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Covid-19 update for GPA patients

Public Health England is advising those who are at increased risk of severe illness from coronavirus (COVID-19), which includes individuals with GPA, to be particularly stringent in following social distancing measures.

The normal clinical practice in the circumstance that a GPA patient catches Covid-19 is to stop their immunosuppressive medication while the patient is being treated for Covid-19 and to resume once clear of Covid-19.

British Society for Rheumatology has released some guidance for patients on immunosuppressive agents:

The advice on the Lupus UK website is also excellent:

https://www.lupusuk.org.uk/coronavirus/

London Marathon 2020

Do you have a friend or family member suffering from Wegener’s (GPA) or are that person suffering from the condition? Are you a keen runner or just fancy a challenge of a lifetime? Applications are now open for London Marathon 2020. You can run in support of the Wegener’s Trust via the Guy’s and St Thomas’ Charity. Every penny of the money you raise by taking part will help further efforts to better understand and treat the condition. You need to raise a minimum of £2,500. Visit the page here and make sure to let them know you want to run for us!

Medical Research Council is funding a three year trial of hydrochloroquine in ANCA vasculitis (mainly GPA)

“If our study is positive, we hope that hydroxychloroquine, an old fashioned and safe drug widely used in other rheumatic conditions, will make a real difference to patients with this complex and challenging disease.” explains Professor David D’Cruz.

He goes on to say “I cannot thank the St Thomas’ Wegener’s Trust enough for your unstinting and generous support over the years while we put this grant application together. Without your support, this trial would simply not have been possible.”

Hydroxychloroquine could be a safe and inexpensive game changer in controlling non-severe ANCA vasculitis, reducing the reliance on steroids and the need for toxic steroid sparing agents like cyclophosphamide or rituximab, as well as reducing associated complications such as infections, blood clots and stiffness of blood vessels. This HAVEN trial is to address the surprising lack of studies of hydroxychloroquine in ANCA vasculitis given its long history of successful use in other auto-immune conditions. Quinine was first used for patients with Lupus by Reverend Payne at St Thomas’ in 1894, and hydroxychloroquine has also been a safe, successful treatment for rheumatoid arthritis for over 50 years.

This 3 year trial will involve 76 patients in 10 specialist study centres across the UK. The Wegener’s Trust has successfully laid the groundwork for the HAVEN trial to get the green light from the Medical Research Council, including a smaller pilot study of 12 ANCA vasculitis patients which had shown benefits of hydroxychloroquine. We thank all those who have donated to the Wegener’s Trust, this is a huge win!

We would like to thank Professor David D’Cruz, Dr Alina Casian, Dr Susan John and Sangmi Kim for keeping the faith despite the many hurdles along the way and bringing this potentially game-changing trial to fruition.

Benkert family raise £270,000 for Wegener’s research

When Alice Benkert died of Wegener’s granulomatosis in 2010, her family were determined to help other sufferers. Wegener’s is a rare but incapacitating disease which causes severe inflammation of the blood vessels and can result in major damage to the lungs, kidneys and other vital organs.

Her brothers Jules and Ollie Benkert wanted to do something in Alice’s memory, raise funds and increase awareness of the disease.

‘We were led to believe by some of the experts in the field that although Wegener’s remains very hard to diagnose, with funding available for key research, treatment may become available quite quickly,’ says Jules. ‘So being able to make a difference or even “find a cure” seems to be within reach.’

With that aim, the family have raised a truly astonishing £270,000 for research at St Thomas’ – an amount which has wildly exceeded their expectations.

To mark the first anniversary of Alice’s death, they organised a sponsored bike ride from Esher in Surrey, where Alice lived, to Mumbles in Wales, one of her favourite places. Approximately 25 of Alice’s family and friends took part in the 200-mile cycle, raising £130,000.

Five years on, the family organised a sponsored bike ride across the Pyrenees from Barcelona to Alice’s favourite holiday location, the Aigua Blava Hotel on the Costa Brava. 25 of them, many from the original team, undertook the 200km ride on 18th May 2016 with more than 3kms in vertical climbing (on bikes) in over just 2 days. They raised £140,000.

‘Athletic ability was very diverse but the consistent factor was that every participant knew Alice and the family and was ready to help,’ says Ollie. ‘The length of this challenge was sufficient to grab many friends of friends’ attention and so the fundraising effort beat many of our expectations.’

For others wishing to fundraise for Guy’s and St Thomas’, Ollie suggests aiming high, ‘especially with people who are close or even one step removed. The impact of these events if they have a personal touch can be meaningful.’

