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Course: Pediatric Nursing

Topic: Nursing Care of Child with

Cystic Fibrosis_ Respiratory Disorder (Part VII)

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Module Goals

Learners will be able to:

Define Cystic Fibrosis
Identify cause, risk factors associated with cystic fibrosis in children
List common diagnostic procedures for Cystic fibrosis
Define the common treatment of cystic fibrosis
Describe nursing management of children experiencing Cystic fibrosis

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Cystic Fibrosis

Cystic fibrosis is an inherited condition that causes thick tenacious ‘sticky’ mucus to build up in the lungs and digestive system.

This causes lung infections and problems with digesting food.

National Health, Lungs, and Blood Institute, 2022

NHS, 2021

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Causes

Caused due to gene default; faulty gene affects the movement of salt and water in and out of cells.

This results in a build-up of thick, sticky mucus in the body's tubes and passageways – particularly the lungs and digestive system.

National Health, Lungs, and Blood Institute, 2022

NHS, 2021

Contact info: info@nursesinternational.org

Cause of cystic fibrosis: Cystic fibrosis is a genetic condition. It's caused by a faulty gene that affects the movement of salt and water in and out of cells. This, along with recurrent infections, my result in a build-up of thick, sticky mucus in the body's tubes and passageways – particularly the lungs and digestive system.

A person with cystic fibrosis is born with the condition. It's not possible to "catch" cystic fibrosis from someone else who has it.

How cystic fibrosis is inherited

To be born with cystic fibrosis, a child has to inherit a copy of the faulty gene from both of their parents.

This may happen if the parents are "carriers" of the faulty gene, which means they don't have cystic fibrosis themselves.

If both parents are carriers, there's a:

1 in 4 chance their child won't inherit any faulty genes and won't have cystic fibrosis or be able to pass it on
1 in 2 chance their child will inherit a faulty gene from one parent and be a carrier
1 in 4 chance their child will inherit the faulty gene from both parents and have cystic fibrosis

If one parent has cystic fibrosis and the other is a carrier, there's a:

1 in 2 chance their child will be a carrier
1 in 2 chance their child will have cystic fibrosis

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Critical Thinking Question

Cystic fibrosis is a genetic disorder, and symptoms depend on the organs affected.

Which of the following systems are majorly affected by the cystic fibrosis? (Select all that apply)

Circulatory
Nervous System
Digestive system
Respiratory System

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Cystic Fibrosis: Signs and Symptoms

Recurring chest infections
wheezing, coughing, shortness of breath and bronchiectasis
Difficulty putting on weight and growing, low BMI
Jaundice
Gastrointestinal problems: Diarrhea, constipation, smelly poo
A bowel obstruction in newborn babies
Malnutrition
Muscle or joint pain
Salty skin (saltier than normal perspiration)

National Health, Lungs, and Blood Institute, 2022

NHS, 2021

Contact info: info@nursesinternational.org

Symptoms of cystic fibrosis

The build-up of sticky mucus in the lungs may cause breathing problems and increases the risk of lung infections. Over time, the lungs may stop working properly.

Mucus also clogs the pancreas (the organ that helps with digestion), which stops enzymes reaching food in the gut and helping with digestion.

This means most children with cystic fibrosis don't absorb nutrients from food properly and need to eat more calories to avoid malnutrition.

Cystic fibrosis most commonly affects the lungs. Some people who have cystic fibrosis may have wheezing and a cough that may produce mucus or blood.

