Porphyria Educational Services

PORPHYRIA EDUCATIONAL SERVICES Bulletin Vol. 1 No. 12 March 1999
Intervention Therapies for Acute Hepatic Porphyria Attacks

For most porphyrics undergoing an acute attack they consume additional carbohydrates. When nausea and vomiting begin and self carbo loading no longer is ideal, a porphyric must turn to a better means of Intervention Therapy. Most ofdten this requires a short hospital stay and the administration of IV glucose. Some patients may also receive intravenous administration of glucose at home through use of ports and self administration or home health care services.

When glucose fails to bring an acute attack under control within 24 to 36 hours, docotrs will often turn to the use of hematin. Hematin is manufactured by Abbott Laboratories in the U.S. .owever, hematin, is used for the most part only as a last resort by knowledgeable clinicians.

Why?
Hematin can cause clotting problems , especially if it is mixed and/or given incorrectly.

This is a quote from Dr. Karl E. Anderson of the University of Texas Medical School at the Galveston, and a leading porphyria researcher and clinician.

"Hematin has a short shelf life and is quite unstable in solution at near neutral pH. Therefore, degredation products begin to form immediately after addition of sterile water. These degradation products are associated with a transient anticoagulant effect and often a plebitis at the site of intravenous infusion. Coagulation abnormalities include prolongation of the prothrombin time, partial thromboplastin time, reptilase time and thrombin time and impaired platelet aggregation. Heme degradation products induce these effects by binding to clotting proteins, platelets and endothelial cells. These adverse effects of hematin occur in normal subjects as well as in patients with porphyria."

That is why the hematin is only good for an hour after it is mixed and it cannot be combined with anything else.

Heme arginate is a much more refined and stable substance.

Unfortinately it is still unavailable for use in the United States as it has not had FDA approval. It may be obtained however in some instances on a per named basis through Dr. Anderson and the clinical trials. Subjects must be at least 18 years of age in order to participate in the study.