Module Two
Question 2
Mr Bingley is a 13-year-old boy with cystic fibrosis. He presents to his GP complaining of cough, which is productive of green sputum. He appears to be short of breath, and can be heard wheezing on physical examination. He also appears to be underweight. Describe the pathophysiology of cystic fibrous. In you answer explain why Mr Bingley appears underweight and has problems with his airways.
Cystic fibrosis is a genetic disorder that affects the endocrine system, renal system, gastro-intestinal system, and reproductive system, but it’s effects are felt mostly in the lungs where chronic infection leads to respiratory failure and eventually death (Craft, Gordon, and Tiziani, 2010). The disease is characterized by it’s viscous mucus, and cycle of
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The airways of patients with cystic fibrosis are eventually obstructed by mucus and hyper-inflated; lung function becomes progressively worse as the airway walls are repeatedly damaged in a cycle of inflammation and infection (Cohen and Prince, 2012). For approximately 90% of patients, respiratory failure will eventually become fatal unless they receive a lung transplant (Davies, Ebdon, and Orchard, 2014). Common symptoms of cystic fibrosis are coughing, diarrhoea, and malnutrition (Haack, Arago, and Novaes, 2013). Patients often experience tachypneoa, wheezing, chronic rhinitis (inflammation of the nose), nasal polyps, haemoptysis (coughing blood) and problems associated with lung inflation such as atelectasis, hyperinflation, or pneumothorax (Lumbamba et Al., 2012). Cystic fibrosis sufferers may also have a barrel chest, club fingers and toes, pulmonary hypertension and low tolerance for physical exercise due to their damaged respiratory system (Lumbamba et Al., 2012). The lack of normal CFTR creates hyper viscous secretions with
Lungs – Mucus plugging, chronic bacterial infections, pronounced inflammatory response, damaged airways leading to respiratory insufficiency, progressive decline in pulmonary function.
Abstract: The main goal of this paper is to explain what Cystic Fibrosis is and also to explain what
Cystic Fibrosis causes abnormal amounts of excessively thick and sticky mucus to develop within the lungs, airways and the digestive system. Due to the thick and sticky mucus, digestive functions in the pancreas are impaired and bacteria is trapped in the lungs causing recurrent lung infections. Cystic Fibrosis Victoria (2015) states that symptoms of Cystic Fibrosis can include:
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
Cystic fibrosis is an acquired malady described by the development of thick, sticky bodily fluid. This bodily fluid can make harm a number of the body's organs. The most widely recognized signs and indications incorporate dynamic harm to the respiratory framework and interminable digestive framework issues. The seriousness and elements of Cystic fibrosis fluctuate amongst each influenced person. The bodily fluid that greases up and secures the linings of the aviation routes, digestive framework, the regenerative framework and different organs and tissues is unusually thick and sticky in patients with Cystic fibrosis. This can obstruct the aviation routes prompting serious breathing issues and bacterial diseases in the lungs. The bacterial
In regards to the digestive tract, organs such as the liver, small and large intestines, gallbladder, mouth, stomach, esophagus, and pancreas are prime locations of complications pertaining to mucus blockage. The pancreas, which is responsible for secreting enzymes assisting in food digestion and insulin for maintaining blood sugar levels, is mainly affected by cystic fibrosis. Thick mucus production creates a blockage within the pancreas ducts and creates a decline in the pancreas’ ability to secrete digestive enzymes to aid in digestion.