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A marathon for Mick

On Sunday 19 April, Richard Brown ran the Greater Manchester Marathon in memory of his brother-in-law, Mick. Mick sadly passed away last year from Wegener’s Granulomatosis, an autoimmune disease that causes organ failure. He raised an astounding £5,000 for Guy’s and St Thomas’ Wegener’s Trust which is undertaking research into the causes of the disease.

After losing their beloved Mick, Richard’s whole family wanted to do something together to remember him.

‘Mick was such a fit and healthy 50-year-old, and then he was diagnosed with this awful illness. He was strong and determined to fight it, but in the end, the treatment he had wasn’t enough. He passed away on the 12th May 2014, with his close family and myself around him.

‘He left behind his wife and two daughters aged 22 and 17. We knew that nothing could bring Mick back, but if we could help by raising money to fund research into this illness, then perhaps in the future we can stop other families going through what we had to.’

Richard has always been a keen runner and has attempted the London Marathon before, but it had to be put on hold when he sustained various injuries.

This time, he struck the balance right and was able to complete the Greater Manchester Marathon without too much trouble – although he couldn’t walk up the stairs at work the day after!

The race went really well and Richard finished with a time of 3 hours 6 minutes – easily beating his target of 3 hours 10 minutes.

Richard says, ‘I had to change my whole eating habits and basically plan my life around runs, but seeing the amount of money we managed to raise and thinking about how proud Mick would be of me is got me through it.

‘A lot of my family came over with me for support and were cheering me on – just what I needed in those final few miles.’

First five years of fundraising

Fundraising events for the Wegener’s Trust by families of patients have ranged from

200 mile cycle ride from Surrey to Swansea

world record 40 hours of 5-a-side football

abseiling off the world’s tallest hospital building

mother of four running the London Marathon

5k run

109km Tour de France cycle through the French Alps

This herculean effort on the fundraising side has raised £240,000 (close to £50,000 a year) in the five years since Professor D’Cruz and James Hancocks set up the Wegener’s Trust, clearly demonstrating the need that the Wegener’s Trust is fulfilling. Affected families are able to respond in a constructive way by helping fund targeted research into the condition.

The various lines of research into the processes and cell biology behind Wegener’s have been highly successful to date (see what we have found out so far) and should lead to better treatment for patients (see current research). This research can only be funded thanks to everyone’s successful fundraising.

Thank you so much.

For help on your fundraising or to put a patient’s story on the website, please email us on [email protected]

Nick’s story

Nick was born in a hospital in the South Wales Valley in 1968.  He attended a Welsh speaking school and was very proud of his welsh heritage.

After school Nick worked around the country in the construction industry – helping build the M25, Canary Wharf and the Docklands Light Railway amongst his day-to-day worklife.  It was whilst working on one of these projects that Nick first experienced a problem with his throat.  He didn’t take much notice of his ‘sore throat’ at first, thinking he had acquired it through over zealous singing at a Wales/England Rugby fixture back home.

Eventually after many tests and bouts of severe breathlessness he was diagnosed with Wegeners.

Nick was treated in the Royal National Throat Nose and Ear Hospital in Kings Cross, London, travelling there more and more as the years passed.  He underwent many procedures to laser and clear his trachea, along with many drug combinations to try and put, and keep, the Wegeners in remission.  He could tell when he had a relapse and would up his steroids accordingly before being seen in London to stabilise them.

A tracheostomy performed to give his windpipe more room and a chance to recover produced many eventful episodes (like when he ‘lost’ his ‘talking tube’ overboard on the Dover/Calais ferry!).  A stent didn’t prove to be any use either – especially when the stitches holding it in place broke!!!

Nick was really pleased to meet someone with Wegeners in the same place as himself recently, as everyone else he encountered seemed to have their Wegeners in different parts of the body to him.  He could talk and advise as well as sympathise and encourage this person, with whom he was glad to just share his experiences with in the knowledge that they could understand exactly what the other was going through.

Most recently Nick was undergoing infusions in Hammersmith Hospital and was due to go for a review after completing the final one in the batch of three.  When asked how he thought they were doing, he said “my breathing is easier, but the sticky stuff is getting really hard to get up”.  This proved to be true as Nick choked on the Wegeners whilst on his Nebuliser!!! Nick passed away at home on 11th July 2012 aged 43.

If anyone reading this would like to know more please get in touch and we can hopefully help by providing more indepth details about drugs/trials etc.

We’ll never forget you, Nick, and will love and miss you until we meet again x x x

Nigel’s Story, by his wife Pauline

Nigel, when I first knew him in our late teens, loved country walks,  was District Long-jump runner up in the sixth form, tried sailing at his polytechnic; later, he regularly played badminton and croquet and was mad about his mountain-bike; he had boundless energy and scarcely knew a day’s sickness.  Having been the driver (and a responsible one) for all social occasions since passing his test at 17, he scarcely drank alcohol.  He loved his food, ate a healthy mixed diet, was never overweight and never smoked a cigarette in his life, let alone touching more potent drugs.

