Introduction Cystic fibrosis is an inherited disease that ultimately leads to death. It affects every racial group worldwide, but its prevalence varies from country to country. In those with cystic fibrosis, the lungs and digestive system are primarily affected by the disease. With the new developments in treatment and management, the 50 percent survival rate from the 1970’s has greatly improved, allowing patients to continue to live their lives longer than ever expected in the past. The new developments in prevention of exacerbations, therapy drugs and methods to preserve lung function have done great things to help patients extend their lives. Education is another important aspect of treating cystic fibrosis. For example, more the patient knows about the disease and the benefits of the treatment, the more likely he, or she, is to comply with their recommended therapy. Though the treatment can be very costly and time consuming, it gives cystic fibrosis patients the opportunity to go to school, have jobs and even have families. These are normal events that go with aging that many cystic fibrosis patients may never have been able to dream of experiencing. Prevalence Cystic fibrosis is a recessive genetic disease. It is seen mainly in the Caucasian population, with 1 in 3,000 Caucasians having cystic fibrosis, but does affect every racial group. According to the Cystic Fibrosis Foundation Patient Registry, as of 2008, there were 30,000 patients with cystic fibrosis in the United States. Keeping this in mind, there are approximately 1,000 patients newly diagnosed with cystic fibrosis each year. The Cystic Fibrosis Foundation also estimates that there are approximately 70,000 children and adults with cystic fibrosis wo... ... middle of paper ... ...fibrosis. In (2011). A.D.A.M medical encyclopedia A.D.A.M., Inc. Retrieved from http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/ Clarithromycin therapy for patients with cystic fibrosis: A randomized controlled trial. Pediatr Pulmonol, Galli, F., Battistoni, A., Gambari, R., Pompella, A., Bragonzi, A., Pilolli, F., Iuliano, L., Piroddi, M., Dechecchi, M. C., Cabrini, G.. Oxidative stress and antioxidant therapy in cystic fibrosis, Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease Dowman, J. K., Watson, D., Loganathan, S., Gunson, B.K., Hodson, J., Mirza, D.F., Clarke, J., Lloyd C., Honeybourne D., Whitehouse, J.L., Nash, E.F., Kelly, D., van Mourik, I., Newsome, P.N.. (2012). Long-term impact of liver transplantation on respiratory function and nutritional status in children and adults with cystic fibrosis. American Journal of Transplantation,
Cystic fibrosis is a genetic disease of the secretory glands that affects the respiratory and digestive system. It mainly effects the lungs, pancreas, liver, intestines, sinuses, and reproductive organs. Cystic fibrosis effects the cells that produce sweat, mucus, and digestive fluids. Mucus becomes thick and sticky causing build-up in the lungs and blocking airways; making it easier for bacteria to develop. This prompts repeated lung infections and can cause severe lung damage after some time. The thick mucus can also block ducts in the pancreas. Therefore, digestive enzymes can't reach the small intestine. Without the digestive compounds used to break down food, the intestines can't completely absorb vital supplements. CF also causes sweat to become salty. When patients with cystic fibrosis sweat, a large amount of salt is secreted, which can agitate the equalization of minerals in the blood and cause numerous health issues.
Visse, M, Abma, T, Oever, HVD, Prins, Y, Gulmans, Y. 2013. Perceptions of hospital admission in patients with cystic fibrosis. Journal of Hospital Administration 2(3), pp. 54-65.
It affects mainly the lungs and digestive tract. Cystic Fibrosis causes a buildup of thick mucus in the lungs, which leads to breathing troubles. Mucus in the lungs also benefits bacteria that are responsible for infections. A child with Cystic Fibrosis
In the Shadow of Illness, the book describes different experiences of families who have or had children with cystic fibrosis (CF). CF is an inherited disease that is passed on from the mother or father who is a carrier, but doesn’t have the condition. Doctors have figured that in this scenario, the parents are likely to have a child with CF. Individuals with CF have to take Cotazymes to help the pancreas digest food. If the person does not take these enzymes, the food goes straight through them as diarrhea. Also, the person’s lungs are affected by a thick mucus that must be removed or thinned before it clogs. Doctors recommend the patient to perform daily breathing exercises that prevent the mucus from thickening; for example, swimming
Chronic obstructive pulmonary disease (COPD) is preventable disease that has a detrimental effects on both the airway and lung parenchyma (Nazir & Erbland, 2009). COPD categorises emphysema and chronic bronchitis, both of which are characterised by a reduced maximum expiratory flow and slow but forced emptying of the lungs (Jeffery 1998). The disease has the one of the highest number of fatalities in the developed world due to the ever increasing amount of tobacco smokers and is associated with significant morbidity and mortality (Marx, Hockberger & Walls, 2014). Signs and symptoms that indicate the presence of the disease include a productive cough, wheezing, dyspnoea and predisposing risk factors (Edelman et al., 1992). The diagnosis of COPD is predominantly based on the results of a lung function assessment (Larsson, 2007). Chronic bronchitis is differentiated from emphysema by it's presentation of a productive cough present for a minimum of three months in two consecutive years that cannot be attributed to other pulmonary or cardiac causes (Marx, Hockberger & Walls, 2014) (Viegi et al., 2007). Whereas emphysema is defined pathologically as as the irreversible destruction without obvious fibrosis of the lung alveoli (Marx, Hockberger & Walls, 2014) (Veigi et al., 2007).It is common for emphysema and chronic bronchitis to be diagnosed concurrently owing to the similarities between the diseases (Marx, Hockberger & Walls, 2014).
