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Improving the Transition from Pediatric to Adult Care for Adolescents and Young Adults with Autoinflammatory Diseases

  • Jonathan S. HausmannEmail author
  • Kitty O’Hare
Chapter

Abstract

The genetic basis for most autoinflammatory diseases (AIDs) makes them unique in rheumatology because most patients will be diagnosed during childhood and will continue to have active disease during adulthood. As a result, adolescents and young adults (AYAs) will need to transition to an adult rheumatologist at some point during their medical care. Transition comes at a particularly sensitive time for patients with AIDs, but it also brings opportunities for emotional growth and social development. In this chapter, we review essential skills that must be mastered by AYAs with AIDs to facilitate the process of transition, including the development of self-management skills and medical decision-making. We also address important aspects of the medical care for patients with AIDs, including long-term sequelae, issues related to pregnancy, primary care, stress management, attending college, and the development of a transition plan for pediatric and adult rheumatology practices.

Keywords

Transition to adult care Autoinflammatory diseases Adolescents and young adults Pediatrics Rheumatology College Self-management Care plan 

References

  1. 1.
    Bodar EJ, van der Hilst JCH, Drenth JPH, et al. Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome: introducing a vaccination provocation model. Neth J Med. 2005;63:260–4.Google Scholar
  2. 2.
    Bodenheimer T, Lorig K, Holman H, Grumbach K. Patient self-management of chronic disease in primary care. JAMA. 2002;288:2469–75.  https://doi.org/10.1080/13814788.2017.1306516.CrossRefPubMedGoogle Scholar
  3. 3.
    Britto MT, Garrett JM, Dugliss MAJ, et al. Preventive services received by adolescents with cystic fibrosis and sickle cell disease. Arch Pediatr Adolesc Med. 1999;153:27–32.  https://doi.org/10.1001/archpedi.153.1.27.CrossRefPubMedGoogle Scholar
  4. 4.
    Centers for Disease Control and Prevention. Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine for adults with immunocompromising conditions: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rep. 2012;61:816–9.Google Scholar
  5. 5.
    Centers for Medicare Medicaid Services (2017) Young Adults and the Affordable Care Act: protecting young adults and eliminating burdens on families and businesses – Centers for Medicare & Medicaid Services. In: www.cms.govCCIIOResourcesFilesadultchildfactsheet.html. Accessed 25 Dec 2017.
  6. 6.
    Christman MP, Castro-Zarraga M, DeFaria Yeh D, et al. Adequacy of cancer screening in adult women with congenital heart disease. ISRN Cardiol. 2013;2013:827696.  https://doi.org/10.1155/2013/827696.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Gabriel P, McManus M, Rogers K, White P. Outcome evidence for structured pediatric to adult health care transition interventions: a systematic review. J Pediatr. 2017;188:263–269.e15.  https://doi.org/10.1016/j.jpeds.2017.05.066.CrossRefPubMedGoogle Scholar
  8. 8.
    ter Haar NM, Jeyaratnam J, Lachmann HJ, et al. The phenotype and genotype of mevalonate kinase deficiency: a series of 114 cases from the Eurofever registry. Arthritis & Rheumatology. 2016;68:2795–805.  https://doi.org/10.1002/art.39763.CrossRefGoogle Scholar
  9. 9.
    Hardin AP, Hackell JM, Committee on Practice and Ambulatory Medicine. Age limit of pediatrics. Pediatrics. 2017.  https://doi.org/10.1542/peds.2017-2151 CrossRefGoogle Scholar
  10. 10.
    Hausmann JS, Dedeoglu F. Autoinflammatory diseases in pediatrics. Dermatol Clin. 2013;31:481–94.  https://doi.org/10.1016/j.det.2013.04.003.CrossRefPubMedGoogle Scholar
  11. 11.
    Hersh AO, Pang S, Curran ML, et al. The challenges of transferring chronic illness patients to adult care: reflections from pediatric and adult rheumatology at a US academic center. Pediatr Rheumatol. 2009;7:13.  https://doi.org/10.1186/1546-0096-7-13.CrossRefGoogle Scholar
  12. 12.
    Hilderson D, Moons P, Van der Elst K, et al. The clinical impact of a brief transition programme for young people with juvenile idiopathic arthritis: results of the DON'T RETARD project. Rheumatology (Oxford). 2016;55:133–42.  https://doi.org/10.1093/rheumatology/kev284.CrossRefGoogle Scholar
  13. 13.
    Jensen PT, Karnes J, Jones K, et al. Quantitative evaluation of a pediatric rheumatology transition program. Pediatr Rheumatol. 2015;13:17.  https://doi.org/10.1186/s12969-015-0013-0.CrossRefGoogle Scholar
  14. 14.
