the role of physical therapy in pediatric hematology: …...1/31/2018 1 the role of physical therapy...

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1

The Role of Physical Therapy in Pediatric HematologyOncology

More Than Just Lab Values

Kelly (OrsquoMara) Rock PT DPT1

Eena Kapoor PT DPT

Childrenrsquos National Health System

Ashley Braswell PT DPT1

Seattle Childrenrsquos Hospital

1 ABPTS Board Certified Pediatric Clinical Specialist

Introduction

bull Ask the right questions

bull Understand the underlying cause of lab value variations

bull Advocate indications and role of physical therapy

bull Educate patientsfamilies

bull Empower patients to take control over their physical and functional potential

Objectives

1 Describe the epidemiology incidence pathology physiology and clinical manifestation of pediatric hematological disorders in infants children adolescents and young adults including bleeding disorders clotting disorders anemias and autoimmune diseases

2 Recognize common pulmonary neurologic cardiovascular gastrointestinal musculoskeletal hematological and integumentary impairments in individuals with these diseases as they relate to function and participation

3 Identify the medical and physical therapy management and measurements of pain functional outcomes and quality of life in persons living with hematological disorders

4 Discuss the role of physical therapy in management of these patients including potential complications of bone marrow transplantation and pharmacological interventions

Hematopoiesis

bull Blood cell production

httplyceumalgonquincollegecomltsonlineCoursesanatomycontentmodule11-8htm

Cells cells and more cellshellip

bull Red blood cells carry oxygen

bull Platelets help blood to clot

bull Granulocytes phagocyte ndash Neutrophils ingest bacteria

ndash Eosinophils allergic reactions and attack parasites

ndash Basophils release histamine

bull Lymphocytes regulate immune system

ndash T-cell

ndash B-cell

bull Monocytes phagocytic WBC ndash Dendritic antigen-presenting

ndash Macrophages larger phagocyte antigen-presenting (histiocyte)

httplifescidlsrutgersedu~babiarzbloodtxhtm

Clotting Cascade

By Joe D - Own work CC BY-SA 30 httpscommonswikimediaorgwindexphpcurid=1983833

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2

Clotting Cascade

httpbaronerockscomindexphpmnemonicsmnemonics-hematology136-baronemnemonic-clotting-cascade (modified)

Co-factors

intrinsic

extrinsic

(heparin)

(warfarin)

INR

Beyond Lab Values The Role of the Physical Therapist in Bleeding and

Clotting Disorders

Eena Kapoor PT DPT


Learning Objectives

bull Identify multi-system impairments found in bleeding disorders and clotting disorders

bull Discuss evaluation tools to use for patients with bleeding and clotting disorders

bull Reflect on treatment strategies when working with persons with bleeding or clotting disorders to improve quality of life

Bleeding Disorders

bull Hemophilia A

bull Hemophilia B

bull Factor VII deficiency

bull Von Willebrand Disease

bull Glanzmannrsquos Thrombasthenia

What Is Hemophilia

bull Genetics bull Presentation

bull Types

ndash Factor VIII deficiency ndash Hemophilia A

ndash Factor IX deficiency ndash Hemophilia B

bull Severity ndash Mild (5) ndash Moderate (1-4) ndash Severe (lt1)

bull Treatment bull Complications

ndash Inhibitor

Goto 2016 Kuijlaars 2017

Hemophilic Arthropathy

Inactivity

Muscle Atrophy

Joint becomes

Vulnerable

Higher risk of injury

and bleed

Carcao 2015

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3

Joint Bleed Development of Hemophilic Arthropathy

Evaluation of Joint Health

bull Hemophilia Joint Health Score

ndash ROM bull Loss of flexionextension

ndash Swelling bull Duration of swelling

ndash Joint pain

ndash Crepitus on motion

ndash Atrophy

ndash Strength

Intervention Techniques

Joint Preservation PRICE

Alignment

Range of motion

Strengthening

Bone Density

Proprioception

Factor Replacement

Kempton 2015

Fearn 2010

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4

MASAC Guidelines

bull httpswwwhemophiliaorgResearchers-Healthcare-ProvidersMedical-and-Scientific-Advisory-Council-MASACMASAC-RecommendationsMASAC-Recommendations-Regarding-Physical-Therapy-Guidelines-in-Patients-with-Bleeding-Disorders

bull Guidelines for treatment of muscle or joint bleed post surgical

Treatment Ideas

CLOTTING DISORDERS

Epidemiology

bull 183 per 100000 (European ancestry)

bull VTE occurs in estimated 1 to 2 per 1000 persons annually in the United States

bull Increased incidence in African Americans

bull Increased incidence with age

Heit 2016 Yang 2012

Risk Factors

bull High risk neonates

bull Pregnancy

bull Immobility

bull Cardiac disease

bull Inflammatory Diseases

bull Obesity

bull Cancer

bull Central Venous Catheter

bull Oral Contraceptives

bull Inherited Thrombophilia

bull Anatomical Risk Factors

Hillegass 2016

Inherited Thrombophilia

bull Factor V Leiden

bull Antiphospholipid syndrome

bull Protein C deficiency

bull Protein S deficiency

bull Antithrombin deficiency

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5

Thromboembolism

bull Venous ndash CNS

ndash Non-CNS

ndash Assess for Post Thrombotic Syndrome

bull Arterial ndash CNS

ndash Non-CNS

ndash Assess for limb length discrepancy and chronic arterial insufficiency

Anatomical Risk Factors May Thurner Anomoly

Left iliac vein compression from the contralateral right common iliac artery against posterior fifth lumbar

vertebral body

Rajachandran 2014

Anatomical Risk Factors Paget Schroetter Syndrome

bull Thoracic Outlet Syndrome

ndash Axillary-subclavian vein thrombosis

ndash Scalene tendon hypertrophy

ndash Costoclavicular ligament abnormal insertion

Venkata 2010

Image Credit ScienceDirectcom

Medical Management

bull Pharmaceutical Management

ndash Low molecular weight heparin

ndash Vitamin K antagonist

ndash Direct acting anticoagulants (Xarelto)

bull Catheter Directed Thrombolysis

Anticoagulation Therapeutic Levels

Hillegass 2016

To Range or not to Range

bull Is it new or old clot

bull In therapeutic range

bull Risk vs benefit

bull Location of clot- Central vs extremity

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6

Precautions if on Anticoagulation

bull Sports

bull Activity Restrictions

bull Diet

ndash Vitamin K

Post Thrombotic Syndrome

bull Most frequent complication of DVT ndash 30-50 of LE Venous DVT

bull Manifestations of PTS

ndash Chronic leg pain

ndash Edema

ndash Leg ulcers

ndash Dilated superficial collateral veins

ndash Skin discoloration

Kahn 2008 2009 van der Velden 2014

Photo Credit Wisegeekcom

Standardized PTS Evaluation

bull Upper Extremity Girth Measurements

ndash Proximal bull Acromion to elbow crease midpoint

ndash Distal bull Elbow crease to wrist crease midpoint

Evaluation of the Duration of Therapy for Thrombosis in Children (Kids-DOTT) Training Video


bull Lower extremity Girth Measurement

ndash Proximal bull Greater trochanter to superior patellar border midpoint

ndash Distal bull Tibial tuberosity to lateral malleolus midpoint

Evaluation of the Duration of Therapy for Thrombosis in Children (Kids-DOTT)

Training Video

Bone Density with Anti Coagulation

bull Vitamin K participates in bone metabolism and since oral anticoagulants antagonize vitamin K their use may also increase the risk of osteoporosis

bull LMWH for 3ndash6 months may not increase the risk of fractures but longer exposure for up to 24 months may adversely affect BMD

Gajic-Veljanoski O et al

Wawrzyńska L1 et al

Physical Therapy Intervention

bull Patient Education

bull Screen for Recurrent VTE

bull Graduated Compression Garment

bull Muscle pump

bull Positioning

bull Fall Risk Assessment

bull Hydration

bull Aquatics

Hillegass 2016

Manco-Johnson l 2006

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7

Clinical Application

bull Outpatient referral indicated if

ndash Pain

ndash Signs of PTS

ndash Decline from PLOF

Case Study

bull 17 yo boy with history of R hemi-hypertrophy RLE DVT psoriasis and complex mental health issues

bull Anticoagulation daily Coumadin

bull Impairments ndash decreased B LE ROM

ndash decreased flexibility of B LE

ndash gait deviations

ndash increased RLE girth (proximal only)

ndash skin discoloration R LE

PTS Assessment

bull Midpoint Girth Measurements of LE

bull January 2017 ndash R LE Distal 415 cm 60 cm proximal ndash L LE Distal 385 cm 56 cm proximal

bull July 2017 ndash R LE Distal 375 cm 62 cm proximal ndash L LE Distal 355 cm 5525 cm proximal bull January 31 2018

bull Distally 3 cm difference R gt L bull Proximally 10 cm difference R gt L

Case Study PT recommendation

bull Graduated Compression Stocking

bull Outpatient PT

bull Aquatics

bull HEP

Take Home Message

bull Perform multi-system assessment of patients with bleeding and clotting disorders

