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Cystic Fibrosis Cheat Sheet by

Med-Surg II

PATHOP­HYS­IOLOGY

A genetic disease that affects many organs and lethally impairs lung function. The underlying problem with CF is blocked chloride transport in the cell membranes. Poor chloride transport causes the formation of muscus that has little water content and is thick. The thick sticky mucus causes problems in lungs, pancreas, liver, salivary glands and tests. The mucus plugs up the airways in the lungs and the granular tissues in non-pu­lmonary organs, causing atrophy and organ dysfun­ction.
CF is most common among whites, and about 4% are carriers. It is rare among African Americans and Asians. Males and females are affected equally. Family history is another risk factor.
CFTR Gene Mutation
Autosomal Recessive

SIGNS & SYMTOMS

Salt tasting skin
Persistent coughing
Dyspnea
Wheezing
Failure to pass Meconium
Weight loss
Frequent resp. infection
clubbing of the fingers.

DIAGNO­STICS

Chloride Sweat Test (>60)
Chest X-Ray
Genetic Screen (CFTR)
 

ASSESSMENT : NURSING

Nonpul­monary
Pulmonary
Abdominal distention
Chest congestion
Gastro­eso­phageal reflux
Limited exercise tolerance
Rectal prolapse
Cough & Sputum production
Foul-s­melling stools
Use of accessory muscles
Steato­rrhea (excessive fat in stools)
Decreased pulmonary function

COMPLI­CATIONS

Infection
Infetility
Diabetes (secon­dary)
Intestinal obstru­ction
Kidney­/Liver
ABG studies show acidosis (low pH), greatly reduced aerial oxygen (PaO2) levels, increased arterial carbon dioxide (PaCo2) levels, and increase bicarb­onate levels.
With infection the patient has fever, an elevated white blood cell count, and decreased oxygen satura­tion.
  

TREATMENT

PHARM
NON-PHARM
Digestive enzymes (vitamins A,D,E, & K)
Aerobic exercise
Lung transplant
Chest physio­therapy
Mucolytics
Exacer­bation therapy
Bronch­odi­lat­ors­/St­eroids
Antibi­otics

EDUCATION

Yearly FLU Vaccine
High Calorie & Protein Diet
Fluids
Genetic counseling prior to having a baby
Cystic Fibrosis Fondation (Resource)

Carrier Testing
 

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  • Published: 8th August, 2019

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