Transitioning to Adult Medical Care for Patients with Dyskeratosis Congenita

Tim Olson M.D. Ph.D.olsont@email.chop.edu

Adult PatientsHospital of the University of

Pennsylvania

Comprehensive Bone Marrow Failure Center

Pediatric Patients Children’s Hospital of

Philadelphia

267-426-9929 215-615-6555

∮ ~ 25% (~10.2 Million) of U.S. children have one or more chronic health conditions

∮ ~ 18% (4.5 Million) of US adolescents (12-18 y/o) have special health care needs

∮ 750,000 young adults with childhood acquired conditions enter adulthood each year

∮ Only 40% have had sufficient discussion about how to transition to adult care

Newacheck & Kim 2005CDC, 2005; Focal Point, 2006

U.S. Department of Health and Human Services, 2013

2

WHAT IS PEDIATRIC – ADULT TRANSITION?

“…a purposeful, planned movement of youth with special health care needs

from pediatric to adult care.”

Blum RW Pediatrics, 2002

∮ The Burdens

∮ Increased rates of depression

∮ Increased social/legal problems

∮ Impaired school/work performance

∮ Increased anxiety about body image, the future, death

∮ The Benefits?

∮Maturity

∮ Perspective

Paediatr Child Health. Nov 2007

BUILDING RESILIENCE IN CHILDREN AND TEENS:

Prepare them for the Adult World!1. Make connections

2. Help your children by having them help others

3. Maintain a daily routine

4. Take a break

5. Teach your child self-care

6. Move towards your goals

7. Nurture a positive self-view

8. Keep things in perspective & maintain a hopeful outlook

9. Look for opportunities for self-discovery

10. Accept that change is part of living

(American Psychological Association http://www.apa.org/ Resilience Guide for Parents and Teachers)

∮ A different crossroads for different patients:

∮ Adjusting to adult responsibilities with a lifelong chronic illness

∮ Coping with a new diagnosis in the context of other major life changes

∮ Experiencing the fear and stress of worsening symptoms that have yet to be explained/diagnosed

-2002 AAP, AFP, ACP, ASIM Consensus Statement

1. Ensure that all young people with special health care needs have an identified health care

professional who attends to the unique challenges of transition and assumes responsibility for

current health care, care coordination, and future health care planning. This responsibility is

executed in partnership with other child and adult health care professionals, the young person, and

his or her family. It is intended to ensure that as transitions occur, all young people have

uninterrupted, comprehensive, and accessible care within their community.

2. Identify the core knowledge and skills required to provide developmentally appropriate health care

transition services to young people with special health care needs and make them part of training

and certification requirements for primary care residents and physicians in practice.

3. Prepare and maintain an up-to-date medical summary that is portable and accessible. This

information is critical for successful health care transition and provides the common knowledge base

for collaboration among health care professionals.

4. Create a written health care transition plan by age 14 together with the young person and family. At a

minimum, this plan should include what services need to be provided, who will provide them, and

how they will be financed. This plan should be reviewed and updated annually and whenever there is

a transfer of care.

5. Apply the same guidelines for primary and preventive care for all adolescents and young adults,

including those with special health care needs, recognizing that young people with special health

care needs may require more resources and services than do other young people to optimize their

health. Examples of such guidelines include the American Medical Association’s Guidelines for

Adolescent Preventive Services (GAPS),8 the National Center for Education in Maternal and Child

Health’s Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents,9 and

the US Public Health Service’s Guidelines to Clinical Preventive Services.10

6. Ensure affordable, continuous health insurance coverage for all young people with special health

care needs throughout adolescence and adulthood. This insurance should cover appropriate

compensation for 1) health care transition planning for all young people with special health care

needs, and 2) care coordination for those who have complex medical conditions.

PEDIATRICADULT TRANSITION GUIDELINES

Find Adult

Providers

Knowledge/

skills

Medical

Summary

Written

Plan

Streamline

Guidelines

Insurance

Coverage

∮ ~Many benefits to young adults with special medical needs transitioning to adult care systems

∮ Eliminated denials based on pre-existing conditions

∮ Can stay on parents policy until age 26

∮ Subsidized private insurance, expanded Medicaid eligibility

∮ But….the system is complex

∮ Only 27% of young adults aware of insurance options (Collins et

al., The Commonwealth Fund, 2013)

∮ Emphasizes need to have formal transition program

➢ Online resources:

National Health Care Transition Center: http://www.gottransition.org/

• Links to state-specific plans to help navigate acquiring insurance for young adults

• Information about how to navigate the changes in the Affordable Care Act to best serve patients with chronic medical needs

➢ Utilize social work services in your local pediatric hematology practice

Transitioning hematology care is only a small part of the challenge!

