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Cystic Fibrosis Care in 2017:
Overcoming Challenges to
Capitalize on Success!
Gregory Sawicki, MD, MPH
Director, Cystic Fibrosis Center
Division of Respiratory Diseases
Boston Children’s Hospital
Assistant Professor of Pediatrics
Harvard Medical School
CF: A Story of Progress
1950
1989
2015
CF: A Changing Population
CF Foundation Patient Registry, 2014
Adolescence is a high risk period in CF
CF Foundation Patient Registry, 2014
Living with CF ……
A Case Study
Diagnosis
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Failure to thrive at age 6 months led PCP to refer for sweat test
Chronic nasal congestion
Positive sweat test with CFTR genotype: F508del homozygous
Social history:
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Has an older sister who does not have CF
Father is an attorney
Mother is a teacher
Has had private health insurance through his father’s employer
– Parents surprised by diagnosis, express need for support, mom
stops working to care for her son instead of utilizing daycare
Infancy - Preschool
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Started pancreatic enzymes at time of diagnosis
Weight increased from 10%ile to 30%ile by age 2
Had reflux as an infant
Described as a “picky eater”, often refusing meals
Started chest PT intermittently at age 8 months
Attended daycare from age 1 until preschool at age 4
Initiated Dornase alfa therapy at age 4
Parents decide to have a third child, request genetic
counseling, no pre-natal screening available at the time
Elementary School
– School requires him to go to nurse to obtain enzymes. Often
forgets.
– First time Pseudomonas detected at age 5, treated with inhaled
antibiotics for eradication therapy
– No further Pseudomonas found in subsequent throat cultures
– In second grade began participating more in soccer, parents of
other children ask whether he is “contagious” due to his cough
– Reported intermittent cough, but was the fastest kid on his team
– Age 5 began daily chest PT with Vest
– First attempts at lung function at age 5 with FEV1 102%
predicted
– Never hospitalized
Middle School
– Age 12: Has soccer practice / games 4 days per week and family
reports that they don’t have time for the Vest on those days
– BMI has dropped from 40%ile to 35%ile
– Denies any new GI symptoms
– Parents state that they pack his lunch every day with enzymes in
his bag
– FEV1 at age 12: 95% predicted
– First hospitalization due to increased symptoms, decreasing
weight, and re-growth of Pseudomonas
High School
– Continues to play competitive soccer, also lacrosse
and basketball
– Prescribed alternate month inhaled antibiotics,
hypertonic saline, dornase alfa, and daily chest PT
– BMI continues to drop – 20%ile at age 14
– Reports “forgetting” his enzymes and doesn’t think he
needs them anymore
– Develops influenza at age 15 requiring hospitalization
– Starts a summer job as a golf caddy
– Straight A student, aspires to attend college to study
engineering
– Starts dating girls at age 16, does not tell friends
(except for his 2 best friends) about CF
College
– Comes to CF clinic only while home on holiday
breaks
– Requests single room accommodation in order to
complete treatments in private
– Reports feeling pretty good, and is surprised with a
decrease in FEV1 (70%) and BMI% 10.
– Reports that living away from home for the first time
while juggling a full load of classes, and social life has
made it difficult to manage all his treatments.
– Parents ask whether he should move back home to
attend a local school instead of living away from
home in a dorm
– Meets adult CF care team for the first time
Post-College
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Baseline FEV1 60% predicted
Chronic infection with Pseudomonas
Care now coordinated by an adult CF Center
Hospitalized yearly since age 23 for CF exacerbation
Works in a research laboratory as a technician, hoping to
apply to graduate schools
Engaged, considers fiancée his primary support person
Considering having children
Medication regimen: Inhaled tobramycin, Dornase alfa,
Azithromycin, Inhaled sodium chloride, Pancreatic
enzymes, Multivitamin plus minerals, Fluticasone
propionate , Esomeprazole
Consideration made for initiation of lumacaftor/ivacaftor
Challenges to CF Care Through the Life Span
Adulthood
Adolescence
Childhood
Infancy
Diagnosis
Adulthood
Adolescence
Childhood
Newborn
Screening
Diagnosis
Infancy
Infant Care
Practices
Nutritional Assessment
Pulmonary Function Assessment
Monitoring for Complications
Adulthood
Prevention of Disease Progression
Education About CF
Assessment of Family Functioning
and Social Supports
Childhood
Newborn
Screening
Diagnosis
Infancy
Infant Care
Practices
Adolescence
Nutritional Assessment
Pulmonary Function Assessment
Monitoring for Complications
Adulthood
Prevention of Disease Progression
Education About CF
Assessment of Family Functioning
and Social Supports
Adolescence
Childhood
Transition
Newborn
Screening
Diagnosis
Infancy
Infant Care
Practices
Self-Management
Shared Decision-Making
Challenges to Achieving
Optimal CF Outcomes
• Adherence to Chronic Therapies
• Transition from Pediatric to Adult-Focused CF Care
Complexity of CF Treatment
Dietary &
vitamin
supplements
Pancreatic
enzyme
replacement
Chest
physiotherapy
Daily medical
treatments
High
Treatment
Burden
Respiratory
medications
Bregnballe, et al. Patient Prefer Adherence. 2011;5:507-15. Sawicki, et al. Pediatr Pulmonol.
