Management Of Cystic Fibrosis Flashcards

1
Q

Cystic fibrosis overview

A

Defect in CFTR proteins and prevents them transporting chloride in and out of cells.

This leads to the following three primary physiological effects:

1) increased chloride and sodium concentration in sweat
2) dehydration of mucus layers and inability to move mucus layers. This leads to chronic infections and destruction of epithelial cells since mucocillary transport is at a halt
3) increased sodium and water reabsorption rates in cells

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2
Q

Primary 4 organ systems affected by cystic fibrosis

A

Respiratory
- increased recurrent upper and lower respiratory infections (especially pneumonia)

GI
- primarily pancreatic and liver

Integumentary

Reproductive

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3
Q

Pulmonary symptoms related to cystic fibrosis

A

Recurrrent pneumonia And/or sinusitis

Chronic coughing

Wheezing

Nasal polyp formations

Bronchiectasis

Obstructive lung disease issues on PFTs

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4
Q

Acute pulmonary exacerbation

A

A consequence of untreated cystic fibrosis

Symptoms

  • increased productive coughing
  • fever
  • change in sputum color
  • hemoptysis
  • dyspnea
  • sinus pain/tenderness
  • fatigue/malaise/lethargy
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5
Q

4 most common infectious agents in cystic fibrosis

A

1) staph aureus
2) haemophilus influenza

3) pseudomonas aeruginosa
* most dangerous*

4) bulkhoideria cepacia
* 3 and 4 are dangerous and harder to cure, but are still rarer than 1 and 2. However cystic fibrosis populations have the highest rates *

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6
Q

GI symptoms related to cystic fibrosis

A

Pancreatic insufficiency/ pancreatitis
most common and will lead to diabetes

Prolonged neonatal jaundice

Biliary cirrhosis

Meconium ileus

  • blockage of the ileum in children
  • “soap bubble” signs on xray that are really black
  • palpable mass in liver area

Steatorrhea

Malabsorption of fat-soluble vitamins
- A,D,E,K

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7
Q

Integumentary symptoms related to cystic fibrosis

A

Salty skin
- babies taste salty

Digital clubbing

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8
Q

Reproductive symptoms related to cystic fibrosis

A

99% of males w/ CF are infertile since the ejaculatory duct and vas deferens are not present as well as hypoplasia of seminal vesicles

Females tend to have difficulty getting pregnant but still can

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9
Q

Diagnosis of cystic fibrosis

A

Newborn screening
- check for immunoreactive trypsinogen levels (will be really high for CF patients)

Sweat test
- patient oversweats w/ prevalence of stupid high chloride levels

Genetic testing

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10
Q

Treatment of cystic fibrosis

A

Goals:

  • increase life expectancy (right now it hovers around 40)
  • improve quality of life
  • optimize nutritional status
  • optimize pulmonary function

Non pharm:

  • OMM (especially rib raising)
  • chest physiotherapy
  • exercise regiments

Pharm:

  • mucinolytics
  • Nebulizer
  • albuterol
  • fat soluble vitamin supplements
  • pancreatic enzyme replacements
  • antibiotics (only if infection is present)
  • NO STEROIDS
  • lung transplant (last resort)
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