AIRWAY CLEARANCE TECHNIQUES

Cystic fibrosis is a congenital disorder of cystic fibrosis transmembrane conductance regulator gene, which creates imbalance in flow of chloride ions across the cell surfaces of respiratory, digestive and reproductive system. In response to high accumulation of salt in the affected cells, the body secretes excess of mucus. A constant flow of mucus on the surface of air passage is observed in normal individual, which is lacking in patients with cystic fibrosis due to extremely thick and sticky mucus. The thick mucus also facilitates rapid bacterial growth and causes frequent bacterial chest infection in CF patients.

Retention of sputum and mucus along with difficulty in breathing is the main characteristic seen in patients with cystic fibrosis. Various forms of airway clearance mechanisms/techniques are employed with patients of cystic fibrosis to overcome the major problem of sputum retention. The main aim of all forms of this technique is to reduce the progression of cystic fibrosis by stimulating the normal mechanism of muco-ciliary clearance in the patient?s body.

CARRIER SCREENING TEST FOR CYSTIC FIBROSIS

Cystic fibrosis is a genetic disorder in people with defective cystic fibrosis transmembrane conductance regulator gene, which in normal individuals helps in controlling the chloride channel in cells of respiratory, digestive and reproductive system. The disorder has significant prevalence in many parts of the world and in united status about 30000 children and adults are affected with this disorder. The disorder induces severe breathing and digestion complication in patients, due to blockage of the pathway or passage due to extreme secretion of mucus. This thick and sticky mucus in turn promotes growth of bacteria and causes life-threatening infection in the patients. The excess of secretions in pancreas also lead to complication with absorption of food and causes complications. Average lifespan of patients diagnosed with cystic fibrosis is till 30-37 years, but in many cases with modern medications and treatment interventions, life expectancy till 40 is also being achieved.

COMPLICATIONS ARISING OF CYSTIC FIBROSIS

Cystic fibrosis is a disorder involving the tissues of respiratory, reproductive and digestive systems, which due to defective CFTR gene, produce impairment in channelizing chloride ion across the affected cells. This in turns causes the body to produce and secrete abnormally excessive mucus , which due to altered chemical composition is thicker and sticky in nature.

? Many complications arise in patients with cystic fibrosis owing to deficient functioning of respiratory, digestive and reproductive system.

? There is less and inadequate absorption of the fat soluble vitamins i.e. vitamin A, D, E and K by the digestive tract of CF patients which ultimately causes rickets, night blindness, bleeding disorders and anemia.

? Rectal prolapse is also seen in about twenty percent of children having cystic fibrosis, which is manifested when rectum lining protrudes out of the anus.

? Due to less capacity of the digestive tract to absorb enough protein, the infants on breast milk or soy protein may develop anemia and edema of the extremities.

CYSTIC FIBROSIS- AT A GLANCE

Cystic fibrosis is an inherited autosomal disease that affects tissues secreting mucus. These tissues line the respiratory, digestive and reproductive tract of patient along with sweat glands. This results in excessive secretion of thick and sticky mucus in the passage of these organs and thereby producing obstruction or blockage causing further complication.

The thick and sticky mucus in the respiratory system causes obstruction in the airway passage due to thick mucus and also facilitate or encourage frequent bacterial proliferation and infections in the lung tissues. Similarly, the abnormally thick mucus secreted in the digestive tract can impairs normal function of primarily pancreas and liver. The altered or deficient functioning of affected digestive organs due to cystic fibrosis causes difficulty in effective digestion of food and absorbs nutrients. The symptoms of cystic fibrosis is varied in patients and may at times is not expressed until the affected child reaches the adolescence.

CYSTIC FIBROSIS AND THE DIGESTIVE SYSTEM

Cystic fibrosis a fatal genetic disorder which targets and affect the working of respiratory, reproductive and digestive system of our body along with sweat glands. It is also in general known as CF or muco-viscidosis, which is characterized by secretion of extremely thick mucus that blocks the respiratory and digestive passage in our body. This disorder affects the exocrine glands of liver, lungs, intestine and pancreas, which causes the secretion of thick mucus in these regions of the body.

Cystic fibrosis can also be transmitted and expressed in child, when both the parents have recessive genes responsible for causing this disease, but do not have this disease. The couples having recessive genes have 25% of possibility of having this disease in their children and 50% chance that the children will possess the genes. The degree of severity of the disorder is variable with few children showing the acute symptoms and with people showing milder symptoms till their mid adulthood.

CYSTIC FIBROSIS AND INFERTILITY

Cystic fibrosis (CF) is an autosomal genetic disorder affecting the epithelial cells lining respiratory, digestive and reproductive system, which in-turn lead to excessive production of thick and sticky mucus in the body. This mucus creates obstruction in the airway, digestive and reproductive passages leading to further complications in the patients with cystic fibrosis.

