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Thera X > 6 Cystic Fibrosis Bensman > Flashcards

6 Cystic Fibrosis Bensman Flashcards

1
Q

What is CF?

A

Chronic, progressive and life limiting autosomal recessive genetic disease characterized by chronic respiratory infection/inflammation, heavy sputum production, pancreatic insufficiency, elevation of sweat electrolytes and male infertility ultimately succumbing to respiratory failure and death

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2
Q

What is used for CF Treatment?

A

Pancreatic Enzyme Replacement Therapy (PERT). High fat, high protein, high calorie diet. Vitamins. Airway clearance therapy. Antibiotic therapy. Management of co-morbidities. Supportive care

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3
Q

What is the median CF survival age as of 2010?

A

38 years

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4
Q

What is Class I CF?

A

No synthesis for CFTR (chloride pump) from mRNA

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5
Q

What is Class II CF?

A

CFTR starts being made, but no maturation. Most common class. Error in Delta 508

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6
Q

What neonatal screening is done for diagnosis of CF?

A

Immunoreactive Trypsinogen (IRT) (blood IRT levels will be elevated). Evidence of CFTR abnormality (Sweat test, Genotyping)

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7
Q

What is Pancreatic Exocrine Insufficiency?

A

CF often results in fat malabsorption - lipase deficiency characterized by steatorrhea and abdominal cramping. Fecal elastase levels low in patients with pancreatic insufficiency (diagnostic test)

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8
Q

What are Pancreatic Enzymes?

A

Enzymes designated to assist in the absorption of food. Lipase (fat), protease (protein), amylase (sugars)

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9
Q

What are enzymes given microencapsulated?

A

Designed for release within alkaline environment of small bowel

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10
Q

How is the lipase component dosed?

A

1500-2500 units/kg/meal

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11
Q

What is done for PERT monitoring?

A

Stool frequency and consistency. BMI. Cramping

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12
Q

What are the ADRs with PERT?

A

Fibrosing colonopathy (most common). Mucosal ulceration. Allergic hypersensitivity (pork). Hyperurecimia

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13
Q

What is the max dose of Lipase PERT to avoid Fibrosing Colonopathy?

A

10,000 lipase units/kg/day. 6,000

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14
Q

How can Mucosal Ulceration be minimized?

A

Wash mouth if opening capsule. Suspend microspheres in acidic food

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15
Q

What are some secondary complications from CF?

A

Malnutrition. Growth failure. Fat-soluble vitamin deficiency. Bone disease (osteoporosis, Kyphosis)

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16
Q

What vitamins need to be replaced?

A

Fat soluble: A, D, E, K

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17
Q

When a patient is on PERT therapy w/ GI upset (bloating, high bowel movements), what can be added?

A

PPIs (only after PERT therapy is maxed)

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18
Q

What part of CF accounts for over 90% of morbidity and mortality?

A

Progressive airway disease

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19
Q

What are the different % FEV predicted ranges for different severities?

A

Normal (> 90). Mild (70-89). Moderate (40-69). Severe (< 40)

20
Q

What is Bronchiectasis?

A

The end result of chronic inflammation and mucus obstruction in the CF lung. As the muscular and elastic components of the bronchial tube are destroyed, the area below the obstruction balloons out to form a perfect hiding place for infection and pus. The end result is progressive loss of lung function leading to eventual respiratory failure

21
Q

What is bad about P. aeruginosa in CF?

A

Reduced susceptibility in biofilms

22
Q

What are the characteristics of inflammation in CF?

A

Exuberant but ineffective response by neutrophils (Proteases cleave CXCR1 surface receptor resulting in impaired phagocytosis). Massive influx of neutrophils into the airway which release: Elastase, proteinase, collagenase, oxidants and IL-8. Excessive proteases cause degradation of airways

23
Q

What can be used in the management of Airway Obstruction?

A

Chest Physiotherapy (CPT). rhDNase (Dornase alfa). Hypertonic saline

24
Q

How does rhDNase (Dornase alfa) work?

A

Hydrolyzes extracellular neutrophil derived DNA. Mucolytic agent

25
Q

How does Hypertonic Saline work for airway obstruction?

A

Rehydrates airways through osmotic effect

26
Q

What is the role of Dornase alfa?

