In treating patients with CF How can we prevent infection from occurring inside their lungs?
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Cystic fibrosis is a serious genetic condition that happened due to a defect in cystic fibrosis transmembrane conductance regulator gene, or CFTR gene that causes severe damage to the respiratory and digestive systems. Movement of water and salt in and out of our body’s cells is controlled by this gene. This damage in the organs regularly results from a build-up of thick, sticky mucus. The most commonly affected organs include the lungs, pancreas, liver, intestines. This may lead to life-threatening complications which include infections, respiratory failure, and malnutrition. For improving quality of life and lengthening the expected lifespan early diagnosis and treatments are necessary.
The symptoms of cystic fibrosis can vary depending on the person’s age at which symptoms develop and the severity of the condition. And also these symptoms may appear at infancy for some people but for other children symptoms may not initiate till after puberty or even later in life. The symptoms associated with the disease may get better or worse with time. Strong salty taste to the skin is one of the very first signs of this disease. Respiratory symptoms are wheezing, a persistent cough that produces thick mucus or phlegm, shortness of breath, especially when exercising, recurrent lung infections, a stuffy nose, stuffy sinuses. The abnormal mucus can also plug up the channels that carry the enzymes produced by the pancreas to the small intestine. Without these digestive enzymes, the intestine can’t absorb the necessary nutrients from food. This can result in greasy, foul-smelling stools, constipation, nausea, a swollen abdomen, loss of appetite, poor weight gain in children, delayed growth in children.
Doctors diagnosed this disease by a genetic test or blood test to check for signs of the disease. This genetic test identifies whether the baby has a defective CFTR gene or not. The blood test determines whether a baby’s pancreas and liver are working properly. There are other diagnostic tests that may be done include Immunoreactive Trypsinogen (IRT) Test, Sweat Chloride Test, Sputum Test, chest X-ray, CT-scan, Pulmonary function tests (PFTs).
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