bull Encourage early mobility with use of graduated compression for VTE

bull Collaborate with interdisciplinary team to provide comprehensive care to these complex patient populations

bull NHF PT Scholarship

bull Questions

ndash ekapoorchildrensnationalorg

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8

References Bleeding and Clotting Disorders bull Anderson I Compression bandaging in patients with venous insufficiency Nurs Stand 2008 Nov 12-1823(10)49-55

bull Carcao Manuel Pamela Hilliard Miguel A Escobar Luigi Solimeno Johnny Mahlangu and Elena Santagostino ldquoOptimising musculoskeletal care for patients with haemophiliardquo European journal of haematology 201595( S81) 11-21

bull Cuesta-Barriuso Ruben Antonia Goacutemez-Conesa and Joseacute Antonio Loacutepez-Pina ldquoPhysiotherapy Treatment in Patients with Hemophilia and Chronic Ankle Arthropathy A Systematic Reviewrdquo Rehabilitation Research and Practice 213 vol 2013 Article ID 305249 10 pages doi1011552013305249

bull FEARN M HILL K WILLIAMS S MUDGE L WALSH C McCARTHY P WALSH M and STREET A) Balance dysfunction in adults with haemophilia Haemophilia 2010 16 606ndash614 doi101111j1365-2516201002200x

bull Flora Peyvandi Prof Isabella Garagiola PhD Guy Young MD The past and future of haemophilia diagnosis treatments and its complications Lancet The 2016 Volume 388 Issue 10040 Pages 187-197 Copyright copy 2016 Elsevier Ltd

bull Gajic-Veljanoski O Phua C Shah P Cheung A Effects of Long-Term Low-Molecular-Weight Heparin on Fractures and Bone Density in Non-Pregnant Adults A Systematic Review With Meta-Analysis Journal Of General Internal Medicine [serial online] 201631(8)947-957 Available from MEDLINE Complete Ipswich MA Accessed January 15 2018

bull Goto M Takedani H Yokota K Haga N Strategies to encourage physical activity in patients with hemophilia to improve quality of life Journal of Blood Medicine 2016785-98 doi102147JBMS84848

bull Heit JA Spencer FA White RH The epidemiology of venous thromboembolism Journal of Thrombosis and Thrombolysis 2016413-14 doi101007s11239-015-1311-6

bull Hillegass Ellen Michael Puthoff Ethel M Frese Mary Thigpen Dennis C Sobush Beth Auten Role of Physical Therapists in the Management of Individuals at Risk for or Diagnosed With Venous Thromboembolism Evidence-Based Clinical Practice Guideline Physical Therapy 2016 Volume 96 Issue 2 Pages 143ndash166 httpsdoiorg102522ptj20150264

bull Kahn Susan R How I treat post thrombotic syndrome BLOOD 2009 19 VOLUME 114 NUMBER 21

bull Kahn SR Shrier I Julian JA et al Determinants and time course of the postthrombotic syndrome after acute deep venous thrombosis Ann Intern Med 2008149698ndash707

bull Kempton Bone health in persons with haemophilia Haemophilia The Official Journal of the World Federation of Haemophilia 2015 Volume 21 Issue 5 SSN 1351-8216 Online ISSN 1365-2516

References Bleeding and Clotting Disorders bull Kuijlaars IAR12 Timmer MA123 de Kleijn P13 Pisters MF234 Fischer K1 Monitoring joint health in haemophilia Factors associated

with deterioration Haemophilia 2017 Nov23(6)934-940 doi 101111hae13327 Epub 2017 Sep 5

bull Manco-Johnson M J Postthrombotic Syndrome in Children Acta Haematol 2006115207-213

bull Pradeep M Poonnoose1 Janjaap van der Net Activity USAMusculoskeletal Outcome in Hemophilia Bleeds Joint Structure and Function Aand Health-Related Fitness Semin Thromb Hemost 2015 41(08) 872-879 DOI 101055s-0034-1543997

bull Rajachandran M et al Diagnosis and Treatment of May-Thurner Syndrome Vascular Disease Management 2014 11(11)E265-E272

bull Revel-Vilk S Branda o LR Journeycake J Goldenberg A Monagle P Sharathkumar A Chan AKC on behalf of the Perinatal and Paediatric Haemostasis Subcommittee of the Scientific and

Standardization Committee of the International Society on Thrombosis and Haemostasis Standardization of post-thrombotic syndrome definition and outcome assessment following upper venous

system thrombosis in pediatric practice J Thromb Haemost 2012 10 2182ndash5

bull Strike K1 Mulder K2 Michael R3Exercise for haemophilia Cochrane Database Syst Rev 2016 Dec 1912CD011180 doi 10100214651858CD011180pub2

bull Wang M Aacutelvarez-Romaacuten MT Chowdary P Quon DV Schafer K Physical activity in individuals with haemophilia and experience with recombinant factor VIII Fc fusion protein and recombinant factor IX Fc fusion protein for the treatment of active patients a literature review and case reports Blood Coagulation amp Fibrinolysis 201627(7)737-744 doi101097MBC0000000000000565

bull Wyseure T Mosnier LO von Drygalski A Advances and Challenges in Hemophilic Arthropathy In Seminars in Hematology 2016 Volume 53 Issue 1 Pages 10-19 ISSN 0037-1963 doi101053jseminhematol201510005

bull van der Velden S Neumann H The post-thrombotic syndrome and compression therapy Phlebology 201429(1 suppl)83ndash89

bull Venkata M Alla MD Et al Paget-Schroetter Syndrome Review of Pathogenesis and Treatment of Effort Thrombosis West J Emerg Med 2010 Sep 11(4) 358-362

bull Wawrzyńska L1 Tomkowski WZ Przedlacki J Hajduk B Torbicki A Changes in bone density during long-term administration of low-molecular-weight heparins or acenocoumarol for secondary prophylaxis of venous thromboembolism Pathophysiol Haemost Thromb 2003 Mar-Apr33(2)64-7

bull Yang G De Staercke C Hooper WC The effects of obesity on venous thromboembolism A review Open journal of preventive medicine 20122(4)499-509 doi104236ojpm201224069

Beyond Lab Values The Role of the Physical Therapist in Anemias and

Autoimmune Diseases

Kelly (OrsquoMara) Rock PT DPT ABPTS Board Certified Pediatric Clinical Specialist


httpsipinimgcom736x77f28c77f28cc1d9a254e98a6fbc8d1883494e--science-humour-biology-humorjpg

httptheawkwardyeticomwp-contentuploads201401Vesselpng

Red Blood Cell Disorders

bull Anemia bull Hemoglobinopathies

ndash sickle cell disease ndash thalassemia

bull Hemolytic anemia bull Red cell enzyme deficiencies (eg G6PD) bull Red cell membrane disorders (eg hereditary spherocytosis) bull Nutritional anemias (eg iron deficiency anemia and folate

deficiency) bull Disorders of heme production (eg sideroblastic anemia) bull Polycythemia (too many red blood cells) bull Hemochromatosis

httpsmyhealthalbertacahealthhealthy-livingPagesconditionsaspxhwid=tp10337

Anemia

Anemia

bull Blood loss bull Bone marrow malignancies

ndash Leukemia lymphoma multiple myeloma

bull Chemotherapies ndash Platinum (cisplatin carboplatin)

bull Radiation bull Nutritional deficiencies

ndash Iron B12 folic acid

bull Hemolytic Anemia ndash Red blood cells destroyed faster than being made ndash Transfusion reaction immune system destroys transfused cells

bull Red blood cell disorders

httpswwwcancernetnavigating-cancer-careside-effectsanemia

Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

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9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia

bull LightheadednessDizziness

bull Headaches

bull Difficulty concentrating

bull Chills

bull Shortness of breath

bull Weakness

bull Yellowing eyesskin

httpswwwtop10homeremediescomwp-contentuploads201408anemia-symptomsjpg

Chronic

Anemia Aplastic anemia Leukemia malignancy

Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute

Anemia Post Op Blood loss

2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease

bull Most common genetically-inherited condition

bull Hemoglobin(Hb) S and C - sickle

bull 1350 African American newborns

bull HbSS homozygous= 65

bull HbSC heterozygous 35 (less severe)

bull Many states with newborn screen

Wang 2007

httpcells4lifecomwp-contentuploads201602sicklecell_185810981jpg

Sickle Cell Anemia

bull Lack flexibility needed to transverse circulation

bull Fragile and shortened life span (hemolytic anemia)

bull Increased adhesiveness to vascular endothelium

bull Vaso-occulsion in small blood vessels

bull Local ischemia results in painful ldquocrisesrdquo

bull Chronic damage to organstissues

bull Inflammatory cascade ndash further tissue damage

bull Low hemoglobin average 8gmdl

Wang 2007

httpwwwblackvoicenewscomwp-contentuploads201606Sickle_Cell_Anemiapng

Sickle Cell Anemia

bull Pain ndash Acute Vaso-occulsive crisis (VOC) or vaso-occulsive events (VOE)

ndash Chronic

ndash Acute on Chronic

bull Musculoskeletal ndash Avascular Necrosis (AVN)

ndash Osteomyelitis

bull Pulmonary ndash Acute Chest Syndrome (ACS)

ndash Asthma

ndash Pulmonary Hypertension

ndash Chronic Lung Disease

bull Central Nervous System ndash Stroke

ndash Transient ischemic attack (TIA)

ndash Silent cerebral infarct

ndash Diminished neuropsychological test performance (Wang 2007)

