Bronchiectasis is one of the most neglected chronic lung diseases despite the fact it impacts 300,000 people in the UK
When Fiona McDonald returned from a holiday in Peru six years ago with a persistent cough the last thing she expected was for it to remain for the next four years.
“I couldn’t really shift it at all,” the 27-year-old medic from Sheffield told The i Paper. “I had a few different treatments, and was referred to a respiratory clinic.”
After some support from her medical team, the cough seemed to clear. Having lived with asthma since she was a child, her condition was put down to that.
McDonald was told she was too well and too young to have anything more serious. When she began experiencing frequent bouts of infection, she was still told it was down to “poorly controlled asthma”.
A respiratory consultant said the results of a CT scan showed signs of bronchiectasis – a condition where the airways in your lungs are damaged and produce a lot of mucus – but a radiologist assessed the scan as “normal”.
‘The symptoms came back within a year’
The condition causes irreversible lung damage – it is incurable and lifelong. Around the world, cases of the condition are rising but it continues to be unknown, undertreated and underprioritised. In the UK, hospital admissions from bronchiectasis cost the NHS around £40m per year.
“Apparently that’s quite common as there can be a mismatch in what the medics think. I was put on antibiotics for three months and my symptoms cleared up so they discharged me,” McDonald said.
McDonald spends most of her free time cycling, hiking, running and swimming in the Peak District, which helps clear her lungs of the mucus caused by bronchiectasis
“But then the symptoms came back within a year and I just didn’t bother pursuing it because it wasn’t really interfering with my health and I was still able to do everything. I kind of got on with things and ignored it.”
It took another two years for McDonald to push her doctors to take the cough seriously and she was finally diagnosed with bronchiectasis last year – one of the most neglected chronic lung diseases despite the fact that it affects 300,000 people in the UK.
‘I’m still on long-term antibiotics’
Asked if she felt she would have got another CT scan if she wasn’t a doctor, McDonald said: “No. It’s not a criticism at all, because I know how difficult it is, but I had to speak to a lot of consultants and push for what I thought was right in order to get the diagnosis.”
Despite its challenges, bronchiectasis has not held McDonald back in any way. She spends most of her free time cycling, hiking, running and swimming in the Peak District, and is currently training for a half Ironman triathlon in September.
Symptoms of bronchiectasis
The main symptoms of bronchiectasis are:
- a cough that does not go away
- coughing up a lot of phlegm (mucus) – the phlegm may be clear, white, yellow or green
- getting frequent chest infections, where your symptoms get worse for a few days or weeks and you may feel generally unwell
Other symptoms can include:
- wheezing
- shortness of breath
- noises when you breathe, such as crackling in your lungs, snoring sounds or high-pitched squeaks
- feeling very tired
- coughing up blood
- chest pain
“Currently, my chest gives me no bother whatsoever, but it will be interesting to see what happens when I come off all the antibiotics because prior to that I was getting a lot of chest infections.
“I’m still on long-term antibiotics for this infection I have, and have been for the last two and a half years and will be for another year. The only issue really is the side effects, as the antibiotics play havoc with your gut,” she said.
McDonald said she is surprised there is no specific treatment for bronchiectasis, given the number of people who have it.
She added: “We need more than that. I’m only 27 so the biggest thing that worries is that I’m ok now, but what will happen in the future? Not many people get diagnosed quite as young, so it does worry me. I work in A&E and see a lot of patients come in with bronchiectasis who are very disabled by it.”
New treatment on the way?
Help may soon be on hand, however, as a US-based pharmaceutical company is on the verge of launching the world’s first treatment for bronchiectasis following almost a decade of research.
Results from Insmed’s phase 3 Aspen study – the largest clinical trial ever conducted in bronchiectasis and published in The New England Journal of Medicine – demonstrated a “statistically significant and clinically meaningful” reduction in both the frequency of pulmonary exacerbations, also known as lung flare-ups, and the rate of lung function decline.
Lead author James Chalmers, Professor and Consultant Respiratory Physician at the University of Dundee, said: “This is an exciting development and represents a potentially transformative breakthrough for people living with bronchiectasis, offering new hope for patients with this challenging condition if [the treatment] is approved.”
The new treatment is currently under “priority review” with the US Food and Drug Administration, with a potential approval date of 12 August. The speeding-up process aims to make potentially life-saving therapies available to patients sooner by shortening the review timeline from the standard 10 months to six months.
Insmed has also filed regulatory submissions for the treatment in the EU and UK and plans to file in Japan by the end of the year, with commercial launches anticipated in 2026, pending approval in each territory. Once approved in the UK, it generally takes up to 90 days for a drug to become routinely available on the NHS, meaning patients like McDonald could have access to the drug within months.
Steven Huysse, Head of Medical Affairs EMEA at Insmed, said: “Bronchiectasis is the third most common chronic airway disease after asthma and COPD. Despite its increasing prevalence, a diagnosis can be delayed and it is often misdiagnosed as asthma or COPD. As such, it can take up to 12 years for a diagnosis in some cases – contributing to it being one of the most neglected respiratory diseases.”
Huysse said the company also has trials under way for the same treatment on two more respiratory conditions, which it hopes will provide further breakthroughs.
McDonald said: “The fact treatments could be soon available is exciting and reassuring and means my condition won’t get so bad in the future.”
For more information on the condition and how to manage it, visit Asthma + Lung UK