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Porphyria Educational Services
Monthly Newsletter
November 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.

Implantable Pain Pump Offers Promise to Chronic Patients

Whether the primary pain is that of neuropathic pain associated with PN due to acute porphyria, or whether it is due to other medical conditions that a porphyria patient may have, pain can now be effectively controlled in most cases.

Manual pain pumps have existed for some time, but now with the new implantable pain pump systems one does not have to push buttoms, set dials or meters. An injection once a month is all it takes for continuous pain medication to block pain signals to the brain.

When oral medications and/or nerve blocks do not sufficiently control the pain, advanced pain therapies or implantable systems may be effective.

These implantable pain systems are designed to interrupt transmission of pain signals from the spinal cord to the brain. If the pain signals do not reach the brain, then the patient does not actually feel the pain.

One form of these implantable pain systems is that of the spinal cord stimulation (SCS) for pain control introduces low levels of electrical current to the dorsal portion of the spinal cord to block the sensation of pain.

This implantable pain pump system is implanted during a surgical procedure, and may include a fully implanted system or a system with an external power source.

Current medical literature indicates that 50 to 60 percent of patients will get a 50 percent or better pain relief with this procedure that has lasted up to 20 years post implant.

Spinal pumps (or pain pumps) delivers pain medication (typically morphine) directly to the intrathecal space around the spinal cord via an implanted pump. The pump is implanted during a surgical procedure, and medication in the pump is added periodically (e.g. monthly) by injecting medication through the skin into the pump reservoir.

Spinal pumps may be used to manage chronic pain from various medical conditions including porphyria. Spinal pumps are also used to treat painful spasticity as is seen in multiple sclerosis.

Most often multiple medications are put into the pump to treat certain specific situations. That is, morphine to treat the nociceptive pain and local anesthetics (such as bipuvicaine) to treat a neuropathic pain component.

Both of the procedures are reversible and the implantable system can be removed.

Lewis Simpsom P.A.
Pain Management

Pain Medications and How They Work

Often it is heard by porphyria patients that they tried one particular pain medication and it worked for awhile and then the pain started to hang around dispite the use of the medication.

The term "tolerance" is a good one to use the foregoing description.

Tolerance is the point at which a person adapts to a specific substance, so larger amounts of the prescribed medication or a new medication is needed to achieve the same results

Another problem with pain medication is when should the next dosage br given.

Each dosage has a given life time of effectiveness, and just prior to the end of that pain management time the next dosage should be given. Most pain medications take a few minutes to 20 minutes to become effective, depending on how they are given.

When dosages are not given in a timely manner it allows for "breakthrough" pain to occur. Such "breakthrough" pain when it presents, makes management of the pain that much harder and longer to control.

There are several types of pain medications that are used.

The analgesics are a medication or agent that reduces pain. There are many of them available.

Another class of drugs are those of the Narcotics. Narcotics are a group of drugs that relieve pain by preventing transmission of pain messages to the brain.

Nonsteroidal anti-inflammatory drugs (NSAIDs) are medications, used to reduce inflammation, that arenít corticosteroid-based. Some are contraindicated for use in acute hepatic porphyrias, but others are well tolerated.

Opioids. are prescription medications that relieve pain by binding to receptors in the brain and spinal cord. Some are natural compounds derived from opium, others are synthetic medications that work in a similar way.

Most anti-seizure medications are contraindicated for porphyria patients. One anti-seizure medication, Neurontin (also known as Gabapentin) has been found to be completely safe for use in acute porphyria patients.

Neuronton is used to prevent seizures. Like several other anti-seizure drugs, this drug has been found to be effective in reducing pain that originates from a damaged nerve (neuropathic pain). This drug is also known as an antiepileptic or anticonvulsant medication.

When needing surgery of various forms there are several forms of anesthesia as well.

Georgia Littleton RNP
Pain Management Team

Stress That Porphyria Patients Need to Avoid

Fortunately for most porphyria patients much of the stress you encounter is under your own control.

By actively taking measures to manage your emotions, you may be able to overcome the anxiety and uncertainty of having a life long medical condition such as porphyria.

Porphyria does not have to consume every minute of your life.

Manage your time, activities and even your reflections. Resist the temptation to focus exclusively on your illness.

Another strategy is to prevent information overload: Porphyria support groups are good and are most useful, but do not spend every waking momement consumed by such a group.

Also evaluate and then control which sources of information that you rely on for porphyria information. Some chats, or general website forums have little or no credibility. Statements are made without source citations. Flaming also takes place leaving all guests facing even more stresss.

You can save yourself stress and time by being selective about your sources.

There is nothing you can do to cure your illness or make it go away. That is a fact and it is something one needs to accept and then go on living their lives to the fullest.

For those whose spirituality is religion based, being active in a parish community or having a religion you feel comfortable returning to, is one way of finding strength through faith. It is important to have a theology that makes sense of the realities of your life, even if you prefer not to attend services.

Have a daily routine. Stick to it. Even if you do not want to get up or do something, do it anyhow.
You have to take responsibility and control of your life. One without the other invites difficulty.

Try to preserve and even strengthen your productive daily routines. This can help reinforce both the value of responsibility and the possibility of control. Your routines can help you get through challenging days by defining your course.

All of these things can make a big difference in your ability to cope with changes

It is always nice if you have reassurance, but most people are unfamiliar with the disease, and really have no concept of the stress you feel in dealing with a life long disease that is so unfamiliar.

Porphyria patients who have a history of depression and anxiety will even have a harder time coping with porphyria.

If anxiety and fear of the uncertain future continue to bother you, talk with your doctor. He or she may be able to suggest other actions to help you reduce your level of distress.

Learning to cope without medications will be most beneficial in adding quality to your life. Porphyria does not have to mean the end of having a quality life.

John Danielson PhD, LSW

Being Aware of P-450 Drugs and Foods with Porphyria

As acute porphyria patients become more knowledgeable about their triggers it is important for them to look at foods as well.

Many of today's modern drugs are as listed as P-450 inhibitors. Acute porphyria patients must avoid all pharmaceuticals that are metabolized through the liver.

Approximately one fifth of all pharmaceuticals are detoxified by the cytochromes, and will thus place additional burdens upon the porphyrin related pathways.

Thus individuals with porphyria may be seriously harmed by doctors without specific training and information related to porphyria.
In addition some foods can be inhibitors of P-450

As acute porphyria patients become more knowledgeable about their triggers it is important for them to look at foods as well.

Grapefruit juice is listed as a P-450 inhibitor.

The inhibition of CYP 450 isoenzymes by grapefruit juice lasts about 24 hours and occurs in all forms of the juice - fresh fruit and fresh and frozen juice.

Caffeine greatly used P-450.

The ingestion of charbroiled meats may induce isoenzymes, resulting in increased clearance of drugs.

Smoking results in exposure to chemicals that induce cytochrome P450 enzymes and heme synthesis.

The benzodiazepines and sodium valproate are porphyrinogenic in experimental models of porphyria.

Most antiepileptic drugs are unsafe for porphyria patients because they have demonstrated porphyrinogenicity.

Porphyrinogens such as estrogens or alcohol, or other inducers of P450 isoenzymes provoke porphyria. Polymorphisms of P450 isoenzymes, iron overload and infection with hepatitis C virus play an important role in the etiopathogenesis of disease manifestation.

Rob Jamison PhD

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.

WELLBUTRIN 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.