Cystic Fibrosis (CF) is a very common, potentially life threatening condition. The disease is caused by inheritance, and affects the exocrine glands of the patient. Cystic fibrosis is found primarily among Caucasians and those of European descent. Those diagnosed with Cystic Fibrosis battle daily to perform simple tasks, such as breathing, as the mucus in their bodies thickens immensely. This mucus will potentially accumulate in the patient’s vital organs, such as the lungs, pancreas, and intestines. One can determine if he/she has cystic fibrosis by analyzing certain symptoms. Cystic Fibrosis can be diagnosed according to the symptoms the patient shows, and can be treated through specific types of treatments, such as gene therapy.
A widely accepted Caucasian disease has since changed; as cases of its existence are appearing in the South Asian Population (Orenstein, Rosenstein and Stern, 2000). First discovered in 1989, Cystic Fibrosis (CF) is a genetically predetermined condition, its presences is lifelong and highly complex, which is why many CF sufferers and families develop various mechanisms in order to adapt to the condition (Tippingemail, Scholes, Cox, 2010). Cystic Fibrosis causes the body to develop thick, sticky mucus which clogs the lungs and affects other organs in the body, mainly the pancreas. The layer of mucus if untreated develops in to a chronic infection which can be detrimental. The pancreas of a CF patient is most vulnerable, once the mucus has reached this organ, it halts digestive enzymes from reaching the intestines which aid in absorbing food, therefore affecting nearby organs (Davies, Alton, and Bush, 2007). Currently there are 9,000 people diagnosed with CF in England (CF Trust, 2011). It has been estimated that there is 1 in 10,000 South Asian sufferers in the UK alone (Kabra, Kabra, Lodha, Ghosh, Kapil et al, 2003; McCormick, Green, Mehta, 2002). Prior research-based literature that focuses on people with CF and their families covers some of the experience of living with the disease but displays some major gaps; none has specifically targeted South Asian individuals. Cross culturally this is also the case; the limited availability of CF research has influenced scientists to devote more attention in this area. For instance, information in regards to CF in Egypt is very limited; firstly CF has been believed to occur infrequently as there has not been a sufficient amount of known CF cases. Naguib, Schrijver, Gardner, Pique, Doss, Ze...
Cystic Fibrosis is an inherited disease characterized by the buildup of thick, sticky mucous that can cause severe damage to the body’s organs. Mucous is usually a slippery substance that lubricates and protects the linings of the airway, digestive system, reproductive system and other organs and tissue. Problems with digestion can lead to diarrhea, malnutrition, poor growth, and weight-loss. Due to the abnormally thick mucous it can can clog airways, leading to breathing problems and bacterial infections in the lungs. Bacterial infections can lead to coughing, wheezing and inflammation. Overtime these infections can lead to permanent damage in the lungs including the formation of scar tissue, known as fibrosis and cysts in the lungs (Genetics Home Reference, 2013). The symptoms and signs of this disease vary but mostly include progressive damage to the respiratory system and chronic digestive system problems. An individuals’ lungs who are infected by cystic fibrosis have bacteria from an early stage. This bacteria can spread to the small airways, leading to the formation of bacterial micro-environments known as biofilms. Biofilms are difficult for antibodies to penetrate, therefore the bacteria repeatedly damage the lung and gradually remodel the airways, resulting in difficultly to eradicate the infection (Welsh, 1995). Cystic fibrosis patients may even have their airways chronically colonized be filamentous fungi and/or yeasts. Most men with cystic fibrosis have congenital bilateral absence of the vas deferens (CBAVD), a condition in which the tubes that carry sperm are blocked by mucous and do not develop properly. As well, women may experience complications in pregnancy. Either the c...
For a baby to have Cystic Fibrosis, he/she has to have precisely two defective cystic fibrosis genes from each of the parents. Both parents must be carriers of this condition. The parents will also not show any signs of having this condition. It is a recessive condition which explains why a majority of the carriers never have a clue when they are carriers. The problem lies on chromosome seven which has the defect in those people living with this condition. Typically, people have 46 chromosomes, which pair up forming 22 pairs in every nucleus in the human body (Wicks, 2007). The remaining two chromosomes are the sex chromosomes hence the seventh chromosome is the one that gets mutated causing Cystic Fibrosis. Some of the common symptoms of Cystic Fibrosis include persistent coughing, salty-tasting skin, wheezing and shortness of breath. Additionally, there is very poor weight gain despite excessive appetite, fleshy growths in the nose and bulky, greasy
Chronic obstructive pulmonary disease (COPD) is an umbrella term used to refer to chronic, progressive diseases affecting the respiratory system including chronic bronchitis and emphysema. COPD is characterised by inflammation, loss of muco-ciliary clearance, fibrosis and loss of elastic recoil in the lung parenchyma. Inflammation increases mucous secretions that cannot be efficiently cleared from the airway due to the destruction of cilia. Damage to lungs and excessive mucous reduces airflow, gas exchange and expiratory volume leading to gas trapping. These changes cause the patient to develop the dyspnoea, cough and sputum production characteristic of COPD (Burt & Corbridge, 2013, p. 34-35).
cleared out of the lungs, but in Cystic Fibrosis the mucus traps the germs in the lungs. The germs
Taussig, Lynn M., M.D., and James C. Cunningham. An Introduction to Cystic Fibrosis for Patient and Families. 5th ed. N.p.: Cystic Fibrosis Foundation & Axcan Pharma, 2003. Print.
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Parker, Steve. "Chronic Pulmonary Diseases." The Human Body Book. New ed. New York: DK Pub., 2007.
Farrell, P. M., Rosenstein, B. J., White, T. B., Accurso, F. J., Castellani, C., Cutting, G. R., ... & Campbell III, P. W. (2008). Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis