Cystic Fibrosis – The diagnosis, treatment and preventive measures

Sixty–five Roses 

Imagine you’re a child, not even five years old, and you’re weak and having difficulty breathing, so you’re rushed to the doctor only to find out that you are sick. You don’t look sick. On the outside, you seem fine and normal like everyone else. Only your doctor and your parents tell you that you are not well, and they even have a name for your illness. For years, you call it “sixty-five roses,” because that makes more sense to the mind of a child. It sounds pleasant, but the name doesn’t match how you feel, nor does it match the countless doctors’ visits, the daily therapies, or the operations you may have to endure. Later in life, you find out that your illness is not as pleasant as the name you’ve been calling it. In reality, you have a rare genetic disorder known as cystic fibrosis–an illness that has been wreaking havoc on your respiratory system since you were a child.

Cystic fibrosis or CF affects about 70,000 people worldwide. Unfortunately, there is no way to prevent the illness, nor is there a cure for it. Brought on by a recessive gene in both parents, it is rare for anyone to develop this illness, but when it does occur, it can be devastating, as cystic fibrosis can lead to respiratory problems that cause lifelong breathing difficulty and eventually lead to death.

What is CF? The cells of the lungs, nose, and stomach all process an electrolyte called chloride. When the process is efficient, chloride helps to regulate the amount of fluid in the body. This is important because our tissues and cells must remain hydrated in order to function properly. In people with cystic fibrosis, the cells don’t process chloride efficiently, which means that the organs of the body are not properly hydrated. This leads to organ malfunction, which can require multiple visits to doctors, excessive treatments, and even surgeries. Our bodies are over 70 percent water. Without proper hydration, system malfunction is imminent. The primary symptom of cystic fibrosis is that the cells are blocked from hydrating the body.

Why cystic fibrosis is so dangerous is due to how the illness affects the lungs. The lungs are naturally coated with mucus, but they also must stay hydrated. There is a fine balance that must be maintained, and when chloride is blocked, the lungs become overflowed with mucus. Not only can this make it difficult to breathe, but it also creates perfect conditions for the proliferation of bacteria. People with cystic fibrosis are at major risk for respiratory infections, making having the illness extremely dangerous.

Cystic fibrosis patients usually have to receive regular treatments to clean out as well as hydrate their lungs. Bronchodilators are used to open the airways, and a sodium chloride solution has to be pumped into the lungs to keep them hydrated. A special vest is often used to loosen mucus within the lungs so it can be discharged. These procedures may have to occur several times a day; otherwise, the battle between bacteria and the body’s own defenses can lead to scar tissue within the lungs, thus worsening the problem.

Scientists are currently working on new treatments for cystic fibrosis. Therapies to allow the cells to release chloride show promise. Other treatments revolve around helping the immune system more effectively eliminate bacteria within the lungs or starving bacteria by preventing them from uptaking nutrients. While all of these therapies seem promising, they are all still in their infancy, and most patients with cystic fibrosis continue to rely on invasive therapies that target symptoms rather than possible cures.

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