Question

A 10-year-old female has tested positive for the sweat-test and has been diagnosed with cystic fibrosis.

Phases: primary, secondary, and tertiary.

What phase is cystic fibrosis? What would be a nursing intervention for a 10-year-old with cystic fibrosis?

Answer 1

Cystic fibrosis is a genetically inherited life threatening disorder that damages the lungs and digestive system. The end stage lung disease is characterized by cysts and fibrosis of lungs and airways and therefore cystic fibrosis could be referred to as the tertiary phase. It causes persistent lung infections and limits the ability to breathe overtime. The cells that produce mucus, digestive juices and sweat are affected severely and there is no permanent cure for the disease as its genetically inherited. Required measures taken could help increase the life span of an individual with cystic fibrosis. The following would be the nursing interventions for a 10 year old with cystic fibrosis.

• Help in clearing out the mucous from the respiratory system to prevent respiratory problems by chest physiotherapy which include postural drainage, huff coughing
• Help in clearing airways by using PEP devices which help in improving breathing
• People with any kind of infection should be restricted from coming in contact with the child
• Children suffering with this condition are at very high risk of being infected quickly therefore good hand washing techniques should be practiced by everybody before coming in contact with the patient to prevent infections
• As this condition affects the digestive system adequate nutrition should be maintained
• Provide the child with high protein, high calorie and high fat diet
• Pancreatic enzymes to be administered before all the meals and snacks. The caregivers need to be educated on this
• Check for any pain in the abdomen due to gas or bowel movements
• Encourage the child to drink plenty of water. This helps in thinning the mucous and also prevent in any kind of GI blockage/obstruction
• Educate the child and the care givers on diet to be taken, medications to be taken as prescribed
• Provide emotional support and engage the child with appropriate activities whenever required so as to treat boredom and anxiety

Above discussed are the nursing interventions for cystic fibrosis.