Allison Lynaugh
Professor Lund
Biology 326 Disease
09 May 2014
Cystic Fibrosis
INTRODUCTION
Cystic fibrosis (CF) is the most common and lethal inherited disease in the United States, currently affecting about 30,000 Americans. Approximately 1,000 new cases are diagnosed each year, with an overall birth prevalence in the United States of 1 in 3,700 (Cystic fibrosis foundation, 2007). CF is an autosomal recessive disorder and approximately 1 in 29 Caucasians are unknowing, symptomless carriers of the defective gene. The defective gene which causes CF is the result of a single point mutation on the CFTR gene which is responsible for regulating sodium chloride transportation throughout epithelial cells. Since chloride ions do not properly move throughout the cell, the epithelial cells in individuals with CF produce abnormally thick and sticky mucus which builds up in the lungs, pancreas, and other organs. Mucus in the lungs eventually blocks the airways and also becomes a breeding ground for bacteria, which ultimately leads to chronic or life-threatening lung infections. This abnormal mucus in CF obstructs the pancreas, which prevents the absorption of fats and fat-soluble vitamins, leading to impaired nutrient absorption. As a result of poor ion regulation in epithelial cells and increased mucus viscosity, individuals with CF have symptoms including “salty-skin,” wheezing or coughing, and recurrent respiratory infections. Although CF is currently an untreatable disease, patients have treatment options including airway clearance techniques, medications, and dietary management which improve the quality of life and life expectancy.
HISTORICAL RELEVANCE
The association between salty skin, pancreatic failure, and lung infections have ...
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...sociation, 2010).
Lung disease is the most common cause of morbidity and mortality in CF.
TREATMENT
Cystic fibrosis remains untreatable however treatment options to clear mucus from the lungs and reduce inflammation include antibiotic and anti-inflammatory medications, gene therapy, and airway clearance techniques. In addition to clinical treatments, lifestyle adjustments such as exercise and dietary management are recommended to improve breathing and digestion. Antibiotic mediations are taken to prevent and treat reoccurring lung infections. To reduce inflammation of the pancreas and lungs, anti-inflammatory medications are prescribed. Respiratory treatment helps loosen secretions and is essential for clearing mucus from the lungs and opening of airways. These techniques literally dislodge mucus secretions which can then be drained from the lungs or coughed out.
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 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.
Suh JD. & Kennedy DW., 2011. Treatment Options for Chronic Rhinosinusitis. American Journal of Respiratory and Critical Care Medicine, 8, 132-140
Sequeiros, IM, Jarad, NA. 2009. Home intravenous antibiotic treatment for acute pulmonary exacerbations in cystic fibrosis-Is it good for the patient?. Annals of Thoracic Medicine 4(3), pp. 111-114.
Chronic obstructive pulmonary disease or COPD is a group of progressive lung diseases that block airflow and make it hard to breathe. Emphysema and chronic bronchitis are the most common types of COPD (Ignatavicius & Workman, 2016, p 557). Primary symptoms include coughing, mucus, chest pain, shortness of breath, and wheezing (Ignatavicius & Workman, 2016, p.557). COPD develops slowly and worsens over time if not treated during early stages. The disease has no cure, but medication and disease management can slow its progress and make one feel better (NIH, 2013)
Chronic suppressive antibiotic therapy is the recommended treatment of chronic P. aeruginosa infections in CF airways. This method of treatment consists of the daily use of nebulized tobramycin or colistin for the remainder of the patient’s life, along with a combination therapy consisting of two antibiotics, administered intravenously, every three months for two weeks[6]. Moreover, azithromycin as well as DNase are taken daily. Since chronic suppressive antibiotic therapy has proven to extend the life of CF patients and delay the weakening of pulmonary functions, some aspects of this therapy are steadily being used in biofilm infection treatment in other areas of the body.[6]
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...
...a are bronchodilators like anticholinergic, beta agonists, theophylline and oxygen, which are for the advance cases of the disease. In addition, the best treatment for people whom have emphysema is for them to stop smoking.
The breast is a muscle connected to the chest wall made up of fatty tissues containing milk-forming glands that drain into the breast ducts during breast-feeding, and then leave the ducts through the nipple (Newson et al). These glands can decrease or increase in number and size. Breast lumps or cysts can form in various tissues or components that make up the breast. The English diction cyst comes from the Ancient Greek word kystis, meaning “bladder” or “pouch”. A cyst is an organ or cavity containing a liquid, semisolid or gaseous secretion. Cysts appear within tissue and can affect any part of the human body. They range in size from minuscule to the mass of team-sport balls such as tennis or baseballs. Cysts are also referred to as any normal bag or sac in the body, such as the bladder.
