Facing life's reality

He looks like any other five-year-old boy. He plays, he runs and jumps; he goes to school.

To look at Clayton Spencer, one would never know that he faces each and every day just a little differently than any other boy in his class. On the outside looking in, most people would never know that the Spencers are different than any other young family of four - and really they aren’t except they face the reality of life with Cystic Fibrosis, a life-threatening genetic disease that currently affects approximately 30,000 in the United States. Clayton is one of those 30,000.

Like most people, Sarah Spencer had never really thought or heard much about CF. She first heard of the disease when she and her husband Robert were dating and he mentioned that his aunt had died of the disease. It was certainly not something Sarah thought would touch her life in a profound way.

“I really didn’t know what CF was,” she said. “I knew he had an aunt that had lung problems, and she died when she was only 22.”

When Sarah and Robert decided to start a family, there was no apparent reason for them to think that their child might have a predisposition for this birth defect. Even if Sarah had known more about the disease and understood that Robert might carry the defective gene, she really didn’t have cause for undo concern. She had no reason to think that she, too, might carry the gene. According to the Cystic Fibrosis Foundation, 10 million - or approximately one in every 31 Americans - are carriers of the defective CF gene but do not have the disease.

To have CF, a person must inherit two copies of the defective CF gene - one from each parent. If both parents are carriers of the CF gene, their child has a 25-percent chance of inheriting both defective copies and having CF. There is a greater chance, 50 percent to be exact, that the child will inherit one copy and be a carrier. A child of two gene-carrying parents has a 25 percent chance of not having CF or inheriting the gene. It was not until just before Clayton’s third birthday that Spencers even realized that they both carried the gene.

Typically, CF presents itself through lung problems. CF causes mucus to build up and clog some of the organs in the body, primarily the lungs and pancreas. Thick mucus causes bacteria to get stuck in airways which causes inflammation and infections that lead to lung damage. The mucus also clogs the lungs impairing breathing. Those affected may have persistent coughing, oftentimes with phlegm, or wheezing and shortness of breath. Symptoms also include frequent lung infections like pneumonia or bronchitis.

The symptoms that eventually led to Clayton’s diagnosis started off in a different manner. For the first nine months of his life, Clayton thrived. He followed the standard growth charts for a child his age - something that is not typical of CF children. Nearing his first birthday, this began to change for Clayton.

“It was touch and go for awhile,” said Sarah. “Clayton began to have stomach problems.” Sarah goes on to explain that it took awhile to figure out what was causing the problems. Early on, Clayton’s problems were thought to be acid reflux. The Spencers tried changing formulas and other medical suggestions - still Clayton was plagued with stomach troubles. After routine medical tests and treatments failed to yield a cause or remedy, Clayton’s local physician referred him to gastroenterologist.

Soon Robert and Sarah heard the five words that would set a new course of life for Clayton and the Spencer family.

“We just looked at each other and said ‘What do we do now?’” said Sarah as she recalled the doctor saying, “Your son has cystic fibrosis.”

As the Spencer family began this new, and not necessarily welcome, journey in life, Sarah began learning about the disease; Clayton began treatments.

A normal day begins at 5:45 a.m. Twice daily - once before school and once in the evening Clayton dons something called The Vest System. The system has an inflatable vest connected by tubes to a generator. During each 30-minute therapy, the vest inflates and deflates rapidly applying pressure to the chest wall. This works to loosen and thin mucus to move it toward larger airways where it can be cleared by coughing or suctioning. In addition to The Vest treatment, Clayton takes five pills with each meal. These are the good days.

On the bad days, when Clayton is sick, his vest treatments increase to four times per day with nebulizer treatments as an added bonus along with a full regime of antibiotics.

Every day includes an 800-calorie shake made by his mother to aid in keeping Clayton’s weight up, an issue with which he constantly struggles. Sometimes even a half-pound increase is cause for a celebration. Another issue affecting those with CF is the loss of sodium chloride otherwise known as salt. It is hard for CF patients to maintain adequate levels of salt in their bodies.

