Unmeasured costs of haemophilia: the economic burden on families with children with haemophilia.

INTRODUCTION AND OBJECTIVES: Although economic evaluations of haemophilia-related care have highlighted both the health care payer and societal perspectives, the costs to families with children with haemophilia have not been examined. This study determined the costs incurred by families of children with haemophilia, attending a haemophilia treatment centre (HTC), servicing a large geographical area in Eastern Canada. METHODS: Families recorded all direct and indirect costs associated with haemophilia-related care for a year. Costs incurred to receive care at the HTC and local health care centres were compared. The relationship between distance to the HTC and costs was modelled using linear regression. RESULTS: Participants included 31/45 children (68%) from 27 families attending the HTC. Median age was 12 years (range: 0.5-17 years); 24/31 (77%) had severe haemophilia. The median distance to the HTC and local health care facility was 230 km (range: 7-600 km) and 33.5 km (range: 2-400 km) respectively. Due to this difference in distance, 23/31 (74%) children do not attend the HTC for management of acute haemorrhage. The median annual total cost per family to attend the HTC is $775.93 (range: $200.00-$5741.00). The total cost to attend the HTC increases by $2.16 (95% CI 1.24-3.9) per kilometer from the HTC. The median total annual cost of haemophilia-related care per family is $1222.50 (range: $396.00-$8037.00). CONCLUSION: Families incur high costs related to haemophilia care. The distance to the HTC is a barrier to care. Improving access to HTCs is paramount in improving haemophilia-related outcomes.

Musculoskeletal health of subjects with hemophilia A treated with tailored prophylaxis: Canadian Hemophilia Primary Prophylaxis (CHPS) Study.

BACKGROUND: Full-dose prophylaxis is very effective at minimizing joint damage but is costly. Tailored prophylaxis has been proposed as a way of reducing costs while still protecting joints. OBJECTIVE: To report detailed findings in index joints of 56 subjects with severe hemophilia A entered into the Canadian Hemophilia Prophylaxis Study, and treated with tailored prophylaxis, after 13 years. METHODS: Boys with severe hemophilia A (< 2% factor) and normal joints were enrolled between the ages of 1 and 2.5 years. Initial treatment consisted of once-weekly factor infusions, with the frequency escalating in a stepwise fashion when breakthrough bleeding occurred. During the first 5 years, subjects were examined every 3 months using the modified Colorado Physical Evaluation (PE) scale; subsequently, every 6 months. The Childhood Health Assessment Questionnaire (CHAQ) was administered at each visit. RESULTS: Median age at study entry was 19 months (range 12-30 months); median follow-up was 92 months (range 2-156). The median PE score was 2, 3 and 3 at ages 3, 6 and 10 years. Persistent findings were related to swelling, muscle atrophy and loss of range of motion. The median score for each of these items (for the six index joints) was 0 at ages 3, 6 and 10 years. The median overall CHAQ score was 0 at ages 3, 6 and 10 years, indicating excellent function. CONCLUSIONS: Canadian boys treated with tailored primary prophylaxis exhibit minimal joint change on physical examination and minimal functional disability.

A qualitative study identifying the knowledge, attitudes and behaviours of young men with mild haemophilia.

The Canadian Physiotherapists in Hemophilia Care (CPHC) sought to learn about attitudes and behaviours of young male adults with mild haemophilia towards their condition and care. Semi-structured in-person or telephone interviews were conducted with 18 young men from and across Canada. This report summarizes the participants' attitudes towards their haemophilia, previous injuries, perceived barriers to seeking treatment, as well as their decision-making process when self-assessing injury. The interviews demonstrated that communication between the young adults and the health care team was not optimal, with common reference to the ineffectiveness of lecture style education. Gaps in knowledge also emerged regarding bleed identification and management.