Cystic Fibrosis

Question 1

How does a patient present with cystic fibrosis?

Answer 1

Failure to gain weight, loose greasy stools, intermittent productive cough, wheezing, past medical history of pneumonia multiple times

Question 2

What are the positive physical examination for this patient?

Answer 2

Emaciated, lower than expected height and weight, generalized hyper-resonance, scattered crepitations bilaterally, expiratory wheezes

Question 3

What are the possible vital signs?

Answer 3

TachyCardia hypertension, fever

Question 4

What is the initial order that should be done for cystic fibrosis patients?

Answer 4

Admit to ward, Pulse oximetry Q4, IV access,Sputum Gram stain, sputum culture and sensitivity, blood culture, CBC with differential, chest x-ray PA/lateral, sinus x-ray, BMP, sweat chloride, 72 hour fecal estimation

Question 5

What is the initial treatment for this patient?

Answer 5

Ambulation at will, regular diet, vitals Q6, D5NS, Chest physiotherapy, multivitamin tablets, nebulized albuterol four times a day, amoxicillin and clavulanic acid Augmentin, oxygen inhalation of saturation is less than 92% on room air

Question 6

What is the findings for CBC, sweat chloride, BMP, x-ray⁉️

Answer 6

CBC has neutrophilic leukocytosis, BMP has low sodium and potassium, x-ray shows hyper inflation of both lung fields and Opacification of paranasal sinuses

Question 7

What are the symptoms for cystic fibrosis?

Answer 7

Persistent or acute respiratory symptoms, failure to thrive, the conium Ellis ileus, diarrhea, rectal prolapse, nasal polyps, electrolyte or acid-based disorders and hepatobiliary disease

Question 8

What is used to diagnose describe roses?

Answer 8

Elevator sweat chloride on two separate occasions as evidence of CFT are dysfunction

Question 9

What is evidence to diagnose malabsorption?

Answer 9

24 hour fecal fat estimation

Question 10

What prophylactic immunizations should be given for cystic fibrosis patients?

Answer 10

Influenza, measles, pertussis

Question 11

What is the expected FEV1 for cystic fibrosis?

Answer 11

30% or less of the predicted value

Question 12

What is the nutrition for cystic fibrosis patients?

Answer 12

high protein and high calories, fat soluble vitamins, pancreatic enzyme replacement

Question 13

What is a drastic measurement for patients with cystic fibrosis who have severe infections and a grossly damaged lungs?

Answer 13

Long transplantation

Question 14

What can be used for patients with CF who have a hyper active airway?

Answer 14

Inhailed glucocorticoids

Question 15

What could be given for patients with daily productive cough plus airway attraction?

Answer 15

DNase

Question 16

What should be given for a patient who exhibits airway obstruction?

Answer 16

Bronchodilators like albuterol for salmeterol

Question 17

We should be done for CF patients with more retained purulent secretions

Answer 17

Physiotherapy, and exercise

Question 18

oxygen therapy should be given to with patients?

Answer 18

Patients with hypoxemia at night or rest or pulmonary hypertension

Question 19

What antibiotic should be given for staph aureus patients?

Answer 19

Cephalexin, dicloxacillin or amoxicillin clavulanate (all oral antibiotics)

Question 20

We should be given for Pseudomonas aeruginosa infections?

Answer 20

Ciprofloxacin as oral antibiotic; If IV regimen then a combination of tobramycin and anti-pseudomonal penicillin like pipercillin