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Hemoglobinopathy Knowledge:Hemoglobinopathy Knowledge:
A S f P di i iA Survey of Pediatricians in Hawai’i, San Francisco,
and Salt Lake City
Kirsty McWalter, MS, CGCCertified Genetic Counselor
Hawai’i Dept of Health Genetics Program
Newborn Screening and Genetic Testing SymposiumMay 2010
Hawaiian sunrise
Genetics in Paradise
Clinical Genetics – population servedp p
Hawai’i Community Genetics
John A. Burns School of Medicine
Pediatric Genetics Services
• Hemoglobinopathy Clinic • ~30 cases of alpha thalassemia per month detected
on Hawai‘i’s newborn screen
Purpose of the Study
HI SF SLC USA
population 1 2 mill 776 000 181 000 281 millpopulation 1.2 mill 776,000 181,000 281 mill
Births/yr (rate)
18,000 (14/1000)
8,300 (10/1000)
3,600 (19/1000)
4 mill (13/1000)
Non-Hispanic
minority (23%)
majority (44%)
majority (71%)
majority (70%)Hispanic
whites(23%) (44%) (71%) (70%)
Asian 42% 31% 4% 4%
Hispanic 7% 14% 19% 13%
2000 US Census
Response Rate
100
80
40
60
20
0
HI=45% SF=23% SLC=34%HI=45% (n=113)
SF=23% (n=54)
SLC=34% (n=59)
Results> 32% give iron
to all patientsto all patients identified with anemiawith anemia
Only 16% had attended aattended a hemoglobinopathy educational session ineducational session in the past six months
Results• The survey included 5 knowledge questions.
• Each knowledge question was analyzed by survey site and other demographics:g p– year of residency completion– whether or not a physician gives iron to all anemic p y g
patients– number of patients with anemia seen each week– sex– attendance at a hemoglobinopathy education session
i hi h l 6 hwithin the last 6 months– type of pediatrician
Knowledge Question 1Question Correct Incorrect
What is the inheritance 84% 16%pattern for sickle cell disease?
Significant Findings
• residency after 1992 more likely than before 1992 (p=0 004)residency after 1992 more likely than before 1992 (p 0.004)• HI less likely than all other participants (p=0.04)
Si ifi t Fi di i H i’i• Significant Findings in Hawai’i:• residency after 1992 more likely than before 1992 (p=0.028)
• those who do not give iron to all anemic patients more likely than who do give iron to all anemic patients (p=0.057)
Knowledge Question 2g
Question Correct Incorrect
Is sickle cell trait detected by newborn screening?
83% 17%g
Significant Findings
• general pediatricians more likely than specialty pediatricians (p=0 010)pediatricians (p 0.010)
• SF general pediatricians more likely than SF specialty di t i i ( 0 009)pediatricians (p=0.009)
Knowledge Question 3
Question Correct Incorrect
Is beta thalassemia detected by newborn screening?
29% 71%
Significant Findings
• SF more likely than all other participants (p=0.002)
• HI less likely than SF (p=0 030)• HI less likely than SF (p=0.030)
• HI more likely than SLC (p=0.022)
• SF more likely than SLC (p=0.000)
Knowledge Question 3 cont’dIs beta thalassemia detected by newborn screening?
Significant Findings in Hawai’i
• those who see >6 anemic patients/week more likely than those who see ≤6 per week (p=0.020)
• those who see >2 anemic patients/week more likely then those who see ≤2 per week (p=0.028)
• those not attending hemoglobinopathy education in the last 6 months more likely than those attending (p=0.032)months more likely than those attending (p 0.032)
• males more likely than females (p=0.052)
Knowledge Question 4Question Correct Incorrect
True or False: sickle cell disease 94% 6%True or False: sickle cell disease only occurs in individuals of African American descent
94% 6%
Significant Findings
• those attending hemoglobinopathy education in the last 6 months more likely than those not attending (p=0.007)
S h i i l i id 200 lik l h• SF physicians completing residency <2005 more likely than SF physicians completing residency ≥2005 (p=0.010)
SF ph sicians not attending hemoglobinopath ed cation in• SF physicians not attending hemoglobinopathy education in the last 6 months more likely than SF attending (p=0.011)
Knowledge Question 5Question Correct Incorrect
True or false: alpha 86% 14%pthalassemia can be diagnosed by ordering a complete blood count or red blood cell index.
Significant FindingsSignificant Findings• HI more likely than all other participants (p=0.050)• HI more likely than SF (p=0.045)y (p )• SLC general peds more likely SLC specialty peds (p=0.057)• HI participants without hemoglobinopathy education in the l t 6 th lik l th HI ith h l bi thlast 6 months more likely than HI with hemoglobinopathy education in the last 6 months (p=0.008)
Results: Total Knowledge Scores
• A total knowledge score was calculated for each participant:“ ” l h 3/5– “poor” = less than 3/5 correct
– “good” = 3/5 or 4/5 correct “perfect” = 5/5 correct– perfect = 5/5 correct
• 68.2% of all participants had either “good” or “perfect” knowledge scores
Significant Results: Total Knowledge Scores
• Both HI and SF physicians were significantly more likely than SLC physicians to have “perfect” total knowledge scores (p=0.035 and p=0.015, respectively)g (p p , p y)
• HI physicians who see anemic patients are significantly more likely than HI physicians who do not see anemicmore likely than HI physicians who do not see anemic patients to have a “good” knowledge score (p=0.053)
h i i h d d h l bi h• HI physicians who attended hemoglobinopathy education in the last six months were significantly more likely to have “good” knowledge scores than those who h d t ( 0 003)had not (p=0.003)
Discussion
• Knowledge gaps between physicians in the three g g p p ypopulations present opportunities to provide targeted education regarding:– hemoglobinopathies, – state NBS panels, and – benefits of a referral to a hemoglobinopathy clinic
• The results will inform the development of public health hemoglobinopathy education materials to
t t h lth kfassure a competent health care workforce.
Kirsty McWalter, MS, CGC
HI Dept of Health Genetics Programkirsty@hawaiigenetics [email protected]
(808)733-8387
Mahalo!