Cystic Fibrosis (Ricci Ch 40)

What medication is given daily to decrease sputum viscosity and help clear secretions?

Recombinant human DNase (Pulmozyme)

The sweat glands produce a larger amount of chloride, leading to...

a salty taste of the skin and alterations in electrolyte balance and dehydration

During percussion, encourage the child to

deep breathe and cough

In infants, breastfeeding should be continued with

enzyme administration

Common signs and symptoms reported during the health history in the undiagnosed child might include:

- A salty taste to the child's skin (resulting from excess chloride loss via perspiration) - Meconium ileus or late, difficult passage of meconium stool in the newborn period - Abdominal pain or difficulty passing stool (infants or toddlers might present with intestinal obstruction or intussusception at the time of diagnosis) - Bulky, greasy stools - Poor weight gain and growth despite good appetite - Chronic or recurrent cough and/or upper or lower respiratory infections

Palpation

- Palpation might yield a finding of asymmetric chest excursion if atelectasis is present. - Tactile fremitus may be decreased over areas of atelectasis. - Note if tenderness is present over the liver (might be an early sign of cor pulmonale).

Percussion

- Percussion over the lung fields usually yields hyperresonance due to air trapping. - Diaphragmatic excursion might be decreased. - Percussion of the abdomen might reveal dullness over an enlarged liver or mass related to intestinal obstruction.

Children known to have cystic fibrosis are often admitted to the hospital for pulmonary exacerbations or other complications of the disease. The health history should include questions related to:

- Respiratory status: has cough, sputum production, or work of breathing increased? - Appetite and weight gain - Activity tolerance - Increased need for pulmonary or pancreatic medications - Presence of fever - Presence of bone pain - Any other changes in physical state or medication regimen

Inspection

- check nasal passages for polyps - note respiratory rate, work of breathing, use of accessory muscles, position of comfort, frequency and severity of cough, and quality and quantity of sputum produced - the child with cystic fibrosis often has a barrel chest - clubbing of the nail beds might also be present - Note whether rectal prolapse is present. - does the child appear small or thin for his or her age? - The child might have a protuberant abdomen and thin extremities, with decreased amounts of subcutaneous fat. - Observe for the presence of edema (sign of cardiac or liver failure). - Note distended neck veins or the presence of a heave (signs of cor pulmonale).

Auscultation

- may reveal a variety of adventitious breath sounds - Fine or coarse crackles and scattered or localized wheezing might be present - With progressive obstructive pulmonary involvement, breath sounds might be diminished - Tachycardia might be present. - Note the presence of a gallop (might occur with cor pulmonale). - Note the adequacy of bowel sounds.

Percuss each segment of the lung for ____________ minutes.

1 to 2

_______________________ is often used as an adjunct therapy in respiratory illnesses, but for children with cystic fibrosis it is a critical intervention.

Chest physiotherapy

What is the responsible gene mutation?

A deletion occurring on the long arm of chromosome 7 at the cystic fibrosis transmembrane regulator (CFTR)

Supplementation with vitamins ________________ is necessary.

A, D, E, and K

__________________ are often prescribed and may be given at home as well as in the hospital.

Aerosolized antibiotics

___________________ can be given at home as well as in the hospital.

Aerosolized antibiotics; Children with frequent or severe respiratory exacerbations might require lengthy courses of intravenous antibiotics.

Most common debilitating disease of childhood among those of

European descent (Caucasians)

What kind of diet is recommended?

Increased-calorie, high-protein diets

What medications are also prescribed for some children?

Inhaled bronchodilators and anti-inflammatory agents

________________ must be administered with all meals and snacks to promote adequate digestion and absorption of nutrients.

Pancreatic enzyme supplements (pancrelipase)

What is common with Cystic Fibrosis?

Nasal polyps and recurrent sinusitis

_________________________ are prescribed to promote adequate digestion and absorption of nutrients and optimize nutritional status.

Pancreatic enzymes and supplemental fat-soluble vitamins (A, D, E, and K)

____________________ involves exhaling through a flow resistor, which creates positive expiratory pressure. The cycles of exhalation are repeated until coughing yields expectoration of secretions.

