Dec 11 2008
Cystic Fibrosis: An insight
Cystic fibrosis is seen in children and is due to a change in a gene (mutation).
Common Manifestations:
1) Recurrent infections of the lung.
2) Other lung disease.
3) Gut problems like malabsorption (whatever food you consume, the nutrients are not absorbed from your intestine).
4) Fatty stools.
5) Child may have excessive crying (due to stomach pain), vomiting.
6) With increasing age, the person may have infertility.
Investigations:
The only investigation is demonstration of high sweat chloride.
Treatment:
1) Respiratory Management
2) Nutritional Care
3) Anticipation and early diagnosis of liver disease and diabetes.
Respiratory Management:
1) Clear the airways - a physiotherapist may be required.
2) Use drugs that break the mucus in the airways.
3) Antibiotics for infection.
4) Use drugs used for asthma.
Nutritional Management:
1) Good diet - eat a lot of dairy products and fried food.
2) Supplementation of Vitamin A,D,K.
3) Enzyme supplementation.
Early Diagnosis:
Cystic fibrosis can be diagnosed prior to birth by genetic testing.
