Autosomal disease that resessif, cystic fibrosis (CF) is the most common genetic disease threatening the lives of white children in the United States. CF affects the respiratory function and other bodily systems, menurnnya production of mucus from the exocrine glands, and thick mucus. In particular, the thick mucus clogging the membrane function and cell membrane that transports multiple organs in the lungs, pancreas, and liver, causing respiratory problems, chronic respiratory infections, nutritional impairment, and cirrhosis.
This disease in varying degrees, associated with many complications, including chronic respiratory infections, ileus due mekoneum (at birth), pancreatitis, gallstones, pneumothorax, prolapsed rectum, and the mother becomes barren. Treatment includes pulmonary therapy, pengoatan pancreatic enzymes and vitamins, and diets high in calories and high in protein. Although many children live to adulthood (the media life expectancy to 28 years), hope to live more in women than men.
• A cough that is productive
• Barrel chest
• Failure of growth
• foul-smelling stool, bulky, no stool or chronic diarrhea.
• Increased appetite
• Vaginal infections
Eyes, ears, nose, and throat
• nasal polyps
• The surface of the skin feeling salty
• The radius of the clubbing experience (against severe cases)
• Development of an obstructed
• Anger (potential)
• Depression (potential)
Impaired gas exchange associated with increased production of mucus.
Children will increase the movement of mucus secretion is characterized by a decrease in respiratory disorders and decrease cyanosis, and cough.
1. Perform chest physiotherapy as needed, every 4 hours
2. Give oxygen using a humidifier with lid; do not use a tent with humidity.
3. Assess the respiratory status of children every 4 hours
4. Instruct children to do deep breathing exercises every 4 hours
5. Give bronchodilators by mouth, nebulizer, or metered-dose inhaler with a spacer, as directed.
1. Chest physiotherapy to help mobilize secretions, maintain lung capacity, and improve oxygenation.
2. Moisturizers will issue and thin the secretions and increase the filling of oxygen to the tissues. Because stimulation of breathing in children often depend on the level of humility oxygen, high oxygen concentration can not be used. Warm, moist environment of the tent moisturizers can increase bacterial growth.
3. Assessment of breathing that often allows early detection of changes in the child’s condition.
4. Practice deep breathing to increase lung expansion.
5. Bronchodilators help thin mucus and improve lung expansion. Spacer devices allow the child to breathe in the full drug.
Risk of infection associated with mucous product.
Children will show no signs of infection characterized by fever and chills do not exist, and the reduction in respiratory distress
1. Give antibiotics as directed.
2. Assess vital signs to assess the increase in respiratory rate, dyspnea, and cyanosis.
3. Monitor the number of white blood cells.
1. Antibiotics can be recommended to help fight infections.
2. These changes indicated the infection gets worse
3. Increased white blood cell count indicated an infection.
Nutritional deficiencies: lack of demand is characterized by decreased absorption of nutrients.
Children will demonstrate improved nutritional status characterized by a minimal weight loss, good skin turgor, and increased intake (eat more than 80% of the food provided)
1. Weigh the child’s weight at the same time every day, and use the same scales scale.
2. Give pancreatic enzymes before each meal and snack provided, give supplements of vitamins A, D, E, and K, as directed.
3. Provide a diet high in calories, protein, and carbohydrates. If necessary, monitor carbohydrate intake in children.
4. Give a breathing treatment before eating.
1. Considering body weight every day to help assess the nutritional status of children.
2. Pancreatic enzymes allows food to digest and is absorbed in the gastrointestinal tract. High-carbohydrate diet can increase diarrhea.
3. Respiratory treatment given after a meal can cause coughing and vomiting, will increase the risk of aspiration.
Anxiety (children) are associated with respiratory problems and stay patient in hospital.
Children will decrease his anxiety is characterized by periods of rest and enough sleep respiratory status is stable.
1. Let the child in the position as comfortable as possible.
2. Delaying the procedure until all the tests and free airway.
3. Encourage parents to stay with the child and participate in nursing care.
1. Allow the child at a given position will increase the anxiety and difficulty breathing
2. Examinations and procedures can increase the anxiety level of children, increasing respiratory distress
3. The presence of parents and participation in nursing care to provide security and reduce anxiety.
Anxiety (parents) are associated with knowledge about the condition of the child.
Parents of children will decrease their anxiety is characterized by the ability to provide support to children and explain the child’s condition.
1. Assess understanding of parents about the child’s condition and treatment provided.
2. Give an explanation of medical conditions, procedures, and treatment received.
3. Provide behavioral support, such as speech and touch the child.
4. Provide emotional support to parents during the child’s stay in hospital.
5. Refer parents and children to such organizations Cystic Fibrosis Foundation; encourage them to work with the management center in order to gain experience in handling CF.
1. The assessment will provide the basis for teaching
2. The explanation given before and during their stay in hospital care will increase knowledge and eliminate various misconceptions, reduce anxiety.
3. Strengthening of encouragement to parents to repeat some behaviors.
4. Hearing the concerns of parents and feelings help parents deal with the crisis hospitalization.
5. Organizations that can provide support and information. This center has been experienced in dealing with CF will be able to provide high quality service and up to date.
Lack of knowledge related to home care.
The parents will express pemahanannya about home-care instructions and demonstrate procedures for home care.
1. Teach parents about giving antibiotics and kemugkinan reaction, including rash, gastrointestinal disturbances, and difficulty breathing.
2. Explain the reasons for granting long-term antibiotics.
3. Teach parents signs and symptoms of respiratory disorders, including dyspnea, Tachypnoea, cyanosis, wheezing, and tachycardia.
4. the need to encourage children to drink two to four 8-oz (240-ml) glasses of fluid each day (depending on the circumstances of children renal and cardiovascular).
5. Teach parents how to give the children before eating enzyme-does not coincide with the food-and how to provide a diet high in calories, protein, and carbohydrates.
1. Parents need to know how to give medication safely and consistently. Knowing what reaction that might occur and the parents as soon as possible memintak bantua doctor if necessary.
2. Long-term treatment to help limit the lung damage from recurrent infections.
3. Know the signs and symptoms of respiratory disorders would enable parents seek immediate medical assistance if necessary.
4. Adequate fluids will thin the mucus and restore fluid lost through the lungs, prevents dehydration which can cause an electrolyte imbalance.
5. Diet will help restore lost nutrients through no absorption of nutrients, enzymes given before meals will help digestion.
Cel List documentation
During his stay in hospital care, notes:
The status of children and mengkajian have been made when admitted to hospital.
Changes in health status of children
Associated with the results of laboratory tests and diagnostic tests
Fluid intake and output
Response of children to treatment
The reaction of children and parents to the disease and stay patient in hospital.
Guidelines for teaching patients and their families
Guidelines for follow-up plan.