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Porphyria Educational Services
Monthly Newsletter
August 2001

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.

FOCUS: Avoiding Phlebitis and Blood Clots During Treatments

Porphyria patients will often experience phlebitis. Quite often porphyria patients undergoing regular intravenous infusion will also experience blood clots.

Painful and potentially debilitating, this complication of I.V. therapy is usually avoidable.....yes AVOIDABLE.

One of the problem with hepatic porphyria patients being on long term intravenous infusions is that so many will develop chemical phlebitis. Furthermore there is the factor that you are at increased risk for vein sclerosis, infiltration, and thrombosis. As a porphyria patient you should learn as much as can about this.

As a hepatic porphyria patient It is your body and veins which are being addressed. Porphyria attacks or even the chronic smoldering porphyria has enough pain of it's own without the added pain. Moreover you do not need to add the medical seriousness of thrombosis or the worsening effects of phlebitis.

Toxicity and the solution's pH will affect your risk of having chemical phlebitis. Ask your physician to always check on this aspect of iv infusion. Also you should always ask the RN dealing with your iv access about this. Many hospitals have specific IV Specialists which will come to oversee your infusion while you are hospitalized.

Medical personnel in charge of your iv. access should know about the toxicity and the pH. Such knowledge will make all of the difference whether you will end up with a blood clot. Also before you start an iv infusion your veins should be reviewed along with your previous hospitalization chartings as to accessibility of veins, and your "boxscore" on phlebitis and other previous vein problems.

If you are on home infusion, permanent PICCs or PORTs will help eliminate most of these problems of phlebitis and blood clots as long as the access lines are routinely flushed.

Today, more than ever before, many hepatic porphyria patients are going to home infusion. Cost effectiveness is one major reason, from the medical provider's standpoint. From a more personal standpoint cost effectiveness is important, but second to the ability to continue a quality of life by taking care of your health matters at home and without being away from one's own bed and comfort settings.

Preventive iv infusion therapy is working well for many porphyria patients. Having a good access line, knowing the basic routine skills involved in iv infusion, and having the security of an emergency telephone number if things should fail, can add much to the quality of a hepatic porphyria patient

It is important to make sure that your care provider has the right vascular access device based on the drug your are receiving and your circulatory condition. Circulatory means the size of your veins and their condition. Many porphyria patients after months or years of IVís just do not have any veins left.

And one type of device does not "fit all". Some patients have tried both glucose and panhematin.

In the hospital glucose for porphyria intervention or preventive therapy should start with veins that are large enough for the volume and duration of infusion. Often access is by the use of Hickmans.

The question of panhematin should be infused in a large vein; use a central line if possible. Using the smallest catheter gauge possible, place the IV device in a vein with enough blood flow to allow hemodilution as the drug infuses.

For many porphyria patients a midline or peripherally inserted central catheter is suffice, rather than using a peripheral IV line. This translates PICC. Many porphyria patients use the PICC line -catheter. Having a PICC requires daily flushing with heparin and prior to and immediately after infusion requires flushing with sodium chloride as well as heparin.

Other porphyria patients who plan long term infusion may opt for a PORT. Some of the more commons ports are BARD, Titanium, Chemoport, and Passport . Flushing of these access lines is required at least once monthly, as well as before and after all infusions.

Another way of reducing the risk of phlebitis or blood clots is by slowing the infusion.

You can also slow the infusion, administering it over a longer period. A fast-running infusion increases the risk of phlebitis by reducing hemodilution time, which allows more concentrated solution to come in contact with the tunica intima.

Whether you are going to receive infusions in the hospital or in the home setting, be sure to discuss all aspects of avoiding phlebitis and blood clots with your physician, and your infusion therapist.

Cherie Stockwell RN, BS, MSN
IV Specialist

FOCUS: Cause & Effect Relationships in Porphyria

From my observations of many porphyria patients and reading many case histories of porphyria patients, medical science is increasingly aware of the effects of porphyria, but are still at a loss for the most part as to the cause of such effects.

There are many illnesses for which doctors can find no cause. And there are still a lot of physicians who will from time to time become frustrated and conclude that such illnesses are all in the patient's head. Such illnesses we are now finding are probably caused by multiple physical, psychological and social factors interacting in complex ways not yet understood.

In porphyria we know that when carbohydrate levels fall that the liver will overproduce an abundance of porphyrins which then manifests in what is known as an acute attack. Such low carbohydrate also produces an abdominal pain. How this happens is still unknown.

Also in porphyria it is known that when electrolytes levels becomes imbalance, mental confusion will often occur. Often seizure activity will occur. What specifically is the cause and what the direct relationship between the two is still unknown.

A bigger question remain as porphyria patients are faced with an ever-growing abundance of pharmaceuticals from which to choose medications. A specific drug will have no reaction in one patient and at the other end of the scale it will cause hallucinations, respiratory paralysis and or peripheral neuropathy in another.

