The work presented in this book is an attempt at a comprehensive statement of the natural history of the sickle cell disease patient and is based upon more than 1500 consecutive patients personally observed and followed up by the author for more than five years. Comments and discussions are, however, based on experience acquired from active supervision of a total of over 3,000 sickle cell disease patients in Ghana between 1965 and 1977, giving a rough experience value of close to 30,000 patient-years.

Sickle cell disease is defined and the various genotypes and phenotypes comprising the disease are clarified. More than 70 years of actively expanding literature has been reviewed, including new information on the molecular pathology and pathophysiology of these haemoglobinopathies. The epidemiology of sickle cell disease, together with a catalogue of clinical features and complications, as witnessed by the author in Accra, have, with the aid of computers, been stated in quantitative terms.

The work shows that sickle cell disease is a problem of great magnitude, deserving of national and international attention. An approach to treatment is given, with greater emphasis on patient management than is usual, paying attention to aggravating factors such as infections within poor socio-economic conditions and stressing the importance of genetic counselling and family planning for patients.