+-----------------+ | CYSTIC FIBROSIS | +-----------------+ +-------------+ | DEFINITIONS | +-------------+ Definition: Cystic Fibrosis: - Genetic disease (chromosome 7) affecting the

exocrine glands, characterized by excessive mucus secretions that impairs the pancreas, intestines, and lungs. Definition: Bronchiectasis: - Irreversible dilation and destruction of the bronchial walls. Definition: Ileus: - Non-mechanical bowel obstruction. Definition: Pulmonary Exacerbation: - Temporary worsening of lung function due to inflammation. - Symptoms: - Dyspnea (shortness of breath). - Coughing. - Fatigue. Definition: Steatorrhea: - Presence of excessive fat in feces. - Bulky, Greasy, Clay-colored (pale), foul-smelling stools. +--------------+ | INTRODUCTION | +--------------+ Cystic Fibrosis: - 37 years. Average lifespan: (Gold Standard):

Cystic Fibrosis: Diagnostic Test: - Sweat chloride test.

Cystic Fibrosis: Electrolyte Disturbances: - Sodium increased. - Chloride increased. Cystic Fibrosis: Sterility: - Male ..... sterile. - Female ... non-sterile. Cystic Fibrosis: - Lungs. - Pancreas. - Intestines. Males vs. Females:

Body organs affected:

Cystic Fibrosis: Clinical Manifestations: - Lungs: - Inflammation. - Mucus secretions. - Bacterial infections. - Bronchiectasis. - Pulmonary exacerbation.

Lungs:

Cystic Fibrosis: Clinical Manifestations: Pancreas: - Pancreas: - Pancreatic insufficiency. - Steatorrhea. - Fat-soluble vitamin deficiencies. - Failure to thrive. - Constipation (chronic). - Intestinal obstructions. - Meconium ileus at birth (20% of CF patients). Cystic Fibrosis: Treatment: Pharmacological: - DNase. - Hypertonic saline (inhaled). - Ibuprofen. - Pancreatic enzymes. Cystic Fibrosis: Treatment: - Before meals and snacks. When to take pancreatic enzymes:

Cystic Fibrosis: Treatment: Mechanical: - Airway clearance therapy: - Chest physiotherapy. - Chest vest. Cystic Fibrosis: Diet: - High calorie, high fat, high protein, high carbohydrate diet. Cystic Fibrosis: Caloric Requirements: - Four times the recommended calories. What are the fat-soluble vitamins: - Vitamin A. - Vitamin D. - Vitamin E. - Vitamin K. Cystic Fibrosis: (names only):

Order and frequency of postural drainage and breathing exercises: - Postural drainage first, then breathing exercises. - Usually done twice daily.

Cystic Fibrosis: Describe patient's stools: - Steatorrhea: - Bulky, Greasy, Clay-colored (pale), foul-smelling stools.

+-----------------------------------------+ | CYSTIC FIBROSIS - NCLEX STYLE QUESTIONS | +-----------------------------------------+

TRUE OR FALSE: - The primary systems affected by cystic fibrosis are the pulmonary and gastrointestinal systems. ANSWER: TRUE.

TRUE OR FALSE: - Lung transplantation is an end-stage treatment for children with cystic fibrosis. ANSWER: TRUE.

QUESTION: - The mother of a child with cystic fibrosis (CF) asks the clinic nurse about the disease. The nurse tells the mother that CF is which of the following? A: B: C: D: A disease that causes the formation of multiple cysts in the lungs A chronic multisystem disorder affecting the exocrine glands Transmitted as an autosomal dominant trait A disease that causes dilation of the passageways of many organs B - A chronic multisystem disorder affecting the exocrine glands. - Cystic Fibrosis is transmitted as an autosomal recessive trait. - Cystic Fibrosis causes obstruction of small pathways.

ANSWER:

QUESTION: - Breathing exercises and postural drainage are prescribed for a hospitalized child with cystic fibrosis (CF). A nurse teaches the child to: A: B: C: D: Perform the postural drainage first and then the breathing exercises. Perform the breathing exercises and then the postural drainage. Schedule the procedures so they are 4 hours apart. Perform postural drainage in the morning and breathing exercises in the evening. A - Perform the postural drainage first and then the breathing exercises. Usually done twice daily.

ANSWER:

QUESTION: - A clinic nurse is providing instructions to a mother of a child with cystic fibrosis regarding the immunization schedule for the child. Which statement would the nurse make to the mother? A: B: C: D: The immunization schedule will need to be altered. The child should not receive any hepatitis vaccines. The child will receive all the immunizations except for the polio series. The child will receive the recommended basic series of immunizations along with a yearly influenza vaccination. ANSWER: D - The child will receive the recommended basic series of immunizations along with a yearly influenza vaccination. Adequately protecting children with cystic fibrosis from communicable diseases by immunization is essential. In addition to the basic series of immunizations, a yearly influenza immunization is recommended for children with cystic fibrosis. - Options A, B, and C are incorrect.

QUESTION: - What symptoms in a child might lead a provider towards testing for cystic fibrosis: A: B: C: D: Chronic cough and wheeze, improves with short-acting beta agonist. Congestion, itchy-eyes, sneezing. Recurrent otitis infections and chronic nasal discharge. Recurrent respiratory infections, poor weight gain, and "mucous-like" stools. D - Recurrent respiratory infections, poor weight gain, and "mucouslike" stools. - Children with cystic fibrosis have chronic respiratory infections and often do not absorb nutrients appropriately due to pancreatic insufficiency, so have problems with failure to thrive and stooling. - Beta-Agonist (short-acting). [HINT: S-B-A]

ANSWER:

QUESTION: - Susie, a 3-year-old with cystic fibrosis. Is beginning her first day at a new day care & preschool center. Which type of stools should the workers at the center be told to expect from her? A: B: C: D: Large amounts of black liquid. Foul smelling liquid. Large, pale. Small, hard, odorless. C - Large, pale stools.

ANSWER:

QUESTION: - Which of the following is NOT a classic treatment for the patient with cystic fibrosis (CF)? A: B: C: D: Airway clearance therapies. Nutritional support. Antibiotic therapy. Tracheostomy. A tracheostomy is not a standard treatment for CF.

ANSWER: D - Tracheostomy -

- The three cornerstones of care for a patient with CF are antibiotic therapy, airway clearance, and nutritional support.

QUESTION: - Your patient, age 3, has just been diagnosed with cystic fibrosis. Her mother asks the nurse about the disease. Which of the following are appropriate responses by the nurse: Select all that apply: A: Cystic fibrosis affects the exocrine glands of the body. B: The pulmonary system is the primary system affected by the disease. C: Cystic fibrosis is usually manifested as an individual experiencing chronic respiratory infections. D: The best diet is a high-fat/low-protein diet. E: Most children with cystic fibrosis die between the ages of 10 and 12. F: Lung transplantation is contraindicated in children with cystic fibrosis. ANSWER: A - B - C

QUESTION: - Which of the following are common symptoms of cystic fibrosis: Select all that apply: A: B: C: D: E: F: Nasal polyps. Pancreatitis. Hyperproteinemia. Respiratory infections by Pseudomonas. Black stools. Rectal prolapse.

ANSWER: A - B - D - F