Comprehensive care for patients with sickle cell disease in Cuba

Sickle cell disease (SCD) is the most frequent hereditary disorder in Cuba. The incidence of sickle cell trait is 13, 25 in blacks, 0, 65 in whites and 3, 085 for the general population.1 There are 4000 sickle cell patients distributed throughout the country, with a greater prevalence in Havana city and in the South-Western provinces.2 In spite of the fact that Cuba is a country with limited resources: our national health system allows a holistic attention to patients with SCD. A nationwide programme for the prevention of sickle cell disorders was initiated in our country in 1983. Couples at risk have been identified by screening pregnant women and the partner of those who carry the S or C haemoglobin, followed by prenatal diagnosis and genetic counselling.3 There is no newborn screening up to now but all the babies with SCD are closely monitored from the first months of their life. There is no data regarding the number of children born with SCD before the implementation of the prenatal diagnosis plan.

Sickle cell patients are treated via specialized consultation by haematologists in all 14 provinces of the country and they have easy access to all kinds of health services including hospitalization and emergency services. They have access to narcotic analgesics in the health care institutions, but they do not habitually take them home. Cienfuegos is a central province of Cuba with about 400,000 inhabitants, 96 of whom suffer with SCD at this moment. Throughout the last 10 years 5 patients have died, all adults, three men of acute chest syndrome, one of hepatic complications and one woman died of pulmonary thromboembolism right after a delivery. The Institute of Haematology and Immunology in Havana city is our referral centre for complex cases. The guidelines for management and treatment as well as many investigations of this disease have been developed there.4–7 In a research done in that centre, it was established that the global survival estimates were 53 years in sickle cell anaemia, 59 years in SC haemoglobinopathy and 48 in Sβ thalassemia. The most frequent cases of death were hepatic complications, stroke and infections.8 This study was performed in 397 adult patients who had been followed during 25 years; the survival was calculated using the Kaplan-Meier curves. This data was not compared to the Cooperative Study for SCD in the USA and it is not sufficient to support a decrease in the mortality rate in our patients due to the fact that there are no previous statistics. The decrease in infancy mortality has been obtained due to early attention and proper treatment from the first postpartum months, prevention of infections by S. pneumoniae with prophyactic use of penicillin in all the children of the country l and patient/family orientation with active involvement in the early diagnosis of complications.8 The all-embracing assistance plan in the patients was carried out with the aid of the Centro Informazione e Educazione allo Sviluppo (CIES) from Italy. Today, transcranial Doppler ultrasound is being realized in children and in adults, screening for pulmonary hypertension.