Dietary advice for patients on GD-specific treatments

Introduction

Eliglustat, an SRT available as first-line treatment for selected adult patients with type I GD, is usually well tolerated; however, it is mandatory to assess the concomitant use of medications, herbal supplements, and fruits that might affect cytochrome P450 (CYP) 2D6 and CYP3A metabolism and thus alter eliglustat plasma levels (9). The SRT miglustat (indicated as second-line therapy for some adult patients with type I GD) is frequently associated with gastrointestinal disturbances such as diarrhea, flatulence, abdominal pain or discomfort, and weight loss (10).

Evidence

Guidelines recommend which medications and food could interfere with eliglustat treatment (9) . Many patients do not tolerate miglustat for gastrointestinal adverse effects; these adverse events (AEs) can be reduced by limiting patients’ intake of disaccharides and a specific diet. There are no particular diet recommendations and dietary restrictions when using ERT.

Recommendations

The panel recommends following the guidelines of which medications and food could interfere with eliglustat treatment.

Eliglustat-treated patients should be advised to restrict or avoid grapefruit products, pomegranate, carambola (star fruit), bitter orange, licorice, and herbal products (9). Herbal supplements and over-the-counter self-medications may interfere with eliglustat metabolism and should be reported to the treating physicians for advice. Regular information to patients regarding compatible medications and supplements is recommended.

For patients treated with miglustat, the panel suggests monitoring for abdominal symptoms and vitamin deficiencies to provide dietary advice when needed.

Miglustat-treated patients should restrict their intake of disaccharides or carbohydrates before initiating therapy. Bloating and diarrhea may be frequent complications during miglustat treatment. Monitoring the effects of spicy foods and dietary fats on symptoms may help to find the best-tolerated diet. Testing for lactose intolerance is advised. Ensuring an adequate intake of non-caffeinated, non-carbonated fluids may help. Short-chain fermentable carbohydrates increase small intestinal water content and colonic gas production, worsening GI symptoms. In some instances, the low fermentable oligosaccharide, disaccharide, monosaccharide, and polyol (FODMAP) diet may benefit patients.

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