It seems doubly cruel and unfair that such a man could be dead at 57 after enduring 15 years of chronic illness, but so it is.

In his early forties, Nigel suffered a series of chest infections, and eventually bronchiectasis was diagnosed.  This was ascribed to his having had a severe bout of whooping cough in childhood.  No longer able to cope with intense exercise, Nigel exchanged badminton for golf, mountain biking for trail-bike riding, and adapted to life with chronic lung disease.  A few years later, he was dogged by gallstones.  After having his gall-bladder removed, he had three months of really good health; he was on top of the bronchiectasis and felt much better in himself.

This was not, alas, to last.

That summer – 2006 – Nigel had constant problems with a sore mouth and, eventually, a sore throat, which was diagnosed as persistent oral thrush.  He also had a small lesion on his right cheek which refused to heal.  Gradually, he gained more lesions on his face, especially round his mouth and nose; his airways became even more congested than usual; his hearing worsened.  Over the next couple of years he was sent from specialist to specialist – dermatologists, ENT, immunologists, even an Aids clinic – eventually ending up back at the lung clinic, no further on, and then doing the rounds of all the others again.  They did countless ever-more-complex blood tests; they took biopsies; no-one could diagnose what exactly was wrong.  Unusual tropical diseases were investigated, despite the fact that Wales was the nearest he’d got to foreign travel since our early twenties!  He was treated with a variety of medicines, some of which seemed to ease symptoms temporarily, but none of which worked long-term.  He developed encrusted lesions on his limbs.  Bone in his face deteriorated; he lost the two upper front teeth and developed a hole in his palate. He also developed nasal septum perforation.  His voice became hoarse from damage to his vocal cords.  He lost weight and could not put it back on, even with food supplements.  Unsurprisingly, he had less and less energy as time went on.

Wegener’s (which we had never heard of before) was first mentioned to us as a possibility in the late summer of 2009.  The problem was, Nigel’s symptoms (particularly the nature of the skin lesions), although they ticked many boxes, did not altogether seem like Wegener’s and at some point the tests had included an ANCA test, which had come up negative.  The alternative “favourite” was some kind of fungal infection, but they could not identify one.  Eventually – with the risks clearly spelled out to us that if he was treated for Wegener’s with immuno-suppressants, then it turned out to be an infection after all, the treatment would allow the infection to become even more rampant, and probably would kill him – we went ahead in November 2009 with Cyclophosphamide pulse therapy which, at first, had really good results; Nigel’s appetite returned, and some of his energy; he was able to enjoy short country walks; some of his skin lesions healed; he put on weight.  We hoped that doctors had found the answer, that the Wegener’s could be contained, its worst symptoms eradicated and we could resume something like a normal life.

Unfortunately, though, during 2010, treatment for Wegener’s was severely curtailed by the infections he developed and Nigel’s symptoms worsened again.  At this point, he took long-term sick leave from his work as a Computer Network Manager in a school.  He was never well enough to go back, and the following year retired early, on the grounds of ill health.  By late autumn, his condition had deteriorated still further and he was finding it very hard to swallow or to speak.  That December, a PEG tube was inserted and by the end of January, he was having all liquids and nutrition through this, as any attempt to swallow resulted in him ingesting some of the food or drink into his lungs.

2011 was marked by Nigel’s self-referral to a London specialist, Valerie Lund, whose paper about Wegener’s he had read on the internet.  She referred us on to Professor Pusey at the Hammersmith hospital.  It was Professor Pusey who recommended treatment with Rituximab and the dermatologists at the Hammersmith who finally confirmed that the skin biopsy they took showed results consistent with Wegener’s.  However, our Midlands doctors were understandably reluctant to begin treatment which would lay him still further open to infection. As an interim measure, he was put on a low dose of mycophenolate (MMF) and also, regular infusions of immuno-globulins were started, though there was a delay in this whilst funding was sought. When the rheumatologist up here agreed to rituximab treatment, funding was again a delaying issue, and when Nigel finally got rituximab in late summer, sadly proved of little benefit to him.

In November 2011 Nigel was rushed into hospital with pseudomonas infection, and then released early in December, but he seemed very low and poorly throughout Christmas and was readmitted to hospital on January 12th with raging infection in his bloodstream.  At this point, we were told his chances were less than 50-50.  He did get through the infection and picked up considerably, but on February 14th his condition suddenly underwent massive deterioration; he had suffered a pulmonary haemorrhage and died that afternoon.  My son and I made it to his bedside just in time.

