What is Acute Intermittent Porphyria?
What is Acute Intermittent Porphyria?
According to Dr. Thomas G. DeLoughery of Oregon Health Sciences University, Acute Intermittent Porphyria is a type of Porphyria that results when there is a problem with the enzyme porphobilinogen-deaminase. This defect causes too much porphyrin to build up in an individual's blood. This accumulation can cause an individual with the disease to experience severe attacks with symptoms that can mimic other illnesses.
What are the Symptoms of Acute Intermittent Porphyria?
It needs to be said that many individuals who carry an inherited gene for Porphyria never develop symptoms. According to the Merck Manuals Online Medical Library, those who do may experience the following:
- Severe abdominal pain
- Nausea
- Vomiting
- Diarrhea
- Constipation
- Difficulty urinating
- Rapid heart rate
- Unusually high blood pressure
- Excessive sweating
- Restlessness
- Insomnia
- Depression
- Agitation
- Fatigue
- Muscle Weakness
- Tremors
- Seizures
Who Gets Acute Intermittent Porphyria?
Anyone who inherits a gene for Acute Intermittent Porphyria can become symptomatic. Women are more likely to inherit the disorder than men and most attacks occur between the ages of 18 and 40 years. Not all individuals with the gene that causes them to have Acute Intermittent Porphyria develop symptomatic attacks. A person can have the disorder without ever realizing it because detection is difficult unless a doctor is looking specifically for Porphyria.
How is Acute Intermittent Porphyria Treated?
Treatment for Acute Intermittent Porphyria usually only occurs during an attack because attacks occur so infrequently. Treatment depends on the severity of the attack. Some patients require hospitalization and infusions of heme in order to prevent permanent nerve damage. Certain drugs can make Acute Intermittent Porphyria attacks worse so a doctor will prescribe medications for anxiety, vomiting, and pain that are safe for patients with Porphyrias.
Patients who know they have Acute Intermittent Porphyria can be proactive. They can make sure each doctor they see knows of the condition so they are not prescribed medications that can bring on an attack. A diet high in carbohydrates also seems to help prevent attacks and individuals with Acute Intermittent Porphyria should avoid fasting or low-calorie diets.
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