Patients share their thoughts and experiences about Royal Brompton research and how getting involved has helped them.
May 2020
I was born in 1973 with a congenital heart defect and kyphoscoliosis (where the spine is abnormally curved). My parents were living overseas at the time and so when the doctors realised I had something seriously wrong with my heart I was flown back to the UK and became a patient at Great Ormond Street Hospital (GOSH). I had open heart surgery in 1977 and in 1984 I had a spinal fusion operation to put a rod in my spine.
After school I undertook a Higher National Diploma in Applied Biology and then gained a place at Oxford Brookes University to do Food Science & Nutrition with Applied Biology, a combined honours degree which I completed in two years. By then I was getting very tired and my cardiac team felt that I would benefit from having a pacemaker. I had one fitted just a week after graduating, in September 1996 and by 1997 I started working at The Royal College of Ophthalmologists where I would go on to work for the next 21 years.
Around 2003/4 I started to feel that I was getting more tired and things that I could previously do quite easily, were becoming more difficult. Despite mentioning this to my cardiologists at the time, it took them a while to really take me seriously and investigate the symptoms I was having, including spells of feeling a fluttering in my chest and sickness.
In 2004 I had an electrophysiological study and ablation done for atrial tachyarrhythmias at University College Hospital (UCH). Two years later I had a cardiac catheterisation and it was then that I was diagnosed with mild pulmonary hypertension (PH). After this diagnosis I was referred to Hammersmith Hospital where it was decided that my PH was due to my severe restrictive lung disease resulting from the kyphoscoliosis and was therefore untreatable. I was subsequently referred to the sleep and ventilatory clinic at the Royal Brompton Hospital and came under the care of Professor Anita Simonds.
Initially it was recommended that I use a CPAP ventilator and then in 2011 I was prescribed ambulatory oxygen which was a bit of a shock to me and I felt very self-conscious using it. The first time I used it in public was when I went to the Opera with my husband. I thought no one there would know me but we bumped into some old friends. At the time I was mortified but they were very kind, and I gradually got used to using it in public.
Over the following few years I steadily deteriorated without really realising it. I was getting more and more chest infections and finding it harder to manage day to day in full time work.
In January 2015 I developed chest pains which were diagnosed as acid reflux but over the next few months the familiar fluttering’s returned, and I knew that my arrhythmia was back. While waiting to get a confirmed diagnosis I went down with pneumonia and was hospitalised on a cardiac ward. It took two and half weeks to recover and I’ve been on oxygen ever since. Later, I had a second much trickier ablation done at St Bartholomew’s Hospital, as by this time my cardiac team had been transferred to the Bart’s Heart Centre, and in December 2016 I had my third pacemaker implanted.
After a lovely holiday in Greece September 2017 my flight home was extremely scary as the oxygen provided by the airline didn’t seem to be working. I was only getting a tiny amount of oxygen and my oxygen saturation fell to really dangerous levels. I felt dreadful over the following days, so I phoned my team at Royal Brompton Hospital and they found I was in heart failure and that my PH had deteriorated. I was referred to Professor Kostas Dimopoulous’ team and he has looked after the PH side of things ever since.
I was quickly admitted for a cardiac catheterisation and started on medication soon after. I felt upset and annoyed that I had been denied any treatment for so long and I had misunderstood and thought that my care at Royal Brompton Hospital was for my PH as well but actually it was solely for my ventilation.
It was so encouraging to be included in the discussions about my PH therapy and for someone to explain all the various intricacies of my heart condition and how it related to PH. Everything made sense at last. I understood what the treatment was for and how to look after myself to try and prevent further deterioration. The support I receive from the PH specialist nurses is wonderful and I can call or email them and get a reply usually the same day, which is very reassuring.
At the end of 2017 I saw my cardiologist at Bart’s where it was stressed that my condition was only going to get worse so I decided that I would retire from work at the end of 2018. The College was celebrating its 30th anniversary and I wanted to be involved in the celebrations and there were projects in progress that I wanted to see completed but it was not to be.
