THE EDITOR, Sir:
A MAJOR study from the Sickle Cell Unit at the University of the West Indies over the last 32 years has been the Jamaican Cohort Study of Sickle Cell Disease.
This is a unique study, known worldwide, in which an unbiased sample of patients with sickle cell disease was developed by the screening of 100,000 deliveries at Victoria Jubilee Hospital between June 1973 and December 1981.
This screening detected 550 children with different forms of sickle cell disease, 315 with the generally severe sickle cell disease, who were closely followed along with 250 normal Jamaican children born at the same time.
As the children have grown up, much has been learnt about the disease, appropriate interventions have been devised, and the quality of life and the survival of Jamaican patients have been improved.
UNIQUE ADVANTAGES
These lessons have also been published in the international medical literature and have contributed to improving the management of sickle cell patients worldwide. Jamaica has unique advantages for this type of long-term study, which include the geographical limitation of an island, the extended family system which has facilitated the tracing of patients, and most of all, the cooperation and understanding of the affected families.
Patients in this study are now aged 24-32 years. They have passed the high-risk period of early childhood, completed formal education, traversed the problems of delayed growth and development in adolescence, and now face the difficulties of obtaining employment, establishing partnerships and raising families.
COMPROMISING SUCCESS
Recently, however, the conditions in Jamaica, which have been so favourable to this study, are beginning to compromise its success and the contribution which this study can make to helping patients everywhere. Of the last eight male deaths in this study, five were violent, three from stabbing and two from gunshot wounds.
The hope that female patients would be immune to this violence was dashed by events at 3 Barnes Avenue, Kingston 13, early on the morning of October 5, ironically in Sickle Cell Awareness Week.
These events led to the violent death of Michelle, a mild-mannered sickle cell patient followed from birth to the age of 30 years, and her life was not claimed by sickle cell disease but by the firebombing of the family home.
Data from the Cohort Study continue to be published in the medical literature, but rather than contributing to the benefit of patients elsewhere, these events will tell the world that living in Jamaica today may be more dangerous to your health than having sickle cell disease.
I am, etc.,
GRAHAM R. SERJEANT
Professor Emeritus, UWI,
Chairman, Sickle Cell Trust.
