Cystic fibrosis.ppt

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Conducted by: Ms.monika Clinical instructor Army college of nursing

Introduction :

Introduction A hereditary disease of the exocrine glands, usually developing during early childhood. it affecting mainly the pancreas, respiratory system, and sweat glands. It is characterized by the production of abnormally viscous mucus by the affected glands, usually resulting in chronic respiratory infections and impaired pancreatic function. Also called mucoviscidosis .

Definition :

Definition Cystic fibrosis (CF) is an inherited disease that affects the lungs, digestive system, sweat glands , and male fertility. Its name derives from the fibrous scar tissue that develops in the pancreas , one of the principal organs affected by the disease.


Cont……………d Inherited metabolic disorder characterized by production of thick, sticky mucus. The secretions interfere with digestive enzymes and block nutrient absorption. Abnormally salty sweat is the basis for diagnosis of cystic fibrosis.

Organs affected by CF:

Organs affected by CF

Pathophysiology of disease:

Pathophysiology of disease Cystic fibrosis affects the body's ability to move salt and water in and out of cells. This defect causes the lungs and pancreas to secrete thick mucus, blocking passageways and preventing proper function. Concentrated mucous secretions in the lungs plug the bronchi, making breathing difficult, promoting infections, and producing chronic cough, recurrent pneumonia, and progressive loss of lung function, the usual cause of death.

Etiology :

Etiology A defect in the CFTR cystic fibrosis transmembrane conductance regulator gene causes cystic fibrosis (CF). This gene makes a protein that controls the movement of salt and water in and out of your body's cells. In client who have cystic fibrosis, the gene makes a protein that doesn't work right. This causes thick, sticky mucus and very salty sweat. The CFTR protein helps to produce mucus. Mucus is a complex mixture of salts, water, sugars, and proteins that cleanses, lubricates, and protects many passageways in the body, including those in the lungs and pancreas.

Clinical manifestation :

Clinical manifestation Respiratory system cystic fibrosis have thick, sticky mucus that builds up in their airways. frequent coughing Sinusitis pneumonia are frequent in CF. tiredness , dyspnea intolerance of exercise become more common recurrent respiratory infections lead to digital clubbing

b) Digestive System :

b) Digestive System Failure of absorption of nutrients in small intestine specially : fats and proteins. This can cause ongoing diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockage also may occur, especially in newborns. constipation stomach pain and discomfort. abdominal swelling and vomiting poorly digested and absorption of food , the person with CF is often experience underweight


Cont…d malnutrition , including anemia and appetite loss Other gastrointestinal symptoms can include a prolapsed rectum, in which part of the rectal lining protrudes through the anus ; intestinal obstruction rarely, intussusception , in which part of the intestinal tube slips over an adjoining part, cutting off blood supply.

Complication of G.I system :

Complication of G.I system Pancreatitis :This is a condition in which the pancreas become inflamed, which causes pain. Rectal prolapse. Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum Liver disease due to inflamed or blocked bile ducts. Gallstones . internal hemorrhaging, accumulation of abdominal fluid ( ascites ), spleen enlargement liver failure.

Reproductive System:

Reproductive System Men who have cystic fibrosis are infertile (because they're born without a vas deferens. This is the tube that delivers sperm from the testicle to the penis.) A woman who has cystic fibrosis may have a hard time getting pregnant because of mucus blocked cervix.

Sweat glands :

Sweat glands The CFTR protein helps to regulate the amount of salt in sweat. People with CF have sweat that is much saltier than normal, measuring the saltiness of a person's sweat is the most important diagnostic test for CF. Parents may notice that their infants taste salty when they kiss them.

Other sign & symptom :

Other sign & symptom Dehydration increased heart rate Tiredness Weakness decreased blood pressure heat stroke

Diagnostic evaluation :

Diagnostic evaluation Diagnose of cystic fibrosis (CF) based on the results from various tests. a) Newborn Screening Screening of newborns for cystic fibrosis is done by using a genetic test or a blood test. The genetic test shows whether a newborn has faulty CFTR genes.

b) Sweat Test :

b) Sweat Test If a genetic test or blood test suggests cystic fibrosis, a physician will confirm a diagnosis using a sweat test . This test is the most useful test for diagnosing cystic fibrosis. It measures the amount of salt in sweat. For this test, doctors trigger sweating on a small patch of skin on an arm or leg. They rub the skin with a sweat-producing chemical and then use an electrode to provide a mild electrical current. This may cause a tingling or warm feeling. Sweat is collected on a pad or paper and then analyzed. The sweat test usually is done twice. High salt levels confirm a diagnosis of cystic fibrosis. Drug used to enhance sweating is Pilocarpine is a cholinergic parasympathomimetic agent. It increase secretion by the exocrine glands

Sweat test using drug pilocarpine:

Sweat test using drug pilocarpine

3) Prenatal Screening :

3) Prenatal Screening In pregnancy , prenatal genetic tests can find out whether your fetus has cystic fibrosis. These tests include amniocentesis. The fluid is tested to screen whether both of the baby's CFTR genes are normal.

Other test :

Other test A chest x ray . A sinus x ray. This test may show signs of sinusitis, a complication of cystic fibrosis. Lung function tests. (spirometery) A sputum culture to check the presence of bacteria mucoid Pseudomonas

Management of CF:

Management of CF The goals of cystic fibrosis treatment are to: Prevent and control lung infections Loosen and remove thick, sticky mucus from the lungs Prevent or treat blockages in the intestines Provide enough nutrition Prevent dehydration

Medical Treatment:

Medical Treatment Treatment includes a diet high in calories, protein, and fat, vigorous physical therapy, antibiotics. Adequate hydration Bronchodilator Postural drainage Chest vibration Administration of oxygen


Cont………….d Anti inflammatory agents Corticosteroids Moderate to severe hemoptysis can occur due to erosion of pulmonary blood vessels so blood replacement should be required. Replacement of fat soluble vitamins particularly vitamin A,D,E,K is required. Chest Physical Therapy High-calorie shakes should be given for additional nutrients.

Nursing management :

Nursing management specialist knowledge of: –nutritional requirements – enteral feeding – intravenous therapy – respiratory complications and support – nebuliser therapy – care of indwelling venous devices – infection control – osteoporosis – liver disease – terminal care and symptom management

Alternative name of cystic fibrosis :

Alternative name of cystic fibrosis CF Cystic fibrosis of the pancreas Fibrocystic disease of the pancreas (MU-ko-vis-i-DO-sis) Mucoviscidosis of the pancreas Pancreas fibrocystic disease Pancreatic cystic fibrosis

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