Updates and news

RAIRDA recently submitted evidence to the Welsh Government in response to their consultation on guidance for services for people with arthritis and long-term musculoskeletal conditions.

We told the Welsh Government that there is a clear need to improve care for people with RAIRDs in Wales. Evidence from our surveys indicates that patients in Wales, on average, have more problems with their care than those in the rest of the UK. Whilst there is some good care in Wales and some clinicians with specific expertise in RAIRDs, we believe the lack of a commissioned specialised centre in Wales is a major reason for this difference.

Current Issues in Wales

The figures outlined in this blog are taken from fresh analysis of a UK-wide survey of people with RAIRDs conducted in 2018, to which we received 2229 responses (125 from people in Wales) and from a new survey conducted in June 2021 of 126 patients living in Wales. In addition we’re extremely grateful to the small group of patients in Wales commented on our draft submission to the Welsh Government.

Read our full 2018 report here.

On average, patients in Wales report worse care than those in the rest of the UK

Our 2018 survey indicated that people with these condition in Wales, on average, have greater issues accessing care for their conditions than in the rest of the UK.

We found that:

• In Wales 35% of respondents said they waited over 6 months to see a specialist when waiting for a diagnosis. In the rest of the UK the figure was 29%.
• 27% of respondents in Wales said they had a coordinated plan in place for their care, compared to 40% across the rest of the UK.
• 9% of respondents living in Wales reported they had been able to access care at a joint clinic with doctors from multiple different specialties compared to a, still low, 17% across the rest of the UK. This is despite the that fact 93% of UK survey respondents reported seeing clinicians from multiple specialties.
• In Wales, 62% of respondents said they weren’t confident in their GP’s understanding of their condition, compared to 53% in the rest of the UK.
• In Wales, 9% of respondents reported being offered psychological support to help them manage the impact of their condition on their mental health, compared to 15% in the rest of the UK.
• Fewer respondents from Wales had a specialist nurse involved in their care, and of those that did, fewer found it easy to contact their nurse compared to the rest of the UK.
• In addition, in Wales, 38% of patients said they had accessed private healthcare for their condition compared to 28% in the rest of the UK. This indicates less patients are able to get the care they need through the NHS.

Meanwhile, comments collected in our 2021 survey made clear how serious issues were in parts of Wales:

“Patients with chronic ill health are being failed in Wales. There is no support, no monitoring, no bloods taken, no medication. I worked hard in my career. For the past eleven years I have become incapacitated by my illness. I have lost my career and am now unable to afford private care.”

“My diagnosis 12 years ago took so long and was traumatic, I became so much worse than I needed to before diagnosis and treatment, this fear stays with me.”

“I have felt totally abandoned for the last 5 years since being diagnosed.”

“Having to fight the health board for minimal contact.”

“I’m often waiting for months despite appealing for help.”

Whilst some patients did report good, responsive, and coordinated care, comments like the above illustrate that many patients in Wales face serious issues.

Given the differences between the rest of the UK and Wales, there need to be specific proposals from the Welsh Government to improve outcomes for people with rare autoimmune rheumatic diseases.

Impact of COVID-19 pandemic on access to care

Our June 2021 survey asked patients what impact the pandemic had on the care they received:

• 41% said it had a major negative impact
• 29% said it had a minor negative impact

There were clear variations in how responsive services had been able to be, and some services had delivered good care despite the pressures of the pandemic. However, some very ill patients reported issues getting care.

“Whilst face to face appointments were swapped to telephone appointments during the pandemic when I had issues and new symptoms I was seen in clinic within the same week.”

“I had my rheumatology appointment cancelled during March 2020. I have not heard anything more from the hospital since. I am unwell and was hoping to start a new treatment for the conditions I’ve had diagnosed. I feel there is no help whatsoever and as if I have fallen off the radar completely. Just left in pain and forgotten about.”

“I went privately as I didn’t know who to contact during a flare. The consultant explained it will be due to COVID that the rheumatology nurses don’t answer the phone, or reply to messages left”

Rheumatology staffing issues affect RAIRD patients

Comments we received from patients in 2021 from patients provide further evidence of the extent of staffing issues in Wales. For example:

“The rheumatology department is vastly understaffed, and the waiting list for clinics are long. Therefore, conditions deteriorate and end up needing more intensive intervention, making the rheumatologists job much more difficult and burdening the waiting list more.

“My rheumatologist left the health board over 2 years ago and I have been left in limbo with care.”

Workforce issues have contributed to overstretched services and it’s evident to patients that healthcare professionals do not have the time to deliver the quality care they aim to.

Issues with access to specialist care in Wales

In Wales, patients may be referred onto another hospital in Wales for care where a consultant or team is understood to have additional expertise caring for a certain rare disease, or onto a specialised centre in England. However, doing this through the NHS is a difficult process and can require an individual patient funding request (IPFR) to be made by clinicians.

In their comments, respondents to our 2021 survey told us:

“I feel the Welsh NHS has abandoned me, I’m fortunate I can pay to access treatment and consultation in England, but there is a desperate need for a centre in Wales to treat patients like myself with specialist care”

“I was under the care of a health board outside of where I lived initially and as it was a specialist centre I had very good care. However, I’ve been repatriated to my local health board and since then the care I’ve had has been awful, in fact it’s been non-existent.”

Given the disparities between care in Wales and care in the rest of the UK, there is a clear need to improve access to specialist care for RAIRDs in Wales.

Key recommendations

We believe there is compelling evidence to support specific actions for patients with rare autoimmune rheumatic diseases.

A specialised, tertiary centre (or centres) for these conditions should be formally commissioned in Wales by the Welsh Health Specialised Services Committee.

Recognising that many people with these diseases cannot get all the care they need from their local hospital, other UK nations specifically commission certain hospitals to deliver specialised centres specifically for these diseases. Patients are referred to these centres by other clinicians in local hospitals and these centres have to meet certain criteria to qualify as specialised.

The Welsh Government can help close the gap between Wales and the rest of the UK by commissioning a specialised centre.

Some patients will continue to need care from hospitals in England – this is because there are some patients who need to see a clinician with further expertise than can exist in Wales. For example, some patients in the north of England travel to Cambridge for their care, despite there being a specialist centre in their region. However, a specialist centre is still needed to drive improvements in care in Wales.

A specialised network could further increase the impact of a new specialised centre

To effectively amplify the benefits of a specialised centre, we support the use of specialised networks to support local rheumatologists to care for these conditions when appropriate. These networks exist in other parts of the UK and often use video conferencing to allow clinicians to seek advice on treatment and make sure care is delivered consistently.

These networks function best when they are centred on a recognised specialised centre, which is properly funded to invest time into supporting the network.

Additional recommendations

Other recommendations in our response include:

• Action on workforce development, education, and recruitment in rheumatology and investment in wider multidisciplinary teams including specialist nurses and clinical psychologists in line with the British Society of Rheumatology’s recent report ‘Rheumatology workforce: a crisis in numbers’.
• Improve access to rapid advice through advice lines and department email inboxes.
• Action to ensure face-to-face appointments when needed.
• Specific recommendations to support rare autoimmune rheumatic diseases, rather than recommendations covering rare diseases in general.

We hope that the evidence provided in our response fosters a sense of urgency. Specific actions to improve care for rare autoimmune rheumatic diseases, and support clinicians to deliver this, need to be taken by the Welsh Government.

Research published today indicates that the 170,000 people in England with rare autoimmune rheumatic diseases like lupus, scleroderma, and vasculitis were more likely to be infected with, and die from, COVID-19 compared to the general population during March-July 2020.

Using data from March to July 2020, during the first wave of the COVID-19 in England, a paper published by researchers from the RECORDER project, has concluded that adults with the included rare autoimmune rheumatic diseases (RAIRDs) had a 54% greater risk of COVID-19 infection and more than twice the risk of COVID-19-related death compared to the general population after age and sex were taken into account.

Whilst the results are sobering, this research is important. Through a novel collaboration with National Congenital Anomaly and Rare Disease Registration Service (NCARDRS), it allows for comparison between everyone in England who has ever been hospitalised for their RAIRD and the general population, giving a far more comprehensive and thorough account of how people with these diseases have been affected.

While previous research had shown that people with rare autoimmune diseases were more likely to die, of any cause, during the first two months of the pandemic, researchers were unsure whether this rise was due to COVID-19 infection or a disruption to peoples’ ongoing care for their RAIRD.

The study only used data from England, however, we believe its conclusions will hold for patients in Wales, Scotland and Northern Ireland.

As a result of this research, RAIRDA are calling for further steps to protect people with these diseases from COVID-19. Including actions to: protect people receiving treatments in hospitals through testing; ensure everyone with these diseases receives a booster jab; and for employers to act to protect people.

