Clinical Trial Gives Hope for a Longer Life: Kathy’s Story

Kathy Castleberry’s 2018 holiday season came with an unexpected bout of pneumonia that did anything but make the season bright. Sick through the entirety of the holidays, Kathy returned to work when she was feeling better, but she was extremely fatigued and had a persistent cough.

The nurse who worked in the office next to Kathy would hear her coughing and was concerned. She recommended that Kathy go back to the doctor to have the cough checked out, and she told Kathy it was important to use her inhaler regularly.

“She knew something wasn’t right and was on my case all the time,” Kathy said.

Kathy recovered somewhat, but less than four months later in March 2019, she was again diagnosed with pneumonia. She questioned if she’d ever really recovered from the initial infection.

In April 2019, Kathy’s daughter convinced her to go to Texas for a vacation with friends. Kathy still wasn’t feeling 100% — she was incredibly fatigued but agreed to go along and take a rest. Despite all the fun activities planned for the vacation, Kathy was too tired to go out.

Kathy went to see her doctor once she was back home as she was still coughing and didn’t feel right. Her primary care doctor listened to her chest and was concerned about a rattling sound in the bottom of her lungs. Kathy had chest X-rays and was sent to OU Health for further testing.

An Interstitial Lung Disease Specialist Makes a Diagnosis

After asthma and other similar respiratory conditions were ruled out, Kathy was referred to pulmonary and critical care physician Jad Kebbe, M.D., who specializes in interstitial lung diseases.

After a thorough evaluation, Dr. Kebbe decided that Kathy needed a lung cryobiopsy to help with the diagnosis. A cryobiopsy is a non-surgical, minimally invasive technique which means that less time is spent in the hospital and in recovery. It’s typically performed under general anesthesia with a bronchoscope, which is a flexible tube that provides access to the lower airways. A flexible cryoprobe is introduced through the bronchoscope into the airway and uses nitrous oxide to freeze and collect lung tissue that can be tested for interstitial lung disease. Dr. Kebbe was one of the first pulmonary specialists in Oklahoma to perform lung cryobiopsy instead of the usual surgical biopsy.

Kathy’s case and biopsy were reviewed by a multidisciplinary team of OU Health specialists that included pulmonology, rheumatology, radiology, and pathology. The results revealed that she had idiopathic pulmonary fibrosis (IPF). Pulmonary fibrosis causes progressive and irreversible lung scarring which leads to worsening shortness of breath, often an unrelenting cough, and prevents people from doing the activities they had previously enjoyed.

Medical studies report that 20-30% of patients who are diagnosed with IPF have a family history of the disease and symptoms typically occur between the ages of 50 and 70 years. Kathy’s mother was diagnosed with pulmonary fibrosis when she was in her 60s and died of it.

“Pulmonary fibrosis would typically lead to death within 2-3 years, which unfortunately is compounded by the fact that doctors can take more than a year to correctly diagnose it. So, it goes untreated in the meantime and patients lose valuable time,” explained Dr. Kebbe, who also serves as the director of Oklahoma's first and only interstitial lung diseases program. “There are two medications that can slow it down, but it has no cure to date.”

A Treatment Plan & Support

Dr. Kebbe gave Kathy a treatment plan and told her about the two available medications. She was assured that while there is currently no cure for IPF, the medications could stop the scarring of the lungs and stop the disease from progressing as fast as it otherwise would. Kathy took one of the medications for over two years, and then switched over to the other medication. She has remained stable since.

Kathy attends pulmonary rehabilitation twice a week, which involves supervised exercise to improve her lung function and reduce symptoms. She also exercises at home with a plan that Dr. Kebbe gave her but finds that the accountability and support at pulmonary rehab helps keep her on track.

As there’s no cure for IPF, Kathy has started to prepare for a lung transplant should it ever be needed. Technically, a lung transplant is the only cure for IPF if the donated lung is healthy and remains functioning.

Clinical Trials Bring New Hope for a Cure

At 72, Kathy wants to do all she can to help advance the treatments available for IPF. Under Dr. Kebbe and team, Kathy is part of a five-year clinical trial to test treatments for the disease.

“I have kids and grandkids and while I know what my prognosis is, if I can do something to help somebody else have a better prognosis, I will do it,” said Kathy. “My mother died from IPF and back then the treatments we now have weren’t available. I want to do something to help others.”

Kathy started by participating in a one-year double-blind trial. The trial qualified her for the current five-year extension of the TETON trial, with a drug called TYVASO (the pharmaceutical name is Treprostinil). TYVASO has shown promising results in prior clinical trials for other types of Interstitial Lung Diseases.

“The hoped-for outcome is that TYVASO will help IPF patients improve their lung function and quality of life, and hopefully slow down the process of lung scarring,” said Dr. Kebbe.

Kathy is doing well since starting the trial in early 2023, and she has noticed some improvement.

“I am managing everything I need to do, and I feel mostly well,” Kathy explained. “The clinical trial team is awesome and no matter what I need or how I feel, I can send one of the nurses a text message and they’ll get straight back to me.”

“At OU Health, we strive to diagnose patients early, start the right treatment, and work hard with the Pulmonary Fibrosis Foundation and other academic centers to find a cure,” said Dr. Kebbe. “We have several trials underway with the hope of finding a cure. We offer patients comprehensive, personalized care and help them freely access antifibrotic medications that can cost more than $100,000 per year.”

Any IPF patient is potentially eligible for this clinical trial, or any of the other IPF clinical trials in which OU Health is a study site. Kathy is 1 out of 12 patients at OU Health participating in the clinical trial. Participants must meet specific criteria to qualify for any clinical trial, but Dr. Kebbe and the team look at every IPF patient’s case individually and help with treatment and possible clinical trial enrollment.

Kathy has benefitted from the care and expertise she receives from Dr. Kebbe and team. She enjoys spending time with her eight grandkids and is due to retire in November.

Learn More & Get Help for Advanced Lung Disease

You can learn more about pulmonary fibrosis and advanced lung disease treatments at OU Health. You can also call (405) 271-6173 and choose option 2 for more information on how to participate in current clinical trials.

Attend the Better Breathers Club, sponsored by OU Health Respiratory Therapy, for support from others who experience respiratory issues. Monthly meetings cover many breathing-related topics.

For information or to RSVP, call (405) 341-6100, ext. 5440.