After getting pneumonia again in May and then going into respiratory failure I knew I wouldn’t be able to return to work, so I took the decision to retire as soon as possible. Thankfully the medical retirement process was swift and straightforward and my last day at work was on the 14th September 2018. It was really difficult saying goodbye to all my lovely friends and colleagues, especially as some had been there as long as I had but it was definitely the right decision.
After that my husband and I moved from West London down to Wiltshire and we are enjoying a slower and healthier way of life. Soon after I was asked if I would like to join the Cardiovascular Lay Advisory Group at Royal Brompton Hospital and I jumped at the chance. I feel it’s important to keep your mind active once you stop working and I love being able to contribute towards research that helps other patients with conditions such as heart failure, cardiomyopathies, COPD and PH.
The research carried out at Royal Brompton Hospital also looks at diagnostics, psychological aspects of heart disease and the greater understanding of how people acquire them in the first place. We have talks from exceptional academics and clinicians too and are able to give feedback on patient information literature, consent forms and how the results of research are disseminated afterwards. It’s really rewarding, and I also enjoy my trips up to town to attend meetings.
January 2019
I first had chest pains and medical investigations back in 1992. The pains stopped after a while but returned with a vengeance in 2000 and have continued, off and on, ever since.
I get acute pain on the left side of my chest, which often spreads to my left arm and that side of my face. I might have difficulty breathing, especially in very cold weather, and feel tired much of the time. The episodes can last anything from a few minutes to hours, occurring day or night, while active or at rest. On a bad day, I can suffer several episodes a day and even wake up in the morning, in pain. It is debilitating and has proved to be life changing.
Between 1992 and 2008, I had a range of tests, including angiograms, cardiac catheterisations, an echocardiogram, exercise tests, 24-hour tape monitor, Thalium Scan and numerous ECGs. All my ECGs were, and still are, abnormal. Despite seeing various cardiologists at several different hospitals, none of the tests showed any heart disease or cardiac conditions. Various letters, hospital reports and discharge forms stated symptoms compatible with everything from myocardial Ischaemia and chronic Ischaemic heart disease, to angina. Some cardiologists even suggested that the pains were psychosomatic – a diagnosis which only deepened my distress. At one point, I was misdiagnosed with cardiomyopathy, although the cardiologists eventually admitted their error and that, at least, was ruled out.
The big breakthrough – a diagnosis at last
My search to determine what was wrong with me came via an unlikely source. In 2008, I read about an American singer called Toni Braxton. She had collapsed on stage due to a condition called CSX/MVA - a condition I’d never heard of. But the description of her medical problems were similar to mine.
In the past, Braxton’s condition had been referred to by at least four different names: Cardiac Syndrome X (CSX), Syndrome X – normal coronary arteries with persistent angina, Microvascular Angina (MVA) and severe chest pain in the absence of coronary disease. It is now generally known as MVA.
After contacting an American professor about my symptoms, he emailed me straight back and told me to get in touch with a cardiologist that he knew in England who might be able to help. Although he wasn't able to help me, I knew that I was on the right track.
In 2010, my cardiologist at Barnet became the first to confirm that I had CSX, although no one knew what the 'x' stood for. He referred me to cardiologist, Peter Collins, a professor at Royal Brompton Hospital, who had been treating people with CSX and researching the condition. Professor Collins was the first person who listened to me properly and confirmed my CSX diagnosis.
Professor Collins has been of immense help and reassurance to me over these last eight years, and has made sure that I’ve tried and taken the medication best suited to me. Above all, he convinced me that I wasn’t imagining my pain or consequent symptoms.
On the humorous side, if there is one, I was initially downcast to discover that MVA mainly affects post-menopausal women – neither of which I am! It also appears that only 5% of people with this relatively rare condition, are men.
In 2011, I started a MVA/CSX discussion group on the British Heart Foundation (BHF) website which allowed many of my fellow sufferers to post comments about their own situation and empathise with each other.
