Chapter 23: Cystic Fibrosis

Question 1

Cystic Fibrosis (CF)

Answer 1

inherited autosomal-recessive disorder that causes the production of thick mucus that blocks exocrine glands and affects several body systems, including respiratory, gastrointestinal, and reproductive systems

• most common cause of chronic respiratory disease in children
• accompanied by multiple, severe respiratory infections
• the increased mucus production in the airways causes obstruction and stasis of fluid, providing a rich habitat for bacterial growth
• the pancreatic ducts are often blocked by mucus, prohibiting the secretions of pancreatic enzymes necessary for the metabolism of food nutrients
• in later childhood, the reproductive system is affected b/c ovarian ducts and the vas deferens may be occluded, leading to infertility
• also an increased loss of sodium , causing salt depletion in children
• more common in Caucasians

Question 2

How is CF Transmitted

Answer 2

transmitted as an autosomal-recessive trait, which means that a child can receive a defective gene from either parent

• when both parents carry the defective gene, there is a 75% chance that the child will inherit one CF gene from each parent and manifest the disease
• there is a 50% chance that the child will inherit one defective gene and one normal gene from each parent and become a carrier of the disease
• there is a 25% chance that the child will inherit only normal genes and be free of CF

Question 3

Where is the CF gene found?

Answer 3

been found on chromosome 7, which encodes cystic fibrosis transmembrane conductor regulator protein (CFTR)
-CTFR normally regulates the chloride channel and facilitates at the cell surface; abnormal functions of CFTR cause a disruption of sodium ion transport across the exocrine and epithelial gland cells and make the cell walls impermeable to chloride ions; this causes an excess of sodium and chloride found in the sweat of children affected by CF
>the loss of sodium and water from the airways increases the viscosity of the mucus and disrupts the ciliary mechanism (hair-like process) that is intended to clear the airways, predisposing the child to recurrent respiratory infections

Question 4

CF and the Transport Defect in the Pancreatic and Bile Ducts

Answer 4

with inadequate excretion of pancreatic enzymes for food breakdown, children experience varying levels of protein and fat absorption

• with reduced protein and fat absorption, there is weight loss and failure to thrive, requiring an affected child’s diet to be high in protein and calories
• fat is excreted in the stool, resulting in abnormal bowel patterns, including steatorrhea (presence of excess fat in feces), diarrhea, and abdominal pain

Question 5

CF and Meconium

Answer 5

the mucus gland produces thin, free-flowing secretions, but in CF it produces thick mucus that accumulates and obstructs the different organs

• in newborns, thick secretions may plug the small intestine and lead to failure in passing meconium (the first feces of a newborn infant, which is greenish black, odorless, and tarry)
• in the GI system, thick secretions impair the digestive system and lead to malnutrition in childhood

Question 6

Meconium Ileus

Answer 6

may be the initial presentation of CF in the neonate

• demonstrated by a meconium stool that is described as thick and sticky and present in 15% of newborns with CF
• may cause obstruction which requires surgical removal

Question 7

Signs + Symptoms

Answer 7

-wide range
-the severity of symptoms varies from child to child; b/c CF is a multisystem disease (failure of two or more organ systems), the symptoms are presented according to the body system affected
>Gastrointestinal Tract:
-Meconium ileus, prolonged jaundice, steatorrhea, rectal prolapsed
>Respiratory Symptoms:
-cracks, wheezes, diminished breath sounds, dry productive cough, tachypnea, hypoxia, and cyanosis
>Integumentary Symptoms:
-salty-tasting tears and skin
>Secondary Consequences:
-dehydration, malnutrition, intestinal atresia, idiopathic pancreatitis, biliary cirrhosis, cholestasis, emphysema and atelectasis, prolonged hypoxia, hemoptysis, bacterial pneumonia, diabetes, anemia, failure to thrive, osteoporosis
>Common Characteristics:
-thin and underweight (less than or equal to 10th percentile for height and weight on a standardized growth chart), barrel chest, protuberant abdomen and distention, wasted buttocks, thin extremities, listless and lethargic, delayed development of secondary sex characteristics and infertility, occlusion of the vas deferens in males, occlusion of the ovarian ducts in females

