Signs and Symptoms of FCS

Symptoms of FCS can vary widely but may include:

• Severe abdominal pain: Often associated with pancreatitis, a potentially life-threatening condition.
• Fatty deposits in the skin: Known as eruptive xanthomas, these are small, yellowish bumps often found on the buttocks, arms, and legs.
• Memory and concentration issues: “Brain fog” is common in patients living with FCS.
• Enlargement of the liver and spleen due to fat buildup.
• Chronic tiredness and weakness are often reported by those with FCS.

Treatment and Management

While there is currently no cure for FCS, treatment focuses on managing symptoms, reducing triglyceride levels, and preventing complications. FCS management can include:

• Dietary management: A very low-fat diet (often below 10-15% of daily calories) is the cornerstone of FCS management. Foods high in fat can increase chylomicrons, so individuals with FCS often avoid foods like oils, nuts, and fatty fish. In patients with FCS, there is no such thing as “Good Fat,” as fat is fat.
• Medications: Fibrates, omega-3 fatty acids (must be included in your daily fat allowance), and niacin are sometimes prescribed, though their effectiveness in FCS is limited. Currently, Redemplo and Tryngolza are the only FDA-approved prescription medicines for adults with FCS in the United States.  Please follow the links for information on Tryngolza and Redemplo. Please discuss with an FCS professional before seeking medication.
• Regular monitoring: Patients need routine blood tests to monitor triglyceride levels and check for signs of pancreatitis or other complications.
• Yearly eye exams are needed to ensure the vessels in your eye and optic nerve are not being negatively affected.

Dietary Management of FCS

• High triglyceride levels are managed by eating an extremely restrictive, low-fat diet (10-15% of total caloric intake). People living with FCS may eat vegetables, fruits, whole grains, egg whites, legumes, fat-free dairy products, seafood, and lean poultry.
• People with FCS can obtain essential fatty acids with supplements that include fat-soluble vitamins (vitamins A, D, E, K), minerals, and medium-chain triglycerides, as needed. Eating small, frequent meals that contain fat-free or low-fat protein is suggested.

• Know Your TGs – FCS-Friendly Recipes
• Learn Your Lipids – Low-Fat Cookbook
• Action FCS – Recipes
• FCS Foundation Recipes

Lifestyle Tips for Living with FCS

Managing FCS involves both lifestyle and dietary adjustments. Below are some helpful tips:

• Maintain a strict low-fat diet (as mentioned above)
• Stay hydrated: Adequate water intake helps reduce blood viscosity.
• Engage in gentle exercise: While intense exercise may increase triglyceride levels, moderate activities like walking or yoga are beneficial.
• Manage stress: Stress can exacerbate symptoms, so practices like meditation and deep breathing exercises can be helpful.
• Support (group or 1:1)

Causes of FCS

FCS occurs when the enzyme lipoprotein lipase (LPL), which breaks down triglycerides, doesn’t work well or is missing. This can be caused by mutations in the LPL gene or in other genes that help LPL function properly.

• Without lipoprotein lipase, triglycerides (a type of fat) build up in the blood. These triglycerides are mainly transported in dietary lipids called chylomicrons. In people with FCS, chylomicrons stay in the blood instead of breaking down, causing them to accumulate. This buildup can block blood flow to the pancreas, triggering acute pancreatitis. Acute pancreatitis can cause serious damage to the pancreas, be life-threatening, and lead to other symptoms and complications.

Diagnosis of FCS

The process of diagnosing FCS can include:

• Blood tests: Measures extremely high triglyceride levels (often over 880 mg/dL) and confirm the presence of chylomicrons. When blood is drawn, the plasma may have a milky appearance due to excessive lipids.
• Lipid profile: Helps rule out other lipid disorders and narrows down diagnosis.
• Early diagnosis is crucial to managing symptoms and reducing the risk of complications.
• Genetic testing: Identifies mutations in genes related to FCS. Key genes associated with FCS:
  • LPL: Code for the enzyme lipoprotein lipase, responsible for breaking down triglycerides.
  • APOA5, GPIHBP1, APOC2, and LMF1: Mutations in these genes can also contribute to FCS.

