Cystic Fibrosis - Exam 2 (6-8Q)

Respiratory Management: Medications Mucolytics (Dornase alfa (Pulmozyme))

acts like scissors by cutting up the long DNA strands contained in white blood cells. Side effects: sore/dry throat and hoarseness, eye redness and irritation, rash, Nursing consideration: take same time everyday, use with other medications to decrease symptoms of CF

Pathophysiology of Cystic Fibrosis

is an inherited autosomal recessive disease characterized by increased viscosity of mucus gland secretions and elevation of sweat electrolytes.

Respiratory Treatment: Airway Clearance Therapy

key part of prevention and treatment of respiratory complications. Mobilizing secretions and expectorating them will help prevent bacterial colonization and damage pulmonary structures. ACT is completed at least twice daily at home and up to four times a day in a hospitalized patient.

Respiratory Labs

uCBC d/p uBMP uSputum culture uChest X ray uPFTs

GI complications associated with CF

uPancreatic insufficiency; pancreatitis uMalabsorption of nutrients uBowel obstruction uSteatorrhea uRectal prolapse uLiver and gallbladder damage uCystic Fibrosis Related Diabetes (CFRD)

Aggressive treatment of pulmonary infections

IV antibiotic therapy Aerosolized antibiotics

Cystic Fibrosis Respiratory:

-leads to bacterial colonization leads to tissue destruction - decrease O2 and CO2 -results in hypoxia and acidosis

Cystic Fibrosis Complication: Pneumothorax

- Accumulation of air in the pleural space Signs -Hypotension -Decreased O2 saturations -Dyspnea, labored breathing -Pain -Tachycardia

A 2-year old has just been diagnosed with CF. The parents ask the nurse what early respiratory symptoms they should initially expect to see in their child. Which is the nurses's best response? -"You can expect your child to be cyanotic" -"You can expect your child to develop a barrel-shaped chest." -"You can expect your child to develop a productive cough." -"You can expect your child to develop bronchiectasis."

-"You can expect your child to develop a productive cough."

Airway Clearance Therapies: Chest Percussion Therapy (CPT)

-Administer 30 minutes before feeding -Encourage coughing -Suction infant -Monitor oxygen sats -Lung sounds before and after

Respiratory Symptoms & Complications Upper:

-Chronic sinusitis -Nasal polyposis

Respiratory Symptoms & Complications Lower:

-Cough -Sputum production -Hemoptysis Bronchiolitis/Bronchitis -Bronchiectasis Pulmonary vascular remodeling Chronic hypoxemia Pulmonary HTN Cor pulmonale Blebs and cysts Pheumothorax

CF reproductive issues:

-Delayed puberty -Sterility -Fertility inhibited

Which of the following statements is appropriate for the nurse to address to the parents of a child with CF who are planning to have a second child? -There is a 50% change the child will be normal. -Genetic counseling is recommended. -There is a 25% chance the child will only be a carrier. -There is a 50% chance the child will be affected.

-Genetic counseling is recommended.

Diagnosis of Cystic Fibrosis

-Positive family history -Newborn screening -DNA identification of mutant genes -Positive sweat test>60Meq/L -Other clinical presentations -Failure to thrive -Chronic respiratory symptoms -Failure to pass meconium -Steatorrhea -Parents report children taste"salty

Goals of CF therapy

-Prevent/minimize pulmonary complications -Adequate nutrition for growth -Physical activity -Psychosocial support -Family Education and support

Which of the following tools is the definitive diagnosis for cystic fibrosis? -Pulmonary function testing. -Sweat Test -Newborn screen -Stool culture

-Sweat Test

Which of the following definitions best describes cystic fibrosis? -An inflammation of the pulmonary parenchyma -A chromosomal abnormality inherited as an autosomal-dominant trait -A chronic lung disease related to high concentrations of oxygen and ventilation. -A multi-symptom disorder affecting the exocrine or mucus producing glands

A multi-symptom disorder affecting the exocrine or mucus producing glands

GI meds for CF: Pancreatic Enzymes •Pancrealipase •Creon •Zenpep

Assist in digestion of nutrients, decreasing fat and bulk. Given prior to all meals and snacks

Which intervention is appropriate for care of the child with cystic fibrosis? -Administer bronchodilator or nebulizer treatments after chest -physiotherapy. -Decrease exercise and limit physical activity. -Complete chest physiotherapy two to four times per day. -Give cough suppressants and anti-histamines.

Complete chest physiotherapy two to four times per day.

GI meds for CF: Multivitamins •ADEK

Cystic Fibrosis interferes with vitamin absorption. Supplements are required in water soluble form for better absorption

ACT techniques include

Mechanical Vests or High -frequency chest compression Percussion and postural drainage Positive expiratory pressure (PEP) Huff-cough method- active cycle of breathing Flutter Valve

Respiratory Management: Medications Corticosteroids (Inhaled - Flovent)

Most effective anti-asthmatic drug Side effects: Thrush Inhaled have less systemic effects so you will not see side effects of increased appetite and mood swings that are seen in oral and IV. Must rinse out mouth after administration.

Respiratory Management: Medications Bronchodilators (Albuteral (Proventil, VentolinProAir HFA)

Most effective drug for relieving bronchospasm Side Effects: Tachycardia, anxiety, tremors

The parents of a 4-month-old with CF asks the nurse what time to begin the child's first CPT each day. Which is the nurse's best response? -"You should do the first CPT only when the child has congestion or coughing." -"You should do the first CPT 30 minutes before feeding the child breakfast." -"You should do the first CPT 30 minutes after feeding the child breakfast." -"You should do CPT after deep-suctioning the child each morning."

You should do the first CPT 30 minutes before feeding the child breakfast."