Responding to the research, Sue Farrington, Co-Chair of the Rare Autoimmune Rheumatic Disease Alliance, and Chief Executive of Scleroderma and Raynaud’s UK, said:

“These diseases are often overlooked because their individual rarity means we lack solid data on them, but thanks to this research we have clear evidence about the impact of COVID-19.”

“We need to make sure we’re taking steps to protect these people. For example, hospitals have been told to test everyone undergoing a surgical procedure, but not all are testing people before they go into a unit where immunosuppressed people are receiving infusions. With higher risk of death among these patients from COVID-19, and uncertainty about how effective vaccines are for people on immunosuppressive treatments, we need to do more to protect these people whilst cases remain high.”

What about vaccines?

The data used in this research dates from the first wave of the pandemic. As such, it shows the need to protect people with RAIRDs through vaccination but does not tell us about how effective vaccines are among these people.

People who are immunosuppressed, which includes many people with RAIRDs, may have a weaker response to the vaccine, which may mean they get less protection from the vaccine. However, there is a lot of uncertainty about to what extent this is the case, and more research is needed.

As well as wider research into vaccine efficacy among people who are immunosuppressed, research is ongoing specifically into vaccine efficacy among people with rare autoimmune rheumatic diseases, and this will also look at the impact of booster jabs due to be given in autumn. Research is also looking at whether specific medications and treatments affect vaccine efficacy.

Has disruption to ongoing care affected people with RAIRDs

As well as showing an increased risk of death from COVID-19, this paper shows there was not an increase in risk of death from other causes. This suggests that, while there were significant disruptions to care for RAIRDs, these did not impact on patients’ risk of death in the first few months of the pandemic.

However, we do know that the impact of disruption may only become apparent over the longer-term. Maintaining responsive care for these conditions will mean fewer serious complications that the health service, and patients, will need to deal with in future.

What about Sjögren’s syndrome?

Patients with Sjögren’s syndrome were not included in this research, so it does not tell us anything about their risk from COVID-19. Whilst patients often have Sjögren’s and another rheumatic disease, people with Sjögren’s alone are less likely to be on immunosuppressive treatments, which would suggest their average risk from COVID-19 is lower than for people with diseases like scleroderma, lupus and vasculitis.

What are we calling for?

The most vulnerable must be protected in hospitals

Some hospitals across the UK have already implemented measures to protect people with RAIRDs coming into hospital to receive strongly immunosuppressive treatments, such as testing for everyone coming into units where people receive treatments.

However, this has not been consistently done across the UK and we are calling for all hospitals to consider having measures in place to protect people in their day case units, such as testing everyone coming into a unit for COVID-19.

The people coming into these units for treatment are often the most immunosuppressed, and therefore the most vulnerable to COVID-19, and having an infusion can involve being inside for many hours in close proximity to other people.

Guidance from NICE currently only recommends testing everyone going into hospitals for surgery, but not other planned procedures. Some hospitals do test in their day case units, but this is not mandated or universal practice. As well as testing, we recommend units consider adopting the relevant measures recommended for, and put in place by, dialysis units.

Booster jabs are vital – people with these disease must be covered

This research shows how important it is that people with RAIRDs have their COVID-19 vaccinations to get a degree of protection from COVID-19.

However, people who are immunosuppressed may have a weaker response to COVID-19 vaccinations than the general popular. Research is ongoing but one study suggests that COVID-19 vaccinations are less effective in people who are immunosuppressed after only one dose, highlighting the importance of the second dose.

All this shows how important it is that people with rare autoimmune rheumatic diseases are vaccinated and are prioritised for booster doses due to be delivered in the autumn. Rare Diseases Europe (EURORDIS) have called for third doses to be a priority for people with certain immune conditions, citing research that says a third dose pushes antibody levels 5-10 levels higher.

Despite being eligible for flu vaccinations through the NHS, people with these conditions have often missed out on these vaccinations. This happens for a variety of reasons including: diagnoses made in hospitals not being recorded in GP data, or healthcare professionals not recognising that these conditions make someone eligible for vaccination. This cannot be allowed to happen with booster jabs for COVID-19.

Employers must protect workers

All of this means that for those people at greatest risk of serious infection and whose response to the vaccine is still the focus of ongoing research, the return to work will not be straightforward. A coalition of charities has written an open letter to employers, asking them to take appropriate steps to keep people who are extremely clinically vulnerable safe. You can find this letter here.

This survey has now closed. Thank you to everyone who completed the survey. We will be the publishing results, and our submission to the Welsh Government, shortly.

