Palliative Care Aspects of Acute Intermittent Porphyria – A Case Report

Abstract

Acute intermitttent porphyria belongs to a rare group of diseases hallmarked by deficient biosynthesis of heme. It carries a significant symptom burden, both physical and emotional,and therefore palliative care has emerged as an essential tool in the armamentarium of porphyria management . It takes care of the patient as a whole and caters to all aspects that the disease process demands. There are many lacunae in the literature regarding the palliative management of porphyria. We are reporting a case of a 16-year-old female who presented with severe abdominal pain, lower backache and symmetrical bilateral lower limb pain to the palliative ward referred by the neurology department for supportive care. This case describes the palliative care aspects of porphyria management which was successfully provided in the palliative care unit right from referral till the last. A multidisciplinary palliative care team managed the patient, and the necessary interventions were provided to the patient and family. Palliative acre in AIP needs to be emphasized, and palliative care services need to be utilized in these cases. The unavailability of specific treatment measure, heme, in countries like India further emphasizes the need for long-term supportive care for the patient and family. The case shows the importance of palliative care throughout the disease course as it is a chronic disease with significant morbidity and carries a heavy symptom burden. This case provides the insight that rather than conventional management alone for such chronic diseases, palliative care should be incorportated. Early integration with palliative care helps in exploring all the domains of disease. This is one of the first cases reported highlighting palliative care in porphyria , bridging the gap in the literature.