Lynne A Wolfe MS CRNP BC Undiagnosed Diseases program Epi743 MitoWorks trial Congenital Disorders of Glycosylation July 26 2014 Metabolic Diseases 3001500 known Inborn Errors of Metabolism ID: 1045340
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1. Transitions: Growing up with a Special Health NeedLynne A. Wolfe, MS, CRNP, BCUndiagnosed Diseases programEpi-743 MitoWorks trialCongenital Disorders of GlycosylationJuly 26, 2014
2. Metabolic Diseases 300-1500 known Inborn Errors of Metabolism50 % of them have been discovered in the last 25 years and more continue to be discoveredIncidence of 1:4000 live births (1000 live births/year)Metabolic disorders are a major cause of chronic illness in childhood
3. Impact Inherited Metabolic DisordersAccount for 5-6 % of SIDS casesRequire Life-long care !Now reaching Childbearing AgeIncreased Maternal risk of HELLP syndrome or Fatty Liver of PregnancyAdult Sequelae – Learning problems, Vision loss, Cardiomyopathy, Peripheral Neuropathy, Chronic Liver disease (?), Depression & Anxiety disorders, pregnancy
4. Impact Inherited Metabolic DisordersChildren with metabolic disorders are hospitalized 3-4 times more often than other childrenAccount for 12% of Pediatric Admissions (50% of Pediatric Admissions have a Genetic Disease) Account for 55% of overnight staysIncrease LOS on average of 3 daysIncur 184% of Inpatient costs4-5% higher in hospital mortality rates40% of overall Childhood mortality related to Genetic diseases
5. Importance of Definitive CareCostly, poor outcomes when not treatedCollectively, not that rareMore and more are treatableFull characterization can be used for future Prenatal DiagnosisProvides opportunity to future care planningLong-term follow provides valuable natural history information that can be used to develop therapies
6. A team is needed to raise these childrenParents/other family membersPCP – Medical HomeMetabolic TeamPT, OT, SLP (local Early Intervention team)Developmental PediatricianGastroenterologyCardiologyOpthalmologyNeurologyTransplant TeamSpecial needs DentistPharmacist/Medical Supply company
7. Maternal and Child Health Bureau (1998)Defined Children with Special Health Needs as: “Those children who have or at increased risk for a chronic physical, developmental, behavioral, or emotional condition and who require health and related services of a type or amount beyond that required by children generally.”
8. Olmstead Act of 1999Operationalized Americans with Disability Act 1990 (ADA)“reasonable efforts” by StatesPresidents New Freedom Initiative 2001Increased assistive technology accessIncreased educational opportunitiesPromoted home ownership/community involvementEncouraged “integrated” workforceIncreased transportation options
9. Surgeon General’s “Call to Action” 2005Assess trajectory of disabilitiesIncrease knowledge of Healthcare providersSupported self-management & health promotionIncreased Healthcare & Social Services access
10. President Bush’s Executive order February 2008Implement strategies to improve health of American youth including encouraging Inter-agency cooperation and improved community-based resourcesEncouraged development of youth oriented websites & support groups
11. Maternal and Child Health BureauCharged with providing services since 1935 through Title V of the Social Security Act2004 Transitional Care became 1 of 6 Core Outcomes: “Youth with special health care needs will receive the services necessary to make appropriate transitions to adult health care, work and independence.”
12. Patient Protection and Affordable Care Act (ACA) 2010Increased AccessNo Pre-existing condition limitations for children under 19 years of ageNo life-time capsNo caps on Out-of-pocket costsEssential services now incldude Dental, Vision & HabitationDependent coverage up to 26 years of age
13. Patient Protection and Affordable Care Act (ACA) 2010Tranformng Pediatric Health Care Mandated Health Care teams which is critical to children with special health needs who need access to multi-disciplinary teamsMandated access to Clinicians with expertise in Pediatrics
14. Scope of the Problem54 million people or 20% of the U.S. population12 million children in the U.S.Pediatric medical care costs $300 billion annually or 4% of the gross domestic product (adult medical care $2.4 trillion)50% of the cost is related to medical expenses50% of the cost is related to lost productivityFor the total population with disabilities there is an increased risk for secondary health problems due to lack of regular health promotion & screening.
