Care client’s with respiratory disorder CYSTIC FIBROSIS
INTRODUCTION• Cystic fibrosis also known as CF or mucoviscidosis is a common recessive genetic disease which affects the entire body,causing progressive disability and often early death.• Approximately 30,000 Americans have CF, making it one of the most common life-shortening inherited diseases in United States.
DEFINITON• Cystic fibrosis is a disease passed down through families that cause thick, sticky mucus to build up in the lungs, digestive tract and other areas of the body.• It is one of the most common chronic lungs disease in children and young adults.
ETIOLOGY• Caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR).• This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb it.
RISK FACTOR• Family history. Because cystic fibrosis is an inherited disorder, it tends to run in families.• Race. Although cystic fibrosis occurs in all races, it is most common in white people of northern European ancestry.
PATHOPHYSIOLOGYCFTR loss of its function as a chloride ion transporter caused by misfolding proteinAbnormal sodium and chloride transport across cell membrane,causing thick tenacious secrection in lung and pancrease Thick,sticky respiratory secretions that are difficult to remove cause airway obstruction,resulting in air trapping and frequent respiratory infection.
CLINICAL MANIFESTATION• Symptoms related to the lungs and sinuses may include : Coughing or increased mucus in the sinuses or lungs Fatigue Nasal congestion caused by the nasal polyps Recurrent episodes of pneumonia.
CON’T…Symptoms in newborns may include :Delayed growthFailure to gain weightNo bowel movement (meconium)Salty-tasting skin
MEDICAL TREATMENT1.Antibiotics to prevent and treat lung and sinusinfections Eg:Azithromycin
CON’T…2.Anti-inflammatory medications may helpreduce the inflammation in your lungs that iscaused by ongoing infections.Eg:Steroid and Ibuprofen.
CON’T…3.Inhaled medicines to help open theairways. (eg:Mucolytics like Bisolven®)
CON’T…4.Gene therapy. attempts to place a normal copyof the CFTR gene into affected cells5.Oxygen therapy may be needed as lungdisease gets worse.
SURGICAL TREATMENT• Lungs transplantation requires surgery to replace one or both of your lungs with healthy lungs from a human donor.
COMPLICATION• Many different types of complication can occur with CF. The most complications affect the respiratory,and digestive tract. Respiratory system include :BronchiectasisChronic infectionCollapsed lungsNasal polyps
CON’T…• Digestive complication• Nutritional deficiencies• Diabetes• Blocked bile duct
PREVENTION• Actually,there is no way to prevent cystic fibrosis because it is genetic disorder.• But,if people or their partner has close relatives who have cystic fibrosis,they both might want to undergo genetic testing before having children of they own.• If woman already pregnant and the test shows that her baby may be at risk of cystic fibrosis, doctor can conduct additional tests on her developing child.
HEALTH TEACHING• Encourage patient avoid smoke, dust, dirt, fumes, household chemicals, fireplace smoke.• Teach patient clearing or bringing up mucus or secretions from the airways.
CON’T…• Encourage patient drinking plenty of fluids.• Encourage patient to do exercising two or three time a week.
NURSING CARE PLAN 1• Nursing diagnosis : Ineffective airway clearance related to excess tenacious mucus.• Expected outcome : To improve pulmonary, GI and pancreatic status.
Nursing Intervention RationalEncourage regular exercise Exercise helps maintain physical wellness and supplements the patients airways clearance strategies by helping to loosen pulmonary secretion.Administer oxygen therapy and receive digoxin To decrease the lungs work.and/or diureticsEncourage coughing, deep breathing exercise Promote lungs expansion, mobilization, andand frequent position changes drainage of secretions.Monitor respiratory rate, depth and work of Respiratory may remain somewhat rapidbreathing because hyperthyroid state, but development of respiratory distress is indicative of tracheal compression from edema or hemorrhage.Assist with repositioning, deep breathing Maintains clear airway and ventilation.exercises, and coughing as indicated
NURSING CARE PLAN 2• Nursing diagnosis : Imbalanced nutrition related to poor appetite and increased calorie expenditure.• Expected outcome : The patient’s weight will be stable at desired weight for height
Nursing Intervention RationalMonitor food intake and weekly weight. Regular monitoring can help identify nutriton problem that are severe.If the patient is too dyspneic to eat, schedule Eating takes a lot of energy and resting canrest periods and bronchodilator treatments help conserve energy before meal.before meals.Create a pleasant eating environment. Unpleasant views or odors can spoil an appetite.Provide smaller and more frequent meals of Eating a lot at one time can fill up the stomachthe patient’s favourite food. and reduce the room for lung.Encourage family members to bring favourite A large tray of unappetizing food may be morefoods from home for hospitalized patient. than a patient can handle and may spoil the appetite.
NURSING CARE PLAN 3• Nursing diagnosis : Anxiety related to dyspnea.• Expected outcome : The patient will state anxiety is controlled. The patient will be able to use technique to control dyspnea and anxiety when they occur.
Nursing Intervention RationalRemain with the patient who is acutely Feeling alone during episodes of dyspnea candyspneic and anxious. increased anxiety.Calmly remind the patient to breath slowly in During acute episodes of dyspnea, the patientthrough the nose and out through pursed lips. may forget that breathing exercises can help.Teach deep breathing exercises during times Relaxation can help reduce muscle tensionwhen anxiety is minimal and remind the patient and distract the patient.to use them during acute anxiety.Administer anti anxiety medication. Medication can reduce anxiety but can also depress respiration, so should be use with caution.
CONCLUSION• Cystic fibrosis transmembrane conductance regulator(CFTR) play important role in function as a chloride ion transporter.• Although there is no cure for CF, treatments have improved greatly in recent years. Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, some people who have CF are living into their forties, fifties, or older.
REFERENCE1. Texts Book:a. Linda S.WILLIAMS, paula D.HOPPER., Understanding medical surgical nursing vol 1, 3rd edition international edition.2.The webside about cystic fibrosis:a.http://www.medicinenet.com/cystic_fibrosis/article.htmb.http://kidshealth.org/teen/diseases_conditions/digestive/cy stic_fibrosis.html#c.http://www.cff.org/treatments/Therapies/d.http://www.youtube.com/