Cystic Fibrosis (CF) is an autosomal recessive genetic disease that causes thickened mucus to form in the lungs, pancreas, and other organs. It affects a specific protein called Cystic Fibrosis Transmembrane Regulator (CFTR) that controls the normal movement of sodium, chloride, and water in and out of the cells within the body. Those diagnosed with CF have either too little or abnormal CFTR. When CFTR is absent or defective, the mucus usually secreted by the cells in the pulmonary airways, pancreatic ducts, and gastrointestinal tract become thickened, leading to obstructions, frequent infection, and loss of function in the affected organs (Cystic Fibrosis Symptoms, Causes & Risk Factors, 2018). According to the Cystic Fibrosis Foundation
In infants symptoms include coughing, wheezing, excess mucus in the lungs, shortness of breath, extremely salty skin and decelerated growth. With infants it is very common for them to develop pneumonia infections because of all the bacteria that resides in the lungs. With treatment, most patients with cystic fibrosis live into their 20’s and 30’s, some individuals with milder cases can live longer. Death is most often due to end-stage lung disease.Thus far no cure for the disease has been found. Although, research on gene therapy is promising. Treatment is generally aimed at alleviating symptoms, preventing infections, and slowing the progress of the disorder. (Egan’s 10th Ed,
Health risks involving the effects of how excess mucus in the lungs causes respiratory problems and how it prevents the pancreas from releasing digestive enzymes that break down food and absorb vital nutrients will be discussed (Cystic Fibrosis Foundation, 2016). An intervention and treatment plan will be tailored to the individual and include specialized management and care of dedicated and specialized medical professionals. Therapies will be discussed and implemented that will provide techniques, equipment, and medicines that will loosen and thin mucus to clear the airways, reduce infections, and improve the absorption of vital
Cystic Fibrosis is a genetic disease that causes the body’s lungs to generate a different type of mucus than a non-infected body would. The contaminated lungs will produce mucus that is thick and adhesive which clogs the lungs and leads to an unpleasant and abhorrent lung infection. CF also interferes with the pancreas, disallowing the digestive enzymes from breaking down and absorbing food in the intestine. This can result in low nutrition, feeble growth, excessive sweat production, difficulties in breathing, and sometimes lung disease. When producing extra sweat and mucus, the body loses salt. If too much salt is lost, it can cause abnormal heart rhythms, disturbance of minerals in the blood, and perhaps, shock.
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.
Between genetics, symptoms, pathology, treatments and new innovations, one could say Cystic Fibrosis is a loaded and complicated illness. However, C.F. effects too many people for it to be forgotten about or underrepresented. With further research and new discoveries, every day we are one set closer to curing this
Cystic Fibrosis (CF) is an autosomal recessive gene that causes a wide range of symptoms because there are over 1,000 changes or mutations that can occur within the cystic fibrosis transmembrane receptor (CFTR) protein. The CFTR protein is generally a chloride ion chain “regulated by cyclic adenosine monophosphate and therefore can act as a regulator of other electrolyte channels”(Grossman, S., & Grossman, L. 2005, p. 46). Typically this protein allows chloride ions to exit mucus-producing cells allowing water to flow in and thin the mucus. However, if the CFTR protein has been mutated, such as in cystic fibrosis, chloride ions cannot exit. This causes the mucus to thicken, become sticky, and obstruct the various channels it passes through. This build up of mucus also prevents bacteria from being cleaned from cells thoroughly increasing the patients risk for infections (Grossman, S., & Grossman, L. 2005). However, the severity of CF depends on whether the patients have complete or partial loss of the CFTR gene. If the person has the classic form of CF abnormalities of CFTR will commonly affect “…the respiratory, gastrointestinal, endocrine and metabolic, and genitourinary systems”(Schram, C. 2012). However, if people have atypical forms of CF their genetic disorder may only affect one of the organ systems and may not be found until the patient develops symptoms in their late childhood, early adolescence, or adulthood
Cystic Fibrosis is a disease that affects the body in many ways throughout the patient's life. Newborns with Cystic Fibrosis may experience delayed growth, inability to gain weight, and salty-tasting skin ("Cystic," umm.edu 1). Older patients may be infertile, have recurring pancreatitis, and respiratory problems ("Cystic," umm.edu 2). Considering that these are just symptoms involving age, the full spectrum of ailments that afflict a patient with Cystic Fibrosis is far more taxing on a patient.
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