Treating AIP Begins with Identifying Potential Triggers
Through patient education and identification of precipitating factors, future attacks may be avoided. However, attacks may occur even in the apparent absence of exogenous precipitating factors.3
- Potential porphyrinogenic agents, illicit drugs including cocaine and amphetamines, and alcohol have been associated with more frequent attacks.1
- Cigarette smoking is associated with more frequent attacks.1
- Crash diets, fasting, or other severe caloric or carbohydrate restriction have all been linked with attacks.1
- Many prescription drugs have been associated with an increase and exacerbation of AIP attacks.1 Drug lists and information on their use in these patients are available on various websites. These lists may not be inclusive and are derived from multiple publications and clinical data and may not be listed in cautionary statements on local labeling.
- Endogenous hormones are important exacerbating factors, which may partially explain why attacks are more common in women and during the luteal phase of the menstrual cycle.1
AIP Treatment Options
Acute attacks associated with AIP require treatment of signs and symptoms and disease-specific therapy to restore heme homeostasis.
Physicians experienced in the management of porphyrias in hospitals where the recommended clinical and laboratory diagnostic and monitoring techniques are available should be consulted.
Manage signs and symptoms1,8
- Many drugs that increase the demand for hepatic heme (particularly for cytochrome P450 enzymes) and induce ALA synthase may be exacerbating factors and should be avoided. For other drugs, there is insufficient information for classification as safe or unsafe.
- Recognize that some treatments commonly used for signs and symptoms of AIP may be linked to exacerbating attacks and should be avoided. Withdraw all unsafe medications and other possible precipitating factors.
- Hospitalization may be required to control acute symptoms.5
- Provide nutritional support, generally intravenously. Carbohydrate loading may provide nutritional replacement and may have some repressive effect on hepatic ALA synthase.
- Monitor for electrolyte imbalances, acute psychiatric manifestations, muscle weakness, bladder distention, and ileus.
Consider PANHEMATIN (hemin for injection)8
PANHEMATIN (hemin for injection) is indicated for the amelioration of recurrent attacks of acute intermittent porphyria temporally related to the menstrual cycle in susceptible women. Manifestations such as pain, hypertension, tachycardia, abnormal mental status, and mild to progressive neurologic signs may be controlled in selected patients with this disorder. Similar findings have been reported in other patients with acute intermittent porphyria, porphyria variegata, and hereditary coproporphyria.
PANHEMATIN should only be used by physicians experienced in the management of porphyrias in hospitals where the recommended clinical and laboratory diagnostic and monitoring techniques are available.
PANHEMATIN therapy should be considered after an appropriate period of alternate therapy (i.e., 400 g glucose/day for 1 to 2 days).
PANHEMATIN (hemin for injection) therapy for the acute porphyrias is not curative. After discontinuation of PANHEMATIN treatment, symptoms generally return although in some cases remission is prolonged. Some neurological symptoms have improved weeks to months after therapy although little or no response was noted at the time of treatment.