Local man raises awareness of rare disease familial chylomicronemia syndrome
Unincorporated Clackamas County resident Russ Cross has become an advocate for those with FCS, a rare genetic disorder that causes a buildup of fats in the blood and increases the risk of pancreatitis.
Cross had watched his father die from pancreatitis, a painful inflamation of the pancreas. Two years ago, Cross, a pharmacist at the Sandy Fred Meyer, was taken to the emergency room with an incident of pancreatitis.
At that time he was referred to a doctor at Oregon Health & Science University, who looked at his symptoms and his family history and told him he had familial chylomicronemia syndrome, or FCS.
"It was such a relief to put a name to it," Cross said.
His medical condition is further complicated by the fact that he has diabetes, which was diagnosed when he was only in his 20s, several years after his father died.
"I attended a pharmacy convention where a company was showing a new diabetic monitor. I volunteered to have my blood tested, and when my triglyceride levels turned out to be high at 400, I was told that I needed to see my doctor when I got home," Cross said, noting that a normal triglyceride level is about 150.
Because of that reading, he went to see his doctor, and when his blood was tested it "separated into a white layer and a red layer; the white layer was fat."
Diagnosed with FCS, Cross was told to follow a strict diet that allows him only 10 grams of fat per day.
"It is impossible to have a fat-free diet; there is virtually nothing I can eat. My nutritionist is trying to get me down to 40 grams of fat a day," Cross said.
"Going to a restaurant is difficult, and the grocery store becomes like a hunt — I have to read labels closely," he said.
He can eat lots of greens, steamed vegetables, turkey, chicken, lean pork and fish.
"He also takes lots of omega-3 fish oil," said Connie Cross, Russ's wife.
She added that she is getting better at finding foods he can eat and reading and understanding food labels.
"It's hard for her; we don't [go out] a lot. If we go to a social event, there is always food and drink," Cross said, noting that alcohol is also forbidden on his diet.
Cross checks his blood often and injects himself with insulin a couple of times a week.
He also takes a daily dose of gemfibrozil, the only drug currently available for those with FCS.
In February, Cross went to Washington, D.C., to join a panel discussion in front of doctors from the Federal Drug Administration. He is hoping that drug companies will take an interest in developing new medications for those with FCS.
"It seemed to go well. It was interesting; I had never experienced that part of the drug world, and [the companies] seemed to be real receptive," he said.
Akcea Therapeutics currently is conducting a clinical-trial program that is designed to evaluate the safety and efficacy of a new medicine called volanesorsen in patients with elevated levels of triglycerides in the blood, Cross said.
"I met two people who are part of the trial, and they said it changed their lives," he said.
Cross wants to reach out to people who have FCS, or who have its symptoms, but have not been diagnosed with it.
"I don't want anyone to go through the pain," he said, adding that emergency room doctors don't always diagnose FCS correctly because the disease is so rare.
It is crucial to have a blood test for triglycerides and an X-ray of the pancreas, Cross said, noting that knowing family history with pancreatitis is helpful.
He added: "I am doing this for my dad. I don't want anyone to go through the pain he did. There is hope out there."
Visit the FCS website at fcsfocus.com for more information about familial chylomicronemia syndrome.
Learn more about drug trials for volanesorsen at apociii.com.
Common symptoms for FCS include:
Pain in the abdomen, often with back pain
Extremely high triglycerides
Frequent or chronic pancreatitis