Condition Info - BotMed Application

ConditionName

Cystic fibrosis

ConditionDescription

Symptoms

Yonge age with symptoms:
Chronic productive cough;
Hemoptysis;
Chronic sinusitis;
Cyanosis;
Finger clubbing;  
Bronchiectasis;
Wheezing;
SOB;
Common: sinus pain and polyps;
Recurrent  pulmonary infections:
Pseudomonas;
Staph A;

PhysicalFindings

ClinicalTests

Initial test: stool fat;
Most accurate test: increased sweat chloride test;

Genetic test not always helpful b/c of spontaneous mutation of CFTR gene;

Sputum culture:
H.influ;
Pseudomonas;
Staph A;
Burkholderia cepacia;

RiskFactors

Autosomal recessive, mutation in CFTR gene;
Caucasian population;
Young adult or child with chronic LU dz who has respited LU infections;

IsRedFlag

IsNPLEX

Consequence

Most common fatal autosomal recessive dz;
Failure to thrive;
Malabsorption, fat soluble vitamins def.--> growth failure;
Nasal polyps, sinusitis;
Fat liver, portal hypotension;
Cholelithiasis, biliary cirrhosis;
A lot of lungs problems!
R. ventricular hypertrophy and pulmonary artery dilation;
Bone, intestinal, pancreatic, ST, SP, reproductive;

ProgressionFromCondition

TreatmentOptions

PharmOptions

Antibiotics (must cover Psedomonas);
Inhaled aminoglycosides (only for CF patients);
Inhaled bronchodilators: albuterol;
Hypertonic saline for chronic cough;
Chest physiotherapy;
Lung transplants;

GI:
Nutrition cancelation; 
Pancreatic enzymes;
Fat soluble vitamins (A, D, E, K);

WebLink

https://www.merckmanuals.com/en-ca/professional/pediatrics/cystic-fibrosis-cf/cystic-fibrosis