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Porphyria Educational Services
Monthly Newsletter
November 2004

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.

Pharmaceuticals and Photosensitivity

Photosensitivity can occur in several of the porphyrias.
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.
While exposure to dirct sunlight or to UV lighting are the main causes of photosensitivity, an increasing number of pharmaceuticals can cause photosensitivity when these drugs are ingested.
Special labels are often attached to the medicine containers of those drugs that have photosensitive properties within them.
These labels are put on drugs that may cause an unusual response to the sun and other sources of ultraviolet (UV) light.
Because photosensitivity is so hard to predict, the best approach is to prevent reactions by always protecting your skin, and whenever possible avoid such pharmaceuticals that produce photosensitivity. Such drugs may also cause hyperpigmentation or/ and dermatitis.
A partial list of pharmaceuticals known to cause photosensitivity includes:
;Diabinese;Diphenhydramine;Doxycycline; Doxepin;Elavil;Fluoroplex;Fluorouracil;
Fulvicin;Furosemide; Griseofulvin; Haldol;Haloperidol; lmipramine;
Lasix;Mexate;Methotrexate; Naprosen;Retin-A;Sulfa drugs;
Terramycin;Tetracyclines;Tretinoin; Veiban; Vinblastine.

Jeff Sanderson RPh

Pain: Companion of Porphyria and PN

Unfortunately but true, pain is often the constant companion of porphyria and PN. The majority of patients with chronic porphyria or the peripheral neuropathy that is associated with porphyria live in daily pain.
Far and few between are the days when chronic porphyria patients can take note that they are actually pain free for a period ot real time.
The pain is often persistent. Such pain is discomforting.
Often porphyria pain is difficult to ease. Also it has been noted that changes in barometric pressure greatly affect PN pain and the deep bone pain of porphyria.
Chronic pain exacts a high toll on the individual patient. In addition it also places a toll on the patient's family, friends and on the health care system. It also plays a role in the patient's workplace and their productivity.
Chronic pain associated with porphyria is very limiting.
Such pain limits functioning in everyday activities, which leads to loss of conditioning and other changes. Because of the pain and it's limiting factors, many porphyria patients with constant pain face depression.
As pain continues endlessly it produces discouragement and frustration. Such discouragement or frustration will aggravate the pain. This is a vicious cycle.
The changes in one's life caused by pain, when combined with sleep problems, medication side effects, mounting medical expenses and strain on important relationships, can have a profound effect on a person's mental and physical well being.
To the observer, it is hard to realize the seveerity of pain in daily life caused by porphyria and PN.
The abdominal pain associated with acute attacks often subsides after the administration of glucose infusion. The neuropathic pain can be somewhat reduced by the use of Neurontin which is used to curtail seizure activity in the porphyria patient.
For more severe chronic pain, the use of narcotic analgesics may give some relief.

Elizabeth Harrell MSN, RN
Pain Management

Alopecia is Painless and Painful

Hair loss is another of the many side effects of porphyria. Ironically while some porphyria patients are dealing with hirsutism (too much hair) alopecia (hair loss) is experienced by others.
The common form of hair loss is Alopecia areata. This form of hair loss is thought to be an auto-immune disease of the hair.
This type of hair loss appears as a rounded bare patch.
Hair loss in porphyria affects men as well as the women on an almost equal basis. Rarely will the hair loss remain permanent.
In addition to the alopecia areata all ready decribed, there are two other forms, both which are far more severe and usually less often present in association with porphyira.
These forms include alopecia totalis where the patient loses all of their scalp hair. The other form is that of Alopecia Universalis whereby there is the loss of scalp and all body hair.
Medical studies relate that hair loss is an auto-immune disease. The connection to porphyria per say is unclear.
WIth such hair loss, the bodyís immune system acts as if the hair follicles are foreign and attacks them. White blood cells called T-lymphocytes attack the hair follicle which causes the hair to stop growing and enter into the telogen (resting) phase, then in about 10 to 12 weeks when the resting phase is over the hair will then fall out. When the T-lymphocytes stop attacking the hair follicle new hair will then grow.
Many of the treatments for hair loss that are used in normally healty populations are contraindicated for use in porphyria.
Topical Minoxidil, Rogaine, Retin A - Tretinoin, and Cortocosteroids.
Such treatments, such as Retin A-Trinoin, increase the skins sensitivity to sunlight, thus off-limits to all forms of cutaneous porphyria.
Oral zinc is known to be benefitial. The down-side is that very high doses are needed for it to be effective and this may result in side effects which can include vomiting and diarrhoea.
The best treatment for alopecia in porphyria patients is to wear a hat. Women can don designer scarves for the short period ot time until hair regrows. Sometimes a change in hair style for women will also cover the patches of hair loss.
Among the many symptoms of poprhyria, alopecia has the benefit of being physically painless however it can be emotionally painful.

Robert Johnson M.D.
Internal Medicine
Retired clinician

Psychosis Basis of Behavorial Change

Behavorial change is often seen during times of acute crises in porphyria.
Porphyria patients during this time are often unsable to communicate effectively.
During acute attacks there is often an extreme impairment of a person's ability to think clearly, causing lots of confusion.
Porphyria patients often find themselves unable to behave appropriately.
Psychotic symptoms can be seen in association with a number of serious mental illnesses, with some forms of drug abuse, which explains why porphyria patients have sometimes been subjected to disbelief and refusal of treatment. Just a few years back it was not all that uncommon for porphyria patients to be directly admitted to psychiatric care units, or to drug treatment centers for observation.
Psychotic symptoms interfere with a personís normal functioning and can be quite debilitating. If a porphyria patient does not receive timely treatment for their acute attacks psychotic symptoms may also include delusions and hallucinations.
Especially during the initial onset of an attack porphyria patients are usually unable to respond emotionally and all too frequently unable to understand reality. Fortunately after commencement of iv carbohydrate infusion and correction of electrolyte balance such mental presentations return to normal.
While such symptoms are psychiatric in presentation, in most porphyria patients who receive treatment for the porphyria, the symptoms correct as chemical imbalances are corrected. It must be made clear that porphyria is not considered a psychiatric condition.

Dr. Kenneth Carlson

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.

URITOL is a brand name for the generic drug FUROSEMIDE. This antihypertensive drug carries a warning for persons who can not tolerate sulfa, and also for persons with liver disease. Sulfa containing drugs are contraindicated for porphyria patients.

DOLMAR is the brand name for the generic drugBUTALBITAL. This drug is classified as a sedative-hypnotic agent and anticonvulsant.The drug carries a WARNING if you have liver disease or have porphyria.

REGLAN is a brand name for the drug METOCLOPRAMIDE. Parkinsonian-like symptoms have occurred. Tardive dyskinesia can occur.Tardive dyskinesia, a syndrome consisting of potentially irreversible, involuntary, dyskinetic movements. Mental depression has occurred in patients with and without prior history of depression. Metoclopramide is listed on several UNSAFE drug lists for use by porphyria patients. It carries a warning for persons with liver disease.

ALLEGRA is the brand name for the generic drug FEXOFENADINE It is an anti-histamine. It is used to reduce allergic symptoms caused by hay fever, chronic idiopathic urticarias [hives] and relief of 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.