Porphyrias: Therapy

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