Other symptoms depend on the organs affected and may include:

Blockage of the intestine in a baby soon after birth
Clubbing of fingers and toes due to less oxygen getting to the hands and feet
Fever, which may include night sweats
Gastrointestinal symptoms, such as severe abdominal pain, chronic diarrhea, or constipation
Jaundice, or yellow skin, for an abnormally long time after birth
Low body mass index (BMI) or being underweight
Muscle and joint pain
Delayed growth or puberty
Salty skin and saltier than normal sweat
Sinus infections

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Cystic Fibrosis and Malnutrition

Epithelial cells (found in sweat glands, pancreas, lungs) help regulate the balance of salt and water
CF adversely impacts the body’s epithelial cells
In children with CF, mucus become sticky and thick
This can cause significant problems with breathing, nutrient absorption, and digestion.
Children with cystic fibrosis develops endocrine pancreatic insufficiency due to excessive mucus in pancreas

Whelan, 2020

Contact info: info@nursesinternational.org

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Cystic Fibrosis and Malnutrition (Continued)

Pancreatic insufficiency leads to digestion problems with as pancreatic enzymes are unable to reach the intestines
This blocks absorption of fat, protein, and carbohydrates, along with the vitamins and minerals they contain.
This may ultimately lead to malnutrition and growth delays

Whelan, 2020

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Diagnosis

Newborn spot test (Heel Prick Test) shortly after birth

If the test suggests cystic fibrosis, additional tests include:

Sweat test – to measure the amount of salt in sweat ( will be abnormally high in someone with cystic fibrosis)

Genetic test – a sample of blood or saliva is checked for the faulty gene that causes cystic fibrosis

National Health, Lungs, and Blood Institute, 2022

NHS, 2021

Contact info: info@nursesinternational.org

Screening for cystic fibrosis

Genetic testing may be performed to look for carriers, as well as to screen relatives of people who have cystic fibrosis. Genetic testing may also be used as prenatal screening tool to look for a mutated CFTR gene. All newborns in the United States are now screened for cystic fibrosis. Since universal screening for cystic fibrosis began relatively recently, there are still young people and adults who have not been screened.

Carrier screening to detect CFTR mutations

Genetic testing may tell if someone is carrying a mutation of the CFTR gene. This is called carrier testing. People who have inherited a mutation of the CFTR gene from one parent are cystic fibrosis carriers. People who have inherited a mutation of the CFTR gene from both parents will have cystic fibrosis.

Siblings of a person who has cystic fibrosis may want to be tested for cystic fibrosis whether or not they have symptoms. Other relatives, such as first cousins and half-siblings, may be tested if they have symptoms, or if the family is concerned that the individual may have cystic fibrosis.

After a positive screening test, the diagnosis should be confirmed with further testing.

Prenatal screening

Couples who are planning to have children may want to be tested to see if they are cystic fibrosis carriers. Genetic testing, such as the carrier screening described above, may be done before or during pregnancy. Similar to standard genetic testing, prenatal screening uses a sample of blood, saliva, or cells from the inside of the mother/father cheek to check the DNA.

Newborn screening

When a child has cystic fibrosis, it is very important to diagnose it early to help prevent complications. Newborn screening for cystic fibrosis is performed during a baby’s first 2 to 3 days of life. A few drops of blood from a heel prick are placed on a special card and analyzed in labs. After a positive screening test, the diagnosis should be confirmed with further testing.

Sweat test

A sweat test checks for high levels of chloride in the child’s sweat. The sweat test is the standard test for diagnosing cystic fibrosis. It may be used if the child has symptoms that may indicate cystic fibrosis or to confirm a positive diagnosis from a screening of the newborn baby.

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Cystic Fibrosis: Complications

Have higher risk of developing other infections:

Osteoporosis
Diabetes Mellitus
Nasal Polyps and sinus infections
Liver diseases/problems
Fertility problems

National Health, Lungs, and Blood Institute, 2022

NHS, 2021

Contact info: info@nursesinternational.org

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Critical Thinking Question

Which symptom in a newborn might indicate CF?

Stools that are pale, foul, or float
Rapid breathing
Jaundice
Bowel obstruction

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Cystic Fibrosis: Treatment

No known cure
Treatments control symptoms, prevent complications and increase quality of life
May need lifelong medications to treat and prevent lung diseases
Physical activities and airway clearance techniques needed to help clear mucus from lungs

National Health, Lungs, and Blood Institute, 2022

NHS, 2021

Contact info: info@nursesinternational.org

Treatments for cystic fibrosis

There's no cure for cystic fibrosis, but a range of treatments may help control the symptoms, prevent or reduce complications, and make the condition easier to live with.

