title: Clinical findings associated with homozygous sickle cell disease in the Barbadian population--do we need a national SCD registry?
abstract: BACKGROUND
  Comprehensive care in homozygous sickle cell disease (HbSS) entails universal neonatal screening and subsequent monitoring of identified patients, a process which has been streamlined in the neighbouring island of Jamaica. In preparation for a similar undertaking in Barbados, we have developed a database of persons with known HbSS, and have piloted processes for documenting clinical manifestations. We now present a brief clinical profile of these findings with comparisons to the Jamaican cohort.
  
  
  METHODS
  HbSS participants were recruited from clinics and support groups. A history of select clinical symptoms was taken and blood and urine samples and echocardiograms were analysed. A re-analysis of data from a previous birth cohort was completed.
  
  
  RESULTS
  Forty-eight persons participated (32 F/16 M); age range 10-62 yrs. 94% had a history of ever having a painful crisis. In the past year, 44% of participants had at least one crisis. There were >69 crises in 21 individuals; 61% were self-managed at home and the majority of the others were treated and discharged from hospital; few were admitted. The prevalence of chronic leg ulceration was 27%. Forty-two persons had urinalysis, 44% were diagnosed with albuminuria (urinary protein/creatinine ratio ≥30 mg/g). Thirty-two participants had echocardiography, 28% had a TRJV ≥ 2.5 m/s. Re-analysis of the incidence study revealed a sickle gene frequency (95% CI) of 2.01% (0.24 to 7.21).
  
  
  CONCLUSIONS
  Although we share a common ancestry, it is thought that HbSS is less common and less severe in Barbados compared to Jamaica. The Jamaican studies reported a sickle gene frequency of 3.15 (2.81 to 3.52); the prevalence of chronic leg ulcers and albuminuria was 29.5% and 42.5% respectively. These comparisons suggest that our initial thoughts may be speculative and that HbSS may be an underestimated clinical problem in Barbados. A prospective neonatal screening programme combined with centralized, routine monitoring of HbSS morbidity and outcomes will definitively answer this question and will improve the evidence-based care and management of HbSS in Barbados.
authors.affiliation: Chronic Disease Research Centre, Tropical Medicine Research Institute, University of the West Indies, Jemmott's Lane, St, Michael, Brigetown, WI, Barbados. Kim.quimby@cavehill.uwi.edu.
authors.forename: Kim R
authors.initials: KR
authors.lastname: Quimby
authors.forename: Stephen
authors.initials: S
authors.lastname: Moe
authors.forename: Ian
authors.initials: I
authors.lastname: Sealy
authors.forename: Christopher
authors.initials: C
authors.lastname: Nicholls
authors.forename: Ian R
authors.initials: IR
authors.lastname: Hambleton
authors.forename: R Clive
authors.initials: RC
authors.lastname: Landis
mesh_terms: D000293:Adolescent; D000328:Adult; D000419:Albuminuria / Q000175:diagnosis / Q000453:epidemiology; D000755:Anemia, Sickle Cell / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics; D001460:Barbados / Q000453:epidemiology; D002648:Child; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D006720:Homozygote; D006801:Humans; D007231:Infant, Newborn; D007563:Jamaica / Q000453:epidemiology; D007871:Leg Ulcer / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D008403:Mass Screening / Q000379:methods*; D008875:Middle Aged; D015997:Neonatal Screening / Q000379:methods; D010865:Pilot Projects; D011159:Population Surveillance / Q000379:methods*; D012042:Registries / Q000706:statistics & numerical data*; D055815:Young Adult