Select syndromes associated with Homozygous Sickle cell disease in the Barbadian Population

Investigators & Affiliations:

KR Quimby¹, S Moe², I Sealy², C Nicholls², IR Hambleton¹, RC Landis¹

1 Chronic Disease Research Centre, TMRI, Barbados
2 Queen Elizabeth Hospital, Barbados

Funding Obtained:

Cave Hill Postgraduate Research Award Fund – 4.2011 – USD $5000.00

Start Date:

Jan 2011

End Date:

Jan 2013

Rationale:

In spite of our knowledge of the clinical complications of Sickle cell disease (SCD), there is no local documentation on the impact of disease progression on the patient or on the healthcare system. In fact the only documented report in the Barbadian population is an incidence study in 1999. In order to continue our molecular work investigating the clinical impact of the haemoglobin scavenging mechanism in SCD, we first needed to document some initial clinical findings associated with this population. For our initial reconnaissance, we focused on albuminuria, pulmonary hypertension, chronic leg ulcers and acute painful crises.

Methods:

Participants were included as outlined in the previous report. A history of albuminuria, pulmonary hypertension, chronic leg ulcers and acute painful crises was ascertained. Urine samples were analysed for albumin:creatinie ratio and the tricuspid regurgitant jet velocity was determined by echocardiogram. Results were compare to those of the Jamaican sickle cohort which is an established SCD registry

Main Results:

93% of participants have ever had a painful crisis, with 44% experiencing at least one within the last year. Forty‐two persons had urinalysis, 44% reported albuminuria (urinary protein/creatinine ratio >30). 27% of participants had a history of chronic leg ulceration. Thirtytwo participants had echocardiograms performed, 28% indicated a raised TRJV. Expected impact: Anecdotally, it is said that the clinical features of SCD are less severe in the Barbadian than in the Jamaican population. This initial observation suggests that the prevalence is similar. This information suggests that centralized, routine monitoring system for SCD morbidity to improve the HbSS evidence base in Barbados may be necessary.

Next Steps / Future Plans:

We are considering implementing universal neonatal screening and database for registration and observation.