Bronchodilator Pulmonary Function

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.

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.

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.

Beta2 agonists act directly on bronchial smooth muscle to cause bronchodilation. They are the most widely used bronchodilators for COPD. Short-acting beta2 agonists are the most commonly used short-acting bronchodilators in COPD.

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 (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).

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

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.

Chronic obstructive pulmonary disease (COPD) is a progressive disease that affects more than 200 million people annually worldwide. It produces significant economic burden in both direct and indirect healthcare costs in addition to causing substantial morbidity and mortality. Distinguishing factors of this disease include progressive dyspnea, cough and sputum production, and breathlessness. This is primarily due to hyperinflation of the lungs, which serves as the chief pathologic mechanism of COPD. The care of patients with COPD has radically over the past 20 years, and several novel therapies have been discovered that have proven to significantly improve the health status of patients. In the case of moderate to severe COPD, exacerbations or persistent symptoms, regular treatment with long-acting bronchodilators is recommended to control symptoms, reduce the occurrence of acute exacerbations, and improve quality of life.

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

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

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 (CF) is an autosomal recessive gene that causes a wide range of symptoms because there are over 1,000 changes or mutations that can occur within the cystic fibrosis transmembrane receptor (CFTR) protein. The CFTR protein is generally a chloride ion chain “regulated by cyclic adenosine monophosphate and therefore can act as a regulator of other electrolyte channels”(Grossman, S., & Grossman, L. 2005, p. 46). Typically this protein allows chloride ions to exit mucus-producing cells allowing water to flow in and thin the mucus. However, if the CFTR protein has been mutated, such as in cystic fibrosis, chloride ions cannot exit. This causes the mucus to thicken, become sticky, and obstruct the various channels it passes through. This build up of mucus also prevents bacteria from being cleaned from cells thoroughly increasing the patients risk for infections (Grossman, S., & Grossman, L. 2005). However, the severity of CF depends on whether the patients have complete or partial loss of the CFTR gene. If the person has the classic form of CF abnormalities of CFTR will commonly affect “…the respiratory, gastrointestinal, endocrine and metabolic, and genitourinary systems”(Schram, C. 2012). However, if people have atypical forms of CF their genetic disorder may only affect one of the organ systems and may not be found until the patient develops symptoms in their late childhood, early adolescence, or adulthood

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