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DEAR DR. GOTT: I would appreciate any information you could give me about hepatic porphyria. I have it and would like to know about treatment.
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DEAR READER: Porphyria is a rare, inherited neurodegenerative condition that results in a buildup of chemicals called porphyrins in the body. these are normal body chemicals. what isn’t normal is when they build up and affect the nervous system, the skin or both. The condition is from a disruption in the body’s production of heme, the nonprotein portion of hemoglobin that contains iron. We all have heme in our tissues, with the greatest amounts found in the red blood cells, liver and bone marrow.
There are eight enzymes that convert porphyrins into heme. In your condition, an inherited mutation in one of your genes has caused a deficiency that leads to the buildup of porphyrins in the body.
You likely received this inherited condition from one of your parents (an autosomal dominant pattern) or from both parents (an autosomal recessive pattern). The interesting thing is that even though you have been diagnosed, you may or may not experience any symptoms. if you are exhibiting symptoms, they may be triggered by exposure to the sun, infection, the use of alcohol, hormonal factors, dieting, excess iron in your system or a host of other possibilities.
Generally speaking, porphyria is divided into two categories — acute and cutaneous.
The acute form generally affects the nervous system and often presents with severe abdominal pain, vomiting and constipation, which then progresses to muscle pain, numbness, weakness, diarrhea, seizures, fever, hallucinations, high blood pressure and red urine.
This form is rare in people prior to puberty or following menopause. The cutaneous form affects the skin with symptoms of blisters and skin swelling within minutes of exposure to the sun, redness and itching.
Because you have the hepatic form, this implies liver and possibly kidney involvement. while I don’t know your specific case, your kidney function could be less than optimal, and your liver may be damaged. The extent of that involvement should determine what your hematologist plans as the next step in your care that may be as simple as discontinuing medication that acts as a trigger or as complicated as dialysis or surgery. good luck.
DEAR DR. GOTT: I have a question regarding treatment of a 5-year-old who has been diagnosed with allergies to dust mites and mold. The allergist prescribed Flonase nasal spray and Zyrtec when he’s bad. this seems a little extreme, as it is a steroid and the only symptom she presented with was frequent colds. Is there any other treatment?
DEAR READER: I cannot, in clear conscience, recommend the use of the steroid Flonase for a child, primarily because the medication can affect his or her growth at such a vulnerable age.
Furthermore, pediatric Zyrtec (an antihistamine) studies involving 168 children aged 2 to 11 found side effects to be mild to moderate. Studies involving children aged 6 to 11 given doses between 1.25 to 10 milligrams daily found symptoms of headache in 11 percent of users, pharyngitis in 6.2 percent, abdominal pain in 4.4 percent and epistaxis (nosebleeds) in 3.7 percent.
As a non-pediatrician, I am not qualified to make such an enormous decision. Speak with your daughter’s pediatrician regarding the allergist’s recommendations. Children are precious and should be treated with the utmost of care.
Dr. Peter H. Gott is a retired physician and the author of several books, including “Live Longer, Live Better,” “Dr. Gott’s no Flour, no Sugar Diet” and “Dr. Gott’s no Flour, no Sugar Cookbook,” which are available at most bookstores or online. His website is AskDr GottMD.com.
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