Bronchodilator Pulmonary Function

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

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]

With CF people lack the special enzyme to break up mucous. Without the mucous breaking up the person cannot breathe because the lungs are filled. In life the body cannot function with enough oxygen.

The study began with 32 patients having stages II to IV COPD. They had to meet the criteria pertaining to the Global Initiative for Chronic Obstructive Pulmonary Disease; total lung capacity >120%, (FEV1/FVC) <70%, FEV1 <80%, RV/TLC >140% and >40% of predicted values in stable conditions. Patients were removed from the study if they had asthma, heart failure, orthopedic impairments of the shoulder girdle, recent surgery, past thoracic fractures, pneumothorax, and claustrophobia.

Cystic fibrosis is known to be one of the most common and deadly diseases in Caucasians, affecting 1 in 2500 children. This percentage results in 30,000 individuals within the United States to be diagnosed with CF. There are over 1900 mutations of this gene that cause a wide variety of severities within this disease. (McCance, Huether, Brashers, & Rote, 2010) Due to its complex mutation and unknown cause, only treating the symptoms of CF have been the main treatment protocol to this disease. Current treatments are cumbersome and expensive providing patients with life expectancy only into their twenties, but usually younger in most cases. There has been specific progress towards a cure involving gene therapy providing hope for a cure to

Discuss the roles of medications, ICS for long term anti-inflammatory effect and Beta adrenergic for immediate bronchial dilation.

Every day, people die from Cystic Fibrosis. That is why Cystic Fibrosis is known as the most deadly inherited disease. There is no real cure of CF but, right now there are many ways to ease your symptoms and help you manage the condition. “Every day, people with CF complete a combination of the following therapies Airway clearance, Inhaled medicines, Pancreatic enzyme supplement and sometime oxygen therapy depending on how severe your condition is”(McLoud). Since there is so many new advancements in treatment, people who have CF now live twice as long as they did 30 years ago.

Target population is the general public, anyone suffering from this condition, or for someone that know of a relative or a friend who is suffering from CF.

According to the American Journal of respiratory and Critical Care Medicine, predicting the disease progression of CF is not as easy or a one risk factor

Cystic fibrosis (CF) is an inherited autosomal recessive disorder that affects the lungs and digestive system most often. In the United States some 30,000 children and adults have CF. There are approximately 1,000 new cases of cystic fibrosis diagnosed each year in the US with 70% of patients diagnosed with CF by the age of two, 40% of patients with CF are 18 or older. In the 1950's most children with CF did not survive to attend elementary school, but in 2006 the median age of survival was 37 years (Cystic Fibrosis Foundation, 2007).

A variety of clinical studies on the G551D mutation have shown a wide range of positive effects from oral dosage of the drug testing a variety of age ranges. Ivacaftor showed improvements in forced expiratory volume in 1 second (FEV1) for CFTR patients after treatment with ivacaftor for 48 weeks ranging from 8.7% median change from baseline in adults (18 years and above) to 12.5% change in children aged 6-11 (P<0.001). Median sweat chloride levels also showed significant decrease in all age groups above 6 years with results ranging from -49.1mmol/L to -59.5mmol/L (P<0.001) (Accurso et al., 2010) (Ramsey et al., 2011) (Davies et al., 2013). Fewer patients on Ivacaftor treatment experienced a pulmonary exacerbation (PEx), however, on those patients who did experience a PEx despite ivacaftor treatment, it did not improve their rate of lung function recovery after the PEx as compared to placebo (Flume et al., 2017). Additionally, patients showed improve weight and BMI following ivacaftor dosage as compared to those on placebo with results showing between 2.7 to 2.8 kg weight gain of patients 6 years and above (Ramsey et al., 2011) (Davies et al., 2013). Interestingly, a new study investigated whether ivacaftor also improved patient outcomes

Cystic Fibrosis, otherwise known as CF, is an inherited genetic disease that affects the lungs and digestive system. The disease causes a build-up of thick mucus in the lungs and other organs. Mucus clogs the airways in the lungs and “traps bacteria, leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.” (#1)

Doctors can then confirm the diagnosis by using what is called a “sweat test.” A sweat test measures the amount of salt that is in the sweat. Doctors will trigger the sweat on a small patch of skin on the arm or leg (MFMER, 2016). Other tests consist of a chest x-ray, lung function tests, or a spit test. There is many treatments that a doctor can do to help lessen the symptoms of CF, but at this point there is no treatment that can cure you from CF. The sooner you catch these symptoms and try to counteract them with treatments the better off you are. Newborn screening helps with early diagnosis. There’s medications such as antibiotics that treat and prevent lung infections. Doctors also use anti inflammatory medications to lessen swellness for airways to the lungs. Patients diagnosed with CF can inhale medications such as mucus thinning drugs to keep their muscles loose. (Diagnosed, 2016). Lastly, there is chest physical therapy, vest therapy, surgery, and other procedures. Doctors are continuing to try to find a cure to CF, but as of right now there is none. New data, research, and technology is helping reduce the amount of people devastated by this

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

Since the cloning of ABCC1 in the early 90s [14,15], progress has been made towards establishing a functional relationship between ABCC1 and CFTR. For example, functional complementation of dysfunctional CFTR by ABCC1 following chemotherapy that resulted in increased expression of ABCC1, was associated with improved lung function in a CF patient [12]. A study analyzing nasal epithelial cells in CF patients also showed that low ABCC1 transcript levels were associated with more