There is no cure for porphyria. Treatment is aimed at getting rid of or managing your symptoms.
Only drugs known to be safe in porphyria can be prescribed for treatment, as many medications, including common sleeping pills and antibiotics can trigger acute attacks. This underlines the importance of making others aware that you have the condition, and the specific form.
Treatment of acute porphyrias may include:
- stopping medications known or suspected to have triggered your symptoms
- medication to control pain. If pain is severe, opiate-based drugs such as pethidine or morphine are safe to be given. A sedative, chlorpromazine (thorazine) may be used to relax you and help you sleep. Similarly, rapid heart beat and hypertension can be helped with propranolol to slow your heart rate. Anti-epileptic drugs may be given to control convulsion or seizures
- oral and intravenous glucose (sugar) to help you maintain a high energy intake
- haem arginate, a medication that is a form of haem, given to reduce the need for your body to produce its own. Combined with glucose, this lowers porphyrin levels by reducing the formation of aminolaevulinic acid. It is used mainly for severe attacks and is likely to be given to you intravenously over several days. Haem arginate has a rapid effect and an improvement may be expected within one week.
Treatment of cutaneous porphyrias is aimed at reducing the amount of porphyrins in your body to help eliminate your symptoms. This may include:
phlebotomy, also called venesection, a procedure similar to that used for blood donation in which a quantity of blood (usually up to 500 millilitres) is drawn into a blood donation bag via a needle placed in a vein in your arm. It is carried out to reduce the amount of iron in your body. Phlebotomy is most commonly used for PCT.
blood transfusion, the opposite treatment to phlebotomy. This is used for CEP in order to treat anaemia, in which you lack enough red blood cells.
a low dose of chloroquine, a drug given in tablet form twice a week.
beta carotene, given daily for EEP. Made from the orangey-yellow pigment that gives many fruits and vegetable their colour, beta carotene is converted by the body into vitamin A, to promote healthy skin and eyes and to increase your skin’s tolerance to sunlight. It may also colour your own skin a slight orangey shade.
a bone marrow transplant may be required for people with CEP, where the abnormal gene involved may also affect the formation of bone marrow.