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Sickling smoothly through life…

Sickling smoothly through life…. Nirmish Shah, MD Assistant Professor Director of Sickle Cell Transition Program Duke University. Educating patients about transition. Outline. Introduction Challenges facing transitioning SCD patients

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Sickling smoothly through life…

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  1. Sickling smoothly through life… Nirmish Shah, MD Assistant Professor Director of Sickle Cell Transition Program Duke University Educating patients about transition

  2. Outline • Introduction • Challenges facing transitioning SCD patients • Model and key components for a successful transition to adulthood • ‘Who, What, Where’ – levels of patient understanding

  3. Who • Medicine-Pediatrics residency at ECU • 2000-2004 • Goal was to do med/peds hem/onc…. ….then reality kicked in and hematology seemed more realistic and interesting

  4. What • Med/Peds Hematologist • Joint appointment between medicine and pediatrics • Have outpatient clinics in both medicine and pediatrics • Do inpatient consults for adult hematology • Majority of effort in research

  5. Where • ‘Where do I go’… • Goal to perform combined pediatric and adult research • Improve transition guidelines and protocols • Advocate co-management of SCD with primary care providers

  6. Introduction • Sickle Cell Disease (SCD) is the most common inherited disease worldwide • Recent advances include prophylactic PCN, improved immunizations, and increased use of hydroxyurea

  7. Survival Deaths due to sickle cell anemia or sickle cell trait in 1967 Scott, R. Health Care Priority and Sickle Cell Anemia, JAMA, 1970, 214(4)

  8. Recent Developments Have Greatly Improved Patient Management and Survival1 60 1998 – STOP trial6 2012– SWiTCH trial8 1986 – Antibiotic prophylaxis in infants6 50 2011– BABY HUG trial7 40 Life expectancy for patients with SCA (years)2,3 1976 – RBC transfusion for treatment of secondary stroke5 30 1995– MSH Trial6 20 1987 – NIH recommends newborn screening6 1949 – Identification of HbS4 1910 - Discovery of SCD4 10 1910 1920 1930 1940 1950 1960 1970 1980 1990 2000 2010 BABY HUG, Pediatric Hydroxyurea Phase III Clinical Trial; NIH, National Institutes of Health; STOP, Stroke Prevention Trial in Sickle Cell Anemia. 1. Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Blood. 2010;115(17):3447–3452. 2. National Heart, Lung, and Blood Institute. Progress in Heart, Lung, and Blood Research: Reducing the burden of Sickle Cell Disease. August, 2011. https://www.nhlbi.nih.gov/news/spotlight/success/sicklecell.pdf. Accessed April 21, 2014. 3. Elmariah H, Garrett ME, De Castro LM, et al. Am J Hematol. 2014;89(5):530-535. 4. Zhou Z, Behymer M, Guchhait P. Anemia2011;2011:918916. 5. Lusher JM, Haghighat H, KhalifaAS. Am J Hematol. 1976;1(2):265-273. 6. National Heart, Lung, and Blood Institute. The Management of Sickle Cell Disease. 4th ed. Bethesda, MD: National Institutes of Health; 2002. NIH publication 02-2117. http://www.nhlbi.nih.gov/health/prof/blood/sickle/sc_mngt.pdf. Accessed April 9, 2014. 7. Wang WC, Ware RE, Miller ST, et al. Lancet. 2011;377(9778):1663–1672. 8. Ware RE, Helms RW. Blood. 2012;119(17):3925-3932.

  9. Challenges facing transitioning SCD patients

  10. Transitioning Poses Many Challenges for Patients Challenges for Any Patient With a Chronic Condition1-2 • Inability to effectively manage their disease • Need for effective coping strategies • Concerns about cost of care and insurance coverage • Uncertainty about future as an independent adult Challenges of Particular Concern for SCD Patients1-4 • Increased socioeconomic challenges • Large number of older SCD patients, placing strain on the system • Adult "transition patients" ending up in the pediatric emergency room • Lack of a medical home with anadult hematologist • Result: suboptimal care with poor tracking of medication and transfusion histories 1. Treadwell M, Telfair J, Gibson RW, et al. Am J Hematol. 2011;86(1):116-120. 2. Paulukonis S, Neumayr L, Coates TD, et al. Blood. 2010;116:Abstract 254. 3. McDonnell WM, Kocolas I, Roosevelt GE, Yetman AT. Arch Pediatr Adolesc Med. 2010;164(6):572-576. 4. National Heart, Lung, and Blood Institute. The Management of Sickle Cell Disease. 4th ed. Bethesda, MD: National Institutes of Health; 2002. NIH publication 02-2117. http://www.nhlbi.nih.gov/health/prof/blood/sickle/sc_mngt.pdf. Accessed August 23, 2013.

