Keep Walking

Going into the hospital this last time Jed and I started to mentally prepare for him not walking back out unless he is able receives a transplant. So we are thoroughly enjoying that he was able to break free yesterday from the hospital and be home for whatever time frame we are given. After 2 weeks of antibiotics, respiratory treatments, and steroid boost he is back home to the “Haas Home Hospital”.

There are days that physically walking and the mental walking forward with CF is difficult yet Jed knows that he cannot stop. Once he stops walking and pushing forward then CF wins the battle. This weekend the kids and I, along with family and friends, helped raise money for CF Foundation through a bake sale, brat fry, and Great Strides walk. I want to say I huge thank you to the individuals who helped bake, serve, donate, walk, or supported us directly through gifts and prayers. Many people I encountered over the weekend, and I know even people following our journey here on PostHope, may not fully understand what Cystic Fibrosis is. So in the spirit of the CF awareness walk I will explain what CF is, how someone gets it, basic treatments, and briefly what lung transplant entails. (Data is taken from CFF.org)

Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. In people with CF, a defective gene causes a thick, 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 nutrients. Symptoms range from very salty-tasting skin, persistent coughing, at times with phlegm, frequent lung infections including pneumonia or bronchitis, wheezing or shortness of breath, poor weight gain, difficulty with bowel movements, increased sinus infections, to male infertility.

Cystic fibrosis is a genetic disease. People with CF have inherited two copies of the defective CF gene -- one copy from each parent. Both parents must have at least one copy of the defective gene. People with only one copy of the defective CF gene are called carriers, but they do not have the disease. Each time two CF carriers have a child, the chances are: 25 percent (1 in 4) the child will have CF, 50 percent (1 in 2) the child will be a carrier but will not have CF, 25 percent (1 in 4) the child will not be a carrier and will not have CF. 

There are more than 1,700 known mutations of the disease. The range of severity of the disease is based on the type of mutations. More than 30,000 people are living with cystic fibrosis (more than 70,000 worldwide). Approximately 1,000 new cases of CF are diagnosed each year. Today screening for CF happens at birth, there is also sweat test that can determine if an individual has CF. This is how Jed was tested when he was ten years old. The life expectancy for CF has increased over the years. When Jed was born the life expectancy for someone with CF was 18 years old, today that life expectancy has increased to 40 years old with the increased knowledge and treatments for CFers.

Due to the various mutations of CF and individuals overall health varying, treatments can widely range for patients. The common treatments for someone with the typical CF mutations can be daily airway clearance to rid the lungs of the built up mucus, this can be done by manual chest compression by another person or by a vest system. For most CFers this is done at least twice a day for a minimum of 30 min, but more if needed. Multiple different nebulized treatments are typical; medications to loosen the mucus, medication to open up airways, and others to fight the bacteria growth. Pancreatic enzymes are also necessary for the body to digest and absorb needed nutrients. CF patients are kept on a high calorie diet and take multiple supplements to get their daily nutrients. Jed on average takes a range of 20-30 pills a day along with all the other treatments. Staying physically active is also important for body and lung health. Exercise is key.

Flare ups for CFers mean that there is bacteria in the lungs that is growing and infection has set in, it is common to get oral or IV antibiotic cocktails to fight the bacteria. Once a bacteria is in the lungs it is unlikely to get rid of it completely. It can be described as putting a wet blanket on a fire within your lungs, yet the ashes are always simmering. The lungs are the perfect breeding ground for bacteria. Over time due to multiple flare ups scar tissue develops on the lungs and lung function decreases. There is currently no cure for CF, it is a terminal disease. 

Once an individual is below 30% lung function they can choose to be evaluated for a double lung transplant. There are many factors that go into whether someone is a good candidate and will be approved to go on the wait list for lung transplant. A transplant is not a cure, it is essentially borrowed time with different lungs that have an unknown expiration date. The recipient’s body will reject the lungs, it is just a matter of when. In a double lung transplant Cystic Fibrosis is still present within the body but not in the lungs, however lung transplants are the most rejected transplants. They are difficult to receive and the organ is exposed to bacteria through the outside environment due to breathing unlike other organ transplants. Rejection is controlled by immune suppressants, steroids, daily vital checking, taking precautions to stay clear of viruses and bacteria, and regular doctor checkups. A lung transplant is trading one disease maintenance for another. Many recipients can have onset diabetes or need future organ transplants due to the medications that are needed to control rejection. Cystic Fibrosis lung transplant national statistic is 88% of patients reach 1 year post transplant, 57% will reach 5 years post-transplant, and 38% reach 10 years post-transplant.

The battle of Cystic Fibrosis and double lung transplant is daily. One must never stop marching forward. The words of one of Jed and CJ’s favorite song by Toby Mac states it best:

Move, keep walkin’ soldier keep movin’ on
Move, keep walkin’ until the mornin’ comes
Move, keep walkin’ soldier keep movin’ on
And lift your head, it ain’t over yet, ain’t over yet.

Statistics can act like a lid sometimes. We know that life is not a statistic. God is the giver of life. Our job is to live sold out making the most of every opportunity until God takes us home. Jed has never lived limited by life expectancy stats, but I wanted to take the opportunity to make you aware of some of the realities of living with Cystic Fibrosis. It is important to remember that with God nothing is impossible. Living by faith is attempting to obey God expecting Him to fulfill our attempt with His power. We must keep walking forward by faith.