Cystic Fibrosis
Cystic Fibrosis is a recessive disorder that is a life threatening disease that causes persistent lung infections and progressively limits the ability to breath. Unfortunately, there is no cure for this genetic disorder, but Airway Clearance, Inhaled Medicines, and Pancreatic enzyme supplements can increase the person’s lifespan incredibly. There are about 70,000 people worldwide with this disease. Approximately 1,000 new cases of cystic fibrosis are diagnosed each year. The average human with cystic fibrosis lives to about 30 if they take their medicine.
Symptoms of this disorder are: salty tasting skin, Persistent coughing, Frequent lung infections, wheezing or shortness of breath, and poor growth or weight gain in spite
Cystic fibrosis is an existence restricting autosomal recessive disorder that influences 70,000 people around the world. The condition is known to affect principally those of European descent, though cystic fibrosis has been accounted for in all races and ethnicities. [12] Unusually viscous emissions in the airway of the lungs and in ducts of the pancreas in people with cystic fibrosis cause hindrances that prompt aggravation, tissue harm and destruction of both organ systems. Studies show that Cystic Fibrosis is more prominent in White Americans than African Americans, and Hispanics. A large number of Americans are carriers of this mutated Cystic Fibrosis gene, however if an individual is affected they must acquire two of these genes keeping
Cystic fibrosis (CF) is a lethal autosomal recessive disorder with a clinical incidence of 1 in every 3500 newborns1. Currently, over 4000 Canadians have CF, with 60% being adults2. In the past, CF was considered a fatal disease with a life expectancy of less than two years, however, advances in enzymatic and antibiotic therapy have greatly improved the life expectancy of patients afflicted with CF3. Currently, the median age of Canadian CF patients is 50 years2.
Cystic Fibrosis is a life threatening condition that causes severe damage to the lungs and digestive system. Developing this condition can change a person's entire life. Depending on the severity of the condition a person can be affected by mild symptoms such as shortness of breath, all the way to severe symptoms such as rectal prolapse, or even death. Screening is done on infants in all 50 states; therefore if a child has inherited Cystic Fibrosis it will be known in the first months of life. As professional staff members of a nursing community it will be necessary to help prepare the child and the family for the life of Cystic Fibrosis.
Today, there is no cure for Cystic Fibrosis, but if the disease is found in an individual early enough, the patient can be recovered. About 85 to 90% of patients will have problems absorbing nutrients from their intestines. And because enzymes are lost when diagnosed, enzyme supplements are required to prevent bad nutrition and malnutrition. There are two main body parts that can be cared for the treatment of Cystic Fibrosis: The lungs and the chest. Lung problems can also be treated, but not cured. Antibiotics and other drugs may loosen the thick mucus in the lungs, but if it fails, a transplant lung may help to extend life. But about four hundred people die each year while waiting for a lung transplant. Also Chest physical therapy is a technique done by vigorous claps on the back and chest to loosen the thick mucus from the lungs. The average lifespan of an individual with CF is about thirty years.
Cystic fibrosis is a disease that is continually affecting children and adults in the United States. This is an inherited and life-threatening disease which affects many organs in the body. According to the Center for Disease Control, there are an estimated 30,000 people affected by this disease today. There are also approximately 2,500 babies born each year with Cystic fibrosis and unfortunately there are millions of people unaware they even carry the gene for this disease.
to a patient of cystic fibrosis. Scientists have always been daunted by this fatal genetic disease
Cystic fibrosis is a genetic disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator protein, which results in a defective chloride channels. Chloride is part of salt that helps transportation of water needed for thin, free flowing mucus. The main characterization of cystic fibrosis is thick, sticky mucus in the lungs, making breathing difficult and allowing pathogens to invade (Ratjen and Döring 2003). Airway mucus creates an iron limiting environment to prevent bacterial growth. However, Pseudomonas aeruginosa is a bacteria able to resist this airway mucus. This proposes a question as to why people with cystic fibrosis are prone to infection when exposed to the same pathogen as any healthy individuals. Research has suggested defective mucus clearance and biofilm formation could contribute to prolonged Pseudomonas aeruginosa infection in people with cystic fibrosis (Gi et al. 2015). Also, targeting cystic fibrosis transmembrane conductance regulator proteins to allow chloride transportation has been proven to decrease mucus volume and Pseudomonas aeruginosa infected tissue (Rowe et al. 2014). The above information suggests increased mucus volume causes prolonged infection in the lungs of cystic fibrosis patients. This paper will describe cystic fibrosis and will focus on the unanswered question as to why cystic fibrosis patients experience chronic infection in their lungs.