Question 8

Diagnosis

Answer 8

based on child’s signs and symptoms, including a positive history of the disease in the family, absence of pancreatic enzymes, increase in the electrolyte concentration of sweat, and chronic pulmonary involvement
-chest x-ray films show patchy atelectasis and obstructive emphysema
-a quantitative sweat chloride test is performed on sweat obtained by iontophoresis of pilocarpine; the results of sweat test are determined differently depending on the age of the child; a chloride concentration of greater than 60 mEq/L is diagnostic of CF
>(normal values of sweat chloride test is less than 40 mEq/L)
>a concentration between 30 to 59 mmol/L for infants less than 6 months = intermediate range
>from 40 to 59 mmol/L in older children = intermediate range
-pancreatic dysfunction is apparent
-test for direct documentation of enzyme secretion are invasive and are not routinely done in older children; these tests help to evaluate the progression of the disorder and provide direction for suitable treatment

Question 9

Prevention

Answer 9

• no known prevention
• in families where there is a known case of CF, identification of carriers may assist the parents in family planning decisions

Question 10

Promoting Respiratory Function

Answer 10

a multidisciplinary approach is taken
-physician, nurse, respiratory therapist, social worker, dietician, and psychologist are important in the management of CF

Question 11

Nursing Care

Answer 11

• goal of treatment is to ensure respiratory function, enhance nutrition, promote growth and development, and encourage independence in an individual child and family
• potential for complications r/t respiratory infection and malnutrition can be reduced or prevented by instructing the parents on proper nutrition, medication compliance, good handwashing, and avoiding contact with persons with respiratory infections

Question 12

Medical Care

Answer 12

-airway clearance and antibiotic use are the key treatment modalities for lung disease r/t CF
>medicated aerosol therapy, chest physiotherapy (percussion and postural drainage to loosen and remove lung secretions), and antibiotic therapy
>some children with CF may have a central venous access device for frequent antibiotic administration
-inhaled mucolytic agents, recombinant human DNAse (Pulmozyme), inhaled hypertonic saline, and
-medication for chronic Pseudomonas infections, which include inhaled tobramycin (TOBI) and oral azithromycin
>most have a complete loss of pancreatic function and inadequate digestion of fats and protein; therefore, an important aspect of management is to replace the pancreatic enzymes; enzyme replacement is administered with meals and snacks so the digestive enzymes are mixed with food in the duodenum; enzyme replacement should not exceed 2500 lipase units/kg per meal

Question 13

Nutrition

Answer 13

well-balanced, high-protein, high-caloric diet

• pancreatic insufficiency results in malabsorption of fat-soluble vitamins (vitamins A, D, E, K)
• daily vitamin supplementation is recommended

Question 14

Caring for the child with Cystic Fibrosis at home

Answer 14

• teach about nature of the disease and prepare them to manage day-to-day minor complaints
• assist family in arranging for the portable suction machine and about the proper suctioning technique at home
• instruct family to do the respiratory therapy before a meal b/c chest physiotherapy may induce vomiting “of the thick tenacious mucus”
• teach different techniques used for chest physiotherapy and postural drainage and coughing exercises based on their child’s age; child needs to be suctioned, followed by chest physiotherapy and inhalation to liquefy the thick secretions
• teach about preferred meal plans, high-caloric diet, and mixing pancreatic enzyme with meal
• instruct the family to monitor the child’s weight to ensure proper growth patterns
• teach how to administer medications properly
• inform family how to access community resources and how to contact their home health nurse

Question 15

Nursing Diagnoses for CF

Answer 15

• ineffective airway clearance r/t thickened secretions
• ineffective breathing patterns r/t tracheobronchial obstructions
• altered nutrition: less than body requirement r/t inability to digest and/or loss of appetite
• altered growth and development r/t inadequate thickening of nutrients
• high risk for infection r/t impaired body defense system
• altered family processes r/t frequent hospitalization/prolonged sickness

