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Cystic fibrosis pathogensis
Cystic fibrosis pathogensis
Pathophysiology for cystic fibrosis
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Hello, I like your post; I had one patient with cystic fibrosis when I work CVICU. It is a very interesting topic, and I really want to know more about the disease process. Thank you for sharing!
Cystic fibrosis is a hereditary disease that affects all exocrine glands and leads to dysfunction in their secretion. These diseases, mainly affect the Caucasian population. Early in the development of the disease, the pancreas of the patient with cystic fibrosis plugs with secretion. As the disease progresses, the lung become involved and starting secreting a thick and viscous mucus that plug the airway and leads to airway obstruction and chronic infection in cystic fibrosis patients. As a result, there are different respiratory complication with
Membranes are involved in Cystic Fibrosis when it comes to the genes that are prone to the disease. In a regular functioning body, the CFTR gene helps make the channel that transports charged chloride ions into and out of cell membranes. In a body with cystic fibrosis, the chloride channels don’t function properly, and do not allow chloride ions into and out of the cell membranes, causing the thick mucus (as mentioned earlier) to be produced. The concentration gradients are involved when it comes to moving these molecules and ions across the cell membranes with passive and active transport. Passive transport substances move down concentration gradients while active transport substances move against their concentration gradients (keep in mind this is in a healthy functioning body). With cystic fibrosis, there is a defect in the transport protein, which does not move through the concentration gradient
Cystic Fibrosis (CF) Pathophysiology: Cystic fibrosis is a genetic disease of the secretory glands that affects the respiratory and digestive system. It mainly affects the lungs, pancreas, liver, intestines, sinuses, and reproductive organs. Cystic fibrosis affects 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.
Pizzignacco, TM, de Mello DF, de Lima, RA. 2010. Stigma and cystic fibrosis. Revista Latino-Americana de Enfermagem 18(1), pp. 139-42.
Along with the problems of chronic illnesses themselves, many other problems may come. Treatments and medications are just the beginning of things when it comes to problems with illnesses. With cystic fibrosis, you start out with a high number of medications and treatments to begin with. The older you get and the worse your condition gets, the more you take. (“Psychological impact,” n.d.) Many struggles come along with taking these medications and treatments. When children are first diagnosed with cystic fibrosis, they are typically very young. From the beginning, there are many medications and treatments that needed to be taken and done. Sometimes, trying to get children to take medications and treatments is like pulling teeth. The medications for cystic fibrosis are extremely important. If cystic fibrosis patients miss medica...
With additional bodily fluid and less cilia to dispose it, patients build up an endless hack that raises sputum, a blend of bodily fluid and cell debris. Stagnant bodily fluid in the respiratory tract gives a development medium to microorganisms. This can prompt disease and bronchial aggravation, with side effects that incorporate dyspnea, hypoxia, cyanosis, slight fever, chills, and assaults of hacking (Saladin, 2010). In addition, bronchitis can cause shortness of breath, wheezing, chest pain, and tickle of the
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
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.
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...
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).
Person, A. & Mintz, M., (2006), Anatomy and Physiology of the Respiratory Tract, Disorders of the Respiratory Tract, pp. 11-17, New Jersey: Human Press Inc.
The clinical manifestation one may see in patients with chronic bronchitis are chronic cough, weight loss, excessive sputum, and dyspnea. Chronic cough is from the body trying to expel the excessive mucus build up to return breathing back to normal. Dyspnea is from the thickening of the bronchial walls causing constriction, thereby altering the breathing pattern. This causes the body to use other surrounding muscles to help with breathing which can be exhausting. These patients ca...
Thomson, Anne H., and Ann Harris. Cystic Fibrosis: the Facts. Oxford: Oxford UP, 2008. Print.
While cystic fibrosis (CF) is not a new disease, there is still a lot to learn about it. In 1938 a pathologist, Dr Dorothy Andersen, provided the first clear description of cystic fibrosis. Before this time there had been reports of people that had the symptoms of someone with CF. During the seventeenth century children with the symptoms of CF were thought to be bewitched and their life expectancy was very short. Dr Dorothy Andersen gave this disease its name because cystic fibrosis refers to the scarring that is found on the pancreas. People with CF also have associated diseases like salt-loss syndrome, obstructive azoospermia, and gastrointestinal abnormalities. CF is inherited from one’s parents, making it a genetic disease. CF is caused by mutations in a certain gene that produces the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This gene was first discovered in 1989 on chromosome pair 7. Cystic fibrosis is a recessive disease meaning both parents have to be a carrier. Whenever two CF carriers have a child together, there’s a 1 in 4 chance that their child will inherit the CF mutation. Although CF produces coughing it cannot be transmitted any other way than hereditary.
In cystic fibrosis, a genetical condition that has a high population of young patients with multiple medical treatment requirements, it is of clinical importance to ensure compliance to their treatments in order to avoid a premature death. Behavioural economics can help here by addressing how we improve motivation with and perceived value of medical treatments – to improve overall patient compliance. In treatment compliance with children, up to 70% of patients with chronic illnesses have poor adherence (Haynes RB, 2002). Poor adherence to cystic fibrosis therapies may result in increased disease symptoms, decrease physical functioning, increased time in hospital, morbidity rates and mortality, as such an increased healthcare costs (Vibeke Bregnballe, 2011). Therefore the more compliant the more cost-effective treatments become, and the healthier the patients stays. It is also important to note that for every drug skipped represents a financial loss. Capgemini group reported in 2013 that the worldwide cost of non-adherence to be estimated at 564 billion dollars. So, if we get childre...
Parker, Steve. "Chronic Pulmonary Diseases." The Human Body Book. New ed. New York: DK Pub., 2007.