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Communication with acute intermittent porphyria patients who have tracheostomy

Author(s): Sevda Arslan | Özlem Altınbaş Akkaş

Journal: International Journal of Human Sciences
ISSN 1303-5134

Volume: 10;
Issue: 2;
Start page: 94;
Date: 2013;
Original page

Keywords: Acute intermittent porphyria | tracheostomy | communication

Objective: Introducing acute intermittent porphyria disease and investigating the communication techniques for acute intermittent porphyria patients who have tracheostomyCase Report: 26 years old woman patient consulted to the hospital when she was 13 years old with nausea and vomiting complaints. In 2008, because of respiratory arrest, tracheostomy was implemented to the patient who had acute intermittent porphyria diagnose. The patient had two bullous lesions on the left hand and hyperpigmentation’s on the face and legs. As a result of metabolic acidosis (because of urine disease), the patient was taken to dialysis. After six months of dialysis, hypernatremia was found in the laboratory tests (Na: 85).Period of acute crisis developed In the patient with hypertension, atrophy in the muscles, fainting, abdominal pain, nausea, vomiting and diarrhea. Because of serious diarrhea, TPN was started for the patient who was fed with gavage before. Laboratory test results are Na:126, K:8.1, BUN:23.83, Urine:51, Creatinine:0.94, Leukocyte:20.4, erytrocyte:2.22, Hb:6.87g/dl, Hct: %20.9, Plt:106. Result: It is really difficult to communicate with these patients with lifespan limited because of an extremely severe clinical condition and tracheostomy.
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