Medication and the pill for HPU

Porphyria – diseases of the haem synthesis recognized by orthodox medicine

Porphyria are serious diseases

Porphyria is also a disease of the haem metabolism. The different forms of porphyria differ in the step in the metabolism on the way from delta-aminolevulinic acid to haem, where the synthesis is disturbed.

In contrast to HPU, in porphyria one of the 8 enzymes required for haem synthesis is genetically defective. In HPU, however, only 3 to 4 steps of the haem synthesis are defective. The most serious symptoms of porphyria include

  • Abdominal cramps,
  • blister-like skin lesions with scarring, especially in light-exposed areas,
  • liver damage, which can lead to scarred liver (cirrhosis of the liver), and
  • a disorder of brain function (encephalopathy) with various psychiatric and neurological symptoms.

Certain drugs can cause severe relapses in people with porphyria by affecting certain enzymes (ALA and urodecarboxylase) of the haem synthesis pathway. Toxic intermediate products of the haem synthesis accumulate and sometimes cause severe neurological symptoms or extreme sensitivity to light. These so-called porphyrinogenic drugs are to be found in the “red list” of classical orthodox medicine. The list of porphyrinogens should not be taken or administered in acute forms of porphyria. Another list indicates the “safe” drugs for people with acute porphyria.

HPU is one of the toxic inducible porphyrias

Why does the HPUler have to be particularly careful when taking medication? There are two reasons:

1. HPU belongs to the toxic inducible porphyrias; this means that symptom outbreaks can be caused by heavy metals such as mercury, lead, cadmium and the light metal aluminium. As a precaution, long-term use of porphyrogenic drugs by people with HPU should also be avoided if possible.

“In general, if you take them for a few days, this is not a problem. But if you have to take a porphyrinogenic medication for a long time, it may very well be a problem”.

Dr. J. Kamsteeg, HPU and Dann, KEAC, 6th edition 2017

On the Porphyria Foundation website, active ingredients can be tested for their safety with regard to porphyria.

2. the HPUler is usually less able to detoxify than the general population. Since drugs are usually broken down or only activated via the liver, the main detoxification organ is always put under additional strain when taking a drug. With the HPUler, this can lead to an overload.
These drugs are considered safe

These drugs are considered safe
(without guarantee)

Analgesics and NSAIDs
Acetaminophen, acetylsalicylic acid, codeine, fentanyl, morphine and related substances, dihydrocodeine, paracetamol
Penicillins, cephalosporins, aminoglycosides, tetracycline
Cardiovascular products
Adrenalin, atropine, digitoxin, metoprolol, propranolol
Psychoactive drugs and sedatives
chloral hydrate, haloperidol, lithium, midazolam, nortryptiline, oxazepam, paraldehyde, promazine
Antihypertensives and diuretics
Amiloride, Atenolol, Ethacrynic acid, Propranolol, Reserpine, Triamterene
Aciclovir, atropine, cimethidine, dicumarol, glucocorticoids, heparin, insulin, neostigmine, pethidine, procaine, thyroxine, chlorpromazine

These drugs should be avoided by HPU patients (no guarantee)

Analgesics and NSAIDs
all NSAIDs except ASS, phenacetin, phenylbutazone, tilidine, pentazocine, tramadol, various antibiotics
chloramphenicol, doxycycline, metronidazole, sulfonamides, griseofulvin
Cardiovascular products
amiodarone, nifedipine, verapamil
Psychoactive drugs and sedatives
barbiturates, diazepam, meprobamate
Antihypertensive and diuretic drugs
Alpha-methyldopa, captopril, clonidine, furosemide, hydralazine, spironolactone, thiazides
ergotamine preparations, imipramine, estrogens, sulfonylureas, theophylline, tolbutamide, metamizole, initially generally all newly approved drugs

General handling of medication for HPU

HPU patients usually have a limited detoxification capacity. This can lead to HPU patients tolerating drugs less well. Since HPU patients react very differently to drugs, no general advice can be given on how to handle drugs.

In principle, the HPU user should carefully consider whether it is absolutely necessary to take a medication or whether the complaints can be alleviated by well-tried household remedies such as peppermint oil on the neck and temples for headaches or warmth, rest and a good tea for stomach ache.

HPU and the pill

Since women with HPU often suffer from hormonal and menstrual problems, gynaecologists are happy to offer the pill as a solution. The pill can certainly help to alleviate symptoms. For example, it can improve the appearance of the skin, make the cycle appear regular and relieve menstrual pain.

However, as the “pill” is considered a vitamin B6, vitamin B12 and folic acid predator*, among other things, its use is particularly counterproductive for women with HPU. This is because HPUers often have a mutation in the MTHFR gene, which makes it harder for them to methylate. Many hormonal complaints improve in the course of HPU therapy as a side effect even without the pill.

* Wilson, SM. et al. Oral contraceptive use: impact on folate, vitamin B₆, and vitamin B₁₂ status. Nutr Rev. 2011 Oct;69(10):572-83.