Nursing Care Plan (NCP) for Cystic Fibrosis

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Outline

Pathophysiology

Cystic fibrosis (CF) is an autosomal recessive genetic disorder that causes abnormalities in the secretory glands that produce mucus and sweat and mostly affects the lungs, pancreas, liver, intestines, and sex organs. The mucus that is produced in the body becomes thick and sticky. Instead of lubricating the lungs and other organs, it clogs the airways in the lungs and the ducts, most frequently in the pancreas and liver. Almost all males with CF are sterile and most females have a hard time getting pregnant. Symptoms can range from mild to severe. CF can lead to other diseases such as diabetes and osteoporosis. Screening for CF is routinely done on newborns and diagnosis is generally made before symptoms are evident, often within the first month of life.

Etiology

Cystic Fibrosis is caused by a recessive defect in the CFTR gene. The CFTR gene makes a protein that regulates the movement of salt and water through the cells. The mutation causes the protein to either not work very well, or not be produced at all. When this happens, there is no regulation of salt and water across the cell membranes and mucus that the body produces becomes dehydrated and salty.  The hallmark sign for this disease is a salty taste to the skin.

Desired Outcome

The patient will maintain adequate ventilation and respiratory status; the patient will have optimal nutritional status; the patient will be free from infection.

Cystic Fibrosis Nursing Care Plan

Subjective Data:

  • Exercise intolerance
  • Nasal congestion
  • Constipation
  • Abdominal pain

Objective Data:

  • Coughing, non-productive or with thick sputum
  • Meconium ileus
  • Failure to thrive
  • Salty taste to skin
  • Oily stools
  • Abdominal distention

Nursing Interventions and Rationales

  • Assess respiratory status; note rate, rhythm, and quality of breathing; auscultate lungs
  CF patients get frequent respiratory infections because the thick mucus in the lungs traps bacteria and becomes infected.
  • Review growth charts, assess changes or decrease in height or weight
  Children may fail to thrive and lose weight or not grow properly because of malabsorption. The body does not absorb nutrients well enough to feed the cells adequately.
  • Assess abdomen
    • Look for distention
    • Listen for active bowel sounds and gas pattern
    • Feel – palpate for mass or signs of constipation
  Lack of water regulation in the cells prevents adequate lubrication in the colon and often results in constipation. Newborns may have a meconium ileus that prevents the infant from passing meconium stools.
  • Insert and monitor nasogastric feeding tubes
  Feeding tubes may be placed to help with nutrition and absorption.
  • Monitor nutritional status
    • Caloric intake
    • Excessive thirst or hunger
    • Fiber intake
    • Use of feeding tube
  Children may have an adequate diet, but the body cannot absorb the nutrients. A blockage of the pancreas prevents digestive juices from being released into the intestines which aid in the absorption of nutrients.
  • Monitor blood glucose
  CF-related diabetes is common due to the impairment of the pancreas
  • Assess for signs of infection
    • Monitor temperature
    • Note cough with or without sputum
    • Obtain sputum culture and sensitivity
  Lung infections are common in patients with CF. Culturing the sputum can help determine bacteria involved and the course of treatment. Educate patients and caregivers that they should avoid sick contacts and stay away from other CF patients who may be ill, due to susceptibility to infections.
  • Monitor for signs of dehydration and encourage fluid and salt intake
  Patients with CF lose excess amounts of fluid and salt and can become dehydrated or develop hyponatremia quickly.
  • Assess for bone malformation, deformity or fractures
  Bones may become thin; the patient develops osteoporosis or osteopenia in the later stages. Fractures are common. Note clubbing, or widening, around the tips of the fingers and toes due to poor oxygenation of tissues
  • Administer medications and supplements appropriately
  • IV antibiotics may be given to treat infections.
  • Antipyretics may be used to manage fever.
  • Anti-inflammatories help to reduce swelling in the airways
  • Inhaled steroids and bronchodilators may be used to decrease inflammation and open airways.
  • Suction secretions as necessary per facility protocol
  Thick mucus in the lungs and weakened muscles make it difficult for patients to expel secretions. Suctioning may be required.
  • Perform chest physical therapy or vest therapy 2 – 4 times per day per facility protocol
  This technique helps loosen mucus within the lungs making it easier to expel or suction
  • Provide education for home treatment and resource information for patients and parents
  • Educate parents on how to spot signs of infection and when to seek medical help.
  • Encourage nutrition with high fiber foods and adequate fluid intake to prevent constipation.
  • Teach the importance of supplements to ensure adequate absorption of nutrients.
  • Provide information for support groups and resources as available.

