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Porphyria Educational Services
June 2003 Newsletter

Disclaimer
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.



Pesticides Exacerbate Dermotosis in Porphyria Patients

When people speak of reactions to pesticides and fungicides and other chemical toxins many will envision a sudden attack, inability to breathe and sometimes respiratory paralysis or even death.

A much more subtle reaction to pesticides and such is that of dermotosis, especially in porphyria patients. Farmers have long noted skin reactions to sprays and chemical toxins used in farming operations. Most farmers are normally healthy persons. In most porphyria patients the same exposure can become a serious matter.

Today environmental medicine is taking a close look at the safety of such chemical toxins and identifying culprits that can cause grave reactions in porphyria patients and other persons with other sensitivity conditions.

Pesticides are chemical substances that are used regularly in agriculture. It is vital to farmers to protect crops against pests., and enhance crop yields.But the other side-of-the-coin is that these same pesticides are capable of causing diseases.

Skin is exposed when spraying of pesticides are underway. Some persons are also exposed to pesticides while mixing, loading the pesticide, or while cleaning the equipment . Just disposing of empty containers can be enough exposure for some individual.

For many porphyria patients just aking a walk through a field or a park that has been sprayed allows for exposure to the pesticides. The same thing occurs when people weed their gardens after the garden has been sprayed with pesticides or fungicides.

Years ago pesticides use saw the risk of acute intoxication among people exposed. Then the chemical manufacturers decreased in the toxicity in pesticides.

Now today with the new improved pesticides, the focus has turned to chronic intoxication and environmental contamination. More and more interest is being shown in the field of Environmental Medicine.

The major problem for porphyria patients and many others lies with dermotoses.

. The majority of pesticide-related dermatoses are contact dermatitis.This includes both allergic or irritant. It also includes porphyria cutanea tarda and skin hypopigmentation, as well as alopecia.

David Lawrence PhD
Dermatological Science



Porphyria Patients Should Use Drug Guide Books

One can not be too careful when contemplating the use of a medicine whether it be prescription or OTC. (over-the-counter) drug.

A handy guide to use is a handbook which is a complete guide to the prescription and nonprescription drugs which has sections on adverse reactions, sun reaction, and other warnings. Such a book will indicate sun sensitiveness.

Another feature of such a guide is that it will have a listing of common types of drugs and how the use of this specific drug will influence the other drugs. It will indicate a decrease or increase in effectiveness [strength of medicine].

The medicine one takes can make 'a world of difference'. Many drugs can trigger porphyria. Some drugs can are contraindicated when used with other drugs. Some drugs have components that cause allergic reactions in some people.

It is important to know your drugs before you take them. They are many excellent drug reference handbooks available which are updated regularly. Take a browse in book store and look at the information and how it is presented. Some very inexpensive paperbacks can be found which specific list the warnings for porphyria patients.

Gary Larson RPH




When Porphyria Is Actually Pseudoporphyria

Many would be poprhyria patients have been wrongly diagnosed with porphyria because of outward appearances or one-time porphyria testing.

Unlike confirmed cases of porphyria, there are no laboratory abnormalities in pseudoporphyria.

Pseudoporphyria is better known as a drug-induced porphyria. Often pseudoporphyria will produce a bullous dermatosis which is also known as drug-induced porphyria or therapy induced bullous photosensitivity.

There are many pharmaceuticals which have been implicated in producing pseudoporphyria. Many of these same pharmaceuticals have also been associated with chronic renal failure patients undergoing hemodialysis.

A common drug known to almost everyone is an OTC pharmaceutical, acetaminophen. Also known for triggering porphyria, is that of barbiturates as well as the sulfonamides.

Much lesser known is that of bumetanide, a commonly prescribed diueretic.

Oral contraceptives are known to trigger PCT, as well as estrogen itself.

Pyridoxinem better known as Vitamin B6, is also known to cause porphyria like symptoms.

In the basic medical textbook, "Harrison's Principles of Internal Medicine" other pharmaceuticals that have been associated with triggering pseudoporphyria include: Carbamazepine, Chlorthalidone, Chlordiazepoxide, Chlorpropamide, Cyclosporine, Damazol, Dapsone, Diflunisal, Ergots, Etretinate, Fluoroquinolones, Furosemide, Glucocorticoids, Glutethimide, Griseofulvin, Hydrochlorthiazide, Insulin, Katoprofen, Meprobamate, Methyprylon, Nalidixic acid, Naprosyn, Naproxen, Narcotic analgesics, Nifedipine, Penicillin and derivatives Phenothiazines, Phenytoin, Photofrin, Progestogens, Pyrazolones, Rifampin, Streptomycin Succinimides, Tetracycline and Valproic acid.

