Cystic Fibrosis

Rectal prolapse:

Although cystic fibrosis can present with constipation and rectal prolapse, the disorder is much less common than functional constipation. In most children, prolapse is associated with malnutrition rather than due to cystic fibrosis alone. In patients who have rectal prolapse, a sweat chloride analysis may be useful.

"- When a kid comes into the ED with CF, who has failed outpt medications, it is most likely that she/he has endobronchitis and need aggressive treatment.  They almost NEVER clear their bacteria from their lungs and this agressiveness will actually keep them from being admitted and lengthen thier life.

When admitted for endobronchitis (in general of course).... always high protein/ high fat diet; and increase vest/percussive treatments during acute illness....

For CF kids, the dosing for Tobramycin is 3mg/kg every 8 hours and then custom tailored to shoot for a peak levels between 8-10.  The pink book has a range, but this seems to work best."  
-Sterling Simpson, MD

 

Hemoptysis
Clubbing
Constipation, functional
Sinusitis, Chronic