Gerene Bauldoff, PhD, RN, MAACVPR, and Connie Paladenech, RRT, RCP, MAACVPR

One topic rarely discussed in pulmonary rehabilitation (PR) is delivery of PR to patients with cystic fibrosis (CF). The septic nature of this disease adds concerns for the patients as well as for the PR professionals treating them, which historically has created challenges in delivery of care.

However, CF management has dramatically changed for the better in the past several decades, and we now see older adults with CF. In 1990, the average life expectancy for CF was age 30. In 2022, that estimate increased to 65 (Cystic Fibrosis Foundation, 2024a). With enhanced infection control protocols, an emphasis on patient education, and the growing availability of tele-rehabilitation, PR programs also are better equipped than ever to service this patient population. 

Infection Prevention

Infection control, affecting all care environments, is paramount both for patients and PR professionals. Concerns regarding cross-contamination raise questions as to the best practices (Cystic Fibrosis Foundation, 2024b). In a recent published pro-con debate about infection control interventions for CF patients, several commonly used interventions are supported (Haggie & Fitzgerald, 2020; Smyth, Smith & Rowbotham, 2020):

• Handwashing by both the healthcare providers and patients/families is CRITICAL
• Use of face masks by CF patients in outpatient settings (like PR) is recommended
• Stringent infection mitigation measures (i.e., careful disinfection of exercise equipment before and after PR for CF patients and use of alcohol-based hand sanitizer throughout each PR session) are also important.   

Addressing care needs of the CF patient who presents with multiply resistant organisms is also of concern. These patients require higher levels of protection regarding infection control and mitigation that may include airborne and/or contact infection control staff interventions such as masks, gowns, and gloves by PR professionals.   

Patient Education

As part of PR education, understanding the recent additions to CF pharmacotherapy is critical. CFTR gene modulators are a dramatic step forward in CF management by improving function of the faulty gene leading to improved lung function, weight maintenance, and reduced salt loss. It is postulated that these drugs can markedly reduce the impact of CF on lung function.

Airway clearance technique education remains a critical component of PR education for the CF patient. These include chest physical therapy (positioning with cupped clapping on chest) and breathing and coughing techniques to promote secretion expectoration. Mechanical devices such as flutter valves and/or vibrating vests also have been shown to be effective.

Tele-Care

Telerehabilitation can expand PR services for CF patients, especially those with more severe disease or those with multi-resistant or pan-resistant airway organisms. The use of home exercise reduces risk for cross-contamination (either to or from the patient with CF and the other patients). As with traditional delivery PR, these programs should be individualized to the patient’s needs and goals. Description of exercise training delivery via telehealth is provided in a recent protocol publication (Lang, et al., 2019).

Real-World Example

The experience of an actual patient in North Carolina illustrates what life and medical care, including PR, looks like for someone living with CF today:

Susan is a young woman who has managed to not only beat the odds of surviving a life-altering disease but to thrive while facing the challenges before her. As an infant and young child, she had difficulty gaining weight and always had a lot of chest congestion with thick, sticky mucus. At age three, she was referred to Wake Forest Baptist Medical Center, where her diagnosis of CF was confirmed. The Cystic Fibrosis Clinic there assumed primary responsibility for Susan’s medical management and now, at age 32, she attends the Adult CF Clinic and continues to do well.

Once enrolled in the CF Clinic and placed on pancreatic enzyme supplementation, Susan’s weight stabilized and fortunately, her sputum cultures were negative for resistant organisms. In addition to nutrition and other medical management issues, airway clearance and prevention of lung infections are her major issues.

PR has played a key role in Susan’s care; and although she does not participate in group classes because of the risk of exposure to resistant bacterial organisms, she does receive the therapy through the CF Clinic — a multidisciplinary environment structured to minimize the risk of cross-contamination between patients.

