Living Beyond the Diagnosis

By Megan Hulen

Caroline Collins and Dallas Hulen, both two years old now, were born just two weeks apart. They each have older sisters who have been friends since birth and their parents have been friends for years. Unfortunately, Caroline and Dallas are not allowed within six feet of each other. Why? You see, both children were diagnosed with cystic fibrosis within their first weeks of life. Cystic Fibrosis (CF) is a progressive genetic disease that causes persistent lung infections and limits the ability to breathe over time. According to the Cystic Fibrosis Foundation, “…in people with CF, a defective gene causes a thick, sticky buildup of mucus in the lungs, pancreas, and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.” Medical studies show that people with CF are at particular risk of spreading certain germs among others with the disease, which is known as cross-infection. Because of this people with CF should stay at least 6 feet away from each other at all times. There are more than 30,000 people in the United States with CF. CF is a genetic disease which occurs when a person inherits two copies of the defective CF gene, one copy from each parent. A person with only one copy of the CF gene is called a carrier.

Caroline and Dallas are patients at Wake Forest Baptist Health Brenner Children’s Hospital and the CF team there has become family. Children with CF require close follow-up with frequent clinic visits. Caroline and Dallas each require sixty minutes every day of airway clearance in the form of an inflatable vest that vibrates the chest at a high frequency to help loosen and thin mucus. When they are sick, they require two hours of vest therapy. They inhale medication to help open the airways and thin the mucus. Both children have pancreatic insufficiency, which prevents their bodies from breaking down food and absorbing vital nutrients, so they must take pancreatic enzymes before eating. They take an average of twenty pills per day. Antibiotics play a large role in treating exacerbations and preventing lung damage due to lung infections. For most people, a cold may last several days and there are no longstanding effects; however, for someone with CF a simple cold can lead to a lengthy hospitalization. As a result of a simple cold/virus, two-year-old Dallas has been hospitalized twice requiring multiple IVs, central lines, and numerous medications.

Many years ago, CF was considered a childhood disease as most affected individuals did not make it past elementary school age. Fortunately, thanks to the research and efforts of the CF Foundation, people with CF are now living into adulthood and the median predicted life expectancy of 47 years old. The CF Foundation does not receive government funding and relies on donations from individuals and businesses to further their efforts as they continue their research and clinical trials that will hopefully lead to a cure. In April, the CF Foundation hosted their 30th annual Great Strides event which is a family friendly walk to raise awareness for CF. Between both children’s teams, a Cure for Caroline and Dallas’ Duel, over $15,000 was raised for the CF Foundation and over 100 people walked in support.

Although advancements have been made in the way of CF treatment, there are heavy associated costs. The inflatable airway vest Caroline and Dallas wear cost anywhere from $10,000-$15,000. The daily medications they require cost approximately $10,000 before insurance and co-pay programs. Thankfully, Caroline and Dallas have medical insurance that covers the bulk of cost; however, the financial burden of CF can be excessive.

Caring for a child with CF is a daily challenge between managing breathing treatments, administering medications, maintaining health, and preventing exposure to germs all while allowing these children to play and act like “normal” toddlers. Both our family and the Collins have taken the approach of raising Caroline and Dallas much like we have raised our first-born children who do not have CF. We do what is necessary to keep them as healthy as possible while encouraging them to live beyond their diagnosis and lead a full life. Our family and the Collins family have decided to “choose joy” despite the challenges that come along with a disease like CF. Our faith in Jesus Christ allows us to choose joy on the good days, the tough days, and all the days in between. Our hope and prayer is for CF to be cured and what a day that will be when Caroline and Dallas can breathe easy. As the CF Foundation says, we will not rest until a cure is found.

One comment on “Living Beyond the Diagnosis

  1. Lauren Sapp

    So happy to see this article because so much information about cystic fibrosis is still misunderstood. My nephew was born right before they started requiring CF testing for newborns. He and my niece both have it and are living wonderful, healthy lives at 10 and 8 years old. Thanks for this informative article!

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