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About Acute Intermittent Porphyria (AIP)

AIP is a rare inherited disease that can cause serious symptoms that affect your body, such as your brain and nervous system. The most common symptom is severe abdominal pain. There is no cure for AIP, but it can be managed.

AIP is caused by a partial lack of activity of an enzyme known as porphobilinogen deaminase (PBGD). The PBGD enzyme is used in the production of heme, a molecule that carries oxygen throughout your body.

If you have AIP, you have about half of the normal amount of PBGD enzyme activity in your body. This is usually enough for your body to do what it is supposed to do. But certain situations can upset your body's chemical balance and disrupt the heme production process, resulting in an AIP attack.

The AIP Attack Cycle

The graphic below illustrates what happens during an AIP attack. Roll over each area of the graphic for more information.

Exposure to triggers increases the need for heme in the body. Many situations may trigger an AIP attack.

See examples below.
The body sends signals to produce more heme. Triggers may upset your body's chemical balance enough to prompt your body to produce more heme.
Heme cannot be produced fast enough due to the enzyme deficiency. In certain situations, there's not enough PBGD enzyme activity in your body to keep up with the demand.
The process gets backed up. The process gets backed up partway through heme production due to the lack of sufficient PBGD enzyme activity.
Harmful molecules are produced. Two molecules - aminolevulinic acid (ALA) and porphobilinogen (PBG) - are produced. The build-up of these toxic or harmful molecules causes the symptoms that occur during an AIP attack.

See more information below.
The heme pool is depleted. The heme pool is depleted because there is not enough PBGD enzyme activity to meet current demand. So your body keeps sending signals to produce more heme, and the dysfunctional cycle continues.

IF AN AIP ATTACK IS NOT TREATED PROMPTLY, IT CAN PROGRESS TO A POINT TO WHERE LASTING DAMAGE MAY OCCUR TO YOUR NERVOUS SYSTEM.

What are common signs and symptoms of AIP?

Severe abdominal pain: the most common AIP symptom
The most common symptom of AIP is severe abdominal pain that usually cannot be relieved with pain medicine such as Advil® (ibuprofen) or Tylenol® (acetaminophen). More than 85% of people who develop AIP symptoms have abdominal pain.

Experiencing symptoms is known as having an "AIP attack." Symptoms may occur for a set period of time, then go away - only to come back later.

People living with AIP have described the pain caused by AIP attacks as being the worst imaginable:

"My insides feel like they're on fire!" - N.T.M.

Other common AIP symptoms

Symptoms can also occur in many different areas of your body during an AIP attack. These AIP symptoms are also common in other conditions. Having one or many of these symptoms does not mean that you have AIP.  But it's important to have your symptoms checked by your doctor right away.

GASTROINTESTINAL (GI) SYMPTOMS

  • Abdominal pain
  • Vomiting
  • Constipation
  • Diarrhea

Other common AIP symptoms

Symptoms can also occur in many different areas of your body during an AIP attack. These AIP symptoms are also common in other conditions. Having one or many of these symptoms does not mean that you have AIP. But it's important to have your symptoms checked by your doctor right away.

URINARY SYSTEM SYMPTOMS

  • Dark-colored urine
  • Red urine

Other common AIP symptoms

Symptoms can also occur in many different areas of your body during an AIP attack. These AIP symptoms are also common in other conditions. Having one or many of these symptoms does not mean that you have AIP. But it's important to have your symptoms checked by your doctor right away.

BRAIN OR NERVOUS SYSTEM SYMPTOMS (NEUROLOGICAL SYMPTOMS)

  • Pain in your arms, hands, legs, feet, back, chest, neck, or head
  • Weak muscles
  • Trouble breathing
  • Psychological symptoms (such as minor behavioral changes, anxiety, confusion or depression)
  • Convulsions

Other common AIP symptoms

Symptoms can also occur in many different areas of your body during an AIP attack. These AIP symptoms are also common in other conditions. Having one or many of these symptoms does not mean that you have AIP. But it's important to have your symptoms checked by your doctor right away.

