Sickling smoothly through life… Educating patients about transition Nirmish Shah, MD Assistant...
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Transcript of 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.
60
50
40
30
20
10
Life
exp
ecta
ncy
for
patie
nts
with
SC
A (
year
s)2
,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
10
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
5
4
3
2
1
0
Mea
n N
umbe
r w
ith >
1 D
iagn
osis
Per
Pat
ient
Per
Qua
rter
0 5045403530252015105
N= (555) (600) (662) (656) (505) (339) (274) (239) (172) (90)
Age
0.6
0.4
0.3
0.2
0.1
0
0.5
Mea
n N
umbe
r of
Tra
nsfu
sion
s E
vent
s an
d P
resc
riptio
ns P
er
Pat
ient
Per
Qua
rter
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)”
23
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
28
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
29
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
30
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
33
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
34
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
35
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:
36
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
37
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
38
Level I13 to 14 years old
Level II15 to 16 years old
Level III17 to 18 years old
Important Screenings for Health Maintenance
39
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:
40
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.
41
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
42
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
43
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