Current Treatments For Cystic Fibrosis

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

Progressive lung disease is the predominant cause of illness and death in people with CF. Mucus blocks the airway passages and results in a predisposition toward chronic bacterial infections. Although the genetic defect underlying CF has been characterized, exactly how and why individuals become infected with Pseudomonas is unknown. The lungs of most children with CF become colonized by Pseudomonas aeruginosa before their 10th birthday. Chronic infection with these bacteria reduces an individuals quality of life, causing acute symptoms of cough, sputum production, and inflammation, which causes repeated exacerbations or episodes of intense breathing problems. Eventually leading to scarring and destruction of lung tissue and, ultimately, death. While it is clear that antibiotic therapy directed against these organisms lengthens the life span of individuals with CF, increasing antibiotic resistance develops. Although antibiotics can decrease the frequency and duration of these attacks, the bacterium establishes a permanent residence and can never be completely

Early infection with P. aeruginosa resembles acute non-CF infections within the airways, however the bacterium can evolve genotypically and phenotypically during infection, and adapt to its human host1. Adaptive mutations resulting in loss of motility and acquired antibiotic resistance support a sessile lifestyle that is associated with persistent infection within the CF lungs9, 12. Chronic infection is characterized by immunity-mediated inflammation from increased recruitment of polymorphonuclear leukocytes to the lungs2. Furthermore, P. aeruginosa utilizes an assortment of virulence factors such as proteases, exotoxin A, and phospholipases to destroy host tissue, by which the symptoms of CF patients are

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.

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

Many years ago people were diagnosed with Cystic Fibrosis but there was no cure. Children were dying from this disease at very young ages. The Cystic Fibrosis Foundation was then formed in 1955 by mothers who were determined to find a cure for their dying children. There is still no cure but there are treatments that help with the infections that grow in the lungs of people with CF. There are people still researching for a way to cure Cystic Fibrosis, but what is the Cystic Fibrosis Foundation all about?

Contact with germs is a concern for people with cystic fibrosis. Sticky mucus build up in the lung allow these germs to thrive and multiple (About Cystic Fibrosis, 2013). Pseudomonas aeruginosa is a gram-negative bacteria very commonly carried around by healthy individuals without any symptoms. But for people who are ill and have weak immune systems, this bacteria can cause a deadly infection. Infection is hard to treat because Pseudomonas aeruginosa can resist many antibiotics. Pseudomonas aeruginosa is spread easily in hospitals by health care professionals and uncleansed medical equipment. This serious infection can cause pneumonia in the lungs and cause septic shock if released into the blood stream (CDC 2014). Therefore, the exposure to

Five year old Ellis Miles undergoes numerous treatments each day to counteract the symptoms that accompany cystic fibrosis including physiotherapy, pills containing digestive enzymes, and a treatment which breaks up the mucus in her lungs. Cystic Fibrosis has numerous life threatening symptoms and while there is no cure, there are several treatments that a typical patient undergoes each day to combat these symptoms. The most prominent symptom associated with Cystic Fibrosis is a thick mucus that clogs up tubes that carry out crucial roles in the human body. This mucus especially affects the respiratory tract. Mucus builds up in the respiratory tract making it difficult for individuals with cystic fibrosis to have a frequent cough that produces a thick

I couldn’t imagine what it would be like growing up with a life threating condition that has no cure. Amanda Estep is a 21-year-old, college student and also a close friend of mine who has been battling Cystic Fibrosis since she was three years old. Cystic Fibrosis is a genetic condition that causes mucus to build up in the lungs and digestive system, making it hard to breath. “Basically everything in my body is thicker, so the mucus in my lungs is harder to break up and get out which causes frequent lung infections.” Amanda explained to me. Cystic Fibrosis also impacts her ability to digest food, resulting in her being underweight most of her life. While I have known Amanda since kindergarten, we have never talked in depth about how much she has to do to remain as healthy as possible. Hearing her explain her story made me realize how lucky I am to be healthy, and that’s something’s no one should take for granted. There is currently no cure for Cystic Fibrosis, but Amanda shared some of the many things she has to do in order to remain as healthy as possible. Some of those things includes being hospitalized for two weeks four or five times a year, taking many medications, and knowing how hard she can push herself.

Cystic Fibrosis (CF) is a common genetic disorder that affects the exocrine gland in the lungs, liver, pancreas, and intestines causing progressive disability due to multisystem failure. It is also known as “mucoviscidosis because of the mucus that builds up and blocks the respiratory system and pancreas”(Bedwell). Cystic Fibrosis affects the cells that produce mucus, sweat, and digestive juices. It causes these fluids to become thick and sticky. With them becoming so thick and sticky they can plug up tubes, duct, and passageways. Unfortunately, there is no real cure for Cystic Fibrosis. “Although, 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. New developments in prevention of exacerbations, therapy drugs and methods to preserve

Cystic fibrosis is an inherited disorder that causes severe damage to the lungs and digestive system. This disorder is among over 70,000 people worldwide, and about 30,000 just in the United States alone. Nearly half of the Cystic Fibrosis population is age 18 or older, and are normally diagnosed by age 2. Though Cystic fibrosis is a complex disease and the types and severity of symptoms can differ widely from person to person. Many different factors, such as age of diagnosis, can affect an individual's health and the course of the disease. Today the median survival age for having this disorder is close to 40 years old. To most that might seem very young, but this is a huge improvement from what it was in past years. New advances in technology makes it possible for people with this disorder to

The main goal of any treatment for someone who has cystic fibrosis is to prevent and/or control lung infections, loosen and remove the mucus build up in the lungs, prevent and treat intestinal blockage and ensuring proper and adequate nutrition. If the treatment includes medications, antibiotics would be used to treat and/or prevent lung infections. To help get the mucus up and out of the lungs, mucus-thinning drugs will be used to improve lung function. By relaxing the muscles around the bronchial tubes, bronchodilators can help keep the airway open. Lastly are oral pancreas enzymes which help the digestive tract absorb nutrients. Non-medication treatments include chest physical therapy which helps the thick mucus loosen and in turn makes it easier to cough up. There is also something available called pulmonary rehabilitation which is something that can help improve lung function in an outpatient setting and this may include exercise training, nutritional education, breathing techniques to conserve energy and counseling and/or support groups. There are also several surgical procedures that can improve quality of life for patients with cystic fibrosis. These in removal of any nasal polys to help breathing, oxygen therapy to help prevent pulmonary hypertension or high blood pressure in the lungs, endoscopy and lavage treatments to

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