sickling smoothly through life… educating patients about transition nirmish shah, md assistant...

Sickling smoothly through life…

Sickling smoothly through life…

Educating patients about transition

Educating patients about transition

Nirmish Shah, MDAssistant Professor

Director of Sickle Cell Transition ProgramDuke University

Outline

Introduction

Challenges facing transitioning SCD patients

Model and key components for a successful transition to adulthood

‘Who, What, Where’ – levels of patient understanding

2

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

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

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

Introduction

Sickle Cell Disease (SCD) is the most common inherited disease worldwide

Recent advances include prophylactic PCN, improved immunizations, and increased use of hydroxyurea

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)

Recent Developments Have Greatly Improved Patient Management and Survival1

8

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. Anemia 2011;2011:918916. 5. Lusher JM, Haghighat H, Khalifa AS. 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.

BABY HUG, Pediatric Hydroxyurea Phase III Clinical Trial; NIH, National Institutes of Health; STOP, Stroke Prevention Trial in Sickle Cell Anemia.

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Life

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A (

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,3

1910 1950 1970 1980 1990 2000 201019601920 1930 1940

1910 - Discovery of SCD4

1986 – Antibiotic prophylaxis in infants6

1949 – Identification of HbS4

1976 – RBC transfusion for treatment of secondary stroke5

1987 – NIH recommends newborn screening6

1998 – STOP trial6

2011 – BABY HUG trial7

2012 – SWiTCH trial8

1995 – MSH Trial6

CHALLENGES FACING TRANSITIONING SCD PATIENTS

Transitioning Poses Many Challenges for Patients

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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.

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

Ineffective use of healthcare system

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

During 5 year study:• Children = <18• Transition = turned 19• Young Adults = 19-30• Adults = >30

Readmission Rates Also Spike After Transition

12Brousseau DC, Owens PL, Mosso AL, et al. JAMA. 2010;303(13):1288-1294.

Approximately 80% of rehospitalizations were to the same hospital– 94% of pediatric readmissions– 77% of adult readmissions

Age range,years n

14-day Rehospitalization,n (95% CI)

30-day Rehospitalization,n (95% CI)

1-9 6895 7.3 (6.7-7.9) 12.8 (12.0-13.6)

10-17 8825 14.1 (13.4-14.8) 23.4 (22.5-24.3)

18-30 24,645 28.4 (27.8-29.0) 41.1 (40.5-41.7)

31-45 15,118 26.0 (25.3-26.7) 38.8 (38.0-39.6)

46-64 5160 15.3 (14.3-16.3) 27.1 (25.9-28.3)

>65 427 6.3 (4.0-8.6) 11.9 (8.8-15.0)

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

13Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Blood. 2010;115(17):3447–3452.

N/A, not applicable.

*Transition was delayed beyond 18 years due to chronic renal failure.

Patient no.

Age at transition, y

Age at death, y

Chronic complication of SCD Circumstances of death

1 N/A* 18.8 Renal failure; dialysis Found dead at home

2 17.9 19.2 None known Acute chest syndrome

3 18.0 18.3 None known Acute chest syndrome, refused transfusion

4 18.2 20.6 None knownFell in hospital while pregnant; cerebral hemorrhage

5 18.3 19.3 None knownAcute chest syndrome complicated by multiorgan failure syndrome

6 18.4 23.7 None known Presumed stroke

7 18.5 18.7 None known Multiorgan failure syndrome

Complications of SCD Increase As Patients Transition and RBC Transfusions Decrease

14Blinder MA, Vekeman F, Sasane M, et al. Pediatr Blood Cancer. 2013;60(5):828–835.

Any Complications

Mean Transfusions

PainInfection

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N= (555) (600) (662) (656) (505) (339) (274) (239) (172) (90)

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Increased frequency of complications correlates with a decrease in the

utilization of RBC transfusions and

hydroxyurea

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 fromPediatric to Adult Sickle Cell Disease Care. Pediatr Blood Cancer 2014;61:697–701

Duke Experience

A retrospective database query was conducted among all Duke patients aged 18-23 years with a SCD outpatient encounters through Dec 2013

– Transfer was 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.

