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Cystic Fibrosis – Nursing Diagnosis and Care Plan

August 16th, 2011 Admin

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.

• Infants use abdominal muscles for breathing. • After 2 years the right bronchus become shorter. Therefore foreign bodies easily lodge in right bronchus. • Newborns have very little respiratory mucus leading to a high risk for infection. Therefore wheezing is absent even with severely compromised lumen Signs and symptoms of respiratory dysfunction .Anatomical differences in children • Frontal and sphenoidal sinuses do not develop until 6-8 yrs. • Excess mucus leads to obstruction in infants and toddlers. • Tonsillar tissue is enlarged in school age children. wider and more vertical than left. • Infants have less cartilage in the walls of the airways and they collapse easily after expiration. • Children use more of accessory muscles for breathing. They have a high risk for respiratory distress. • As the cartilages of the airways are not well developed infants do not develop bronchospasm easily.

wheezing) • Tachypnea and tachycardia • Retraction of supraclavicular.Physical examination • Purulent rhinitis • Reddened nasal mucosa • Flaring of alae nasi • Petechiae on palate • Red. rales. 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 . swollen tonsillar tissue • Harsh or ineffective speech • Swollen and tender cervical lymph nodes • Adventitious lung sounds (rhonchi.

minute volume. Therapeutic techniques in the treatment of respiratory illness • Expectorant therapy .• 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. 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.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.35 – 7. residual volume and forced expiratory volume. vital capacity.

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 .Vaporizers .– Oral fluids – Liquefying agents (expectorant) – Humidification .Mist tents .Nebulizers .

These are located in the axillae and pubic region and they open into hair follicles rather than directly onto the surface of the skin. Eccrine sweat glands: .An inherited disease of exocrine glands (glands whose secretion reaches an epithelial surface either directly or through a duct). They appear after puberty. as found in the liver.CF usually begins in infancy and is characterized by chronic respiratory infection. pancreatic insufficiency and increased electrolytes in sweat. Apocrine sweat glands: . • They produce a variety of secretions along with varying amounts of cellular materials. respiratory system and apocrine sweat glands. .The diagnosis of CF is by finding high concentration of sodium chloride in sweat test of these children. saliva. or complex.• Endotracheal intubation • Assisted ventilation CYSTIC FIBROSIS (CF) . . . or to the outside through the ducts. SWEAT GLANDS There are 2 types of sweat glands in the body: 1. resulting in high concentration of sodium chloride in the sweat.CF is the major cause of severe chronic lung disease in children. tree like systems of ducts. The exocrine gland • These are glands that release their secretions into organs. . • Examples of exocrine secretions are sweat. body cavities.In CF there is defect in the electrolyte of the eccrine sweat glands. tube like structures as found in the stomach wall and in the lining of the intestine.These open directly onto the skin surface. 2. . mucus and other digestive juices. affecting the pancreas. pancreas and salivary glands. • These glands may be simple .

Lungs: • Bronchiectasis • Bronchitis • Pneumonia • Atelectasis • Hyper inflation . Mucus secretions of the body particularly pancreas and lungs are affected. 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.A disease in which there is generalized dysfunction of the exocrine glands. Pathophysilogy of Cystic fibrosis Effects of cystic fibrosis on various organs . Involvement of sweat glands leads to electrolyte imbalances.Cystic fibrosis .

• Pneumothorax • Sinusitis • Nasal polyps • Secondary corpulmonale .Liver: • Sub clinical cirrhosis • Portal hypertension .Pancreas: • Pancreatic insufficiency • Malaabsorption • Steatorrhoea • Pancreatitis • Diabetes mellitus • Meconium ileus (Newborn) .

then productive • Greenish sputum with infection • Altered sleep and activity from cough • Repeted bronchitis.• Esophageal varices .Gall bladder: • Gall stones . bronchiolitis • Wheezing • Shortness of breath • Decreased exercise tolerence • Slowing of weight gain and physical growth • Barrel shaped chest • Clubbing of fingers. 1st dry and hacking. toes.Salivary glands: • Plugging and dilatation of ducts • Abnormal electrolyte concentrations in saliva Assessment findings of Cystic Fibrosis • Respiratory • Cough.Genital: • Sterility .Sweat glands: • Salt depletion • Electrolyte imbalances . . bronchopneumonia.

– Decreased muscle mass and thin extremities – Poor weight gain – Bulky. • Abdominal distention and vomiting • Pancreatic enzyme deficiency leading to – Impaired conversion of food into absorbable nutrients. – Impaired fat absorption – Deficiency of vit.E and K.D. foul smelling. 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 .• Cyanosis • Hemoptysis • Atelectasis. A. emphysema. frothy stool (steatorhhea) – Prolapse of rectum. pneumothorax. respiratory failure an .GIT • Meconium ileus • Newborn will not pass meconium in 24 hrs.

– 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. – Supplement with synthetic pancreatic enzymes before each snack and meal. – Use mucolytic agents and do not give cough suppressants. – Aerosol therapy 3-4 times a day. It should not be added to bottle or formula. • Interventions – Provide moistened oxygen (30-40%) to correct hypoxia and acidosis. of food or warm water and give. ADE and K as water miscible forms – Add extra salt in diet. – Supplement vit. • Interventions – Provide a high calorie. high protein. – In small infants open the capsule and mix the contents to 1 tsp. – 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 . especially during summer. moderate fat diet.• 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.

com/2011/08/cystic-fibrosis-nursing-diagnosis-and-careplan. • Interventions – Teach the home care – Regular follow up visits – Arrange for schooling or a home tutor – Encourage all family members in giving care Source: http://www.nursing-lectures. – After a bowel movement check the child’s rectum for prolapse.html . – After replacement tape the buttocks together to maintain gentle pressure on the anus. – If prolapse is present with a gloved lubricated finger replace it gently back.– For infants change diapers immediately when the stool is passed. • Nursing diagnosis 4: High risk for ineffective family coping R/T chronic illness of a child.