A

Recommended in patients Age > 6 years with moderate-severe lung disease to improve lung function and reduce frequency of exacerbations

27
Q

What is the role of Hypertonic Saline?

A

Recommended in patients > 6 years of age. Alternative to improve airway clearance in patients who fail to respond, are ineligible, or are unable to tolerate rhDNase. Most beneficial in patients with multiple RTEs

28
Q

What can be used to manage airway infections?

A

Inhaled Tobramycin. Inhaled Aztreonam. Can use Azithro in combo

29
Q

What is the role of Inhaled Tobramycin?

A

Recommended in patients with age > 6 years chronically infected with P. aeruginosa and have moderate-severe lung disease. Can also be used for same patient that has asymptomatic-mild lung disease (Grade B)

30
Q

What is the role of Inhaled Aztreonam?

A

Primary therapy in patients with P. aeruginosa and are intolerant of inhaled tobramycin. Add-on therapy in patients with decline in lung function or frequent exacerbations despite inhaled tobramycin (cycle each month)

31
Q

What is the role of Azithromycin?

A

Recommended for patients > 6 years with CHRONIC P. aeruginosa infection (Grade B). Screen for non-tuberculosis mycobacteria

32
Q

What is usually done for Reduced baseline airway surface liquid/clearance and increased obstruction (CF Stage 1)?

A

7% HS rehydrates mucin and expands the ASL. rhDNase reduces viscosity of sputum

33
Q

What is usually done for Initial Infection/Hyperinflammation (CF Stage 2)?

A

Eradication of pathogen with antibiotics. Ibuprofen reduces magnitude of PMN migration to airways

34
Q

What is used for Augmented Obstruction (CF Stage 3)?

A

rhDNase reduces viscosity of mucous. LABAs dilate airways

35
Q

What is used for Chronic infection/Inflammation established?

A

Inhaled antibiotics for infection. Azithromycin for anti-virulent and anti-inflammatory response

36
Q

What is the Aerosol Administration Sequence?

A

1) Bronchodilator (quick onset). 2) rhDNase. 3) Hypertonic Saline. 4) Airway clearance. 5) Inhaled antibiotics. 6) Inhaled corticosteroids

37
Q

When is Peak Flow Monitoring done?

A

Patients should perform twice a day every day at same time recording highest value for each occasion in diary. A 20% decrease of personal best may indicate pulmonary exacerbation and requires additional spirometry

38
Q

What is Pulmonary Exacerbation?

A

Increased cough. Increased sputum. Change in sputum (more purulent, hemoptysis). Decline in lung function (spirometry). New infiltrate on CXR. Fever. Decreased appetite. Loss of weight. Tachycardia. Reduced exercise tolerance

39
Q

What is the impact of RTEs?

A

Each RTE within a year had the same negative impact on 5-year survival as a 12% reduction in FEV. The annual number of acute RTEs, with FEV and 7 other covariates together may accurately predict 5-year survival. Reduction of RTEs is therefore a critical goal of the respiratory management of CF

40
Q

What are the choices for treatment of acute exacerbation?

A

Intravenous antibiotics (10-14 days w/ dual AP therapy: beta-lactam + tobramycin or cipro). Airway clearance therapy. Nutrition

41
Q

What airways clearance therapy is there for treating an acute exacerbation?

A

SABA Q4h. Dornase alfa 2.5mg inhaled QD. HS 7% inhaled BID. Chest percussion or high frequency chest oscillation QID

42
Q

What is useful for MDR P. aeurginosa in acute exacerbation?

A

Intravenous Colistimethate, monitor for nephrotoxicity and neurotoxicity

43
Q

What is the rationale for larger antibiotic doses in CF?

A

Reduced penetration into pulmonary secretions. Reduced antibiotic susceptibility. Altered PK (higher drug clearance)

44
Q

What is Kalydeco (Ivacaftor)?

A

First CFTR potentiator. CF patients > 6 with at least one G551D mutation. Add-on therapy

45
Q

What are the ADRs with Kalydeco (Ivacaftor)?

A

HA, dizziness. URT symptoms. Abdominal symptoms. Rash

46
Q

What needs to be monitored while on Kalydeco (Ivacaftor)?

A

Liver function (baseline, Q3 months for first year, annually after, DC when > 5x UNL)

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