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Porphyria Educational Services
Monthly Newsletter
October 2002

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.

Controlling Pain in Multiple Conditions Collectively

For many acute porphyria patients there is the pain that accompanies the acute attacks. With 48 hours of glucose infusion the pain usually goes into remission as does the acute attack itself.

However for many acute porphyria patients they become ridden with the PN pain, a deep bone pain in the limbs that becomes chronic.

Fentanyl patches have worked for many but skin sensitivities often find the patches cast aside, leaving the patient having to make multiple trips to clinics or hospital emergency rooms to get the break through pain under control.

Of course the best treatment for pain is one that is constant, the right strength and is given in a timely fashion to avoid any break through pain which makes it that much harder to get pain under any type of control.

Added to the pain dilemna for many porphyria patients is pain from other medical conditions such as MS, FM, migraine, arthritis, and many other

Originally designed for use in cancer patients, but now a "given" in pain control for countless others, are the inplanted pain pumps.

With the inplanted pain pumps porphyria patients no longer have to use the pain pumps attached to the iv poles. They can avoid patches, trips to the clinic for injections, or being hooked to iv pain pumps. Most of all they can be relatively pain free.

As documented with many porphyria patients, the pain from other medical conditions often becomes the trigger of acute porphyria attacks.

An example is that of a person with extremely painful FM, migraines, PN secondary to the porphyria and porphyria its self.

FM is painful and without timely or proper medication = stress
Stress = Migraine headache onset
Migraine = acute attacks (nausea, vomiting, abdominal pain and PN)
PN = Peripheral neuropathy with deep bone pain

All four conditions now excerbate extreme pain. How does one treat these painfful conditions presenting with pain?

Do you treat each pain cause individually? Do you treat pain in a combined pain treatment? If so, how so?

Many porphyria patients have skin sensitivities which do not allow for long term use of Fentanyl patches. Many also have logistical problems in receiving treatment especially in rural areas due to the great distances the patient must travel.

Even during hospitalizations pain reaches breakthrough before additional dosages are given, causing the situation to be worse than needed and allowing for the porphyria to kick in.

Because all four medical conditions present with severe pain, PAIN is the focal point of concern.

With proper pain control the likelihood of triggering further porphyria acute attacks will most likely drop significantly.

WIth many other patients with multiple medical conditions all exhibiting pain the use of an implanted pain pump has produced relative pain free living and greatly reduced acute attacks of porphyria.

Combining the use of the implanted pain pump along with the use of home infusion of glucose and electrolyte supplementation (as needed) acute porphjyria patients can now be free of repeated hospitalizations and continuous suffering due to uncontrolled pain

. Dr. Robert Johnson M.D.
Retired Clinician

The Different Carbohydrates and Porphyria

If you have a diagnosis of an acute hepatic porphyria then you need to learn as much as possible about carbohydrates.

Low intake of carbohydrate is known to trigger acute attacks of porphyria. Acute attacks are caused when an overproduction of porphyrins occurs within the liver for the forms of AIP, HCP and VP. While the exact science or reason for glucose is not fully understood, it is known that the intake of glucose (the purest form of carbohydrate) will stop the over production of porphyrins and thus bring an acute attack into remission..

Carbohydrates are your body’s main energy source. Carbohydrates are used not only in your liver to maintain the right level of porphyrin production which takes place there, but carbohydrate are used throughout the entire body.

Carbohydrates are used in the brain as its primary source of fuel. That gives meaning to the old caption stating "Brain Power".

And what makes carbohydrates? At the base of all carbohydrates are sugar components. There are different kinds of carbohydrates and this important for porphyria patients to remember.

Depending on the number of components and how they’re linked, a carbohydrate is classified as a simple carbohydrate (sugar) or a complex carbohydrate (starch).

After being consumed the body breaks down complex carbohydrates into simple sugars.
And what makes up simple sugars? Sweets, milk, fruit and some vegetables contain simple sugars. .

And what makes up complex carbohydrates? Grain products and certain vegetables contain complex carbohydrates.

Another name for the complex carbohydrates could be that of starches. Starches are complex carbohydrates and include bread, cereal, rice, pasta, beans and certain vegetables, such as corn, potatoes and squash.

Simple sugars are contained in fruit and dairy products. Every form of fruit, from the familiar apples, bananas and oranges to pears contains simple sugars. Milk and milk products contain simple sugars.

The vegetables with simple sugars includes all nonstarchy vegetables, such as lettuce, asparagus and zucchini.

When planning your meals for throughout the day is best to eat a mixture of complex and simple carbohydrates. The advantage of complex carbohydrates is that it takes your body longer to break them down into sugar. This means sugar enters your bloodstream at a prolonged rate. With some simple carbohydrates, sugar may enter your bloodstream quickly.

For porphyria patients this is excellent for maintaining a continuous intake of carbohydrate into the liver. For those porphyria patients who are also diabetic, this continuous flow of carbohydrates is excellent in maintaining a rather constant blood serum glucose level. .

The more fiber the food contains, the more slowly it’s digested and the more slowly your blood sugar level rises which benefits diabetes, but is not beneficial in carbo-loading pending the onset of an attack, however it is good in maintaining a constant carbohydrate level for controlling porphyrin production as long as there is a continuous carbohydrate intake.

By spreading carbohydrate intake every 2-3 hours through your waking hours and also including a carbohydrate "middle of the night" snack, you will effectively achieve maintaining a continuous carbohydrate level in the blood stream and liver. At the same time you will achieve maintaining a steady blood serum glucose level which will keep your diabetes under control.

