Cystic fibrosis (CF) is a common genetic disease that is inherited from your biological parents and occurs because of abnormalities in our genes. The CF gene is the instructions on how to build a channel (or pore) in our cells that allows salt to move into and out of the cell. This salt channel is called CFTR, the cystic fibrosis transmembrane regulator. A mutation in this gene means that this salt channel does not work properly.
The lungs are the most commonly affected organ in patients with cystic fibrosis. As a result, patients with CF can have a chronic cough, phlegm production, shortness of breath, chest tightness, sinus problems and occasionally cough up blood.
A standard treatment regimen includes:
- airway clearance and exercise,
- chest physiotherapy,
- anti-inflammatory agents,
- supplemental oxygen, and
- nutritional support.
Nearly half of CF sufferers report poor sleep quality. Your oxygen levels may lower during an exacerbation and this is most noticeable during sleep. In some people these changes in breathing and oxygen during sleep can affect their performance during the day. They may have difficulties with concentration, memory and feeling tired during the day. People who have low oxygen levels while awake will usually also have low oxygen levels during sleep. In some cases, patients who may not require oxygen while awake may need extra oxygen while sleeping.
Individuals with CF can have low levels of oxygen in their bodies and some need to use supplemental oxygen to bring their oxygen levels up to a healthier level. Supplemental oxygen protects the body from the effects of low oxygen levels. It also helps your body to function better and allows you to stay more active. Some individuals only need oxygen when they are active or while sleeping, however, in most cases, oxygen should be used 24 hours a day.