Reyzelin M.

Endrenal 04/ 08/2022

BSN-4A

CYSTIC FIBROSIS

PATHOPHYSIOLOGY SIGN AND SYMPTOMS

RISK FACTOR Autosomal recessive o Persisted cough and
disorder that is caused phlegm
by mutation on o Postnasal drip
MODIFIABLE FACTOR NON-MODIFIABLE chromosome 7 o Salty tasting skin
FACTOR o Wheezing
o Vitamin D This mutation result in a o Shortness of breath
deficiency o Common in male defective membrane CI o Sinuses
o CF related o Common in channel (CFTR), causing o GI problems
diabetes mellitus Caucasians defective chloride and o GU problems
o Low BMI o Genetics water transport in o Clubbing of digits
o Post lung epithelial cells o Steatorrhea
transplant o Meconium ileus

PHARMACOLOGICAL
LABORATORY AND
o Nebulized antibiotics include MEDICAL DIAGNOSIS
DIAGNOSTIC PROCEDURE
tobramycin, colistin,
gentamicin, and ceftazidime CYSTIC FIBROSIS o Immunoreactive
o Mucus-thinning meds
trypsinogen (IRT)
o NSAIDS
o Sweat chloride test
o Bronchodilators
o Sputum test
o Chest X-ray
o CT-scan
o Pulmonary function tests
NURSING DIAGNOSIS

o Infection r/t airway pathogens s/t cystic fibrosis NURSING INTERVENTIONS

o Ineffective airway clearance r/t increased sputum production
s/t cystic fibrosis o Assess respiratory rate
o Impaired gas exchange r/t bronchiectasis with decreased o Encourage frequent
surface area for gas exchange and loss of lung function s/t effective cough
cystic fibrosis o Administer oxygen as
o Imbalanced nutrition: less than body requirement r/t poor prescribed
nutritional status resulting to anorexia s/t cystic fibrosis o Monitor pulmonary
function test
EXPECTED OUTCOMES o Administer medication
as prescribed
o Patient demonstrate healthy hygiene through handwashing o Administer all fat-soluble
o Patient does not show signs of infection vitamins and enzymes
o Patient maintains adequate nutritional intake o Assess baseline
o Patient maintains compliance in health routine knowledge of disease
o Patient maintained a healthy airway pathophysiology
Reyzelin M. Endrenal 04/ 08/2022
BSN-4A

CYSTIC FIBROSIS

REFERENCES:
https://prezi.com/zod3aac4zw7l/respiratory-concept-map-cystic-fibrosis/
https://www.healthline.com/health/cystic-fibrosis#treatments
https://www.researchgate.net/figure/Perceived-risk-factors-for-increased-bone-loss-in-people-
with-cystic-fibrosis-CF_tbl1_45151719