Special Considerations
The Glucose Effect in the Acute Porphyrias
 The disorders Acute Intermittent Porphyria (AIP), Hereditary Coproporphyria
(HCP), Variegate Porphyria (VP) and ALAD porphyria (ADP) are treated
initially with the administration of carbohydrate/glucose. This therapy
has its basis in the ability of glucose to decrease porphyrin biosynthesis
in the liver.
Glucose can diminish excess excretion of heme precursors, which,
in turn, can prevent an attack or can hasten recovery from an attack
of the acute porphyrias. Therefore, it is suggested that when patients
cannot consume carbohydrates due to nausea or vomiting, glucose should
be administered intravenously. Some physicians have prepared a standing
order for patients who are prone to attacks to help facilitate intravenous
glucose in the emergency room. Often this prevents further hospitalization.
Most patients are knowledgeable about the deleterious affect the
wrong drug can have on their bodies and are consequently careful
about the medication they ingest, but some do not understand the
importance of carbohydrates for prevention and treatment of a porphyria
attack.
Because it is a simple therapy, many patients ignore the significance
of their carbohydrate (sugar) intake to suppress disease activity.
When The Dr. Atkin's Diet, which was a high protein/low carbohydrate
diet, became popular a number of years ago, many porphyria patients
who adhered to this diet became ill. It soon became apparent that
their severely reduced daily carbohydrate count exacerbated their
porphyria. In fact, complying with the Atkin's plan precipitated
attacks in some previously undiagnosed and non-symptomatic individuals.
Please note that the Diet and Nutrition section
provides information to assist you, your physician and your dietitian
in preparing and maintaining a healthy diet that includes the amount
of carbohydrates suggested for porphyria patients. It has also proven
helpful to patients who want to lose weight without jeopardizing
their health.
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