Life expectancy and cause of death in individuals with haemophilia A and B in Norway, 1986‐2018

OBJECTIVES: Evaluate trends over time in age- and cause of death in males with haemophilia (PWH) in Norway compared with the general male population and investigate its correlates with improvements in haemophilia treatment. METHODS: Data about age and cause of death in the period of 1986-2018, from two independent, high-quality national registries: the Norwegian Cause of Death Registry (NCoDR) and the patient registry at Centre for Rare Disorders (CRD), Oslo University Hospital. RESULTS: Life expectancy increased significantly from 1986 to 2018. However, PWH still had a decreased mean age at death of 56.8 years (SD = 24.7) in the NCoDR and 58.6 years (SD = 21.7) in the CRD data, compared with 73.9 years (SD = 16.3) in the general male population. There was a distinct shift in the most frequently reported haemophilia-related causes of death, such as haemorrhage and AIDS, to more age-related causes of death, such as cancer, reflecting an ageing population. CONCLUSION: Haemophilia treatment has improved significantly in the last three decades. Despite treatment-related improvements, PWH in Norway still have a decreased life expectancy compared with the general male population.