Acute Intermittent Porphyria

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Diseases defined by the abnormal metabolism of hemoglobin are classified as porphyrias. Porphyrias cause an abnormal buildup of porphyrin ring compounds that are essential for proper function on hemoglobin. There are multiple different classifications of porphyria, with acute intermittent porphyria being the most common. Porphyrins, the building blocks of hemoglobin, are excreted in the urine of affected people, causing extreme symptoms that range from pain to neurologic disturbances. While there are ways to treat symptoms and methods to avoid frequent attacks, there is no cure do to the foundation of the disease beginning at the genomic level. Symptoms from acute attacks can be treated by avoiding and suppressing stressors. The disease …show more content…
Because the foundation of acute intermittent porphyria is a genetic deficiency, there is currently no known cure, but many steps to treat the disease can be taken. The first sept in treating this disease is acute attack management. This includes determining medications that induce attacks, addressing proper hydration, and proper diet (Bissell & Wang, 2015). Intravenous fluids should be administered along with glucose, in an attempt to alleviate primary attack symptoms. By maintaining healthy levels of hydration and a consistent glucose level patients are able to reduce the onset of an acute attack. Because this disease primarily affects women, gonadotropin releasing hormone analogues will help avoid acute attacks prior to menstruation (Bissell & Wang, 2015). Gonadotropin releasing hormone analogues can help alleviate menstrual cycle related attacks by suppressing the secretion of luteinizing hormones. By preventing the natural cyclic pattern of menstruation, the hormone levels in the body are permitted to remain constant, removing the added stress from the body. Women diagnosed with acute intermittent porphyria need to be aware that attacks prior to menstruation are common but may still occur when using gonadotropin releasing hormone analogues. It has been found that women should avoid oral contraceptives as they have been linked with acute attacks, most likely due to the …show more content…
Prophylactic hemin is a porphyrin chelate of iron, derived from red blood cells. Panhematin® is one of many prescribed medications for the treatment of acute intermittent porphyria attacks. Hemin is administered to avoid the progression of generalized pain to a neurological crisis. Because delta-aminolevulinic acid, the first step in heme synthesis, is regulated by a heme feedback mechanism, it was determined that acute porphyrias represent a deficiency in hepatocytes (Bissell & Wang, 2015). Hemin helps reduce 5’-aminolevulinate synthase-1 activity allowing the liver to reduce production of delta-aminolevulinic acid and porphobilinogen (Besur, Hou, Schmeltzer, & Bonkovsky,. 2014). Due to the issue originating in the liver, hemin is most effective best when given intravenously, to avoid the first pass mechanism in the body. This allows hemin to reach the liver before the body is able to metabolize the drug. Like any drug, prophylactic hemin has side effects that should be monitored by medical

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