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10

Pain

bull Vaso-Occlusive Crisis (VOC) or Events (VOE)

bull ldquoUnpredictable and relentlessrdquo

bull May be provoked by

ndash extremes or changes of temperature

ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation

ndash no identified precipitating cause

Elander et al 1996 Yaster et al 200o

Acute Pain

bull ldquoBone painrdquo

bull Younger children ndash hands feet fingers toes ldquodactylisisrdquo (hand-foot syndrome)

bull Adolescents ndash abdominal chest low back

Yaster 200o

httphealthfixitcomwp-contentuploads201706Dactylitis-in-infant-with-sickle-cell-diseasephototjpg

Pain ndash Admissions

bull 5 of patients account for 30 of hospitalizations

bull ldquoRevolving doorrdquo ndash frequent admissions

bull Up to 10 or more hospitalizations a year

bull Earlier death

bull Pain intensity scores remain elevated during hospitalization in youth with SCD

bull Physical function improves over the course of hospital stay in youth

Yaster et al 200o Platt et al 1991 Zempsky et al 2008 Zempksy et al 2012

Acute Care - Pain Management

Goals

bull Decrease Pain

ndash Visual Analog Scale(VAS) MCID 097thinspcm

ndash Numeric Pain Rating Scale(NPRS) MCID 09

ndash gt745thinspcm (VAS) or 75 (NPRS) need for pain medication

bull Vasodilation

ndash Heat and exercise

Myrvik et 2013 Zempsky et al 2008 Zempksy et al 2012

Chronic Pain

bull May be a result of

ndash avascular necrosis

ndash vertebral collapse

ndash chronic leg ulceration

ndash chronic low back pain

bull Goals of management improve functional capacities while decreasing pain

Yaster et al 200o

Pain Management

Cognitive Therapies ndash Psychological

bull Distraction guided imagery hypnotherapy education and psychotherapy

ndash Behavioral bull Relaxation biofeedback behavioral modification and deep

breathing

Physical Therapies ndash Physical therapy hydration heat massage

transcutaneous electrical nerve stimulation (TENS) acupuncture

bull Limited publications

Yaster et al 200o

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11

Avascular Necrosis (AVN)

bull As many as 50 have AVN or osteonecrosis ndash Temporary or permanent loss of blood supply to bones

bull head of femur

bull head of humerus - Equal occurrence ndash 2-17

- Combo occurring in 8 out of 10

bull temporomandibular vertebral bodies

bull Collapse of femoral head 90 of patient within

5 years after diagnosis

bull Decreased range of motion (ROM) pain with ROM or weight bearing

Martiacute-Carvajal et al 2016 Neumayr et al 2006

Avascular Necrosis

Treatment

bull Non-surgical ndash Observation analgesics limited weight bearing (WB)

ndash Physical Therapy

bull Surgical ndash Joint reconstruction

ndash Nucleus decompression

ndash Bone graft

ndash Vascularized bone graft

ndash Electrical Stimulation

ndash Osteotomy

Martiacute-Carvajal et al 2016

Avascular Necrosis ndash PT plusmn Surgery (Neumayr et al)

Randomized Control Trial bull 420 participants (32 centers June 1998 ndash June 2002)

ndash 176 met inclusion criterion ndash 46 enrolled in study ndash Mean age 26 ndash If bilateral more involved hip was included

bull Intervention (17 of 23) hip core decompression and physical therapy bull Control (21 of 23) physical therapy alone bull Matched Steinberg staging system bull Outcomes

ndash Childrenrsquos Hospital Oakland Hip Evaluation Scale (CHOHES) score ndash Hip survival


AVNndash PT plusmn Surgery (continued)

bull Childrenrsquos Hospital Oakland Hip Evaluation Scale (CHOHES) score ndash Average follow-up 27 months ndash Intervention 18 pt (181 +- 198) ndash Control 15 pt (157 +- 196) ndash RR 095 (95 CI 058 to 160)

Complications bull Intervention

ndash 2 re-admissions bilateral hip pain (lt30 days post-op) ndash 1 post-op infection ndash 6 required additional surgical intervention ndash 3 required total hip replacement

bull Control ndash 3 hip core decompression during the study

bull No significant difference between VOC or ACS


3 year ndash Follow-up Study

bull Control (PT only)

ndash Hip survival 86

ndash Harris hip score 157 mean improvement

bull Intervention (PT + surgery)



ldquoPhysical therapy alone appears to be just as effective as core decompression plus physical therapy in improving hip function and postponing the need for additional surgical interventionsrdquo

Neumayr et al 2006

Pulmonary

bull Accounts for 27 of pediatric SCD hospitalizations

bull Acute Chest Syndrome (ACS)

bull Pulmonary Hypertension

bull Asthma

bull Chronic Lung Disease

Ahmad et al 2011 Steiner 2006

1312018

12

Acute Chest Syndrome (ACS)

bull Second most common cause of hospitalization

bull Most common post-operative complication

bull Develops in as high as 50 of admitted patients

bull A leading cause of death

Ahmad et al 2011 Khoury et al 2011 Vichinsky et al 2000

Myers et al 2012 Driscoll 2007

Acute Chest Syndrome

bull Multifactorial

ndash Infection

ndash Fat embolization from bone marrow

ndash Sequestration of sickled red blood cells bull Direct lung infarction ldquowedge-shapedrdquo

bull Back pain ACS risk factor

Ahmad et al 2011 Khoury et al 2011 Vichinksy et al 1994

Pulmonary Hypertension

bull 11-31 children and adolescents

bull Decline in exercise capacity

Khoury et al 2011 Dhoui et al 2010 Minniti et al 2009 Gordeuk et al 2011

Asthma

bull Incidence 168-53

bull ACS 2x more episodes

bull 2x higher mortality

Khoury et al 2011 Boyd et al 2006 Boyd et al 2007

Chronic Lung Disease

bull Recurrent ACS Adults

ndash 74 restrictive

ndash 10 normal

bull Obstructive pattern might start in infancy

Khoury et al 2011 Klings et al 2006 Koumbourlis et al 2007

Pulmonary Intervention

bull Incentive Spirometry

ndash Ahmad (2011) mandatory IS for non-pulmonary admissions

bull Decreased requirements oxygen antibiotics and blood transfusions

bull PEP

bull Blowing bubbles

bull AmbulationActivity

bull Education

1312018

13

Exercise Assessment

bull VO2 max ndash Decreased (less than 80 predicted)

bull Anaerobic threshold (AT) ndash Less than predicted

bull Low O2 pulse (rate of VO2 to heart rate = mimic stroke volume) bull Low VO2-work bull No mechanical ventilation impairments

Reasons bull Anemia

ndash Low peak VO2 low AT low O2 pulse elevated heart rate for level of work increased respiratory equivalent of CO2

bull Pulmonary vascular disease ndash Gas exchange abnormalities

bull Peripheral vascular disease andor myopathy ndash High heart rate reserve

Connes et al 2011

Exercise - Testing

bull 6 minute walk test

ndash Pulmonary hypertension

ndash Pulmonary or peripheral vascular disease (severe SCA)

bull Symptom-limited cardiopulmonary exercise testing (CPET)

ndash Pulmonary or peripheral vascular disease bull severe SCA- less than AT


ndash Heart disease (with EKG)

ndash Bronchial hyper-reactivity (with spirometry)

Connes et al 2011

Exercise Prescription

bull Moderate exercise (50 of maximal aerobic power) of 20 minutes did not cause marked hematological alterations (Balayssac-Syransy et al 2011)

bull Exercise therapy may contribute to a reduction in the length of hospitalization in sickle cell anemia children with painful VOC

(Alcorn et al 1984) ndash Moderate strength and endurance exercise 10-30 minutes duration and included recreational

gymnastics stationary bike riding and games

bull Decreased pain increased respiratory muscles strength improved quality of life (Tinti et al 2010)

ndash Kinesiology and aquatic rehabilitation 2xweek for 5 weeks (warm water exercise stretching aerobic exercise and relaxation)

bull Regular exercise at moderate intensity could decrease the risk of inflammatory reaction related to exercise and could increased exercise intensity (Barbeau et al 2001)

ndash Used 60-75 of predicted maximum bull AT of SCA ranges between 35-60 VO2max (10 24 exercise)


bull Start exercise gradually

bull Avoid intense exercise

bull Stop exercising at first sign of fatigue

bull Should not practice during illness

bull Avoid prolonged exercise for more than 20 minutes without rest

bull Drink water during and after exercise

bull Outdoors prevent cold and heat stress

bull Intensity of exercise should be at or below anaerobic threshold (AT)

