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Porphyria Educational Services
Monthly Newsletter
November 2003

All information published in the Porphyria Educational Services Monthly Newsletter is to provide information on the various aspects of the disease porphyria and it's associated symptoms, triggers, and treatment.

Columnist and contributors and the information that they provide are not intended as a substitute for the medical advice of physicians. The diagnosis and treatment of the porphyrias are based upon the entire encounter between a physician and the individual patient.

Specific recommendations for the confirmed diagnosis and treatment of any individual must be accomplished by that individual and their personal physician, acting together cooperatively.

Porphyria Educational Services in no way shall be held responsible in part or whole for any injury, misinformation, negligence, or loss incurred by you. In reading the monthly newsletters you need to agree not to hold liable any contributing writers.

Weakness is Common Symptom of Acute Porphyrias

Weakness is a commonly experienced malady in porphyria. Porphyria patients complain endlessly of having a lack of strength or a general weakness of their muscles.

Weakness is a reduction in the strength of one or more muscles. Much of weakness may be subjective. The porphyria patient feels weak, but has no measurable loss of strength. And then again the porphyria patient may indeed have a measurable loss of strength as noted in a physical exam.

Weakness may be generalized emcompassing most of the body or such weakness can be localized to a specific area, side of the body, limb, or muscle. Weakness is more notable when it occurs in only one area of the body.

Measurable weakness may result from a variety of conditions including metabolic and neurologic diseases of the porphyrias. Weakness can also be attributed to the exposure to chemical toxins.

Sometimes porphyria patients will have weakness mimicing multiple sclerosis (MS) or that of Guillain-Barre syndrome. When such weakness is due to chemical toxins it is usually associated with exposure to insecticides.

When discussing your muscle weakness with your PCP it is best to keep a record of your weakness and it's progression. Your PCP will want to know is the weakness is confined to one area of the body, if the weakness is prolonged, if it is an unexplained weakness, if there was a sudden . onset of weakness, or if it was following another illness.

General body weakness often presents in the acute porphyrias, but other causes of weakness need to be ruled out, if such weakness has just presented.

Virginia Dobmier MNS, RN

Porphyrias Accompanying Peripheral Neuropathy

Many porphyria patients are plagued with problems associated with porphyric peripheral neuropathy.

Neuropathy, is a general term for disorders of the peripheral nervous system. In the most of the porphyrias we hear alot about the CNS (central Nervous System) and the ANS (Autonomic Nervous System).

The majority of porphyria patients sooner or later find themselves consulting with neurologists as PN begins to progress. Often it is the pain associated with the PN that will make them first seek medical assistance.

Neuropathies may affect just one nerve (mononeuropathy) or several nerves (polyneuropathy).

Symptoms vary according to what nerves are affected. Symptoms can range from a mild tingling or numbness in the fingers or toes to searing hand or foot pain. You may lose some of the feeling in your feet or hands. Many porphyria patients also experience weakness or even paralysis of certain muscles, or problems with your bowels or bladder.

PN symptoms result from damage to the nerves that provide communication between your brain and your muscles, skin, internal organs and blood vessels. Peripheral neuropathy (PN)often affects people with diabetes and autoimmune diseases such as rheumatoid arthritis and lupus as well as those with porphyria.

Certain vitamin deficiencies, some medications and alcoholism can also damage peripheral nerves. B5 is among the vitamin deficiences that can cause PN.

In porphyria is hard to treat the underlying condition other than through the use of Gabapentin or better known as Neurontin, the use of TENS, physical therapy and general exercise.

For many porphyria patients treatment may focus on managing pain.

Leonard Simpson PA

Wood's Lamp Test Used for Validating PCT

Wood's Lamp testing has been used for years in the testing of possible porphyria patients thought to have Porphyria cutanea tarda (PCT). Some medical professionals will refer to such testing as a black light test; or an ultraviolet light test.

Like a lot of the other porphyria tests, there are many pitfalls which can render such a test as invalid. For example, a room that is not dark enough may alter results.

It is important not to bathe or shampoo for 24-hours before your test, because porphyrins (a substance found in the suspect organisms) are removed with soap and water. The reason for not washing immediately before the test may cause a false negative result.

Deordorants, and some forms of cosmetic makeup may also alter the results.

There are no risks involved with the Wood's Lamp test however a [atient should avoid looking directly into the ultraviolet light, as you avoid looking into the sun.

While there really is much to a Wood's Lamp test, the test involves sitting while your health care provider shines the ultraviolet light over the skin being studied. You will be seated, and the lights will be turned off. After your eyes adjust to the darkness you will be reminded that you are not to look at the Wood's Lamp light directly.

The Wood's lamp will then be turned on and held a short distance from the area being evaluated. . When the lamp is on it will detect the presence of infectious organisms which contain substances that glow on the skin in the presence of ultraviolet light.

