barriers, blessings and solutions in adult metabolic
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BARRIERS, BLESSINGS AND SOLUTIONS IN ADULT METABOLIC DIETARY CARE KIMBERLY A CHAPMAN, MD PHDDANIELLE STARIN, MS RD LN
May 3, 2021
LEARNING OBJECTIVES
Be able to discuss how adults physiologically differ from children and how this impacts metabolic diet
Be able to list the barriers that adults present
Be able to discuss management for life changes
ADULTS ARE NOT JUST BIG CHILDREN-PHYSICAL DIFFERENCES
Water volume differs (Peds: ml/kg/h; Adults 120 ml/h)
Metabolism of medication differs (mg/kg/d vs mg/d)
Less plastic brains and closed fontanelles
More end organ damage
Females can have babies and males can father children
ADULTS ARE NOT JUST BIG CHILDREN- SYSTEM DIFFERENCES
Adult care focuses on taking ownership of care
Parents/caregivers not as involved
Less is done for the patient (support services)
Usually, shorter appointments
No lollypops or stickers
ADULTS ARE NOT CHILDREN- PSYCHIATRIC AND PSYCHOLOGY
Adults expect autonomous role in decision making
Can make decisions
Some of our patients do not have the ability to do this (their decision making is not always the best or their intellectual disabilities are limiting)
Disease and non-disease-related psychiatric disease
Relationship (and Sexuality) issues
ISSUES IN ADULT CARE
Legal – guardianship
Long term complications and specialist visits
Negotiating health care with challenges
Pediatric versus adult hospital and caregivers
Insurance
Transplant
Where to admit
INSURANCE ISSUES IN
ADULT CARE
Some with intellectual challenges ( we have a few high-power attorneys in our PKU group)
Most age out of Medicaid
Expect they will need some level of help
FORMULA ACCESS FOR
ADULTS
Patient assistance programs are limited
Not many insurance cover low protein foods (maybe 20% after deductible)
Varies by locations Medicare does not cover formula which is consumed by mouth;
must be by tube for swallowing dysfunction Medicaid as a secondary will not pick up if Medicare denies Problematic if one is covered by both
Individual can age out of special insurances at 18 – 21 years old (i.e. REM program)
EXAMPLE- OUT OF POCKET FORMULA COST
Adult with MSUD Nutrition Prescription ~60 grams medical protein per day One month’s supply of ketonex-2 = $1126.68 per month
Valine and Isoleucine supplementation Straight powder is relatively cheap but difficult to measure ($10/month) Compounded forms or premeasured sachets = $100 – 1000 per month
Low Protein Foods One box of low protein pasta = $10.99 + shipping One loaf of bread = $10.99 + shipping
CASE STUDY: THE TRANSITIONING TEEN
A is 17-year-old female with PKU
What do I need her to know and what do I need to know about her? Does she make her own formula? Can she could protein (phe)? Does she know how to make appointments and order
formula? Is she going to college, getting more training or starting a job
when done with HS?
Does she want children, what is her sexual identity, sexual orientation?
She can make formula and take meds
She does not know how to order formula and has never made an appointment on her own
She does not want to have children at this time
She has had sex with men only
RELATIONSHIPS & SEXUALITY
Many of our patients have questions about sexual relationships
Their parents may or may not acknowledge this
Be culturally aware
This is normal development
They are not going to tell you (unless you are Danie) unless you ask
Don't forget those who have intellectual challenges…
EXAMPLE- THE FIRST DATE
Hi P__,
Congrats on hitting the dating scene! Below are some low protein ideas for items at Ihop. Remember to drink your formula ahead of time so you wont be hungry should the date go long. I hope you have a great time!
2 gram:
Hash browns with side of tomato’s
Pancake with fruit topping
Gluten free English muffin with side of fruit
…
Cheers,
Danie
EXAMPLE- THE FIRST DATE
Patient consented to share photos
BARRIERS AND BLESSINGS: TRANSITIONING TO ADULT CARE
These are still adolescents They have strong attitudes and opinions (!) Getting through to them is extremely rewarding
Parents accepting child is growing up Playing middle-man during this difficult time Opportunity to solidify patients understanding of disease
management
CASE STUDY: A IS GETTING MARRIED (SHE IS NOW 28 YEARS)
Now what?
Would she like her fiancé at an appointment? Does he have questions?
They would like to try to start having kids in the next 1 year following wedding
PREGNANCY AND BIRTH
What we do depends on the diagnosis?
