Acute intermittent porphyria

Acute intermittent porphyria attacks can last several days to weeks. Most acute intermittent porphyria attacks last less than 2 weeks.

Women with acute intermittent porphyria (AIP) have attacks more often than men. Also, people with AIP from Sweden are at an increased risk of developing liver cancer. People with AIP may also have different symptoms or triggers.

You can take steps to lower the chance of having an attack of acute intermittent porphyria by avoiding triggers and living a healthy lifestyle. Triggers to avoid include medications not prescribed by your doctor, illegal or street drugs, too much alcohol, smoking, and going long periods of time without eating. A healthy lifestyle includes a balanced diet, regular meal times, visiting the doctor and getting treated for any illnesses, and reducing stress. There are also treatments such as heme therapy that can prevent attacks. You can speak to your doctor to see if it is right for you.

Preventing ovulation in women can prevent attacks of acute intermittent porphyria, since hormones associated with the menstrual cycle can trigger an attack. Also, regular infusions of hemin (usually weekly) can help decrease the risk of attacks. Maintaining a good diet that includes a healthy level of carbohydrates and calories can also help decrease the chance of an attack. In most severe cases, the patient may need a liver transplant to cure the disease but this is the last resort.

It is very rare to have an attack of acute intermittent porphyria before puberty. Usually, people start having symptoms of the condition or attacks in their 30s to 40s.

Attacks of acute intermittent porphyria can range from mild to life threatening. If someone is having an attack, they should seek medical attention to make sure they get the care they need. People who have had serious attacks should also wear a medical alert bracelet so that their condition can be properly treated if an attack occurs.

It may only take a few days to recover from an attack of acute intermittent porphyria. However, recovery from severe attacks can take weeks to months. Sometimes, long term, or chronic damage like nerve damage and muscle weakness can occur.

Mental changes in attacks of acute intermittent porphyria can include insomnia or difficulty sleeping, anxiety, depression, hallucinations, confusion, paranoia, and amnesia or memory loss. It is very rare for these to be the only symptoms of an attack, but it may happen. See your doctor if you have acute intermittent porphyria and are having mental changes.

If you have acute intermittent porphyria, some medications/drugs you should not take are barbiturates, sulfa-containing antibiotics, some antiepileptic drugs, progestagens, and synthetic estrogens.

A woman's menstrual cycle can impact the risk of having an attack. Acute attacks are common in the luteal phase of the menstrual cycle, which occurs right after ovulation and before the woman's period. In other words, attacks can be common in the middle of a woman's cycle.

In general, women seem not to have more attacks during pregnancy. However, it is possible that pregnancy can trigger an attack. Pregnant women with symptoms of acute intermittent porphyria should first be evaluated for other complications of pregnancy. If a woman is having an attack, her doctor should be careful to only treat her with drugs that are not dangerous to the baby, if possible. Hemin infusions appear to be safe in pregnancy.

While it is possible that an acute attack can be deadly, it is very rare for a person with acute intermittent porphyria to die from an attack. This is because we have good treatments for the most dangerous symptoms. However, death may occur if a person develops liver cancer or if they need a liver transplant and it is not successful.

You and your doctor can work together to decide the best path to treat your acute intermittent porphyria (AIP). Usually, patients who need liver transplants are those that are having a life-threatening attack, don't respond to medicines, or have a poor quality of life because of the disease. This is mostly a last resort treatment.

Smoking is a known trigger of acute intermittent porphyria attacks and should be avoided, if possible.

Most doctors with experience caring for people with acute intermittent porphyria strongly recommend that you avoid alcohol as much as possible. If it is not possible for you to complete avoid alcohol, it is best that you try to drink as little as possible. Heavy red wines, brandy, and other liqueurs are known to be particularly bad for triggering attacks.

One of the known triggers of an attack of acute intermittent porphyria (AIP) is having a low calorie intake or going too long between meals. Because of this, it may not be safe for you to go on a new diet to lose weight if you have AIP. If you have concerns about your weight, you should talk to your doctor and a dietician about how to safely change your eating habits without risking an attack of AIP.

You can learn more about diet and nutrition information for people with AIP here.

It is important that you have something that informs people of your acute intermittent porphyria in case of an emergency. This can be a wrist bracelet, a neck pendant, or an information card. This is important because you might not be able to explain that you have acute intermittent porphyria during an emergency.

Female hormones, especially progesterone, are known triggers of attacks of acute intermittent porphyria. Progesterone is found in many hormonal birth controls, like the pill. For this reason, birth control pills with progesterone should be avoided if you have acute intermittent porphyria. Also, long-term birth control medicines that are injected or implanted (like in intrauterine device, or IUD) can be very dangerous and should always be avoided by women with acute intermittent porphyria. You should speak to your doctor about the birth control options that may be right for you.

Some genetic tests specifically look at what are called SNPs (single nucleotide polymorphisms) in the HMBS gene. SNPs are the most common type of genetic variation among people. Each SNP represents a single DNA building block (nucleotide). Most SNPs do not have an effect on the health or development of the individual. However, they can sometimes help determine someone's response to certain drugs or their risk of developing particular diseases.

A genetic counselor can help with ordering further diagnostic testing and determine which labs in the U.S. conduct testing for porphyria would be best in your situation if testing is appropriate. (Take a look at "Find a Genetic Counselor" in your area http://nsgc.org/p/cm/ld/fid=164).

There is also an excellent organization called the American Porphyria Foundation that has some great resources for people with various types of porphyria. They may be able to assist in determining different potential triggers or how other people avoid them.