Respiratory Care for Cystic Fibrosis


Early diagnosis, proper care and thorough education can improve and extend the lives of babies born with cystic fibrosis (CF). Treatment of this patient population has improved exponentially in the past few decades, most notably with the universal implementation of newborn screening.

“The impact of early diagnosis and treatment of cystic fibrosis is profound,” said Catherine O’Malley, RRT-NPS, cystic fibrosis specialist at Ann and Robert H. Lurie Children’s Hospital of Chicago. “Patients diagnosed early due to newborn screening will benefit from early evaluation, monitoring and treatment strategies.”

Over the last 25 years, the median age of survival for patients with CF has steadily increased. Since 2002, life expectancy for this patient population has increased by nearly 10 years to the median age of 41.1 years and is expected to be even higher for the cohort of CF patients born in the last 2 to 5 years, according to data from the Cystic Fibrosis Foundation’s Patient Registry Report.

Three strategies that contributed to the rise in life expectancy are aggressive treatment of respiratory infections; improved nutrition strategies and treatment of pancreatic malabsorption and malnourishment; and airway clearance, according to Fadel E. Ruiz, MD, a pediatric pulmonologist and the interim director of the cystic fibrosis clinic at Texas Children’s Hospital.

“Proper respiratory care started early in the life of CF patients has a significant impact controlling secretions and recurrent respiratory infections,” Ruiz said. “I like to tell patients and families that the effort they put into proper daily respiratory care is their investment into a more stable disease process from the respiratory perspective.”

Early Diagnosis

The United States made newborn screen (NBS) testing for cystic fibrosis a standard in December 2009. “This allows for earlier diagnosis, usually within the first 2 months of life,” Ruiz said. “And the earlier the diagnosis, the sooner the treatment can begin.”

According to 2012 CF Foundation data, 61% of people with CF were diagnosed because of abnormal newborn screens. “Research has shown than people diagnosed via NBS have better weight and healthier lungs later in life than those diagnosed because of symptoms,” Ruiz said.

When patients are diagnosed with CF later in life, pulmonary function and growth may be compromised because the patients haven’t been treated. “Years ago, it would not have been unusual to diagnose a toddler or older child who was already malnourished, which is a problem,” O’Malley explained.

Patients who have gone undiagnosed may not be able to catch up and meet their genetic potential, according to O’Malley. Today, patients that are diagnosed late were born before newborn screening began.

O’Malley treats patients with CF ranging from newborn to young adults who are close to transition. The transition age varies based on individual needs, but generally, a patient will transition to an adult facility by the age of 21 years.

Education Is Paramount

With advancements in screening, education and awareness, patients are living longer than ever with cystic fibrosis but preparing the family for the long road ahead remains an ongoing challenge for providers.

“When a baby has been screened and receives a confirmed diagnosis through sweat testing, we have a strategic plan to introduce the family to CF care,” O’Malley said. “It’s important that we are careful and appropriate with this introduction because we don’t want to overwhelm the parents who are initially dealing with the news of the diagnosis.”

Patients and family members play a key role in the treatment plan, explained Ruiz. “Communication needs to be open so families can participate and be fully involved in care decisions,” he said. “Adherence to the prescribed regimen improves when patients and families understand the reasons for treatments and therapies.”

Lurie Children’s aims to optimize the growth and health of each baby diagnosed with CF. The center employs a multidisciplinary team comprised of pulmonologists, nurses, nutritionists, respiratory therapists and social workers to address the needs of patients and families. Frequent visits are an important part of the care plan so patients and families visit the center every 2 to 4 weeks initially, and then every 1 to 2 months until about 1 year of age. After the first year, visits are scheduled once every 2 to 3 months to monitor the child’s growth and progress.

For a newly diagnosed patient, the initial clinic visits are planned out to focus on one topic at a time so the parents are not inundated with too much information. “The first visit focuses on genetics and answers the parents questions about what a gene is, what the altered CF gene (mutation) is and what it means and how this happened,” O’Malley said.

Because malnutrition is closely related to poor lung function, the second visit focuses on nutrition to ensure the child’s dietary requirements are met. The goal is for the child to have normal growth and development and to meet his genetic potential in height and weight. The other visits focus on respiratory care and social work.