The best way to maintain porphyrin production in the liver and at the same time to control blood serum glucose levels is by eating the same amount of carbohydrates at similar times throughout the day.

Nutritional Guidelines for Porphyria
AIP Medical Guide
Sheryl Wilson (HCP), MSN, RD

Porphyria Patients with the Added Hepatitus C (HCV)

Having various forms of porhyria is a whole learning experience and something that one needs to deal with medically and most likely for the rest of their lives.

For some poprhyria patients, especially those with PCT, many find themselves with chronic hepatitis C (HCV) as well.

Often these porphyria patients are surprised to find out that they harbor this virus, because for the most part symptoms are usually absent. The absence of symptoms is even true in the more advanced stages of the disease manifestations.

Even porphyria patients who have progressed to cirrhosis find themselves with HCV and are shocked to find out this diagnosis.

And just like with many other liver diseases, if the symptoms of HCV are present, they are usually non-specific. Porphyria patients are very familiar with the terminology "non-specific" because porphyria in any forms is generally very non-specific.

It is noted that only about 20% of people with chronic HCV experience symptoms. Those symptoms most generally noted include fatigue and generalized weakness, and are often masked as a generalized weakness and fatigue often accompany the symptoms of porphyria as well.

And as hepatic porphyria patients complain of abdominal pain, it is easy to be unaware of the vague abdominal discomfort, often in the area over the liver that is a part of HCV.

Weight loss, which is contraindicated for acute hepatic porphyria patients, is often a sign of HCV. Decreased appetite, weight loss, and depression all have been noted in HCV patients.

WIth HCV there is liver damage and inflammation of the liver itself. With the usual signs and symptoms of poprhyria the signs and symptoms of HCV are often masked.

Physical findings are often normal in people with chronic HCV.

Unless a porphyria patient's physician is looking for an enlarged and tender liver, such signs and symptoms of HCV go unnoted, allowing for further liver damage to occur.

In porphyria cutanea tarda (PCT) in which there is a skin abnormality that may present as easy bruising of the skin, in addition to blisters that are sensitive to the sun and bleed easily. In addition there may be areas of increased or decreased skin pigmentation and increased hair growth, known as hirsuitism.

Both hypothyroid and hyperthyroid have been indicated to occur in approximately 5% of the individuals with chronic HCV. These disorders often worsen once therapy with interferon has been initiated. Elevated blood sugar (glucose) levels – has been found to be present in many people with chronic hepatitis C.

Gregory Jackson PA
Internal Medicine

Knowing the Specific Types of Pain

All porphyria patients experience pain. But not all pain is the same. Nor do all porphyria patient experience the same types of pain.

Acute hepatic porphyria patients are all too familiar with the colicky gnawing and sometimes severe abdominal pain in the lower right quadrant adjacent to the liver and often mistaken for liver pain.

Pain is an unpleasant sensory and emotional experience associated with actual or potential tissue damage or described in terms of such damage. Pain can be acute or it can be chronic. It can be mild or it can be severe. Pain can wax and wane.

When pain is acute it usually occurs immediately and then resolves quickly.

When pain is chronic like it is with many aucte hepatic porphyria patients who are termed as chronic smoldering porphyrics, pain is there almost all of the time. It may be mild, but you still know it is there. For other, all too often the pain is severe during the acute attacks and then the PN pain comes and can easily bring one to their knees.

Chronic pain persists beyond the time of normal healing and can last from a few months to many years. For porphyria patients it mostly results from the peripheral neuropathy that acompanies ongoing acute attacks. And many acute porphyrias patients also become diabetic and in time experience painful diabetic neuropathy as well.

The pain from PN or diabetic neuropathy is known as Neuropathic pain. Neuropathic Pain originates from a damaged nerve or nervous system.

Myofascial pain is still another type of pain. Myofascial Pain has tenderness in the muscles and adjacent fibrous tissues (fascia).

Some acute porphyria patients unfortunately also have the medical condition known as fibromyalgia. Again this is painful and in normally healthy people can be treated with anti-inflamatories. However in acute porphyria patients the use of most anti-inflammatories is contraindicated.

One of the best things porphyria patients can learn is that of tolerance, and keeping busy.

Pain most likely is here to stay, so one needs to makes the most of their life and work around the pain.

There are many levels of pain medication, physical therapy, massage, TENS, theurapeutic pools and relaxation techniques that can help with the pain. For the most severe and chronic pain there is a implanted spinal pain pump.

Work through the options and find those most appropriate for your pain.

Georgia Littleton RNP
Pain Management Team

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 judgement 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.

ALLEGRA is a brand name for the generic drug FEXOFENADINE which is a non-sedating antihistamine The drug carries a warning with it for those who have liver or kidney disease, or electrolyte abnormalities or low potassium.

ZYBAN is the brand name for the generic drug BUPROPION. It is classified as an aminoketone drug and is used as an anti-depresant and for smoking cessation. Side effects of this drug include abnormal liver funtion, photosensitivity, jaundice, hepatitus, pancreatitis, edema, peripheral edema, leukocytosis, muscle rigidity, leg cramps, muscle weakness, depersonalization, neuropathy, and liver damage. WARNINGS & PRECAUTIONS: Not recommended for persons with liver disease.

REACTINE is a brand name for the generic drug CETIRIZINE which is a non-sedating antihistamine The drug carries a warning with it for those who have liver or kidney disease, or electrolyte abnormalities or low potassium.