Cystic Fibrosis – Nursing Diagnosis and Care Plan


Physiology of Respiratory System

Inspiration: Intake of warmed moistened air to the lungs leading to alveolar exchange of oxygen to hemoglobin and diffusion of carbon dioxide into alveoli.

Expiration: Discharge of carbon dioxide filled air to outside.

Control of respiratory center: Located in the medulla. Changes in pH, percentage of carbon dioxide and oxygen, temperature and blood pressure stimulate the respiratory center to slow or increase respiratory activity.





Anatomical differences in children

• Frontal and sphenoidal sinuses do not develop until 6-8 yrs.

• Tonsillar tissue is enlarged in school age children.

• Newborns have very little respiratory mucus leading to a high risk for infection.

• Excess mucus leads to obstruction in infants and toddlers.

• After 2 years the right bronchus become shorter, wider and more vertical than left. Therefore foreign bodies easily lodge in right bronchus.

• Infants use abdominal muscles for breathing.

• Children use more of accessory muscles for breathing. They have a high risk for respiratory distress.

• Infants have less cartilage in the walls of the airways and they collapse easily after expiration.

• As the cartilages of the airways are not well developed infants do not develop bronchospasm easily. Therefore wheezing is absent even with severely compromised lumen


Signs and symptoms of respiratory dysfunction

Physical examination

• Purulent rhinitis

• Reddened nasal mucosa

• Flaring of alae nasi

• Petechiae on palate

• Red, swollen tonsillar tissue

• Harsh or ineffective speech

• Swollen and tender cervical lymph nodes

• Adventitious lung sounds (rhonchi, rales, wheezing)

• Tachypnea and tachycardia

• Retraction of supraclavicular, intercostal or subcostal muscles

• Hyperresonance (distended alveoli)

• Dull sound with percussion (consolidated alveoli)

• Headache from sinusitis

• Rubbing ear from ear pain

• Fever

• Coughing

• Cyanosis

• Non-midline trachea

• Grunting sound on expiration

• Dyspnea or apnea

• Crowing sound on inspiration (stridor)

• Enlarged antero-posterior chest diameter

• Increased or decreased vocal fremitus

• Clubbing of fingers


Laboratory tests and investigations

Blood gas values

• Partial pressure of oxygen (PaO2) – 80 – 100 mm Hg

• Partial pressure of carbon dioxide (PaCO2) – 35 – 45 mm Hg

• pH 7.35 – 7.45

• Oxygen saturation (SaO2) – 96 – 98%

• Bicarbonate – (HCO3) – 22 – 26 mEq/liter

• Pulse oximetry

• Transcutaneous oxygen monitoring

• Nasopharyngeal culture

• Nasal washing

• Sputum analysis

Diagnostic procedures

• Chest X-ray

• Bronchography

• Pneumogram: A continuous monitor read out showing the respiratory rate and rhythm, amplitude of inspiration and frequency and duration of apnea using thoracic impedance monitoring.

• Thoracentesis

• Pulmonary function test: Study of lung capacities and volumes includes tidal volume, vital capacity, minute volume, residual volume and forced expiratory volume.


Therapeutic techniques in the treatment of respiratory illness

• Expectorant therapy

– Oral fluids

– Liquefying agents (expectorant)

–  Humidification
– Vaporizers
– Nebulizers
– Mist tents
– Metered dose inhalers

• Coughing

• Chest physiotherapy

– Postural drainage with percussion

– Vibration


Therapies to improve Oxygenation

• Oxygen administration

• Pharmacologic therapy

– Antihistamines (nasal sprays or drops)

– Bronchodilators

• Salbutamol (Ventolin)

• Theophylline

• epinephrine

– Antiinflammatory

– Incentive spirometry

• Breathing techniques

– Blowing through straw or balloon

• Suctioning

• Tracheostomy

• Endotracheal intubation

• Assisted ventilation



– An inherited disease of exocrine glands (glands whose secretion reaches an epithelial surface either directly or through a duct), affecting the pancreas, respiratory system and apocrine sweat glands.

– CF usually begins in infancy and is characterized by chronic respiratory infection, pancreatic insufficiency and increased electrolytes in sweat.

– CF is the major cause of severe chronic lung disease in children.


The exocrine gland

• These are glands that release their secretions into organs, body cavities, or to the outside through the ducts.

• These glands may be simple , tube like structures as found in the stomach wall and in the lining of the intestine, or complex, tree like systems of ducts, as found in the liver, pancreas and salivary glands.

• They produce a variety of secretions along with varying amounts of cellular materials.

• Examples of exocrine secretions are sweat, saliva, mucus and other digestive juices.


