Little fighter realises his dream

LYDENBURG – You die once, you live every day. This is the slogan by which terminally ill Rynhardt Viljoen (8) lives. Rynhardt is a grade two learner at Laerskool Lydenburg and suffers from primary sclerosing cholangitis (PSC) for which there is no cure, except a liver transplant.

This little fighter was, however, recently chosen as one of the children for the Reach for a Dream foundation in fulfilling his lifelong aspiration: flying in a helicopter. Rynhardt was awarded this on September 4. Henley Air offered not only him, but his whole family a scenic helicopter ride over Johannesburg.

He also had the opportunity to play pilot in the aviation company’s helicopter simulator and held a real helicopter’s joystick flying solo for a couple of minutes.

To use his words, “It was beyond awesome!” According to his mother, Alice, Rynhardt was an ICU baby and was in and out of the hospital for most of his baby life.

“He was first diagnosed with portal hypertension, an increase in blood pressure within a system of veins called the portal venous system. His first operation was a shunt transplanted into his brain. It failed and Rynhardt’s heart failed shortly after the operation.”

A doctor at the hospital said the family could not go on like this and suggested a bone marrow transplant. “We were referred to the Wits Donald Gordon Medical Centre where various scans were conducted. The results showed not only PSC, but also fibrosis, sclerosis, liver failure and Chrone’s disease. Even though a transplant is his only option, Rynhardt also has a delayed heart rhythm.”

His bone marrow is green and doctors first suspected stage four leukaemia, but he was declared safe from cancer. According to Alice, there is no explanation for this occurrence. PSC has no cure. There is no treatment, only maintenance and trail and error. Even if he does get the transplant, there is a chance that the PSC could return.

As Alice explained, transplant lists can take anything from five to 15 years and even then, donors have to undergo several tests to establish if they are suitable. Another option is a liver donation from a deceased child. “You don’t get children’s livers. A lot of people do not want to donate their child’s organs. A suitable liver for Rynhardt will be between the age of four and 12.” She said Rynhardt knows that every day could be his last.

“His older brother and sister also know that he could die any day. It is difficult, my daughter asks for a birthday party every year. We just cannot afford it. We have to budget for everything – his medication, some of which the medical aid does not cover, our petrol money to Johannesburg every time, toll money… the list goes on.”

Even if he gets the transplant, Rynhardt will be on medication for the rest of his life. He will never play sport or have the opportunities other children will have. The road ahead is a difficult one, but the family take it day to day. “It is part of our life.”

Rynhardt has two other wishes left: to meet singer Bok van Blerk and to own and play an electric guitar.

MORE ABOUT PSC:
According to www.pscpartners.org, PSC is a rare liver disease that damages the bile ducts inside and outside the liver. With PSC, bile ducts are inflamed and the inflammation leads to scarring and narrowing of the affected ducts. Eventually, blockages may occur.

As the scarring blocks more and more ducts, bile becomes trapped in the liver. This damages the liver and can result in fibrosis and cirrhosis of the liver and liver failure. Patients may eventually require a liver transplant. PSC does not discriminate based on age or gender, but affects about twice as many males as females. Many patients are asymptomatic at the time of diagnosis.

It is estimated that there are more than 30 000 PSC patients in the United States. It often is found in people with inflammatory bowel disease, most often ulcerative colitis and sometimes Crohn’s disease. More than 75 per cent of PSC patients have ulcerative colitis. It is also associated with other autoimmune diseases.

There are currently no proven treatments that change PSC’s course or slow its progression, but there are medications and procedures that help relieve some of the symptoms.