AIP, HCP, VP, & ADP

Precautions

Most individuals who inherit AIP seldom have symptoms if certain precautions are taken. The following general recommendations are made for individuals with low PBG-D blood test results:

1. Avoid harmful drugs, smoking, alcohol, hormones, and fasting. Make sure you and your physician review your medications for safety before you take them. Both of you should feel free to consult one of the experts in porphyria for this purpose since information about drugs and porphyria is difficult to find.

2. Inform all of your physicians that you have AIP. You should know whether your urine is usually positive for PBG. Many physicians are not experts on porphyria, thus, it is helpful for patients with AIP to carry information about themselves. A Medic Alert card and bracelet or necklace are also recommended for those who are susceptible to AIP attacks. In case unexpected emergencies occur, they could prevent harmful drugs from being administered.

3. Review requirements for surgery with your physician before the procedure. Surgery should be done without barbiturate anesthesia. The anesthetic gases are probably safe in AIP. Since major surgery interferes with nutrition, at least 300 grams of glucose should be infused intravenously during surgery and on a daily basis for a period of time thereafter.

4. Check urine periodically for PBG, particularly in children at the time of puberty. If the urine does become positive for PBG, most individuals still remain asymptomatic. However, since AIP symptoms are almost always associated with high PBG output, it is useful to have such information for future reference.

5. Contact porphyria specialists or medical centers that have expertise in porphyria in case unforeseen questions arise concerning drugs, treatments and other matters.

6. Be your own best advocate by educating yourself about porphyria. Challenge your health care providers to also become very knowledgeable. It is essential to transfer from any health care providers who refuse to acknowledge the importance of this disease.

7. Seek medical attention immediately when you feel ill with anything other than ordinary illnesses.