Mortality, health care utilization and associated diagnoses in hospitalized patients with haemophilia in the United States: first reported nationwide estimates

Abstract

To describe the in-hospital epidemiology of haemophilia A and B in the US we analysed the National Inpatient Sample (NIS), a stratified probability sample of 20% of all hospital discharges in the US for the year 2007. We applied sampling weights to represent all hospital discharges for haemophilia A and B identified using ICD-9 codes 286.0 and 286.1, respectively. Haemophilia (A or B) was one of all the listed diagnoses in 9737 discharges and principal diagnosis in 1684 discharges. The most common associated diagnoses in discharges with Haemophilia in adults and children were hypertension (28.1 ± 1.6%) and central line infections (15.2 ± 1.8%) respectively. No Hepatitis C or HIV was reported in children. Among 212 deaths, associated diagnoses included sepsis (37.9%), heart failure (30.2%), respiratory failure (28.3%), pneumonia (24.5%), HIV (14.2%), hepatic coma (5.2%) and intracranial haemorrhage (2.3%). All fifteen reported paediatric deaths occurred on day zero of life, the commonest associated diagnoses being Intraventricular haemorrhage and newborn haemorrhage-NOS (33% each). Median age of in-hospital mortality for diagnosis of Haemophilia was 68.3 years as compared to 72.3 years for all males for all hospitalizations in NIS combined. Mean hospital charges for haemophilia of $76823 ± 5530 were significantly higher than those from all causes of hospitalization of $26,120 ± 562. In-hospital mortality is rare in children with haemophilia beyond the neonatal period and age of mortality in adults is approaching that of the general male population. Hospitalization in children is most often due to central line infections and hospitalization and death in adults is primarily due to age-related illnesses.