Cystic fibrosis is a chronic condition in which a mutation of the cystic fibrosis trans-membrane regulator (CFTR) protein causes the body to produce abnormally thick, sticky mucus. When this mucus becomes lodged in the respiratory passages, infection is likely to result. Blockage and subsequent infection can cause the airways to become weak and stretched out, scarred, and otherwise deformed. This condition is called bronchiectasis.
Nearly 50% of all reported instances of bronchiectasis are associated with the inflammation and infections associated with cystic fibrosis. Sometimes, the diagnosis of bronchiectasis is made prior to a formal diagnosis of cystic fibrosis. Bronchiectasis can also develop in patients who have allergic bronchopulmonary aspergilliosis (ABPA).
The severity of bronchiectasis is related to which of three types of the condition are present.
1) Fusiform (Cylindrical)
3) (Saccular) Cystic
Cylindrical forms are often caused by acute bronchitis or other lung infection. In this form, the respiratory passages are only slightly widened, similar to how a garden hose would expand when filled with water. This type of bronchiectasis can be reversed, as there are no collapsed sections of the bronchial wall. Varicose bronchiectasis, is a more serious form, characterized by extended and collapsed portions of the bronchial wall. The most serious and irreversible form is cystic bronchiectasis, in which the bronchi are greatly ballooned.
Bronchiectasis is usually identified during a routine chest x-ray as part of a cystic fibrosis patient’s annual lab tests. Further imaging such as a CT scan as well as pulmonary function tests and a sputum cultures will help the doctor determine the underlying cause, extent and severity of lung damage.
Bronchodilators such as albuterol, are prescribed to help open the airways. Fluticason and ipatropium are useful in controlling inflammation. Percussive respiratory therapy and postural drainage designed to dislodge the mucus is also used. Patients with cystic fibrosis will often use a combination of respiratory therapy including nebulized antibiotics to help control infection, bronchodilators, mucolytics and a vibrating vest such as the InCourage or ThAIRapy vest. Each of these therapies is important not only to control infection, but to slow the rate of declining lung function. Bronchiectasis is less likely to develop if infections are treated early.
In 2005 the FDA approved an inhalation powder called Bronchitol . Phase II clinical studies demonstrated adequate safety and effectiveness in patients at risk for developing bronchiectasis. The medication works by stimulating mucus hydration, enabling the usually thick, sticky mucus to be cleared more easily. Nebulized hypertonic saline produces a similar effect.
My 8 year old son has this year had two bouts of bronchialpneumonia 1st May this year and then again in July when he ended up in hospital for a week. His pedeatrician sent us to Ancona (we live in Italy) to a specialist cystic fibrosis center (Salesi ospitale). They have done several tests also the sweat test for cystic fibrosis where he resulted negativ. However, he constantly has an abnormal amount of mucus in his left lung and we do physiotherapy every day to drain his lungs. He has had a ct scan and has been diagnosed with a dilation of his bronchial tubes in his left lung. At the moment I think the diagnosis is of non-cystic fibrosis bronchiectasis but the doctor now wants to do some genetic testing to see if my son has a mild form of cystic fibrosis. Most of the time he is a very healthy little boy who likes sports, however my worry is that whereas a "normal" child would get a mild cold he immediately has something more serious. Also now he is on antibiotics because he had a mild sniffle which two days later resulted in a mucosy green cough with a light temperature of 37.7 and the doctor immediately put him on antibiotics due to the worry that it might again turn into bronchopneumonia. Please would you advise on a course of action to avoid him getting ill all the time or to nip any illness in the bud. What alternative medicines are most effective and reccommended. Basically what can I do to as much as possible stop him from getting ill constantly, taking antibiotics and making him well, which would be the best sport for him to strengthen his lungs (at the moment he does tennis and swimming) are there any food supplements he can take to help him, which would be the best alternative medicine to look into.