2 diseases of the newborn

Diseases of the NewbornPrepared by: Hans Christian f. Vitug RN, MANc

Faculty/Clinical Instructor/ReviewerOur Lady of Fatima University – Antipolo Campus

"Necrotizing" means the death of tissue, "entero" refers to the small intestine, "colo" to the large intestine, and "itis" means inflammation.

An acute inflammatory disease of the bowel with increased incidence in preterm and high risk infants

Theories to Support that Explains NEC1.Little supply of oxygenation -> plus feeding ->

stress to the intestinal wall -> allowing bacteria to invade intestinal wall and bloodstream -> necrosis/perforation of the intestinal wall -> decrease absorption of the vitamins and minerals and Leak of bacteria into abdomen causing peritonitis.

2.Difficult deliveries -> deprived oxygenation -> vital organs receives more oxygen

3.Increased RBC counts – thickens the blood and impaired circulation -> hinder the transport of oxgenation

How does it happen?

• Prematurity remains the most prominent risk factor

• Great damage to mucosal lining diminished blood supply stop secreting protective lubricating mucus unprotected bowel attacked by proteolytic enzymes unable to synthesize IgM Gas-forming bacteria invades damaged area produce intestinal pneumatosis (presence of air in the submucosal or subserosal surfaces of the bowel)

Signs and Symptoms

• Infant may “not look well”• Poor feeding• Apnea • Vomiting (often bile stained)• Decreased U/O• Hypothermia • Distended abdomen• Gastric residuals• Blood in the stools

Diagnostic Evaluation

RadiographySausage-shaped dilation of the intestines“Soap suds” or bubbly appearance o f thickened bowel wall

Diagnostic Evaluation

Laboratory examinations–Anemia– Leukopenia– Leukocytosis–Metabolic acidosis– Electrolyte

imbalance

Therapeutic Management

• Oral feedings withheld for at least 24 to 48 hours

• Breast feeding is preferred

• Antibiotics • Probiotics– Lactobacillus acidophilus– Bifidobacterium infantis

Nursing Management

• Observation and assessment• Infants left undiapered• Position infant supine or on the

side• Vital signs including blood

pressure–Avoid rectal temperature

TRANSIENT TACHYPNEA OF THE NEWBORN

Some newborns' breathing during the first hours of life is more rapid and labored than normal because of a lung condition called transient tachypnea of the newborn (TTN).

Definition• A respiratory problem seen in the newborn

shortly after delivery• It is likely due to retained lung fluid• Common in 35+ week gestation babies who are

delivered by caesarian section without labor• Resolves over 24-48 hours• Causative Factors– Pulmonary immaturity– Mild surfactant deficiency

Causes of TTNTTN, also called "wet lungs" or type II respiratory distress syndrome, usually can be diagnosed in the hours after birth.

TTN can occur in both preemies (because their lungs are not yet fully developed) and full-term babies.

New borns at higher risk for TTN include those who are:

delivered by cesarean section (C-section)born to mothers with diabetesborn to mothers with asthmasmall for gestational age (small at birth)

Pathophysiology

• Lower levels of circulating catecholamines after a caesarean section which are necessary to alter the function of channels that absorb excess fluid from the lungs

• Delayed absorption of fetal lung fluid from the pulmonary lymphatic system increased fluid in the lungs increased airway resistance and reduced lung compliance

Clinical Manifestations

• Period of rapid breathing• Tachypnea• Intracostal and subcostal

retractions• Grunting• Nasal flaring• Possible cyanosis

Diagnostic Evaluation and Therapeutic Management

• Diagnostic evaluation–Chest X-ray– Levels of PG were found to be negative in

certain newborns

• Management– Supplemental oxygen–Antibiotics

ERYTHROBLASTOSIS FETALIS/HEMOLYTIC DISEASE OF THE NEWBORN

Abnormal, rapid destruction of RBCHyperbilirubinemia in the first 24 hours of life is most often the result

Hemolytic disease of the newborn (HDN)

