Cystic fibrosis
Cystic fibrosis is an inherited illness that leads to severe damage to the lungs, sinuses intestinal tract, pancreas and other body parts. These health problems commonly take place during early childhood, however, these can also happen in childhood or adulthood.
SYMPTOMS OF CYSTIC FIBROSIS
Symptoms of Cystic Fibrosis most often point to multisystem involvement. Symptoms also vary according to the age of onset of disease. Single system involvement is more common in late childhood or early adulthood. The most common symptoms include:
- Intestinal blockage, mainly in new-borns (meconium ileus)
- Severe constipation and repeated intestinal blockage
- Foul-smelling and greasy stools
- Repeated episodes of abdominal pain due to Pancreatitis
- Poor weight gain and growth
- Rectal Prolapse
- A constant cough that produces thick and copious sputum
- Repeated chest and sinus infections with multiple organisms
- Breathlessness, Wheezing and increasing exercise intolerance
- Infertility in males and reduced fertility in females
- Diabetes Mellitus
- Multiple Vitamin Deficiency
- Multiple fractures and skeletal deformities
TREATMENT OPTIONS FOR CYSTIC FIBROSIS
CFTR modulators are a class of drugs that have a role in reducing rapidity of progression of the disease to a great extent if introduced early in life. It is possible for specific treatments to help in controlling the symptoms, preventing or reducing complications, and making the illness easier to live with. An individual with cystic fibrosis is usually supported by a team of healthcare professionals at a specialist cystic fibrosis centre. A care plan is drawn up that is tailored according to their personal needs. A variety of treatments are used, and often treated in a hospital is required. Regular appointments to monitor the illness is also recommended.
- Medications for Lung Problems - An individual with CF needs a combination of medications for sinopulmonary infections, clearance and thinning of secretions and improvement in wheezing.
- Airway Clearance Techniques - In addition to medication, specific methods are extremely important to keep the sinuses, airways and lungs clear. Physiotherapist plays a very important role in guiding and assisting a patient in airways clearing techniques and also in improving effort tolerance.
- Dietary and Nutritional Advice - For patients with cystic fibrosis, getting a healthy diet and right nutrition with vitamin supplements keeping malabsorption in mind is crucial in helping them develop normally and preventing them from becoming repeatedly ill. Also, prevention of constipation is very important. All these need a trained nutritionist counselling.
- Infertility - Counselling for male infertility and reduced fertility in females helps couples to have a successful pregnancy.
Lung Transplants - In advanced lung disease with Cystic Fibrosis Lung Transplant is an important option with good results.