Haemophilia: diagnosis, management and nursing care of patients

This article has been double-blind peer reviewed

In this article…

• Causes of haemophilia and how it affects patients

• Current treatments and future developments

• Nurse's role in supporting people with haemophilia

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Key points

Congenital haemophilia is a lifelong, inherited bleeding disorder associated with a lack of clotting factor

There are two main types of haemophilia, which have identical symptoms, but treatment depends on which clotting factor is missing

The hallmark of severe haemophilia is recurrent and spontaneous joint bleeds which, if left untreated, can cause deformity

Some bleeds, such as those in the skull and soft tissue around airways or other internal organs, can be life threatening

Recombinant clotting factor and non-factor replacement therapies mean more people with haemophilia are living into old age

Abstract

Haemophilia is a rare bleeding disorder associated with a lack of coagulation factor VIII (type A) or IX (type B). Haemophilia A is the most common, affecting 1 in 5,000 male births. The hallmark of severe haemophilia is recurrent and spontaneous joint bleeds, which can cause permanent joint damage and bleeding in other locations, such as the skull; they can be life threatening. However, recombinant clotting factor and non-factor replacement therapies are transforming the outlook for people with haemophilia, and more people are living with the disorder into old age. This article describes haemophilia and its management.