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.

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

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

Duke Experience

5 (2%) are deceased (all <10 yo)

Continued care in pediatrics- Less than 6 months overdue for f/u

28 (14%)

First visit in adults 14 (6%)

Transferred to a non-Duke provider 19 (9%)

Transferred to adult hematology 60 (30%)

Lost to follow up 78 (38%)

Duke QI

Lost to follow up– Communicate with patient/family– Document status

– PCP– Specialty provider– Barriers to care– Insurance

An Educational Model to Promote Successful Transition to Adulthood

Blum RW, Garell D, Hodgman CH, et al. J Adolesc Health. 1993;14(7):570-576.

A Definition of Transition

“Transition is a multifaceted, active process that attends to the medical, 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 in other areas as well (e.g., work, community, and the school).”

“Transition proceeds at different rates for different individuals and families (and programs)”

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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

24Schwartz M, Tuchman LK, Hobbie WL, Ginsberg JP. Child Care Health Dev. 2011;37(6):883-895.

Components of a Transition Program

Components:– Medical Care – Adolescent/young adult Health Focus– Support - Case Management, Social, Psychological,

volunteers/educators– Education - Patient, Family, Providers

25Schwartz M, Tuchman LK, Hobbie WL, Ginsberg JP. Child Care Health Dev. 2011;37(6):883-895.

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

Level I 13-14 year old

Level II 15-16 year old

Level III 17-18 year old

How much do you know about your Sickle Cell?

Planning for the Future:Levels of Patient Understanding

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Level I(WHO)

Know who they are

Level II(WHAT)

Know what to do in different

Level II(WHERE)

Know where they are going

Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

Level I(WHO)

Know who they are

WHOLevel I of Understanding

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Level I13 to 14 years old

“I am _____, and I have sickle cell type ____.”

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

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Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

What causes more ‘Sickling’?

Any stress on your body– Dehydration– Infection– Not breathing well– Temperature changes

Body happy = Blood happy!

WHATLevel II of Understanding

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Level II(WHAT)

Know what to do in different

Level II15 to 16 years old

“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 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

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Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

Patients Should Understand Implications of Their Lab Results

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Hemoglobin

Carries Oxygen ‘Energy Cells’

White Blood Cells Transfusions, SF, LIC

Reticulocyte Count

Platelets

MCV

How big Reflects fetal hemoglobin HU effect

Reflect risk of chronic iron overload

How much blood is made

Stop bleeding Fight infection

MCV, mean corpuscular volume.

Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

What Patients Need to Know About:

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Painmanagement

Shortnessof breath

Neurologic complications

• Know their medications– Have a pain action plan

• Take main pain medication withanti-inflammatory

• Drink fluids, rest, reduce‘stress’ to body

• Interplay between stress, swelling, and pain

• Can be an emergency

• Risk for acute chest syndrome

• Asthma increases their chances of having issues

• May need immediate transfusion or exchange transfusion

• Emergency

• Weakness, slurred speech, visual changes

• Need immediate transfusion or exchange transfusion

Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

Level II(WHERE)

Know where they are going

WHERELEVEL III of Understanding

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Level III17 to 18 years old

“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 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

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Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

Important Screenings for Health Maintenance

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Routine dental exams Dilated eye exam

Urine microalbumin

Vaccinations

Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

What Patients Need to Know About:

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School Work or volunteering

• Absences should be excused if they are absent for SCD issues

• Schools should work with them to get missed assignments

• Goals should be modified during illness or recovery

• Patients need to make sure they and their employers know their limits

• Patients should

– Hydrate

– Take breaks

– Understand when they need medications

Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

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.

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Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

Inpatient care Medications Transfusions/exchange transfusion decisions

and tracking Screening and monitoring Resources

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

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Adult provider

(hematologist)Pediatric

provider

Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

Know your Sickle Cell

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Level I13 to 14 years old

Level II15 to 16 years old

Level III17 to 18 years old

I am ____ and I have sickle cell type ____.

WHO: WHAT:

I know what to do when ___ happens.

WHERE:

I know where I am going, to ___ college/university and/or working at ___

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

Questions???

Transition to adulthood