Care of Mother and Child at Risk ( Acute and Chronic )

NCM 109

Report Manuscript
“CYSTIC FIBROSIS”
Submitted by :

Olantes, Josiah

Agarpao, Maria Angelika

Cimatu, Alexandria

Espejo, Heleighna

Gerada, Valerie

Navarro, Rose Anne

Recta, Cesmari

Soliva, Marie Liz

Vergara, April Joy

Villanueva, Jullana

Submitted to:
Mrs. Marilyn M. Racca
Instructor
Definition:

Cystic fibrosis (CF) is a genetic disorder that primarily affects the lungs and digestive system. It

is caused by mutations in the CFTR gene, which regulates the flow of salt and fluids in and out

of cells. These mutations lead to the production of thick and sticky mucus, which clogs the

airways and traps bacteria, leading to frequent lung infections and difficulty breathing. CF also

affects the pancreas, causing digestive problems due to the thickened mucus blocking the ducts

that carry digestive enzymes.

Statistics:

 Cystic fibrosis affects approximately 70,000 people worldwide.

 In the United States, about 30,000 people live with cystic fibrosis.

 It is estimated that 1 in every 3,500 newborns in the US is born with cystic fibrosis. There

are about 30,000 people with cystic fibrosis in the United States.

Year of Birth Median Predicted Survival Age

1993-1997 31

2003-2007 37

2013-2017 44

 There is no cure for CF, but treatment is available. Because of improved care, the average

life expectancy has been steadily increasing since the 1950s and is currently close to 40

years.
Risk

Factors:

Cystic Fibrosis (CF) is due to a mutation in the cystic fibrosis transmembrane conductance

regulator (CFTR) gene, which results in dysfunction of the CFTR protein.

1. Age of Parent - Advanced maternal age has been associated with a slightly higher risk of

having a child with cystic fibrosis.

2. Genetics - Both parents must carry a mutated CFTR gene for a child to inherit cystic

fibrosis. If both parents are carriers, there is a 25% chance with each pregnancy that the

child will have cystic fibrosis.

3. Ethnicity - Cystic fibrosis is more common in Caucasians of Northern European descent,

but it can affect individuals of any race or ethnicity.

Signs and Symptoms:

In pediatric patients, signs and symptoms of cystic fibrosis may include:

 Persistent coughing

 Frequent lung infections, such as pneumonia or bronchitis

 Wheezing or shortness of breath

 Poor weight gain

 Difficulty passing stools or constipation

 Frequent sinus infections

 Infants may experience intestinal blockage (meconium ileus) shortly after birth.
Pharmacologic Treatment:

 Bronchodilators

- Works by binding to receptors on the smooth muscle cells of the airways.

- Examples: Albuterol and Ipratroprium

 Mucolytics

- Works by breaking down the long strands of DNA found in thick mucus, thereby

reducing its viscosity and making it easier to clear from the airways.

- Example: Pulmozyme/Dornase Alfa

 Antibiotics

-Works by targeting and killing bacteria that cause respiratory infections in cystic fibrosis

patients.

- Example: Azithromycin and Tobramycin

 Pancreatic Enzyme Replacement Therapy (PERT)

- Provides supplemental pancreatic enzymes, including lipase, protease, and amylase, to

replace those that are deficient in cystic fibrosis patients with pancreatic insufficiency.

- Examples: Creon and ZenPep

 CFTR Modulators

- Target the underlying cause of cystic fibrosis by improving the function of the defective

CFTR protein.

- Ivacaftor/Kalydeco, lumacaftor/ivacaftor/Orkambi, tezacaftor/ivacaftor/Symdeko,

elexacaftor/tezacaftor/ivacaftor/Trikafta
Laboratory Treatment:

 Sweat Test – to measure the amount of salt in sweat, as the sweat of someone with

cystic fibrosis has higher levels of salt than normal. It’s done by placing a solution on

the forearm (or the thigh, if your child is too small) and attaching electrodes. The skin

is stimulated to sweat with a mild electric current. This does not cause pain or harm

your child. The sweat is collected onto a gauze pad and checked. Higher than normal

amounts of chloride may suggest CF. The sweat test is not painful. It often causes

only minor discomfort.

 Genetic Test – where a sample of blood or saliva is checked for the faulty gene that

causes cystic fibrosis. Blood, or cells taken from a cheek scraping, can be tested for

mutations in the CFTR gene.

Nursing Management

NURSING CARE PLAN

Nursing Diagnosis:

 Ineffective airway clearance related to increased mucus production, inflammation

of the bronchioles and infectious process as evidenced by persistent coughing

and wheezing or shortness of breath.

