define cystic fibrosis
- inherited lung disorder
-autosomol recessive gene disorder
-results in thick and sticky mucus accumulation in the lungs
-blocks airways and hard to clear out
-Cystic Fibrosis Transmembrane Conductance Regulator (gene)
-systemic disorder
Chromosome 7 gene mutation
how is cystic fibrosis gen inherited
-recessive gene disorder
-inherited two copies of defective CFTR gene from both parents that are carriers
-if both parents carriers 25% child inherited it
-10 million carry gene
anatomical alterations of the lungs
-Excessive production and accumulation of thick, tenacious mucus in the tracheobranchial tree
-Partial bronchial obstruction
-Hyperinflation of alveoli
-Total bronchial obstruction
-Atelectasis
what is newborn screening
-most infants with CF have an elevated blood level of immunoreactive tryspin
-IRT is measured from blood dots collects on a newborn infant and guthrie card
who should get a sweat test
1.all patients with the following should get a sweat test
*infants w.positive CF newborn screening results
*infants w/ symptoms suggesting CF
*members of pt family w/confirmed CF
what is a sweat test
- gold standard diagnostic test for CF
-98% of CF identification
-measures the amount of sodium and chloride in pt sweat
-perform 2 times
sweat test interpretations
infants <6 months
≤ 29mmol/L= normal
30-59mmol/L=intermediate
≥ 60mmol/L= abnormal-CF
infants >6 months old,children,adults
≤ 39mmol/L= normal
40-59mmol/L=intermediate
≥ 60mmol/L=abnormal-CF
what are the clinical manifestations and physical exam
- excessive bronchial secretions
- bronchospasm
- atelectasis
what are the physical examinations of pt w/ Cystic fibrosis
- vital sign:
-tachypnea
-tachycardia
-hypertension - use of accessory muscles of inspiration/expiration
- purse lip breathing
- barrel chest
5.cyanosis - digital clubbing
- cough:
-sputum production
-hemoptysis
8.chest findings:
-decresed fremitus
-hyperresonat percusion
-diminshed breath sounds
-diminished heart sounds
-bronchial breath sounds (over atelectasis)
-crackles
-wheezing
clinical data: pulmonary function test
- obstructive lung disease
-Forced expiratory volume and flow rate findings
: all decreased
-lung volume and capacity
:↑ RV, FRC
:↓ VC
:N or↓- IRV, IC,
:N or↑- Vt, TLC
Clinical data: arterial blood gas
Mild-moderate stages:
Acute respiratory alkalosis
pH↑, PaCO2↓HCO3↓ PaO2↓ SaO2/SpO2↓
severe stage:
chronic respiratory acidosis
pH N, PaCO2↑, HCO3↑, PaO2↓, SaO2/SpO2↓
clinical data: hemodynamics
mod-severe stages
CVP↑, PAP↑, due to extra effort to pump blood into lungs and mucus obstruction
PVR↑as obstruction in lungs rise resistance rises contributing to stain on R.heart=cor Pulmonale
PCWP N, CO N, SV N, CI N, SVR N
Clinical data- laboratory test
1.hematology:
-↑ Hct & Hb( polycythimia)
-↑ WBC
- electrolytes
- hypocloremia - sputum culture
-↑ WBC
-pseudomonas aeruginosa
what are the radiograph findings for a pt w/ cystic fibrosis
- depressed or flattened diaphragm
- right ventricular enlargement
- ares of atelectasis
- tram-tracks-parallel lines indicating thickened bronchial walls due to chronic inflammation *hallmark
what is the primary goal to manage CF
Prevent:
1. pulmonary infections
2. reduce the amount of thick bronchial secretion
3. improve air flow
4. provide adequate nutrition -malnutrition worsens cf outcomes
5. family education
what are the respiratory care treatment protocols
-Oxygen therapy
-airway clearance:
CPT w/ PD,HFCWO,PEP
-lung expansion therapy:
deep breathing
effective cough
-aersolized medications
bronchodilators
mucolytic agenst: inhaled dornase alfa,hypertonic saline:thin mucus make it easier to expel
antibacterial: tobi, aztreonam(cayston)-treat pseudomonas aeruginosa
-CTFR modifier medication
improve cell function of defective gene
ivacaftor: CFTR modulator improves function :improving hydration and transport of Cl
Why do parents say baby’s skin is salty
High chloride levels in skin