Writing a Nursing Care Plan (NCP) for Cystic Fibrosis

A Nursing Care Plan (NCP) for Cystic Fibrosis starts when at patient admission and documents all activities and changes in the patient’s condition. The goal of an NCP is to create a treatment plan that is specific to the patient. They should be anchored in evidence-based practices and accurately record existing data and identify potential needs or risks.


References

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Transcript

Today we are going to be tackling cystic fibrosis. Cystic fibrosis is a genetic disorder of the secretary of glands. So the glands that secrete different mucus and sweat. It causes it to overproduce mucus, sweat, and it causes it to affect the lungs, pancreas, liver, intestines, and sex organs. The mucus is very thick and it impedes the normal processes of the body. So instead of lubricating the lungs and other organs, it actually clogs the airways and the ducts; the most, frequently affected organs are the pancreas and the liver. Some things we want to look for, we want to look for signs of infection. These patients tend to have some respiratory infections. We also want to just think in the back of our mind vest therapy, that’s some therapy that the cystic fibrosis patients undertake. We’re going to monitor the vital signs. 

We’re going to draw some labs. We’re going to administer medications as ordered. We’re going to do a good abdominal assessment, and we’re going to do a glucose check because the blocked pancreas is also affected and that can cause some diabetes. And also we’re going to monitor their fluid intake. This patient is going to maintain adequate ventilation and respiratory status as well as have optimal, nutritional status. And the cystic fibrosis patient is going to be free of infection. 

So cystic fibrosis: subjective. What is the patient telling us? Well, the patient is going to have some exercise intolerance. They’re going to get tired and that’s because a lot of their ducts are blocked. So their lungs are going to be tired. They’re going to have some nasal congestion. Remember the thick mucus. They’re also going to have some constipation because their GI tract is going to also be blocked and they are going to have some abdominal pain. 

Okay. Um, some objective things that we’re going to observe as nurses, we’re going to see coughing, but it’s going to be non productive. Or if it’s productive, it’s going to be thick sputum. They’re going to have what we call meconium ileus. If you remember, meconium is that first stool that is passed as a child. So they’re going to have ileus, which is, it’s not going to come. They’re going to have a failure to thrive. These patients also have what we call a salty taste to the skin, and they’re going to have oily stools and some abdominal distention. So what are some things that we can do as nurses and intervene? Well, the first thing I think we should do is assess the respiratory status. Very important that we note a few things. We want to note the rate, the rhythm and the quality of breathing, because those things can give us some good information. 

So cystic fibrosis patients tend to get frequent respiratory infections because of the thick mucus that traps bacteria. We want to perform chest physical therapy or vest therapy, two to four to four times a day. And that’s all based on the facility’s protocol. We’re going to look at the abdomen. We’re going to do a good abdominal assessment. We’re going to feel or palpate for signs of constipation. In these patients, the mucus that usually lubricates the GI tract becomes an area for distension and constipation. Okay. We’re going to monitor their blood sugar. These patients tend to have increased blood glucose levels. CF related diabetes is very common. 

We’re going to, again, assess for signs of infection. So because lung infections are common, we’re going to culture the sputum. We’re going to have determined which bacteria is involved. If an infection is present, because we want to be able to pick the correct antibiotics in order to develop a course of treatment. Last, but certainly not least, we are going to monitor for signs of dehydration, and we’re going to encourage fluid and salt intake. So patients with cystic fibrosis tend to lose an excess amount of fluids and salt. So salt leaves and fluid goes right behind it. They can become dehydrated or develop hyponatremia, which is another word for low sodium. They can develop low sodium and that develops quickly. We want to avoid all of those complications. We talked about this, but we want to make sure that we perform the vest therapy two to four times a day. Like I said, per the protocol, this technique is just shaking up the body and loosening up that thick mucus and making it easy to either suction out or expel with a cough. 

Here are some key points that we want to focus on when we are taking care of the cystic fibrosis patient. Okay. Remember, cystic fibrosis is an autosomal, recessive genetic disorder. Okay. It just makes your secretary glands produce thick mucus. Okay. The subjective and objective details that we want to focus on are subjective. These patients are going to have some exercise and tolerance, constipation, abdominal pain, and congestion. Objective: we’re going to notice a few things. Few of them, a meconium ileus, and abdominal distension, as well as increased blood sugar. So let’s add that here. Increase B G blood glucose. We’re going to monitor glucose for CF related diabetes and best therapies should be done two to four times per day. That’s going to help break up that thick mucus. We love you guys; go out and be your best self today. And, as always, happy nursing.

 

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