  11. Ineffective use of healthcare system • During 5 year study: • Children = <18 • Transition = turned 19 • Young Adults = 19-30 • Adults = >30 Hemker BG, Brousseau DC, Yan K, Hoffmann RG, Panepinto JA. When children with sickle-cell disease becomes adults: lack of outpatient care leads to increased use of the emergency department. Am J Hematol. 2011 Oct;86(10):863-5. doi: 10.1002/ajh.22106. Epub 2011 Aug 3

  12. Readmission Rates Also Spike After Transition • Approximately 80% of rehospitalizations were to the same hospital • 94% of pediatric readmissions • 77% of adult readmissions • Brousseau DC, Owens PL, Mosso AL, et al. JAMA. 2010;303(13):1288-1294.

  13. The Transition Years Are A Vulnerable Time in Life For Many SCD Patients • Deaths in patients 18 years of age or older in the 940-patient Dallas newborn cohort • There were 7 deaths between 2002 and 2007, all of which occurred in transition-age patients • Mean time between transition and death was 1.8 years *Transition was delayed beyond 18 years due to chronic renal failure. N/A, not applicable. • Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Blood. 2010;115(17):3447–3452.

  14. Complications of SCD Increase As Patients Transition and RBC Transfusions Decrease 5 0.6 Increased frequency of complications correlates with a decrease in the utilization of RBC transfusions and hydroxyurea 0.5 4 0.4 3 Mean Number with > 1 Diagnosis Per Patient Per Quarter 0.3 Mean Number of Transfusions Events and Prescriptions Per Patient Per Quarter 2 Any Complications 0.2 Mean Transfusions Pain Infection 1 0.1 0 0 40 50 5 35 45 15 30 0 10 25 20 Age N= (555) (600) (662) (656) (505) (339) (274) (239) (172) (90) • Blinder MA, Vekeman F, Sasane M, et al. PediatrBlood Cancer. 2013;60(5):828–835.

  15. Risk factors for unsuccessful transition • Associated with successful transition (1 adult visit): • Transferring to adult care <21 (p=0.008) • Distance <20 miles (p=0.01) • Severe phenotype - SS/Sβ0 (p=0.04) • Being on chronic transfusions (p=0.02) 32% did not transfer successfully Andemariam et al. Identification of Risk Factors for an Unsuccessful Transition from Pediatric to Adult Sickle Cell Disease Care. PediatrBlood Cancer 2014;61:697–701

  16. Duke Experience • A retrospective database query was conducted among all Duke patients aged 18-23 years with a SCD outpatient encounters through Dec 2013 • Transferwas defined as having clinic visit in pediatrics followed by a subsequent visit in the adult clinic. • Successful transfer was defined as having follow up care in adult hematology clinic for 1 year post-transfer. • Hill, S; Maslow, G; Walker, L; Johnson, S; Shah, N. Growing Pains – Determination of Transfer and Transition from Pediatrics to Adult Outpatient Clinics for Patients with Sickle Cell Disease (SCD). ASH annual meeting 2014.

  17. Duke ExperienceDatabase results • Hill, S; Maslow, G; Walker, L; Johnson, S; Shah, N. Growing Pains – Determination of Transfer and Transition from Pediatrics to Adult Outpatient Clinics for Patients with Sickle Cell Disease (SCD). ASH annual meeting 2014.

  18. Duke ExperienceValidation • Chart review of these patients • 26 (8%) had missing data on outpatient SCD encounters • 48 (14%) SCD patients not followed by Duke Hematology • 65 (19%) did not have a SCD diagnosis.  _______________________________________ Total: • 139 (41%) records were excluded • 203 patients followed at Duke

  19. Duke Experience • Classification: • Continued care in pediatrics • First visit in adults • Transferred to a non-Duke provider • Documented transfer to Duke adult provider (with continued visits for 1 year) • “Lost to follow-up” status as greater than 6 months overdue

  20. Duke Experience • 5 (2%) are deceased (all <10 yo)

  21. Duke QI • Lost to follow up • Communicate with patient/family • Document status • PCP • Specialty provider • Barriers to care • Insurance

  22. An Educational Model to Promote Successful Transition to Adulthood

  23. A Definition of Transition • “Transitionis a multifaceted, active process that attends to themedical, psychosocial, and educational/vocational needs of adolescents as they move from the child-focused to the adult- focused health-care system. Health transition facilitates transition inother areas as well (e.g., work, community, and the school).” • “Transition proceeds at different rates for different individuals and families (and programs)” Blum RW, Garell D, Hodgman CH, et al. J Adolesc Health. 1993;14(7):570-576.

  24. Components of a Transition Program • Comprehensive Approach • Must be a pre-defined program • Policies • Age = 18yo and graduation from high school • 20yo if delayed graduation • 1-2 years in peds if attending college • Education = must complete level 1 and 2 education in pediatrics • mastery >80% on knowledge questionnaires Schwartz M, Tuchman LK, Hobbie WL, Ginsberg JP. Child Care Health Dev. 2011;37(6):883-895.

  25. Components of a Transition Program • Components: • Medical Care – Adolescent/young adult Health Focus • Support - Case Management, Social, Psychological, volunteers/educators • Education - Patient, Family, Providers Schwartz M, Tuchman LK, Hobbie WL, Ginsberg JP. Child Care Health Dev. 2011;37(6):883-895.

