Maple syrup urine disease

Maple syrup urine disease (MSUD for short) is a rare inherited metabolic disease. This is a condition that affects the body's ability to break down branched chain amino acids, particularly leucine such as leucine, isoleucine and valine. If left untreated these amino acids accumulate which may result in irreversible neurological impairment and death. Treatment involves maintaining an appropriate low-protein diet and monitoring the level of amino acids in the blood. Dietary management aims to prevent the accumulation by using a low protein diet. Protein requirements are supplemented with a leucine, isoleucine and valine free amino acid mixture (protein substitute / supplement), although it is sometimes necessary to take additional valine and isoleucine. Therefore during their travels people diagnosed with MSUD must carry on board and/or checked luggage:

• Special protein substitute formulas and/or vitamins (liquid, powder or pills)
• Low protein foods
• Pharmaceutical products dedicated to MSUD treatment