Cystic fibrosis is a disease that has no cure and affects approximately 30,000 children in the United States. The life expectancy of patients with cystic fibrosis has increased over the past 50 years dramatically. Advancements in medications and therapies have allowed patients a longer and more productive life. Genetic research, pharmaceutical technology, and therapy advancements have been the forefront in increasing patient age. The drug Ivacaftor is helping some patients by treating the missing link in the DNA of a cystic fibrosis patient, it does not just help the symptoms of CF, but actually treats the underlying condition itself. Advancements in bronchial hygiene, such as the chest vest have also greatly impacted the lives of CF patients.
…show more content…
CF is an autosomal recessive gene disorder caused by mutations in a pair of genes located on chromosome 7.1 There are thousands of combinations of gene mutations that can lead to a diagnosis of cystic fibrosis. These are referred to as cystic fibrosis transmembrane conductance regulator (CFTR).1 Cystic fibrosis affects about 30,000 children and adults in the United States, and about 70,000 worldwide per the Cystic Fibrosis Foundation.1 Cystic fibrosis does not just affect the respiratory system, it also encompasses other body systems. These include exocrine gland function, pancreas insufficiency, high chloride concentrations in the sweat, kidneys, liver, and intestines. Cystic fibrosis patients also have abnormalities in sodium and chloride production which aids in the dysfunction of the mucociliary transport …show more content…
Antibiotics are commonly used to treat chronic respiratory tract infections and prevent them. By the time most CF patients reach adulthood, many are colonized with mucoid producing strains of Pseudomonas aeruginosa.5 Numerous patients may also have chronic infections with Staphylococcus aureus. Quarterly culture and sensitivity results can be essential in directing acute antibiotic therapy.5 Antibiotics can be aerosolized (inhaled), taken orally, or given intravenously depending on the severity of the exacerbation and what infecting organism is being combatted. Common antibiotics that are given to cystic fibrosis patients are azithromycin, tobramycin, aztreonam (Cayston), ciprofloxacin, and levofloxacin. Aerosolized antibiotics can help suppress chronic infections, improve lung function, decrease inflammation, and decrease pulmonary secretions.5 Azithromycin is most commonly used to treat Pseudomonas aeruginosa and tobramycin (TOBI) is also used to treat P. aeruginosa.1 The use of long term antibiotics comes at a cost though, bacteria can become resistant to the antibiotics. Thus, the antibiotic treatments are less effective and bacterial overgrowth is an issue. Long term use of antibiotics can also increase the risk for fungal infections in the patient’s
CF is an autosomal recessive gene disorder caused by mutations in a pair of genes located on chromosome 7.1 There are thousands of combinations of gene mutations that can lead to a diagnosis of cystic fibrosis. These are referred to as cystic fibrosis transmembrane conductance regulator (CFTR).1 Cystic fibrosis affects about 30,000 children and adults in the United States, and about 70,000 worldwide per the Cystic Fibrosis Foundation.1 Cystic fibrosis does not just affect the respiratory system, it also encompasses other body systems. These include exocrine gland function, pancreas insufficiency, high chloride concentrations in the sweat, kidneys, liver, and intestines. Cystic fibrosis patients also have abnormalities in sodium and chloride production which aids in the dysfunction of the mucociliary transport …show more content…
Antibiotics are commonly used to treat chronic respiratory tract infections and prevent them. By the time most CF patients reach adulthood, many are colonized with mucoid producing strains of Pseudomonas aeruginosa.5 Numerous patients may also have chronic infections with Staphylococcus aureus. Quarterly culture and sensitivity results can be essential in directing acute antibiotic therapy.5 Antibiotics can be aerosolized (inhaled), taken orally, or given intravenously depending on the severity of the exacerbation and what infecting organism is being combatted. Common antibiotics that are given to cystic fibrosis patients are azithromycin, tobramycin, aztreonam (Cayston), ciprofloxacin, and levofloxacin. Aerosolized antibiotics can help suppress chronic infections, improve lung function, decrease inflammation, and decrease pulmonary secretions.5 Azithromycin is most commonly used to treat Pseudomonas aeruginosa and tobramycin (TOBI) is also used to treat P. aeruginosa.1 The use of long term antibiotics comes at a cost though, bacteria can become resistant to the antibiotics. Thus, the antibiotic treatments are less effective and bacterial overgrowth is an issue. Long term use of antibiotics can also increase the risk for fungal infections in the patient’s