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#PAW2018 Medical Moments on EPP & XLP


Important Considerations

Consult a  specialist.  Because EPP is a rare condition, most physicians are not knowledgeable about it.  Primary care or Emergency Room doctors can contact EPP expert: Dr. Micheline Mathews-Roth, Harvard Medical School, 617-525-8249. The American Porphyria Foundation, 713-266-9617 can also provide further information. 
Alert the anesthesiologist to use an anesthetic that doesn’t block bile flow.
Be careful with medication. Avoid damage to the liver.  Avoid griseofulvin, estrogen or any drugs that block bile flow.
Be careful with surgical lights. Strong operating room lights can cause photosensitivity in internal organs. Cover lights with clear window film, such as CL5-200-X from Madico Co. Keep skin covered with surgical drapes and keep exposed internal organs as covered as possible.   It is extremely important to shield the surgical lights during abdominal surgery.  The approach for superficial surgeries is to shield the skin as much as possible, and to work quickly.  In an emergency situation, where better shielding materials are not available, use as much cloth as possible to shield the site. 
Be careful with lights.  Problems with fluorescent lights are common. Incandescent bulbs are generally acceptable.  However, companies may be changing the filaments and thus varying output.
The risks from surgical lights, anesthetics and drugs are great enough that all EPP patients should wear Medic Alert bracelets, even those who have mild enough cases that they can lead quite normal lives with just a few adjustments.


Vitamins:  Taking Vitamins A and D in excess can cause major health problems. Taking beta carotene, even in large doses as for EPP, does not lead to Vitamin A toxicity, because the body stops splitting beta-carotene into Vitamin A when it does not need the Vitamin A any more.  Vitamins E and K can also be toxic at several times the recommended dose.  
Celebrex:  It has been reported that Celebrex, a non-steroidal anti-inflammatory drug (NSAID), helped reduce photosensitivity.  This has not been proven.  NSAIDs are powerful drugs and can cause very severe complications, such as stomach and esophageal ulcers and hemorrhages, and some liver problems.  Thus, if you need to take NSAIDs for arthritis and other conditions and you find that they happen to decrease your photosensitivity, it is important to ask your doctor to monitor your liver chemistries and porphyrin levels every 4-6 months.

Laser Treatments 

Laser treatments for hair removal or eye surgery have been a subject for question.  According to Dr. Roth, laser treatments have not been a problem for EPP people, as long as they check with their doctor as to the output of the laser.  It should not be between 400 and 650 nanometers. In addition, if the person is having hair removal treatment, the doctor should irradiate a small area of the skin to be treated for the length of time it will take to do the hair removal to ascertain if the patient will get any EPP symptoms from the laser radiation. It is important to wait for as long as it usually takes your symptoms to develop following sun exposure.

Dietary Considerations 

There have been a number of reports to suggest that dietary fish oils seem to lessen the symptoms of EPP. At this point, none of these claims can be validated until careful studies under medical supervision are conducted and FDA approval is obtained. This would be a treatment, not a cure.  The oils may work by acting as anti-oxidants.  One major brand of fish oils is Maxepa, distributed by Twin Labs. EPP people sometimes have low hemoglobin. Therefore, it is suggested that you have enough iron in your diet to meet the recommended dietary allowance (RDA) for this nutrient to avoid becoming anemic. The RDA is 10 mg/day for men, 15 mg/day for women 25-50 years, and 10 mg/day for women over 50. For pregnant women, the suggested allowance is 30 mg/day.  It is important to check food labels for iron content.

Women and Osteoporosis 

There are several methods of treating osteoporosis, including increasing calcium intake, exercise, hormone (estrogen/progesterone) replacement therapy in postmenopausal women, testosterone therapy for certain men, and calcitonin. There are some experimental drugs being studied at this time, but they have not yet been approved by the FDA.
For women with EPP, hormone replacement therapy (HRT) can be used if you feel that the benefits outweigh the risks, according to our liver consultant Dr. Joseph Bloomer. The risk is that these hormones may impair protoporphyrin excretion from the liver and thus can lead to liver problems (the reason that EPP people are discouraged from taking birth-control pills).  If you do decide to take HRT, it is crucial to follow closely your protoporphyrin levels and your liver chemistry blood tests (at least twice a year). If there is any increase in either liver chemistry or protoporphyrin levels, HRT should be stopped immediately. Each case must be judged on an individual basis, so do discuss this with your personal physician. For men, osteoporosis is sometimes treated by giving testosterone—this hormone also affects the liver in the same way that estrogen does, thus the same cautions apply.

Youngsters with EPP

Camp Discovery
Camp Discovery offers a wonderful summer camping experience for young people with skin disorders like Porphyria.
Every year, the American Academy of Dermatology sponsors a week of fishing, boating, swimming, water skiing, arts and crafts, and just plain fun.
Under the expert care of dermatologists and nurses, Camp Discovery offers the opportunity to spend a week with other young people who have similar skin conditions. Many of the counselors have serious skin conditions as well, and can provide support and advice to campers.
There is no fee for camp. Full scholarships, including transportation, are provided by the American Academy of Dermatology through generous donations of their members and other organizations. Members of the Academy are asked to recommend candidates for Camp Discovery, so ask your child's doctor about sending your child to Camp Discovery.
Disneyland and Disney World for EPP Kids
Disneyland and Disney World are responsive to people with sun sensitivity. They will provide a pass to enable you to enter attractions without waiting in line in the sun.
Go to "City Hall" and explain your problem with photosensitivity. You should bring a physician's letter with you as well as an APF brochure explaining the type of Porphyria you have.
Disney World
Proceed to the "Guest Relations" office at any park (Magic Kingdom, EPCOT, etc.) and request the Special Assistance Pass.
Remember to bring a doctor's note and explanation of your condition, because it is not necessarily visible. People on duty may not be familiar with light sensitivity and its consequences.


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 The following questions were submitted to Dr. Wang for his responses ... Q. Does EPP give us bad teeth? Also, do people with EPP get stomach pains or is that with the other porphyias? A. The porphyrin that accumulates in EPP patients is protoporphyrin IX, which does not cause discoloration to teeth or abdominal pain.
 The type of porphyria that leads to discolored teeth is Congenital Erythropoietic Protoporphyria. The porphyrias that lead to episodic abdominal pain attacks are the acute hepatic porphyrias. Q. I have EPP and I have a severe reaction on my hands and lips. Do I seek urgent care? Also, what can you even do when you burn your lips? A. The acute reactions to sunlight in EPP can be very severe and, unfortunately, there are not many effective options to treat the symptoms. Nonsteroidal anti-inflammatory drugs (NSAIDS such as ibup…