July 2003 PES Bulletin

Abdominal Pain in the Acute Hepatic Porphyrias

Abdominal pain is is almost universal among patients who are diagnosed with one of the various forms of acute hepatic porphyhria, those being AIP, VP and HCP.

In a study published in "Scientific American" on "The Porphyrias" in 1996 it was found that 90 percent of acute porphyria patients experience the abdominal pain associated with the onset of an acute attack. The abdominal pain is experienced by those who never fully go into remission and are termed "chronic smoldering" porphyrics.

Ab pain, as it is most often called can be described as an old fasioned "belly ache.; Women often will relate that it is like menstrual or labor associated abdominal cramps. Men will most often call it stomach pain or gut pain. In the acute porphyrias however it is all related to abdominal pain of which there remains very little medical understanding. It is known that it is real, but little is know of it's exact cause, except it presence.

Most often it is referred to as upper right quadrant pain, some the lower right quadrant. Such abdominal pain is a nonspecific symptom. This means that such abdominal pain may be associated with a multitude of conditions.

In general abdominal pain origin's can be quite varied. Some abdominal pain does not occur within the abdomen itself but cause will cause abdominal discomfort.. Other abdominal pain will originate within the abdomen but is not related to the gastrointestinal tract. . Other pain is related directly to the gastrointestinal tract.

And many porphyria patients have experienced emrgency room evaluations where first response physicians thought such pain to be the appendix and some porphyrics have indeed gone under the knife and had the appendix removed to no avail. It was not the appendix causing the pain. However If theattending physician finds evidence of peritoneal inflammation, the abdominal pain may be classified as an "acute abdomen" which often requires prompt surgical intervention. Unfortunately, all too many porphyria patients have had to undergo unnecessary surgical procedures only to find that the pain presents over and over again.

Emergency room personnel should always have a high degree of suspicion when a patient presents with such abdominal pain without any specific diagnosis. If the patient is a repeated admit with this the same pain manifestation, appropriate testing for the porphyrias should be immediately started.

Because abdominal pain is nonspecific, the attending physician will require specific information regarding the time of onset, duration of pain (minutes, hours, days, or even months), location of pain, nature of pain (dull, sharp, steady, crampy, off and on), severity of pain and relationship to normal functions such as menstruation and ovulation Often in women acute attacks of porphyria have an onset a week to 10days prior to the menses.

When porphyria patients present to the emergency room they should carry with them charting which describes their overall medical history. Often hospitals do not have access to clinical charts where a patient's overall medical history is contained.

The reason for carrying such charting is that the attending physician will try to relate the abdominal tenderness to other general symptoms such as fever, fatigue, weakness, nausea, vomiting, malaise, or changes in stool. They will also almost always ask about increasingly specific symptoms as the diagnostic considerations are narrowed.

Even if a patient is sure that is still another porphyria attack beginning, it is never to be assumed that it is just porphyria. Unfortunately, like everybody else, porphyria patients experience other medical conditions as well. For instance, abdominal pain, nausea and bloating or constipation may indicate a bowel obstruction. And as you read this, it reads just like porphyria. And again, abdominal pain followed by nausea/ vomiting and fever may indicate appendicitis.

When experiencing the abdominal pain, sip water or other clear fluids. Do avoid solid food. until after you have been examined. Also avoid narcotic pain medications., aspirin and NSAIDS.Antacids may provide some relief.

A physical examination with a focus on the abdomen will be performed. Observation in the hospital may be required in severe cases. If pain persists, re-evaluation will be necessary.

Once other conditions have been ruled out administration of iv glucose can begin either at the hospital or at home, depending on what porphyria treatment plans you have inplace.

For those who only think it may be porphyria or even if you know it to be but are without a confirmed diagnosis, chances are great that some medical professional will want to further explores the abominal pain.

It is not unusual for many porphyria patients to have a history that has included blood, urine, and stool tests which are normally done anyhow; barium enemas, upper GI and small bowel series, endoscopy, ultrasound of the abdomen, upper GI (gastrointestinal) tract (EGD) probes, X-rays of the abdomen or an ultrasound of the abdomen.

Abdominal pain for most porphyria patients unfortunately is a signal that another acute attack is pending

William Simpson, PA

Always Read Drug Labeling for Safe Use

It is very important to read the auxiliary labeling of a prescription drug.

An auxiliary label that says "avoid prolonged exposure to sunlight" means both: protect yourself from the sun any time you are outdoors and, protect yourself from sun that shines through a window

These labels are used for drugs that may cause photosensitivity - an exaggerated response to UV rays from the sun and other sources.

The reaction, which may happen very quickly, will most likely appear as a burn, rash or other skin problem.

This is often caused by UV-A rays, which are present throughout the day and can go through glass.

Auxiliary labels are the small colored labels on your prescription bottle. They are usually placed secondary to your main label which has your name, the name of your physician and the name of the medicine.

Be sure to ask your pharmacist if you don't know exactly what these labels mean. Most pharmacists are more than happy to explain unfamiliar terminology with a patient.

When taking any medicine, it is very important to fully understand the labeling. Not everyone is familiar with "P.R.N." and what it means. Always ask if you are unsure.