Most days find Clayton involved in regular boy activities. He enjoys karate and playing with friends and his four-year-old brother Nicholas. He can participate in any sport or activities he chooses with the exception of football. The football limitation is only to protect the mediport that resides just beneath his skin in his chest enabling medical personal to easily deliver medication and draw blood.

“It’s sometimes hard to tell anything is wrong,” said his mother. “He is sick in a way that doesn’t usually keep him down. It threw us for a loop at first. Now things have become pretty normal. It’s important to us that he not be treated differently.”

Testing has indicated that Clayton’s brother Nicholas does not have CF. As little brothers do, Nicholas looks up to Clayton. Sometimes he is envious and wants to do just as his brother does treatment and all.

Just as any boy might, Clayton sometimes balks at the treatment that has become a way of life. It’s not much fun for an active boy to spend time in a vest during treatment - even if he does get to choose the television show he wants to watch during treatment. One thing his parents remind him of is that the time spent doing this often delays what is known as a “tune up” trip to Driscoll Children’s Center in Corpus Christi. These trips are necessary to help lengthen and improve the quality of life for CF patients. During these visits, a minimum of 14 days each, the patient is given numerous treatments and high doses of intravenous antibiotics. However, a hospital stay does not necessarily mean confinement to bed for Clayton. He can take his IV along with him to the playroom which is set up for enjoyment of the youngsters that find themselves in the hospital for long periods of time.

Clayton had two such trips in 2006 and has already had one this year. As he grows older, these trips are likely to increase as infections tend to get worse and are more difficult to treat as an individual gets older. Infection and the resultant lung damage are the leading causes of death in individuals with CF.

Hospital trips aren’t fun and often find Clayton and Nicholas missing each other as the family becomes separated - paired as Daddy and Nicholas in Port Lavaca and Sarah and Clayton at Driscoll. Yet, Sarah is thankful.

“There are so many kids that are much worse off than he is,” she said. “We see children at the hospital that are so sick with other diseases. It could be so much worse, and we are thankful it isn’t.”

Currently, there is no cure for CF - though medical advances have greatly improved life expectancy. The life expectancy of someone with CF in the 1930s was only five years. In 1969 it increased to 14 years and jumped to 32 years in the year 2000. Today, the median age is 36.8 according to the Cystic Fibrosis Foundation.

“Great strides are being made,” said Sarah. “They are so close to a cure.” Sarah, Robert and their families have found a way to help through Clayton’s Angels. Monday, April 30, Clayton’s Angels are holding their 3rd Annual Spaghetti Dinner benefiting the Cystic

Fibrosis Foundation. The team will also participate in the Cystic Fibrosis Foundation’s National Great Strides: Taking Steps to Cure Cystic Fibrosis walk event to be held May 19. The proceeds of the dinner will go to the Cystic Fibrosis Foundation Gulf Coast Chapter in Houston for medical research to find a cure and to the care of Cystic Fibrosis patients.

The dinner will be held from 4 p.m. to 7 p.m. at the Bauer Exhibit Building at the Calhoun County Fairgrounds. The cost is $7 per plate. Lots of Layers will accept money donations for the foundation for cake by the slice. Drive-thru and dine-in plates will be available. For more information or lunch plates, call 552-9403. Advanced tickets may be purchased at Pfeil Insurance Agency, Twin Dolphin Cinemas, King Fisher Marine and the Calhoun County Extension Office at the fairgrounds.

The passion in Sarah’s eyes is evident as she talks about the motivation behind her family’s fundraising efforts.

“We fundraise because we wouldn’t be here without the Cystic Fibrosis Foundation,” she said. “The outlook is so much better for us because of their fundraising efforts and research. People are living so much longer, and this is so very doable.”

The question of mortality hangs in the air and Sarah and Robert know that one day they will face having to answer the tough questions that Clayton may ask.

For now they just remind him that following his treatments helps to keep the hospital stays at bay - and they continue to do their part to help researchers get one step closer to a cure for their son and little boys and girls everywhere.