Connes et al 2011

Central Nervous System

bull Stroke

bull Transient ischemic attack (TIA)

bull ldquoSilentrdquo cerebral infarct

Wang 2007

Stroke

bull Children with SCD 500x more likely to have stroke

ndash 11 in first 2 decades of life

ndash Peak incidence 1st stroke 2-5 years old and 6-9 years old

ndash Great risk in HbSS than HbSC (51)

bull Ischemic ndash majority

bull Hemorrhagic ndash 38

ndash Moyamoya ndashcollateral circulation related to obstructed anterior portion of Circle of Willis

Wang 2007 Ohene-Frampong et al 1998

1312018

14

CNS - Medical diagnosis

bull Risk factors

ndash Previous TIA

ndash Recent or recurrent ACS

ndash Increased blood pressure

ndash Overnight oxygen saturation

ndash Hemoglobin (low-steady)

ndash High leukocytes

ndash High platelets


PT Management

bull Detailed sensorymotor exam

bull Neurological rehabilitation

bull Recognize decreased neuropsychological performance

Hydroxyurea (HU)

bull S phase-specific chemotherapeutic agent ndash Increased Hb F

ndash Decrease adhesion of sickle cells to endothelium

ndash Myelosuppressive decreased WBC and likely number of adherent leukocytes

bull Decreased

ndash painful crises

ndash ACS bull BABY HUG (73 reduction)

ndash transfusion requirements

ndash hospitalization

bull Increased ndash Survival

ndash Anaerobic and aerobic exercise capacity

bull Compliance is an issue

Frenette et al 2007 Connes et al 2011 Hackney et al 1996 Platt et al 1991 https4imimgcomdata4FBCEMY-2745020hydroxyurea-capsules-250x250jpeg

Outcome Measurements

bull Youth Acute Pain Functional Ability Questionnaire (YAPFAQ) ndash Self-report measure of physical function in youth experiencing acute pain ndash Measures of physical activity pain impact pain location and quality of life ndash Pain intensity

bull Childrenrsquos Hospital Oakland Hip Evaluation Scale (CHOHES) bull Functional Independence Measure (FIM) bull Sickle Cell Pain Burden Interview Youth (SCPBI-Y) bull Functional Disability Inventory (FDI) bull Child Activities Limitations Interview (CALI) bull Pediatric Quality of Life Inventory (PedsQL)

ndash Acute ndash Sickle Cell (pain)

bull Pain ndash Adolescent Pediatric Pain Tool (APPT) ndash Visual Analogue Scale (VAS) ndash Numeric Pain Rating Scale (NPRS)

SCD PT Summary

bull Pain ndash Patient-centered Hot packs whirlpool TENS relaxationmassage deep

breathing distraction impairment-specific exercise

bull Musculoskeletal ndash AVN screening and intervention

bull Pulmonary ndash Incentive spirometry ndash Bubbles PEP ndash Mobility

bull Exercise Tolerance ndash Exercise screening and prescription ndash Outpatient services and monitoring

bull Neurological ndash Stroke screening and intervention

Early Detection and Prevention

Integrated Sickle Cell Clinic Childrenrsquos National bull Hematology Providers bull Pain Medicine bull Palliative Medicine bull Psychology bull Physical Therapy bull Social Work

Goal Comprehensive management and prevention of chronic pain in childrenadolescents with chronic pain risk factors

1312018

15

Thalassemia

Thalassemia

bull Italian Greek Middle Eastern South Asian and African descent

bull Alpha Thalassemia

bull Beta Thalassemia

bull Major or Minor

bull Hemoglobin S associated with Beta thalassemia

bull Anemia

httpsghrnlmnihgovartlargethalassemia-red-blood-cellsjpeg

Medical Management

bull Regular blood transfusions

bull Iron chelation

ndash Deferoxamine prevent iron accumulation

bull Folic acid

bull Hematopoietic stem cell transplantation (HSCT)

Lal 2016

Thalassemia

bull Thin brittle bones bull Scoliosis

ndash More common in preadolescents with thalassemia ndash Unknown

bull Bone marrow expansion bull Delayed puberty bull Deferoxamine prevent iron accumulation

ndash metaphyseal changes long bones slow growth ndash Worse when started before age 2 or high dosage

bull Pain (last 7 days) ndash Brief Pain Inventory (BPI) ndash 12-17y 8 ndash 25-34y 36 ndash Greater than 35y 56-58 ndash Low back (common) legs head hips upper back

Lal 2016

Autoimmune Diseases

Autoimmune Diseases

bull Hemophagocytic lymphohistiocytosis (HLH)

bull Lupus

bull Severe Combined Immunodeficiency (SCID)

bull Immune thrombocytopenia (ITP)

bull Autoimmune hemolytic anemia

bull Evans syndrome

1312018

16

Hemophagocytic Lymphohistiocytosis

(HLH)

Hemophagocytic Lymphohistiocytosis (HLH)

bull Disordered immune regulation

bull Cytokine storm

ndash Macrophages

ndash T-Lymphocytes bull Natural Killer T cells (NK-cells)

bull Cytotoxic T-lymphocytes

bull Severe cytopenias

bull CNS involvement -inflammatory

George 2014

httpsiytimgcomviVxoy5328MZcmaxresdefaultjpg

HLH

bull 1939 ndash Scott and Robb-Smith first described

bull 1983 ndash long-term survival 4 median survival untreated lt2 months

bull Children (under 18) = 1 in 100000

George 2014 Niece et al 2010


bull PrimaryFamilial (25)

ndash Genetic abnormality causing the disorder

bull Secondary

ndash Infection most common

ndash Malignancy bull acute B-lymphoblastic leukemia

ndash Metabolic

bull Macrophage activation syndrome (MAS)

ndash Autoimmune

(George 2014)

HLH diagnosis

bull Molecular diagnosis andor

bull Five of the following 1 Fever

2 Enlarged spleen

3 Cytopenias (2 or more cell lines)

4 High triglyercides

5 Partial deficiency of fibrinogen

6 Elevated ferritin

7 Hemophagocytosis (bone marrowspleenlymph nodes)

8 Low or absent natural killer (NK)-cell activity

9 Elevated soluble CD25 (interleukin-2 receptor)

George 2014

Medical Management

bull Immune-suppressive and modulatory agents ndash Chemotherapy

bull Etoposide bull Intrathecal (IT) methotrexate (CNS disease)

ndash Corticosteroids bull Glucocorticoids dexamethasone prednisone

ndash Cyclosporine (CSA) ndash Immunoglobulins

bull Biological response modifiers ndash Rituximab Epstein-Barr Virus (EBV)

bull Treatment of illness (secondary) ndash Antibiotics


George 2014 Weitzman 2011

1312018

17

Glucocorticoid Steroids

bull Inhibit glucose uptake in skeletal muscle ndash Breakdown of muscle proteins

bull Stimulate protein degradation and inhibit protein synthesis ndash Directly affect protein synthesis

bull Stimulate production of glutamine ndash Amino acid ndash generate glucose in the liver

bull Muscle atrophy ndash catabolic effects

bull Hyperglycemia

LaPier 1997

Muscle Atrophy

bull Insidious bull Painless bull Symmetrical

bull Type II fibers bull Locomotion muscles less susceptible bull Diaphragm can be affected 1 Proximal lower extremity 2 Proximal upper extremity 3 Distal extremities

(LaPier 1997) (Falduto et al 1992)

Figure 1 (LaPier 1997)

Glucocorticoid Steroids ndash Exercise

bull Endurance exercise training

ndash Decrease muscle atrophy predominately in the most highly recruited type I skeletal muscle fibers

bull Strength Fifty days of isokinetic training (3xweek)

ndash Increased thigh muscle area

ndash Decreased thigh fat area

ndash Normalized mean peak torque and total work output

LaPier 1997 Hickson et al 1993 Horber et al 1985)

