Biology

Mucus is constantly produced by some cells in the body, which helps in keeping the linings of specific organs moist thus preventing them from getting infected and drying out (MedlinePlus). In disease condition, the mucus is usually thick and gum-like. The mucus will build up in the lungs and block the critical airways, which make available the essential oxygen and expel carbon dioxide. The airways that are blocked are the tubes that are largely responsible for gaseous exchange. The mucus buildup makes the lungs to be vulnerable to bacterial attack and encourage bacterial growth. A repeated serious infection ensues which severely destroys the lungs leading to a condition known as cystic fibrosis or CF (MedlinePlus). Description of Cystic Fibrosis (CF)

Cystic fibrosis is a genetic disease that affects almost the whole body causing serious gradual disability that often leads to death (National Heart Lung and Blood Institute). It is normally known to be a disorder of the sweat and mucus gland which normally attacks the lungs, liver and pancreas (National Heart Lung and Blood Institute). The sex organs, sinuses and intestines are other organs which are sometimes affected by CF.   In the pancreas, the thick and gum-like mucus blocks ducts or tubes blocking the digestive enzymes made in the pancreas from reaching the small intestines (MedlinePlus). The enzymes that are produced in the pancreas are important in the process of breaking down food particles into forms that can be assimilated. Lack of the enzymes implies that the absorption of fats and proteins cannot fully be achieved in the intestines (MedlinePlus).

A severe vitamin deficiency results accompanied by serious malnutrition since the nutrients often leave the body without being assimilated. CF patients often experience bulky stools, swollen bellies which results from severe constipation, intestinal gas and great discomfort or pain (National Institute of Health). All what CF patients experience are as a result of the inability of the ducts or tubes to pass the enzymes that are essential in the breakdown of food substances into simpler molecules that the body can utilize for life processes such as energy metabolism (National Institute of Health).

CF can also cause the afflicted individuals to produce sweat, which is salty. This leads to the patients to lose large amounts of salts following sweating which usually upsets the mineral balance in blood (National Heart Lung and Blood Institute). This imbalance can lead to an array of health complications such as increased heart rate, dehydration, tiredness, reduced blood pressure, weakness, heat stroke and in rare situations, death. CF patients have a higher risk for diabetes and some disorders such as osteoporosis (National Heart Lung and Blood Institute). CF can cause infertility in male patients and can make it difficult for women to conceive.Causes of CF CF is caused by a defective gene known as cystic fibrosis transmembrane conductance regulator (CFTR) (National institutes of Health). When there is a mutation in the gene, the correct type of protein will not be transcribed and translated. CFTR gene codes for a special type of protein which regulates mucus, digestive juices and sweat components (National Institutes of Health). In normal humans, there are two copies of fully functional CFTR gene although a single copy is sufficient in preventing CF from occurring. This implies that CF will only occur when neither of the genes is working in a normal way (National Institutes of Health). This makes CF to be considered a special type of autosomal recessive condition.Symptoms of CF The signs of CF vary from one person to another. In some patients CF can result to serious digestive and lung complications (Murphy, and Schubert 13). Other patients will have more mild forms of CF which will not show any symptoms until they become fully adults or in their adolescent stage. The symptoms may also vary over time. Patients may show varying symptoms with some showing fewer symptoms which become severe as the disease advances. Lung function will decline during early childhood among people suffering from CF (Murphy, and Schubert 13). As the disease advances there is a permanent lung damage which often causes serious breathing difficulties (Murphy, and Schubert 14). Breathing problems have been identified to be the main causes of CF deaths. The symptoms will also seem to improve with various medical interventions. The patients who are provided with proper medical care often have improved life expectancy of 40-50 years or even more. With early treatment, the lifespan and the quality of life can greatly be improved (Murphy, and Schubert 14). Specifically, CF causes problems in respiratory, reproductive and digestive systems and these can be presented as signs at advanced stage of disease development.

In the respiratory system, CF individuals often present thick and gum-like mucus which build up in the airways (Murphy, and Schubert 14). It is the buildup which makes it easier for the bacteria to cause infections and grow. Bacterial infections can block the air passages and result to constant coughing that bring about very thick sputum or sputum with blood stains. Respiratory infections among the CF individuals dont respond to conventional antibiotics. Bouts of infections of the spaces behind the forehead, nose and eyes and bronchitis and pneumonia are some of the problems faced by CF individuals (National Heart Lung and Blood Institute). CF also affects the digestive system. Patients will always present thick mucus, which develops in pancreatic ducts (MedlinePlus). The mucus prevents various enzymes from reaching the small intestines. Because of blocked ducts, patients may present foul-smelling, bulky and greasy stool or serious diarrhea. Children can show signs of losing weight and having a slowed growth. Other complications typical among CF patients in worse situations include gallstones, diabetes, liver problems due to blocked or inflamed bile ducts, rectal prolapsed and pancreatitis (MedlinePlus).

There are other symptoms which usually occur in the reproductive systems. The men born with CF are always infertile since in CF, the vas deferens does not form during fetal developmental stages (National Institutes of Health). Lack of vas deferens tube implies that the sperms will not be delivered to the penis from the testicles. Women also may experience difficult moments getting pregnant since the mucus always blocks her cervix or causes other CF-related complications (National Institutes of Health). Treatment of CF Treatment options for CF have always taken the dimension of preventive medicine than the intervention to use medicines to cure the CF (Roberts 41). these efforts are primarily meant to prevent lung infection, intestinal blockages and dehydration. Treatment goals are also directed to loosening and removing thick and gum-like mucus and provide sufficient nutrition. Chest therapy may involve using a mechanical precursor also called an electric chest clapper (Murphy, and Schubert 14). Exercise is also necessary for CF patients to loosen the mucus. Medicines usually used include antibiotics, bronchodilators, anti-inflammatory agents and mucus-thinning agents. The goal of using medicines is to prevent and treat infections on the lungs, open the airways, thin the mucus and reduce swellings. Antibiotics are important in preventing and treating lung infections (Roberts 45). They can be administered as oral, intravenous or can be inhaled. In advanced cases of CF, oral pancreatic enzymes can be administered and the patient given vitamins A, D, E and K which are fat soluble vitamins that their intestines cannot absorb (Roberts 48).Conclusion

In general, CF is disorder that affects largely the quality of life among those afflicted and the family members, workmates, friends and the relatives of the patients. The disease can cause great reduction in labor output and hence it is considered to be of great economic importance. With the advanced recombinant DNA technology and various molecular biology tools, gene therapy is a possibility in the near future and this will mark the triumphant over CF (MedlinePlus).