Saving young lungs - Dan’s story

Dan was diagnosed with Cystic Fibrosis (CF) at 18 months old and spent a lot of his childhood in hospital. He received home tuition, as it was deemed too risky during winter months for him to be at school. He always felt different to everybody else, which was difficult growing up. Luckily, he was able to attend secondary school full time, but it was still really hard. Dan and his family were always cautious of what the next issue might be. 

Nowadays, people with CF are recommended not to meet face to face due to cross infection risks, but as a child, Dan spent a lot of time on open CF wards with other people with CF at the Sheffield Children’s Hospital. 

After university, Dan had to be hospitalised again after coughing up some blood. This was a terrifying experience, and living with a condition like CF, there is always that lingering element that things could get bad, very quickly.

There are numerous treatments that people with CF need to take. Many of these treatments have stayed broadly the same, but they’ve evolved to be more efficient, and the type of medication has changed. These include Physiotherapy, which helps with the drainage and clearing the mucus. People who have CF often have to have IV antibiotics. Repeat IVs can damage your veins and so those who need regular IV antibiotics will often have a device put into their chest to make the process easier. Modulator therapy has been the biggest change to treatments in recent years. Modulator therapy targets the CFTR protein. The faulty CFTR protein in CF leads to the buildup of sticky mucus in the lungs and other organs, causing infection and damage. Modulator therapy makes the mucus thinner and less sticky. 

Although the modulator therapy has been transformative, it is not a cure, and not everybody has access to the modulators. About 5% of people with CF are ineligible for this therapy due to genotype, and others are unable to use it due to side effects. 

In the UK, modulator therapy is available on the NHS, so anyone in the age range or genotype can access this. However, not all countries have the same levels of access. 

With people with CF now living longer, we’re starting to see new issues emerging.This is why MRI lung imaging developed by Sheffield is so essential. It will mean we can better assess how the lungs are reacting to new treatments in the long-term, keeping people with CF as healthy as possible. 

There are lots of misconceptions around CF. For example it’s a multi-system condition - not just a lung disease. CF affects many areas of the body. Almost all men with CF are infertile, so having children can be really difficult. Dan had his first child through IVF, and then adopted his second, but this can be a very difficult process. It’s just another thing for people with CF to have to think about. 

How is MRI helping?

MRI enables doctors to spot the more nuanced changes we are seeing in the health of people’s health. The historical lung function test used in CF can often say that lung function is stable, but doesn’t identify the smaller changes that can be going on - MRI changes this. 

Anecdotally, following modulator therapy some CF patients feel less attuned to when something is going wrong with their health because the usual triggers that they’ve been familiar with in the past now aren’t happening due to alleviated symptoms. This means that the detail from the MRI scans is now really important. It gives insight into what’s actually happening in peoples' bodies, allowing doctors to catch when an issue is arising. 

MRI helps with early intervention as it can spot problems earlier than other tests. It’s a multi-organ technology that can tell us so much about so many different parts of the body. 

MRI also doesn’t use radiation. With a long term condition like CF, people may have to have repeat scans. With MRI, people with CF wouldn’t be exposed to repeat radiation. Dan would like to see MRI used more frequently in CF care, for instance as part of the CF annual review, a detailed assessment people with CF go through on a yearly basis.

Dan is so grateful for the fundraising efforts and community support:

“I think it's incredible. People giving of their time and energy and their money for good causes is a really incredible thing to do and I’m very appreciative of everyone who's getting involved in the Big Walk. I've been very grateful for all the developments that I've seen in my lifetime in CF and undoubtedly public donations have been an enormous part of that. The Big Walk is another really good example of people doing that.” 

Dan Beever, Research Fellow on the MAGNIFY project and person with CF.