Cystic Fibrosis: Associated Issues and Management

Question 1

Sinusitis Signs and Symptoms (9)

Answer 1

1. Mucopurulent discharge
2. Nasal congestion
3. Facial pain – eye pain or retroorbital pain
4. Decreased sense of smell
5. Can have persistent fever
6. Persistent colored nasal discharge for >4 days
7. Mucosal inflammation on nasal endoscopy
8. X-ray findings not reliable
9. Same organisms as lung

Question 2

Sinusitis Treatment (3)

Answer 2

1. Sinus rinse
2. Antibiotics
3. Surgery in new guidelines

Question 3

Polyposis (5 with tx)

Answer 3

1. 6-40%
2. Nasal air obstruction
3. Loss of taste or appetite
4. Persistent epistaxis
5. Treatment – Topical steroids, macrolide antibiotics, antibiotics, surgery

Question 4

CF: Gastrointestinal Disease (6)

Answer 4

1. Pancreatic insufficiency/malabsorption
2. Lipo-soluble vitamin deficiency (ADEK)
3. Failure to thrive - hypoproteinemia and edema
4. Neonatal intestinal obstruction (15%)
5. Recurrent distal intestinal obstruction syndrome (DIOS)
6. Biliary stasis - portal hypertension; 2-5% pts

Question 5

Pancreatic Insufficiency: Clinical Manifestations (5)

Answer 5

1. 85% rate of pancreatic insufficiency
2. Frequent bulky, greasy stools, floating stools
3. Failure to thrive
4. Abdominal distention
5. Crampy abdominal pain

Question 6

Nutritional Complications (4)

Answer 6

1. Edema and hypoproteinemia
2. Acute salt depletion
3. Anemia
4. Zinc deficiency – acrodermatitis enteropathica
   a. A baby that cannot absorb zinc in breastmilk
   b. Unusually severe diaper and/or facial rash
   c. May have edema → check/test for edema on sacral area

Question 7

Cystic Fibrosis Liver Disease (5)

Answer 7

Diagnosed when there are at least 2 of the following 4:

1. Hepatomegaly and/or splenomegaly
2. Liver function tests abnormalities on 3 tests in a 12 month period
3. Portal hypertension or abnormal liver echo texture on ultrasound
4. Cirrhosis on liver tissue biopsy.
5. Treatment of ursodeoxycholic acid (ursodiol) is the recommended treatment

Question 8

Other GI Complications (2)

Answer 8

1. Distal intestinal obstruction syndrome (DIOS): DIOS is managed using osmotic laxatives to promote lower bowel clearance. The use of sodium meglumin diatrizoate (Gastrografin) enemas can be used for a near complete obstruction by an experienced radiologist
2. Rectal prolapse

Question 9

Pancreatitis as only manifestation (4)

Answer 9

1. 18 year-old with acute abdominal pain. Amylase-792; lipase-950. Diagnosed with acute pancreatitis. Mutation analysis – 1717-1G>A and 3849+10kbC>T
2. Sweat chloride – 84 meq/l
3. Chest x-ray normal
4. Pulmonary function tests normal; Indicates longer life expectancy if no lung disease

Question 10

Endocrine Manifesation: Cystic Fibrosis Diabetes mellitus (CFRD) (8)

Answer 10

1. Result of the fatty infiltration and destruction off the islet cells due to the thick viscous secretions in the pancreas.
2. Microvascular complication of diabetes due to hyperglycemia
3. Renal disease
4. Retinopathy
5. Peripheral neuropathy are associated.
6. Average age of onset – 18-21 years; 16%
7. Insidious onset and mild clinical course
8. Ketoacidosis is rare.

Question 11

Oral glucose test with CFRD (2)

Answer 11

At age 10, an oral glucose tolerance test is done annually to screen for CFDR.

1. Hemoglobin A1C is not recommended since is underestimates overall glycemic control.
   * Drink set amount of glucose then do blood draws q30 min
2. Insulin is used to treat CFRD

Question 12

Preventative Measures w/ CF (4)

Answer 12

1. Routine immunizations
2. Influenza vaccine
3. Children with Down syndrome or cystic fibrosis also are not recommended to receive palivizumab, even though some evidence shows that these children may be at higher risk for RSV
4. Pneumococcal vaccine? – variable recommendations on PCV23 (the addition of pneumococcal 23 because it is not a conjugated vaccine; cannot be given until 2 years old)

Question 13

CF Management/Treatment (9)

Answer 13

1. Table salt supplementation
2. Fluoride supplementation
3. Education of caregivers regarding harms of cigarette smoke exposure for children and provision of a smoke-free environment
4. Airway clearance therapy
5. Albuterol before percussion and postural drainage
6. Infection control education
7. Infection control measures to minimize transmission of bacterial infections to infants
8. Influenza vaccination
9. Palivizumab (for prophylaxis of respiratory syncytial virus)- Unlikely

Question 14

Pulmonary Treatment (7)

Answer 14

1. Aim of pulmonary disease treatment
2. Optimize lung functioning
3. Prevent disease progression
4. Avoid complications.