A person must learn to find out what all of the fillers in a medication are. For persons with a corn allergy, or other types of allergies, such fillers can be deterimental.

The same drug, manufactured by a different company, may use different fillers and yet be called the same drug.

It is the fillers that often cause the adverse reactions to patients who are chemically sensitive.

Always read the pamphlets before you take any of the medicine.. Many drugs on the market today can cause a chemical change in the body. In porphyria, drugs are most likely the primary trigger of acute attacks for many patients.

Gary Larson RPH
Pharmacist

Avoidance of Sulfites is Essential for Acute Porphyrics

Porphyria patients routinely face the dilemna of what is safe and unsafe to use as a pharmaceutical. They also have to be extremely careful in selection the foods that they ingest. One of these problems is that of sulfites.

When a porphyria patient's protect Sulfite Oxidase enzyme is low, the ingestion of sulfites can hurt a person. Sulfite oxidase is a chemical that converts one chemical to another chemical. WHen the body is low in this chemical, sulfites can be harmful.

Sulfite Oxidase converts sulfites to sulfates, which are not harmful. If your Sulfite Oxidase enzyme has a diminished level, the sulfite will be carried around in your blood and inhibit important enzymes such as Tyrosinase, polyphenoloxidase, and ascorbate oxidase.

When this chemical conditions presents it can result in the malfunctioning of the synthesis of Dopamine and the conversion of Dopamine to Noradrenaline. When this conversion takes place, it can lead to neurological fatigue. Neurological greatly present in acute porphyria patients, which are all ready in a weakened condition. Ingestion of sulfites can only add harm to the acute porphyric.

Sulfites can impair liver cell ATP energy production. In addition sulfites can inhibit 90% of lung ATP energy production. Moreover sulfites can deplete glutathione. Glutathione is a chemical that helps the liver filter the blood and helps protect cell enzymes from damage.

It is well known that anything that lowers your production of ATP energy can cause fatigue, since low levels of energy are synonymous with fatigue.

Besides sulfites themselves other elements can cause damage to the protective sulfite oxidase. Such elements include heavy metal molecules such as lead and mercury,

Sulfa-drugs likewise cause damage, as well as having a molybdenum deficiency.

It is important to remember that "sulfites" are also known as "sulphiites". This chemical agent is often used as a form of food preservative. Many food contain sulfite preservatives. Some such foods include red wine, grape juice, bottled lemon and lime juices, packaged dried fruits, and molasses.

To know when you may be affected by sulfites a person will experience fatigue after eating, and often starts coughing. The development of low blood serum glucose will present due to disruption of the regulation of blood sugars. Many experience headache and also have rash or reddening of the skin and itching present.

Porphyria patients can avoid sulphite side effects by avoiding sulphites. Always check prservatives that are in prepackaged foods as well as double-checking with your pharmacist about any sulphite containing pharmaceuticals.

Dr. Lester Sinclair PhD
Biochemistry & Metabolism

Anxiety Often a ANS Presention During Attacks

When acute porphyria patients experience acute attacks, mental changes can occur. Such autonomic nervous system (ANS) changes take place during porphyria attacks and can include several different expressions of mental change mimicing many common psychological maladies.

Anxiety is one of the many mental changes that are regularly experienced. And just what is this anxiety? It is in itself a multisystem response to a perceived threat or danger.

Anxiety is the product of a collection of biochemical changes in the body. Basically the porphyria patient's memory, lifestyle, hardships, medical condition, and other personal history. Medical researchers to date believe that anxiety is a uniquely human experience. Other animals clearly know fear, but human anxiety involves an ability, to use memory and imagination to move backward and forward in time, that animals do not appear to have.

When anxiety presents in a porphyria patient it is important to distinguish between anxiety as a feeling or experience, and an anxiety disorder as a psychiatric diagnosis.

In normally healthy persons, a person may feel anxious without having an anxiety disorder.

And it is important to remember that a person facing a clear and present danger or a realistic fear is not usually considered to be in a state of anxiety.

In most porphyria patients the presence of anxiety during an acute attack is not considered a psychiatric disturbance.

Anxiety is a commonplace experience that everyone has from time to time, however it is difficult to give a firm description because it has so many different potential causes and degrees of intensity. Anxiety can be viewed as either "emotion" or "affect" and sometimes both.

Anxiety can be related to fear, but it is not "fear" itself. Anxiety is a multidimensional response to stimuli in the person's environment, or a response to an internal stimulus resulting from a combination of general biological and individual psychological processes.

In the case of acute porphyrias, anxiety is produced by physical responses to the disease processes as well as often the medication prescribed for the various aspects of porphyria.

Elizabeth Upjohn MSN, RN
Critical Care Nursing

PES Monthly Drug Update

Disclaimer
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. TRAMOL is a brand name for the generic drug TRAMADOL. It is an analgesic. Seizures have beenreported 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.

SERAX is a brand name for the generic drug OXAZEPAM. It belongs to the BENZODIAZEPINE class of drugs. The drug contains sulfates. The drug carries a warning against use in persons with the disease porphyria.

LEVANXOL is a brand name for the generic drug TEMAZEPAM. It belongs to the BENZODIAZEPINE class of drugs. The drug contains sulfates. The drug carries a warning against use in persons with the disease porphyria.