Porphyria Educational Services
Newsletter Summer 2007
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
Specific recommendations for the confirmed diagnosis and treatment of any individual
must be accomplished by that individual and their personal physician, acting together
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.
Chronic Pain and the Porphyric
Chronic pain exacts a high toll on the individual porphyria patient.
In addition chronic pain also places a toll on the patient's family, friends and on the health care system.
Chronic pain also plays a role in the patient's workplace and their productivity.
Chronic pain associated with porphyria is very limiting.
Such pain limits functioning in everyday activities, which leads to loss of
conditioning and other changes.
Often porphyrics with constant pain face depression.
As pain continues endlessly it produces discouragement and frustration.
Such discouragement or frustration will aggravate the pain.
This is a vicious cycle.
The changes caused by pain, when combined with sleep problems, medication side effects, mounting medical expenses and strain on important relationships, can have a profound effect on a person's mental and physical well being.
When a porphyria patient begins to explain their pain it is important to realize that there are two types of pain: acute and chronic.
With the acute pain, it is a type of pain that is triggered by tissue damage. It may be slight and last a moment, or for a few weeks or maybe a couple of months.
However with acute pain, the source is obvious and the pain eventually fades away.
Contrastly chronic pain is a type of pain that can result from injury or it can occur for no apparent reason.
Such is the case with the chronic pain that is experienced by mnay porphyria patients. It can occur for no apparent reason.
Chronic pain can be mild or severe. It can hurt all the time, or it can come and go.Unlike acute pain, it is often more difficult to find a cause for chronic pain. In addition chronic pain can last an indefinite period of time, in some cases years or decades.
With the upper right quadrantr abdominal pain which is experienced by so many porphyria patients, usually in a matter of hours of the administration of glucose, pain will subside or go into complete remission.
Neuropathic pain is also a part of porphyria. This pain can be somewhat reduced by the use of Neurontin which is used to curtail seizure activity in the porphyria patient.
Bonnie Hedgewick RN
Chronic Pain associated with Acute Porphyrias
Besides porphyria chronic pain, some pporphyria patients will experience chronic pain from other medical conditions that they may have such as arthritis: Inflammation of joints, back pain, muscle strain, nerve damage, muscle loss, osteoporosis, or a herniated disk
Some specific chronic pains comes is located in the mouth, and jaw. Often along with this is face pain: Dental pain is another factor. Neck pain: Muscle strain, nerve or joint problems.
With porphyria there is often Peripheral neuropathy: Here you will find a porphyria patient with Tingling, numbness and pain in the hands and feet.
Having chronic pain often causes the porphyria patient to experience inactivity. Inactivity can cause weight gain and loss of strength and stamina.
Loss of sleep can cause irritability, fatigue and dependency on medications and alcohol in an attempt to facilitate sleep.
Mood swings can lead to damaged relationships, depression, inability to handle stress, and difficulties at work and at home.
Financial strain also can result from efforts to meet medical bills, pay for medications and loss of income due to time off from work.
While porphyria patients are to avoid alcohol, some porphyria patients have a Chemical dependency resulting from chronic pain. Reliance on medications can impair clear thinking, cause physical complications and emotional distress.
Choices for pain medications for use by porphyria patients are very limited.
Alternative solutions such as hot tubs, massage, exercise and electric mattress covers along with the use of a TENS unit can greatly benefit a porphyria patient
Rosalie Schumacher M.S.,N.P
Depression, Fatigue and Porphyria
Another aspects of fatigue is that of depression.
And depression is a common symptom among porphyria patients at one time or another.
Fatigue is a typical symptom of depression.
Porphyria patients often relate that they have a lack of motivation or a loss of energy.
Many anti-depressant drugs are considered unsafe for the porphyria patients. However when a patient is treated with such a drug which is safe for them, they often begin to feel less tired.
Once treatment has begun, the patient will also be more willing to participate fully in a day's activities.
Lack of sleep itself is also a cause of fatigue.
Dr. Kenneth Carlson
Liver cancer (HCC)
HCC has a 67 % increase risk in patients diagnosed with any of the acute hepatic forms of porphyria. There is also an elevated risk for those with PCT.
Such porphyria patients are advised by the American Porphyria Foundation to undergo AFP (alpha-fetoprotein) blood testing annually as well as periodic CT Scans to check for the development of HCC.
Screening and diagnosis
No completely accurate screening test for liver cancer exists. Doctors sometimes use a blood test that checks for the presence of alpha-fetoprotein (AFP) — a type of protein not normally found in adults — to screen people at high risk of the disease.
But the test isn't perfect. Not all malignant liver tumors produce AFP, and those that do may be advanced by the time protein levels become elevated. In addition, other types of cancer and even some noncancerous liver diseases can raise AFP levels.
Although AFP screening can detect small tumors in some people, most liver cancer isn't diagnosed early. That's because symptoms usually don't appear until late in the disease and because liver cancers grow quickly.
As an acute hepatic porphyria patient or a PCT patient, one should remember to have medical exams which cover HCC screenings as porphyria patients are at high risk for HCC.
The most common type of liver cancer, HCC, doubles in size every four months.
Diagnosis is also difficult because the effects of some liver tumors may resemble those of other medical conditions, and because liver cancer can't be diagnosed using a standard panel of liver tests.
If you experience any of the symptoms of liver cancer, such as unexplained weight loss, persistent abdominal pain or swelling, or jaundice, see your doctor as soon as possible.
Bonnie Hedwick NP
Oncology & Heptology