HEART OR BLOOD VESSEL SYMPTOMS (CARDIOVASCULAR SYMPTOMS)

  • Fast heart rate
  • High blood pressure
  • GASTROINTESTINAL (GI)
  • URINARY SYSTEM
  • BRAIN OR NERVOUS SYSTEM
  • HEART OR BLOOD VESSELS

What triggers AIP attacks?

  1. Endogenous (internal) hormones, particularly estrogen and progesterone. These hormones fluctuate the most during the 2 weeks before a woman’s menstrual period starts.
  2. Behaviors such as drinking alcohol, smoking cigarettes, using marijuana or certain illicit drugs.
  3. Stress on the body caused by infections, surgery, or physical exhaustion.
  1. Certain prescription drugs. Attacks can also be triggered by starting a new prescription drug.
  2. Changes in eating patterns such as fasting or crash dieting.
  3. Mental stress or emotional exhaustion.

If you have attacks of severe abdominal pain or other symptoms that seem to be triggered by any of the factors above, talk to your doctor right away.

If you have attacks of severe abdominal pain or other symptoms that seem to be triggered by any of the factors above, talk to your doctor right away.

Early diagnosis and treatment of AIP are critical

AIP attacks can be very serious. And symptoms may get worse over time. Untreated attacks can cause serious damage to your nervous system —including paralysis, and even death. That's why early diagnosis and treatment of AIP is so important.

AIP is rare and symptoms are nonspecific. Unfortunately, it's common for people with AIP to be misdiagnosed and to have symptoms for more than 10 years before getting an accurate diagnosis of AIP.

If you have any of the symptoms listed above, download and complete a symptoms checklist download and complete a symptoms checklist and take it to your doctor right away.


Find a doctor

The American Porphyria Foundation (APF) keeps a list of porphyria experts. You can contact the APF to find an expert in your area.

Contact APF
About PANHEMATIN Learn how PANHEMATIN treats AIP LIVING WITH AIP Create a plan to help manage your AIP attacks

Indications And Usage

PANHEMATIN® is a hemin for injection prescription medication used to relieve repeated attacks of acute intermittent porphyria related to the menstrual cycle in affected women, after initial carbohydrate therapy is known or suspected to be inadequate.

Limitations of Use

  • Before giving PANHEMATIN, consider an appropriate trial of carbohydrates [i.e., 400 grams of glucose (a carbohydrate or sugar) per day for 1 to 2 days].
  • Attacks of porphyria may progress to a point where irreversible nerve damage has occurred. PANHEMATIN therapy is intended to prevent an attack from reaching the critical stage of nerve breakdown. PANHEMATIN is not effective in repairing nerve damage that has already occurred.

Indications and Important
Safety Information

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Indications And Usage

PANHEMATIN is a hemin for injection prescription medication used to relieve repeated attacks of acute intermittent porphyria related to the menstrual cycle in affected women, after initial carbohydrate therapy is known or suspected to be inadequate.

Important Safety Information

  • PANHEMATIN is not for patients known to be allergic to this drug.
  • Vein inflammation is possible. Use a large arm vein or a central line to administer PANHEMATIN to minimize the possibility of vein inflammation.
  • Elevated iron levels may occur. Your doctor must monitor your iron levels if you receive multiple courses of PANHEMATIN.
  • PANHEMATIN has a passing and mild blood thinning effect. Avoid blood thinners during PANHEMATIN therapy.
  • Reversible kidney shutdown has occurred when too high a dose of PANHEMATIN was given in a single dose.
  • PANHEMATIN may carry a risk of transmitting agents that can cause infections, such as viruses, and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
  • Most common side effects are headache, fever, infusion site reactions, and vein inflammation.
  • To report SUSPECTED SIDE EFFECTS, contact Recordati Rare Diseases Inc. at 1-888-575-8344, or FDA at 1800-FDA-1088 or www.fda.gov/medwatch.
  • When taking PANHEMATIN, do not take drugs such as estrogens (e.g., oral contraceptives), barbiturates (drugs that help with sleep and used to treat epilepsy) or steroids (body hormone-like drugs), because such drugs can trigger an attack or make an attack worse.
For more information, please see PANHEMATIN Full Prescribing Information at www.recordatirarediseases.com.

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