Summary: Cystic Fibrosis Noncompliance

Hello, today I will be explaining a brief description of my I.S.P topic on Cystic Fibrosis also known (CF). What I will be talking about is what Cystic Fibrosis is, the causes of Cystic Fibrosis, the Symptoms of CF, Diagnosing CF, when to see a doctor, The Cystic Fibrosis Foundation, where does Cystic Fibrosis affect, risk factors, respiratory signs and symptoms, digestive signs and symptoms, respiratory system complications, digestive system complications, reproductive system complications, and lastly prevention as how to prevent. So first I will talk about what Cystic Fibrosis is. {Click}

Nearly 1 in every 30 Americans are a carrier of the recessive gene that leads to CF (About Cystic Fibrosis, n.d.). Although CF is a life-threatening condition, through the advancement of treatments and care, there has been a steady increase in life expectancy and improvement in the quality of life. The current mean age of survival is now 40. Although that does not seem very high, sixty years ago, a child diagnosed with CF did not survive childhood (About Cystic Fibrosis, n.d.). CF is a complex disease, where the types and severity of symptoms can differ widely from person to person. Symptoms may include fatigue, salty-tasting skin, persistent cough with phlegm, wheezing and shortness of breath, lung infections, and poor growth and weight loss (Cystic Fibrosis Symptoms, Causes & Risk Factors, 2018). Many different factors, such as the age of diagnosis, can affect an individual's health and the course of the

They are also accompanied by many medicines and other treatments to help them with the comfort of cystic fibrosis.

Brand PL, van der Ent CK. The Practical Application and Interpretation of Simple Lung Function Tests in Cystic Fibrosis. J R Soc Med. 1999;92 (Suppl 37):2–12. [PMC free article] [PubMed]

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.

There is no cure for cystic fibrosis, but treatment can ease symptoms and reduce complications. Close monitoring and early, aggressive intervention is recommended. Medications can be used such as antibiotics to treat and prevent lung infections, bronchodilators to help keep airways open, mucus-thinning drugs to help loosen the mucus so the patient can cough it out, and oral pancreatic enzymes to help the digestive tract. Chest PT helps to loosen the mucus.

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,

A person with Cystic Fibrosis is affected by these symptoms on a daily basis, making their life harder. These symptoms force people with Cystic Fibrosis to live with a daily routine, where they are required to have one or two nebulisers, every morning and night with physio sessions in between. According to Cystic Fibrosis Australia (2015), they are also required to take up to 40 tablets a day, consisting of enzymes each time they eat. They also have keep their lungs and body active with exercise and a healthy diet, making sure they eat nutrients and healthy fats to maintain a healthy body weight and avoid

     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.

Abstract: The main goal of this paper is to explain what Cystic Fibrosis is and also to explain what

The average life span of a person with Cystic Fibrosis is 25-30 years of age. Although the more traditional treatments of this disease are adequate, is there something else that could be even better? Gene therapy is fast becoming one of the more studied aspects of genetics today. Let's take a look at some details of Cystic Fibrosis and gene therapy.

Armoni, S., Rony, R., & Kerem, E. (2009). Quality of care and quality of life: Patient/healthcare perspectives. Journal Cystic Fibrosis, 8(2), 99. doi: 10.1016/S1569-1993(09)60383-0

Here are some other changes affecting CF patients: Crackles, non-productive or productive cough, physical changes to the chest cavity, cyanosis, and clubbing of the fingernails. Genetic testing should be done since cystic fibrosis is an inherited disorder. Those affected with cystic fibrosis have parents “who do not have cystic fibrosis but are heterozygotes or carriers of the disease. A heterozygote has 1 dominant allele and 1 recessive allele.” (Grossman & Grossman, 2005, p. 46) My co-worker and her spouse are heterozygotes, and they have a 50/50 chance of having a cystic fibrosis child, they were not lucky with the odds and both their sons are affected with cystic fibrosis. These

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