It is probably a good idea for people with EPP to wear (or carry) a MedicAlert bracelet, mainly because of the danger of operating room lights (they can cause severe photosensitivity of internal organs as well as skin), and because of the importance of avoiding medications that slow down bile flow in the liver (cholestasis). You should contact MedicAlert to get an application form, and fill it out as we suggest below. Their toll-free phone number is 1-800-432-5378. We have also printed here a page of information we have prepared for them on EPP. We suggest that you apply by mail, so that you can enclose the form and the material we have prepared. Their nurse-consultant, Andrea Terry, R.N., is working with EPPREF to make sure MedicAlert has accurate information on EPP.
We suggest that you put the following information on the application:
Question 3, Emblem engraving: As stated on the application, the emblem has a limited number of spaces on it. If you have any medical conditions or allergies in addition to EPP, list them (for example, diabetes, penicillin allergy, asthma, etc.). For EPP, write: Protoporphyria: extremely light-sensitive. If you have trouble getting everything you think should be listed in the limited number of spaces, call Andrea Terry, R.N. at MedicAlert, 1-800-432-5378, extension 2408.
Question 4, Information for medical file: If you have other conditions, list medications you are on, etc. For EPP, write: "erythropoietic protoporphyria - see attached sheet". Staple the following page, "Information on Erythropoietic Protoporphyria" to your application.
The other questions on the application are straightforward, and do not need specialized information about EPP.
Send your completed application and membership fee to
- MedicAlert
- Attention: Andrea Terry, R.N.
- 2323 Colorado Avenue
- Turlock, CA 95382
If you have any questions, do not hesitate to contact EPPREF at (617)-525-8249.
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(Print out the following document to MedicAlert with your application)
INFORMATION ON ERYTHROPOIETIC PROTOPORPHYRIA FOR MEDICALERT
Erythropoietic protoporphyria (EPP) is a rare genetic disease. EPP patients have a defect in ferrochelatase, the enzyme which inserts iron into protoporphyrin to form heme. As a result of protoporphyrin build-up in erythrocytes and its leakage into serum, EPP patients are sensitive to most of the visible light spectrum (400-600 nm). Exposure to this light, from the sun when outdoors, through window glass when indoors and in an automobile, and in some patients, from indoor fluorescent lights, makes their skin itch, burn and ulcerate. EPP is a bone-marrow-based porphyria, for which at present there is no cure. Taking beta-carotene in doses up to 300 mg/day by mouth is the FDA-approved treatment for EPP: in this case, beta-carotene is not acting as a vitamin, but as a quencher of light-induced excited species. Clinical experience has shown that preparations of crystalline carotene in oil do not give high enough blood levels of beta-carotene: the best blood levels are achieved by taking Hoffmann-LaRoche "10% dry beta- carotene beadlets". At present, the only beta-carotene preparation containing these exclusively is LUMITENE (Tishcon; 1-800-848-8442).
It is also essential that EPP patients see their physicians annually for a physical examination, blood count and liver function tests, as in rare instances, anemia or severe liver disease can develop. These conditions can usually be treated if discovered at an early stage.
Emergency personnel need to be especially concerned about the potential for EPP patients to develop potentially fatal liver disease and to develop serious photosensitivity.
1) DO NOT administer any anesthetic agents or drugs which induce cholestasis.
2) Shield skin and internal organs as much as possible from operating room lights, other strong lights, and light coming through the windows of ambulances, and patient rooms. This latter precaution arises from reports that during liver transplant operations, serious burns have been caused to viscera exposed to OR lights. (See for example, Meerman et al., Transplantation, Vol. 57, pp. 155-158, 1994). Also - some patients report sensitivity to fluorescent lights, so exposure to these lights should be lessened if necessary.
In addition to beta-carotene ingestion, EPP patients still must protect themselves from light. Light protection is best achieved by wearing long-sleeved shirts and long pants or skirts. The patient may also have to adjust the lighting in the workplace, such as by exchanging fluorescent tubes for incandescent ones, cutting down direct light, etc. It is also advised that patients put light-filtering material (for example, "Scotchtint" films by 3M) on home and car windows, if they are bothered by the light coming through the window glass. This light-filtering film is usually obtainable as a tinted film but on special order can be obtained as a clear film. Use of this film has enabled EPP patients to travel in cars without suffering great discomfort. Use on the side windows is sufficient to provide relief for most patients, but for those bothered by the light coming through the windshield, use of a strip of the clear film along the top of the windshield is usually sufficient to provide relief. Since the film is not tinted, it does not reduce visibility through the windshield. However, in spite of taking the precautions given here, in some cases, the patient's light sensitivity is so severe that light exposure outside the home cannot be sufficiently controlled, forcing the patient to be essentially confined to his/her home, leaving it only at night.
If you have any questions about EPP, you can contact Micheline Mathews-Roth, M.D., Brigham & Women's Hospital, Boston, MA (617-525-8249) or the American Porphyria Foundation (713-266-9617).
Suggested Bracelet Imprint for EPP: Protoporphyria: extremely light-sensitive.
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