Essay on Cystic Fibrosis Awareness

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.

Cystic Fibrosis (CF) is a genetic disorder in which mucus glands produce abnormally thick secretions. These secretions can lead to chronic infections of the lungs and eventually lead to obstruction of the pancreas, resulting in digestive enzyme deficiency, the liver is also sometimes affected. Secretions from the sweat and salivary glands of a CF patient frequently contain abnormally high amounts of sodium and chloride. Because the body produces a high amount of salt, a sweat test is generally used to diagnose the disorder.

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 the most common lethal genetic disease that affects children and young adults. The disease is inherited and mainly affects the lungs and the digestive system. It is estimated that there is one case of cystic fibrosis in the country in every 3,600 children. More than 4,100 children and young adults suffering from cystic fibrosis attend specialized cystic fibrosis clinics (Horsley, Cunningham, & Innes, 2015).

This increases bacteria growth and causes infections (NHLBI, "What Are the Signs and Symptoms of Cystic Fibrosis?", 2013). These infections can clog the airways and damage the lungs making it difficult to breath (NHLBI, "What Are the Signs and Symptoms of Cystic Fibrosis?", 2013). The signs and symptoms of cystic fibrosis according to the Cystic Fibrosis Foundation are very salty tasting skin, persistent cough with sputum, frequent lung infections, wheezing, shortness of breath, poor growth, constipation, greasy,bulky stools(Cystic Fibrosis Foundation [CFF], "About CF: Causes, Signs & Symptoms of Cystic Fibrosis | CF Foundation",

Cystic Fibrosis cannot be developed or contracted it is something you’re born with. CF is a recessive disease, it occurs when a child inherits one defective copy of the gene from each parent. This gene mutation is responsible for cystic fibrosis. ("Cystic fibrosis Canada," 2011) This gene is known as the CFTR gene ("www.medincinet.com," 1996). Reference Figure 3. The “gene makes a protein that controls the movement of salt and water in and out of your body's cells. In people who have cystic fibrosis, the gene makes a protein that doesn't work right. This causes the thick, sticky mucus” ("www.medincinet.com," 1996).

Cystic Fibrosis affects 1 in every 2,500 children at birth. (ncbi.nlm.nih.gov) Cystic Fibrosis, or CF, is a genetic disorder. A genetic disorder is inherited, and is contributed to by both parents. (lung.org) Each parent has one abnormal gene. (lung.org) Cystic Fibrosis is where the body can not make, or it makes an abnormal version of the CFTR protein. CFTR is also known as cystic fibrosis transmembrane regulator. There are several symptoms, and causes of Cystic Fibrosis. These symptoms are diagnosed, according to what causes CF. Cystic Fibrosis is an inherited disease, that when after diagnosed, doctors will suggest treatments to help with the condition. Cystic Fibrosis is most common in white infants, but can

     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.

In the respiratory system, the mucus causes breathing difficulties, frequent respiratory infections and eventually permanent lung damage. The mucus builds up providing bacteria with a place to flourish. The most common infection comes from a bacterium called Pseudomonas aeruginosa. The body's response to P. aeruginosa includes inflammation, which causes episodes of intense breathing problems (Pseudomonas Genome Project, 1998). Normally the body will get rid of excess mucus by coughing before it's a problem but with the thick mucus involved in CF the body has a harder time to get rid of the thicker mucus. Lung disease is the usual cause of death in most patients (National Institutes of Health, 1995). In the digestive system, mucus can block the supply of enzymes used to break down food. The result of the blockage of such enzymes is malnutrition. The patient will have an excessive appetite but will not experience any weight gain. What is also evident is a failure to grow, CF was often misdiagnosed vaguely as "failure to thrive" when physicians didn't know the reason for this poor growth. Sweating is also a problem in people with CF. One of the basic defects in CF is the faulty transport of sodium and chloride (salt). People with CF lose excessive amounts of salt when they sweat. This causes the natural balance of salt in the body to be off balance, which may cause abnormal heart rhythms (National Institutes of Health, 1995).

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

Cystic Fibrosis is a disorder where the exocrine glands secrete abnormally thick mucus, leading to obstruction of the pancreas and chronic infections of the lungs, which usually cause death in childhood or early adulthood. Some mildly affected patients may survive longer. Doctors can diagnose the disease by testing the patients perspiration because people with Cystic Fibrosis have high amounts of salt in their perspiration. Those with respiratory infections are treated with antibiotics, with aerosols that relieve constriction of the airways and liquefy the thick mucus, and by physical therapy to help patients cough up the obstructing secretions. Patients with pancreatic insufficiency can take pancreatic enzymes with meals.

*Specific Purpose: To inform my classmates about cystic fibrosis and how severe it really is.

Cystic Fibrosis, a very serious inherited genetic disease, is also known as CF and sixty-five roses. This disease affects one in every 3,000 live births. It may first appear in a newborn, but can appear all the way up until a young adult. However, ten percent of most cases are apparent at birth. CF affects the lungs and causes a build-up of abnormally thick mucus which leads to chest infections, and CF also affects the reproductive system. Doctors do not know what causes the mucus to thicken. CF’s infections usually lead to death in childhood and early adulthood. Most people infected with CF had a life span into their teens long ago. Now, due to advanced technology, the life span is in the fifties or older.