  26. Levels of understanding • Level I (WHO) • Know who you are • Level II (WHAT) • Know what to do in different situations • Level III (WHERE) • Know where you are going

  27. How much do you know about your Sickle Cell?

  28. Planning for the Future:Levels of Patient Understanding • Level II(WHERE) • Know where they are going • Level I(WHO) • Know who they are • Level II(WHAT) • Know what to do in different Level I 13 to 14 years old Level II 15 to 16 years old Level III 17 to 18 years old

  29. WHOLevel I of Understanding “I am _____, and I have sickle cell type ____.” • Level I(WHO) • Know who they are Level I 13 to 14 years old

  30. Level I UnderstandingPatients Aged 13 to 14 Years Basic points to understand • Shape of a sickle cell • Genetics • Types of sickle cell • How sickle cell causes complications • ‘Stressors’ that cause sickling Level I 13 to 14 years old Level II 15 to 16 years old Level III 17 to 18 years old

  31. What causes more ‘Sickling’? • Any stress on your body • Dehydration • Infection • Not breathing well • Temperature changes

  32. Body happy = Blood happy!

  33. WHATLevel II of Understanding “I am _____, and I have sickle cell type ____. Sickle cell is when your blood looks like a banana and can cause blood to have problems going places. Stress to the body can cause issues. I know what I need to do when ____ happens with sickle cell.” • Level II(WHAT) • Know what to do in different Level II 15 to 16 years old

  34. Level II UnderstandingPatients Aged 15 to 16 Years Basic points to understand • Types of blood cells • Management of pain • Medications taken daily • Warning signs for complications • Fever, shortness of breath, neurologic symptoms • Complications • Acute chest syndrome, stroke, infection, priapism • Sports and hydration Level I 13 to 14 years old Level II 15 to 16 years old Level III 17 to 18 years old

  35. Patients Should Understand Implications of Their Lab Results Hemoglobin MCV Reticulocyte Count • Carries Oxygen • ‘Energy Cells’ • How big • Reflects fetal hemoglobin • HU effect • How much blood is made White Blood Cells Platelets Transfusions, SF, LIC • Fight infection • Stop bleeding • Reflect risk of chronic iron overload Level I 13 to 14 years old Level II 15 to 16 years old Level III 17 to 18 years old MCV, mean corpuscular volume.

  36. What Patients Need to Know About: Level I 13 to 14 years old Level II 15 to 16 years old Level III 17 to 18 years old

  37. WHERELEVEL III of Understanding “I am _____, and I have sickle cell type ____. Sickle cell is when your blood looks like a banana and can cause blood to have problems going places. Stress to the body causes issues. I know what I need to do when ____ happens with sickle cell. I am going to ___ college/university and/or I am going to work ____. I know about what is bad for me and who/what are bad influences.” • Level II(WHERE) • Know where they are going Level III 17 to 18 years old

  38. Level III understandingPatients Aged 17 to 18 Years Basic points to understand • Health Maintenance • School and/or work (limits and resources) • Insurance • Adult provider • Coordination of transfer • Inpatient management differences • Transfusions/exchange transfusion Level II 15 to 16 years old Level I 13 to 14 years old Level III 17 to 18 years old

  39. Important Screenings for Health Maintenance Routine dental exams Dilated eye exam Vaccinations Urine microalbumin Level II 15 to 16 years old Level I 13 to 14 years old Level III 17 to 18 years old

  40. What Patients Need to Know About: Level II 15 to 16 years old Level I 13 to 14 years old Level III 17 to 18 years old

  41. Make Sure Patients Understand Their Insurance Options • Patients need to understand that seeking treatment without insurance will result in large medical bills • Leading cause for young adults to end up bankrupt is large, unexpected medical bills • Patients should talk to a social worker or regional public health sickle cell educators about resources and options. Level II 15 to 16 years old Level I 13 to 14 years old Level III 17 to 18 years old

  42. Coordination of Care With the Adult Provider is Crucial • Agree upon the transfer process and time necessary for transition should be agreed upon by both • Outline the coordination of care before and after Adult provider (hematologist) Pediatric provider • Inpatient care • Medications • Transfusions/exchange transfusion decisions and tracking • Screening and monitoring • Resources Level II 15 to 16 years old Level I 13 to 14 years old Level III 17 to 18 years old

  43. Know your Sickle Cell WHO: WHAT: WHERE: I know where I am going, to ___ college/university and/or working at ___ I am ____ and I have sickle cell type ____. I know what to do when ___ happens. Level II 15 to 16 years old Level I 13 to 14 years old Level III 17 to 18 years old

  44. Evidence of knowledge • Quizzes • Level 1 - Who, median 9 (range 7-10) • Level 2 - What, median 10 (range 8-10) • Level 3 - Where, median 8 (range 4-10) • Working on ‘All star’ Quizzes

  45. Transition to adulthood Questions???

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