(Living with Cystic Fibrosis)
Cystic Fibrosis is an Autosomal recessive disorder affecting the exocrine glands and also causing blockage in bronchi, intestines and pancreatic ducts by the abnormal production of thick mucus.
The patient is recommended to have regular follow-ups with the doctor because of the complexity and multisystemic involvement of the disease.
Management at the Diagnosis Stage:
At the time of diagnosis (initial stage), the patients are recommended to go through a baseline assessment before the initiation of therapy. Parents/patients are also counseled and educated about the use of pieces of equipment for airway clearance (spacers, nebulizers).
Management at the Treatment Stage:
Although there is no cure for CF with the recent advances now, the patients are able to live a fuller lifeless hampered by their condition. The first and foremost thing to be done in the management of CF is a nutritious diet and an active lifestyle.
Managing the symptoms related to the disease
A continuous pulmonary rehabilitation throughout a patient’s life actually maximizes the organ functions and also the quality of life. The rehabilitation program includes:
- Breathing techniques to loosen the mucus
- Nutritional counseling
- Education about the disease
There are some other disease management techniques like oral antibiotic which aid in chronic and acute infections. Also, some mechanical devices and inhalation medications have proven to be effective as they clear the thickened mucus, though they are time-consuming.
Patients can acquire other lung infections like pneumonia which can be prevented by antibiotics or pneumococcal vaccines. Anti-inflammatory drugs aid in reducing the swelling in the airway tracks.
Medications and other medical procedures
Medications for diminishing the mucus are likewise accessible to ease in the coughing. Patients can likewise utilize bronchodilators to open up the aviation routes and loosen up the muscles around the bronchial tubes.
Patients are likewise encouraged to take oral pancreatic catalysts to help supplement ingestion process in the stomach related tract.
For the patients who have quality transformations, specialists prompt more current drug accessible which are helpful in lessening the measure of salt in perspiration, lung contaminations and enhancing weight issues.
Notwithstanding the medications, Chest treatment likewise helps in diminishing the mucus and makes it simple for the lungs to hack up mucus.
The therapy is to be performed one to four times each day. Clapping with measured turns in the front and back of the chest is a typical method utilized in the Chest treatment.
Some Surgical procedures are also suggested which include:
- Nasal polyp removal (if any) as they obstruct the airway
- Oxygen therapy to prevent pulmonary hypertension
- Endoscopy and lavage as the mucus can be suctioned from the obstructed airways through an endoscope.
- Feeding tubes to aid in proper nutrient absorption even while the patient is asleep, Bowel surgery to remove, if there is any, blockage in the bowel.
- Lung Transplant is suggested in severe cases if the patient has any resistance to the antibiotics or develops life-threatening lung infections.
Other things to be taken care by the patients:
- Adequate water intake
- Extra fiber intake to prevent intestinal blockage
- Some antacids as advised by a doctor
- Special fat-soluble vitamins
- Extra salt intake especially in hot weather conditions and also after exercise.
- Good nutritious and a high-calorie diet.
- Avoid smoking and also passive smoking as it is harmful to the lungs of the patients.
- Continuous monitoring of the patient through the liver function test (LFT).
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