Pulmonology

Gastroenterology

Hepatology

Endocrinology

ENT/Oral surgery

Neurology

Psychology

Gynecology/ Urology

What are an individual patient’s major DC-related medical problems?

Expertise

Pediatric Care Adult Care

Failure to Thrive

Developmental Delay

Inherited Immune Deficiency

Bone Marrow Failure/ BMT

Enteropathy/Enterocolitis

MDS/Leukemia

Head/Neck Cancer

Liver Cirrhosis

Pulmonary Fibrosis

Expertise and patient-specific needs should

help determine when to

transition!

What is a Medical Home?

☤Easier to access services

☤Consistent and coordinated care

☤Efficient and effective use of resources

☤Provides support to individuals and families

☤Improves health, developmental, educational, vocational, psychosocial and functional outcomes

A way of delivering health care that is coordinated, patient and family centered and culturally appropriate.

Where Should your Medical Home Be?

For Patients with Dyskeratosis Congenita:

❖ Children: Typically easy to identify

❖ Pediatric Hematologist

❖ Pediatric BMT/BMF team

❖ Adults: Much more difficult

❖ Adult Hematologist?

❖ Primary Care Physician + Regional Specialist?

GI

Hepatology

ENT

GynMedical

Home

Tip: Avoid transitioning medical home and subspecialty care at the same time

If the young adult remains near hometown:

❖ Medical home first then specialists

❖ Also ok to transition specialty care first Medical home

But… if the young adult is relocating:

❖ Best to identify medical home first (prior to move)!!

❖ Organizations like DCO can help to identify local/regional experts to serve as medical home

When is it time to transition?

✓ When your pediatric team can’t address all of your

health problems

✓ When you are fully ready to be a partner in your medical

decision-making, not a passive participant

✓ When the office equipment is too small

✓ At the end of education or development

❖ There is not one specific age! It depends……

Age Provider Parent/Family Patient

Early

Childhood

Major

responsibilityProvides care Receives care

Grade SchoolAge

Support to family &

patientManages Participates

Secondary School Age

Consultant Supervisor Manager

Adulthood Resource Consultant Supervisor

Adapted from Kieckhefer & Trahms, Pediatr Nursing 2000

SHARED MANAGEMENT MODEL

(AN EVOLUTION IN HOW CARE IS PROVIDED)

How/when to prepare for transition?

17

Modifiable Factors: can frequently be changed• Knowledge• Skills/self efficiency• Believes/expectations• Developmental maturity• Goal/ motivation• Relationship/communication• Psychological emotions

Patient

Provider(s) Parent(s)

Transfer to adult care

Preexisting Factors: can frequently not be changed• Access/ insurance• Medical status and medical risk factors• Neurocognition/ IQ• Sociodemographic/ culture

JAMA Pediatr. 2013;167(10):939-946.

Social-ecological Model of Adolescent and Young Adult Readiness to

Transition (SMART) Model

❖ Access/ insurance

➢ Employment/ access to private insurance

➢ State Medicaid programs- which providers are you allowed to see?

❖ Medical status and medical risk factors

➢ Severely affected? (Hoyeraal-Hreidarsson, Revesz)

➢ How many subspecialists are needed?

➢ How complex will the coordination of care be?

❖ Neurocognitive abilities will affect:

➢ Time to transition

➢ Extent to which responsibility can be shifted

❖ Sociodemographics/ culture

➢ Income/ availability of support

➢ Access to regional expertise

➢ Cultural attitudes toward the medical system

Preexisting Factors Affecting Transition for DC Patients

Modifiable Factors

Key Knowledge and Skills!

• Understanding your condition and health needs

• Having an emergency plan

• Communicating with your healthcare professional

• Managing medications and treatments

• Scheduling and getting to appointments

• Obtaining health insurance

• Keeping a health summary

• Finding community resources

• Setting goals

• Knowing what to expect in the adult clinic and hospital

Tweens(~ 11-13 years)

Key skills:

Understanding condition

• Learn about condition(s) and reasons for tests, procedures, and medications

• Know how to briefly explain them to others• Know any allergies

Managing medications and treatments• Know medication names • Take medications on own without reminders • Know how to read labels on medication bottles

My Health: 3 Sentence Summary

What is it?

Easy way to remember and recite important health information with healthcare providers or other adults during an acute event

Sentence 1: My age, diagnosis and brief medical history

Sentence 2: My treatment plan

Sentence 3: My question/concern/symptom now

• Practice with parents and health care providers

• Ask if your 3-Sentence Summary is correct and includes all the important information

Good 2 Go Transition Program www.sickkids.ca/good2go

Example:

“ Hi, my name is ____”.

1) I am 16 and have Dyskeratosis Congenita. I have low platelets and low white blood cells

2) My doctors provide platelet transfusions when my platelets are low, and sometimes I often need to go to the hospital when I have an infection.

3) I have a fever, cough, and just don’t feel well

Early to mid teens(~14-16 yrs)

Key Skills:

Communicating with healthcare team

• Comfortable doing most of the talking during visits• Spends some time with health care provider without parents at each

visit

Appointments• Know names of health care providers and why they are seen• Practice making appointments with coaching from parent

Know emergency plan• Learn plan for important acute contingencies• Carry emergency management card

Setting goals • Consider plans for the future (driving, post high school education,

employment/career, living away from home, relationships, etc.)

An approach to learning how to communicate with health care professionals (G.L.A.D.D.):

G Give information: How you’ve been feeling, steps you’ve taken for your health.

L Listen: Learn from health care providers.

A Ask: Questions about your health.

D Decisions: Learn how to make and participate in treatment decisions.

D Do: Your part in following the plan that is made!

http://hctransitions.ichp.ufl.edu/gladd/

In Case of Emergency (ICE) contact in mobile phone address book

Assists emergency personnel in reaching emergency contact:

• Choose a person to be your “ICE” contact

• Provide them with information that may affect your treatment

• Add emergency contact’s phone number in your mobile phone address book under the heading “ICE” (example: ICE-Mom)

❖ Carry an Emergency Card

Portable customized wallet-sized documentwith:

➢ Address/contacts

➢ Medical history

➢ Prescription information

➢ Allergies

➢ Emergency plan

• Available at:

➢www.sickkids.ca/MyHealthPassport

➢https://www.healthcareready.org/rx-on-the-run

Older teen/young adult(~17-22 yrs)

Key Skills:

Independent appointments

• Take responsibility for scheduling appointments • Arranging transportation to get to appointments

Navigate health insurance

• Carries insurance card (or a copy) and co-pay• Makes plans for obtaining health insurance as an adult

Taking ownership of medical records

• Utilize an organized system• Maintains an up-to-date health care summary with help from

pediatric team

Learning about and planning for transition

• Learns about the adult health system• Asks pediatric team and support network for recommendations

regarding adult providers• Creates a written comprehensive transition plan

Pediatric Care – Where you are now Adult Care – Where you will be

Parents and Medical Providers are in chargeCare is self-directed and self monitored by

patients

Parents schedule appointmentsPatient must schedule and keep

appointments

Transportation provided by parents Patient must provide own transportation

Support services are offered for financial and emotional issues

Patient must seek support services independently

Parents manage medications/treatmentsPatient must obtain prescriptions and

manage home care

Pediatric clinic staff: typically engaging, patient, and tolerant

Adult clinic staff: likely to be intolerant of immature behavior

Parents shield patients from specifics regarding prognosis and decision-making

Patients must make informed decisions and must be able to handle bad news

Pediatric vs. Adult care: How do they differ?

We don’t expect your children to have mastered all of these skills…yet.

Creating a Personal Health Record• Step 1: Work with your providers to determine which parts of the medical record

you need.

• Step 2: Organize paper copies in a file folder or binder. Use tools to help organize sections.

• Step 3: Keep electronic copies on personal storage devices or use an internet-based storage service.

• Step 4: Ask for and complete an “authorization for the release of information” form and keep with health records

• Step 5: Work with your care team to create a Health Care Summary and Emergency care plan.

• Step 6: Bring the personal health record to all health care appointments.

• Step 7: Keep your Health Care summary up-to-date. Add new health records to your file with each health care encounter.

