High triglycerides may point to FCS

Dear Dr. Roach: Recently I was diagnosed with FCS (familial chylomicronemia syndrome) by my primary physician. I have extremely high triglycerides and Type 2 diabetes. I have brought my A1C from 10 percent to 6 percent, as well as lost 40 pounds and completely changed my way of eating. I also walk.

Despite this, I have numerous bouts of acute pancreatitis every year. I am hospitalized at least every three months. I am extremely discouraged. During my last bout, my triglycerides were 10,000. Currently they are 1,200. Is there any hope of relief ? — T.R.

Familial chylomicronemia syndrome, also called Type 1a hyperlipoproteinemia, is a very rare disorder of fat metabolism caused by the loss of the enzyme lipoprotein lipase, normally made in the pancreas.

The major symptom is abdominal pain, and bouts of acute pancreatitis are common. Repeated episodes of pancreatitis can lead to damage to the pancreas and subsequent diabetes.

I am impressed with your primary physician. This diagnosis frequently is not made or is made incorrectly. It is confirmed with genetic tests.

Unfortunately, there is no current effective drug treatment available for the high cholesterol and high triglycerides of FCS. The only effective therapy available right now is a diet that is very low in fat, with almost no simple sugars or alcohol. Fats are taken as a medium-chain triglyceride oil.

You have done extremely well in dropping your triglycerides, but I understand you are still frustrated with persistent bouts of pancreatitis. Once people have had several bouts of pancreatitis, the damage done to the pancreas puts them at risk for further episodes, even if the underlying cause (triglycerides in your case, alcohol in some other people’s) is under better control.