Resp Care
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Cystic fibrosis (CF) patients use several therapies to treat chronic inflammation and infection in the lungs and to improve airway clearance. Inhaled therapies in CF typically include bronchodilators, airway wetting agents, mucus-active agents, and antibiotics, among others. ⋯ Fortunately, novel aerosol delivery systems and drug formulations are being developed to tackle the many challenges of aerosol delivery in CF. If successful, these systems will reduce the time burden and improve the clinical outcomes for the CF community.
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The Cystic Fibrosis Foundation is a voluntary, nonprofit, health organization whose mission is "to assure the development of the means to cure and control cystic fibrosis and to improve the quality of life for those with the disease." While substantial progress has been made, as evidenced by a marked increase in the median predicted age of survival, much work remains to be done. Ongoing medical programs and activities of the Cystic Fibrosis Foundation, which span basic science, drug discovery, drug development, clinical care, patient education, and advocacy, will be described in this article. The key role of respiratory therapists in the cystic fibrosis community will be highlighted.
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Although some clinicians still believe that cystic fibrosis (CF) lung disease is largely due to hypersecretion of very viscous mucus, it has never been demonstrated that there is mucus hypersecretion in CF and it is clear that there is almost no intact mucin (the principal polymeric component of normal mucus) in CF sputum. CF sputum has lower viscosity when compared to asthma or bronchitis sputa, but is highly tenacious and biochemically most closely resembles pus. ⋯ There are many medications and devices either in use or under development that are meant to improve airway hygiene in CF by assisting with sputum expectoration. This paper discusses the scientific basis and potential mechanism of action for many of these interventions and briefly reviews the clinical evidence of their safety and effectiveness.
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The clearance of secretions from the lungs of patients with cystic fibrosis (CF) is an important component in the fight to preserve their lung function. There is excessive inflammation in the airways of these patients, which is thought to be exaggerated by ineffective mucociliary clearance and bacterial infection. In 2005 the Cystic Fibrosis Foundation formed the Pulmonary Therapies Committee to review all of the medical literature on the various airway-clearance therapies used in treating CF lung disease. ⋯ The respiratory and physical therapists are integral in helping patients and families develop airway-clearance routines that aid in the removal of the secretions that cause airway obstruction. There is a wide range of airway-clearance therapies that therapists can choose from when they are teaching the patients and family members the strategies of secretion removal. The questions are: What therapy is best for what age or stage of lung disease? What therapies will the patient do? And which therapies will be covered by medical insurance? These are all fundamental questions that must be answered when guiding families in finding therapies that are effective and appropriate for each CF patient's unique situation.
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Lung transplantation has become a viable option for those cystic fibrosis (CF) patients with end-stage lung disease. Despite the challenges that the CF patients present, the survival seen after lung transplantation is more favorable than seen in patients with chronic obstructive pulmonary disease and pulmonary fibrosis. Although the CF patients with severe respiratory disease usually are infected with organisms that display in vitro resistance to the commonly used antibiotics, these patients usually have successful outcomes with transplantation. ⋯ The limiting factor now in lung transplantation is the number of organs available. Efforts to increase the donor pool, such as alveolar recruitment strategies to improve gas exchange, have been effective in allowing more patients to be transplanted. Lung transplantation is now an accepted form of therapy in those patients who are developing progressive respiratory failure.