All the many doctors we saw agreed Nigel’s case was unusual, maybe even unique, and was complicated by him having more than one serious condition.  This undoubtedly was a major factor in the delay in diagnosis.  Fortunately most Wegener’s sufferers will enjoy a better outcome than Nigel.  However, further research and publicity are both vital to early diagnosis and effective treatment; even more importantly, we hope that research will pinpoint what causes it and how it may be cured and even prevented.  All who knew Nigel and the remarkable courage he showed in the face of terrible adversity wish to support the Wegener’s Trust in any way we can.  Nigel himself wished that in studying his illness, doctors would learn more and disseminate that information so that his legacy would give hope to other sufferers.

Gary’s Story

My name is Gary, I am 57 years old and in 2010 after what seemed like a lifetime of illness I was diagnosed with Wegener’s granulomatosis.

It all started in October 2009 when I noticed my eye kept watering and my sinuses were constantly blocked, I made an appointment to see the doctor and was given nose and eye drops, the symptoms continued and got worse and by early December I started to get flu like symptoms which included aching joints and pains in my ears I was so tired all the time I could hardly do anything without feeling drained.

Christmas came and I tried my best to join in with the family celebration but by this time I was having difficulty with my breathing and had to give in and go back to bed.  I spent the rest of Christmas and New Year in bed and in early January I knew I had to go back to the doctors.  The doctor had me admitted to Medway maritime hospital where they treated me for pneumonia, after 5 days and still not feeling any better they sent me home with antibiotics.

The next 2 weeks I carried on getting worse, most days I couldn’t even get out of bed, I was suffering with nose bleeds, I was weak because I couldn’t eat and had lost so much weight and also I had started to lose my voice.

On January 30th I awoke with chest pains, my wife called an ambulance and whilst on route I suffered a heart attack, on arrival at Medway hospital I was stabilised but because they thought I also had pneumonia I was transferred to St Thomas’s hospital where I was taken into theatre and fitted with a stent.  They also tested me for swine flu but this came back clear.

A few days later I was starting to feel a bit better, I was hoping this would be it and I was going to be well again but no that didn’t happen the next day I had the same chest pains and was taken back into theatre to be fitted with yet another stent.

Due to my condition and because I was so ill my family requested I remained at St Thomas’s and not be returned to Medway hospital, thankfully they agreed as the next day I suffered internal bleeding and was rushed to intensive care and put on life support under the care of Dr Gibson, 2 days later my condition started to improve but I was still critical and remained in intensive care for a further 2 weeks where I was diagnosed with Wegener’s granulomatosis, which we later found out was the reason for my heart attack.

My treatment consisted of 8 courses of cyclophosphamide which was given every 2 weeks, after the first course of treatment I started to feel better straight away and was moved from intensive care to HDU where i started a course of physio to help me walk again as I was so weak and had lost so much weight, after my second session of cyclophosphamide I continued to do well and was allowed to go home.  After I had finished my 8 courses of cyclophosphamide I was put onto 25mg of methotrexate and steroids.

The Wegener’s did affect my airways and my breathing was slowly getting worse, a few weeks later I collapsed at home unable to get enough oxygen, my wife again called an ambulance and I was taken back to Medway hospital, I was assessed on arrival and transferred to charring cross hospital under the care of Dr Sandu, I underwent laser treatment on my airways, and was sent home 2 days later but had to return a further 4 times for laser treatment, until march 2011 where I underwent major surgery to rebuild my airway, they used cartilage from my ribs and a skin graft from my leg to reconstruct the airway, I was fitted with a trachea for 4 weeks and finally after 5 long weeks I was allowed home.

I have now improved considerably and have returned to work full time.  I am still under the care of Dr Gibson and the brilliant Wegener’s team at St Thomas’s hospital and I have regular check-ups every 6 months, I am happy to say after all that I am still living a full and happy life with my wonderful family.

Marathon 5-a-side Football World Record

Two squads of eight lads, playing 5-a-side football for 36 hours in order to get into the Guinness Book of Records.  Sounds easy doesn’t it? That’s a day and a half.  72 episodes of Eastenders, 720 boiled eggs or 24 eleven a side matches back to back.

Over the 13th, 14th and 15th May 2011, Paul and his friends, backed by an amazing group of volunteers passed the 36 hour record, passed the 38 hour target and went straight on to 40 hours!

All of this was in memory of Billy Newton, Paul’s father, who he lost to Wegener’s Granulomatosis in 2010. They raised just a shade under £15,000 for the Wegener’s Trust, an amazing contribution!

Read the blog on http://soccerampmam.wordpress.com/

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