June 2004 PES Bulletin

What Are The Opiods?

Opioids are prescription medications. These drugs are also regulated drugs. Opioids are classified as narcotics.
Such regulated drugs are termed "controlled substances". Controlled substances are regulated by the Drug Enforcement Administration.
A doctor must have a special license in order to prescribe these drugs.
A porphyria patient must have a prescription in order to acquire the opiods.
Prescriptions for opiods are also time limited, meaning that the patient will need to have a new prescription to get a refill as such drugs do not allow for automatic refills.
Opioids are often used to relieve severe pain such as the two main types of pain experienced in porphyria.
There are a variety of forms of opiods.
The basis of such drugs are now available in both the natural forms and in synthetic form.
The natural form is derived from the opium poppy. Such forms are called opiates.
There are also synthetic opioids which work closely in the same fashion.
Opiods that are regularly prescribed in the treating of both abdominal pain during acute attacks and the neuropathic pain that persists into periods of remission include the following:
Codeine
Fentanyl (Duragesic)
Hydrocodone
Hydromorphone (Dilaudid)
Levorphanol (Levo-Dromoran)
Meperidine (Demerol)
Methadone (Dolophine)
Morphine (MS Contin, Oramorph SR, others)
Oxymorphone (Numorphan)
Propoxyphene (Darvon, Darvocet)

Robin Lamberton RPH

Hepatocellular Cancer a Risk Factor

Hepatocellular carcinoma [HCC] is the most common form of primary liver cancer. HCC starts in the hepatocytes which are the main type of liver cell.
HCC can spread in different ways. In some HCC it can spread as a single tumor. This type of tumor can slowly spread throughout the rest of the liver.
Other HCC, especially associated with porphyria spreads throughout the liver immediately rather than being confined to a single tumor or as nodules.
In hepatic porphyria, a porphyria patient has a higher risk of HCC that the normally healthy population.
Some studies have indicated that there is a 67% increase in risk factor for acute intermittent porphyria patients [AIP] in developing hepatoceullar carcinoma [HCC].
In addition, among porphyria cutanea tarda [PCT] porphyrics, there is a higher risk of developing hepatocellular carcinoma if you have cirrhosis, hepatitis B or C, which is often associated with their PCT. Alcohol, tobacco and birth control pills are other risk factors for PCT patients in association with HCC.
Some HCC has been associated with exposure to industrial chemicals such as vinyl chloride.
The American Porphyria Foundation recommends that porphyria patients at risk be screened annually for HCC.
Through use of a simple blood test that checks for the presence of alpha-fetoprotein (AFP) porphyria patients can be screened for HCC. Other screening can be obtained through use of CT scans or MRIs.

Girard Weber PhD
Hematology

Adequate Potassium Vital in Porphyria
Potassium is a vital mineral. Potassium is one of many elements that make up the elctrolytes.
Potassium is one of the minerals responsible for maintaining the electrical stability of the cells of your cardiac system and the central nervous system.
Another vital function of the role of potassium is that of cell and muscle growth.
Potassium is an important factor in maintaining normal fluid balance. In acute porphyria attacks, fluid balance is crucial if there is nausea and vomiting, and/or diarrhea, and profused sweating.
Because many porphyria patients also experience edema or hypertension associated with their porphyria, many are prescribed diuretics. Some anti-hypertensive medications (diuretics) increase urination, which often lead to a potassium deficiency.
Couple potassium loss due to diuretics along with potassium loss due to nausea and vomiting or diarrhea and sweating and the patient can face dehydration and mental confusion.
In the acute porphyrias mental confusion presents with little warning and can range from general confusion, disorientation, to hallucinations, paranoia, insomnia, tremor, agitation and anxiety.
Interesting enough, research have found that low potassium may contribute to high blood pressure.
While in normally healthy persons, potassium deficiency is rare, in acute porphyria patients, care providers may prescribe potassium supplements if bloof serum potassium tests show diminished levels. Often such supplementation is due to the use of certain medications, mainly diuretics.
However, porphyria patients often are prescribed potassium supplementation piggy-backed with their glucose infusions during treatment for acute attacks in order to maintain a normal potassium level and counteract against loss of K through continuous vomiting and other fluid loss.
Porphyria patients are not to take potassium supplementation without direction from their care provider. It is vital that blood levels be routinely monitored on a regular basis and that the potassium be checked throughout any porphyic crisis.
For normally healthy people, eating a balanced diet is all that is necessary to get all the potassium they need. For the porphyria patient, one needs to have electrolytes periodically and routinely checked to avoid problems.

Robert Johnson M.D.
Internal Medicine
Retired Clinician

Cortical Aspects of the Porphyrias Cortical aspects of porphyria can be divided into those secondary to photosensitization and those related to neurological dysfunction.
The neuro-ophthalmological manifestations found in the porphyrias include optic neuritis, optic atrophy, cranial nerve palsies, retinal hemorrhages and ptosis.
In the porphyrias with skin manifetations, inflammation of the skin and eyelids can lead to vesicle and bulla formation, scarring, hyperpigmentation, and superinfection.
When the eyelids are affected by sun exposure, photosensitization of the eyelid skin can lead to erythema.
Vesicle or bulla formation can occur. When this happens, there can be scarring, hyperpigmentation, hypertrichosis, and superinfection.
Photosensitivity in the porphyrias affect the eyelids, the retina, conjunctiva, cornea, and sclera.
Conjunctiva Conjunctival photosensitization commonly is manifest as hyperemia and chemosis.
In the case of EPP patients, Purpura can also be observed.
In PCT patients there can be clinical findings of a diffuse purple-red "heliotropic" changes of the central face, most manifesting in the periorbital skin.
Lacrimation, and photophobia often are present.
Blepharospasm can occur.
Corneal ulceration, opacification, and vascularization has been noted.
In AIP, fifth cranial nerve paralysis is often known to occur. This can follow with corneal complications of hypoesthesia.
Retina Retinal findings in porphyria include retinal hemorrhages, cotton-woolspots, edema, chorioretinal pigment proliferation or atrophy, or well as a disc edema.

Shannon Mallory PA
Qphthalmology