Summary
Cystic fibrosis [CF] is a chronic disease in which preventative treatment with nebulised antibiotics can reduce pulmonary exacerbations that otherwise require rescue therapy. However, adherence is low. Making adherence to maintenance treatment visible is a crucial step towards improving adherence. In this article, we discuss how adherence data can be used to support Quality
Improvement in CF through behaviour change in both people with cystic fibrosis and their clinical teams. Keywords: Adherence, Cystic Fibrosis, Telemedicine
The problem with treatment adherence in long-term conditions
Adherence to medication among patients with long-term conditions is a major problem. An estimated 30-50% of medicines are not taken as recommended
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Although the discrepancy between self-report and objective measurement is often interpreted as evidence of patient’s attempting to deceive clinicians on many occasions it will simply reflect difficulties in recall. Self-monitoring and feedback provide information to increase awareness of the target behaviour and are therefore important for good adherence.[6] With advances in technology, more self-monitoring gadgets are commercially available and self-monitoring is now increasingly popular. An example is the Quantified Self movement whereby interested users collaborate with tool makers to develop products for self-monitoring.[7]
The CF community is particularly well placed to make adherence visible using the principles of self-monitoring because of the close contact between people with CF (PWCF) and their CF centres – unlike other long-term conditions which are predominantly managed by general practitioners in the community, healthcare for PWCF are delivered almost exclusively via specialist
CF centres.[8] CF registries also exist at national and international level to coordinate CF-related data and help drive improvement.
Quality improvement and the impact of adherence visibility
Understanding the quality of care delivered by CF units is crucial. If we can measure the quality provided by the best units, we can understand what is possible. If we can build structures that routinely measure quality in all units, we
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
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.
Patient Adherence: There is no known cure for chronic disease, the progressive nature of chronic disease the patient and family must adjust to continual treatment changes, and the chronic disease continues throughout the patient’s lifetime developmental and lifestyle changes often influence or pose additional challenges to the person with a chronic
Cystic Fibrosis, also known as CF, is one of the most common life-threatening genetic disorders found in people. Discovered in 1938 by Dr. Dorothy Hansine Andersen, Cystic Fibrosis severely affects two parts of the human body, including the respiratory system and the digestive system. Cystic Fibrosis causes the mucus glands to secrete very thick and sticky mucus. This mucus then clogs the tiny air passages and traps bacteria in the lungs. The thick mucus also stops the release of digestive enzymes in the pancreas from reaching the small intestine. According to Cystic Fibrosis Queensland (2015) more than one million Australians carry the Cystic Fibrosis Gene, with one in every 25 people, often not realising they are carrying the deadly gene.
The Cystic Fibrosis Foundation provides support to help improve the lives of people who live with the disease. The foundation’s main goal is to find a cure to Cystic Fibrosis is an inherited disease caused by changed in a gene on chromosome seven. It is described as a buildup of mucus in the lungs and organs. The mucus in the lungs clogs the airways and bacteria grows, gets blocked in and eventually leads to infections that causes lung damage. After lung damage has occurred, respiratory failure then happens.
While Andrea still loves the annual walk and “seeing the kids running to the finish line,” learning more about cystic fibrosis made the event meaningful, especially when they learned that a cure might be found within their lifetime.
The Main treatment for someone with lung problems with CF is chest physical therapy, which is also called chest clapping or percussion. CPT is done by pounding your chest and back repeatedly with your hands or device to loosen the mucus from your lungs so that you can cough it up. Exercise is also a choice of a treatment plan, exercised that make you breathe harder can help loosen the mucus in your airways so you can cough it up. Medication can also be used to treat Cystic Fibrosis, your doctor may prescribe antibiotics, anti-inflammatory medicines, bronchodilators, or other medication that can help clear the mucus buildup in the lungs(“Cystic
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.
NIH-03: Advisory and Management Support for the P5 (Opportunity) – Anne, Don, Marni, Natasha and Ryan
Like all of us, those with cystic fibrosis want to live. To experience. Gold speaks of the looming mortality that all cystic fibrosis clients are living with every day. We are all mortal, although those with this disease just know that they are more likely to live a shorter life and they want to live it to the fullest (Picard, 2000). Personal knowledge allows a nurse to better understand a client’s needs and the things we may want to do when we ourselves are a little older, the adolescent with Cystic fibrosis may want to do earlier, before they no longer have that
Cystic Fibrosis, a very serious inherited genetic disease, is also known as CF and sixty-five roses. This disease affects one in every 3,000 live births. It may first appear in a newborn, but can appear all the way up until a young adult. However, ten percent of most cases are apparent at birth. CF affects the lungs and causes a build-up of abnormally thick mucus which leads to chest infections, and CF also affects the reproductive system. Doctors do not know what causes the mucus to thicken. CF’s infections usually lead to death in childhood and early adulthood. Most people infected with CF had a life span into their teens long ago. Now, due to advanced technology, the life span is in the fifties or older.
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 (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
Quality indicators enable the health care system to identify inferior care in both process or outcome and structure while enhancing quality improvement in health care (De Vos et. al, 2009, p.1).