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Porphyria Educational Services
Monthly Newsletter
October 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.

Narcotics Use and Risk of Addiction

Chronic porphyria patients are always aware of the risk of possible addiction with the use of narcotics. At the same time these same patients know that in order to have any quality of life, chronic pain must be managed.
Often patients are not aware of the variables involved in addiction. Often various terminology is wrongly applied.
Addiction is not synonymous with physical dependence or tolerance. Each are separate medication conditions in relation to the use of narcotics.
(1) Addiction is a primary disease marked by unsatisfied desire for a drug and compulsive use of that drug irregardless of the repeated and harmful consequences.
(2) Tolerance happens when the initial dose of a narcotic is no longer effective, which means having to take higher doses of the drug to produce the desired effect.
(3) Physical dependence occurs when your body adapts to a drug. After the drug is stopped, the patient may then experience anxiety, spasms, and other physical withdrawal symptoms. People with chronic pain or illness who take narcotics may, over a period of time, develop tolerance and even physical dependence. But please note, these persons are not addicted.
A small number of porphyria patients could potentially experience addiction over several years of regular narcotic use. Such addiction however is based on many factors which include many variables ranging from genetic, psychological and environmental.
Studies of patients and drug use consistently show that —people treated with opioids never become addicted.
When porphyria patients are seen in emergency room settings or by clinician other than their own primary physician, the chronic pain causes such patients to act in ways that are mistakenly for addiction. An example is when a patient is in so much pain that they are seeking emergency help and that is their sole focus and can not think of anything else. This is not an addictive behavior pseudoaddiction, a behavior that stops immediately as the porphyria patient receive satisfactory pain relief.
All too frequently porphyria patients and their caretakers have complained that medical staff often tend to view the patient as "drug-seeking".
Regardless of debates over the use of narcotics, the use of narcotics can and should be a key part of the treatment plan for porphyria patients with chronic neuropathic pain.
Porphyria patients should always discuss with their pain management physician in regard to combining opioids with simple analgesics for maximum pain relief. Always bear in mind that many non-narcotic drugs are contraindicated for porphyria patients.
If the porphyria patient uses alternative means of pain control at night, such as an electric mattress cover or TENS unit, or heating pad, the patient and the physician should look at the beenfits of short-acting and sustained-release medications for daytime use, skipping medication at night when alternative treatment may be used.
Another factor that needs to be discussed with your physician is whether you should take opioids on a regular schedule or simply on an as-needed basis.
Keep a written written record of all narcotic use and any symptoms that you experience. The porphyria patient should compare their function and activity levels with how you were functioning before you began taking the medication.
I good deciding factor is that the patient should be able to see a marked improvement before deciding to continue using a given narcotic for a long term period.

Robin Lamberton RPH

Dealing With Agitation and Restlessness

Agitation and restlessness are often present at the initial onset of acute porphyria attacks.
There seems to be a high state of extreme arousal. Patients often state that they become more tense and irritable. Often such symptoms are associated with stresss, and in some instances it is the stress itself that triggers the acute attack.
Agitation by itself may not have much clinical significance; but, if viewed with other symptoms, it can be a good indicator of a disease state. It is almost always present at the onset of the acute attack.
Extreme agitation can lead to confusion or disorientation. Unfortunately all too often agigitation associated with porphyria can lead to outright hostility.
Agitation can come on suddenly or gradually. Porphyria patients have often commented that when acute attacks are triggered by infections such as urinary tract infections, the agitation gradually builds right along with the discomfort of the UTI itself.
When agitation is associated with a PN pain, such agitation can last for just a few minutes or for weeks and even months. External stimuli such as pain and anxiety both increase agitation.
During times of agitation it is important that the porphyria patients be able to speak out their feelings.
Reduction of stress is very important in ovecoming the agitation.
There is need for a calm environment, quiet, quality sleep, no bright lights, no sudden movement,or toxic smells. In the clinical setting it is important that a porphyria patient not be restrained since this usually makes the problem worse.
Once adequate treatment of an acute attack is underway, the symptom of agitation usually goes into remission.