Chart Review

bull Lab values

ndash Blood glucose levels

ndash Serum creatine kinase (CK)

bull HLH

ndash All cell lines bull Platelets

bull Hemoglobin

LaPier 1997

Exercise

bull Individualized

bull Hydration ndash electrolyte imbalances

bull Monitor signssymptoms of hyperglycemia and hypoglycemia

bull Resistance training

ndash Low weight and high repetitions

bull Avoid repetitive shearing forces (skin breakdown)

bull Monitor blood pressure

LaPier 1997

Posterior reversible encephalopathy syndrome (PRES)

bull Headache bull Altered consciousness bull Visual disturbances bull Seizures

bull MRI vasogenic cerebral edema predominantly in the

posterior cerebral hemispheres

bull High blood pressure bull Most associated cyclosporine

bull Reversible

1312018

18

HLH PT Summary

bull Chart review

ndash Monitor lab value trends

bull Optimize function

bull Adaptindividualize exercise

bull Educate on importance of function

Hematopoietic stem cell transplantation (HSCT)

bull Sickle Cell Disease

bull Thalassemia

bull HLH

bull Other immune diseases

References Anemias and Autoimmune

bull Aguilar CM Neumayr LD Eggleston BE et al Clinical evaluation of avascular necrosis in patients with sickle cell disease Childrenrsquos Hospital Oakland Hip Evaluation Scale ndasha modification of the Harris Hip Score Archives of Physical Medicine and Rehabilitation 200586(7)369-375

bull Ahmad A Macias C Allen J The use of incentive spirometry in pediatric patient with sickle cell disease to reduce the incidence of acute chest syndrome J Pediatr Hematol Oncol 201133(6) 415-420

bull Albers GW Caplan LR Easton JD et al Transient ischemic attack- proposal for new definition New England Journal of Medicine 20023471713-1716

bull Alcorn A Bowser B Henley EJ Holloway V Fluidotherapy and exercise in the management of sickle cell anemia A clinical report Phys Ther 1984641520-1522

bull Balayssac-Syransky EA Connes P Tuo N et al Mild hemorheological changes induced by moderate endurance exercise in patients with sickle cell anemia Br J Haematol 2011154398-407

bull Barbeau P Woods KF Ramsey LT et al Exercise in sickle cell anemia effect on inflammatory and vasoactive mediators Endotheliuem 20018147-155

bull Boyd JH Macklin EA Strunk RC DeBaun MR Asthma is associated with increased mortality in individuals with sickle cell anemia Haematologica 200792(8)1115-1118

bull Boyd JH Macklin EA Strunk RC DeBaun MR Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia Blood 2006108(9)2923-2927

bull Connes P Machado R Hue O Reid H Exercise limitation exercise testing and exercise recommendations in sickle cell anemia Clinical Hemorheology and Microcirculation 201149 152-163

bull Dahoui HA Hayek MN Nietert PJ et al Pulmonary hypertension in children and young adults with sickle cell disease evidence for familial clustering Pediatr Blood Cancer 201054(3)398-402

bull Driscoll MC Sickle cell disease Pediatr Rev 200728259-268 bull Elander J Midence K A review of evidence about factors affecting quality of pain management in sickle cell disease Clin J Pain 199612180-193 bull Embury SH The clinical pathophysiology of sickle cell disease Annu Rev Med 198637361-376 bull Falduto MT Young AP Hickson RC Interruption of ongoing glucocorticoid-induced muscle atrophy and glutamine synthetase induction by

exercise Med Sci Sports Exerc 199224(suppl)S3 bull Frenette P Atweh G Sickle cell disease Old discoveries new concepts and future promise The Journal of Clinical Investigation 2007 117(4) 850-

858 bull George MR Hemophagocytic lymphohistiocytosis a review of etiologies and management Journal of Blood Medicine 2014569-86 bull Gordeuk VR Minniti CP Nouraie M et al Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in

children and adolescents with sickle cell anemia Haematologica 201196(1)33-40


bull Hackney AC Hezier W Gulledge TP et al Effects of hydroyurea administration on the body weight body composition and exercise performance of patients with sickle-cell anaemia Clin Sci (Lond) 199792481-486

bull Hickson RC Marone JR Exercise and inhibition of glucocorticoid-induced muscle atrophy Exerc Sports Sci Rev 199321135-167 bull Horber FF et al Evidence that prednisone- induced myopathy is reversed by physical training Journal of Clinical Endocrinology amp Metabolism

19856183-88 bull Khoury RA Musallam KM Mroueh S Abboud MR Pulmonary complications of sickle cell disease Hemoglobin 201135(5)625-635 bull Klings ES Wyszynski DF Nolen VG Steinberg MH Abnormal pulmonary function in adults with sickle cell anemia Am J Respir Crit Care Med

2006173(11)1264-1269 bull Koumbourlis AC Lee DJ Lee A Longitudinal changes in lung function and somatic growth in children with sickle cell disease Pedatri Pulmonol

200742(6)483-488 bull Lal A Assessment and treatment of pain in thalessemia Ann NY Acad Sci 2016136865-72 bull LaPier TK Glucocorticoid-induced muscle atrophy The role of exercise in the treatment and prevention Journal of Cardiopulmonary Rehabilitation

MarchApril 1997 17(2)76-84 bull Marti-Carvajal AJ Sola I Agreda-Perez LH Treatment for avascular necrosis of bone in people with sickle cell disease Cochrane Database of

Systematic Reviews 20147CD004344 bull Minniti CP Sable C Campbell A et al Elevated tricuspid regurgitant jet velocity in children and adolscents with sicke cell disease association with

hemolysis and hemoglobin oxygen desaturation Haematologicia 100994(3)340-347 bull Moser FG Miller ST Bello JA et al The spectrum of brain MR abnormalities in sickle-cell disease A report from the Cooperative Study of Sickle Cell

Disease American Journal of Neuroradiology 199617965-972 bull Myers M Eckes EJ A novel approach to pain management in person with sickle cell disease Medsurg Nurs 2012 Sep-Oct21(5)293-298) bull Myrvik MP Brandow AM Drendel AL Clinically meaningful measurement of pain in children with sickle cell disease Pediatr Blood Cancer

201360(10)1689-1695 bull Neumayr LD Aguilar C Earles AN et al Physical therapy alone compared with core decompression and physical therapy for femoral head

osteonecrosis in sickle cell disease results of a multicenter study at a mean of three years after treatment The Journal of bone and joint surgery 2006 Dec 88( 12)2573-2582

bull Niece JA Rogers ZR Ahmad N Langevin AM McClain KL Hemophagocytic lymphohistiocytosis in Texas observations on ethnicity and race Pediatr Blood Cancer 201054(3)424-428

bull Noguchi CT Schechter AN Rodgers GP Sickle cell disease pathophysiology Baillierersquos Clin Haematol 19936(1)57-91 bull Ohene-Frempong K Weiner SJ Sleeper LA et al Cerebrovascular accidents in sickle cell disease rates and risk factors Blood 199891288-294 bull Platt OS Thorington BD Brambilla DJ et al Pain in sickle cell disease Rates and risk factors N Engl J Med 199132511-16 bull Short KR Nygren J Bigelow ML et al Effect of short-term prednisone use on blood flow muscle protein metabolism and function Journal of

Clinical Endocrinology amp Metabolism December 2004 89(12)6198-6207


bull Tinti G Somera R Valente FM Domingos CR Benefits of kinesiotherapy and aquatic rehabilitation on sickle cell anemia a case report Genet Mol Res 20109360-264

bull Vichinsky EP Neumayr LD Earles AN et al Causes and outcomes of the acute chest syndrome in sickle cell disease National Acute Chest Syndrome Study Group N Engl J Med 2000342(25)1855-1865

bull Vichinsky EP Williams R Das M et al Pulmonary fat embolism a distinct cause of severe acute chest syndrome in sickle cell anemia Blood 199483(11)3107-3112

bull Wang W Central nervous system complications of sickle cell disease in children an overview Child Neuropsychology 200713103-109

bull Weitzman S Approach to hemophagocytic syndromes Hematology Am Soc Hematol Edu Program 20112011178-183

bull Yaster M Kost-Byerly S Maxwell LG The management of pain in sickle cell disease Pediatr Clin North Am 2000 Jun47(3)699-710

bull Zempsky WT Loiselle KA McKay K Blake GL Hagstrom NJ Schechter NL Kain ZN Retrospective evaluation of pain assessment and treatment for acute vasoocclusive episodes in children with sickle cell disease Pediatr Blood Cancer 200851265-268

bull Zempsky WT OrsquoHara EA Santanelli JP et al Development and Validation of the Youth Acute Pain Functional Ability Questionnaire (YAPFAQ) The journal of pain  official journal of the American Pain Society 201415(12)1319-1327

bull Zempsky WT Palermo TM Corsi JM Lewandowski AS Zhou C Casella JF Daily changes in pain mood and physical function in children hospitalized for sickle cell pain Pain Res Mana 20121833-38

bull Zhang L Zhou J Sokol L Hereditary and acquired hemophagocytic lymphohistiocytosis Cancer Control October 2014 21(4)301-312

Hematopoietic Stem Cell Transplant (HSCT)

httpasctreatmentcomauwhat-are-

stem-cells

Ashley Braswell PT DPT ABPTS Board Certified Pediatric Clinical

Specialist

February 22 2018

1312018

19

HSCT or HCT

bull Intravenous (IV) infusion of autologous or allogeneic (donor) stem cells in order to restore inadequate bone marrow or immune system bull Autologous not used to treat hematological disorders

httpsemedicinemedscapecomarticle20

8954-overview

Donor Types

bull Matched sibling donor bull Matched unrelated donor (MUD) bull Mismatched unrelated donor bull Single or double umbilical cord bull Haploidentical donor