Question 16

When Conducting Research in the Pediatric Setting, be sure to:

Answer 16

-obtain written consent from the parents or legal guardian
-obtain written assent from mature children (age 12-13 and older)
-use the ethical principles of beneficence, non-malfeasance, autonomy, veracity, justice, fidelity, and professional integrity
-review institutional protocols carefully, ensuring that the highest standards of research are addressed
-secure clearance from the institutional review board at the institution
>Be sure to design the research so that: children’s rights are respected, children protected from harm or discomfort, children’s legal guardians are provided the information necessary for them to give their informed consent, and children’s rights to anonymity and confidentiality ensured

Question 17

ATI: Cystic Fibrosis

Answer 17

• respiratory disorder that results from inheriting a mutated gene
• characterized by mucus glands that secrete an increase in the quantity of thick, tenacious mucus, which leads to mechanical obstruction of organs (pancreas, lungs, liver, small intestine, and reproductive system)
• an increase in organic and enzymatic constituents in the saliva
• increase in sodium and chloride content of sweat
• causes autonomic nervous system abnormalities

Question 18

ATI: Expected Fidnings

Answer 18

-family history of cystic fibrosis
-medical history of respiratory infections, growth failure
-meconium ileus at birth manifested as distention of the abdomen, vomiting, and inability to pass stool
>meconium ileus is the earliest indication of cystic fibrosis in the newborn

Question 19

ATI: Respiratory Findings

Answer 19

> Stasis of mucus increases the risk for respiratory infections

> Early Manifestations:

• wheezing, rhonchi
• dry, nonproductive cough

> Increased Involvement:

• dyspnea
• paroxysmal cough
• obstructive emphysema and atelectasis on chest x-ray

> Advanced Involvement:

• cyanosis
• barrel-shaped chest
• clubbing of fingers and toes
• multiple episodes of bronchitis or bronchopneumonia

Question 20

ATI: Gastrointestinal Findings

Answer 20

• Large, frothy, bulky, greasy, foul-smelling stools (steatorrhea)
• voracious appetite (early sign), loss of appetite (late sign)
• failure to gain weight or weight loss
• delayed growth patterns
• distended abdomen (infant)
• thin arms and legs (infant)
• deficiency of fat soluble vitamins
• anemia
• reflux
• prolapse rectum (infant, child)

Question 21

ATI: Integumentary Findings

Answer 21

sweat, tears, and saliva have an excessively high content of sodium and chloride

Question 22

ATI: Endocrine and Reproductive System Findings

Answer 22

• viscous cervical mucus
• decreased or absent sperm
• decreased insulin production

Question 23

ATI: Laboratory Tests

Answer 23

• Blood Specimen: nutritional panel to detect a deficiency of fat-soluble vitamins (A, D, E, and K)
• Sputum Culture for detection of infection

Question 24

ATI: Diagnostic Procedures

Answer 24

• DNA Testing: to isolate the mutation
• Pulmonary Function Tests (PFTs): evaluate the small airways
• Chest x-ray: indicate diffuse atelectasis and obstructive emphysema
• Abdominal x-ray: detect meconium ileus
• Stool analysis: for presence of fat and enzymes
• Duodenal analysis: analyze pancreatic trypsin levels (NG tube)
• Sweat Chloride Test

Question 25

ATI: Sweat Chloride Test

Answer 25

-must be well hydrated to ensure accurate results
-a device that uses an electrical current stimulates sweat production
-collection of sweat from 2 different sites for adequate sample
-Normal Chloride Content: less than 40 mEq/L
-Normal Sodium Content: less than 70 mEq/L
>Diagnostic Confirmation of CF:
-Chloride greater than 40 mEq/L for infants less than 3 months; greater than 60 mEq/L for others
-Sodium greater than 90 mEq/L

