The symptoms associated with Cystic Fibrosis varies between individuals with the disease. For some people, the symptoms appear soon after they were born, while in other cases the symptoms may not appear for months or even years. Cystic Fibrosis Symptoms in babies and infants include pale stools, persistent diarrhea, smelling, greasy stools large and foul, gassiness, and abdominal swelling. Other symptoms include chronic cough, frequent wheezing, pneumonia, or chronic cough with thick mucus. Infants and babies with Cystic Fibrosis may also be vomiting, dehydration, salty-tasting skin, and intestinal blockage.
Children with Cystic Fibrosis experiencing symptoms including difficulty breathing, frequent respiratory infections, cough, rapid breathing, and a barrel-chested appearance. They also may have a fever, nasal flaring, and poor growth. Additional symptoms include poor appetite, malnutrition, gassiness, and abdominal discomfort and pain.
There are three stages of Cystic Fibrosis which can be determined, prenatal, postnatal, and in early childhood. The amniotic fluid that surrounds the fetus in pregnant women is something that can be tested for fetal intestinal enzymes using a procedure called, 'Amniocentesis.' During the procedure, a sample of amniotic fluid is extracted and analyzed for levels of intestinal enzymes, if the fetus has Cystic Fibrosis, the rate will decrease.
Mechanical treatment of airway clearance found three devices are available for therapy. One device, 'Positive Expiratory Pressure Treatment,' which involves positive expiratory pressure treatment using the mouthpiece or mask, which is attached to the one-way valve that has a resistance set of five to twenty centimeters of water. Other devices known as, 'Flutter,' which is a handheld device resembling a pipe. There is a steel ball in a bowl of the pipe, generating oscillations during expiratory breathing resistance variables. A third mechanical treatment involves an inflatable vest and high-frequency chest compression.
Nursing Care Plan for Cystic Fibrosis
Nursing Diagnosis : Ineffective airway clearance related to thick mucus secretions and coughing bad effort.
Goal: Client not aspiration
Outcomes: Shows an effective cough and increased air exchange in the lungs.
Nursing Interventions :
1. Auscultation of breath. Note the presence of breath sounds, for example: wheezing, crackles.
Rationale: Some degree of bronchospasm with obstruction and airway obstruction may / not indicated the presence of abnormal breath sounds or crackles eg absence of breath sounds.
2. Perform physiotherapy to issue secret and give the patient a comfortable position, eg, elevation of the head of the bed, sitting on the back of the bed (position semi-Fowler / Fowler).
Rationale: Elevation headboard facilitate respiratory function by using gravity.
3. Assist clients to dilute sputum, with the collaboration expectorant administration to improve airway clearance.
Rationale: Giving expectorants may help thin the secret so secret is more easily removed.
4. Provide nebulizer with a solution and in accordance with the right tools.
Rational: Nebulization can help spending viscous secretions.
5. Observations clients closely after aerosol therapy and chest physiotherapy to prevent aspiration due to many sputum suddenly become watery.
Rsaional: To prevent aspiration.
6. Provide postural drainage (adjust the area where there is a buildup of mucus) as prescribed to reduce the viscosity of mucus.
Rationale: Postural drainage helps remove thick mucus.