8954-overview

Source of stem cells

bull Bone marrow bull Peripheral blood bull Umbilical cord


8954-overview

Diagnoses

bull Bone Marrow FailureDysfunction bull Aplastic anemia bull Fanconi anemia bull Shwachman-Diamond syndrome bull Diamond-Blackfan anemia

all have an increased likelihood to develop myelodysplastic syndrome or leukemia (usually acute myeloid leukemia)

httpsemedicinemedsc

apecom

Diagnoses

bull Blood Disorders bull Beta Thalassemia

bull Decreased production of hemoglobin

bull Sickle Cell Anemia bull Abnormal hemoglobin

httpsemedicinemedsc

apecom

Diagnoses

bull Immunodeficiency bull Severe combined immunodeficiency (SCID)

bull Absence of T-lymphocyte and B lymphocyte function

bull Severe congenital neutropenia bull Hemophagocytic lymphohistiocytosis (HLH)

bull Too many immune cells (macrophages and lymphocytes)

bull Wiskott-Aldrich Syndrome

httpsemedicinemedsc

apecom

1312018

20

Pre-transplant testing

bull History and physical exam bull Liver function electrolyte and creatinine bull Viral

bull cytomegalovirus (CMV) herpes virus HIV anti-HIV antibodies hepatitis B and C syphilis human T-cell lymphotropic virus-1 and 2 (HTLV-III)

bull ABO blood typing bull Human leukocyte antigen (HLA) typing


54-overviewa1


bull Chest radiography bull Electrocardiography (ECG) Echocardiogram bull Pulmonary function tests


54-overviewa1


bull PhysicalOccupational Therapy Evaluation bull Functional mobility bull Range of Motion bull Strength bull Endurance bull Familypatient education bull ADL routine

Performance Evaluation

bull Scales used to measure functional capacity bull Found to predict survival in patients with cancer bull Used as entry criteria for clinical trials

Lee amp Smith

2017

httpswwwcibmtrorgDataManagementTrainingReferenc

eManualsDataManagementDocumentsappendix-lpdf

Eastern Cooperative Oncology Group (ECOG Zubrod World Health Organization) performance scale

Performa

nce

Status

Definition

0 Fully active no performance restrictions

1 Strenuous physical activity restricted fully

ambulatory and able to carry out light work

2 Capable of all self-care but unable to carry out

any work activities Up and about gt50 of

waking hours

3 Capable of only limited self-care confined to bed

or chair gt50 of waking hours

4 Completely disabled cannot carry out any self-

care totally confined to bed or chair

Lee amp Smith

2017

Excerpted from Oken MM Creech RH Tormey DC et al Toxicity and response criteria of the

Eastern Cooperative Oncology Group Am J Clin Oncol 1982 5649

1312018

21

PreparativeConditioning Regimens

bull Myeloablative bull Can include total body irradiation bull Cause immunosuppression for engraftment

bull Nonmyeloablative bull Used for graft-versus-tumor effect which is helpful for cancers bull Decreased toxicity

httpsemedicinemeds

capecomarticle208954-overview


bull Reduced Intensity Regimens bull Can be myeloablative or nonmyeloablative bull Reduced acute and chronic toxicities bull Onset of graft-versus-host disease (GVHD) occurs later bull Incidence of GVHD is similar to myeloablative

httpsemedicineme

dscapecomarticle208954-overview

Acute complications

bull Mucositis bull Infection bull Prolonged severe pancytopenia bull Acute Graft-versus-host disease (aGVHD) bull Graft failure bull Hepatic veno-occlusive disease (VOD) or sinusoidal obstructive

syndrome (SOS) bull Thrombotic microangiopathy (TMA)

httpsemedicinemedscapecomarti

cle208954-overviewa1

httpsemedicinemedscapecomar

ticle1079570-overview

Chronic complications

bull Chronic graft-versus-host disease (cGVHD) bull Eyes bull Endocrine bull Immune bull Pulmonary bull Musculoskeletal bull Neurological



Infection Prophylaxis

bull Hand hygiene HEPA-filtered positive-air-pressure sealed rooms bull Facility dependent isolation

bull Antifungal bull fluconazole or amphotericin Bvorixonazole

bull Antibacterial prophylaxis bull fluoroquinolone




bull Antiviral bull Acyclovir ndash herpes simplex positive patients bull Ganciclovir intravenous immunoglobulin (IVIg) and CMV negative

blood products for CMV negative patients bull Pneumocystis prophylaxis

bull trimethoprim-sulfamethoxazolepentamidine bull Gastrointestinal prophylaxis

bull metronidazolefluoroquinolones bull Hepatitis B vaccine for all negative patients



1312018

22

Typical Timeline

httpsafepharmacysh

opsu

Acute graft-versus-host disease (aGVHD)

bull Multisystem complication bull Donor immune cells recognize recipient cells as foreign initiating an

immune reaction causing disease bull Occurs in allogeneic transplants bull Pathophysiology

bull Tissue injury and up-regulation of inflammatory cytokines with T-cell alloreactivity (Filipovich 2008)

Chao

2017

aGVHD definition

bull lsquoClassicrsquo within 100 days with symptoms of maculopapular rash nausea vomiting diarrhea ileus anorexia and cholestatic hepatitis (rising serum bilirubin concentration)

bull Persistent recurrent or late onset aGVHD without diagnostic manifestations of chronic GVHD (cGHVD) beyond +100 days bull Often occurs during withdrawal of immune suppression

bull Skin gastrointestinal tract or liver abnormalities are classified as aGVHD regardless of time

Chao

2017

aGVDH

bull Exact incidence is unknown bull 9 to 50 in HLA-identical sibling transplants bull Common in matched unrelated and haploidentical transplants

Chao

2017

aGVHD risk factors

bull Female donor to male recipient bull Mismatched or unrelated donor bull Conditioning regimen intensity bull Prophylactic regimen used bull Peripheral blood and bone marrow gt umbilical cord

Chao

2017

aGVHD risk factors continued

bull Lesser established risk bull Recipient age CMV status of recipient and donor donor Epstein-Barr

virus (EBV) status gut decontamination bull Can differ depending on underlying disease bull Incidence and severity can depend on pre-transplant

comorbidities

Chao

2017

1312018

23

Chronic Graft-versus-host disease (cGVHD)

bull Immunoregulatory disorder

bull Features of the following bull Autoimmunity

bull Similar to Sjoren syndrome scleroderma primary biliary cirrhosis bull Immunodeficiency

bull Thymic injury bull Poor T-cell immunoreconstitution

bull Usually develops within 3 years of HSCT bull Major cause of late non-relapse mortality after allogenic HSCT

Filipovich

2008

cGVHD Classification

bull Classic bull At least one diagnosticdistinctive manifestation without features of aGVHD

bull Overlap syndrome bull Chronic and acute features appear together

bull Both can be present at anytime after allogenic HSCT bull Sometimes diagnosis is missed as patients are at home and do not see

their specialty transplant providers but once per year bull Physical therapy implications

Filipovich

2008

Diagnostic clinical signs of cGVHD

bull Skin bull Lichen planus-like features purplish itchy flat top bumps bull Morphea-like features painless discolored patches

bull Mouth bull Hyperkeratotic plaques bull Restriction of opening (sclerosis)

bull Genitalia bull Vaginal scarringstenosis

Filipovich

2008


bull GI bull Esophageal web bull Stricturesstenosis of uppermid third of esophagus

bull Lungs bull Bronchiolitis obliterans dx by biopsy

bull Muscles bull Fasciitis bull Joint stiffnesscontractures (sclerosis)

bull all listed sufficient to diagnosis cGVHD Filipovich

2008

Skin Lichen planus-like

httpsemedicinemedscapeco

marticle1050580-overview

aGVHD grade 2 cGVHD grade 4

Skin Morphea-like features

httpsbethematchclinical

orgpost-transplant-carechronic-gvhdskin

ldquoLocalized patchy

areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24

Mouth Lichen-type features

httpwwwnejmorgdoif

ull101056NEJMicm010397

Distinctive Clinical Manifestation by Test

bull Skin depigmentation bull Nails

bull Dystophy longitudinal splitting bull Scalp

bull New onset scarring alopecia (after recovery) bull Scaling lesions

bull Mouth bull Mucosal atrophy ulcers pseudomembranes

Filipovich

2008


bull Eyes bull New-onset dry gritty painful bull Cicatrical conjunctivitis (inflammationscarring) bull Keratoconjunctivitis sicca (dry eye)

bull Genitalia bull Erosions fissures ulcers

bull Lung bull Bronchiolitis obliterans by PFTsimaging

bull Muscles fascia joints bull myositispolymyositis

Filipovich

2008

Eyes Keratoconjunctivitis sicca

httpsbethematchclinicalorgpost-

transplant-carechronic-gvhdeyes

Transplant Specific Medications

bull Immunosuppression bull Cyclosporine amp Tacrolimus

bull Burningtingling hands and feet

bull Granulocyte-colony stimulating factor (G-CSF) bull Bony pain fatigue

bull Steroids

httpsmedlinepl

usgov

Research disclaimer

bull Most studies do not separate out malignancies and non-malignancies

bull Predominately adult research but some pediatric research exists bull Pilot studies or small number of participants