Major causes of RBC destruction– Rh Incompatibility (Isoimmunization)• Mother is Rh negative, and infant is Rh positive• May not occur in first pregnancy• Increased risk of fetal blood being transferred to

maternal circulation subsequent pregnancy with Rh (+) fetus maternal antibodies formed will attach and destroy fetal erythrocytes• Progressive hemolysis in utero fetus compensates,

accelerates rate of erythropoesis immature RBCs appear erythroblastosis fetalis (hydrops fetalis)

Hemolytic disease of the newborn (HDN)

Major causes of RBC destruction–ABO Incompatibility• Between a mother with type O and an infant

with A or B blood groups.• Anti-A and Anti-B already present in the

maternal circulation cross the placenta and attach to fetal RBCs hemolysis• Less severe hemolytic reaction the Rh

incompatibility• May occur in first pregnancy

Signs and Symptoms

Jaundice –Most not

jaundice at birth

AnemiaHypovolemic shock may developHypoglycemia

Diagnostic Evaluation

•Maternal antibody titer (Indirect Coomb’s test)•Amniocentesis•Ultrasound

Therapeutic Management

Prevention of Rh Isoimmunization–Administration of RhIg–RhIg (RhoGAM) – must be administered

to unsensitized mothers within 72 hours after the first delivery–Admin of RhIg at 26 to 28 weeks of

gestation further reduces risk of isoimmunization

Therapeutic Management

• Exchange Transfusion–Infants blood is removed in small

amounts (5 to 10 ml at a time) and replaced with compatible blood–For severe hydrops

• ABO incompatibility–Early detection and phototherapy

Nursing Management• Recognizing jaundice – initial nursing

responsibility• Prepares family incase of transfusion and

assist practitioner– Infant remains NPO during procedures–Maintain documentation of blood volume

exchange, time, cumulative record of the total blood exchanged–Vital signs– Signs of transfusion reactions

DOWN SYNDROME

Down Syndrome

• The genetic disorder most frequently seen as causing moderate to severe mental retardation

• Etiology is unknown– Genetic predisposition– Exposure to radiation before conception– Immunologic problems– Infection

Clinical Manifestations• Bradycephaly• Back of the head is flat• Epicanthal folds• Palpebral fissure slanting

laterally upward• Tongue may protrude• Narrow palate• Low-set ears• Short broad hands• Transpalmar crease (simian line)• Short stature• Rag-doll appearance• IQ of 50-70

Diagnostic Evaluation

• Evident at birth• Prenatal testing• Chromosomal

analysis

Therapeutic Management

• Surgery to correct cardiac abnormalities, GI malformations and craniofacial deviations• Neck radiography before the child

participates in any sports

Nursing Management

• Options for fluid and calorie intake– Breastfeeding may not be possible, immature

sucking reflex– Special bottles and utensils

• Routine– Changes causes frustration and decreased coping

abilities• Encourage self-care• Advise X-rays before participating in sports

TEMPERATURE CONTROL

Cold Stress

• Infants lack shivering response• Norepinephrine (SNS) stimulates fat metabolism

to produce internal heat blood surface tissues

• Increased in metabolism increased oxygen consumption

• Norepinephrine vasoconstriction decrease oxygen decreased glucose metabolism

• Results: Hypoxia, Metabolic acidosis, Hypoglycemia

Three primary methods for maintaining a neutral thermal environment

• Incubator• Radiant warming

panel• Open bassinet

with cotton blankets

Temperature control

•Warm items first • Plastic wrap • Careful drying• Kangaroo care

Incubator Care

• Double walled incubators –improve infants ability to maintain a

desirable temp reduces energy expenditure r/t heat regulation

• Pre-warm incubator first• Head covering when outside of

the incubator

THE NEWBORN SCREENING PROGRAM

Essential public strategy that enables the early detection and management of several congenital metabolic disorders, which if left untreated , may lead to mental retardation and even death

For early detection and management of congenital metabolic disorders

Newborn Screening Program (NBS)

• Mandated through RA 9288 (The Newborn Screening Act of 2004)• Done between 24-72 hours after

birth

Collection of NBS Samples• Through heel prick method: 4

drops of blood is drawn from heel puncture blotted onto a filter paper

• Air dry 4-6 hours• Sent to laboratory within 24

hours• BEST - 48th to 72nd hours of life • ACCEPTABLE - anytime after 24

hours from birth until 2 weeks of age

Sample collection done before the ideal time may result in:

