Summer 2006 PES Bulletin

Porphyria Pain and Use of Opiod Analgesics

Opioid analgesics are centrally acting agents. These opiods provide fast pain relief by either binding or blocking opiate receptors in both the brain and the spinal cord. An agonist effect is known as binding. The blocking effect is known as an antagonist effect.

Opiods are also known as narcotics.
Opiods can play a role in the management of som chronic pain conditions and this includes many of the acute hepatic porphyric pain.
It is thought by many chronic pain specialists that non-addictive personalities of porphyria patients who use the opiods specifically for their analgesic effect have a very low possibility of addiction. Those patients however who use such opiods because of the their euphoric effects have a far greater possibility of becoming addicted to such drugs.

Nonetheless, because of the social stigma and also in many places the legal issues that focus on the chronic use of opiods, there continues to be a barrier to both the patient's ability to comply and the physician's ability in prescribing. For these reasons non-narcotic analgesics are often preferred as the first-line of therapy for porphyric patients for their often chronic pain.

Beverly Thompson MNS
Pharmacology Department

Electrolyte Balance Essential in Acute Porphyrias

"Electrolyte": A substance that when dissolved in water conducts an electric current. And what does this have to do with a porphyric? What makes up the electrolytes? The normal electrolytes include sodium, chloride, potassium and to some degree calcium. When a porphyric loses their electrokyte balance small electric shocks sent through the nervous system signal changes ahead.

Many of these changes exascebate in changes in our mental well being. They are also thought to contribute to seizure activity, and muscle spasms.
Various disturbances of fluid and electrolyte balance are seen during the acute attack.Dehydration may occur, owing to persistent vomiting. Hyponatraemia, secondary to inappropriate antidiuretic hormone secretion, may also occur, sometimes first becoming apparent after commencing intravenous fluids.
Hyponatraemia can usually be controlled by restricting fluid intake.
To maintain adequate carbohydrate intake while restricting fluid intake, it may be necessary to use higher concentrations of glucose, administered via a central venous line.
When beginning the onset of an acute attack of porphyria it is important to safeguiard one's self by avoiding the nausea and vomiting by the use of safe suppositories to avoid the loss of them. The most commonly used and known as safe for porphyrics is that of compazine.

Potassium is another factor and if potassium is falling in lab tests then an added bag of iv potassium along with the glucose can be administered.
When in remission potassium can be easily restored to proper levels by the oral intake of potassium supplements as prescribed by your physician.
Also it well for a person to drink water often to avoid dehydration.
Keep your body well hydrated - in the hot summer months as well as in the dry heated air indoors in the winter. Drink often. It is better to drink 8 - 8 oz glasses of water throughout the day than to drink three or four 15 - 20 oz glasses. The fluid will stay in your body better.
Furthermore, If you are chronically dehydrated, it may take a week of "forcing" fluids before you notice a difference in your hydration. Until then you may just find yourself going to the bathroom more often. On average, however, a person should drink eight 8 oz. glasses per day (about 2 quarts).

An overweight person should drink and additional glass for every 25 pounds of excess weight; those who exercise or are experiencing hot or dry weather should increase their intake as well.

Water is best cold, but not ice water. Cold water instead of ice water is good not only for taste, but the cold water is adsorbed into the system more rapidly.
Electrolytes are very important to porphyric patients and they should be sure to have them checked regularly and at the beginning of any acute porphyric attack in order to avoid the unnecessary mental changes and other neurological changes that can occur.

Elizabeth Reuter NP
Infusion Therapy

Using NSAIDS in Treating Porphyria Pain

NSAIDS: The Non-Narcotic Analgesics NSAID reduce inflamation and relieve pain by affecting arachidonic acid metabolism.
While the NSAIDS are used safely and effectively by millions of people they are often associated with adverse effects, particularly in patients who are in high-risk groups, including porphyrics.
GI complications are the most common adverse efect of NSAIDS.
One of the safest NSAIDS is Acetaminophen [Tylenol].

Beverly Thompson MNS
Pharmacology Department

Porphyria Cutaneous Tardea and Blistering

In the PCT type of porphyria the most common skin problem is that of vesicles which are otherwise known as blisters.
A vesicle is a thin-walled sac filled with a fluid. The fluid is usually clear and ranging in size from pinpoint to 10 millimeters in diameter. As a rule, the term vesicle is usually used to describe small blisters, while the term bullae is used to describe larger blisters.
In addition the vesicle is an important term used to describe the appearance of many rashes that typically consist of or begin with tiny-to-small fluid-filled blisters.
Typical illnesses beside porphyria cutaneous tarda that begin with vesicular eruptions are cold sore (herpes labialis), genital herpes, shingles (herpes zoster), and chicken pox.
These have the appearance of area or patches or otherwise called crops of vesicles. Because of the similar crops of vesicles it sometimes makes a diagnosis of PCT hard when based upon' the skin appearance alone.

Contact dermatitis may first show up with tiny vesicles that itch or burn. A typical example of contact dermatitis would be something like poison ivy. It begins with tiny vesicles that enlarge rapidly, rupture, ooze for a period of time, and finally crust over and heal.
In PCT these eruptions often lead to massive scarring.
In PCT the skin remains thin and fragile and will easily break.

Josie Revere NP
Dermatology

Analgesics Used for Treating Porphyria Pain

Analgesics are presecribed for giving effective pain relief in the acute porphyrias. At least that is the theory. Often porphyrics still endure a life of ongoing pain.

Analgesics can be classified by the site of their action.
There are three types: [1] centrally acting; [2] peripherally acting; and [3] locally acting.
The centrally acting analgesics include both opiod analgesics and non-narcotic agents such as Tramadol a.k.a. Ultram.

In porphyria we must avoid the Tramadol/Ultram non-narcotic pain medication because of the side effects of seizures and being noted by some as a "trigger" for acute attacks. It is also contraindicated with the use of some medications for seizures, muscle relaxants, pain and nausea medications.

It must be said that no one drug is perfect. Every drug known has their benefits and at the same time has some risks involved with its use.
Healthcare medical providers must make a determination which drug to use in any given situation.
Often drugs are given to a patient and the medical provider then assesses the individual p[atient's reaction to a specific drug.
With the porphyric patient, it is much better to use drugs that have been approved for a period of no less than five years.
The reason for this is that it takes a couple of years to assess the general problems with any new pharmaceutical product. Porphyrics by the very nature of their disease need to be ever mindful of the use of drugs and double check all information on any drug prescribed for them whether it be oral, suppository, injection or intravenous.

The majority of drugs on the market today are newer drugs, and each years countless numbers of new drugs and especially drug samples are left with medical care providers to give to patients to try out.
Be care of such medications. Ask for and demand to use pharmaceuticals known to be safe for porphyrics.
Even then, because each person is different and has different sensitivities, a "safe" drug can not be tolerated by everone. Regardless of whether one uses a non-narcotic, an opiod, or NSAID, and informed decision making in the prescribing of such drugs requires an understanding of the pharmacology, efficiacy and more importantly, the safety profile of these agents.

Every porphyric patient should familiarize themselves with the Drugs List by Dr. Michael Moore. In addition to his endless list of drug names, one would be advised to state the variables of each drug name, whether it be the generic, brand, trade or classification name of a drug.
BuSpar for instance is known as busprione. It does not appear on any drug list, unsafe or safe. However the classification is such that one would refuse to take the drug. BuSpar is an antianxiety drug, a sedative.Most drugs dealing with the mental abnormalities such as anxiety, or insomnia are unsafe for porphyrics. For pain associated with inflammation an NSAID is more often prescribed.

Beverly Thompson MNS
Pharmacology Department