2012;47(6):523-33.
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Daily Treatment Burden for Adults
Median Number of Therapies
Median Number of Minutes
(Total = 108)
Inhaled
Nebulized
Oral
1
Exercise
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2
4
Airway
Clearance
29
Total
Nebulized
41
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Sawicki, et al. J Cyst Fibros. 2009;8(2):91-6.
Oral
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Adherence in CF:
What do we know?
Some Terminology
• “Compliance”
– The accuracy with which a person follows the
regimen prescribed by a health professional
• “Adherence”
– Extent to which a person’s behavior coincides
with medical advice
• “Self-management”
– Health behaviors of individuals and families
use to care for a chronic condition
http://apps.who.int/iris/bitstream/10665/42682/1/9241545992.pdf
Modi AC et al. Pediatrics. 2012;129(2)e473–485.
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Adherence rates to CF respiratory
medications are low
MPR=0.5
Quittner AL et al. Chest 2014;146(1):142–151.
Adherence rates vary by age
Quittner AL et al. Chest 2014;146(1):142–151.
Adherence in adolescents is best on
weekdays during school term time
Fig. 1 Comparison of adherence to treatment for individual patients during a) weekdays and weekends and
b) holidays and term-times. The horizontal thickened bars represent mean adherence for the group (*p<0.001).
Ball R et al. Journal of Cystic Fibrosis 2013;12(5) 440–444.
Challenges to adherence and
self-management
Individual
Family
• Age
• Gender
• Health literacy
• Disease & treatment knowledge
• Mental health / behavioral problems
• Coping style
• Health beliefs & perceptions
• Family structure
• Income / health insurance
• Disease knowledge
• Mental health / behavioral problems
• Coping style
• Health beliefs & perceptions
• Relationship quality
• Involvement in care
Health Care System
Community
• Access to care
• Continuity of care
• Patient-provider communication
• Shared decision making
• Frequency of clinic visits
• Provider biases
• Neighborhood
• Work (hours & policies)
• School
• Peer support
• Illness stigma
Adapted from Modi AC et al. Pediatrics. 2012;129(2)e473–485.
Adherence Typologies
Unwitting
– Patient and provider mistakenly believe that the
patient is adherent
Erratic
– Patient understands and agrees with therapy but
has difficulty consistently maintaining regimen
“Rationalized”
– Patient deliberately alters or discontinues
therapy
Adherence in CF:
Measurement matters!
• Self-report
– Daily diaries
– Questionnaires
– Interviews
• Clinician-report
– Questionnaires
• Pharmacy records
– Medication Possession
Ratio (MPR)
– Proportion of days covered
(PDC)
– Number of refills
• Electronic monitors
– MEMS Caps
– “Chipped” devices
– MDI monitors
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Identifying non-adherence is challenging
PATIENT REPORT
PROVIDER REPORT
0
Daniels T et al. Chest 2011;140(2):425–432.
Adherence in CF:
Adolescent Perspectives
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Barriers
Immediate time pressures
– Lack of time
– Uncertain schedules
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Competing priorities
– Balancing time trade-offs
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Privacy concerns
– Wanting to be “normal”; not wanting
to seem different or disabled
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Lack of perceived
consequences
– Not seeing an impact on one's
health right away from skipping
treatments or medications
Facilitators
• Improving understanding of
the importance of therapies
• Fostering relationships with
the CF care team
– CF team should be creative in
problem-solving with the
adolescent and parent
• Empowering adolescents
– Enabling parents to cede control
and entrust responsibility to
adolescents
• Establish a structure
– Having a daily routine, “making it
a ritual”
Sawicki GS et al. Pediatr Pulmonol , 2015 Feb;50(2):127-36.
What Can Be Done?
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Reframe Adherence to Sustain Daily Care
Shift from adherence in aAdherence
linear view  Sustaining Daily Care
Care team –
Patient Family
Interaction
Treatment
plan
Expectation of
adherence to
treatment
plan
To a cyclical view of Sustaining Daily Care
Ongoing
collaborative
conversation
between care
team and
patient/family
Adapting
plan into
daily life
Co-create
the right
treatment
plan
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CFF Partnerships for Sustaining Daily Care
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CFF Success with Therapies
Research Consortium
To facilitate the clinical study of interventions to improve
adherence and CF disease self-management in order to
optimize health outcomes and quality of life.
Interventions will be developed, implemented, and disseminated to:
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Include measures of adherence and self-management that are validated
for use in CF.
Be patient-centered and empower individuals with CF.
Be tailored to people’s unique strengths and needs.
Be feasible, practical and adaptable.
Leverage collaborations with family, CF Care team, and community.