People with cystic fibrosis may need to take different medicines to treat and prevent lung problems.

Physical activity and the use of airway clearance techniques may also be recommended to help clear mucus from the lungs.

Medicines to treat cystic fibrosis include those used to maintain and improve lung function, fight infections, clear mucus and help breathing, and work on the faulty CFTR protein.

Antibiotics prevent or treat lung infections and improve lung function. Doctor may prescribe oral, inhaled, or intravenous (IV) antibiotics.
Anti-inflammatory medicines, such as ibuprofen or corticosteroids, reduce inflammation. Inflammation causes many of the changes in cystic fibrosis, such as lung disease. Ibuprofen is especially beneficial for children, but side effects may include kidney and stomach problems. Corticosteroids may cause bone thinning and increased blood sugar and blood pressure.
Bronchodilators relax and open airways. These treatments are taken by inhaling them.
CFTR modulators improve the function of the faulty CFTR protein. They improve lung function and help prevent lung problems and other complications. Examples include ivacaftor and lumacaftor and a triple combination medicine (elexacaftor–tezacaftor–ivacaftor). The combination medicine is the first approved treatment that may help up to 90% of people who have cystic fibrosis. It is currently approved to use in adults and children older than 12 years.
Mucus thinners make it easier to clear the mucus from the airways. These treatments are taken by inhaling them.

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Cystic Fibrosis: Treatment

Surgery may be an option for people with advanced conditions:

Lung transplant: with advanced lung disease and respiratory failure.
Liver transplant: Option for advanced liver disease such as cirrhosis

Balanced nutrition:

High-calorie diet
Vitamin and mineral supplements, digestive enzyme capsules to help with digestion and prevent malnutrition

National Health, Lungs, and Blood Institute, 2022

NHS, 2021

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Nurses’ Role for Hospitalized Child

Improve airway clearance

Suctioning
Chest physiotherapy
Deep breathing exercises
Teach effective coughing

Administer prescribed medications
Prepare child and family for laboratory, diagnostic testing or surgery
Ensure hydration: Monitor and record intake and output

Belleza, 2021

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Nurses’ Role for Hospitalized Child

Ensure adequate nutrition:

High calorie food with vitamins and mineral supplements
Improve ventilation and breathing:

High fowler’s position
Assess oxygen level
Administer oxygen when as needed
Encourage or perform mouth care every 3-4 hours
Age appropriate diversional activities
Gentle exercises to loosen mucus
Nebulization

Belleza, 2021

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Nurses’ Role for Hospitalized Child

Prevent infection:

Good hand hygiene
Standard precautions
Restrict visitors

Address and reduce child’s anxiety

Age appropriate activities, allow favorite toys/blanket
Allow caregivers/parents by their side,

Provide support to the family: Provide space to vent their anxiety and concerns

Belleza, 2021

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Client Education

Importance of health lifestyles: Health food, vitamin supplements and active lifestyle
Chest physiotherapy and postural drainage
Diseases and its process (delayed development)
Prevent infection (Hand washing, maintaining distance, avoiding crowds)
Encourage to join the support groups
Encourage to remain in regular check ups and follow ups

Belleza, 2021

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Dietary Needs

Energy needs of people with CF are estimated to be 1 ½ to 2 times the needs of those without CF
Enteral/parenteral nutrition might be required for the hospitalized children
Enzyme Supplements, Vitamins and Salt:

Enzymes: Pancreatic enzymes to absorb and digest fat and protein
Vitamin like: A, D, E, K and extra calcium
Extra table salt for children in hot climates

Icahn School of Medicine at Mount Sinai, 2020

John Hopkins n.d.

