Cystic Fibrosis

nursing assessment

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Incidence

• transmission is 25% with each pregnancy

• highest in people of northern European ancestry.

• common autosomal-recessive disease in the white population.

• age for diagnosis is 6 to 8 months

• equally common in both sexes

Pathophysiology

• lead to decreased secretion of chloride and increased reabsorption of sodium and water across epithelial cells

• viscosity of mucous gland secretions increases

• secretions in the bronchioles and alveoli causes respiratory changes

• Pancreatic insufficiency results in malabsorption

• reduced water content of secretions

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Nursing Diagnosis

• ineffective airway clearance r/t mucopurulent secretions AEB pt. reports in ability to remove secretions with coughing

• impaired gas exchange r/t airway obstruction AEB pt. is dyspneic with cyanotic skin color

• imbalance nutrition: less than body requirement r/t chronic malabsorption AEB low fat-soluble vitamin levels and muscle wasting

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Wheezy respirations

Hyperresonance on percussion

Dry, nonproductive, paroxysmal cough

Dyspnea

Tachypnea

Bibasilar crackles

Barrel chest

Cyanosis

Clubbing of nails

Distended abdomen

Thin extremities

Sallow skin with poor turgor

Delayed sexual development

Hepatosplenomegaly

Rectal prolapse

medications

Etiology

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IV fluids

Antibiotics

O2 therapy

Beta-andrenergic agonists (albuterol)

Inhaled hypertonic saline

Mucoytics

Sucralfatee

Anti-inflammatories

Corticosteroidds

Oral pancreatic enzymes

Vitamin A,D,E,K supplements

Mystic fibrosis transmembrane regulator modulator (ivacaftor)

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Family history

Race (occurs most often in whites of northern European ancestry)

Autosomal recessive mutation of the CFTR protein found on all exocrine tissues

Diagnostics

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Sputum culture

ABG’s

Sweat Test

CX-ray

Medical surgical Managment

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VTE prophylaxis

HIGH Fat High protein

Nutritional supplements

Activity as tolerated

PT for joint maintenance

Iv fluids

Antibiotics

O2 therapy

Corticosteriods

Pancreatic enzymes

Mineral supplemets