Pancreatic enzyme supplements, vitamins, salts are required to balance the absorption of nutrients. Since CF can cause impaired growth, a high fat, high-calorie diet is key for normal growth and development in kids with this condition. These diets can aid in maintaining optimal growth. Additionally, CF can cause increased lung infections, thus good nutrition is imperative to maintain a great defense against these infections. Other methods of managing this condition include implanted devices which allow long-term access to the bloodstream for the common administration of drugs (Cohen-Cymberknoh, Shoseyov & Kerem, 2011). With this method, management of this condition is less intrusive and more efficient. The treatment that targets the defective CF-causing gene is CF transmembrane conductance regulator modulators (CFTR). This medication facilitates effective flow of fluids and salts on the lung surface, thus thinning the thick mucus, which people with CF have built in their lungs. The commonly used modulator brands are Orkambi and Kalydeco which are prescribed for kids with ten various mutations of the gene causing the condition (Wicks, 2007). Future treatments are also focusing on gene therapy such as CF transmembrane conductance regulator
Cystic fibrosis is a genetic disease; it is passed down through families. Cystic Fibrosis causes sticky mucus to build up in lungs, digestive track, and other areas of the body. The mucus clogs can lead to life threatening mucus infections. {Board, A.D.A.M. Editorial. Cystic fibrosis. U.S. National Library of Medicine, 16 May 2012. Web. 11 Jan. 2014.} Some of the most common symptoms of Cystic Fibrosis are salty skin, wheezing or shortness of breath, and persistent cough, sometimes with thick mucus called phlegm. {Phlegm. Dictionary .com. Dictionary.com, n.d. Web. 13 Jan. 2014.} Other symptoms are poor growth or weight gain even with a good diet. {Children's Health." Symptoms of Cystic fibrosis. N.p., n.d. Web. 13 Jan. 2014.} Current treatments of Cystic fibrosis include chest physical therapy; where they pound chest and back allover with device to loosen mucus from lungs, or wear and inflatable therapy vest that uses airwaves to force mucus upper airways. Exercise, and Antibiosis, both oral and IV tube are also used as treatment. {"How Is Cystic fibrosis Treated?" - NHLBI, NIH. N....
There is currently no cure but there are effective lifestyle changes that can be implemented to slow the progression of my disease. I will have to avoid respiratory irritants such as fumes, cooking odors, and certain fumes. It is also imperative for me to exercise regularly, despite my symptoms. I will also have to make sure to protect myself from cold air, which can cause spasms of the bronchial passages, and prevent respiratory infections by getting an annual flu shot and practicing sanitary procedures such as washing my hands and wearing a face mask if needed. I will make sure to follow all of these steps so that I will be able to see my kids
Chronic bronchitis is a disorder that causes inflammation to the airway, mainly the bronchial tubules. It produces a chronic cough that lasts three consecutive months for more than two successive years (Vijayan,2013). Chronic Bronchitis is a member of the COPD family and is prominently seen in cigarette smokers. Other factors such as air pollutants, Asbestos, and working in coal mines contributes to inflammation. Once the irritant comes in contact with the mucosa of the bronchi it alters the composition causing hyperplasia of the glands and producing excessive sputum (Viayan,2013). Goblet cells also enlarge to contribute to the excessive secretion of sputum. This effects the cilia that carry out the mechanism of trapping foreign bodies to allow it to be expelled in the sputum, which are now damaged by the irritant making it impossible for the person to clear their airway. Since the mechanism of airway clearance is ineffective, the secretion builds up a thickened wall of the bronchioles causing constriction and increasing the work of breathing. The excessive build up of mucous could set up pneumonia. The alveoli are also damaged enabling the macrophages to eliminate bacteria putting the patient at risk for acquiring an infection.
Cystitis is the medical term for inflammation of the urinary bladder. Most of the time, the inflammation is caused by a bacterial infection, and it’s called a urinary tract infection. A bladder infection can be painful and annoying, and it can become a serious health problem if the infection spreads to your kidney.
One of the best ways to clear up a sinus infection or chronic sinusitis is to use saline nasal spray. The saline nasal spray helps dry up the mucus and relieves some of the pain and inflammation in the sinus cavity. Some people also find relief by using a room humidifier and adding vapor rub for congestion. It is also highly recommended to drink plenty of water when you suffer from chronic sinusitis because is helps to rid the boy of mucus. Water helps loosen and expel the mucus in both the sinus cavity and the lungs. Some people also use essential oils on their chest and in their humidifier for a touch of relief from the