Reed, 2020

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Dietary Needs (Continued)

Eating strategies:

Provide small but several frequent meals a day
Provide variety of foods
Provide small snacks between meals
Encourage child to eat regularly

Icahn School of Medicine at Mount Sinai, 2020

John Hopkins n.d.

Reed, 2020

Contact info: info@nursesinternational.org

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Cystic Fibrosis: Dietary Needs (Continued)

Adding high calorie and high protein foods

Cheese, whole milk, butter
Yogurt
Breaded meat, chicken, fish
Hard boiled eggs, cheese cubes
Shrimps, ground beefs, diced ham
Raisins, dates or chopped nuts
Brown sugar

Icahn School of Medicine at Mount Sinai, 2020

John Hopkins n.d.

Reed, 2020

Contact info: info@nursesinternational.org

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Case Study

A child is admitted to the unit with cystic fibrosis.

The nurse should understand that the genetic defect of cystic fibrosis causing which changes in mucus secretions?

Excessive mucus secretion that is thin and watery
Excessive mucus secretion that has blood stains
Excessive mucus secretion that is thick and sticky
Thick, yellow and sticky mucus secretion

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Red Flags

Osteoporosis
Diabetes
Nasal polyps and sinus infections
Liver problems
Fertility problems

Best-Brandt, 2019

Contact info: info@nursesinternational.org

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Cultural Considerations

Religion, culture, beliefs, and ethnic customs may influence how families understand and use health concepts:

Health beliefs: In some cultures talking about a possible poor health outcome will cause that outcome to occur.
Health customs: In some cultures family members play a large role in health care decision-making.
Ethnic customs: Differing gender roles may determine who makes decisions about accepting & following treatment recommendations.

AHRQ, 2020

Contact info: info@nursesinternational.org

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Cultural Considerations (con.):

Religion, culture, beliefs, and ethnic customs may influence how families understand and use health concepts:

Religious beliefs: Faith and spiritual beliefs may affect health seeking behavior and willingness to accept treatment.
Dietary customs: Dietary advice may be difficult to follow if it does not fit the foods or cooking methods of the family.
Interpersonal customs: Eye contact or physical touch may be ok in some cultures but inappropriate or offensive in others.

AHRQ, 2020

Contact info: info@nursesinternational.org

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References

Andrews, M.M., Boyle, J.S. & Collings, J. W. (2020). Transcultural Concepts in Nursing Care (8th Ed.). Wolters Kluwer, Philadelphia, PA.

Belleza, M. (2021, February 11). Cystic Fibrosis. https://nurseslabs.com/cystic-fibrosis/

Icahn School of Medicine at Mount Sinai. (2020, May 27). Cystic fibrosis - nutrition. https://www.mountsinai.org/health-library/nutrition/cystic-fibrosis-nutrition#:~:text=Cystic%20fibrosis%20is%20a%20disease,from%20the%20foods%20you%20eat.

John Hopkins. (n.d. ). Cystic Fibrosis: Diet and Nutrition. https://www.hopkinsallchildrens.org/Patients-Families/Health-Library/HealthDocNew/Cystic-Fibrosis-Diet-and-Nutrition

NHLBI- National Health, Lungs, and Blood Institute. (2022, March 24). Cystic Fibrosis: What is Cystic Fibrosis? https://www.nhlbi.nih.gov/health/cystic-fibrosis

Contact info: info@nursesinternational.org

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References

NHS- National Health Services. (2021, March 16). Cystic Fibrosis: Overview. https://www.nhs.uk/conditions/cystic-fibrosis/

Reed, M. (202, January). Cystic Fibrosis and Nutrition. https://kidshealth.org/en/parents/cf-nutrition.html

Whelan, C. (2020, August 3). What’s in a Cystic Fibrosis-Friendly Diet? https://www.healthline.com/health/cystic-fibrosis-diet

Contact info: info@nursesinternational.org

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