‘Unmasking the cure’

When you have a story to tell like Christian Goodpaster’s, the young man from Elizabeth who recently underwent double lung transplant surgery in Chapel Hill, N.C., just about everything else pales in comparison.
We first reported his need for the transplant several years ago. In December and again three times in January, Christian nearly had the operation. But the lungs were either defective or had been held too long after the death of the donor. On Feb. 16, everything was perfect, including surgery, Christian said he was told by the doctors.
What is this disease we know so little about?
Christian was born with Cystic Fibrosis (CF), a genetic disease that today affects some 30,000 children and adults in the United States. There are an additional 3,000 cases in Canada and 30,000 in other places in the world. Those numbers are low, compared to diseases like diabetes, which affects millions and for that reason perhaps attracts more research. Nevertheless, the Cystic Fibrosis Foundation, begun in 1955, supports CF research, which has led to great strides into the cause, treatment and possible cure.
When the CF Foundation was formed, few children born with the disease lived long enough to attend elementary school. Now a person with CF is expected to live into the early 30s, and many people live into their 50s and 60s.
According to the CF Foundation, giant steps continue to be made. In 1989, scientists supported by the Foundation discovered the cause: two defective genes are inherited, one from each parent. More than 10 million Americans (one in 31) are carriers of the defective gene, and do not know it, because there are no symptoms.
But the CF disease itself has a diverse range of symptoms and they vary in severity. The body produces a thick, sticky mucous which clogs the airways and can lead to life-threatening lung infections. The mucous also obstructs the pancreas which prevents enzymes from reaching the intestines to break down and digest food.
‘All CF patients take enzymes to help digest their food,’ Christian said late last week. ‘The digestive system lacks the proper acids and natural enzymes to digest their food so enzymes are given to supplement digestion.
‘For this reason, most CF patients are malnutritioned. Many look half their age and are very small and skinny. Along with medications and inhaled treatments, it’s of the uppermost importance for a CF patient to eat a high calorie diet and take supplements to help maintain and gain health weight.’
Bleeding is a frequent problem for patients when lung damage is advanced, Christian said, and immediate treatment is necessary. The source of bleeding in the patient’s airway must be stopped. Also, surgeries are sometimes necessary to place a gastric tube in a malnutritioned patient’s stomach to help them gain weight.
‘As you can tell, CF patients undergo a lot of stress to take care of themselves throughout their lives,’ Christian said. ‘Family and friends are the biggest supports of these individuals.’
That is the part we need to remember most, not just for Christian’s sake but for all those who suffer from debilitating, life-threatening diseases.
We know the courage they have. We’ve seen it in Christian and in many, perhaps far too many others for such a small community.
‘CF patients have been known to be courageous and strong fighters and can be put in a category of their own, some say,’ Christian said.
Perhaps ironically, our daughter, Vickie McDougal, who lives in a Los Angeles suburb, not long ago became a volunteer for the Cystic Fibrosis Foundation. A friend and neighbor, Jessica Plance, 15, suffered from the disease. She received her second chance at life with a double lung transplant a couple of years ago on Vickie’s birthday, Oct. 22. Jessica died a year later.
Vickie, like others, decided that is not OK, especially when the right amount of money for research could be standing in the way of a cure. Fund-raising events are being held, such as black-tie galas, which Vickie dearly loves to help organize. ‘It’s all in the organization,’ she says. ‘There’s nothing to it.’
The theme for the next such event is ‘Unmasking the cure.’
Christian Goodpaster and his family need our prayers, and they also need our financial help to get them through these long days and the years ahead.
A fund has been set up at First Harrison Bank for those who wish to help.
Christian can be reached by e-mail at [email protected]
And for those who would like to take a step further, the CF Foundation’s address is 6931 Arlington Road, Bethesda, MD 20814.
For additional information on the disease, the CF Foundation’s Web site is ww.cff.org.
The ‘Sixty-five Roses’ story
’65 Roses” is what little children suffering from Cystic Fibrosis call their disease.
(As the following true story illustrates, the words are much easier for children to pronounce.)
Mary G. Weiss became a volunteer for the Cystic Fibrosis Foundation in 1965 after learning that her three little boys had Cystic Fibrosis. Her duty was to call every civic club, social and service organization seeking financial support for Cystic Fibrosis research.
Mary’s 4-year-old son, Richard, listened closely to his mother as she made each call. After several calls, Richard came into the room and told his mom, ‘I know what you are working for.’
Mary was dumbstruck because Richard did not know what she was doing, nor did he know that he had Cystic Fibrosis.
With some trepidation, Mary posed the question back to Richard, ‘What am I working for, Richard?’
‘You are working for 65 Roses’,’ he answered so sweetly. Mary was speechless. She went over to him and tenderly pressed his tiny body to hers. He could not see the tears running down Mary’s cheeks as she stammered, ‘Yes, Richard, I’m working for 65 Roses’!’
For 38 years, 65 Roses’ has been used by children of all ages to describe their disease. But making it easier to say doesn’t make Cystic Fibrosis any easier to live with.
The ugly fact is Cystic Fibrosis is the number-one genetic killer of children and young adults in America today.
The ’65 Roses” story has captured the hearts and emotions of all who have heard it. The rose, appropriately the ancient symbol of love, has become a symbol of the Cystic Fibrosis Foundation.