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Chronic shortage of clotting factor concentrates, exorbitant treatment costs and the poor economic status of patients makes disability an inevitable consequence of haemophilia in India. The prevalence and risk factors for disability were studied in 148 patients with severe haemophilia A registered at five haemophilia clinics in the country. Disability was measured using a scoring method that used a compilation of functional ability, mobility and range-of-motion scores for each patient. Patients ranged in age from 5 to 55 years. Only nine of 148 patients were free of disability. The proportion of disability free patients in the 5-12, 13-24 and 25+ age groups were 14.3%, 4.4% and 0% respectively. The risk factors significantly associated with disability were patients age, socio-economic status, number of persons in the family, family history of haemophilia, frequency of physiotherapy exercises, home use of coagulation factor concentrate and type of blood product(s) used, that is clotting factor concentrate or cryoprecipitate. The study highlights the need to provide coagulation factor concentrates in sufficient amounts to prevent disability, the beneficial role of physiotherapy exercises and the advantage of older patients as peer educators for younger patients. The most important utility of the data is to demonstrate the widespread prevalence of disability amongst patients, its social costs in terms of incomplete education and unemployment, which justifies the need to include haemophilia as one of the conditions under the Disability Act of India.

Secondary data on haemophilia cases in the state of Maharashtra, India were compiled and analysed. A total of 2192 haemophiliacs could be accounted from case papers and pedigrees, representing approximately 60% of the estimated number of cases in the state. Ratio of haemophilia A to haemophilia B was 4.2:1. Severe haemophiliacs constituted the majority of haemophilia A and B cases. About 70% of patients did not report any family history. Age distribution of cases suggested that the longevity of severe haemophiliacs was less than 30 years. Geographical distribution revealed clustering of cases around three established haemophilia clinics, with large parts of the state showing no cases. Although the cumulative number of cases showed an ascending trend, there was a 90% decrease in the number of cases registered between 1989 and 2000, suggesting that case recruitment from areas around the facilities had been achieved. Most cases showed delayed diagnosis, as the majority of severe haemophiliacs was being diagnosed at around 11-15 years of age. However, increasing awareness about haemophilia was reflected in a four-fold increase in referrals of severe cases over a 10-year period and increased registration of cases under 5 years of age. This study highlights that haemophilia services in India are still in their infancy, and increased awareness among healthcare providers as well as expansion of treatment facilities are still the primary areas for improvement of treatment.