Falsely elevated thyroid stimulating hormone (TSH) = false (+) screen for CHFalsely elevated 17 hydroxyprogesterone (17-OH-P) = false (+) screeen for CHFalsely low galactose and phenylalalnine = false (-) screen for GAL and PKU

Disorders tested for newborn screening

• Congenital Hypothyroidism (CH)• Congenital Adrenal Hyperplasia (CAH)• Galactosemia (GAL)• Phenylketonuria (PKU)• Glucose-6-Phospate-Dehydrogenase

Deficiency (G6PD)

Congenital Hypothyroidism (CH)

Congenital Hypothyroidism (CH)

• aka Cretinism• Lack or

absence of thyroid hormone

(H-U-Mi-D)

Hereditary conditions

Underdevelopment of the fetal thyroid glands

Maternal Intake of anti thyroid drugs

during pregancy

Deficiency, Maternal Iodine

*ManifestationsPoor suck and feedingJaundiceHypotoniaCool pale dry skinSwelling around the eyesLarge swollen tongueLarge fontanels with late closurePoor weight gain and growthHoare sounding cryDelayed milestone (sitting, crawling, walking and talking)

*TreatmentLifetime oral doses of thyroid hormone. L-Thyroxine

Nursing Considerations:Instruct parents to avoid Soy-based formulas and iron supplements. Avoid adjusting medications without MD’s order to prevent under medication or over medication.

Excessive medication can cause : D-I-T-S DiarrheaInability to sleepTachycardiaShakiness in the child

*TreatmentRegular monitoring of the child’s weight, overall health and thyroid hormones level

Congenital Adrenal Hyperplasia (CAH)

Congenital Adrenal Hyperplasia (CAH)

• Excessive or deficient production of sex steroids• Severe salt loss, dehydration and

abnormally high levels of male sex hormones in both boys and girls• If not detected and treated early,

infants may die within 7-14 days

Congenital Adrenal Hyperplasia (CAH)CAH is caused by a deficiency of adrenal gland hormones.

21 hydroxylase is missing or not working correctly.

Congenital Adrenal Hyperplasia (CAH)

21-OH is responsible for the production of hormones :CORTISOL is involved in glucose metabolism and in normal inflammation and immune response. ALDOSTERONE is responsible for blood pressure and sodium retention.

Congenital Adrenal Hyperplasia (CAH)

*Manifestations• Poor feeding• Listlessness and drowsiness• Vomiting• Diarrhea• Weight loss• Hypotension• Hyponatremia• Metabolic acidosis

Muscle growth at an early age

Enlargement of penis during childhood

Early deepening of the voice

Early beard

Pubic hair and underarm hair during childhood

Severe acne

M-E-E-E-P-S-S

Muscle growth at an early age

Enlargement of penis during childhood

Early deepening of the voice

Early beard

Pubic hair and underarm hair during childhood

Smaller than normal testicles

Severe acne

Male pattern baldness

Severe acne

Early puberty changes such as hair in airmpits and pubic area

Lack of menstrual periods or scanty or irregular periods

Excess hair on the face and body

Deep, husky voice

(S-M-E-L-E-D)Severe acne

Male pattern baldness

Early puberty changes such as hair in airmpits and pubic areaLack of menstrual periods or scanty or irregular periodsExcess hair on the face and bodyDeep, husky voice

TREATMENT

*TreatmentLifetime administration of the deficient or missing hormonesHYDROCORTISONES lessens the amount of androgens (prevents early puberty and allows for more typical growth and development).

Over medication can results to Cushing’s syndrome (Stretch marks, rounded face, weight gain, hypertension and bone loss).

Under medication can occur during periods of stress and illness when higher doses of the drug are required by the body

Corrective surgery for enlarged clitoris (can be done as early as one to three years of age. To separate labia and to create a normal vagina

Galactosemia (GAL)

Galactosemia (GAL)

This hereditary disorder is characterized by the lack of the enzyme Galactose-1-Phosphate uridyl Transferase (GALT) that converts galactose to glucose, the form of sugar that can be used by body cells.

