Family disorganization and role changes. ![]() Rapidly expanding clinical knowledge base with new interventions available.Ineffective or incomplete education in past.First exacerbation/hospitalization experience.Poor nutritional status resulting from anorexia.New acquisition of common cystic fibrosis airway pathogens.Growth plume of known colonized CF airway pathogens.Increased mucopurulent production and mucous plugging in response to new or increased airway bacterial growth.Infection with lung consolidation, alveolar collapse.Bronchiectasis with decreased surface area for gas exchange and loss of lung function.Airway obstruction by nasal obstruction.Gastrointestinal tract abnormalities (recurring pancreatitis, biliary cirrhosis)Īssess for factors related to the cause of cystic fibrosis (CF):.Crackles and rhonchi (abnormal lung sounds).Decline in pulmonary function testing (spirometry).Hypoxemia (low oxygen levels in the blood).Hypercapnia (high carbon dioxide levels in the blood).Nonpulmonary manifestations include gastrointestinal issues, CF-related diabetes, and genitourinary problems affecting fertility.Īssess for the following subjective and objective data: CF also presents with upper respiratory symptoms like sinusitis and nasal polyps. Chronic inflammation and infection result from impaired mucus clearance, with early colonization by S. Unrealistic expectations and emotional challengesĬystic fibrosis (CF) is characterized by pulmonary symptoms such as a productive cough, wheezing, hyperinflation on chest x-ray, and obstructive airway disease.Malabsorption and nutritional deficiencies.Preventing chronic lung infections and complications.Managing decreased respiratory function.Managing airway obstruction and inflammation.The following are the nursing priorities for patients with cystic fibrosis (CF): Palliative care and end-of-life discussions should be addressed when necessary, providing support to patients and families facing a shortened lifespan and uncertainty. As CF is a lifelong disorder, patients adapt daily activities as the disease progresses. Adequate fluid and dietary intake are emphasized for secretion removal and nutrition. Patients are educated on reducing respiratory infection risks and recognizing early signs of infection. Nurses help patients manage pulmonary symptoms, prevent complications, and promote secretion removal through strategies like chest physiotherapy and breathing exercises. Nursing management is essential in the interdisciplinary care of adults with cystic fibrosis (CF). The impact on the sweat gland gives rise to abnormally high chloride secretion a simple analysis of sweat chloride content continues to be the gold standard for CF diagnosis. Newborn screening for cystic fibrosis is performed in all US states and the District of Columbia. The sweat glands and reproductive glands are also affected almost all males diagnosed with CF are sterile because of the absence or impairment of vas deferens, whereas females have reduced fertility. Pulmonary symptoms can range from nearly asymptomatic disease with undetectable changes in the lung function to severe obstructive disease early in childhood, whereas gastrointestinal symptoms may range from mild constipation, normal liver function, and intermittent pancreatitis to severe loss of pancreatic function, malabsorption with subsequent malnutrition, CF-related diabetes, and end-stage liver diseases. However, there is a wide range of disease varieties. The hallmark manifestation of CF is chronic, progressive lung disease, resulting from the secretion of dehydrated mucus with airway obstruction, and malnutrition from pancreatic insufficiency. The basic defect in CF is an exocrine gland dysfunction that includes increased viscosity of mucous gland secretions and a loss of electrolytes in sweat because of an abnormal chloride movement. Providing Patient Education & Health TeachingsĬystic fibrosis (CF) is an autosomal recessive genetic disorder, with both parents carrying a gene for the disease, that is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR) present in the mucus-secreting cells of the body, primarily affecting the respiratory, reproductive, and gastrointestinal tracts. Monitoring Diagnostic Procedures and Laboratory Studies Administering Medications and Pharmacological Support Maintaining Patent Airways & Improving Gas Exchange ![]() Gain valuable insights on nursing assessment, interventions, goals, and nursing diagnosis specifically tailored for cystic fibrosis in this guide. Utilize this comprehensive nursing care plan and management guide to provide effective care for patients with cystic fibrosis.
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