Transcript Sickling Smoothly through Life

Sickling smoothly
through life…
Educating patients about
transition
Nirmish Shah, MD
Assistant Professor
Director of Sickle Cell Transition Program
Duke 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
Life expectancy for patients with SCA (years)2,3
2012 – SWiTCH trial8
60
1998 – STOP trial6
1986 – Antibiotic
prophylaxis in infants6
50
2011 – BABY HUG trial7
40
1976 – RBC transfusion for
treatment of secondary stroke5
30
1949 – Identification of HbS4
20
1995 – MSH Trial6
1987 – NIH recommends
newborn screening6
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.
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.
8
CHALLENGES FACING
TRANSITIONING SCD PATIENTS
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 an
adult 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.
10
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
Readmission Rates Also Spike After Transition
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)
Age range,
years
 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.
12
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
–
–
Patient
no.
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
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 known
Fell in hospital while pregnant; cerebral
hemorrhage
5
18.3
19.3
None known
Acute 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
*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.
13
Complications of SCD Increase As Patients
Transition and RBC Transfusions Decrease
0.6
0.5
4
0.4
3
0.3
2
0.2
1
0.1
0
0
0
N=
5
(555)
10
15
20
25
Age
30
35
40
45
50
(600)
(662)
(656)
(505)
(339)
(274)
(239)
(172)
(90)
Blinder MA, Vekeman F, Sasane M, et al. Pediatr Blood Cancer. 2013;60(5):828–835.
Mean Number of Transfusions Events and Prescriptions Per
Patient Per Quarter
Mean Number with > 1 Diagnosis Per Patient Per Quarter
5
Increased frequency
of complications
correlates with a
decrease in the
utilization of RBC
transfusions and
hydroxyurea
Any Complications
Mean Transfusions
Pain
Infection
14
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. 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 posttransfer.
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 Experience
Database 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 Experience
Validation
 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
Continued care in pediatrics
28 (14%)
- Less than 6 months overdue for f/u
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%)
 5 (2%) are deceased (all <10 yo)
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
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 adultfocused 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)”
Blum RW, Garell D, Hodgman CH, et al. J Adolesc Health. 1993;14(7):570-576.
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
Schwartz M, Tuchman LK, Hobbie WL, Ginsberg JP. Child Care Health Dev. 2011;37(6):883-895.
24
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.
25
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
Level I
(WHO)
Level II
(WHAT)
Level II
(WHERE)
Know who
they are
Know what to do in
different
Know where they are
going
Level I
13 to 14 years old
Level II
15 to 16 years old
Level III
17 to 18 years old
28
WHO
Level I of Understanding
Level I
(WHO)
Know who
they are
“I am _____, and
I have sickle cell
type ____.”
Level I
13 to 14 years old
29
Level I Understanding
Patients 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
30
What causes more ‘Sickling’?
 Any stress on your body
–
–
–
–
Dehydration
Infection
Not breathing well
Temperature changes
Body happy = Blood happy!
WHAT
Level II of Understanding
Level II
(WHAT)
Know what to do in
different
“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
15 to 16 years old
33
Level II Understanding
Patients 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

Level I
13 to 14 years old
Sports and hydration
Level II
15 to 16 years old
Level III
17 to 18 years old
34
Patients Should Understand Implications
of Their Lab Results
Hemoglobin
 Carries Oxygen
 ‘Energy Cells’
White Blood Cells
 Fight infection
Level I
13 to 14 years old
MCV
Reticulocyte Count
 How big
 Reflects fetal hemoglobin
 HU effect
 How much blood is made
Platelets
Transfusions, SF, LIC
 Stop bleeding
Level II
15 to 16 years old
 Reflect risk of chronic
iron overload
Level III
17 to 18 years old
MCV, mean corpuscular volume.
35
What Patients Need to Know About:
Pain
management
•
Know their medications
–
•
Have a pain action plan
Take main pain medication
with
anti-inflammatory
•
Drink fluids, rest, reduce
‘stress’ to body
•
Interplay between stress,
swelling, and pain
Level I
13 to 14 years old
Shortness
of breath
•
Can be an emergency
•
Risk for acute chest
syndrome
•
•
Neurologic
complications
•
Emergency
Asthma increases their
chances of having issues
•
Weakness, slurred speech,
visual changes
May need immediate
transfusion or exchange
transfusion
•
Need immediate transfusion
or
exchange transfusion
Level II
15 to 16 years old
Level III
17 to 18 years old
36
WHERE
LEVEL III of Understanding
Level II
(WHERE)
Know where they are
going
“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
17 to 18 years old
37
Level III understanding
Patients Aged 17 to 18 Years
Basic points to understand
 Health Maintenance
 School and/or work
(limits and resources)
 Insurance
Level I
13 to 14 years old
 Adult provider
– Coordination of transfer
– Inpatient management differences
– Transfusions/exchange transfusion
Level II
15 to 16 years old
Level III
17 to 18 years old
38
Important Screenings for Health Maintenance
Routine dental exams
Dilated eye exam
Vaccinations
Urine microalbumin
Level I
13 to 14 years old
Level II
15 to 16 years old
Level III
17 to 18 years old
39
What Patients Need to Know About:
School
Work or volunteering
•
Absences should be excused if they are
absent for SCD issues
•
Patients need to make sure they and their
employers know their limits
•
Schools should work with them to get
missed assignments
•
Patients should
•
Goals should be modified during illness or
recovery
Level I
13 to 14 years old
– Hydrate
– Take breaks
– Understand when they need
medications
Level II
15 to 16 years old
Level III
17 to 18 years old
40
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 I
13 to 14 years old
Level II
15 to 16 years old
Level III
17 to 18 years old
41
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
Pediatric
provider
 Inpatient care
 Medications
 Transfusions/exchange transfusion decisions
and tracking
 Screening and monitoring
 Resources
Level I
13 to 14 years old
Level II
15 to 16 years old
Adult provider
(hematologist)
Level III
17 to 18 years old
42
Know your Sickle Cell
WHO:
I am ____ and I have
sickle cell type ____.
Level I
13 to 14 years old
WHAT:
I know what to do
when ___ happens.
Level II
15 to 16 years old
WHERE:
I know where I am
going, to ___ college/
university
and/or working
at ___
Level III
17 to 18 years old
43
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
Transition to adulthood
Questions???