1312018

25

Role of Physical Therapy

bull During transplant patients have a decline in physical functioning which results in muscle atrophy and a loss of strength (Wolin et al 2010)

bull Mobilization bull Prolonged isolation reinforces immobility (Baumann et al 2009)

bull Endurance bull Quality of Life (QoL)

Strenk et al

2014

Pediatric Program Considerations

bull Supervised interventions versus home based program (limited research)

bull More intense contact on a regular basis in order to achieve benefit

bull If possible perform in treating hospital bull Alleviate parent concerns

Wolin et al

2010

PT Interventions During HSCT

bull Begin during conditioning and continue through discharge bull Supervised low to moderate intensity exercise addressing

bull Aerobic endurance training bull Strength bull Balance bull Coordination bull Flexibility

bull Focus on positively affecting QoL bull Functional mobility transfers walking and stair climbing bull Higher frequency 5x per week is beneficial to maintain physical

performance Strenk et al

2014

Low to Moderate Intensity Exercise Program

bull 30-60 minutes with rest breaks as needed bull Aerobic exercise at 50 to 75 of max heart rate with rate of

perceived exertion (RPE) at 10 to 13 bull Strengtheningstretching ndash RPE of 10 to 13 bull Progressive relaxation exercises at RPE 6 to 9

Strenk et al

2014

Discharge from Hospital Recommendations

bull Supervised scheduled moderate intensity exercise program bull Goal of returning to prior level of function bull Designed to address

bull Fatigue bull Physical function bull QoL

bull Minimum frequency of 2x per week for 6 to 12 weeks bull Moderate intensity defined as

bull Aerobic exercise starting at 50 to 60 and progressing up to 70 or 80 max HR with RPE up to 15 bull Strengtheningstretching at RPE of 10 to 13

Strenk et al 2014

Wolin et al 2010 bull Systematic Review bull Two studies of kids receiving HSCT

bull Pediatric survivors with ALLAML who received HSCT the previous year completed 8 week intervention which showed (San Juan et al 2008)

bull Improvement in muscle strength functional mobility aerobic fitness and QoL bull Aerobic and resistance training program for 3 weeks during inpatient stay

(Chamorro-Vina et al 2010) bull Increased fitness bull Increased body mass bull No negative effects on immune recovery

1312018

26

Wiskemann and Huber 2008

bull Review with meta-analysis bull 609 adults with half autologous and half allogeneic transplants bull During inpatient admission exercise stabilizes physical performance

bull Decreases endurance loss when compared to controls bull During outpatient period aerobic exercise and resistance training

bull Improvements in endurance and strength bull Improved QoL in those who participated in exercise programs

Physical Exercise Interventions vs Treatment Phases potential effects

During HSCT After HSCT

Endurance performance Stabilization

effect

Performance improving

effect

Strength performance Stabilization

effect


effect

Psychosocial parameters

(QoL fatigue)

Improving

effect

Improving effect

Body composition No data

available

(2008)

Improving effect

Immune systemfunction (+) effect on

duration of

recovery

No data available

(2008) Wiskemann and

Huber 2008 Little data available

Physical Exercise Interventions tentative Recommendations

Phase of Medical

Therapy

Type of Exercise

Before HSCT Mixed Exercise (3-5x per

week)

Duration up to 30 min

Intensity Moderate (12-

14 Borg 70-80 max HR

Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise

During HSCT Start with endurance

training (5x per week up

to 7x) adding resistance

training with increased

platelet counts in last third

of hospitalization (2-3x per

week)

Duration 10-15 min up to

30 min

Intensity Moderate (12-14

Borg 70-80 max HR) Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise

After HSCT Mixed Exercise (3-5x per

week)

Duration up to 30 min or

more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011

bull Pilot study of 19 adults undergoing HSCT bull Exercise intervention versus control group bull Intervention included strength training including

concentriceccentric exercises body weight resistance and elastic band resistance if able

bull Assessment included Timed stair climb (functional quad strength) Handgrip strength (dynamometer) Thirty-Second Chair-Stand Test Time Needed to stand up from Bed Rest Exam Fatigue Health Status Perceptions QoL

bull Strength-training group reported less fatigue compared with usual-activity group was only statistically significant result

1312018

27

Baumann et al 2009

bull 64 adults with cancer undergoing allogeneicautologous HSCT bull Controlled randomized study bull Training group (TG) versus control group (CG) bull TG lost 10 strength CG lost 24 bull TG maintained endurance TG decreased bull TG had improved QoL and physical functioning bull CG showed increased fatigue

Baumann et al 2009

Take Home

bull HSCT is a complicated process that is continually changing as new research and medicines are found

bull Physical therapists can be instrumental in stabilizing secondary negative effects of intensive medical treatment and prolonged hospitalizations

bull Research to support supervised exercise program improving QoL and fatigue

Resources

bull Medscape bull Overview with specifics

bull Bethematchclinicalorg bull Clinicians resource

bull UpToDate bull Cincinnati Childrenrsquos Best Evidence Statement

References HSCT Perumbeti A Hematopoetic Stem Cell Transplantation Practice Essentials Overview and Indications for HSCT Medscapehttpsemedicinemedscapecomarticle208954-overviewa1 Updated November 13 2017 Accessed January 21 2017 Lee S amp Smith A Survival estimates in advanced terminal cancer Morrison RS amp Savarese D MF Eds UpToDate Waltham MA UpToDate Inc httpswwwuptodatecomcontentssurvival-estimates-in-advanced-terminal-cancersearch=survival20estimates20in20advanced20terminal20cancerampsource=search_resultampselectedTitle=1~150ampusage_type=defaultampdisplay_rank=1 (Accessed January 21 2017) Chao NJ Clinical Manifestations diagnosis and grading of acute graft-versus-host disease Negrin RS amp Rosmarin AG eds UpToDate Waltham MA UpToDate Inc httpswwwuptodatecomcontentsclinical-manifestations-diagnosis-and-grading-of-acute-graft-versus-host-diseasesearch=clinical20manifestations20diagnosis20and20grading20of20acute20graftampsource=search_resultampselectedTitle=1~150ampusage_type=defaultampdisplay_rank=1 (Accessed January 21 2017) Filipovich AH Diagnosis and manifestations of chronic graft-versus-host disease Best Pract Res Clin Haematol 2008 Jun 21(2)251-7 Wolin KY Ruiz JR Tuchman H et al Exercise in adult and pediatric hematological cancer survivors an intervention review Leukemia 2010 24(6) 1113-1120

References HSCT Strenk M Gevedon A Monfreda J Cincinnati Childrens Hospital Medical Center Best Evidence Statement Physical therapy during the hemopoietic stem cell transplant process to improve quality of life httpwwwcincinnatichildrensorgservicejanderson-centerevidence-based-carerecommendationsdefault BESt 177 pages 1-9 March 18 2014 Wiskemann J amp Huber G Physical exercise as adjuvant therapy for patients undergoing hematopoietic stem cell transplantation Bone Marrow Transplantation 2008 41 321-329 Hacker ED Larson J Kujath A et al Strength training following hematopoietic stem cell transplantation Cancer Nurs 2011 34 (3) 238-249 Baumann FT Kraut L Schule K et al A controlled randomized study examining the effects of exercise therapy on patients undergoing haematopoietic stem cell transplantation Bone Marrow Transplantation 2009 1-8

1312018

2

Clotting Cascade

httpbaronerockscomindexphpmnemonicsmnemonics-hematology136-baronemnemonic-clotting-cascade (modified)

Co-factors

intrinsic

extrinsic

(heparin)

(warfarin)

INR

Beyond Lab Values The Role of the Physical Therapist in Bleeding and

Clotting Disorders

Eena Kapoor PT DPT


Learning Objectives

bull Identify multi-system impairments found in bleeding disorders and clotting disorders

bull Discuss evaluation tools to use for patients with bleeding and clotting disorders

bull Reflect on treatment strategies when working with persons with bleeding or clotting disorders to improve quality of life

