Impact Of Prophylaxis And Quality Of Life Determinants In Paediatric Haemophilia A Patients: A Prospective Study From South India

Abstract

Back ground: Bleeding disorders, such as haemophilia, can greatly influence the health-related quality of life (HRQoL) of both children and their caregivers. Evaluating HRQoL helps healthcare providers to pinpoint specific areas where additional support is needed. It also offers a valuable way to assess the impact of new treatments. There are only very few researches which assessed the HRQoL of paediatric haemophilia population of South India. Here we aimed at assessing the HRQoL of paediatric haemophilia A patients and its improvement following prophylactic treatment.

Materials and methods: HRQoL was assessed at two points of time, one year apart, in 46 male children with haemophilia A less than 17years using haemophilia specific instrument- Haemo-Qol. We have used parent proxy reported short form for 4 to 7years and paediatric short form for 8 to 17years. Demographic variables were compared with QoL scores. Difference in scores at the end of the study between prophylactic group and ‘on demand’ treatment group was also compared.

Results:  Base line mean HRQoL  scores improved from(52.044±17.14) to(47.71±20.01)at the end of the study reflecting the positive impact of prophylaxis(p value-0.000). Base line QoL scores in 4-7 yrs was (50.19±15.36) when compared to (52.88±18.03) in 8-17years, which when reassessed after 1year showed improvement in values as follows (37.11±15.06 vs 52.34±20.33).QoL scores were higher indicating poor QoL with presence inhibitors at base line and after 1 year(p value=0.000).

Conclusion:  In haemophilia A patients QoL mainly depended on the joint health which in turn is negatively influenced by presence of inhibitors and  positively by prophylactic treatment.