Porphyria is a group of inherited disorders in which affected individuals have a deficiency of certain enzymes which convert chemicals known as porphyrins into heme. Heme is a protein substance which is present in almost all body tissues but is most commonly found in red blood cells, bone marrow, and liver. A faulty and incomplete conversion of porphyrins results in a buildup of these substances in the body and causes symptoms such as abdominal pain, constipation, vomiting, muscular pain, high blood pressure and an increased heart rate. Cutaneous manifestations of the disease result in itching and skin rash. Severe manifestations of this condition result in convulsions, confusion and hallucinations.
Acute intermittent porphyria can be precipitated by medications, fasting, smoking, infections, surgery, stress, menstrual hormones, and excessive use of alcohol or exposure to the sun. This results in intermittent and acute presentation of the above mentioned symptoms usually requiring hospitalization and intensive care. Such patients are treated using intravenous glucose to maintain adequate carbohydrate levels and achieve rehydration, A medication known as hematin, if available, is used to convert the excess porphyrins into heme, thus resulting in reduced symptoms. The buildup of porphyrins in the body can also be reduced by phlebotomy (removal of blood from a vein) and the use of activated charcoal which combine with porphyrins and help eliminate them from the body.
While patients can be managed conservatively in hospital for the acute presentation, there is no long-term cure for this condition. Ayurvedic treatment can be used safely and effective in the management of porphyria-like conditions. The presenting symptoms in affected individuals are interpreted in the light of Ayurvedic pathophysiology and treated accordingly. Most such symptoms correspond to the disease known in Ayurvedic terminology as Adhog (going downwards) Raktapitta, with symptoms predominantly caused due to a joint dysfunction of Vata and Pitta doshas. Ayurvedic treatment is therefore aimed at treating Vata dysfunction and reducing excessive Pitta. Mild herbal medicines which have a digestive and anti-spasmodic action are used in the stage of acute presentation of symptoms. Long term treatment includes the use of herbal medicines which have a Pitta-reducing and mild laxative action. Panchkarma procedures which include full body massage and light fomentation and basti (enema) using medicated oils are also highly effective in treating as well as preventing acute episodes of this disease.
Depending upon the presentation of symptoms, medicines also need to be given to treat and prevent damage to the liver, kidneys, as well as the nervous system. Ayurvedic herbal treatment needs to be taken regularly for periods ranging from six months to a year, depending upon the severity of the condition and associated complications. Once the patient achieves a complete remission of symptoms, medicines can be gradually tapered and then stopped altogether. The frequency of Panchkarma procedures can also be gradually reduced proportionately. Overall, most patients need to be monitored for a couple of years in order to prevent further attacks and/or relapses of the condition. Diet control is very important in preventing or aggravating attacks. Adequate modifications in lifestyle also need to be strictly implemented. All the known precipitating factors for this condition need to be avoided in order to prevent acute episodes and relapses. Ayurvedic herbal treatment, combined with preventive measures, can thus go a long way in successfully treating acute porphyria as well as managing the medical condition on a long-term basis.
by Abdulmubeen Mundewadi