Cystic Fibrosis Brought Them Together but Keeps Them Apart

This is a story about a sister and a brother and how their cystic fibrosis (CF) keeps them apart in certain areas of their lives. It is also a story of exclusion on a bigger scale for the CF population as a whole.

There is a community exclusion issue for those with CF. As a whole, the CF community is prevented from having face-to-face interaction with others having CF.

In 2013, the Cystic Fibrosis Foundation (CFF) formulated a policy that people with CF are not to mingle in non-medical settings due to concerns of cross-infections with certain bacteria peculiar to the cystic fibrosis population (Point Counterpoint Editorials/CHEST Journal).

The siblings in this story currently suffer from cystic fibrosis and live together in their family home in Great Britain. Their names are Faye and Alfie. The parents got the children tested because the congestion in the lungs was interfering with feeding and sleeping.

The main two focuses of the parents is to manage the mucus buildup in the lungs and to improve the quality of life of their children. Due to lung congestion, both the kids sleep poorly and with physical exertion become very tired. To prevent the sharing of lung bacteria, the parents closely watch both the children. To loosen and remove the mucus form the lungs, they do physiotherapy exercises daily. But they do it in separate rooms.

The family is fundraising for an airway clearance vest machine. This machine can help to remove the mucus. Despite their challenges, the kids live a relatively normal life. They play and enjoy their time with each other. Faye now goes to primary school and Alfie goes to nursery.

Standard CF care consists of the following:

• Standard precaution: People with CF must be separated from others with CF, and those who are sick, at all times.
• Transmission based precautions: In a hospital treatment setting, people with CF should wear a mask.
• Hand hygiene among medical staff: People around those with CF should clean their hands. They should use alcohol-based hand rub or they can use soap with water.
• Care of respiratory equipment: There should be audits for multi-use equipment and cleaning of surfaces in medical settings.

Over time, the lifespan of those with CF has increased. From 10 years in 1962, the predicted median survival has become 37 years in 2017. The discovery of new medications, antibiotics, and mechanical physical therapy has helped. For people with CF whose lungs have been damaged, lung transplantation can be a viable option and that, too, has advanced over the last several decades.

Read on to learn more about Faye and Alfie.

Photo credit: PA Real Life