Cystic fibrosis Flashcards
2 y/o boy c/o failure to gain weight and loose stools
VS: 100, 80/56, Temp: 101.2, 18 bpm, 27.2”, 7.2 kg
C/o loose greasy stools, failure to gain weight despite adequate nutrition. Intermittent productive cough and rhinorrhea. Has been wheezing and coughing purulent mucous x 4 days. Deny F/C. Has had pna 4x since birth. Hx of CF on both sides.
What’s your approach?
Problem: FTT. can be organic and non-organic. Etiology consistent with CF.
Clues for CF: + family hx, repeated chest infections, malabsorption diarrhea.
What should you do first?
Complete physical exam
Results: emaciated. Height and weight lower than expected for age
Chest exam: hyper resonance and scattered crepitations b/l
Wheezing with expiration.
What do you order?
Admit to floor Pulse ox stat and q4 IV access, stat Sputum cx with sens and gram stain, stat Blood cultures stat CBC with diff stat BMP stat CXR AP and lat stat X-ray sinuses routine Sweat chloride routine 72-hour decal fat estimation O2 for sats <92%, continuous Augmentin oral continuous Neb albuterol inhalation QID, continuous Chest PT Vitals q6 D5NS IV continuous Reg diet Up at lib
Lab results: CBC shows neutrophilic leukocytosis Sweat 85 Low Na and K CXR: hyperinflation b/l Opacification of para nasal sinuses GS: no predominant organism Cx: NGTD Fecal fat pending
What do you make of these results?
CF: clinical mani: acute or Persistent respiratory symptoms, failure to thrive, meconium ileus, diarrhea, rectal prolapse, nasal polyps, electrolyte or acid base disorders and hepatobiliary disease
Dx: evidence of CTFR dysfunction (Sweat elevated x 2) + typical clinical features or positive family hx
Also important to get gram stain, cx, sensitivities, CXR, sinus films. Fecal fat = malabsorption. Genetic testing not required.
What do you do with a child presenting with CF?
Admit to hospital
Baseline testing, accurate dx, initiation of treatment and education of caregivers
F/u q 2-3 months for monitoring
- H&P
- staining and cx of sputum or pharyngeal swab
Ppx immunization against flu, measles, pertussis
Treatment of CF?
Abx for chest infection. Select depending on cx
- S. aureus: cephalexin, dicloxacillin, augmentin
- P. Aeruginosa oral: cipro, IV: tobra + piperacillin
Bronchodilator if have airflow obstruction
DNase if daily cough + airflow obstruction
Combo chest PT + exercise for pts with more retained purulent secretions
Inhaled steroids if have airway hyperactivity
O2 if have hypoxemia at night, at rest, or with pulm HTN
Lung transplant only definitive treatment with FEV1 30% or less
Diet: high protein and high cal. Fat soluble vitamin supplementation. Pancreatic enzyme replacement
Fecal fat results elevated
Sputum grew Staph A sens to cephalexin
What do order now?
D/C augmentin start cephalexin D/C IVF Flu shot Pneumococcal shot Consult dietitian Pancreatic enzymes PO cont Genetic counseling
Follow up 2-3 no