In November 2012, having had an MRI Scan of my heart at Royal Brompton Hospital, I was invited to take part in a research study for the Trust’s Biobank, led by Dr Elianna Reyes and Professor Collins. The study aimed to help identify genes that may be associated with heart problems.
In 2014, I began attending monthly meetings of the MVA support group and met others grappling with the condition. Meetings have included guest speakers, who have taught us a lot about the technical aspects of MVA and the best ways to cope with it. I’m grateful to Dr Carolyn Webb, for her efforts, in running this excellent group.
The last meeting featured Dr Tina Khan, who spoke about a new clinical trial proposal for a treatment which may potentially help people with MVA. If, and when, funding has been finalised, I will be keen to take part in this.
My wonderful, supportive wife has been pivotal in getting me through the ordeal of suffering while not knowing what was wrong. Along with my equally supportive close family and understanding friends, she helped me to come to terms with, and pursue, the vastly different lifestyle that I had to adopt.
A short poem
Since early retirement from a career in the financial services industry, I have developed a love of writing. I have written and published books, letters to the media and a lot of poetry, including:
Research is surely the key
To a better lifestyle for me
So that, as somebody with MVA
I will no longer suffer the way
That I regularly do, today
And I’m sure that this applies
To all my fellow sufferers, likewise
So, to the powers that be in the medical profession
This is my plea for more research into this condition
If you would like to find out more about how you can get involved in research, either as a participant or as a lay reviewer, please email us.
March 2018
Idiopathic pulmonary fibrosis (IPF) is a condition which causes scars in the lungs and reduces the efficiency of breathing. The build-up of scar tissue is called fibrosis; this causes the lungs to become stiffer and lose their elasticity so they’re less able to inflate and take oxygen from the air.
IPF is a progressive condition and usually gets worse over time. In some people the symptoms gradually get worse over several years. For others, the symptoms get worse more quickly.
Jose Luis Gonzalez Nieto, a patient who suffers from IPF, writes about his experience of living with IPF and how he became involved in research at Royal Brompton Hospital.
Life is not complicated, we make it complicated. We go through this journey called life trying to achieve objectives, enduring what life throws at us the best way we can, often taking things for granted. In many cases we pay little attention to the functions of our bodies necessary for everyday living, such as breathing, and usually it takes a problem or serious condition to make us realise the importance of being in good health.
I didn’t look sick, nor did I really feel any severe symptoms associated with IPF. I just had some shortness of breath when climbing the stairs and some lower back pain, both of which I initially blamed on my lack of fitness and being 64 years of age. So I joined a local gym and began swimming at weekends.
As the weeks passed the pain did not subside so I was referred to the local hospital for a CT scan of my waist and lower back. Although the result of the scan did not show any issues with my kidneys or spine, the radiologist did report some irregularity and inflammation at the base of my lungs. I was therefore referred to my local chest physician and after a few appointments I was diagnosed with IPF.
I was dumbfounded. It’s so very distressing to have a lung disease. It’s natural to be shocked and distraught when you first hear the words.
My wonderful wife attended most of the medical appointments with me and her love and support is always a huge help. Within an hour of receiving the diagnosis, she had found out that Royal Brompton Hospital is part of the largest specialist heart and lung centre in the UK, delivering world-class care, and we made an appointment for me to see one of their main consultants.
From the first moment I visited Royal Brompton Hospital I felt I was in expert hands, and very soon afterwards I was receiving excellent advice on medication. My morale was boosted and by gaining a better understanding of the disease, I was able to start doing the best I could to take care of my health.
In addition to taking the medication I attended a pulmonary rehabilitation (PR) programme lasting a couple of months, which I found extremely beneficial as it helped me get fitter, lose some weight and set me on a path to exercising on a regular basis, which I continue to do.
The programme included physical exercises to help me breathe better and education on how to stay healthy and active after completing the course. It was not just focused on patients with IPF, and the team of specialists set goals and monitored my heart rate, blood pressure and oxygen level so that I was able to exercise safely. They also provided advice on how to manage the symptoms of my condition and advice on the best diet.