PKU vs MSUD vs FAO vs PA/MMA vs CBS deficiency vs UCD vs not small molecule
Peripartum period lasts up to 8 weeks following delivery
Wilcox GReviews in Endocrine and Metabolic Disorders (2018) 19:13–33
BARRIERS AND BLESSINGS: PREGNANCY CARE
Close contact with a patient through a life change Takes a lot of time to manage Get to experience the joy of new life
Patient getting coverage for meds and formula May have been off diet and has to learn to tolerate the diet again Many organizations will support pregnant women with IEM’s
EXAMPLE- RD TIME COMMITMENT WITH PREGNANT PATIENT
• Diet immediately post-partum
• Diet during breastfeeding
• Weekly-monthly blood spots
• Weight management
• Medication discussions
• Decisions on long-term diet
Post-partum
• Delivery planning• Weight
management counseling
• Weekly blood spots
• Weekly food record analysis
• Education on higher protein foods
3rd trimester
• Weight management counseling
• Weekly blood spots
• Weekly food record analysis
• Education on higher protein foods
• Counseling
2nd trimester
• Nausea management
• Coordination with OBGYN
• Formula intolerance
• Weight management counseling
• Weekly blood spots
• Weekly food record analysis
• Counseling
1st trimester
• Returning to diet education
• Formula trials• Medications
discussions• Weekly blood
spots• Weekly food
record analysis• Educating partner• Counseling
Pre conception
EXAMPLE- RD TIME COMMITMENT WITH PREGNANT PATIENT
Patient consented to share photos
CASE STUDY: ADULT WITH ID
N is a 52-year-old with CBS deficiency
Intellectual disability (IQ is about 49); hypertension, ectopic lens
Lives in protected living group home
No formula coverage
Power of attorney plays very small role in day to day living
Be ready for positive regular screening (lump on mammogram or need for colonoscopy)
History may be limited, but more time for all the forms.
BARRIERS AND BLESSINGS: CARING FOR ADULT WITH ID
Working with group homes Frequent staffing turn overs means frequent re-education Staff usually excited to learn. Very happy to tips to make their jobs easier Tend to actually do what you tell them! (unlike parents/family)
Insurance- Cannot get formula covered Most IEM organizations do not support formula Opportunity to RD to flex skills and get creative
EXAMPLE- CREATIVE FORMULA SOLUTION
Desired formula- any HCU formula- not covered by insurance Patient has both Medicaid and Medicare
Primary goal- protein supplement with no methionine
Recipe: 2 scoops GNC BCAA powder ¼ tsp GNC L-cystine ¼ tsp GNC L-phenylalanine à Makes up ~50% of protein needs and 30% calorie needs, saving ~$1000/month
CASE STUDY: OLDER ADULT WITH COMORBIDITIES
Mr. S is a 65-year-old with partial pyridoxine-responsive CBS deficiency (Pyridoxine + milddiet restriction)
He is retiring from his job as cook at a private school
He will be transitioning to Medicare and has a very limited income
He also has obesity, bilateral ectopic lens, peripheral neuropathy, rheumatoid arthritis, osteoporosis
ADULT SPECIFIC CHALLENGES
Other providers may not be familiar with diagnosis
Patient wants to help make decisions
Adherence (Anyone here who can’t remember to take a full course of antibiotics understands)
Education: sometimes what you think they know; they don’t know
OBESITY AND WEIGHT LOSS
How to treat the disordered eating that we have given these patients
Embracing the body type that the disease has created … or that genetics gave you… Health at every size
Weight loss education- with the metabolic RD or outside RD?
BARRIERS AND BLESSINGS: THE ADULT WITH COMORBIDITIES
Set in their ways, often inflexible to new treatment recommendations Have worked with multiple genetics teams Opportunity for you to stand out
Age related comorbidities Managing IEM with other nutrition related disorder is hard Creative problem solving for one patient can lead to ideas for others
WAYS TO IMPROVE THE ADULT EXPERIENCE
Adult onlyclinic days
Flexible schedules for morning and
evening appointments
Weekend appointments
Clinic rooms designed for adult
patients
Ability to do anthropometrics in
the room
Arrangements with community providers
EXPECT THEM TO BE
ADULTS!
QUESTIONS?
Our Contact information:
Kimberly A Chapman and Danielle Starin
Children’s National Rare Disease Institute, Washington DC
KChapman@childrensnational.org; Dstarin@childrensnational.org
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