Dr. Kenneth Carlson

Unwanted Hair Problems in Porphyria

In various forms of the porphyrias the massive growth of hair in unwanted places occurs. This manifestation is known as hirsutism.
The complaint of hirsutism is common among porphyria patients and often accompanied by severe anxiety and social stress.
Because of the change in physical appearance, many porphyria patients become very self concsious in addition to the stress they all ready have in dealing with lack of public and care provider understandings of their disease condition.
Therefore, hirsutism has important issues to consider.
The extent and severity of hirsutism should be well charted along with the method and frequency of physical removal of the unwanted hair.
Most patients who complain of hirsutism will have an objective excess of hair on examination. It can be in a small specific area or can be wide-spread.
Normally hirsutism usually begins around the time of the menarche and increases slowly and steadily throughout the teen years and into early adulthood. Other hirsutism growth patterns need further evaluatuion beyond that associated with the porphyrias.
Rapid progression and prepubertal or late onset suggests a more serious cause. Female porphyrics with hirsutism will have some disturbance of menstruation. The greater the disrution the more likely it is that there is a serious cause.
Clinical findings also indicate that overweight or obsese patients also have more abundance of hair growth.
In treating hirsutism, the underlying cause should be removed in the rare instances where this is possible, other words, reducing the manifestation of porphyria.
Other treatment depends upon whether the aim is to reduce hirsutism through regularizing menstrual periods in female porphyrics.
Shaving, plucking, bleaching, depilatory cream or wax and shaving may all help and are often underused.
Electrolysis is slow and expensive.
Topical ointments are also available for use.
Naire-Three-In-One has been used successfully by a number of porphyria patients without causing any cutaneous porphyria symptoms.
Eflornithine Cream is the first topical prescription treatment for women with unwanted facial hair.
Eflornithine works by inhibiting the growth of facial hair and was shown in controlled clinical trials to provide clinically meaningful and statistically significant improvement in the reduction of facial hair growth in women.
Keeping the skin clean and free from oil, as well as avoiding the sun also help is reducing hitsutism.

Robert Johnson M.D.
Internal Medicine
Retired Clinician

Tachycardia Often Present in Attacks

Tachycardia is listed nearly in every medical writing concerning acute attacks of porphyria.
Tachycardia is the medical term for a fast or rapid heart rate, galloping heatbeat, or bounding pulse.
Tachycardia is a heart rate that is faster than normal. It can occur alone, or it can accompany a bounding pulse which can be described as a very forceful pulse.
A very fast pulse can be a symptom of arrhythmias. Cardiac arrythmias are comonly associated with the acute porphyrias
A rapid heart rate and bounding pulse can occur together, but can also occur separately.
A bounding pulse is often associated with high blood pressure or fluid overload, both of which can be found during porphyric crisis.
A fast heart beat and bounding pulse both occur normally with high anxiety ehich is often present at the onset of an acute attack.
In the porphyrias the presence of tachycardia and hypertension have most often been treated with the use of propranolol which is known safe for use in acute porphyria patients. Propranolol also is known to help in the treatment of depression which sometimes can ocur in association with porphyric attacks.

Phillipe Beauchamp PA

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.

AMAPHEN is the brand name for the generic drug BUTALBITAL. This drug is classified as a sedative-hypnotic agent and anticonvulsant.The drug carries a WARNING if you have liver disease or have porphyria. DANOCRINE is a brand name for the generic drug DANAZOL. Danazol appears on several of the UNSAFE drug lists for porphyria patients. The drug carries a WARNING for persons with liver disease. MYROSEMIDE 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 XANAX is the brand name for the generic drug ALPRAZOLAN whish is a tranquilier, anti-convulsant, and a benzodiazepine. It is used for the treatment of muscle spasms, anxiety disorders, seizure risorders, alcohol withdrawal,and insomnia. The drug is photosensitive. The side effects of this drug include: Clumsiness, drowsiness, dizziness, signs of addiction, hallucinations,confusion, depression, irritablity, rash, itch, vision changes, sore throat,fever, chills, constipation, diarrhea, nausea, vomiting, difficult urination,. vivid dreams, behavior changes, abdominal pain, heache, dry mouth. The drug carries a WARNING. Do not use if your have Porphyria or liver disease.