Skip to main content

101 Acute Hepatic Porphyrias 3 of 3

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 Recordati Rare Diseases 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 (and are available  from the APF as part of the ER Kit). 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 that 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 those who inherit the gene 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.


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 and the APF drug database 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.


Popular posts from this blog

Amazing patient advocacy....and needed media for this issue. Thank you Terri Witter!

Meet Shadow Jumper Mitchell Felts

Meet Shadow Jumper
Mitchell FeltsMitchell Felts, age 12 How old were you when you were diagnosed?
I was 9 Years old. Do you remember your first flare/reaction?
No, I do not recall my first flare & reaction. What did it feel like to you?
It was tingling bad on my skin. What things help you feel better? (cool water, ice, shade, bath, clothes)
It helps me when I use cold rags and ice packs on my skin. How long does it take before you start to feel better?
Sometimes, I start to feel better after the first day, but sometimes it takes 2-3 days or longer to feel better. What kind of clothing/trends do you wear when you go outside or in bad lighting?
I can use long sleeves, hats and umbrellas. What is your most favorite sport to play or watch? Do you play it inside or outside? During the day or at night?
I enjoy playing baseball late in the day and it feels great to play at night. What ways are you able to adapt to do certain activities outside?
I must cover up, its hot outside so I must take breaks to c…

Medical Moment: Patient/Physician Relationship

Medical Moment: Patient/Physician Relationship
We all want a great relationship with our doctors, right? You, as the patient have a responsibility to establish a solid rapport with your physician and other members of the healthcare team. This can have a positive impact on the quality of care and better access to treatment. Yes, there could be a stigma associated with having Porphyria but don’t let it stop you from receiving the proper treatment that you deserve. Communication is KEY! Below you will find some key elements and tips that will not only prepare you for your visit with your doctor, but also build a strong relationship.
Here are a few tips for your doctor’s visit: 1. Plan – Be prepared! Prepare your questions and concerns beforehand. You want to be courteous of your physician’s time with you. 2. Make a list – Make a list of your questions, concerns and any other relating information. 3. Communication is key – Make sure that you understand fully what the doctor is explaining/a…