At the gym, where the PR took place, I met a group of patients who have become friends. We meet socially on a regular basis for a meal or coffee and we call ourselves Team Brompton!
Since my diagnosis I have made significant changes to my lifestyle, follow a healthy diet, adopted a routine of exercising every day (including three or four visits a week to the local gym to attend classes on pilates, tai chi and aqua aerobics) and I try my best to avoid colds and chest infections to keep myself as healthy as I possibly can.
The Interstitial Lung Disease (ILD) Patients Support Group, which meets every two months at the hospital, is an excellent opportunity to get advice on medication, oxygen therapy and healthy diets from consultants, specialist nurses and external experts. The meetings also provide an opportunity for patients to meet as a group and exchange information.
The battle to beat IPF requires participation from us, the patients, in order to find an effective and definitive cure for this disease that is robbing us of our lives. Research is the key to finding this cure and we cannot afford to waste any time.
So far, I have enrolled in the PROFILE study and other projects, and I am considering joining other trials to help find a cure for IPF. Every patient who makes a contribution to research for finding the cure for this disease is taking another step towards reaching that objective, and we all want to get there as soon as possible.
If you would like to find out more about how you can get involved in research, either as a participant or as a lay reviewer, please email us.
September 2017
I am in my 68th year as a chronic atopic asthmatic and have never let it hold me back.
Born with hereditary asthma (my grandfather had it); it became evident at 18 months old when I struggled to keep up with my brother’s pram on walks out. Breathing was a noisy, suffocating nuisance! Soon I was visiting the hospital in Bury St Edmunds where my doctor had me blowing feathers suspended on cotton threads and ping-pong balls down a couch to encourage me to breathe out.
Treatment was sporadic. I recall bright blue, bitter tablets hidden in a spoonful of demerara sugar and a large Rybarvin Inhaler. It could only be administered by an adult, and this meant painful anxious waits for its relief. This lasted well into my teens when Ventolin Inhalers arrived. Oh, the bliss of self-administration at last!
At home in those early years we had lots of cats, chickens and geese. It was later discovered that I was highly allergic to these; cats in particular. It will be no surprise that I was often in hospital as an emergency admission.
My mother said I could gallop before I could walk. All my life I have loved music and dancing. I was soon in my first show in the Abbey Gardens, Bury St Edmunds, as a rabbit. I was two and a half! I was picked out for ballet classes as having an aptitude and this became my passion.
Milford Chest Hospital was a pivotal moment as they introduced Prednisolone into my life when I was aged 12. It enabled me to follow my dream of becoming a ballet dancer, which I did, surviving the grueling training, and on into several ballet companies. I learned to cover up my proneness to attacks and bronchitis in my determination to keep working. Widening my experience with contemporary dance companies, musical theatre, choreography, plays and television, I worked in the theatre for 15 years, marrying and bringing up two wonderful children.
While they were growing up, I ran my own ballet school in West Wimbledon and continued for 35 years with the help of my husband. With the regular routine of terms and holidays, I found I relished teaching and bringing out the potential in children, enabling them to realise their dreams. We did shows and exams and festivals and I worked non-stop 6 days a week till last year; still dogged by bouts of acute asthma; unpredictable, cyclical and hard to manage.
I was eventually referred to the Royal Brompton Hospital under Professor Chung and discovered the wonders of new approaches, attitudes, and their interest in each individual. Above all else, it was the feeling of relief that here were people who understood me. I did not have to keep explaining. There was a plan, aims and encouragement. I have been on Omalizumab (Xolair) for the last 6 years, which has made a huge improvement to my reaction to allergens; my grandchildren’s cat, Floribunda Button in particular!
The care and attention to detail I have experienced at the Brompton has been so heartening and helpful. Especially those received through the department of research into exacerbations run by Dr Hoda. This is enabling me to keep a closer monitor on my asthma and to better understand what is going on.
I feel privileged to be involved in the research of treatments for future generations so they may be free to pursue their passions as I have.
If you would like to find out more about how you can get involved in research, either as a participant or as a lay reviewer, please email us.