In the cutaneous aspects of pseudoporphyria, the clinical lesions resemble porphyria cutanea tarda with spontaneous blisters and skin fragility, usually on the dorsum of the hands. Lesions may develop from 1 week to several months after the start of the drugs.

Unlike true PCT, in cases of pseudoporphyria, there usually is no hypertrichosis, hyperpigmentation, or sclerodermoid changes. The microscopic changes are similar to that seen in porphyria cutanea tarda.

Dr. William Morris
Department of Dermatology



The Role of Potassium in Acute Porphyria

Potassium plays many roles in the healthy well being of a person who is considered "normally healthy". So potassium in is even more vital in the well-being of an acute porphyria patient.

So just what is this thing called potassium? Potassium is a mineral that helps the kidneys function normally. Potassium is vital to good smooth muscle contraction.

Potassium also is a vital nutrient for normal heart, digestive, and muscular function. It has been found that potassium helps lower blood pressure, and that it can help reduce the risk of death from an acute heart attack when administered by a health care provider along with insulin and glucose.

Some terminology one must remember in regard to the use or lack of use of potassium is that If you take ingest too much potassium in your diet, you run the risk of getting "hyperkalemia". On the otherhand, and most noticeable in acute porphyrias is too little potassium intake and not enough potassium in the blood. This is known as "hypokalemia".

With the hypokalemia in the acute porphyrias, often patients begin to experience CNS disturbances. Such disturbances can range from seizure activity to mental confusion., and also include weakness, lack of energy, stomach disturbances, an irregular heartbeat, and an abnormal EKG (electrocardiogram).

Many porphyria patients are well-advised to monitor their blood serum potassium on a regular basis. Their PCP can prescribe potassium to be used to lower blood pressure, prevent stroke, treat muscle weakness, and help prevent death from an acute heart attack, as well as prevent the CNS symptoms of acute porphyria during attacks. If you have kidney problems along with the porphyria, you should not take potassium supplements unless told to do so by your PCP. AS you head into the "golden years" one should take potassium supplements as directed by their PCP due to having decreased kidney function with age.

When using potassium supplementation it is good to remember that nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen may raise levels of potassium in the blood. ACE inhibitors (such as enalapril, captopril,and lisinopril) can also increase blood levels of potassium.

For those with kidney abnormalities a rise in potassium from ACE inhibitors may also be more likely in cases of decreased kidney function and diabetes.

Heparin, cyclosporine, trimethoprim, and beta-blockers (propranolol) that are used to treat high blood pressure) may also raise blood levels of potassium. Both heparin and propranolol are drugs normally prescribed and used in the treatment of acute porphyrias.

Corticosteroids, amphotericin B, antacids, insulin, loop diuretics (such as furosemide and bumetanide), and thiazide diuretics (such as hydrochlorothiazide) can lower potassium levels. Remember that Bumetanide is another normally prescribed drug for acute porphyria patients in the treatment of edema as well as controlling blood pressure.

Always check with your PCP before increasing your dosages of potassium. Many PCP's include potassium supplementation in the intravenous fluids given porphyria patients during acute attacks.

Robert Johnson M.D.
Retired Clinician


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.

BACTRIM is a brand name for the generic drug combination of SULFAMETHOXAZOLE and TRIMETHOPRIM. It contains sulfa as an ingredient. The drug carries a warning against use in persons with the disease porphyria.

INTENSOL is a brand name for the generic drug ALPRAZOLAM and is a part of the drug classification of BENZODIAZEPINES. The following adverse events have been reported in association with the use of benzodiazepines: dystonia, irritability, concentration difficulties, anorexia, transient amnesia or memory impairment, loss of coordination, fatigue, seizures, sedation, slurred speech, jaundice, musculoskeletal weakness, pruritus, diplopia, dysarthria, changes in libido, menstrual irregularities, incontinence, and urinary retention This drug has a warning on withdrawal reactions including seizures and dependence. The drug is metabolized in the liver. There is a warning concerning use in patients with liver disease.

PANZAC is a brand name for the generic drug OMEPRAZOLE. In clinical trials this drug was knownto elevate liver functions. Some hepatic failure was noted. The drug is metabolized in the liver. Caution is listed for persons with liver impairment.

ULTRAM is a brand name for the generic drug TRAMADOL. It is an analgesic. Seizures have been reported in patients receiving this drug. Respiratory depression may also occur. The drug is metabolized in the liver. Besides the possibility of seizures, paresthesia, cognitive dysfunction, hallucinations, tremor, amnesia, difficulty in concentration, abnormal gait, and depression have occurred in the central nervous system. Deaths have been noted with this drug.