AIP, HCP, VP, & ADP

Treatment During Attacks

Pain can be the primary and only complaint of a patient in a porphyric crisis and sometimes for some time beyond. Appropriate pain measures should be applied and it would be unwise and not good medicine to overrule a patient by withholding medication.

Hospitalization is often necessary for acute attacks. Medicine for pain, nausea and vomiting along with close observation are required. Initial treatment of AIP attacks consists of stopping harmful drugs the patient may be taking and providing a high intake of carbohydrate, 300 grams or more per day. Carbohydrate can be given either in the form of an oral carbohydrate or by intravenous infusion. Intravenous infusion is better in moderate or severe attacks or for patients who are unable to ingest enough carbohydrate orally. Pain, anxiety and emotional symptoms should be treated with safe drugs. Attacks with muscle weakness occasionally require respiratory support, but this is unusual unless an attack is brought on by prolonged administration of harmful drugs. After recovering from an attack, a patient should continue to eat regularly, because there is good evidence that skipping meals or fasting is harmful.

Heme therapy is a treatment for AIP that is given intravenously. It is useful during severe attacks. The best results are achieved if heme therapy is started early in the attack. It is also administered to prevent attacks that are readily predictable. Heme therapy is commercially available through Ovation Pharmaceuticals, Inc. as Panhematin^®. Panhematin^® is less likely to produce phlebitis if it is mixed with human albumin before it is given. Directions for preparing Panhematin^® in this manner can be obtained from porphyria specialists. Panhematin^® therapy is seldom indicated unless the diagnosis of acute porphyria is proven by a marked increase in urine PBG. How heme therapy should be used to prevent attacks is not well established.

Heme arginate, which is marketed in some other countries, is another preparation of heme therapy for intravenous administration, but it is not available in the United States.

AIP is particularly dangerous if the diagnosis has not been made and if harmful drugs are continued. The prognosis is usually good if the disease is recognized and if treatment and preventive measures are begun before severe nerve damage has occurred. Although symptoms usually resolve after an attack, some patients develop chronic pain. Nerve damage and associated muscle weakness can improve over a period of months or longer after a severe attack. Long term expert physical therapy and other rehabilitation therapies are essential. In addition, mental symptoms may occur during attacks but are usually not chronic.

AIP patients prone to attacks should eat a normal or high carbohydrate diet and should not greatly restrict their intakes of carbohydrate and calories, even for short periods of time. If weight loss is desired, it is advisable to consult a physician who may then request that a dietitian estimate an individual's normal caloric intake (this varies greatly from one person to another). Then it may be appropriate to prescribe a diet, which is approximately 10% below the normal level of calories for the patient. This should result in gradual weight loss and usually will not cause an attack of porphyria.

Pregnancy is tolerated much better than was formerly believed. Offspring have a 50% chance of inheriting the gene for AIP, but the great majority of them remain "latent" for all or most of their lifetimes.

Patients with frequent intermittent symptoms should have access to physicians who are familiar with AIP and with the patient's specific medical problems. Psychiatric support is sometimes helpful as well, because emotional problems may continue even with appropriate medical treatment. If new symptoms arise, diseases unrelated to porphyria should be suspected. Like anyone else, AIP patients may develop other illnesses.