    Lachmann HJ, Papa R, Gerhold K, et al. The phenotype of TNF receptor-associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the Eurofever/EUROTRAPS international registry. Ann Rheum Dis. 2014;73:2160–7.  https://doi.org/10.1136/annrheumdis-2013-204184.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Lawson EF, Hersh AO, Applebaum MA, et al. Self-management skills in adolescents with chronic rheumatic disease: a cross-sectional survey. Pediatr Rheumatol. 2011;9:35.  https://doi.org/10.1186/1546-0096-9-35.CrossRefGoogle Scholar
  16. 16.
    Lemly DC, Lawlor K, Scherer EA, et al. College health service capacity to support youth with chronic medical conditions. Pediatrics. 2014;134:885–91.  https://doi.org/10.1542/peds.2014-1304.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Leslie KS, Lachmann HJ, Bruning E, et al. Phenotype, genotype, and sustained response to anakinra in 22 patients with autoinflammatory disease associated with CIAS-1/NALP3 mutations. Arch Dermatol. 2006;142:1591–7.  https://doi.org/10.1001/archderm.142.12.1591.CrossRefPubMedGoogle Scholar
  18. 18.
    McDonagh JE, Southwood TR, Shaw KL, British Society of Paediatric and Adolescent Rheumatology. The impact of a coordinated transitional care programme on adolescents with juvenile idiopathic arthritis. Rheumatology. 2007;46:161–8.  https://doi.org/10.1093/rheumatology/kel198.CrossRefPubMedGoogle Scholar
  19. 19.
    McManus M, White P, Barbour A, et al. Pediatric to adult transition: a quality improvement model for primary care. J Adolesc Health. 2015;56:73–8.  https://doi.org/10.1016/j.jadohealth.2014.08.006.CrossRefPubMedGoogle Scholar
  20. 20.
    McManus MA, Pollack LR, Cooley WC, et al. Current status of transition preparation among youth with special needs in the United States. Pediatrics. 2013;131:1090–7.  https://doi.org/10.1542/peds.2012-3050.CrossRefPubMedGoogle Scholar
  21. 21.
    Park MJ, Scott JT, Adams SH, et al. Adolescent and young adult health in the United States in the past decade: little improvement and young adults remain worse off than adolescents. J Adolesc Health. 2014;55:3–16.  https://doi.org/10.1016/j.jadohealth.2014.04.003.CrossRefPubMedGoogle Scholar
  22. 22.
    Pelagatti MA, Meini A, Caorsi R, et al. Long-term clinical profile of children with the low-penetrance R92Q mutation of the TNFRSF1A gene. Arthritis Rheum. 2011;63:1141–50.  https://doi.org/10.1002/art.30237.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Sharma N, O'Hare K, Antonelli RC, Sawicki GS. Transition care: future directions in education, health policy, and outcomes research. Acad Pediatr. 2014;14:120–7.  https://doi.org/10.1016/j.acap.2013.11.007.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Sharma N, O'Hare K, O'Connor KG, et al. Care coordination and comprehensive electronic health records are associated with increased transition planning activities. Acad Pediatr. 2017.  https://doi.org/10.1016/j.acap.2017.04.005.CrossRefGoogle Scholar
  25. 25.
    Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med. 1967;43:227–53.CrossRefGoogle Scholar
  26. 26.
    The Six Core Elements of Health Care Transition. URL: http://www.gottransition.org/resourceGet.cfm?id=206. Accessed 30 Dec 2017.
  27. 27.
    Toplak N, Frenkel J, Ozen S, et al. An international registry on autoinflammatory diseases: the Eurofever experience. Ann Rheum Dis. 2012;71:1177–82.  https://doi.org/10.1136/annrheumdis-2011-200549.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Tran T-A, Koné-Paut I, Marie I, et al. Muckle-Wells syndrome and male hypofertility: a case series. Semin Arthritis Rheum. 2012;42:327–31.  https://doi.org/10.1016/j.semarthrit.2012.03.005.CrossRefPubMedGoogle Scholar
  29. 29.
    van der Hilst JCH, Bodar EJ, Barron KS, et al. Long-term follow-up, clinical Features, and quality of life in a series of 103 patients with hyperimmunoglobulinemia D syndrome. Medicine. 2008;87:301–10.  https://doi.org/10.1097/MD.0b013e318190cfb7.CrossRefPubMedGoogle Scholar
  30. 30.
    Walker UA, Hoffman HM, Williams R, et al. Brief report: severe inflammation following vaccination against streptococcus pneumoniae in patients with cryopyrin-associated periodic syndromes. Arthritis Rheumatol. 2016;68:516–20.  https://doi.org/10.1002/art.39482.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Wurster VM, Carlucci JG, Feder HM, Edwards KM. Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. J Pediatr. 2011;159:958–64.  https://doi.org/10.1016/j.jpeds.2011.06.004.CrossRefPubMedGoogle Scholar
  32. 32.
    Youngstein T, Hoffmann P, Lane T, et al. International experience of pregnancy outcomes in auto-inflammatory syndromes treated with Interleukin-1 inhibitors. Pediatr Rheumatol. 2015;13:O67.CrossRefGoogle Scholar
  33. 33.
    Zemer D, Livneh A, Danon YL, et al. Long-term colchicine treatment in children with familial mediterranean fever. Arthritis Rheum. 1991;34:973–7.  https://doi.org/10.1002/art.1780340806.CrossRefPubMedPubMedCentralGoogle Scholar