The way to determine if one has Cystic Fibrosis, which is caused by the mucus in the body’s organs, varies from patient to patient and depends on the severity of the disease. “CF is characterized by several clinical features: increased viscosity of mucous gland secretions, a striking elevation of sweat electrolytes, an increase in several organic and enzymatic constituents of saliva, and abnormalities in autonomic nervous system function” (Essentials of Pediatric Nursing 795). Failure to grow, persistent cough with mucus production, and large frequent bowel movements are early manifestations of CF in childhood. Additionally, one of the first signs of cystic fibrosis is an excessively salty taste to the skin, and parents often can taste the salt when they kiss their child. Nonetheless, problems with breathing are among the most common symptoms. In adults, a frequent cough is the first symptom of Cystic Fibrosis. Pneumonia, bronchiolitis, and bronchitis are among other respiratory problems that may be indicative of Cystic Fibrosis. “The disease progresses from being a disease of the small airways (Chronic bronchiolitis) to involvement of the larger airways, and finally causes destruction of lung tissue. CF also progresses to a restrictive lung disease
Cystic fibrosis, an inherited disease of the secretory glands that affects the liver, pancreas, intestines, lungs, sinuses, and sex organs, affects about 30,000 Americans with 1,000 new cases diagnosed each year. Normal mucus is a watery, slick substance made by the tissues that keeps the inside of organs moist, preventing infection. People with cystic fibrosis have thick and sticky mucus that builds up in their lungs, blocking the airways. This buildup can cause serious lung diseases from bacteria growth that damages the lungs. This damage to the lungs can cause severe breathing problems which can lead to respiratory failure. Respiratory failure is the most common cause of death in people with cystic fibrosis. The thick and sticky mucus also can block the tubes that carry important enzymes from the pancreas to the small intestines that are needed to break down food and for your body to absorb the nutrients essential for one’s health.
There are many diseases that greatly affect our respiratory physiology; one of those diseases is cystic fibrosis. Cystic fibrosis, according to the National Institute of Health (2013), is an inherited disease of the secretory glands or exocrine glands. Not only does this disease affect our respiratory system, it takes a toll on our digestive system. The respiratory system includes the gas exchange from our external atmosphere and our internal environment. This gas exchange of oxygen and carbon dioxide through our atmosphere and lungs does play a roll with cystic fibrosis. Cystic fibrosis is inherited, it affects many parts of the body mainly the respiratory system, and there are many signs and symptoms to this overwhelming disease.
Cystic Fibrosis is a defective gene that causes the mucus to become thick and build up in the lungs, pancreas, and other organs. This is a genetic disorder caused by a defective gene. People that have Cystic Fibrosis have two copies of the recessive Cystic Fibrosis gene (one from each parent). When parents are both carriers their children’s chances are 25% have Cystic Fibrosis, 50% will be a carrier, and 25% will not have Cystic Fibrosis. This causes lung infections and enzymes not to be digested so that nutrients and fats are not absorbed. People are at risk for having lung infection and it stops the absorption of food and key nutrients.
Kaneshiro describes cystic Fibrosis as a system wide disease process that affects the mucous production of organs from the time of birth, most commonly affecting the lungs and intestines (2014). He goes on to say that this is a hereditary disease that is most commonly found in the first 2 years of a child’s life and the symptoms start out as mild and can be easily overlooked, which accounts for the late diagnosis even though the disease started at birth (2014). The most common early life symptoms include a lack of stools in the first couple of days after birth and salty tasting skin though as the child ages there is a significant lack of developmental growth and lack of weight gain along with frequent respiratory infections and constipation (Kaneshiro, 2014). The life expectancy of a child with CF currently is at around 37 years old when that child receives extensive early treatment for the side effects of CF, though there is no cure at this time, though the treatments are getting more effective every year (Kaneshiro, 2014).
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 due to a mutation in the gene that encodes cystic fibrosis transmembrane conductance regulator (CFTR) protein. It effects the exocrine glands which are responsible for making mucus and sweat. It causes a thickening of the mucus in your body and increases the salt content of your sweat. These can lead to problems including problems absorbing oxygen, lung infections, inhibit digestive enzymes from reaching your small intestine, dehydration, increased heart rate, lower blood pressure, and infertility. It can also have an effect on reproduction, causing difficulty for women to conceive and infertility in men. People who suffer with cystic fibrosis are also at a higher risk of getting diabetes, liver disease, osteopenia and
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