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Pulmonary > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

Cystic fibrosis =

A

Most common autosomal recessive disease among Caucasian Americans
Affects lungs and GI system
Defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food

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2
Q

Symptoms

A

Salty-tasting skin
Persistent cough at times with phlegm
Frequent lung infections
Wheezing or SOB
Poor growth/wt gain in spite of good appetite
Frequent greasy, bulky stools or difficulty with BMs

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3
Q

Survival

A

Overall trend is imrpoved

Female survival rate worse than male b/t 2-20yo

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4
Q

Dx

A

Sweat chloride concentration >60mEq/L
In presence of 1 or more typical features:
Chronic sinopulmonary dz
Pancreatic insufficiency
Salt loss syndromes
Obstructive azoospermia
Appropriate fam hx (sibling or 1st cousin)

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5
Q

Who should undergo sweat testing

A

Infants c positive newborn screening results
Infants, older children, and adults c symptoms suggestive of CF
Siblings of a pt c confirmed CF

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6
Q

Organs affected

A 
Airways
Lungs
Pancreas
Liver
Small intestine
Reproductive tract
Skin
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7
Q

Clinical Manifestations

A 
Bronchiolitis/asthma
Pseudomonas aeruginosa colonization of the respiratory tract
Staphylococcal pneumonia
Nasal polyposis
Chronic productive cough
Sinusitis
Digital clubbing
As dz progresses, chronic bronchitis c or s bronchiloectasis develops and can be accompanies by acute exacerbations
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8
Q

Sino-Pulmonary dz

A

Endobronchial dz: cough, sputum production, air obstruction - evidence of obstruction on PFTs, CXR anomolies, digital clubbing

Sinus dz: nasal polyps, CT or XR findings of panopacifications of paranasal sinuses

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9
Q

Colonization of Airway

A

80% of CF pts eventually end up with Pseudomonas infection

Association b/t acquiring Pseudomonas and clinical decline

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10
Q

Schamroth Sign

A

normal angle b/t nail and nailbed lost (digital clubbing)

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11
Q

Nutritional deficit

A

Insufficient secretion of digestive enzymes such as lipase leads to malabsorption of fat (with steatorrhea) and protein

Malabsorption presented as FTT or fat soluble vitamin insuffeciency (fat soluble vitamins = A, D, E, K)

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12
Q

Intestinal abnormalities
Hepatobiliary dz
Pancreatic endocrine dysfxn

A

Intestinal abnormality
Meconium ileus
Distal intestinal obstruction syndrome (DIOS)
Rectal prolapse

Hepatobiliary disease
Focal biliary cirrhosis
Multilobular cirrhosis

Pancreatic endocrine dysfunction
Cystic fibrosis related diabetes

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13
Q

CF related DM
Screening
Initiating insulin tx

A

Screening
Oral glucose tolerance test (OGTT)
Every two years in patients 10-16 years
Any patient with random plasma glucose >180

Fasting>=200 mg/dl
initiate insulin treatment

Fasting 140-200 mg/dl
Home glucose monitoring; consider insulin

Fasting <140
Normal glucose tolerance

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14
Q

Infertility

A

Common
Men:
Abnormal embryologic development of the epididymal duct and vas deferens—may be incomplete or absent
Congenital bilateral absence of vas deferans in 97-98%

Women:
Lower fertility rate than non-CF women
Viscid mucoid cervical secretions of low volume in women with CF

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15
Q

Sweat Chloride Test

A

Primary test for dx of CF

Positive Sweat chloride: 60-165 meq/L

Borderine sweat chloride: 40-60 meq/L

Normal sweat chloride:
0-40

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16
Q

Sweat chloride test:
False Positives
False Negatives

A 
False positives:
Hypothyroidism
Addison disease
Ectodermal dysplasia
Glycogen storage disease
Edema
Malnutrition
Lab error (evaporation or contamination of sample)
False negatives:
Edema
Malnutrition
Some CF mutations
Sample diluted
17
Q

Genetic Testing

A

Mutation analysis available

Testing varies

18
Q

Prenatal Screening

A

Option of prenatal screening for CF is recommended

Sensitivity of prenatal screen in white population < or =78

19
Q

Newborn Screening

A

Goal: dx early

Evidence that early dx leads to better nutritional outcome and chest radiographic scores

20
Q

Tx

A

While the focus of this discussion is on pulmonary therapies, it must be kept in mind that management is often suboptimal unless the multisystem nature of the disease is considered. Sinus infection, nutritional status, glucose control, and psychosocial issues must all be assessed at regular intervals. This requires a multidisciplinary approach to care that, in the United States, is best provided at one of more than 130 CF Care Centers

21
Q

Aerosolized Abx

Tobramycin

A

The most common airway pathogen in patients with CF is Pseudomonas aeruginosa.
For patients with CF, 6 years of age and older, who have moderate to severe lung disease and with P. aeruginosa persistently present in cultures of the airways, the Cystic Fibrosis Foundation strongly recommends the chronic use of inhaled tobramycin to improve lung function and reduce exacerbations.

22
Q

Azithromycin

A

For patients with CF, 6 years of age and older, and with Pseudomonas aeruginosa persistently present in cultures of the airways, the Cystic Fibrosis Foundation recommends the chronic use of azithromycin to improve lung function and to reduce exacerbations

23
Q

Tx

Pulmonary Toilet/Airway Clearance

A

Chest physiotherapy
Postural drainage and percussion
P.E.P valve, Acapella valve, Flutter valve
High frequency chest wall oscillation

Albuterol
Bronchodilation
Increase ciliary efficiency

Dornase alpha/recombinant DNase
Hypertonic Saline by nebulization

24
Q

Tx
Chronic infection
Pulmonary Exacerbation

A

Inhaled antibiotics
Inhaled tobramycin in patients with pseudomonas
Sputum cultures

Treatment of pulmonary exacerbation
Pulmonary exacerbation—change in symptoms and signs from baseline (cough, sputum production, lung function, increased crackles on physical exam)
Requires hospitalization for antibiotics IV, as well as increased airway clearance

25
Q

Dornase Alpha

A

Recombinant human DNase (dornase alfa) was developed to degrade the large amount of free DNA that accumulates within CF mucus, thereby improving the viscoelastic properties of airway secretions and promoting airway clearance.

26
Q

Hypertonic Saline Inhalation

A

increase hydration of airway surface liquid in patients with CF, thereby improving mucociliary clearance

27
Q

Anti-Inflammatory Agents

A

Regular Ibuprofen use recommended
showed slower decrease in FEV1 and better weight maintenance
Best effect in pt’s <13yo

28
Q

Cromolyn

A

Evidence insuficient to recommend for or against cromolyn use in CF

29
Q

Corticosteroids

A

Evidence is insufficient to recommend for or against use of PO corticosteroids

Recommended against routine use of inhaled corticosteroids

30
Q

Psychosocial Issues

A 
QOL affected by: 
Frequent hospitalizations
Time spent on therapies
Morbidity from disease
Restrictions secondary to disease

Discuss c pt:
Adherence to therapies
Family planning
Endo of life issues

Pulmonary (9 decks)

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