Respiratory Treatment Plan

The goal of respiratory care in CF is to maintain healthy lungs and prevent a decline in lung function. Early lung treatment includes the use of airway clearance techniques (ACT) that mobilize secretions, inhalation therapy to deliver medications to the lungs, early treatment of respiratory illnesses and the use of medications that have proven to be effective.

Therapies are introduced depending on age, ability to perform them, patient preference and stage of respiratory disease process, Ruiz told ADVANCE. The plan of care, he said, should include physical education and exercise to maintain good respiratory health and also education about the disease and the reasons behind the complex regimen.

Respiratory treatments include a variety of airway clearance techniques including Percussion and postural drainage (chest PT), Vest, flutter, PEP and Acapella, according to Ruiz.

“Airway clearance is usually the first thing I teach parents,” O’Malley said. She encourages the parents to train other family caregivers on the technique, if possible, because it helps to have others share in the responsibility.

O’Malley demonstrates chest PT on babies with CF for the parents. “While I am doing chest PT, I describe the lungs and the bronchial tree, the purpose of the procedure and how often I recommend they do it,” she explained. “I always hope the baby sleeps through my demonstration or at least relaxes. If the baby cries, then it’s difficult for parents to absorb the information because they will be concerned about why the baby is crying.”

Most often, O’Malley shared, babies tolerate chest PT with no problem because they like to be held and touched. “I review the 5 S’s of comforting a baby which include side positioning, shushing, swaddling, sucking and swinging, so the parents are prepared with techniques,” she said.

There are several inhaled medications used to treat CF. Inhalation therapies with hypertonic saline (Hypersal) and dornase alfa (Pulmozyme) can help mobilize mucus. Bronchodilators like albuterol can open up the airways, and inhaled antibiotics such as tobramycin and aztreonam (Cayston) control/suppress respiratory infections.

By following infection control principles you will create a safe environment for you, your co-workers and your patients.

“We introduce an inhaled bronchodilator early because it’s one of the recommended chronic medications in CF care,” O’Malley said. “Parents may be ready to learn both chest PT and inhalation therapy right at the start, or we can wait until the family is ready to handle both interventions,” she explained. “Knowing that the child needs a medication can be difficult for parents to digest.”

Vaccines, CPT and albuterol are preventive therapies that need to be monitored with frequent visits to reassess. “Caring for a child with CF can be a struggle for families so we want them to have stability and support. We intervene when we can to provide emotional and educational support or whatever is needed,” O’Malley shared.

Infection control is another important part of the preventive care plan. “This includes immunizing against viral infections that can cause immediate and long term complications in patients with CF,” Ruiz said. “Annual influenza immunizations are important as well as staying up-to-date with all traditional immunizations including Pneumococcus.”

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One of the biggest concerns for this patient population is access to all appropriate therapies and treatments, Ruiz told ADVANCE. “It’s important for patients to have proper medical coverage, access all programs available to CF patients and families, and maintain long-term adherence,” he said.

Is a Cure on the Horizon?

The CF community is excited about recent developments in the care of patients with CF including therapies developed to target the specific CF protein, Ruiz said. Kalydeco, approved by the U.S. Food and Drug Administration in 2012, is the first drug available that targets the underlying cause of CF – a faulty gene and its protein product, CFTR. The drug was specifically developed for people 6 years and older with the G551D mutation of CF.

According to O’Malley, the CFTR protein is a chloride channel that allows chloride to exit the cells in the body, which is an important function in regulating fluids in the body. In CF, the CFTR protein doesn’t function properly or it does not function at all. Those that have the G551D mutation have a CFTR protein that happens to be in the right place in the cell, but it doesn’t work.

“The medication Kalydeko makes it work,” O’Malley said. “The drug opens the channel to let chloride exit the cell. Only about 4% of the CF population has the G551D mutation, but this medical breakthrough holds so much promise for the future.”

Exciting and promising studies are currently underway looking at medications to correct the most common mutation in CF: the F508del mutation.

Rebecca Mayer Knutsen is on staff at ADVANCE. Contact: rknutsen@advanceweb.com

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