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Porphyria
How is it treated?
Acute Porphyrias: Avoiding precipitating factors; maintaining regular meals and a balanced diet is important and wide dissemination of prohibited agents is recommended. Avoid alcohol, starvation and unwarranted physical or surgical strain. Acute attacks can also be managed by the use of increased calorie intake either by mouth or parenterally, scrupulous attention to fluid balance is required for the management of hyponatraemia (due to syndrome of inappropriate anti-diuretic hormone release); safe control of intercurrent hypertension. In severe attacks parenteral administration of haem arginate is recommended which suppresses the haem formation pathway and reduces the biochemical abnormalities that underpin the acute attack. Seizure control requires special consideration since many conventional anti-convulsants are porphyrinogenic.
Acute attacks should be rapidly evaluated and hospital admission considered for assessment. In the patient with progressive acute disease, ventilation and intensive care therapy followed by active rehabilitation may be needed for paralysis.
An updated list of safe drugs is available from the Welsh Medicines Information Centre, University Hospital of Wales, Tel: 029 2074 3877 e-mail: welshmedicines.information@cardiffandvale.wales.nhs.uk. The Information Centre's list is available to patients and their health care professionals. Advice can be sought on the safe use of medicines for Porphyria.
See also MedicAlert, Web: http://www.medicalert.org.uk the Emergency Identification System for people with hidden medical conditions.
What are the symptoms?
| Inheritance patterns and prenatal diagnosis 
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