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

Abstract

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 agerelated 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.

All‐cause mortality and causes of death in persons with haemophilia: A systematic review and meta‐analysis

“…Thirty studies describing causes of death in the overall PWH population were used to examine proportionate mortality 1–2,7–12,14,17–18,25–43 . Causes of mortality were extracted for a total of 7703 deaths.…”

“…Altogether 47 studies reported causes of death. Of them, 30 non‐overlapping studies 1–2,7–12,14,17–18,25–43 reported proportionate mortality in the overall PWH population and are presented in Tables 3–4. Results from 17 studies that reported causes of death in subpopulations of PWH or are overlapping with other studies are presented in the supplementary appendix 13,15,19–20,44–58 …”

“…Thirty studies describing causes of death in the overall PWH population were used to examine proportionate mortality. [1][2][7][8][9][10][11][12]14,[17][18][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43] Causes of mortality were extracted for a total of 7703 deaths. Before the year 2000, 31.2% deaths occurred due to HIV followed by haemorrhage (26.0%), cardiovascular disease (18.2%), liver disease (9.0%) and cancer (8.9%) (Table 3).…”

Comorbidities, Health-Related Quality of Life, Health-care Utilization in Older Persons with Hemophilia—Hematology Utilization Group Study Part VII (HUGS VII)

“… 19 This is also noted in an analysis of life expectancy and cause of death in individuals with hemophilia A and B in Norway from 1986-2018, which showed that the persons with severe hemophilia died at a mean age of 53.4±21.7 compared to 71.5±19.6 for mild and moderate hemophilia. 20 …”

Applicability of the European Society of Cardiology Guidelines on the management of acute coronary syndromes to older people with haemophilia A – A modified Delphi consensus by the ADVANCE Working Group

“…Major advances in therapy and treatment strategies have been reflected in an increased life expectancy and improved quality of life for people with haemophilia (PWH). [1][2][3] However, as PWH of all severities without inhibitors now have longer life expectancy, an increase in the incidence of cardiovascular diseases (CVDs), cancer, and other comorbidities is to be expected, 4,5 especially as PWH are equally or even more prone to CV risk factors such as obesity, hypertension, and diabetes than the general population. 6 - 11 For example, a retrospective study of PWHA/B aged ≥40 years who attended the Gulf States Hemophilia and Thrombophilia Center (GSHTC) reported that 78% of the study population had at least one CV risk factor including hypertension (46%), overweight/obesity (65%), high cholesterol (16%), or/and diabetes mellitus (14%).…”