Proper Management of FCS is Critical

While there is research underway regarding potential therapies, no FDA-approved cure or treatment currently exists for FCS.² Drugs used to lower triglyceride levels, such as fibrates or niacin, work in part by targeting LPL activity. In FCS, LPL does not function, making these drugs ineffective.^1,22-25 In addition, enzyme replacement therapy is not a therapeutic option for FCS due to the short half-life of the LPL protein (approximately 15-30 minutes).³²

Currently, the standard of care for patients living with FCS is to maintain a strict, life-long diet that is extremely low in fat (<10-15 g/day; a single tablespoon of olive oil has 12 g of fat) and restricts the intake of simple carbohydrates and alcohol.^{24, 26-28} Even with strict dietary supervision, however, the results of a recent study show that 67% of patients with FCS being treated by the foremost lipid centers still experience episodes of acute pancreatitis.³³

Women with FCS may face serious additional risks. Women of childbearing age with FCS face an increased risk of acute pancreatitis, miscarriage, and maternal fatality ³². Triglyceride (TG) levels can increase due to contraceptive use of estrogen and due to pregnancy where a 2-3 fold increase in TG is normal course in the third trimester.

People with FCS are encouraged to work with a registered dietician to support the lifelong dietary management of FCS that is extremely low in fat, low in simple carbohydrates, limits total carbohydrates, eliminates alcohol, and meets requirements for essential fatty acids and other nutrients. A dietician may recommend the use of MCT oil in food preparation, for instance. Medium chain triglyceride (MCT) oil is a safe fat source and source of calories for FCS patients and should be obtained by prescription. When consumed, MCTs are rapidly hydrolyzed and removed through the portal circulation to the liver and are used as a source of energy. Therapy to address psychosocial concerns (eg, depression/anxiety) is also recommended.²⁹