Galactosemia (GAL)

Initial symptoms also include: (F-L-I-P)

Failure to gain weight

Lethargy

Irritability

Poor feeding and poor suck

Galactosemia (GAL)

Treatment: Giving the child a special lactose free formula and exclusion of lactose and galactose foods such as milk (including breast milk) and other dairy products from the diet through out life.

Galactosemia (GAL)

Foods that should be avoided:Milk and all dairy productsProcessed and pre packaged foodsTomato saucesCertain medicationsAny foods or drugs which contain the ingredients Lactulose, Casein, Caseinate, Lactalbumin, Curds, Whey or Whey solids

Galactosemia (GAL)

Calcium and Vitamin D deficiency is likely to develop in a child on a lactose free. Therefor, the child is given supplements to prevent deficiencies

Phenylketonuria (PKU)

A metabolic disorder characterized by lack of enzyme Phenylalanine hydroxylase (PAH) needed to process the amino acid phenylalanine

The resultant build up of the said protein in the body leads to mental retardation

Phenylketonuria (PKU)

Phenylketonuria (PKU)

• Excessive accumulation of phenylalanine = brain damage• Dx – Guthrie test•Mx - Low protein diet;

breastmilk

Glucose-6-Phospate-Dehydrogenase Deficiency (G6PD)

G6PD is one of many enzymes that help the body process carbohydrates and turn them into energy.

Glucose-6-Phospate-Dehydrogenase Deficiency (G6PD)

• A condition where the body lacks the enzyme called G6PD a metabolic enzyme especially important in RBC metabolism• Hemolytic anemia resulting from

exposure to certain drug, food and chemical

Child during Hospitalization

STRESSORS AND THE CHILD’S REACTION

Illness and Hospitalization:

Children are particularly vulnerable to the crises of illness and hospitalization because:

1.Stress represents a change from the usual state of health and environmental routine

2.Children have limited number of coping mechanisms to resolve stressors

SEPARATION ANXIETY• Also known as Anaclitic depression• Major stress especially for children

ages 16 to 30 months• Three Phases:–Phase of Protest–Phase of Despair–Phase of Detachment

SEPARATION ANXIETY

Phase of Protest•React

aggressively to separation from parent•Behavior is from

a few hours to several days

Three Phases:

SEPARATION ANXIETY

Phase of Despair•Child is less active•Withdraws from others

Three Phases:

SEPARATION ANXIETY

Phase of Detachment• Also called denial• Appears detached

and uninterested in parents’ visits• Appears to finally

adjust to the surroundings

Three Phases:

Loss of ControlINFANTS– Trust– Inconsistent care and deviations from daily routine

• TODDLERS– Autonomy– Egocentric pleasures– Rely on the consistency and familiarity of daily rituals– Altered routine and rituals– Regression

• PRESCHOOLERS – Egocentrism and magical thinking– Physical restriction, altered routines and enforced dependency

Bodily Injury and Pain

• INFANTS– Infants younger than 6 months

• no obvious memory of previous pain

– Facial expression of discomfort– React with physical resistance– Distraction and anticipatory preparation does little to lessen

immediate reaction to pain• TODDLERS

– Intrusive experience produce anxiety– React with intense emotional upset and physical resistance– Communicate about their pain

Bodily Injury and Pain • PRESCHOOLERS– Cause of illness is seen as concrete action the child

does or the child fails to do self-blame– Contagion – proximity of two object or persons causes

the illness– Injection - fear that the puncture will not close

• SCHOOL-AGE CHILDREN– May be less concerned with pain than disability– Major concern is their fear of being told that

something is wrong with them– Aware of the significance of different illnesses

Bodily Injury and Pain• SCHOOL-AGE CHILDREN– Passive acceptance of pain– Nondirective request for support – When someone identifies unspoken messages and

offers support, they readily accept it• ADOLESCENTS– The nature of bodily injury may be more important

based on the adolescents’ perception rather than the actual degree of severity of the illness

– Changes in body image is their concern– Privacy– Reluctance to disclose pain

NURSING CARE OF THE CHILD WHO IS HOSPITALIZED

Communication

• Speak in quiet pleasant tones• Bend down• Do not use clichés.• Explain all procedures • Be honest• Be careful in making promises• Observe nonverbal communication for clues to level of

understanding• Do not threaten• Allow child to show feelings• Provide time to talk

