The ins and outs of cystic fibrosis

Here is a reasonably common childhood disorder which you may have encountered before. It affects around 105 000 people in the world today.

Cystic Fibrosis. This disorder sometimes known as CF is a genetic disorder which is progressive. It affects the lungs, pancreas and other key organs in the body and it does become worse over time.

What is Cystic Fibrosis?

Individuals with CF have an issue with producing a certain protein known as CFTR protein. The CFTR protein is responsible for taking chloride to the cell surface. Chloride is a component that is present in salt and it has a vital role in the cells of our body. It comes up to the cell surface to attract water to enter into the cell. Without it being present, the body can build up mucus within its organs which can affect functionality.

The main organs affected are the lungs, pancreas, and liver. The lungs fill with mucus, making it difficult to breath and increasing your likelihood of getting chest infections and pneumonia. The pancreas fills with mucus which prevents the release of key enzymes that help your body absorb nutrients. The liver and other organs can also build up mucus and stop functioning properly.

The biggest thing that individuals with CF need to monitor is their lung function. The most common cause of death is respiratory failure, so breathing is not taken lightly at all. The second thing which is quite important is nutrition and this is due to the impact CF has on the absorption of food. So what does this have to do with physiotherapy?

Treatment of Cystic Fibrosis:

Physios actually have a really key role in the treatment of cystic fibrosis. One of the things that we are really good at is lung health and building respiratory capacity. There is a test known as the Pulmonary Function test. This indicates what your current lung capacity is and the medical team use this to help individuals with CF track how their disease is progressing. The main treatment technique is to work on clearing the mucus (sputum) from the lungs and increasing the size and function of the alveoli (lung cells) to increase lung capacity and fitness. These are both treatment techniques that we do as physiotherapists.

The vital technique that is implemented is Airway Clearance Techniques. This involves shifting the mucus from the corners of your lungs and drawing it to the carina which is the joining point between your two lungs so the mucus can be coughed up. This helps clear additional mucus from your lungs and enable more of your lung cells to absorb oxygen.

The second vital technique is working on respiratory fitness which can involve general fitness techniques, in the form of building strength, increasing cardiorespiratory fitness, increasing endurance. This involves a tailored exercise program that pushes your lungs to increase their capacity whilst also being manageable.

These are the main two areas that physios are involved in. However, once your disease progresses and more mucus gathers within the lungs. Physiotherapy can help use more intense techniques to shift the mucus to that central point. This can be through coughing, huffing, and combinations of different breathing to move mucus. Physiotherapists can also use manual techniques such as percussion, vibration, in combination with positioning to loosen the mucus droplets from the lung tissue and help move it out of the lungs. There are other techniques such as bubbles and Positive expiration Pressure (PEP) therapy that are instigated when required. The main goal is to shift the mucus out of the lungs and into the correct position to be coughed up.

This is why a physiotherapist is a key role in the team managing your CF. We play a vital role in keeping the lungs healthy and slowing the deterioration of respiratory function. We also look at the broader picture by helping the whole body stay strong and active to help build and maintain cardiorespiratory fitness.