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PPC3 Exam 1 > Cystic Fibrosis: Associated Issues and Management > Flashcards

Cystic Fibrosis: Associated Issues and Management Flashcards

1
Q

Sinusitis Signs and Symptoms (9)

A
  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
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2
Q

Sinusitis Treatment (3)

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

Polyposis (5 with tx)

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

CF: Gastrointestinal Disease (6)

A
  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
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5
Q

Pancreatic Insufficiency: Clinical Manifestations (5)

A
  1. 85% rate of pancreatic insufficiency
  2. Frequent bulky, greasy stools, floating stools
  3. Failure to thrive
  4. Abdominal distention
  5. Crampy abdominal pain
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6
Q

Nutritional Complications (4)

A
  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
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7
Q

Cystic Fibrosis Liver Disease (5)

A

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
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8
Q

Other GI Complications (2)

A
  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
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9
Q

Pancreatitis as only manifestation (4)

A
  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
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10
Q

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

A
  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.
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11
Q

Oral glucose test with CFRD (2)

A

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
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12
Q

Preventative Measures w/ CF (4)

A
  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)
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13
Q

CF Management/Treatment (9)

A
  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
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14
Q

Pulmonary Treatment (7)

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

Must

  1. Control of airway infections
  2. Clearance of airway secretions
  3. Decreasing inflammation in the lung.
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15
Q

CF Respiratory Management (6 w/ targeted genetic tx)

A
  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
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16
Q

Chronic Pulmonary Treatment (3)

A
  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
17
Q

Airway clearance (6)

A
  1. Chest Physical therapy
  2. Vest – mechanical percussion
  3. Flutter, Acapella
  4. Breathing techniques: ACB
  5. Exercise
  6. None is superior
18
Q

Chronic Pulmonary Treatment: Mucus Modifying Agents (4)

A
  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
19
Q

Chronic Pulmonary Treatment: Hypertonic saline 7%

A

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

20
Q

Chronic Pulmonary Treatment: Anti-inflammatory drugs

A

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.

21
Q

Chronic Pulmonary Treatment: inhaled antimicrobial agents (

A
  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)
22
Q

Chronic Pulmonary Treatment: Other (3)

A
  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
23
Q

Acute Pulmonary Exacerbations: Main treatment (3)

A
  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
24
Q

Acute Pulmonary Exacerbations: Pneumothroax (6)

A
  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
25
Q

Acute Pulmonary Exacerbations: Hemoptysis (5)

A
  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
26
Q

Acute Pulmonary Exacerbations: Management (3)

A
  1. Antibiotic therapy
  2. Cessation of the anti-inflammatory drugs
  3. Limiting therapies for airway clearance
27
Q

Management of GI Symptoms (6)

A
  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
28
Q

Ivacaftor (Kalydeco) and CF (5)

A
  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.
29
Q

CF Primary care (9)

A
  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
30
Q

Transplantation (7)

A
  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
31
Q

Transitioning in CF (2)

A
  1. Should begin at around age 12

2. It is important to have a multidisciplinary team

PPC3 Exam 1 (13 decks)

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