September 2004 PES Bulletin

Delirium Often Present at Attack Onset

In the acute porphyrias mental change is usually present during the times of acute excerbation of the disease. One form of mental change that is often observed is that od delirium.
In delirium there is reduced ability to maintain attention to external stimuli and to appropriately shift attention to new external stimuli. Attention will wander.
It is very important for the care provider accompanying a porphyria patient to a clinical setting during an acute crisis to realize that the porphyria patient often can not stay focused and follow through on answering a lot of questions that are repeatedly asked by medical staff.
Often confusion will develop in just short time span. The porphyria patients will present with disorganized thinking. This is especially true when being asked to tell medical staff a long list of medications and for what they were prescribed.
If the porphyria patients does not begin timely intervention treatment for the porphyria the patients will progress to a reduced level of consciousness, or ANS (altered neurologic state).
Memory impairment and disorientation to place, time or person can take place. Often porphyria patients need to ask where they are if ANS has occurred or time in triage has been long.

Dr. Kenneth Carlson
Neuropsychiatric

Porphyrics at Greater Risk For HCC

Hepatocellular carcinoma (liver cancer) usually known as HCC, has an increased risk factor in acute intermittent porphyria patients.
Porphyria cutanea tarda patients are also subject to HCC but due more so to the associated cirrhosis, hepititus, and alcoholism.
Porphyria specialists are encouraging porphyria patients who are at high risk for HCC to have regular annual serum alpha fetoprotein, Phase 3 CT scans and ultrasound.
HCC has long been associated with AIP. Research studies have shown there AIP run a 67% greater risk of progressing to HCC.
Triggering factors of PCT are also associated with risk of HCC.
The most important risk factors for HCC include chronic infection with HBV or HCV which often accompanies PCT. Studies show that HBV infection causes 80 percent of cases of hepatocellular carcinoma.
Liver cancer can affect people of all ages and races, but certain factors may increase your risk of developing the disease.
Risks factors that can have a great impact on risk of HCC include:
Smoking
Alcohol consumption
Cirrhosis
Birth control pills
Arsenic exposure
Vinyl chloride exposure
Thorium dioxide exposure
CIrrhosis often develops in hepatic porphyria patients. Cirrhosis is a progressive and irreversible condition that causes scar tissue to form in y the liver. Because of such scarring it can increase your chances of developing HCC.
Smoking, alcohol consumption and use of birth control pills are contraindicated in porphyria patients, however for many such patients the use of these triggers before diagnosis of porphyria elevates their risk factor for HCC.
Today with ever increasing chemical toxins in our environment there will be more chances of exposure to elements which can put a person at risk for HCC.

Girard Weber PhD
Hematology

Using Narcotic Analgesics for Pain Control

Systemic narcotic analgesics are used to relieve pain. And often pain is "the name of the game" in managing the pain of porphyria.
Many physicians are relunctant to treat porphyria patients with narcotics, but porphyria patients do experience real pain, and that pain needs to be managed. Management of such pain requires the use of systemic marcotic analgesics.
The action of narcotic analgesics takes place in the central nervous system (CNS). The outcome of that action is to relieve pain.
Unfortunately some of the side effects of narcotic analgesics are also caused by the CNS. There is always a possibility of dependence on a drug if the drug is used for long periods of time.
If a narcotic is used for a long time, it may become habit-forming. When a drug becomes habit-forming it can cause a psychological dependence and sometimes a physical dependence.
When a physical dependence occurs, it may lead to withdrawal side effects when you stop taking the medicine. However it must be noted that such dependence is rare if the patient is well monitored by their care provider and variation of pain management are used.
Narcotic analgesics are available only with a prescription from your medical care provider. Depending of which drug you are being prescribed, prescriptions cannot be refilled and you must obtain a new prescription from your medical doctor each time you need the medicine.
The following are a listing of the most commonly prescribed narcotic analgesics.
Cotanal
Darvocet
Darvon
Demerol
Dilaudid
Dolophine
Duramorph
Hydrostat
Methadose
M S Contin
MSIR
MS/L
MS/S
Nubain
Numorphan
OxyContin
Rescudose
Roxanol
Stadol
Talwin

Robert Johnson M.D.
Internal Medicine
Retired Clinician

Visual Defects Found in Porphyria

Like a host of other manifestations in the porphyrias, visual problems can also present.
Many acute porphyria patients complain of "fuzzy eyes" from time to time.
Visual change is often noted during acute attacks and sometimes will remain during the periods of remission.
Some patients will develop optic neuritis, other optic atrophy.
Some patients have developed bilateral blindness after recovery from coma secondary to severe acute porphyria. Sight however nearly always returns.
Sometimes the sight is slow in returning to normal levels of vision. Gradual return of vision usually will take place however there may be a visual field defect or/and optic atrophy.
When persistant problems remain there may be a pathophysiologic mechanism, a spasm of the vessels supplying the optic disk leading to ischemia and infarction of the optic nerve. This is atypical generally. however optic atrophy has been observed in many porphyria patients.
Porphyria patients should take a few simple steps in always safeguarding their eyes from UV lighting, direct exposure to sun, glare from snow and bright reflections from bodies of water.
In the home study lamps should always be tilted away from direct control with the eyes.
During acute attacks, porphyria patients should always close shades, dim lighting and try resting the eyes. It is not a time to read or focus on things with fine detail. "Fuzzy eyes" are commonly experienced during times of acute attacks.
Taking a few simple precautions can help avoid eye problems associated with porphyria.

Shannon Mallory PA
Ophthalmology

PES Monthly Drug Update:
DISCLAIMER

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 judgment 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.

PHRENILIN is the brand name for the generic drug BUTALBITAL. This drug is classified as a sedative-hypnotic agent and anticonvulsant.The drug carries a WARNING if you have liver disease or have porphyria.
RECLOPMIDE is a brand name for the drug METOCLOPRAMIDE. Parkinsonian-like symptoms have occurred. Tardive dyskinesia can occur.Tardive dyskinesia, a syndrome consisting of potentially irreversible, involuntary, dyskinetic movements. Mental depression has occurred in patients with and without prior history of depression. Metoclopramide is listed on several UNSAFE drug lists for use by porphyria patients. It carries a warning for persons with liver disease.
GLUCOTROL is a brand name for the generic drug GLIPIZIDE It is a sulfonylurea. SIDE EFFECTS: Hypoglycemia, Gastrointestinal disturbances are most common reactions.Porphyria cutanea tarda and photosensitivity reactions have been reported with sulfonylureas. Skin eruptions rarely progressing to erythema multiforme and exfoliative dermatitis have also been reported. Metabolic REACTIONS - Hepatic porphyria and disulfiram-like reactions have been reported with chlorpropamide.WARNING:This drug is UNSAFE for porphyria patients