A five-year study of a haemophilia reference centre

Area of Science:

• Hematology
• Clinical Medicine
• Public Health

Background:

• Haemophilia A (factor VIII deficiency) and Haemophilia B (factor IX deficiency) are genetic bleeding disorders.
• The Regional Haemophilia Reference Centre in Edinburgh Royal Infirmary provides specialized care for these patients.
• Treatment availability and patterns can influence patient outcomes and healthcare resource utilization.

Purpose of the Study:

• To analyze trends in patient attendances and treatment patterns at a Haemophilia Reference Centre over a five-year period (1969-1973).
• To assess the impact of increased factor VIII availability on the management of haemarthrosis.
• To evaluate changes in the utilization of replacement therapy for haemophilia.

Main Methods:

• Retrospective analysis of patient attendances and admissions data from 1969 to 1973.
• Review of records on the types and quantities of replacement therapy used.
• Comparison of data between the initial and final years of the study period.

Main Results:

• Patient attendances increased significantly from 123 in 1969 to 624 in 1973.
• This increase was primarily driven by more frequent outpatient treatment for haemarthroses, facilitated by greater factor VIII availability.
• Total replacement therapy units increased from 2704 to 8778, with cryoprecipitate showing the largest rise. Inpatient admissions remained stable.
• Knee haemarthrosis was the most common joint affected.

Conclusions:

• Enhanced availability of factor VIII has shifted haemophilia management towards earlier and more frequent outpatient treatment of haemarthroses.
• This change in treatment patterns has led to increased centre utilization and a substantial rise in factor VIII concentrate usage.
• Future implications for resource allocation and treatment strategies in haemophilia care need consideration.