Risks of FCS & Care Team

It is crucial for patients managing FCS to have a diverse care team in place because:

• Repeated episodes of acute pancreatitis can lead to chronic pancreatitis and signs of exocrine or endocrine pancreatic insufficiency, including pancreatic (type 3c) diabetes.
• Patients with extremely high levels of triglycerides may have a more severe case of pancreatitis, leading to worse outcomes, including but not limited to longer hospital stays, a higher rate of pancreatic necrosis, more frequent, persistent organ failure, and higher mortality rates.
• This risk may be lowered by treatment with a healthcare team that understands all aspects of the disease, which may include a lipidologist, pancreatologist, gastroenterologist, primary care physician, registered dietician, psychologist, and/or social worker.

FCS Resources

• • FCS Foundation: Living with FCS is the site for all FCS patients, caregivers and family members that offers information, resources and support.
  • Action FCS: Action FCS offers resources for patients and caregivers to learn more about FCS.
  • Animated Pancreas Patient: The NPF’s Animated Pancreas Patient offers short, easy-to-understand animations with audio narration, expert video explanations, patient interviews, slide shows, and downloadable information on major pancreatic disease topics. This resource offers expert advice to help you discuss key issues with your healthcare provider and make important decisions related to management and treatment of pancreas disease.
  • EatRight.org:  The Academy of Nutrition and Dietetics offers information on nutrition and health, from meal planning and prep to choices that can help prevent or manage health conditions and more.
  • Know Your TGs: Ionis has created a free suite of tools designed to ease the diagnosis, management, and understanding of FCS and the lifestyle it requires. Their helpful resources provide insight into FCS, pancreatitis and diet and lifestyle, in addition to downloadable resources, including the Patient Guide to FCS, the FCS CareBook, and the FCS Nutrition Toolkit. Download available resources HERE.
  • Learn Your Lipids:  Learn Your Lipids is the foundation of the National Lipid Association. It is a non-profit focused on providing education and resources to help patients and their families manage and overcome lipid-related health problems that may put them at risk for a heart attack or stroke. They aim to translate scientific and medical progress into effective evidence based guidance for optimal patient care. The Foundation’s mission is to improve the welfare of patients and families affected by cholesterol and triglyceride problems. Their website provides resources for FCS patients and families.
  • Lower My TGs: Arrowhead Pharmaceuticals has created a patient and caregiver disease state website that serves as a comprehensive platform offering resources, education, and patient stories. Patients can learn more about lowering Triglycerides to reduce acute pancreatitis attacks. Read their “Spotlight on FCS” paper HERE.
  • National Lipid Association: The National Lipid Association website is a resource dedicated to promoting the understanding and management of lipid disorders and cardiovascular health. It serves healthcare professionals, patients, and researchers with: educational resources, patient care information, and research and advocacy.

FCS Patient Education

“Pull up a chair in our virtual living room for honest, supportive conversations with people who understand what it means to live with FCS. In this peer-led video series, patients, caregivers, and advocacy leaders 
share practical tips, everyday strategies, and personal stories—offering tools and perspectives to help you feel more informed, connected, and empowered on your FCS journey.”

Together, we’ll explore topics like:

• Building your FCS care team and communicating effectively with healthcare providers
• The importance of understanding FCS to become your own FCS expert and advocating for your needs
• Managing mental health, stress, and the emotional realities of FCS
• Navigating dining out, finding food ideas, and managing social settings
• Finding community, peer support, and trusted resources for living with FCS

This educational resource is provided by Arrowhead Pharmaceuticals as a part of their commitment to supporting the FCS community through shared experiences and trusted information.

Approved Therapies, Emerging Therapies and Research Studies

• • Tryngolza: In December 2024, the U.S. Food and Drug Administration (FDA) approved Tryngolza (olezarsen), used with diet, to reduce triglycerides (TG) in adults with FCS. Tryngolza is injected under the skin one time per month and it works differently in the body than other therapies currently used to treat FCS
  • Redemplo: In November 2025, the FDA approved Redemplo (plozasiran) used together with a low-fat diet to reduce triglycerides (fat in the blood) in adults with familial chylomicronemia syndrome (FCS). Redemplo is the first and only FDA-approved siRNA medicine for people living with FCS and can be self-administered at home with a simple subcutaneous injection once every three months. In the registrational clinical study (PALISADE), Redemplo achieved deep and durable reductions in triglycerides, with a median change from baseline of -80% versus -17% in the pooled placebo group, and a lower numerical incidence of acute pancreatitis compared with placebo. To read more about the PALISADE trial, please click HERE

FCS Patient Stories