Question 26

ATI: Nursing Care

Answer 26

• assess lung sounds
• assess respiratory status
• obtain IV access; use of a peripherally inserted central catheter (PICC) or IV port allows for home IV antibiotic therapy
• obtain sputum for culture and sensitivity
• provide support to child and family

Question 27

ATI: Pulmonary Management

Answer 27

> Assist in providing airway clearance therapy (ACT) to promote expectoration of pulmonary secretions; usually prescribed twice a day (morning and evening)
-AVOID ACT immediately before or after meals
Methods of ACT:
-Chest Physiotherapy (CPT) with postural drainage as prescribed (manual or mechanical percussion)
-Positive Expiratory Therapy (PEP): uses a device (flutter mucus clearing device) to encourage the client to breathe with forceful exhalations
-Active-cycle-of-breathing Technique (“huffing” or forced expiration) encouraged
-Autogenic Drainage: uses an electronic chest vibrator or handheld percussor along with breathing techniques
-High Frequency Chest Compression: uses a mechanical chest device combined with nebulization therapy
Administer aerosol therapy as prescribed (bronchodilator, human deoxyribonuclease); recommended prior to ACT
Administer IV or aerosolized antibiotics
Physical aerobic exercise
Provide oxygen as prescribed (assess for CO2 retention)
-Monitor for hemoptysis or pneumothorax

Question 28

ATI: Gastroinestinal Management

Answer 28

• Well-balanced diet high in protein and calories
• Give 3 meals a day with snacks
• Oral fluid intake
• Administer Pancreatic Enzymes within 30 minutes of eating a meal or snack
• Administer water-soluble vitamin supplements: multivitamin; vitamins A, D, E, and K
• Laxatives or Stool softeners for constipation
• Polyethylene-glycol electrolyte solution is administered orally or via nasogastric tube
• Administer histamine-receptor antagonist and motility medications for GERD
• Administer possible formula supplements in addition to breast feedings or via gastric tube
• Add salt to food during hot weather (dehydration)
• consult dietician

Question 29

ATI: Endocrine Management

Answer 29

• CF r/t diabetes (CFRD) requires monitoring of blood glucose
• administer insulin; oral glycemic medications nor effective for CFRD

Question 30

ATI: Respiratory Medications

Answer 30

>Short-acting beta 2 agonists: Albuterol
-monitor for tremors and tachycardia
>Cholinergic antagonists (anti-cholinergics [ipratropium])
-monitor for dry mouth
>Fluticasone/ Salmeterol
-rinse mouth after
>Dornase alfa (aerosol)
-decreases the viscosity of mucus and improves lung function
-monitor of sputum thickness and ability of client to expectorate
-monitor for improvement of PFTs
-educate on how to use nebulize
-administer once or twice a day
-can cause laryngitis

Question 31

ATI: Dornase alfa

Answer 31

aerosol
>decreases viscosity of mucus and improves lung function
-monitor of sputum thickness and ability of client to expectorate
-monitor for improvement of PFTs
-educate on how to use nebulizer
-administer once or twice a day
-can cause laryngitis

Question 32

ATI: Antibitoics

Answer 32

specific to treat pulmonary infection
-tobramycin, ticarcillin, gentamicin
-administer through IV or aerosol
>assess for allergies
>high doses may be prescribed; collect blood specimens before and after some IV antibiotics to maintain therapeutic levels

Question 33

ATI: Pancreatic Enzymes

Answer 33

Pancrelipase treats pancreatic insufficiency associated with CF

• monitor stools for adequate dosing (1 to 2 stools/day)
• monitor weight
• administer capsules with ALL meals and snacks
• child can swallow or sprinkle capsules on food; DO NOT sprinkle on hot foods or add to bottles or formula
• increase dosage of enzymes when eating high-fat foods

Question 34

ATI: Vitamins

Answer 34

daily multivitamin and water soluble vitamins A, D, E, and K