(Adapted from American Health Information Management Association and Healthytransitionsny.org)

Keeping track of health information

• Health Care Transition Summary: 2 page health summary from FloridaHATS program: http://www.floridahats.org/wp-content/uploads/2010/03/HCT-Summary.pdf

• Healthy Transitions: Electronic Transition Information Form: more detailed form for summarizing health information. www.healthytransitionsny.org/

• CHOP Care Binder: A place to keep track of important medical and other related information: www.chop.edu/transition

➢ Ask your pediatric providers and support networks for help

➢ Find a primary practice to be medical home

➢ Anticipate and address changes in health insurance

➢ Be sure to bring medical summary, copies of relevant records, and written transition plan (use a care binder!)

➢ Maintain an emergency care letter (if needed)

➢ Address decision-making, guardianship issues if indicated

Identifying adult health care providers

Legal Adulthood (Age 18)

At age 18, a person has the right to:

• Vote

• Make medical decisions and sign consent forms

• Control who has access to their medical information

• Designate a Health Care Power of Attorney/ Health Care Agent

• Create an Advanced Directive/Living Will

➢ Advanced Care Planning

• Healthcare power of attorney ("surrogate"): Documents the person you select to be your voice for your healthcare decisions if you cannot speak for yourself.

• Living will and advanced directives:

Documents the kinds of medical treatments you would or would not want at the end of life.

Tools and resources:

• National Health Decisions Day web site: http://www.nhdd.org• Health Decisions Resources (ABA Commission on Law and Aging):

http://www.americanbar.org/content/dam/aba/uncategorized/2011/2011_aging_hcdec_art_advplarescources.authcheckdam.pdf

Tips for College

• Check out health facilities at potential schools before enrolling!

• Medical records should be on file at college’s health services

• Have a plan for how you will order medications

• Consider registering with the Office of Disability/Special Accommodations (if indicated)

– Letter from your doctor explaining need for accommodation

– 504 plan?

– Disclosure: who else needs to know?

(Modified from (http://www.chop.edu/ser

vice/gastroenterology-hepatology-and-

nutrition/transition/)

Tips for Employment

• Identify employment goals and learn how to apply for a job or vocational services

• Consider disclosure issues: if, when, who

• Obtain accommodation and support needs

• Great resource: Job Accommodation Network (JAN): www.askjan.org

Optimizing Transition: Take Home Tips for Parents

➢ Start early

➢ Provide age-level appropriate information

➢ Don’t be afraid to give your child responsibilities

➢ Role model skills you want your child to develop

➢ Provide coaching and opportunities for practice

➢ Encourage teen to communicate directly with health care providers

➢ Encourage teen to prepare for and make their appointments

➢ Plan for transition in insurance coverage

➢ Help teen understand the future health implications of their condition

➢ Discuss career and educational options

Conclusions

• Pediatric-Adult Transition is the purposeful, planned movement of youth with special health care needs from child-centered to adult-oriented care

• Transition-related knowledge and skills are ideally learned over time (a process not an event) and should be tailored to the individual and their developmental stage

• There are many excellent tools available to assist youth and their parents with this process

• Youth who experience a successful transition are more likely to live up to their full potential, have better health outcomes, higher self-esteem, and lead more fulfilling lives.

Pediatric & Adult

Comprehensive BMF Center

CHOP & UPENN

Pediatric patients:

Physicians: Tim Olson MD/PhD, Helge Hartung MDResource Coordinator: Millicent MurrayNurse Coordinator: Beverly PaulThe Children's Hospital of Philadelphia, 3615 Civic Center Blvd, ARC 302Philadelphia, PA 19104Phone: 267-426-9889 Fax: 267-426-9892Email: MurrayM3@email.chop.eduwww.chop.edu/service/hematology/our-programs/bone-marrow-failure/home.html

Adult patients:

Physician: Daria Babushok MD/PhDPerelman Center for Advanced Medicine West Pavilion, 2nd Floor 3400 Civic Center BLVDPhiladelphia, PA 19104Phone: (215) 615-5858Online appointments: https://www.pennmedicine.org/providers/profile/daria-babushok

Selected Resources:

• Got Transition? National Health Care Transition Center: http://www.gottransition.org/

• Health Care Transitions Initiative http://hctransitions.ichp.ufl.edu/

• Florida Health and Transition Services (Florida HATS) http://www.floridahats.org/

• The Hospital for Sick Children (Toronto)- SickKids, Good 2 Go Transition Program http://www.sickkids.ca/good2go/