In a normally healthy person your skin will not fluoresce, or shine under the ultraviolet light.

Under the ultraviolet light, different infections and conditions show different colors: If a person has PCT the colors that will present are pink to pink orange.

Renae DiSalvo MSN, RN

Many Drugs Carry Photosensitive Warning Labels

When you are prescribed a new drug, be sure to double check the label as well as the drug information pamhlet or print out that accompanies the product. DO this BEFORE you begin your taking your prescribed dosages. If you are a porphyria patient with cutaenous symptoms, photosensitivity is all ready a problem and you will not want to add to that problem by taking a drug that will enhance photosensitivity.

Special labels are often attached to the medicine containers of those drugs that have photosensitive properties within them. My pharmacy uses a different color little stickers that goes on the side of the basic pharmaceutical information. These labels are put on drugs that may cause an unusual response to the sun and other sources of ultraviolet (UV) light.

This is called "photosensitivity,. There are several reactions that can occur in dealing with photosensitivity.. The most common reaction is an intense or exaggerated sunburn. However rashes, blistering, swelling and other skin problems can also occur. Skin can become very fragile.

It is important to note here that not everyone who takes these drugs has a reaction. Many people have no problems, and others react only mildly. It is known that photosensitivity is hard to predict. Please remember that just because someone else hasn't had a reaction doesn't mean it won't happen with you. And this includes from one time to the next time.

It is known that the same person can also respond in different ways at different times. Many factors enter into this. The amount of medication you take, other drugs or products you're using, and the intensity of UV light -- determine whether reaction will occur. It also makes a difference if you have Rosacea or if you have PCT, VP or HCP. Here you must be warned-don't be fooled if you haven't reacted while taking one of these drugs in the past.

Photosensitivity can still occur the next time around.

It must be also noted that in some cases, photosensitivity may also continue after a drug is stopped. With tetracycline for example, some people stay sensitive for several weeks. This includes the minocycline which is a common medication for Rosacea. With some patients it can sometimes up to several months) after they've finished the medication.

Because photosensitivity is so hard to predict, the best approach is to prevent reactions by always protecting your skin. In VP and HCP along with the PCT, avoiding the sun and sun reflection such as snow glare or water glare from a lake, is important. And just because you are separated from the sun by a pane of window glass does not mean you are protected.

Photosensitivity may happen quickly (sometimes in as little as 10 minutes), so even the time it takes to walk to the store, wash the car, or mow the lawn or shovel the snow out of the driveway on a bright winter's day, it can be enough to cause a reaction in some people.

Diana Deats-O'Reilly
CEO Porphyria Educational Services

PES Monthly Drug Update

PES drug information does not endorse drugs, diagnose patients or recommend therapy. PES drug information is a reference resource designed as a supplement to, and not a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners in patient care. The absence of a warning for a given drug or drug combination in no way should be construed to indicate that the drug or drug combination is safe, effective or appropriate for any given patient.

ALLEGRA is the brand name for the generic drug FEXOFENADINE anti-histamine. It is used to reduce allergic symptoms caused by hay fever, chronic idiopathic urticarias [hives] and relief asthma symptoms. The drug is photosensive. This drug carries a WARNING that it is not recommended for persons with any type of heart disorder, glaucoma, prostate or urinary retention, asthma, kidney disease, peptic ulcer, electrolyte imbalance [low potassium,] or liver disease.

ZAPEX is the brand name for the generic drug OXAZEPAM. This drug is classified as a tranquilizer, anti-convulsant and is a benzodiazepine. It is sued for the treament of muscle spasms, anxiety disorders, seizure disorders, alcohol withdrawal and insomnia. Zapex is has photosensitivity. This drug carries a WARNING: ***Do not use if your have Porphyria or liver disease.

NEMBUTAL is the brand name for the generic drug PENTOBARBITAL which is a barbituarate, sedative-hypnotic, and an anti-convulsant. It carries a WARNING: ****Do not use if you have Porphyria.

VASOTEC is the brand name for the generic drug ENALAPRIL. Angiotensin-Converting Enzyme [ACE] Inhibitors This drug is used for the treatment of high blood pressure, kidney disease in diabetic patients. and treatment of acute myocardial infarction within 24 hours of occurence. Some of the side effects of palpitation, Ileus, pancreatitis, hepatitis hepatocellular cholestatic jaundice, constipation, muscle cramps, depression, confusion, ataxia, somnolence, insomnia, nervousness, peripheral neuropathy, paresthesia, dysesthesia,. alopecia, flushing, photosensitivity, blurred vision, photosensitivity, rash and other dermatologic manifestations and mortality. WARNING - Not recommended for those with autoimmune disease, for those with liver disease or lupus. Please note: Hepatic failure has occurred.