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An inherited chronic disease affecting lungs and digestive system is known as Cystic Fibrosis. Around 70,000 patients are afflicted with this disease worldwide. It is caused by a defective gene and protein of that gene produces sticky, thick mucus that clogs the lungs causing life threatening infections of lungs.
The protein also hinders the functioning of pancreas and interferes in proper metabolism and assimilation of food, by blocking the associated enzymes. Symptoms of cystic fibrosis include frequent and persistent coughing, breathlessness, poor growth (even with good appetite), salty skin and difficult bowel movements with bulky and greasy stools.
Recent research advancements and better medical treatments have extended life of children and adults with CF. Now these patients could live much longer, till their 40s and beyond.
Chest physical therapy involves tapping the chest as well as back with hands to dislodge the loosen mucus from lungs, which can be easily removed on coughing. Few devices to assist CPT are developed like electric chest clapper, an inflatable therapy vest, vibrating mask and handheld device to exhale through and remove mucus. Perform aerobic exercises or breathing techniques that include forceful short breaths with intermittent relaxed breathing. These help to open the airways and loosen mucus from the lungs that gets easily dislodged off. Exercise improves overall health by building up stamina and immunity of the patient.
Bronchodilators on inhalation, relax the muscles thereby opening airways passage and clearing off the mucus. These are usually taken before CPT to assist the process. Anti-inflammatory medicines are used to lower swelling within airways formed due to lung infections and antibiotics drugs are used to treat such infections. Mucus thinning drugs are also administered to remove mucus by decreasing its sticky nature and improving lung functioning.
In advanced lung disease cases, oxygen therapy is given through mask or nasal prongs. This helps to deliver oxygen rich blood to heart thereby decreasing fatigue and shortness of breath.
With use of portable oxygen system, one can easily move around and perform daily activities simultaneously. In case of severe lung disease, when other treatments have failed, lung transplant could be the option, wherein the diseased lung is removed and a healthy lung is placed from a deceased donor.
To counter digestive problems due to CF, nutritional therapy need to be included that improves the ability and strength to remain active. It helps in better child growth and development and one become resistant to newer lung infections. Nutritionist will help to create a well balanced nutritional plan that is rich in fat, calories and protein.
In addition, it includes oral intake of pancreatic enzymes that support fats and protein digestion and assist in absorption of vitamins. Fat insoluble vitamins A, D, E and K supplements are included to ensure vitamin supply, since fat soluble vitamins are not absorbed by the intestine in CF patients.
High calorie shakes and high salt diet are included in the therapy to maintain body energy, more so before exercising. At night also, the nutritional demands are being fulfilled with help of a feeding tube that is placed either through surgically hole into the stomach or through nose and throat into the stomach.
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