Make your own free website on

Porphyria Educational Services
Monthly Newsletter
December 2003

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.

Sulphite Sensitivity High Among Acute Porphyrics

Sulfite sensitivity appears to occur in severe asthmatics, although some reports of sulfite reactions indicate that a small number of non-asthmatic individuals such as those with acute porphyria may also be at risk.

Persons at risk for sulfite sensitivity among the affected population vary widely. Some individuals with higher tolerate level are not likely to have problems with dietary sulfites, but some porphyria patients with a lower tolerance level could ingest a sulphite dose which could ultimately trigger an acute attack of porphyria all contained within one meal.

Another factor regarding sulphites is that some porphyrics may respond one day and not another day , depending on the natural course of their illness.

Some sulfited foods do not elicit a triggering effect in sulfite-sensitive individuals, but for others it compounds to the list of on-going side effects that a porphyric experiences.

Sulfites pose no health hazard to most Americans, but to acute porphyria patients sulfites are known to be a problem for many, but not all.

The sulphites are used primarily to reduce or prevent spoilage and discoloration during the preparation, storage and distribution of foods. The products so treated include processed potatoes, many dried fruits, dehydrated vegetables and some seafood, especially shellfish. Sulfites retard browning and inhibit the deterioration of such nutrients as vitamin C.

For a number of years sulfites were used to help maintain the fresh appearance of fresh fruits and vegetables in salad bars and produce departments but were later banned.

Sulfites are used in a substantial number of prescription and (OTC) over-the-counter drugs to help maintain their potency and stability. Drugs handbooks or packaging pamphlets will indicate the chemical elements contained within a drug. These are important to read and to double check when trying a new drug for the first time. Most porphyria patients need to avoid such chemical containing drugs.

Sulfites, or sulfiting agents, are sulfur-based substances used primarily as preservatives. Six are used by the food industry: Porphyria patients should check food labels for any of the following terms as indicators of sulfites: sulfur dioxide, sodium sulfite, sodium bisulfite, potassium bisulfite, sodium metabisulfite, potassium metabisulfite or the term "sulfiting agent."

Overall the most reported reactions to sulphites cover a wide variety of symptoms - from hives, nausea and diarrhea to respiratory failure. They have in some instances been thought to be the provoking factors of an acute porphyria attack.

Sheryl WIlson MSN, RD

Cranial Nerve Involvement in Porphyria

The cranial nerves often come into play during acute attacks of porphyria, along with many other symptoms associated with the CNS (central nervous system) and the ANS (autonomic nervous system)

Each of the nerves has it's own specific roles and affects specific senses or bodily parts.

To familiarize porphyria patients with the cranial nerve involvement that often affects them it is beneficial to know the type and function of the nerves.

The olfactory sensory controls olfaction which is known as the function of smelling.

Optic nerve is again a sensory nerve and deals with our vision. Often porphyria patients will refer to this problem as having "fuzzy eyes" or as optic neuritis.

Oculomotor motor deals with the nerves of eyelid and eyeball muscles. Trochlear motor nerves deal directly with the eyeball muscles.Abducens motor nerves actually control the eyeball movement.

Another common experience of porphyria patients is having trouble chewing and swallowing during an acute attack.Trigeminal mixed nerves which are again a part of the sensory: nerves affect the facial and mouth sensation that a person has. Motor nerves affect the ability of chewing and swallowing.

Facial mixed sensory nerves will effect the taste we experience while eating.: The motor: facial muscles and salivary glands are active in this process, while the glossopharyngeal sensory nerves affect taste and the motor affect the ability to swallow.

Our ability to hear is controlled by the auditory nerves which are sensory . In addition to our hearing these nerves also control our ability to balance

Cranial nerve involvement is indeed a part of porphyria acute attacks.Not all attacks will involve the same cranial nerves, nor will the affects be the same each time.

Bulbar paralysis and respiratory paralysis are severe effects of an acute attack, and many cranial nerve symptoms may preclude the bulbar paralysis and may also serve as a warning.

Leslie Goodwin PA

Electroencephalograms Used to Rule Out Conditions

Electro encephalograms are commonly known as an EEG. EEGs are used for diagnostic purposes in many medical conditions, but not to diagnose porphyria. EEGs however rule out many other medical conditions that may present similar to neurological symptoms experienced in porphyria.

In porphyria patients the EEG is not a test for peripheral neuropathy but is carried out to exclude other causes of similar symptoms.

The EEG measures the electrical activity of your brain, as a series of brain waves recorded by an instrument as paper traces. It can display the general state of arousal of different parts of the brain. It can also show areas of the brain that are not functioning correctly either because of too little or too much electrical activity.

Several electrodes will be attached to your scalp using adhesive paste. The test may take up to an hour to complete. It is painless. There are generally no risks involved with an EEG.

Lance Driscoll, MT

Disorientation Part of CNS Symptoms in Porphyria

Being disoriented and having a sense of loss of position can be quite scary for the porphyria patient. You simply at times do not know which end is up, or which way to go.

This is another unusual feeling, and porphyria patients run into all kinds of unusual feelings which are hard to describe, or furthermore for others to understand.

Loss of sense of position is describe by porphyric patients as " your brain knows where your feet should go, but your feet have a mind of their own.

Many times the porphyric patient may become conscious about the way that they walk, but they can not for the life of them understand why it has altered.

Without realizing it the average porphyria patient will probably re-educate the way that they walk, possibly widening their gait or throwing their leg.

They will probably have difficulty keeping their balance especially in the dark and find that they will have to look at the ground to compensate for the loss of position sense.

Yes, it will at times seem like you are going every which way but the way you think you are going or wanting to go.

Renae Stillman PTA
ReHabilitative 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.

VALIUM is the brand name for the generic drug DIAZEPAM. This drug is a Tranquilizer, Anti-convulsant, and a Benzodiazepine. It is used for the treament of muscle spasms, anxiety disorders, seizure disorders, alcohol withdrawal, and insomnia. Side effects include clumsiness, drowsiness, dizziness, hallucinations, confusion, depression, irritablity, rash, itch, vision changes, constipation, diarrhea, nausea, vomiting, difficult urination,. vivid dreams, behavior changes, abdominal pain, headache and is photosensitive. It carries a WARNING: ***Do not use if your have Porphyria or liver disease.

CAPOTEN is the brand name for the generic drug CAPTOPRIL. Is is classified as a Angiotensin-Converting Enzyme [ACE] Inhibitor. It is used for the treament of high blood pressure, kidney disease in diabetic patients, and treatment of acute myocardial infarction within 24 hours of occurence. There are many side effects to this drug including rash, fever, flushing, hypotension, tachycardia, chest pain, palpitations, vomiting, diarrhea, anorexia, constipation, dizziness, headache, malaise, fatigue, insomnia, alopecia, paresthesias, Pancreatitis, jaundice, hepatitis, hyponatremia, confusion, depression, nervousness, and blurred vision. This drug carries a WARNING that it is not recommended for those with autoimmune disease.and for those with liver disease or lupus, and causes photosensitivity when exposed to sun or ultraviolet light. Avoid exposure.

XANAX is the brand name for the generic drug ALPRAZOLAN which 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