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abstract 89

144. Comorbidities in the Young and Aging should consider the likely higher prevalence of dis-
Populations with Haemophilia A eases such as diabetes, mental health issues, pain, ar-
thropathy and asthma. Use of different types of data
Ceri Hirst and James Black source provides a comprehensive overview that may
enhance the recognition of comorbidities.
Real World Data Science, F. Hoffmann La Roche,
Basel, Switzerland 145. Epidemiologic and Clinical Characteristics of
Thalassemia (THAL) Intermedia (TI) in the US
Background: Haemophilia A (HA), a rare hereditary
condition, impacts ~1/5000 male births globally. Elliott Vichinsky1, Janet L. Kwiatkowski2, Patricia-
Treatment advances in recent decades have improved Jane Giardina3, Carole Paley4, Francis Vekeman5,
life expectancy for affected individuals, now ap- Wendy Y. Cheng6, Joseph Damron6, Nora
proaching normality. Thus, an aging population of McCormick6, Medha Sasane4, Ying Qiu4, Mei Sheng
people with HA (PWHA) is emerging, but with little Duh6 and Alexis Thompson7
published research on their comorbidity prole.
1
Childrens Hospital and Research Center Oakland,
Objectives: We evaluated comorbidity rates from real Oakland; 2 The Childrens Hospital of Philadelphia,
world data sources to show key comorbidities in Philadelphia; 3 Weill Cornell Medical College, New
PWHA by age strata. York; 4 Novartis Pharmaceuticals Corporation, East
Hanover; 5 Groupe dAnalyse, Ltee, Montreal; 6 Analy-
Methods: Analyses used 3 data sources, and their con- sis Group, Inc., Boston; 7 Ann Robert H. Lurie Chil-
cordance was evaluated. Clinical Practice Research drens Hospital of Chicago, Chicago
Database (CPRD) provides a comprehensive record
from a nationwide representative sample of UK gen- Background: TI is increasingly prevalent in the US
eral practitioners. MarketScan provides insurance due to changing immigration patterns. It is
claims data for ~46 million US patients. Patients from underdiagnosed, leading to inadequate or delayed
CPRD and MarketScan were included if they had a management.
HA diagnosis on/before 1 Jan 2014 (index date), with
12 mo of medical history. An age- and gender- Objectives: To review the epidemiology and clinical
matched sample of the general population was derived characteristics of TI in patients (pts) in the US.
in the CPRD. Diagnoses for 5 years preceding were
evaluated. A cross-sectional physician survey, Methods: Medical records of pts with a TI diagnosis
CHESS, collected data for EU patients with severe (8 mean packed red blood cell transfusions (tx) per
HA. year (yr) over a 3-yr period) and 12 months of fol-
low-up from 1/1997- 4/2014 at 4 US hematology cen-
Results: The population included MarketScan 1903, ters were reviewed for demographic and clinical
CPRD 310, CHESS 996; mean age was 39 y (CPRD characteristics by TI subtype.
and MarketScan) and 36 y (CHESS). In adults, hepati-
tis C, pain, arthropathy, chronic pulmonary disease Results: Of 138 pts reviewed, 84 had -thal, 39 had -
(including asthma), diabetes, cancer and anxiety/de- thal, and 15 had E/-thal. 74% of -thal pts had
pression appeared to be reported more often in PWHA deletional (del) Hb H, and 26% had non-del (ndel)
than the general population, though prevalence was in- Hb H. Median age was 2.3 yr (1.64 del; 6.1 ndel) in
consistent across data sources. Prevalence of diabetes, the -thal group, 9.2 yr in the -thal group, and 2.2 yr
pain, depression/anxiety increased with age, while in the E/-thal group. Most -thal (77%) and E/-thal
HIV and hepatitis C prevalence was highest in 30-40 (87%) pts were Asian, and -thal pts were White
y group (likely due to childhood exposure to contami- (46%) or African-American (AA; 36%). 21%, 10%,
nated blood products). Arthropathy was reported in and 20% of , , and E/-thal pts were foreign-born,
MarketScan in PWHA aged 3-5 y (8%) and 6-11 y and 5%, 3% and 7% were transfused outside of the
(27%), but not CPRD, maybe due to lack of reporting US.
in primary care. Top 5 comorbidities were splenomegaly,
extramedullary hematopoiesis, growth retardation, he-
Conclusions: PWHA, across age groups, suffer con- patomegaly, and infections needing hospitalization/IV
siderable comorbidities. Management of PWHA antibiotics. 22% of pts had 1 tx, while 7% of pts had

Copyright 2016 John Wiley & Sons, Ltd. Pharmacoepidemiology and Drug Safety, 2016; 25(Suppl. 3): 3681
DOI: 10.1002/pds