Must

5. Control of airway infections
6. Clearance of airway secretions
7. Decreasing inflammation in the lung.

Question 15

CF Respiratory Management (6 w/ targeted genetic tx)

Answer 15

1. Regular visits to CF Center
2. Airway clearance
3. Mucus thinners (DNase, hypertonic saline)
4. Antibiotics (PO, IV)
5. Anti inflammatory agents
6. Targeted genetic treatment—
   a. Ivacaftor
   b. Combination of Ivacaftor/lumacaftor

Question 16

Chronic Pulmonary Treatment (3)

Answer 16

1. Airway Clearance
2. Chest percussion and drainage is only treatment for infants and young children
3. Postural drainage, active cycle of breathing, autogenic drainage, percussion, positive expiratory pressure, exercise high frequency chest wall oscillation; Twice a day to facilitate secretion removal

Question 17

Airway clearance (6)

Answer 17

1. Chest Physical therapy
2. Vest – mechanical percussion
3. Flutter, Acapella
4. Breathing techniques: ACB
5. Exercise
6. None is superior

Question 18

Chronic Pulmonary Treatment: Mucus Modifying Agents (4)

Answer 18

1. Inhaled dornase alfa (recombinant human deoxyribonuclease); DNAse
2. Digests the extracellular neutrophil derived DNA
3. Assists with secretions in the lungs, along with hypertonic saline
4. Very expensive

Question 19

Chronic Pulmonary Treatment: Hypertonic saline 7%

Answer 19

Works by drawing water into secretions and is used to thin secretions to allow removal of
*Not good for bronchiolitis

Question 20

Chronic Pulmonary Treatment: Anti-inflammatory drugs

Answer 20

High-dose ibuprofen

*While high dose ibuprofen decreases neutrophil migration in children from 6 years to 17 years, the therapy is not widely used due to risk of GI bleeding and frequent drug blood level measurements.

Question 21

Chronic Pulmonary Treatment: inhaled antimicrobial agents (

Answer 21

1. Eradicates initial P. aeruginosa growth
2. Inhaled tobramycin for 28 days as part of eradication protocol
   i. Tobramycin inhaled
3. > 6 years
   ii. DRUG OF CHOICE
4. Aztreonam (2nd choice)
   - Alternating inhaled antibiotics every months to decrease frequency
5. Colistin (3rd choice)

Question 22

Chronic Pulmonary Treatment: Other (3)

Answer 22

1. The use of Ivacaftor is limited to patients who carry at least one G551D mutation
2. No corticosteroids
3. Oral azithromycin dosed three times a week are used; Must be screened for atypical mycobacterial infection before starting long-term azithromycin

Question 23

Acute Pulmonary Exacerbations: Main treatment (3)

Answer 23

1. For 13 days
2. Aminoglycosides are most common class of antipseudomonials
   * Side effects → ototoxicity and nephrotoxicity
3. Beta-Lactam antibiotics—Not recommended in the CF guidelines

Question 24

Acute Pulmonary Exacerbations: Pneumothroax (6)

Answer 24

1. Increased intrapleural pressure due to inflammation and obstruction
2. Acute onset of chest pain and dyspnea and is confirmed by chest X-ray
3. Larger pneumothorax >2 cm Admit and chest tube
4. Smaller pneumothoraxes (<2 cm)
   * Managed by observation and discontinuation of positive pressure.
5. Surgical or chemical pleurodesis is used in recurrent large pneumothoraxes.
6. Lung transplantation is a viable therapy for selected patients who have terminal lung disease

Question 25

Acute Pulmonary Exacerbations: Hemoptysis (5)

Answer 25

1. Results from the hypertrophy and proliferation of the bronchial arteries rupturing into the airways as a result of the disease process (Hurt and Simmonds, 2012). 2. Scant (<5ml) 3. Moderate (5-240 ml) 4. Massive (>240 ml) 5. Associated with advancing lung disease as well as vitamin K deficiency

Question 26

Acute Pulmonary Exacerbations: Management (3)

Answer 26

1. Antibiotic therapy 2. Cessation of the anti-inflammatory drugs 3. Limiting therapies for airway clearance

Question 27

Management of GI Symptoms (6)

Answer 27

1. Pancreatic enzyme replacement therapy (PERT) a. Replacement with enzymes in the dosage ranges of 2000 to 2500 U/kg of lipase to a maximum of 10,000 U/kg/day. b. Not over 24,000 units a day * Risk of severe fibrosing colonopathy 2. Feeding (human milk, standard infant formulas, calorie- dense feedings, provision of educational resources for caregivers) 3. Nutritional counseling 4. Multivitamins a. Vitamin A and K levels need to be monitored!! – unable to absorb these 5. Monitoring of fat-soluble vitamin levels 6. Zinc supplementation

Question 28

Ivacaftor (Kalydeco) and CF (5)

Answer 28

1. People ages 2 and over with the following 5 mutations: a. 3849+10kbC->T b. 2789+5G->A c. 3272-26A->G d. 711+3A->G E831X. 2. Improve the function of a defective CFTR protein 3. With this mutation the CFTR protein acts like a locked gate, preventing the proper flow of salt and fluids in and out of the cell. 4. Kalydeco helps unlock that gate and restore the function of the CFTR protein, allowing a proper flow of salt and fluids on the surface of the lungs. 5. Thins the thick, sticky mucus caused by CF that builds up in the lungs.

Question 29

CF Primary care (9)

Answer 29

1. Nutrition including use of Cycloheptadine 2. Development 3. Sleep 4. Elimination 5. Immunizations 6. School 7. Psychosocial 8. Sexuality/Reproductive 9. Routine Screening as recommended by AAP

Question 30

Transplantation (7)

Answer 30

1. Declining lung function FEV1 ≤ 30 2. Pulmonary hypertension (>35 mmHg) 3. Infections with poor recovery from pulmonary exacerbations 4. Nutrition; Worsening status despite supplements and appetite stimulants 5. Life threatening hemoptysis 6. Pneumothorax 7. Diabetes

Question 31

Transitioning in CF (2)

Answer 31

1. Should begin at around age 12 | 2. It is important to have a multidisciplinary team