In the clinic, patients are seen individually and never cohorted. Infection control practices are strictly enforced and include disinfection of exam rooms between patients, leaving exam rooms unoccupied for a minimum of 30 minutes between patients to allow for aerosol settling, use of HEPA filters, disposable masks and gowns that staff change out of in between seeing patients, and patient masking. Everyone (patients, families, staff) is expected to practice meticulous handwashing: “wash in, wash out.”

A member of the pulmonary rehab team provides education on airway clearance techniques and exercise at each visit and adapts exercise prescriptions to best meet patient needs. One of the goals for CF patients is to help them understand the critical importance of establishing a treatment plan and encourage them to be as active as possible. This not only provides the physical benefits of exercise but also aids greatly in airway clearance, as patients are more likely to perform activities if they are fun. One of Susan’s favorite activities was bouncing on a Hoppit ball that she continued to use until she was about 10 years old. She has now transitioned to camping and hiking trails in the Blue Ridge mountains with her husband and two Bernese Mountain dogs.  

Not only has Susan thrived in her personal life, she also has flourished at the professional level. As a licensed practical nurse working in an outpatient orthopedic clinic, she provides patient care but is at a lower risk of encountering infectious organisms. She has recently started Trikafta® and will be monitoring her FEV1. By protecting against infection, participating in PR, and taking advantage of the latest treatment interventions, she is an example of how CF patients can avoid premature mortality and instead live healthy, happy, and productive lives.

Pearls for the PR Professional

Enrollment of CF patients into your PR program does not need to be onerous or frightening.  Practicing pristine infection control and following your institution’s protocols regarding multiply resistant organisms mitigates concerns for cross-contamination. Modifying educational information specific to the patient and their disease process is central, including medications and nutritional evaluation and intervention as indicated. Finally, the option of tele-rehabilitation can provide alternative delivery that best meets the needs of your patients and your program.

References:

WEBSITES:

American Lung Association. (2024). Treating and Managing Cystic Fibrosis. https://www.lung.org/lung-health-diseases/lung-disease-lookup/cystic-fibrosis/treating-and-managing

Cystic Fibrosis Foundation (2024a). Understanding changes in Life Expectancy.  https://www.cff.org/managing-cf/understanding-changes-life-expectancy

Cystic Fibrosis Foundation. (2024b). How can you reduce the risk of cross-contamination? https://www.cff.org/managing-cf/how-can-you-reduce-risk-cross-infection

Mayo Clinic. (2024). Cystic Fibrosis—Diagnosis and Treatment. https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/diagnosis-treatment/drc-20353706

ACADEMIC PAPERS:

Haggie. S & Fitzgerald. (2020). Pro-con debates in clinical medicine infection prevention and control in cystic fibrosis: One size fits all? The argument in favour. Paediatric Respiratory Reviews 36: 97-99. https://doi.org/10.1016/j.prrv.3030.06.008

Smyth, AR, Smith SJ, Rowbotham NJ. (2020). Pro-con debates in clinical medicine infection prevention and control in cystic fibrosis: One size fits all. The argument against.  Paediatric Respiratory Reviews 36: 94-96. https://doi.org/10.1016/j.prrv.2019.01.001

Lang, RL, Wilson C, Stockton K, Russell T & Johnston LM. (2019). CyFIT telehealth: protocol for a randomized controlled trial of an online outpatient physiotherapy service for children with cystic fibrosis. BMC Pulmonary Medicine 19 (21). https://doi.org/10.1186/s12890-019-0784-z

(Left): Gerene Bauldoff, PhD, RN, MAACVPR, has been a member of AACVPR since 1992. She has served two terms on the Board of Directors and currently serves on multiple committees. She also holds fellowship in the American Academy of Nursing and the American College of Chest Physicians.

(Right): Connie Paladenech, RRT, RCP, FAARC, MAACVPR, has been a member of AACVPR since 2000. She is currently a member of the Board of Directors. She has held numerous offices and served on many AARC and AACVPR committees.