Cystic fibrosis is an autosomal recessive genetic disorder 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. The hallmark manifestation of CF is chronic, progressive lung disease, resulting from secretion of dehydrated mucus with airway obstruction, and malnutrition from pancreatic insufficiency. However, there is a wide range of disease variety. 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. The sweat glands and reproductive glands are also affected; almost all males with CF are sterile because of absence or impairment of vas deferens, whereas females have reduced fertility. The impact on the sweat gland give rise to abnormally high chloride secretion; a simple analysis of sweat chloride content continues to be the gold standard for CF diagnosis. Nursing care plan for clients with cystic fibrosis includes maintaining adequate oxygenation, promoting measures
to remove pulmonary secretions, emphasizing the importance of adequate fluid and dietary intake, ensuring adequate nutrition, and preventing complications. Here are five (5) nursing care plans (NCP) and nursing diagnosis (NDx) for cystic fibrosis:
1. Impaired Gas ExchangeImpaired Gas ExchangeNursing Diagnosis
May be related to
Possibly evidenced by
Desired Outcomes
1. Impaired Gas ExchangeRecommended ResourcesRecommended nursing diagnosis and nursing care plan books and resources. Disclosure: Included below are affiliate links from Amazon at no additional cost from you. We may earn a small commission from your purchase. For more information, check out our privacy policy.
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Other nursing care plans related to respiratory system disorders:
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Paul Martin is a registered nurse with a bachelor of science in nursing since 2007. Having worked as a medical-surgical nurse for five years, he handled different kinds of patients and learned how to provide individualized care to them. Now, his experiences working in the hospital is carried over to his writings to help aspiring students achieve their goals. He is currently working as a nursing instructor and have a particular interest in nursing management, emergency care, critical care, infection control, and public health. As a writer at Nurseslabs, his goal is to impart his clinical knowledge and skills to students and nurses helping them become the best version of themselves and ultimately make an impact in uplifting the nursing profession. What will a nurse teach a child with cystic fibrosis to take in order to facilitate digestion and absorption of nutrients?To help compensate, almost everyone with cystic fibrosis needs to take pancreatic enzyme supplements with meals and snacks. Taken by mouth, the enzymes Proteins that help make and increase certain chemical processes in the body. go to work in the intestines to help digest food so it can be absorbed by the body.
What is an important nursing intervention during the care of a hospitalized child with cystic fibrosis?Nursing care plan for clients with cystic fibrosis includes maintaining adequate oxygenation, promoting measures to remove pulmonary secretions, emphasizing the importance of adequate fluid and dietary intake, ensuring adequate nutrition, and preventing complications.
Which would the nurse teach the parents about preventing sudden infant death syndrome?Practice room-sharing without bed-sharing. Experts recommend that infants sleep in their parents' room — but on a separate surface, like a bassinet or crib next to the bed — until the child's first birthday, or for at least 6 months, when the risk of SIDS is highest.
Which assessment findings would alert the nurse that the child is in respiratory distress?Respiratory distress in the newborn is recognized as one or more signs of increased work of breathing, such as tachypnea, nasal flaring, chest retractions, or grunting.
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Which is the most appropriate nursing action when planning care for a child with cystic fibrosis
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