Bleeding Disorders

bull Hemophilia A

bull Hemophilia B

bull Factor VII deficiency

bull Von Willebrand Disease

bull Glanzmannrsquos Thrombasthenia

What Is Hemophilia

bull Genetics bull Presentation

bull Types

ndash Factor VIII deficiency ndash Hemophilia A

ndash Factor IX deficiency ndash Hemophilia B

bull Severity ndash Mild (5) ndash Moderate (1-4) ndash Severe (lt1)

bull Treatment bull Complications

ndash Inhibitor

Goto 2016 Kuijlaars 2017

Hemophilic Arthropathy

Inactivity

Muscle Atrophy

Joint becomes

Vulnerable

Higher risk of injury

and bleed

Carcao 2015

1312018

3






ndash Joint pain


ndash Atrophy

ndash Strength



Alignment

Range of motion

Strengthening

Bone Density

Proprioception

Factor Replacement

Kempton 2015

Fearn 2010

1312018

4

MASAC Guidelines



Treatment Ideas

CLOTTING DISORDERS

Epidemiology





Heit 2016 Yang 2012

Risk Factors


bull Pregnancy

bull Immobility



bull Obesity

bull Cancer





Hillegass 2016







1312018

5

Thromboembolism


ndash Non-CNS



ndash Non-CNS




vertebral body

Rajachandran 2014






Venkata 2010


Medical Management







Hillegass 2016






1312018

6


bull Sports


bull Diet

ndash Vitamin K





ndash Edema

ndash Leg ulcers















Training Video










bull Muscle pump

bull Positioning


bull Hydration

bull Aquatics

Hillegass 2016


1312018

7



ndash Pain

ndash Signs of PTS


Case Study








PTS Assessment







bull Outpatient PT

bull Aquatics

bull HEP

Take Home Message





bull Questions


1312018

8





























Autoimmune Diseases











Anemia

Anemia









Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

3






ndash Joint pain


ndash Atrophy

ndash Strength



Alignment

Range of motion

Strengthening

Bone Density

Proprioception

Factor Replacement

Kempton 2015

Fearn 2010

1312018

4

MASAC Guidelines



Treatment Ideas

CLOTTING DISORDERS

Epidemiology





Heit 2016 Yang 2012

Risk Factors


bull Pregnancy

bull Immobility



bull Obesity

bull Cancer





Hillegass 2016







1312018

5

Thromboembolism


ndash Non-CNS



ndash Non-CNS




vertebral body

Rajachandran 2014






Venkata 2010


Medical Management







Hillegass 2016






1312018

6


bull Sports


bull Diet

ndash Vitamin K





ndash Edema

ndash Leg ulcers















Training Video










bull Muscle pump

bull Positioning


bull Hydration

bull Aquatics

Hillegass 2016


1312018

7



ndash Pain

ndash Signs of PTS


Case Study








PTS Assessment







bull Outpatient PT

bull Aquatics

bull HEP

Take Home Message





bull Questions


1312018

8





























Autoimmune Diseases











Anemia

Anemia









Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

4

MASAC Guidelines



Treatment Ideas

CLOTTING DISORDERS

Epidemiology





Heit 2016 Yang 2012

Risk Factors


bull Pregnancy

bull Immobility



bull Obesity

bull Cancer





Hillegass 2016







1312018

5

Thromboembolism


ndash Non-CNS



ndash Non-CNS




vertebral body

Rajachandran 2014






Venkata 2010


Medical Management







Hillegass 2016






1312018

6


bull Sports


bull Diet

ndash Vitamin K





ndash Edema

ndash Leg ulcers















Training Video










bull Muscle pump

bull Positioning


bull Hydration

bull Aquatics

Hillegass 2016


1312018

7



ndash Pain

ndash Signs of PTS


Case Study








PTS Assessment







bull Outpatient PT

bull Aquatics

bull HEP

Take Home Message





bull Questions


1312018

8





























Autoimmune Diseases











Anemia

Anemia









Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

5

Thromboembolism


ndash Non-CNS



ndash Non-CNS




vertebral body

Rajachandran 2014






Venkata 2010


Medical Management







Hillegass 2016






1312018

6


bull Sports


bull Diet

ndash Vitamin K





ndash Edema

ndash Leg ulcers















Training Video










bull Muscle pump

bull Positioning


bull Hydration

bull Aquatics

Hillegass 2016


1312018

7



ndash Pain

ndash Signs of PTS


Case Study








PTS Assessment







bull Outpatient PT

bull Aquatics

bull HEP

Take Home Message





bull Questions


1312018

8





























Autoimmune Diseases











Anemia

Anemia









Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

6


bull Sports


bull Diet

ndash Vitamin K





ndash Edema

ndash Leg ulcers















Training Video










bull Muscle pump

bull Positioning


bull Hydration

bull Aquatics

Hillegass 2016


1312018

7



ndash Pain

ndash Signs of PTS


Case Study








PTS Assessment







bull Outpatient PT

bull Aquatics

bull HEP

Take Home Message





bull Questions


1312018

8





























Autoimmune Diseases











Anemia

Anemia









Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

7



ndash Pain

ndash Signs of PTS


Case Study








PTS Assessment







bull Outpatient PT

bull Aquatics

bull HEP

Take Home Message





bull Questions


1312018

8





























Autoimmune Diseases











Anemia

Anemia









Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

8





























Autoimmune Diseases











Anemia

Anemia









Normal Hbg

Newborn 17-22gdl

1 week 12-20gdl

1 month 11-15gdl

Children 11-13gdl

Adult Males

14-18gdl

Adult Females

12-16gdl

1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

9

Symptoms of Anemia

bull Fatigue

bull Pallor

bull Tachycardia


bull Headaches


bull Chills


bull Weakness



Chronic


Pts on chemo

radiation

SCD

Renalliver failure

Post- BMT

Acute


2g drop from

baseline

Asymptomatic

Symptomatic SOB

darrBP

Fatigue uarrHR

DOE

Dizziness

No Cardiac

comorbidities

Cardiac Toxicity

Cardiac

Comorbidities

Cardiac Toxicity

Hgb within

2g from

baseline

Hgb gt2 g

from

baseline

Exercise within

tolerance VS

monitored

Average Hgb during

the previous month

CNrsquos guideline

Sickle Cell Disease

Sickle Cell Disease







Wang 2007


Sickle Cell Anemia









Wang 2007


Sickle Cell Anemia


ndash Chronic



ndash Osteomyelitis


ndash Asthma







1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

10

Pain





ndash Infection

ndash dehydration

ndash high altitude

ndash stress

ndash fatigue

ndash menstruation



Acute Pain




Yaster 200o






bull Earlier death





Goals

bull Decrease Pain




bull Vasodilation



Chronic Pain







Yaster et al 200o

Pain Management




breathing




Yaster et al 200o

1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

11



bull head of femur








Avascular Necrosis

Treatment





ndash Bone graft



ndash Osteotomy























Neumayr et al 2006

Pulmonary




bull Asthma



1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

12









bull Multifactorial

ndash Infection









Asthma








ndash 10 normal







bull PEP



bull Education

1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

13

Exercise Assessment




Reasons bull Anemia




Connes et al 2011

Exercise - Testing









Connes et al 2011



















Connes et al 2011


bull Stroke



Wang 2007

Stroke









1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

14


bull Risk factors

ndash Previous TIA








PT Management




Hydroxyurea (HU)




bull Decreased














SCD PT Summary










1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

15

Thalassemia

Thalassemia




bull Major or Minor


bull Anemia


Medical Management


bull Iron chelation


bull Folic acid


Lal 2016

Thalassemia






Lal 2016

Autoimmune Diseases

Autoimmune Diseases


bull Lupus




bull Evans syndrome

1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

16


(HLH)



bull Cytokine storm

ndash Macrophages





George 2014


HLH








bull Secondary



ndash Metabolic


ndash Autoimmune

(George 2014)

HLH diagnosis



2 Enlarged spleen




6 Elevated ferritin




George 2014

Medical Management









1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

17






bull Hyperglycemia

LaPier 1997

Muscle Atrophy













Chart Review

bull Lab values



bull HLH


bull Hemoglobin

LaPier 1997

Exercise

bull Individualized







LaPier 1997






bull Reversible

1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

18

HLH PT Summary

bull Chart review







bull Thalassemia

bull HLH













































stem-cells


Specialist

February 22 2018

1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

19

HSCT or HCT



8954-overview

Donor Types



8954-overview




8954-overview

Diagnoses



httpsemedicinemedsc

apecom

Diagnoses




httpsemedicinemedsc

apecom

Diagnoses






httpsemedicinemedsc

apecom

1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

20






54-overviewa1




54-overviewa1





Lee amp Smith

2017




Performa

nce

Status

Definition






waking hours





Lee amp Smith

2017



1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

21




httpsemedicinemeds




httpsemedicineme


Acute complications
























1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

22

Typical Timeline

httpsafepharmacysh

opsu





Chao

2017

aGVHD definition




Chao

2017

aGVDH


Chao

2017

aGVHD risk factors


Chao

2017




comorbidities

Chao

2017

1312018

23







Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

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1312018

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Filipovich

2008






Filipovich

2008





Filipovich

2008






2008









areas of moveable

smooth or shiny

skin with leather-

like consistency

often with

depigmentationrdquo

1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

24


httpwwwnejmorgdoif







Filipovich

2008






Filipovich

2008








bull Steroids

httpsmedlinepl

usgov

Research disclaimer



1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

25





Strenk et al

2014





Wolin et al

2010






2014




Strenk et al

2014






Strenk et al 2014





1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

26








effect


effect


effect


effect


(QoL fatigue)

Improving

effect

Improving effect


available

(2008)

Improving effect


duration of

recovery

No data available




Phase of Medical

Therapy

Type of Exercise


week)




Wiskemann and

Huber 2008


Phase of Medical

Therapy

Type of Exercise







week)


30 min



Huber 2008


Phase of Medical

Therapy

Type of Exercise


week)


more


Borg 70-80 max HR)

Wiskemann and

Huber 2008

Hacker et al 2011





1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources






1312018

27

Baumann et al 2009


Baumann et al 2009

Take Home




Resources