Additional Resources

  1. American College of Rheumatology Pediatric to Adult Rheumatology Care Transition Toolkit.https://www.rheumatology.org/Practice-Quality/Pediatric-to-Adult-Rheumatology-Care-Transition.
  2. Arthritis Foundation Transition Toolkit. http://www.jiatransition.org.
  3. Autoinflammatory Diseases Transition Tools and Templates. http://autoinflammatorydiseases.org/transition.
  4. BC Children’s Hospital ON TRAC, Transitioning Responsibly to Adult Care. http://ontracbc.ca.
  5. George Washington University Directory of Transition Websites. https://www.heath.gwu.edu/directory-of-transition-websites.
  6. Got Transition. http://gottransition.org.
  7. National Collaborative on Workforce and Disability A Young Person’s Guide to Health Care Transition. http://www.ncwd-youth.info/sites/default/files/Young-Persons-Guide-to-Health-Care-Transition.pdf.
  8. Transition Readiness Assessment Questionnaire. www.etsu.edu/com/pediatrics/traq/.
  9. University of Washington Adolescent Health Transition Project. http://depts.washington.edu/healthtr.

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Autoinflammatory Diseases Clinic, Boston Children’s HospitalBostonUSA
  2. 2.Autoinflammatory Disease Center, Beth Israel Deaconess Medical CenterBostonUSA
  3. 3.Harvard Medical SchoolBostonUSA
  4. 4.Transition Medicine for Primary CareWeitzman Family BRIDGES Adult Transition Program, Boston Children’s HospitalBostonUSA
  5. 5.Pediatric Population Health, Brigham and Women’s HospitalBostonUSA

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