Communication

• Teach parents to anticipate next stage of development• If teaching is interrupted, start over from the beginning• Provide independence• Do not compare child’s progress to that of anyone else• Provide praise• Instead of asking what something is, ask child to give it

a name or tell you about it• Allow choices where possible• Involve parents in child’s care

Communication

• Keep routines • If parents cannot stay with child, encourage

them to bring in a favorite toy, pictures of family members or to make tape to played for the child

Play

• Toddler– Enjoys repetition– Solitary play–Parallel play

• Preschooler–Role play, make believe, associative play

• School-age–Group, organized activities–Group goals with interaction

Play• Play is a very important part of development for your

growing child.

• Not only is play time entertaining for your child, but it also provides stimulation, increases skills and coordination, provides an outlet for your child's energy, and helps to encourage exploration by your child.

Play is also important for the following reasons (Lippincott Williams & Wilkins, 2005)

Play is an excellent stress reducer and tension reliever. It allows the child freedom of expression to act out his fears, concerns and anxieties

Play provides a source of diversional activity, alleviating separation anxiety

Play is also important for the following reasons (Lippincott Williams & Wilkins, 2005)

Play is also important for the following reasons (Lippincott Williams & Wilkins, 2005)

Play provides the child with a sense of safety and security because while he is engaging in play, he knows that no painful procedures will occur.

Play is also important for the following reasons (Lippincott Williams & Wilkins, 2005)

Developmentally appropriate play fosters the child’s normal growth and development, especially for children who are repeatedly hospitalized for chronic conditions

Play is also important for the following reasons (Lippincott Williams & Wilkins, 2005)

• Play puts the child in the driver’s seat, allowing him to make choices and giving him a sense of control

Play– Way to solve problems– Express creativity– Decrease stress– Prepare for procedures– Enhance fine and motor skills

• Make play appropriate for mental age and physical/disease state

• Multisensory stimulation• Safe toys for mental age• Offer play specific to age group

NURSING CARE OF THE CHILD IN PAIN

Concept of PainPreoperational Thought (2-7 yrs)Relates to pain primarily as physical concrete experienceMagical disappearance of painPain as punishmentHold someone accountable for own pain

Concrete Operational Thought (7-10 yrs)Relates to pain physicallyPerceive psychologic painFears bodily harm & annihilationPain as punishment

Formal Operational Thought (13 yrs and older)Give reason for painPerceives several types of psychologic painFears losing control during painful experienceHas limited life expereinces to cope with pain

Q-U-E-S-T (PAIN ASSESSMENT)

QUESTION the child’s parents and child too, if he is old enough to respondUSE appropriate pain assessmentEVALUATE the child’s behaviourSECURE the parent’s active participation in treatmentTAKE the cause of the pain into consideration

ASSESSMENT

• Wong-Baker FACES Pain Rating Scale

ASSESSMENT• FLACC (Face, Legs, Activity, Cry and

Consolability) – for infants and very young children

• Behavior is observed to assess painMeasures each of the five identified categories on a 0 to 2 scale• The higher the total score, the more pain

CryingRequires oxygen to maintain saturation about 95%Increased heart rate and blood pressureExpressionSleeplessness

CRIES Neonatal Postoperative Pain Measurement Scale

Facial ExpressionCryingBreathing patternsState of arousal Movement of arms and legs

Neonatal Infant Pain Scale

Gestational ageHeart rateOxygen saturationBehavioral stateBrow bulgeEye squeezeNasolabial furrow

Premature Infant pain Profile

Behavioral Responses to pain

Facial expression

Mouth stretched open

Eyes tightly shut

Brows and forehead knitted

Cheeks raised high enough

INFANT

Narrowing of the eyesGrimace or fearful appearanceFrequent and longer lasting bouts of crying with a tone that is higher and louder than normalLess receptiveness to comforting by parents or other caregivers Holding or protecting the painful areas

Younger Children

Pharmacologic interventions

Anticipate and prevent or minimize pain related to hospitalization, procedure and treatment

Identify and relieve existing pain

Non pharmacologic interventions to reduce stress, increase comfort and enhance healing

P-A-I-N Management

Pharmacologic Intervention – mainstay of pain management and it depends on the specific needs of the patient

Opioid analgesics-Highly effective pain relievers and constitute the core of most pharmacologic interventions to manage acute pain in infants and children-Oral, sublingual, rectal, nasal, subcutaneous, transdermal, IV and intraspinal*Morphine (MS contin)*Fetanyl (Duragesic)

Non Opioid Analgesics-are prescribed to manage mild to moderate pain.-infants and children metabolize non opioid analgesics in the same manner and at the same rate as adult.