15. Scope of the Problem60% male68% Non-Hispanic white45% have “Medical Home” resources84% have normal activities disrupted42% have thought about shifting care62% have “some knowledge” of anticipated health problems34% have some knowledge of insurance issues
16. Scope of the Problem78% of children with special health needs are encouraged to take responsibility for their own careNon-english speaking, low-income at greatest risk of not making successful transitionMost significantly impacted by special health needs are also at risk for unsuccessful transitionsFemales more likely than males to have successful transitions
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18. ConsiderationsAssume responsibility for personal careAnticipate future health needsObtain future healthcare providersPlan for Health Insurance changes
19. PCP – Medical HomePatient SafetyEffectiveness* Efficiency Family-Provider Partnership*Health Status*Timeliness*EquityCost*
20. PCP – Medical HomeEffectiveness Adherence to plan of care (medications, diet)Fewer hospitalizations and/or decreased length of stayFewer ED visitsFamily-Provider PartnershipIncreased Self managementImproved SatisfactionDecreased Family stress/improved Family functioningHealth StatusFewer School or Work days missedFewer unmet needs
21. PCP – Medical HomeTimelinessLess time to have phone calls returnedLess time to get an appointmentSame day appointment availabilityAccess to Provider after hours CostDecreased Short-term costsDecreased Long-term costs
22. The Hand-off: Goal is a seamless hand-offTraditional separation of Pediatric and Adult careInternal Medicine specialists most comfortable with disorders they see in Adult patients already: Diabetes, Obesity, Cancer, Intellectual disability etc…Lack of preparation at the receiving end Lack of knowledge at the hand-off end
23. The Hand-off: Goal is a seamless hand-offEmotional Rapport between Pediatric Providers & families is a strength & weaknessReimbursement and Administrative Barriers to Co-managementModels exist: Geriatric-End of Life Case Management Childhood Cancer Survivor careCystic FibrosisCongenital Heart Disease
24. What is needed ?Children with Special Health Care needsParents/GuardianshipPCP (Medical Home)Current SpecialistsProfessional OrganizationsState & Federal Resources
25. Role of Child with the Special Health NeedBe aware of Age of MajorityLearn about their health care needsActively participate in keeping themselves healthyAcquire necessary skills to implement the health care planBecome their own advocateBe able to obtain all necessary services
26. Parents RoleTeach, encourage as we do with every other transitionCall for an appointment, lab test or to refill a medicationArrange transportationKnow Medications & dosages like you know your address & phone numberDispense medications, mix formulas, cook meal for the family, make lunch for school or workDiscuss sexuality & pregnancyHave an emergency plan in place
27. Birth to ThreeDevelop trustTake breaks to maintain your energy & healthBegin record keeping of early childhood interventions, medical history, surgeries and injuries, immunizations, medications, special diets, allergies & adverse drug eventsCreate list of Specialists involved in your child’s careApply for Medicaid Waiver whenever possible
28. Three to FiveDevelop decision-making skills by offering choicesEncourage participation in household choresGet involved in recreational & community activitiesBegin to teach self-care related to chronic illness Encourage interaction with therapists, nurses & doctors Begin teaching about personal space & relationships“What do you want to do when you grow up?”
29. Six to ElevenStrengthen knowledge of chronic illnessStrengthen self-care abilitiesDiscuss personal safetyConsider 504 Plan or IEP needsEncourage Hobbies & Leisure activitiesBegin shopping with the childDiscuss consequences of poor choicesTeach self-advocacy skills
30. Twelve to EighteenFill in gaps about special health needsEncourage self-careInclude adolescent in 504 & IEP plansSupport ordering their own medications & supplies, calling for appointments etc…Discuss sexualityConsider special needs Camp attendenceExplore insurance changesPlan for Provider changes
31. After EighteenAssume responsibility for getting health needs metParent should remain resource & social supportContinue Hobbies & leisure activitiesEncourage Support Group involvementConnect to community-based or college-based disability servicesConsider contacting Department of Vocational Rehabilitation
32. Parents RoleLong-term PlanningGuardianshipIEP inclusionVocational training/other secondary educationSupervised Living ArrangementsEstate Planning
33. Barriers to the Medical Home and Transitional Care MovementLack of TrainingSpecific conditionsCultural SensitivityLack of Care Coordination ToolsLack of knowledge or access to Patient Registeries & Support organizationsLack of key personnel: Care Coordinator, Social Worker, DietitianTime
34. Resourceswww.childhealthdata.org/learn/NS-CSHCNhttp://www.medicalhomeinfo.org/how/care_delivery/cyshcn.aspxhttp://www.cms.gov/www.familyvoices.orghttp://www.socialsecurity.gov/disabilityresearch/index.htmlhttp://hdwg.org/catalyst/
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