Men patients with cystic fibrosis can definitely enjoy normal sex life but almost all males with this disorder are in fertile. Earlier, many of the patients with Cystic fibrosis moved into their adolescence stage, without even knowing and counseled about their almost certain infertility. This has been overcome now by various campaigns and foundations formed to create awareness on various aspects of cystic fibrosis among the patients. In these patients sperms are produced as in normal individuals, the infertility is mainly due to obstruction in Vas deferens, which is used to carry the sperms from testes. In such cases, however it is possible to aspirate the sperms of the patients from epididymis and can be used for in vitro fertilization. There is 20-30% of success rate reported through such technique to induce pregnancy with infertile CF patients.

CYSTIC FIBROSIS AND THE DIGESTIVE SYSTEM

The patient with cystic fibrosis shows abnormity in function of cystic fibrosis transmembrane conductance regulator gene in their body, control the flow of chloride ions and water across few body cells. The mutation in the gene causes the cell protein to behave abnormally thus altering the chloride flow in the body and thus excess of mucus, sweat and water is secreted as body reflex in patients. The patients with cystic fibrosis may have complications in their respiratory, digestive and reproductive system. Thick and sticky mucous is secreted by the cell of the affected system, which in turn clogs the passage and causes obstruction in respiration, digestion and reproduction.

The pancreas is mostly affected under the digestive system complication in patients with cystic fibrosis. Pancreas is an exocrine gland which secretes certain digestive enzymes which aids in food absorption and digestion. The deficiency of such enzymatic secretion by pancreas of patients with cystic fibrosis in turn lead to improper fat absorption in the body due to incomplete breakdown of fat content in the food. The pancreatic duct also gets obstructed due to increased thick secretions from pancreases, which causes difficulty in absorption of fats, certain proteins and vitamins like A, B, D, E and K. The pancreatic insufficiency in cystic fibrosis leads to passing large quantity, mushy and foul smelling stool. The stool is generally greasy and pale in color, which due to excess of fat in it, floats on the water of the toilet.

CYSTIC FIBROSIS: SYMPTOMS

The degree of severity of symptoms in patients with cystic fibrosis is variable and doesn?t show any specific pattern of occurrence or expression. The basic problem in all patients with cystic fibrosis is defective cystic fibrosis transmembrane conductance regulator gene, which causes abnormal function of exocrine glands that secretes mucus or sweat. The excessive secretion of sweat in patients causes imbalance and excessive loss of mineral in the body, which produced abnormal heart functioning and can lead to going in into shock. Similarly, excessive secretion of thick and sticky mucus intestine and pancreas, lead to occurrence of digestive problems, stunted growth and greasy stools. The clogging of respiratory tract due to excessive secretion of mucus in patient with cystic fibrosis can clog the lungs, cause frequent respiratory infections, breathing problem and permanent lung damage.

CYSTIC FIBROSIS AND THE DIGESTIVE SYSTEM

Cystic fibrosis a fatal genetic disorder which targets and affect the working of respiratory, reproductive and digestive system of our body along with sweat glands. It is also in general known as CF or muco-viscidosis, which is characterized by secretion of extremely thick mucus that blocks the respiratory and digestive passage in our body. This disorder affects the exocrine glands of liver, lungs, intestine and pancreas, which causes the secretion of thick mucus in these regions of the body.

Cystic fibrosis can also be transmitted and expressed in child, when both the parents have recessive genes responsible for causing this disease, but do not have this disease. The couples having recessive genes have 25% of possibility of having this disease in their children and 50% chance that the children will possess the genes. The degree of severity of the disorder is variable with few children showing the acute symptoms and with people showing milder symptoms till their mid adulthood.

It is a fatal disorder and an average lifespan of patient with cystic fibrosis is 28 to 30 years of age. Now-a-days, with improved medications and mucus drainage procedural interventions, the average lifespan of children with this disorder is increased and they are able to live till mid-adulthood or beyond that. Earlier, children manifested with cystic fibrosis were not able to reach their adulthood. The ultimate cause of death in patients with cystic fibrosis is acute respiratory tract infection along with swelling/enlargement of the right side of heart.

DIET OF KIDS WITH CYSTIC FIBROSIS

Cystic fibrosis is an autosomal disease affecting the epithelial cells lining respiratory, digestive and reproductive tract. The disease manifests itself by excessive secretion of thick and sticky mucus in to the respiratory and digestive passage, which causes obstruction and thereby impairs functioning of the system. This disease may also impairment in salt and water balance in the body. The extremely thick and sticky mucus in the airway passage of respiratory system makes it difficult for child with cystic fibrosis to breathe.

Due to obstruction of the digestive tract, the fat soluble vitamin and fats are not absorbed in the body , which causes stunted growth and weight loss in kids. The recurrent respiratory and digestive problems may make the kid weak and feel sick most of the time.
The CF kids can keep themselves healthier and fit by eating well and taking the prescribed medicine regularly. It is recommended that the parents of kids with cystic fibrosis consult a dietician to prepare the food plan for their child.