Congress Abstracts 2006

110

UPDATE ON NURSING MANAGEMENT OF PATIENTS WITH ACUTE PORPHYRIAS. Joyce Loyal, RN, BSN, OCN^®, Wishard Health Services, Indianapolis, IN.

The porphyrias are genetic disorders of heme biosynthesis, which clinically manifest themselves in a variety of ways, all yielding unique challenges to nurses in diagnosis, treatment and patient education. Porphyria is not a single disease, but a group of at least eight disorders all due to deficiencies in the same metabolic pathway, having the common feature of accumulation in the body of porphyrins or porphyrin precursors. Porphyrins combine with iron to create heme, which attaches to protein to form hemoglobin.

Though the acute porphyrias are relatively rare disorders, occurring in approximately 1 per 100,000 persons, oncology nurses are often involved in the management, treatment and education of these patients. Therefore it is crucial that oncology nurses have an understanding of this disease and the implications for nursing care. The purpose of this poster is to present the pathophysiology, common clinical features, exacerbating factors and the nurse's role in the management of porphyrias. After viewing this poster, the nurse will be able to:

1. Describe basic pathophysiology of porphyria.
2. Describe common clinical presentation of patients experiencing acute exacerbations.
3. Discuss treatment of porphyria attacks.
4. Discuss critical patient education components.
5. Identify patient risk factors for the development of hepatocellular carcinoma.

In our center, nursing care for patients with acute porphyrias, includes the administration of glucose containing IV solution for mild attacks, the administration of analgesics for the management of pain, and the administration of hemin(Panhematin) to replenish cellular heme pools. Patient education is a necessary component of nursing care and should include signs and symptoms of exacerbation, information regarding precipitating factors, analgesic and treatment regimens, and also possible long term effects.

Prevention of future attacks or decrease in frequency can be significantly impacted by the patient's ability to recognize early signs and symptoms of an impending attack, and education in the avoidance of precipitating factors.

Oncology Nurses have a critical role in the management of symptoms associated with acute porphyria, and providing support and education to assist patients in coping with the long-term effects of these disorders.