Positive expiratory pressure therapy

Ensure that ______________ is administered, as well as ___________________________ if prescribed

Pulmozyme; inhaled bronchodilators and anti-inflammatory agents

Secondary bacterial infection with ______________________________ often occurs.

Staphylococcus aureus, Pseudomonas aeruginosa, and Burkholderia cepacia

What can also become become obstructed by viscous mucus and eosinophilic material?

The pancreas, intrahepatic bile ducts, intestinal glands, gallbladder, and submaxillary glands

True or false: Lung transplantation has been successful in some children with cystic fibrosis.

True

What is critical to prevent infection?

Vigorous pulmonary hygiene for mobilization of secretions

The dosage of pancreatic enzyme can be adjusted until

an adequate growth pattern is established and the number of stools is consistent at one or two per day.

Cystic Fibrosis

autosomal recessive disorder that affects 30,000 children and adults in the United States

When percussing, you should put what part of the hand on the lung segment and keep what straight? Vibrate by doing what with your arms during the child's exhalation? Vibrate each lung segment for at least _____ exhalations.

ball of the hand; arm and shoulder; tensing and relaxing your arms; five

Abnormally thick mucus plugs the small airways, and then ______________________ occur

bronchiolitis and further plugging of the airways

All children with cystic fibrosis who have pulmonary involvement require

chest physiotherapy with postural drainage several times daily to mobilize secretions from the lungs

The epithelial cells fail to conduct _______________, and ________________ abnormalities occur

chloride; water transport

the CFTR mutation causes alterations in ______________ on _________________, resulting in generalized dysfunction of the _________________.

epithelial ion transport; mucosal surfaces; exocrine glands

Pancreatic enzyme activity is lost and malabsorption of _____________________ occurs, resulting in _________________

fats, proteins, and carbohydrates; poor growth and large, malodorous stools.

The __________________ provides high-frequency oscillation to the airway as the child exhales into a mouthpiece that contains a steel ball.

flutter valve device

Complications include

hemoptysis, pneumothorax, bacterial colonization, cor pulmonale, volvulus, intussusception, intestinal obstruction, rectal prolapse, gastroesophageal reflux disease, diabetes, portal hypertension, liver failure, gallstones, and decreased fertility

Children will need additional enzyme capsules when

high-fat foods are being eaten

Appropriate percussion yields a _____________ sound, not a ___________ sound.

hollow; slapping

Chest physiotherapy may be preceded by an __________________ and should not be performed ___________________.

inhalation treatment; after eating

Take note!!! Children 6 and older who have the G551D, G1244E, G1349D, G178R, G551 S, S1251 N, S1255P, S549 N, or S549R mutation of the cystic fibrosis gene may be prescribed _______________.

ivacaftor

Median age for survival is

late 30s

Therapeutic management is aimed toward

minimizing pulmonary complications, maximizing lung function, preventing infection, and facilitating growth

Nursing management focuses on

minimizing pulmonary complications, promoting growth and development, and facilitating coping and adjustment by the child and family

Secondary bacterial infection can contribute to ____________________, leading to ______________________

obstruction and inflammation; chronic infection, tissue damage, and respiratory failure

In the infant or young child, the enzyme capsule can be

opened and sprinkled on cereal or applesauce

Screening available for

preconception or prenatal care and newborn

Chest physiotherapy involves percussion, vibration, and postural drainage, and either it or another bronchial hygiene therapy must be performed ___________ times a day to assist with mobilization of secretions.

several

The number of pancreatic enzyme capsules required depends on

the extent of pancreatic insufficiency and the amount of food being ingested

Water transport abnormalities result in

thickened, tenacious secretions in the sweat glands, gastrointestinal tract, pancreas, respiratory tract, and other exocrine tissues; the increased viscosity of these secretions makes them difficult to clear

Use of ivacaftor results in ______________________. It is the first drug to act directly on the defective gene.

thinning of lung mucous, resulting in easier airway clearance via coughing

Some children may require ____________ to maintain or gain weight.

total parenteral nutrition

Excess mucus is produced by the ______________ glands.

tracheobronchial

The ______________________ provides high-frequency chest wall oscillation to increase airflow velocity to create repetitive cough-like shear forces and to decrease the viscosity of secretions.

vest airway clearance system

A ___________________________ is necessary to ensure adequate growth

well-balanced, high-calorie, high-protein diet