-relieve for mild to moderate pain and anti inflammatory effects-Ibuprofen (advil); Naproxen (Naprosyn); Tolmetin (Tolectin); Indomethacin (Indocin) & Ketorolac (Toradol) are approved for use in children-S.E: Inhibition of platelet aggregation and GI irritation

NSAIDS

-Is the DOC for treating mild pain. Available in suppository, liquid and table form.

-it has the added benefit of helping reduce fever and is very safe, even for neonates.

-long term can cause risk of liver damage

Acetaminophen

EMLA Cream (lidocaine 2.5% and prilocaine 2.5%),

applied to intact skin under occlusive dressing, provides dermal analgesia by the release of lidocaine and prilocaine from the cream into the epidermal and dermal layers of the skin

• Complementary and alternative medicine (CAM)– Complementary Pain Medicine• Music Therapy• Hypnosis • TENS (transcutaneous electric nerve stimulation) unit • Acupuncture

– Non-Pharmacologic Care• Comfort, positioning and non-nutritive sucking• Distraction • Relaxation • Guided Imagery • Biofeedback • Behavioral Contracting

Pediatric Surgery

Pediatric Surgery

• Preoperative classes– Younger – simple and as close to the time of the

procedure as possible• Allow to play with equipment• Teach and provide time to practice• Show pictures• Describe sensations• Detect misconceptions or fantasies• Parents can often be helpful in preparing

PEDIATRIC SURGERY

• Preoperative class• Listen to child for clarifying misunderstandings• Give simple information about the system that will

be affected• Use of anatomically correct dolls• Preschool boys: allow to look at penis after surgery• Post surgery: helping child master a threatening

situation and minimizing physical and psychological complications

CHRONICALLY ILL PEDIATRIC CLIENTS: CONCEPTS OF DEATH DYING AND GRIEVING

Concept of Death in Childhood (Lippincotts William and Wilkins, 2005)

Infancy Concept of death – NONENursing considerations: Be aware that the older infant will experience separation anxietyHelp the family cope with death so they can be available to the infant

Concept of Death in Childhood (Lippincotts William and Wilkins, 2005)

Early childhood Knows the words “DEAD” and “DEATH”. Reactions are influenced by the attitude of the parents.Nursing considerations:Help the family members including siblings cope with their feelingsAllow the child to express his own feelings in an open and honest manner.

Concept of Death in Childhood (Lippincotts William and Wilkins, 2005)

Middle childhood.Understands universality and irreversibility of death. May have a fear of parents dying.Nursing considerationsUse play to facilitate the child’s understanding of deathAllow siblings to express their feelings.

Concept of Death in Childhood (Lippincotts William and Wilkins, 2005)

Late childhood Beings to incorporate family and cultural beliefs about death. Explores views of an afterlife and faces the reality of own mortalityNursing considerationsProvide opportunities for the child to verbalize his fearsHelp the child discuss his concerns with the family

Concept of Death in Childhood (Lippincotts William and Wilkins, 2005)

Adolescence Adult perception of death, but still focused on the HERE and NOWNursing considerationsUse opportunities to open discussion about deathAllow expression of feelings of guilt, confusion and anxietySupport and maintain self esteem

Helping Families to Cope

• Accept and support participants• Be available and express your availability• Encourage parents to assist in the care of

their child• Encourage involvement of siblings• Religious associations as source of

strength and support

Helping parents to talk with their child about dying if he is ready to do soEncouraging all family members to express their feelings, even though they might be difficult to hearAllowing families to spend as much time as possible with the dying childAllowing and encouraging parents to continue to take an active role in their child’s careReminding parents that they don’t always have to be strong and ask for help

Thank you for Listening!