Porphyria

The following post is a question and answer post on Porphyria

I am a registered nurse aged 49, and have been in poor health for several years. Within two years I had two serious operations. After the second surgery my blood pressure remained over 210/120, and it was discovered I was suffering from porphyria with mental confusion. The doctors tell me I am sensitive or allergic to morphine and its derivatives, barbiturates, phenergan, sulfas; that I will always have porphyria, and that probably I have had it all my life. How serious is porphyria? What can be done for it? I eat well, and I gained almost 25 pounds after surgery, but I still have high blood pressure, edema, and poor coordination at times. I take only one medication, but I feel no benefit from it. I have been told porphyria can be fatal.

Porphyria includes a group of diseases in which large amounts of abnormal sub­stances called uroporphyrins occur in the urine. Porphyria is one of several condi­tions that could cause inherited meta­bolic irregularities, ranging in severity from a mild condition to a pronounced

disease process.

There are two major groups of porphyrias. One involves the production of red cells in the bone marrow, and the other type involves the liver, in which excessive uroporphyrins are formed.

The first group, or erythropoietic por­phyria, a condition people are born with,often is discovered in infancy or child­hood. It may cause certain skin lesions or a rash.

The second group, the hepatic (liver-related) type, may be called acute inter­mittent porphyria. Your condition may refer to it. This condition usually is first noticed between the ages of 20 and 40,and it is more likely to occur in women than in men. This type of porphyria may cause attacks of severe abdominal pain associated with such related symptoms as vomiting, diarrhea, and bloating of the abdomen. The colicky nature of the pain may simulate acute abdominal conditions, and it may even result in exploratory surgery.

Patients with this disease can develop various nervous-system symptoms such as anxiety, irritability, restlessness, and even more severe symptoms; for example, con­fusion or even delirium, according to the severity of the condition. High blood pres­sure, a fast heart rate, liver insufficiency, and fever may develop. In such cases the urine may become dark red or black on exposure to sunlight, owing to the large amount of abnormal end-products formed

in the liver.

Treatment should be adjusted to the needs of the patient by the doctor. There are medications that can give rapid relief of the symptoms of porphyria.