'˜Taking part in clinical trial has totally changed my life'.

She had no idea she actually had an extremely rare and often life-limiting disease.

Jenny was diagnosed with a type of vasculitis known as Churg-Strauss Syndrome, which can lead to heart attacks and lung failure.

But now her life has been transformed after she took part in a clinical trial for a new drug.

Her condition is also known as Eosinophilic Granulomatosis with Polyangiitis (EGPA).

It is an inflammatory disease of small and medium-sized blood vessels which affects the lungs and skin, but can also affect organs such as the heart and kidneys.

The main symptoms, apart from severe asthma, include sinus and nose problems, nerve damage, bowel trouble, rashes and heart problems.

‘It can affect the brain. Some people have died from it,’ says Jenny, 38, from Horndean.

One of the worst symptoms for Jenny was fatigue.

‘That was a massive thing for me. I always thought that was a bit of a myth.

‘I had just met my husband when I first started to get it. It made me really miserable. I was really lethargic.

‘I didn’t want to do anything. I had constant chest infections.

‘When events were coming up, I just hoped that I wasn’t going to be ill for them.’

Lifelong asthma sufferer Jenny, who works in sales for IBM, had to have some time off work.

She says: ‘I was stressed worrying about work and how I was going to manage that. I had a very understanding manager though. My company were very good.

‘At that point you couldn’t physically see that I wasn’t very well. I was really run down, but you can’t explain to people how rubbish you feel because there aren’t any physical symptoms.’

She adds: ‘I didn’t know what was wrong with me. I just kept getting ill. No-one could offer me an explanation.’

Jenny first started to feel ill in 2009, when she regularly found herself suffering from sinus infections and bronchitis.

After several trips to the doctor, she was referred to a respiratory specialist. He happened to be an expert in her illness and picked up on the symptoms straight away.

‘It’s quite rare so it’s very difficult to diagnose and there’s not a lot of awareness of it,’ she explains.

‘I had to hit a criteria first and they had to rule out a few things.

‘As soon as he saw me he got my illness under control, even though we didn’t know exactly what it was at that stage.’

Jenny was put on a course of steroids, but in 2011 she suffered a bad bout of pneumonia. At the time she was taking part in a hospital study in relation to her asthma.

After that she was officially diagnosed with EGPA and had to take a series of drugs including both nasal and oral steroids and antihistamines, plus regularly use inhalers.

It was around that point in her life that the trial was first mentioned.

‘They knew that there was a medical trial coming up for a drug and that I would potentially be an ideal candidate for it,’ says Jenny.

‘I was getting married and so having children was the next step. They said to crack on with it because they didn’t know what might happen in the future.’

In December 2012, Jenny gave birth to her daughter, Erin, having got married to Lee at the start of that year.

‘I was very closely monitored throughout my pregnancy,’ she adds.

In February, 2014 Jenny was asked by the respiratory research team at the Queen Alexandra Hospital in Cosham whether she would like to participate in a clinical trial testing the efficacy of a new drug.

‘The year-long trial meant I had to have injections every month. But I didn’t know whether I was receiving the actual drug or a placebo. I had to give feedback on any symptoms.

‘As soon as I started the trial I began to feel better and was able to reduce the steroids I was taking.’

She is now involved in a follow-up study. Jenny says: ‘I feel normal now. I can’t remember the last time I had a flare-up – it must have been almost three years ago now.

‘Before taking part in this study, it felt like I constantly had a flare-up and it did have a big impact on my life. Now I don’t feel ill. I still get tired but everybody gets tired. I feel like my old self again.

‘This has completely changed my life. For me, it was right place, right time.

‘Now I have long-term access to the drug. Hopefully it will be available on the NHS some day, but that could take years.’

Now Jenny wants others to be aware of the symptoms of this very rare illness.

She adds: ‘I can’t praise the research team at QA enough. They have been there for me pretty much since day one. That’s what you get from taking part in studies and trials.

‘I have found out lots more about my illness. I’ve been educated. And if this trial helps others, then that’s fantastic.’

EGPA

EGPA tends to affect both men and women equally. Around 11-13 people per million are diagnosed with it and the average age of someone with a new diagnosis is 40.

It is very rare for children or those over 65 to be diagnosed.

The cause of EGPA is not known. It is likely that a combination of factors lead to its development.

Studies looking at genetics have shown some genes that are linked to EGPA.

Meanwhile some people have Anti Neutrophil Cytoplasmic Antibodies (ANCA), which may cause some types of auto-immune disease.

Unusual levels of some types of hormone-like chemicals in the blood (cytokines) have also been found in people with EGPA.

The most up-to-date diagnostic rules, written by the European Respiratory Society (ERS), say that someone with EGPA should have:

n Asthma

n High levels of eosinophils in the blood (over 10% of all white blood cells)

n Damage from vasculitis to the skin, nerves, kidney, lung, heart or blood tests suggesting vasculitis

The ERS also suggested a sub-type of EGPA called Hypereosinophilic Asthma with Systemic Manifestations (HASM), where vasculitis itself was not present, but patients had other types of organ damage from eosinophils.

For more information on EGPA, please visit vasculitis.org.uk