sickle cell anemia -striving to provide comprehensive care at cham library/global navigation/for...
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Sickle Cell Anemia -striving to
provide comprehensive care at
CHAM
Kerry Morrone, MD
Director of the Pediatric Sickle Cell Program
11-2-16
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Objectives
• Demonstrate how to interpret a hemoglobin
electrophoresis
• Update on treatment guidelines from the NHLBI
• Review vaccination guidelines
• Overview on special considerations in prescribing
medications or performing surgical procedures in
sickle cell disease
•
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Prevalence in U.S 90-100,000 Incidence in Africa 200,000 infants/year
Sickle Cell Disease: A Public Health Problem
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Hemoglobin Structure
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Pathophysiology of vaso-occulsion
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Name Genotype Percent
Homozygous SS (Sickle Cell Anemia) S-S 65
Heterozygous SC (Hb SC Disease)
S- C 24
Heterozygous S-+ thal S- + thal 7
Heterozygous S-° thal
S- ° thal
3
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Mortality in sickle cell disease-more to be done
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Perspectives from a clinical case
• Two siblings present with their parents to clinic
after recently immigrating from Liberia.
• They think their children may have a blood
disorder, but are unsure.
• Fareed the 10 year old child had to be
hospitalized several times for infection and gets
pain intermittently
• Kadida is 2 years old and has not had any
complications
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How would you test this family? What are the
methods to screen for sickle cell trait/disease?
• As of 2006 all 50 states now require new born screen
testing for sickle cell disease
• Best screening methods: hemoglobin electrophoresis,
HPLC, isoelectric focusing
• Sickle cell solubility test has many potential errors
• NCAA offers testing of all athletes in Divisions I-III
• 16 % of individuals and 37 % of parents of children
tested are aware of their sickle cell carrier status
Treadwell et al. J Nat Med Assoc . 2006
Naik and Haywood. 2015. Ash Education Program
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Perspectives from a clinical case
• Fareed’s labs:
• 15>7/24<550
• MCV: 75
• Retic: 10
• LDH: 400
• TB/DB: 2.5/.4
• Hemoglobin electrophoresis:
• S%: 90 A2%: 3 F%: 7
• Kadida’s labs:
• 5>12/36<300
• MCV: 75
• Retic: 1
• LDH: 200
• TB/DB: .4/.2
• Hemoglobin electrophoresis:
• S%: 25 A%: 65 A2%: 3 F%: 7
What do you inform this family?
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Perspectives from a clinical case
• The family is informed that Fareed has SS
disease and that Kadida has sickle trait .
• The family wants to discuss the risk factors for
Kadida and want to know what does the
literature say?
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Are there additional risks of having sickle cell trait ?
• No systematic review of complications
• Exertional related injury documented in
retrospective reports
• NEJM- 2016 not increased
• Chronic kidney disease and albuminuira – 1.57
OR (CI 1.34-1.84)
Naik et al. 2014 JAMA
Kark JA et al. 1987 NEJM
Harmon KG et al. 2012 Br J Sports
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Another common scenario…
• Destiny is a 2 month old born in Florida whose
family has moved to the Bronx this week and
mom wants to establish care
• Mom knows there is sikcle cell trait in her family
and missed her follow up appointment to go
over newborn screen results. She asks for her
daughter to be tested
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Another common scenario…
• 10>12<350 MCV: 75 retic: 1.5
• Hemoglobin electrophoresis: S-20 % F->40%
A2:5%
• Does this patient have sickle cell disease or
trait?
• Do they need any additional follow up or
treatments ?
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Another common scenario…
• This patient has sickle cell disease and should
be started on penicillin prophylaxis and referred
to a hematologist
• Fetal hemoglobin is elevated in the newborn
period
• In sickle cell disease the level of fetal
hemoglobin is not stabilized even up until 5
years old
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30 years later from the landmark prophylaxis
study …
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Vaccination recommendations
Steinberg. 2015 BJH
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What is the rate of infection
post vaccination era?
• Post vaccine rate of bacteremia: .8 % in setting
of febrile episodes
• Presence of a central venous line, band count,
clinical appearance and age helped to predict
bacteremia
• Patients still require a blood culture, broad
spectrum antibiotics and ability to have close
follow up
Chang et al. 2013
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What does the CDC suggest for
meningococcal vaccinations?
• New updates for children with anatomic or functional
asplenia (including sickle cell disease):
• Can start vaccinating at 2 months old with Menveo or
Menhibirix
• If start vaccinating at 2 months then should receive
vaccines at 2,4, 6 and 12 months
• Catch up vaccine schedule
• Menactra can be used in over 2 years of age
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At 430 pm on a Friday afternoon
• Jose and his mother present to the Adolescent
doctor for his routine visit
• He is noted to be tachypneic and febrile to 102
• He states he was febrile this morning but felt
better after motrin.
• Besides the tachypnea he is very well
appearing and states he wants to go home..
• What do you do ?
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NHLBI guidlelines for fever
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• Jose is seen in the emergency room and as the resident
is doing medication reconciliation they notice he is on
hydroxyurea
• On further questioning Jose states he has had very few
crises and very minimal complications
• The family states his hematologist suggested he be on it
for his general health
• Is there an indication to do that?
Hydroxyurea- who , when and why ?
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Randomized phase III trial
9-18 months
N=193
18 month follow up
Excellent safety profile
Decreased pain crisis
Decreased dactylitis
Improved hemoglobin
Decreased inpatient hospitalization costs
Wang Pediatrics 2013
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< 5 yrs
5 <10yrs
10 <15yrs
>15yrs
Never
Death
Stroke
Renal Disease
Hepatic disease
Sepsis/Infection
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NHLBI 2014 Guidelines:
Hydroxyurea
****
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• Jose tells you he is also upset because he is supposed to
have surgery tomorrow to help with his snoring
• He is afraid his fever will prevent him from getting the
surgery
• After checking his labs his hemoglobin returns at 8 , retic:
10
• If he is feeling better should be just go to the ambulatory
suite tomorrow to remove his tonsils?
Surgical considerations
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What are common surgeries or procedures
for patients with sickle cell anemia?
• Tonsillectomy & adenoidectomy
• Cholecystectomy
• Orthopedic procedures (core decompression of
hip)
• Splenectomy
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Procedural/Surgical Preparations for Sickle
Cell Disease
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Procedure/Surgical Preparation
• Refer to your patient’s hematologist
• Blood work is done and patients are optimized
by blood transfusions and/or IVF
• Patients typically admitted the night prior to
surgery or procedure if it requires sedation
• Medical clearance should be provided by
hematologist
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By request: special considerations
• No estrogen containing contraception
• Steriods- to be used judiciously and tapered for
risk of VOC and ACS rebound
• Headache restrictions (triptans)
Whitley- ASH education book
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Objectives
• Demonstrate how to interpret a hemoglobin
electrophoresis
• Update on treatment guidelines from the NHLBI
• Review vaccination guidelines
• Overview on special considerations in prescribing
medications or performing surgical procedures in
sickle cell disease
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CHAM Pediatric Hematology team