NUR 01.8 - Nursing management of patients with Acquired Haemophilia A

Background: Acquired Haemophilia A (AHA) is a rare bleeding disorder whereby antibodies are made against plasma coagulation factors that can manifest in severe or sometimes fatal bleeding. Diagnosis can be delayed due to limited knowledge amongst general practitioners and general haematologists. Treatment includes initial bleed management, treat underlying idiopathic condition if identified and suppression of the immune system to attempt to eliminate the antibody.

Aims: Specialised nursing management can enhance patient outcomes.

Methods: A reflection of 15 years of experience at the Queensland Haemophilia Centre (QHC) with patients with AHA. Nursing management consisted of acute bleed management, management of side effects of steroid therapy and education and liaising with patients, caregivers, and medical and nursing staff at local and regional hospitals.

Results: Patients were managed locally or at regional hospitals under the care of Haematologists with the input from QHC. Due to the rarity of the disease patients were unprepared for the acute upon chronic aspect of the disease process. Follow up was intense with most patients attending weekly outpatient clinic for blood test and review, as symptoms improved, and blood tests normalised reviews became less frequent. Patients and care givers were educated on how to recognise bleeding episodes and provided with criteria for when to seek medical advice. Treatment with immunosuppression therapy to eradicate the inhibitor can lead to other complications such as sepsis, worsening diabetes mellitus, mucocutaneous ulcers and worsening of psychiatric disorders. Patients were instructed to monitor symptoms and to have regular follow up with their specialists and general practitioners.

Conclusion(s): A specialised haemophilia nurse is pivotal to the management for patients with AHA as they provide patients with reassurance, facilitate care and can assist to navigate an unfamiliar health care system.