Causal therapy does not exist for either the acute or cutaneous forms of porphyria.Therapy in reducing the risk of an attack or episode.
General measures
- For acute porphyrias during an episode:
- Move affected person to a darkened and quiet room.
- Intensive medical monitoring may be required because of the risk of respiratory paralysis!
- In the context of cutaneous porphyrias, the sun should be avoided if possible:
- Sun protection by clothing: long-sleeved and tightly woven clothing, closed shoes, gloves, headgear.
- Use special sunscreens → based on titanium dioxide and zinc oxide, so protection from UVA and UVB rays and visible (blue) light is possible; Note: Normal sunscreens are unsuitable, as they do not absorb the blue portion of visible light!
- Alcohol restriction (abstain from alcohol consumption).
- Review of permanent medication due topossible effect on the existing disease.
- Avoidance of psychosocial stress:
- Stress
- Avoidance of environmental stress:
- Lead → lead poisoning
- Organic solvents, such as those found in painting businesses and dry cleaning.
- Toxic chemicals (hepatotoxic/liver damaging).
Vaccinations
The following vaccinations are advised, as infection can often lead to worsening of the present disease:
- Flu vaccination
- Pneumococcal vaccination
Regular checkups
- Regular medical checkups
- Regular liver screening and sonography (ultrasound examination) of the liver in acute porphyrias and age > 50 years due to increased risk of hepatocellular carcinoma
Nutritional medicine
- Nutritional counseling based on nutritional analysis
- Nutritional recommendations according to a mixed diet taking into account the disease at hand. This means, among other things:
- Daily total of 5 servings of fresh vegetables and fruits (≥ 400 g; 3 servings of vegetables and 2 servings of fruits).
- Once or twice a week fresh sea fish, i.e. fatty marine fish (omega-3 fatty acids) such as salmon, herring, mackerel.
- High-fiber diet (whole grains, vegetables).
- Observance of the following special dietary recommendations in the context of acute porphyrias:
- High-carbohydrate diet in the latency phase (phase with no or only minor symptoms) – the risk of an attack can be reduced
- Avoid starvation states – pay attention to regular eating; perform diets only in phases of remission (temporary (transient) or permanent subsidence of disease symptoms) and aim for slow weight reduction
- Carry glucose – at the onset of abdominal pain (abdominal pain) can avoid critical symptom development
- Selection of appropriate food based on the nutritional analysis
- See also under “Therapy with micronutrients (vital substances)” – if necessary, taking a suitable dietary supplement.
- Detailed information on nutritional medicine you will receive from us.
Physical therapy (including physiotherapy)
- Preventive phototherapy (in spring): the skin becomes more tolerant by irradiation with artificial UV light several times a week.
Psychotherapy
- Stress management, if necessary
- Detailed information on psychosomatics (including stress management) can be obtained from us.