• COVID-19
    Cystic Fibrosis
    Read the story of ASCP Patient Champion Leland and learn about the role
    of laboratory testing in the diagnosis and treatment of Cystic Fibrosis.

WHAT IS CYSTIC FIBROSIS?

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system, and other organs in the body. Cystic fibrosis affects the cells that produce mucus, sweat, and digestive juices. These secreted fluids are normally thin and slippery. But in people with CF, a defective gene causes the secretions to become sticky and thick. Instead of acting as lubricants, the secretions plug up tubes, ducts, and passageways, especially in the lungs and pancreas.

Cystic Fibrosis

This Cystic Fibrosis slide is showing mucoid pseudomonas which is the most common infection in CF patients.

ASCP Patient Champion Leland

“Be patient, tactful, appreciative, and kind to your care team. It’s important to also ask the right questions. These are simple things to do, but they go a long way in maintaining a good relationship with your medical team and caregivers.”

LELAND’S STORY

At 4 years old, Leland was diagnosed with Cystic Fibrosis (CF), a chronic disease causing severe damage to his lungs. Because he was so young when he was diagnosed, Leland does not remember a life without having CF. Leland’s parents raised him to be just like any other kid playing in the park or hanging out with friends, the only difference was that he would have to take some precautions and live life with some modifications. Since Leland was diagnosed with CF, he lived at a much greater risk of getting a lung infection because the thick mucus built up in his lungs allowed germs to thrive and multiply. Being exposed to more germs and other infections posed a detrimental threat to his health, so Leland had to modify his interactions with environments that put him at risk.

When he was 28, Leland received a lung transplant to replace his damaged lungs. Laboratory testing played an important role in the transplant process both before and after his lung transplant. Blood tests were performed to determine the blood type of the donor to see if their blood type would be compatible with Leland’s. These blood tests also involved tissue typing, which are a series of tests performed in the laboratory that evaluate the compatibility of tissue between the organ donor and recipient. The tissue typing lab identified and compared information about Leland’s antigens (the “markers” in cells that stimulate antibody production) so that a donor lung could be matched to Leland. The transplant was successful, however it was followed with a series of new complicating diagnoses, including osteoporosis, diabetes, and skin cancer which he then needed to manage.

Leland continues to receive follow-up care and testing three to four times a year. He has CBC and CMP lab tests done once a month at a minimum which are critical in making sure his health post-transplant is monitored regularly and maintained, especially having been diagnosed with further health complications. He also has magnesium blood tests performed along other tests that monitor how his treatment drugs are affecting him. Despite the serious health challenges that Leland has faced since he was a child, Leland is optimistic. In the meantime, as he continues to navigate his diagnoses, Leland and his family are focused on enjoying life to the fullest every day. He continues to have ongoing support from his wife and two adopted children. Their faith has played an integral role in managing his cystic fibrosis, lung transplant, and everything else that has come their way.