Safe/Unsafe drugs

Treatment anecdotes

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Fields of Study

Administrators (Hospital), Anaesthesiology, Biochemistry, Biology, Chirality, Community nursing, Environmental studies, Enzymology, Epidemiology, Evolution, Gene therapy, Genetic counselling, General Practice , Geography, Haematology, History, Journalism, Molecular Physiology, Neurology, Neuroscience, Oncology, Paediatrics, Pain therapy, Pharmacology, Physicians, Psychiatry, Veterinary Science, And finally...

Administrators (Hospital):

If haem-arginate[11,12] can save hospital-bed days why is it not used more often in Australia ?

Anaesthesiology:

Twelve hours after an anaesthetic, a patient goes into extreme pain and agitation. It can progress to paralysis sometimes reversible, sometimes leading to death. . . But People with Porphyria need operations too.

Biochemistry:

The red blood cells, erythrocytes, are 8um in diameter and each contains about 280x10⁶ molecules of haemoglobin[13].(Each haem is a protoporphyrin nest with an Iron in the middle)

Biology:

Is it the glucose treatment effect[14](see Physicians) a similar process to the fundamentals of energy synthesis/catabolism in the same way as a plant makes & uses starch?

Chirality:

Are porphyrins involved in the “new carbon dating”? Building porphyrins involves a chain of six, then four make a ring, one doing a flip[15]. (Adding an iron atom for haem or a magnesium atom for chlorophyll[16]).

Community nursing:

Invent a program akin to cardiac coaching to assist people to live with a prevention regime that can be more disciplined than diabetes.

Environmental studies:

Is Porphyria becoming more prevalent as there are more triggers in the environment?[17] Is acquired Porphyria a canary effect[18], and raised porphyrins a toxicity marker[19]?

Enzymology:

Is it possible to find replacement enzymes for the deficient 'synthesases' for all of the Porphyrias?

Epidemiology:

A grandfather who has 60 known descendants - none have been tested, 50% are at risk of Porphyria. With over 200 medication triggers[20] are they in 'clear and present danger'?

Evolution:

Are People with Porphyria the nocturnal of the human species? (Insomnia) Or an adaptation to the Scandinavian twilight? (Severe reactions to light.) Were porphyrins around in primordial times [21]? (chlorophyll 2.8 billion years ago, haemoglobin 400mya)

Gene therapy[22]:

Is Porphyria one of the “easy ones” with a single coding error on one of identified chromosomes (9, 10 or 11)[23] with biochemically measurable benefits? (Is it T to A for Hereditary CoproPorphyria?[24, 25]) But with Acute Intermittent Porphyria “ nearly every family has its own mutation”.[26, 27]]

General Practice:

If the acute porphyrias affect one in 10,000 of the population in the United Kingdom[28] and the French have a 1 in 1700 chance of inheriting the gene for Acute Intermittent Porphyria[29] should an Australian doctor expect to meet a patent with Porphyria at least every few years. Testing for Porphyria is cheaper than a colonoscopy.

Genetic counselling:

Never underestimate the power of denial[30]. Even though 1 in 100 attacks are still fatal and the potential pain is some of the worst known, extended family members decline testing.

Geography:

Read about the founder [33] population of people with Porphyria Variegata in South Africa[35] , DNA attributed to just two fertile Dutch immigrants

Haematology:

Slow video a bruise and hit reverse to see the colours of porphyrin synthesis.

History:

If haem-arginate had been available to George III[35] would there have been a regency crisis? Would America still be British? Trace Porphyria though that genetic bottleneck, European royalty[36]. Did Queen Victoria have Porphyria, passing it on to 50% of her large family, including the last Czarina?[37 , 38]

Journalism:

Did the current Queen’s uncle Johnny have seizures from it?[39] Did her father die from Porphyria induced lung failure? And did her sister Margaret have it? Is Charles[40] afraid of it[41]?

Molecular Physiology:

Do the eight different porphyrias make research complicated … or are they built-in effect controls? Which have skin presentations and which have acute crises? What is the role of melanin?

Neurology: [42]

Think of it along with other disorders: Parkinson’s, Guillain Barre[43] , MS [44] … and also Porphyria[45].

Neuroscience:

MRI reveals multiple reversible cerebral lesions in an attack of acute intermittent Porphyria. Both the cerebral clinical abnormalities and the MRI lesions resolved following treatment. [46 , 47]

Oncology:

Injecting tumors with porphyrins and then zapping them with light . . .[48, 49, 50]

Paediatrics:

Is it a myth that most Porphyrias don’t present till puberty?[51] Bactrim and most anti-seizure[52] drugs are triggers. Would it be possible to population-screen for Porphyria in infants? Cost effectively?

Pain therapy:

Episodic, intense and extreme pain, when currently the only safe pain relief is the opioids. Provided an attack of Porphyria is treated effectively the danger of addiction is considered minimal.[53]

Pharmacology:

Temazepam is on the Safe List for Porphyria and Valium on the Unsafe List[54, 55, 56] – the role of molecular shape and cytochrome P450. [57]

Physicians:

Some Porphyrias can be treated with intravenous glucose10%[58], sometimes giving relief within two hours. BSL’s go up at first then drop to normal. How does it work?

Psychiatry:

Tishler [59] and others said Porphyrics were over-represented in psychiatric hospitals … until someone tested a healthy cohort of blood donors . [60]

Veterinary Science:

Apparently cats get Porphyria[61] ,[62]. Do dogs? Mice can be induced porphyric.

And finally:

Urine that can change colour when left in the sun. Faeces[63] or teeth[64], which can glow under UV lamp. Why is the colour purple the worst?