There are 2 types of sweat glands in the body:

1. Eccrine sweat glands:

– These open directly onto the skin surface.

– In CF there is defect in the electrolyte of the eccrine sweat glands, resulting in high concentration of sodium chloride in the sweat.

– The diagnosis of CF is by finding high concentration of sodium chloride in sweat test of these children.


2. Apocrine sweat glands:

– These are located in the axillae and pubic region and they open into hair follicles rather than directly onto the surface of the skin. They appear after puberty.


Cystic fibrosis

– A disease in which there is generalized dysfunction of the exocrine glands. Mucus secretions of the body particularly pancreas and lungs are affected. Involvement of sweat glands leads to electrolyte imbalances.

Etiolgy of Cystic fibrosis

Chromosomal abnormality of the long arm of the chromosome 7 leads to an inability to transport molecules across cell membranes resulting in dehydration of epithelial cells in airways and pancreas.

Pathophysilogy of Cystic fibrosis



Effects of cystic fibrosis  on various organs

– Lungs:

• Bronchiectasis

• Bronchitis

• Pneumonia

• Atelectasis

• Hyper inflation

• Pneumothorax

• Sinusitis

• Nasal polyps

• Secondary corpulmonale

– Pancreas:

• Pancreatic insufficiency

• Malaabsorption

• Steatorrhoea

• Pancreatitis

• Diabetes mellitus

• Meconium ileus (Newborn)


– Liver:

• Sub clinical cirrhosis

• Portal hypertension

• Esophageal varices

– Gall bladder:

• Gall stones

– Genital:

• Sterility

– Sweat glands:

• Salt depletion

• Electrolyte imbalances

– Salivary glands:

• Plugging and dilatation of ducts

• Abnormal electrolyte concentrations in saliva


Assessment findings of Cystic Fibrosis

• Respiratory

• Cough, 1st dry and hacking, then productive

• Greenish sputum with infection

• Altered sleep and activity from cough

• Repeted bronchitis, bronchopneumonia, bronchiolitis

• Wheezing

• Shortness of breath

• Decreased exercise tolerence

• Slowing of weight gain and physical growth

• Barrel shaped chest

• Clubbing of fingers, toes,

• Cyanosis

• Hemoptysis

• Atelectasis, pneumothorax, emphysema, respiratory failure an


• Meconium ileus

• Newborn will not pass meconium in 24 hrs.

• Abdominal distention and vomiting

• Pancreatic enzyme deficiency leading to

– Impaired conversion of food into absorbable nutrients.

– Impaired fat absorption

– Deficiency of vit. A,D,E and K.

– Decreased muscle mass and thin extremities

– Poor weight gain

– Bulky, foul smelling, frothy stool (steatorhhea)

– Prolapse of rectum, fecal impaction and intussusception

• Pancreas

• Acute pancreatitis & diabetes mellitus

• Genito urinary

• Delayed sexual functions

• Secondary amenorrhoea and cevicitis

• Infertility in male and female

• Sweat glands

• Infant tastes salty when kissed

• Salt frosting on skin

• Salt depletion specially in summer


Nursing Diagnosis and Care Plan  for Cystic Fibrosis

• Nursing diagnoses 1: Ineffective airway clearance R/T inability to clear mucus.

• Interventions

– Provide moistened oxygen (30-40%) to correct hypoxia and acidosis.

– Use mucolytic agents and do not give cough suppressants.

– Aerosol therapy 3-4 times a day.

– Postural drainage

– Encourage activity

– Provide respiratory hygiene

– Frequent mouth washes

– Frequent observation of the child

• Nursing diagnosis 2: Altered nutrition less than body requirement R/T inability to digest fat.

• Interventions

– Provide a high calorie, high protein, moderate fat diet.

– Supplement vit. ADE and K as water miscible forms

– Add extra salt in diet, especially during summer.

– Supplement with synthetic pancreatic enzymes before each snack and meal.

– In small infants open the capsule and mix the contents to 1 tsp. of food or warm water and give. It should not be added to bottle or formula.

– Make sure the room temperature is always below 720F to prevent excessive perspiration


• Nursing diagnosis 3: Risk for altered skin integrity R/T acid stools

• Interventions

– For infants change diapers immediately when the stool is passed.

– After a bowel movement check the child’s rectum for prolapse.

– If prolapse is present with a gloved lubricated finger replace it gently back.

– After replacement tape the buttocks together to maintain gentle pressure on the anus.


• Nursing diagnosis 4: High risk for ineffective family coping R/T chronic illness of a child.

• Interventions

– Teach the home care

– Regular follow up visits

– Arrange for schooling or a home tutor

– Encourage all family members in giving care

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