Cystic Fibrosis: A Day in the Life
Everyday tasks like walking up a set of stairs can be a daunting and debilitating endeavor for individuals with cystic fibrosis. The progressive, lifelong illness causes thick mucus to build up on the inside of critical organs, most notably the lungs. People who live with cystic fibrosis are constantly falling into a cycle of airway obstruction, infection, and inflammation, which all serve to destroy a person’s airway and send their lung functioning into decline.
According to Corbus Pharmaceuticals, an industry leader in medicines for the treatment of cystic fibrosis, it is the most common genetically inherited disorder among Caucasians. Without treatment or early intervention, the disease is able to cause respiratory failure in a child before they reach elementary school, and before the establishment of the Cystic Fibrosis Foundation in the 1950s, this was regularly the case.
Even with all of the advancements in understanding and treating the disease, the median age of survival for individuals with cystic fibrosis is estimated to be 40. Living with the disease is a constant battle to maintain health, and the burden of care on patients is high. Every day, an individual must follow a strict regimen to prevent decline and maintain a baseline of health.
Medicines and therapies
People living with cystic fibrosis will live most of their lives on medications. The number medications can range between 10 and 64 different prescriptions, each one serving to tackle a different angle of the complex and constantly progressing disorder. In addition to the cocktail of solid pills that an individual must take, lifelong therapies that require the use of supplementary devices is common.
Two of the main prescriptions that cystic fibrosis patients are put on are antibiotics and mucus thinners. Infections are both common and incredibly destructive in cystic fibrosis patients. Anything that can help prevent infection, or assist the body in fighting infections is a valuable resource to be adding to the body. Inhaled antibiotics are often taken after other medicines that help to clear and open the airways, such as bronchodilators and mucus thinners.
Mucus thinners are typically inhaled, and make it easier for cystic fibrosis patients to clear out the thick buildup of mucus inside the lungs. The two most common types of mucus thinners are dornase alfa, and hypertonic saline. People with cystic fibrosis do not transport sodium and chloride ions—gained primarily through salt, or saline solutions—into the lungs, which draws water to the lungs and helps to clear out mucus. Taking hypertonic saline solution remedies this, but the effects only last for a short while, and must be repeated once or twice a day.
Dornase alfa requires the use of a nebulizer, one of the most widely utilized devices in cystic fibrosis therapy. A nebulizer is used to administer medications to the lungs by taking a liquid medication and transforming the medication into a breathable mist. Patients typically use the nebulizer at home two or three times a day. They must also clean and disinfect the nebulizer after every use, to avoid the risk of infection.
There are many other medications and therapies available to cystic fibrosis patients, and each additional layer of treatment adds to the overall burden of care that the individual experiences. Some opt into vascular access devices, which allow permanent access to the bloodstream. This is useful if a person knows that they will frequently or regularly receive an IV medication. Most patients will take medications their entire lives.
Navigating daily life
Cystic fibrosis affects everything from a person’s social life, to their financial circumstances. As the number of adults and children with cystic fibrosis continues to rise, the Cystic Fibrosis Foundation and other supporting organizations are working to combat the disease and find a cure. There is currently no cure for the disorder. Researchers are also dealing with a shifting demographic of CF patients, as extended lifespans have led adults to recently outnumber children with the disease.
The emotional wellbeing of a person with cystic fibrosis is an incredibly important part of their overall quality of life. It is hard to watch other people handle everyday tasks so easily, especially when it is so difficult for a person with cystic fibrosis. Part of navigating daily life is finding and doing something that the person loves. Anxiety and depression rates are higher in cystic fibrosis patients than in the average person, and should be treated as part of a patient’s overall health and wellness.
Staying on top of cystic fibrosis is a daily struggle, the first part of which is taking medicines and keeping up on therapies. After this comes maintaining overall health, which is primarily achieved through diet and fitness. In the back of every CF patient’s mind are germs and the risk of infection. Lung infections in particular can cause long-term, severe damage to the lungs, and so CF patients do everything that they can on a daily basis to lower their risk of infection.
Cystic fibrosis patients are put on a high-calorie, high-fat diet that allows their bodies to maximize energy production. In addition to the diet, a person may also opt into vitamin and mineral supplements. Cystic fibrosis often affects the pancreas, which releases enzymes necessary to breaking down and properly digesting foods. Consequently, people with CF must take additional precautions to ensure that their dietary needs are being met.
Individuals with CF can experience the most benefits by performing regular, cardiovascular exercises. Regular exercise has been shown to provide a wide array of health benefits to everyone, and for individuals living with CF, the benefits include slower lung decline and increased respiratory functioning. Other benefits that are key in keeping quality of life high are strengthened bones, lower risk of heart disease, and improved mood.
Continued burden of treatment
One of the hardest parts of having cystic fibrosis is the amount of necessary treatments and therapies that one must go through on a daily basis. It is not unusual for a person’s daily regimen to take up three, four, or even more hours of a person’s day. Even more difficult is the knowledge that none of this treatment will solve the underlying condition, or reverse any of the symptoms. It can be difficult knowing that fighting the disease is a constant battle to stay healthy and maintain your current condition.
As research and new information comes to light, treating the disease is beginning to shift to treating the inflammation that the underlying condition can cause. Traditional anti-inflammatory medications actually exacerbate the condition, leading many pharmaceutical companies to rise to the challenge of addressing this unmet need. Inflammation is one of the factors leading to lung decline, and finding a way to treat it without adding to the burden of treatment could drastically alleviate the burden of treatment for individuals living with cystic fibrosis.
The important thing to remember as a person with cystic fibrosis, or as a person caring for someone with cystic fibrosis, is that the disease is conquerable. It may not yet be curable, but finding a way to pursue the things that you enjoy, and finding a way to enjoy life is well within reach. The disease is no longer the insurmountable obstacle that it once was, and living a fulfilling, satisfying life is a question of finding what you enjoy and pursuing it to the best of your ability, while staying on top of your treatments and meetings with your healthcare team.
References
https://www.cff.org/Life-With-CF/Daily-Life/Fitness-and-Nutrition/Fitness/
https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Medications/
https://www.corbuspharma.com/pipeline/cystic-fibrosis
