Definition of Cystic Fibrosis
Cystic fibrosis is a monogenic disorder that is found as a multisystem disease. The disease is characterized by chronic bacterial infection of the airways, which in turn will lead to bronciectasis and bronchiolectasis, exocrine pancreatic insufficiency, and intestinal dysfunction, abnormal function of the sweat glands, and urogenital dysfunction.
Cystic fibrosis is a chronic multisystem disorder characterized by recurrent endobronchial infection, progressive obstructive lung disease and pancreatic insufficiency with impaired absorption / intestinal malabsorption. This disorder is a recessive genetic disorder that is heterogeneous with an overview of pathobiology that reflects mutations in the gene cystic fibrosis transmembrane conductance regulator / CFTR.
Clinical Manifestations of Cystic Fibrosis
Common manifestations of cystic fibrosis in the first year or two of life in the most frequent respiratory tract cough and / or pulmonary infiltrates. Most of the symptoms of cystic fibrosis is caused by the mucus. The most common symptoms are:
- Persistent cough with sputum and getting worse.
- Coughing from the effects of bronchitis and pneumonia which can cause inflammation and permanent lung damage.
- Peningktan sputum volume.
- The decrease in pulmonary function.
- Nasal obstruction.
- Dyspnea.
- Worsening of nasal discharge.
- Fever.
- Dehydration.
- Diarrhea.
- Big appetite but no weight gain and growth (downward trend). It results from chronic malnutrition due to not getting enough nutrients from food.
- Pain and discomfort due to too much gas in the intestines. This could be caused by intestinal dysfunction.
Complications of Cystic Fibrosis
Complications that can occur in cystic fibrosis are:
- Sinusitis. Caused by excessive production of mucus that covers and infect the sinuses.
- Bronchiectasis. Bronchi will be stretched and form pockets when the accumulated mucus. This mucus is where the development of bacteria that are potentially causing lung infections. This infection will be more damaging and if untreated bronchial bronchiectasis can progress to severe illness including respiratory failure.
- Pancreatitis.
- Nasal polyps.
- Clubbing. It occurs in the absence of oxygen transfer from the lungs to the bloodstream.
- Lung collapse.
- Rectal prolapse. Persistent cough or suppression may cause rectal tissue arising out.
- Liver disease.
- Diabetes.
- Pneumothorax occurs frequently (more than 10% of patients)
- The worst complication of cystic fibrosis is respiratory failure and cor pulmonale.
Nursing Care Plan for Cystic Fibrosis
Assessment
Anamnesis
Data collected during the assessment is used as a basis for nursing care plans. Nursing assessment process must be done very individualized (corresponding problems and current needs). In reviewing the status of breathing, the nurse did an interview and a physical examination to maximize the collected data without having to increase respiratory distress. After the initial assessment the nurse choose the appropriate component inspection with respiratory distress levels experienced by the client.
The data were collected or studied include:
1. Patient Identity.
At this stage the nurse needs to know about: name, age, gender, home address, religion or belief, ethnicity, languages spoken, education and employment status of patients.
2. Primary complaint.
Patients with cystic fibrosis found complaints of chronic respiratory tract infections such as chronic cough with phlegm is often repeated, can be accompanied by coughing up blood (hemoptysis), shortness of breath, decreased appetite, fever, glandular exocrine pancreatic insufficiency and abnomalitas sweat glands.
3. History of present illness.
History of present illness in cystic fibrosis patients showed a genetic mutation that forms the CF transmembrane conductance regulator protein (CFTR) located on chromosome 7.
4. Past medical history.
Both parents are carriers of the recessive gene CFTR or one of the parents is suffering from cystic fibrosis. Additionally it also needs to be asked whether the patient had been suffering from lung diseases such as tuberculosis, pneumonia, heart failure, Tauma and so on. It is necessary to determine possible predisposing factors.
5. History of family illness.
It should be asked whether there are family members who suffer from diseases that are pointed out as the cause of cystic fibfosis.
6. Psychosocial history.
Includes feelings of the patient against the disease, how to cope and how the patient's behavior, the actions taken against him.
Physical Examination
B1 (Breath)
Includes shortness of breath, pulmonary oxygen deprivation that damaged tissue and bluish colored skin (cyanosis) and a cough that is increasingly worse.
B2 (Blood)
Allow for hyperglycemia due to the pancreas can not produce insulin properly due to excessive mucus to damage the pancreas.
B3 (Brain): -
B4 (Bladder): -
B5 (Bowel)
In bowel disorder include; diarrhea, dehydration, abdominal pain and discomfort due to too much gas in the intestines as a result of dysfunction of digestive enzymes. In addition, abnormalities can be found besr appetite but no weight gain and growth (downward trend).
B6 (Bone): -
Nursing Diagnosis for Cystic Fibrosis
1. Ineffective Airway Clearance
related to the viscous mucus secretions and a lot of effort and a bad cough.
2. Impaired gas exchange
related to airway obstruction.
3. Ineffective breathing pattern
related to tracheobronchial obstruction.
4. Risk for infection
related to damage to the body's defenses, the presence of mucus as a medium for growth of the organism.
5. Imbalanced Nutrition Less than Body Requirements
related to the inability to digest nutrients.