STRC Sites and Members
17 Institutions
 13 Pediatric programs
 8 Adult programs
31 Principal Investigators
 20 Physicans
 10 PhDs – Psychology, Health Economics,
Health Education
 1 PharmD
Health Care Transition:
A Health System Dilemma
The Current State of Transition in the U.S.
Pediatric
Care
Faith, Trust,
and Pixie
Dust
Adult
Care
Operational Definition of
Health Care Transition
A purposeful planned process that supports
adolescents and young adults with chronic health
conditions and disabilities to move from childcentered (pediatric) to adult-oriented health-care
practices, providers, programs, and facilities.
Reiss & Gibson, Pediatrics. 2002 Dec;110(6 Pt 2):1307-14.
Goals of Health Care Transition
• Promote autonomy, self-care and selfdetermination
• Allow assumption of adult roles and responsibilities
• Maximize life-long functioning and potential
• Incorporate lifespan developmental perspectives
and addresses knowledge and skills
• Transfer of care from child-oriented to adultoriented services with uninterrupted services (when
appropriate)
TRANSITION
≠ TRANSFER
Should we be transferring everyone?
Adults, including those with childhoodacquired chronic conditions, should
receive adult-oriented primary health
care from appropriately trained and
certified providers, in adult health care
settings
Journal of Adolescent Health 2003; 33:309–311
Consensus Statements on
Health Care Transition
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Identify a health provider to coordinate transition
Train health care providers in transition services
Maintain a portable medical summary
Create a written health transition plan
Apply standard primary care guidelines to CSHCN
Ensure affordable, continuous health insurance
Pediatrics 2011;128:182-200
Time for a Reality Check!
National Survey of US CF Centers: 2007
• Transfer of care occurs at median age of 19, but initial discussion
begins at median age of 17
• Fewer than half of CF programs perform readiness assessments
• Fewer than 10% have written list of desirable self-management skills
• Fewer than 2/3rds discuss current sexual activity or fertility intention
• Half of programs never or rarely prepare medical summaries
McLaughlin, S. E. et al. Pediatrics 2008;121:e1160-e1166
What is the Problem?
Obstacles to Health Care Transition
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The youth
The parent(s) / family
The pediatric team
The adult team
The health system
Patient-Family Level Barriers
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Accessing care: primary and specialty care
Knowledge and Skills
Insurance
Family / Individual resistance
• Familiarity with the pediatrician / Fear of a new team or facility
• Need for family to maintain control
• Perception adolescent cannot handle condition
Health System Barriers:
Are Pediatricians are From Mars,
and Internists From Venus?
Barriers for Pediatricians
• Lack of available adult specialists
• Lack of pediatric staff skills in transition planning
• Difficulty in breaking bond between pediatricians and
adolescents and their parents
• Lack sufficient pediatric staff time to provide transition
services
• Lack of adolescent knowledge about health conditions
and/or skills to self advocate during physician visits
Barriers for Internists
• Training
• lack of training in congenital and childhood-onset conditions
• lack of adolescent medicine training
• Working with families
• lack of family involvement
• families' high expectations
• Practice management
• difficulty meeting patients' psychosocial needs
• facing disability/end-of-life issues during youth and early in
the relationship
• financial pressures limiting visit time
There Are Many System Barriers
System
Providers
Patient Protection and Affordable Care Act:
Can it Improve Transition?
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Subsidies for 133%-400% Federal Poverty Level
No lifetime coverage limits
Can’t deny coverage for pre-existing conditions
Can keep children on parents’ policies until age 26
Can’t cancel policies because of illness
Encouragement of Patient Centered Medical Home
Key Questions to Consider
• What can parents do from an early age, to encourage a
smooth transition?
• What parenting traits and styles inhibit a smooth
transition?
• How can families and medical teams work with an
adolescent or young adult who resists, protests or
otherwise makes transition more difficult?
• By what ages would you expect patients to be
independent in certain treatment tasks?
• How can patients and families work with medical team
members in their transition process?
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The Goal: A Comprehensive
HCT Process
Active Transfer
Therapeutic Discharge
Empathic Intake
Pediatrics
Preparation
for Transfer
Adult care
Planned
Hand-off
Establish YA
in Adult System
A non-CF focused resource:
Got Transition!
www.gottransition.org
CF R.I.S.E.:
A CF-Specific Resource
THIS INFORMATION MEETS THE GUIDELINES AND
STANDARDS OF THE OF THE CF FOUNDATION’S
EDUCATION COMMITTEE.
CF R.I.S.E. was developed in collaboration with
a multidisciplinary team of CF experts and is
sponsored by Gilead.
Summary
• Health outcomes in CF have been improving for decades
• Many more adolescents with CF continue to reach adulthood
• Novel therapies continue to be developed
• Non-adherence to chronic CF therapies is common and is
linked to poor health outcomes
• “Precision medicine” for CF needs to focus beyond genetic
therapies
• Interventions to improve adherence and transitional care need
to be developed, studied, and implemented on a larger scale
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Thank You!
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