Nursing inference:

 It implies that the patient is struggling to expel mucus effectively, leading to

compromised airway function. It may include administering bronchodilators and

mucolytics to address mucus viscosity, promoting effective coughing techniques, and

providing chest physiotherapy to aid in mucus mobilization.

Nursing Goal:

 Patient will maintain a patent airway and will demonstrate an improvement in airway

clearance as observed by vital signs and chest x-ray within normal limits.

Nursing Intervention

Interventions Rationale

Independent

Assist with airway clearance Help with clearing the airway by assisting

moving mucous out of the lungs that can be

done by patient education and breathing

exercise such as huff coughing.

Position accordingly Proper positioning makes a huge difference in

clearing the airways. Chest x-rays can help

determine where secretions are in the lungs so

the patient can position for the mobilization of

mucus.

Dependent

Administer bronchodilators, mucolytics and Hypertonic saline is commonly given inhaled

antibiotics as ordered and thins the mucus. Guaifenesin is an

expectorant and will help cough up the mucus.

Bronchodilators should be given before

hypertonic saline as these will open the

airways and make coughing up mucus easier.

Antibiotics are given to control the infection.

Collaborative

Collaborate with a physical therapist A physical therapist is a great help in chest

physiotherapy for they have a huge knowledge

in breathing exercises.
Nursing Diagnosis:

 Imbalanced nutrition related to malabsorption and nutrient deficiencies as evidenced by

poor weight gain and difficulty passing stools or constipation.

Nursing Goal:

 Patient will display improvement in nutritional status as evidenced by lab work.

Nursing Intervention:

Interventions Rationale
Independent
Provide a high calorie, protein and fat diet Patients require high-calorie and high-protein

diets. Encourage dairy, breaded meats, peanut

butter, pasta, and eggs.

Dependent
Administer pancreatic enzymes as ordered Pancreatic enzymes help the body digest

carbs, fats, and protein. These should be taken

before eating to aid digestion.

Collaborative
Collaborate with a dietician Dieticians can assist with finding the correct

amount of enzymes required as well as

recommending additional vitamins and

supplements and adding calories to the diet.

Pathophysiology:

Cystic Fibrosis
Non-Modifiable Risk Factors: Modifiable Risk Factors:
Family History/Genetic Smoking/drug abuse
Old age between 40-60y/o Exposure to dust or other fumes
Pre-existing asthma condition Allergens

Genetic Mutations (CFTR)

Defective chloride transport

Increase viscosity of mucus

Pancreatic insufficiency Airway obstruction and inflammation

Malnutrition and failure to thrive Recurrent infections

Systemic manifestation

Signs/Symptoms:
 Persistent coughing
 Frequent lung infections, such as pneumonia or
bronchitis
 Wheezing or shortness of breath
 Poor weight gain
 Difficulty passing stools or constipation
 Frequent sinus infections
 Infants may experience intestinal blockage
(meconium ileus) shortly after birth.
References:

Faap, G. D. S. M. F. (n.d.). Cystic Fibrosis Medication: Enzymes, Pancreatic, Vitamins,

Bronchodilators, Mucolytic Agents, CFTR Potentiators and Correctors, Antibiotics.

https://emedicine.medscape.com/article/1001602medication?

fbclid=IwAR0dC7JG9JpIrZ8D8owOCSMxmebCThKKM1xWd436-

VsxGzdUKrQfg4hBV0w&form=fpf

Diagnosis of Cystic Fibrosis. (n.d.). Stanford Medicine Children’s Health.

https://www.stanfordchildrens.org/en/topic/default?id=diagnosis-of-cystic-fibrosis-90-

P02931&fbclid=IwAR0OjRagQHt6N1aembBKYiAcgvWp0-mp6guuPAPU96vfhj34nBImiJj0K-

Bsn, P. M. (2023, October 12). 7 Cystic Fibrosis Nursing Care Plans. Nurseslabs.

https://nurseslabs.com/cystic-fibrosis-nursing-care-plans/?

fbclid=IwAR3iz49BAVQu2189_Pwg9Aer7M7wZ9jdzFfHB9qd-YaTZvToj_inBImWXIM

What Is Cystic Fibrosis? | NHLBI, NIH. (2023, November 21). NHLBI, NIH.

https://www.nhlbi.nih.gov/health/cysticfibrosis#:~:text=Cystic%20fibrosis%20(CF)%20is

%20a,and%20other%20organs%20and%20tissues.

About Cystic Fibrosis. (n.d.). Cystic Fibrosis Foundation. https://www.cff.org/intro-cf/about-

cystic-fibrosis

Cystic fibrosis - Symptoms and causes - Mayo Clinic. (2021, November 23). Mayo Clinic.

https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700