Reasons for the large variation is not known.

Another example of unknown factors is that of porphyria based peripheral neuropathy. Often porphyria patients undergo CT Scans, MRIs, various forms of neurological testing and most of the results will come back negative, yet paralysis, numbness and tingling effects are noticeable.

In porphyria medical science has had many breakthroughs, yet overall research is just "scratching the surface".

Dr. Robert Johnson M.D.

Focus: Quality of Life and Chronic Smoldering Porphyria

An ever-growing percentage of hepatic porphyria patients are now being termed as "chronic smoldering" patients. The condition seems to be more and more common.

Chronic smoldering porphyria without a doubt has a direct impact on the patients' quality of life while they are symptomatic. To date there have been no studies that attempt to quantify quality of life in patients with chronic smoldering symptoms.

Quality of life is the subjective value that a person places on satisfaction with his or her life and it is influenced by several factors. Several aspects of quality of life have been documented and defined as being measurable with consistent, reproducible findings.

And what are these aspects?

These aspects are physical functioning, role limitation caused by physical health, role limitation caused by mental health, vitality, emotional well-being, social functioning, pain, and patient perception of general health.

In conclusion it would suffice to state that suffering from a chronic smoldering porphyria has a detrimental influence on patient quality of life.

Dr. Kenneth Carson

Focus: ICD # for Porphyria

Will medical insurance pay for testing and treatment of Porphyrin Disorders?

It should.

Porphyria as well as the vague area of MCS are all coded under Disorders of porphyrin metabolism.

While some forms are rare and we only represent up to 9% of the world's population, Disorders of Porphyrin Metabolism are widely accepted by the medical community.

Porphyrin disorders and "porphyrinurias" have been listed by the International Classification of Diseases since 1920 (currently ICD 277.1).

This ID # is important for using in trying to obtain SSI as well.

BTW, the diagnostic need for detailed biochemical testing is well established in the medical literature (urine and/or stool testing alone are insufficient).

There also is broad agreement that the primary focus of treatment in acute cases must be on the avoidance of exposures that may trigger an attack, and using glucose as intervention.

Since so many types of porphyria have such potentially serious consequences, there is no excuse for denying complete and prompt evaluation in any suspected case.

Please, please let your physician know if your insurance company refuses to pay for porphyria diagnosis and/or treatment.

You may want to appeal, and there are several sources that may be able to help. The MCS Referral Services is one of them. Patient Advocacy Services is another.

Diana Deats-O'Reilly, CEO
Porphyria Educational Services

FOCUS: Headaches as a Manifestation of Porphyria

Many porphyria patients believe that they suffer from migraines, however there is little clinical indication for this premise. These porphyrics consider any bad headache a migraine. However in truth the majority of headaches are not migraines. However in the actual cases of a migraine condition there is today many new and very effective treatments available.

Many of the headaches experienced by hepatic porphyria patients are headaches which are caused by muscle contractions. Such contractions are often due to stress or the lack of quality sleep. The contractions will appear in the shoulders, neck and the head. Often they will be accompanied by stress, tension or/ and anxiety.

In the general population it is noted that headache is one of the most common reasons people see a doctor. Surprisingly in porphyria headaches rank relatively low with such things as abdominal pain, ongoing nausea, vomiting and constipation as more prominent.

It must be kept in mind that there are a variety of things that porphyria patients can do for themselves in order to avoid headaches or recover from headaches of any variety.

Regardless of the fact that one has porphyria, one needs to continue to exercise. It is necessary for circulation, emotional health, easing pain, and ridding peripheral neuropathy.

Another factor in headaches is avoiding certain foods, especially those with sulfites, or any food known to an individual to cause distress.

Sometimes behavioral and lifestyle changes are necessary. One area that needs to be accessed is that of quality sleep. Many porphyria patients complain of restlessness or insomnia. Fatigue will often lead to headaches. Avoiding unnecessary stress is another area that can bring on headache because of the tension involved.

Dr. Kenneth Carson
Neuropsychiatric Medicine

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.

RELAXOL 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.

ZIPAN 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.

MAXZINE is a brand name for the generic drug HYDROCHLOROTHIAZIDE TRIAMTERENE. It is classified as a dieuretic. It can cause liver enzyme abnormalities. It can also cause renal failure. This drug can also reduce levels of blood serum potassium essential to electrolyte balance. This drug also contains the ingredient of sulfate.

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

SETPRIN 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.

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

DAZID is a brand name for the generic drug HYDROCHLOROTHIAZIDE TRIAMTERENE. It is classified as a diuretic. It can cause liver enzyme abnormalities. It can also cause renal failure. This drug can also reduce levels of blood serum potassium essential to electrolyte balance. This drug also contains the ingredient of sulfate.