From the Guidelines
Patients with Hereditary Coproporphyria (HCP) should avoid prolonged fasting and instead maintain a balanced diet, as fasting can trigger acute attacks by activating the heme biosynthetic pathway 1.
Key Considerations
- The most recent and highest quality study recommends that patients with HCP should be counseled to avoid identifiable triggers that may precipitate acute attacks, such as caloric deprivation 1.
- Fasting can upregulate ALA synthase, potentially precipitating acute porphyria attacks, so any fasting approach must prioritize metabolic stability over weight loss or other potential benefits.
- A conservative 12:12 schedule (12 hours fasting, 12 hours eating) may be considered, but patients should maintain consistent carbohydrate intake during eating periods and monitor symptoms closely before attempting more restrictive windows.
Management Recommendations
- Patients should work with both a hematologist experienced in porphyria and a nutritionist to develop a personalized plan.
- Morning glucose monitoring is essential, aiming to keep levels above 70 mg/dL.
- Patients should take their prescribed porphyria medications (such as hemin at 3-4 mg/kg/day during acute attacks) according to their regular schedule, regardless of fasting windows.
- A medical alert bracelet should be worn at all times.
- Patients should keep a detailed symptom diary correlating fasting periods with any neurological symptoms, abdominal pain, or skin manifestations.
Prioritizing Metabolic Stability
- The underlying concern with fasting in HCP is that reduced glucose availability can trigger acute attacks, so metabolic stability should be prioritized over weight loss or other potential benefits 1.
- Patients should be counseled to avoid crash diets and instead aim for gradual weight loss with the guidance of a dietitian, if necessary.
From the Research
Optimal Fasting Window for Hereditary Coproporphyria (HCP)
There are no specific studies that discuss the optimal fasting window for patients with Hereditary Coproporphyria (HCP). However, some general information about HCP and its management can be found:
- HCP is a rare acute hepatic porphyria that can be triggered by certain factors such as low caloric intake 2.
- Attacks of HCP can be precipitated by factors such as certain drugs, alcohol, infections, or low caloric intake 2.
- Management of acute porphyria attacks includes pain relief, elimination of triggering agents, and intravenous glucose administration for mild episodes 3, 4.
- Intravenous hemin is needed for management of moderate to severe episodes of acute porphyria 3, 4.
- Preventive treatment options include hormone suppression therapy, off-label prophylactic hemin, Givosiran, and exceptionally liver transplantation 5.
Triggering Factors
Some factors that can trigger HCP attacks include:
- Certain drugs
- Alcohol
- Infections
- Low caloric intake 2
- It is essential to avoid these triggering factors to prevent HCP attacks.
Management of Acute Attacks
Management of acute HCP attacks includes: