Royal Brompton and Harefield hospitals are world leaders in the diagnosis, management and treatment of lung disease.
Our expert teams treat patients from the UK and overseas who have respiratory disorders including severe asthma, chronic obstructive pulmonary disorder (COPD), interstitial lung disease (ILD), allergies, occupational lung disease, cystic fibrosis (CF), sleep disorders and lung cancer.
They run the largest ILD clinic in the UK, the largest asthma clinic in London and the south east, and the largest occupational lung disease service in the UK. Our COPD clinic for patients with bronchitis and emphysema treats patients from all over the UK. The Trust is home to the largest adult cystic fibrosis centre in the UK, and the largest centre for the surgical treatment of lung cancer.
Interstitial lung disease
Interstitial lung disease (ILD) is an umbrella term that covers many different conditions. ‘Interstitial’ means the disease affects the ‘interstitium’, a lace-like network of tissue that supports the alveoli (air sacs) in the lungs.
ILD causes inflammation or scar tissue to build up in the lungs, making them thick and hard. This build-up of scar tissue is called fibrosis. As the lungs become stiffer and lose their elasticity, they are less able to take in oxygen. People with ILD can feel breathless from simple everyday activities like walking. Coughing is another common symptom.
There are more than 200 different types of ILD. All of them are rare, but the most common is idiopathic pulmonary fibrosis (IPF).
The interstitial lung disease unit at Royal Brompton Hospital is the largest unit of its kind in Europe, with almost 5,000 patients.
Dr Felix Chua, consultant respiratory physician and divisional lead for ILD says: “For most types of ILD, the cause is unknown. Some types are linked to other conditions such as rheumatoid arthritis, or it can be a side-effect of medication such as chemotherapy drugs. It can also be caused by exposure to certain types of dust and other allergens such as birds, mould and metal dusts.
“Most patients who come to us have experienced symptoms for years, and many have been misdiagnosed in the past. There is no cure, but it can be managed.”
In the past decade, teams at Royal Brompton Hospital have been at the forefront of developments made in the diagnosis and treatment of ILD. Two new drugs for ILD were approved by NICE in 2014.
Sarcoidosis
Sarcoidosis is a form of interstitial lung disease. It’s a rare condition that causes small patches of red and swollen tissue, called granulomas, to develop in the organs of the body. It usually affects the lungs and skin but it can affect other parts of the body, such as the heart.
The symptoms of sarcoidosis depend on which organs are affected, but typically include tender, red bumps on the skin, shortness of breath and a persistent cough.
For many people with sarcoidosis, symptoms often improve without treatment within a few months or years. For these people, the symptoms aren’t usually severe. However, a few people find their symptoms develop gradually and get worse over time, to the point where they become severely affected. This is known as chronic sarcoidosis.
Sarcoidosis is estimated to affect about one in every 10,000 people in the UK. There is currently no cure but, as with IPF, it can be managed.
Royal Brompton’s ILD team runs a weekly sarcoidosis clinic, the largest of its kind in the UK. We have particular expertise in the management of chronic and multi-organ sarcoidosis and run monthly combined cardiac-sarcoid and neuro-sarcoid clinics – a first for the UK.
Teams at the Trust also have close links with dermatologists and ear nose and throat surgeons based at Chelsea and Westminster Hospital, for the shared care of individuals with sarcoid affecting the skin and upper respiratory tract.
Cystic fibrosis (CF)
The cystic fibrosis centre at Royal Brompton Hospital is one of the largest in Europe, caring for around 700 adults and 340 children. In 1965, the Trust established the UK’s first adult cystic fibrosis service in response to the increasing number of patients living with the condition into adulthood.
Cystic fibrosis is a life-limiting inherited condition caused by a faulty gene that controls the movement of salt and water in and out of cells. This causes mucus to gather in the lungs and digestive system, which can lead to infections and other problems.
Gene therapy for CF
A therapy that replaces the faulty gene responsible for cystic fibrosis (CF) in patients’ lungs has produced encouraging results in a trial at Royal Brompton Hospital.
During the clinical trial, 136 patients aged 12 and over received monthly doses of either the gene therapy or a placebo for one year. The results showed that patients who received the therapy had a modest, but significant, improvement in lung function compared with those receiving a placebo.
Patients in Scotland also took part in the trial and were treated at the Western General Hospital in Edinburgh.
The cause of CF – mutations in a gene located on chromosome 7 – was identified in 1989. This discovery opened the door to a gene therapy, which introduces a normal copy of this gene. In the trial, patients inhaled molecules of DNA wrapped in fat globules (liposomes) that delivered the gene into the cells in the lung lining.
The study was carried out by the UK Cystic Fibrosis Gene Therapy Consortium, a group of scientists and clinical teams from Royal Brompton & Harefield NHS Foundation Trust, Imperial College London, the Universities of Oxford and Edinburgh and NHS Lothian. The group came together in 2001 to develop a gene therapy and were supported by the Cystic Fibrosis Trust.
The trial launched in 2012 and was funded by the National Institute for Health Research (NIHR) and the Medical Research Council (MRC). The findings were published in July 2015 in The Lancet Respiratory Medicine.
The trial is the first to show that repeated doses of gene therapy can have a meaningful effect on CF and change the lung function of patients. However, further research is needed to make the therapy more effective before it is suitable for clinical use.
Professor Eric Alton, consultant physician at Royal Brompton Hospital and co-ordinator of the Imperial College consortium, says:
“The results are encouraging and lay the groundwork for further trials that we hope can improve the effect. We are looking to carry out follow-up studies to assess higher and more frequent doses, combinations with other treatments and different methods of delivering the gene into the cells.
“Our aim is to achieve a step change in the treatment of CF that focuses on the basic defect rather than just addressing the symptoms. Eventually we hope gene therapy will push CF patients towards a normal life expectancy and improve their quality of life significantly.”
Chronic obstructive pulmonary disease (COPD)
COPD is an umbrella term that includes chronic bronchitis and emphysema. It’s a condition in which the airways become inflamed and the air sacs in the lungs are damaged. This causes the airways to narrow, resulting in breathing difficulties.
The main cause is smoking and it’s thought to affect around three million people in the UK. There is currently no cure, but the condition can be managed with exercise and medication. The Trust offers a world-class COPD service to patients from the local community and across the country.
New freezing treatment for chronic bronchitis
Chronic bronchitis is the most common form of COPD. In March 2016, a pioneering treatment for patients with chronic bronchitis was carried out for the first time in the UK as part of a trial led by experts at Royal Brompton and Chelsea and Westminster Hospitals in London.
The 30-minute procedure, which uses liquid nitrogen to freeze diseased lung tissue from the upper layer of cells in the airways, was carried out by consultant respiratory physician and chief UK investigator, Dr Pallav Shah, as part of the RejuvenAir System clinical trial.
The minimally-invasive treatment targets the goblet cells, which are found in the top layer of airway tissue (the epithelium) and produce excessive amounts of mucus in patients with chronic bronchitis. The procedure aims to destroy these cells using liquid nitrogen while preserving the stem cells underneath. The stem cells allow new, healthy tissue to quickly form within a few days.
Dr Shah says: “The main cause of chronic bronchitis is smoking because the immune system triggers the goblet cells to produce too much mucus. The excess mucus blocks the airways, causing coughing and breathlessness, and attracts bacteria, which can lead to infections. Even when these patients stop smoking, the cells still produce excess mucus – it is as though a switch is broken and is permanently turned on.
“While some medications available to patients can thin the mucus or open the airways, this new procedure is the only treatment that has the potential to stop excess mucus production and restore it to a normal level.”
During the procedure, a narrow tube with a light and camera at the tip – known as a bronchoscope – is inserted into the patient’s airways that lead to the lungs. A thin tube (catheter) is then manoeuvred into the part of the lung targeted for treatment. The catheter is connected to equipment that delivers liquid nitrogen at a carefully-controlled dose, which depends on the patient and the area being treated. The liquid nitrogen is about -196˚C when applied to the cells and at this temperature the structures within the cells freeze and die.
In March, Jayne Hewitt, 49, from London, became the first patient in the UK to have the novel procedure. She was diagnosed with COPD around four years ago after suffering from breathlessness, a tight chest, frequent chest infections and a persistent cough. Her condition progressively worsened until it got to the point where simple daily tasks, such as having a bath, carrying shopping, talking and eating, were a struggle.
She says: “It’s amazing to be the first UK patient to have this treatment and I feel so privileged to be given this opportunity. Days after the procedure my chest already didn’t feel as tight, I was coughing less than before and breathing a bit better, even though only one part of my right lung had been treated. It used to feel like I had an elephant sitting on my chest and now it doesn’t. It has given me such a boost and I feel more hopeful about my future now.”
The clinical trial, which is taking place at Royal Brompton Hospital, in collaboration with Chelsea and Westminster Hospital NHS Foundation Trust, and at the University Medical Center Groningen in the Netherlands, aims to evaluate the safety and feasibility of the treatment. It is funded by CSA Medical, the company that manufactures the RejuvenAir System.
Singing for breathing
The Trust’s weekly Singing for Breathing workshops have been running since 2008. Singing can be beneficial to people with damaged or weak lungs as it encourages them to focus on breathing techniques and use their lung capacity as best they can. A survey of 500 Trust patients found that 70 per cent felt significantly physically better after taking part in Singing for Breathing workshops.
The sessions also boosted their wellbeing, making them less anxious, depressed and isolated.