Acute intermittent porphyria is a rare genetic disorder that causes porphyrins to build up in the body. This can lead to acute AIP attacks, characterized by severe abdominal pain, nausea and vomiting, psychosis and, in severe cases, paralysis and heart arrhythmia. Acute intermittent porphyria does not have a cure. Diet, however, plays a large part in the treatment and prevention of acute attacks and certain kinds of foods can lessen the length and severity of an attack. You should always consult your hematologist before attempting to control an acute attack through diet, as AIP can be life-threatening if it is treated incorrectly.
Glucose and AIP
Acute intermittent porphyria is caused by a defect in an enzyme called porphobilinogen deaminase, or simply PBGD. When other factors such as diet come into play, an attack occurs. If you don’t eat enough carbs, your body will activate proliferator-activated receptor y coactivator 1a. PGC-1a in turn increases the expression of aminolevulinate synthase-1 in the body, which results in an acute attack. Taking in a large amount of carbs reverses this process at least partially, which is why carb loading is used to treat and prevent acute attacks.
There are a variety of different foods and drinks that you can take in. You will want to incorporate a mixture of simple and complex carbohydrates. The simple carbohydrates enter your bloodstream almost immediately, helping to reduce porphyrin production in your body right when you need it. The complex carbohydrates give your body a steady time-released supply of glucose to keep levels high and stable. Recommended simple carbohydrates include fruit, honey, syrup and other sugary foods. Honey is especially good because it contains a large amount of carbohydrates and it enters the bloodstream straight from the stomach. This makes it a good choice if you are nauseous. Complex carbohydrates that are recommended for acute attacks include beans, oats and whole-grain breads and pasta. If you are feeling too nauseous to eat, try taking small, but frequent, sips of sugary drinks, such as fruit juices and sports drinks.
During an attack, you will need to take in a minimum of 300 g of carbohydrates a day, according to a study published in the August 2005 issue of “Cell.” This translates into 1,200 calories of carbohydrate daily. It is best to consume small amounts evenly throughout the day, taking in at least 50 grams of carbs every three hours. The first dose should comprise mostly simple carbohydrates. The makeup of the following meals and snacks can be mixed. It is important that you monitor your blood sugar at this time to make sure it is not too high. You’ll need to obtain a home monitor and check your sugar roughly every three to four hours. Consult your hematologist if your blood sugar measures above 150.
Diet and Prevention
A proper diet is not only useful in treating acute attacks, but also in preventing them. While maintaining proper levels of carbohydrates is important, a well-balanced diet is key. Carbohydrates should make up 60 percent of your diet, according to the American Porphyria Foundation. You’ll also need to take in a large amount of fiber, 40 grams to 50 grams daily. This is especially true if you are female, as women are more prone to attacks due to hormonal fluctuations. There are also certain dietary restrictions you must adhere to if you want to decrease your likelihood of getting an attack. You should avoid eating excessive amounts of protein, as a high-protein diet can be triggering. Alcohol must also be avoided.
Precautions and Considerations
Unless the attack is mild and glucose loading is done at the onset, hospitalization is usually required. You will need medication to control your pain and to make you feel less nauseous. If you cannot eat due to nausea, you will get glucose infused directly intravenously. If your attack is severe, you will probably need treatment with Panhematin, the only medication available that is specifically targeted to treating acute porphyrias. Always consult your hematologist when you think you are experiencing an attack. An acute episode can be life-threatening. You should not self-treat at home without first obtaining guidance and advice from your physician.
- “Clinics in Liver Disease”; Hepatic Porphyrias: Diagnosis and Management; Annie Chemmanur, et al.; November 2004
- Rare Diseases Clinical Research Network: Acute Intermittent Porphyria
- American Porphyria Foundation: Nutrition in the Acute Porphyrias