We have launched a new survey which aims to gather the views and experiences of people living in Wales with rare autoimmune rheumatic diseases (RAIRDs). This will support our submission to the Welsh Government as part of their consultation on new guidance for services for arthritis and long-term musculoskeletal conditions. This guidance is meant to advise and guide NHS health boards on the way they design services for people with these diseases, including RAIRDs like lupus, scleroderma, vasculitis and Sjögren’s syndrome.

Through sharing the results of this survey with the Welsh Government we hope to highlight the needs and experiences of people with RAIRDs and give a clearer picture of how people with these diseases are currently cared for, and their priorities, so that this informs new guidance.

This survey is anonymous. Most questions are optional and can be skipped if you prefer not to answer. There are 20 questions, most of which are multiple choice.

The information collected through our survey will be combined with the responses RAIRDA collected in 2018 and 2020 from patients across the UK, including Wales. Previous survey reports have been cited by Members of Parliament, clinicians, and researchers looking to improve the way people with these diseases are cared for. If you answered one or both of these surveys, please do fill this one in too – this survey builds on these and asks different questions specifically relevant to people living in Wales.

In addition to this survey, we are also looking to involve a small group of patients in commenting on our final submission to the Welsh Government. We hope this will make sure our submission is as guided by patients as possible. Please email RAIRDAuk@gmail.com if you would be interested in finding out more about what this would involve.

What is a RAIRD?

RAIRDA is a coalition of charities (Scleroderma and Raynaud’s UK, LUPUS UK, Vasculitis UK and the British Sjogren’s Syndrome Association). Whilst we tend to focus on the four conditions these charities represent, a full list of conditions which might be considered a rare autoimmune rheumatic disease (RAIRD) is below. If you have a diagnosis of any of these, and live in Wales, please do fill in our survey.

• Systemic lupus erythematosus (SLE)
• Antiphospholipid syndrome (APS)
• Systemic sclerosis (scleroderma)
• Sjogren’s syndrome
• Relapsing polychondritis
• Myositis/inflammatory muscle disease.
• Undifferentiated or Mixed Connective Tissue Disease
• Systemic vasculitis, including:
  • Giant cell arteritis
  • ANCA-associated vasculitis including: Granulomatosis with polyangiitis (Wegener’s), Microscopic polyangiitis, Eosinophilic Granulomatosis with polyangiitis (Churg Strauss)
  • Behcet’s disease
  • Takayasu’s arteritis
  • IgA vasculitis (Henoch-Schonlein purpura)
  • Cryoglobulinaemia

Avtar Gill has Raynaud’s and Scleroderma and lives with his partner in Birmingham. His career within the training team of the AA ended early when he took redundancy due to the impact of his diagnosis, and he now works for Tesco on a part-time basis. Avtar is very active in supporting others who are living with scleroderma and set up the SRUK Support Group Midlands in 2018.

Before I turned 40, I was one of those people who never went to the the doctor and was not taking any medications. Today, it is a very different story. I was diagnosed with secondary Raynaud’s in 2016, after my symptoms began without warning when I had a sudden, severe Raynaud’s attack. I saw my GP, who made a referral to Solihull Hospital, where I was diagnosed with systemic sclerosis (scleroderma). Today, I am prescribed a cocktail of different medications to help manage this condition.

It was during the process of my diagnosis that I first reached out to try to find others who may be in a similar situation, and I soon realized that there was a gap at local level in finding peer support for people with Raynaud’s and Scleroderma.

After making some contacts online I eventually suggested that we all try to meet in person, so myself, my partner and three others met in a pub in Birmingham for the very first time. This was the beginning of the SRUK Support Group Midlands.

It was enlightening to talk to other people who were also living with Scleroderma and Raynaud’s, and since everyone felt the same way, we quickly agreed that our meetings should continue. Partners are still very welcome, as these conditions can cause a great deal of stress and anxiety, so all-round support remains incredibly important.

Our little group has quickly grown to around 30 members. We promote our meetings on social media including via our Facebook group; that is a closed group and a safe space to share any concerns or seek advice. As well as a place to meet new people and find friendship, the group is also somewhere to share our struggles, which can be very helpful since there may well be someone else who has been through something similar.

Before the pandemic, we would meet in person every quarter on a Sunday. During the first lockdown, we found that Zoom was the best platform on which to stay connected. When life eventually returns to normal, we will probably combine our online catch-ups with face-to-face meetings, so that we can include anyone who cannot get there in person but would still like to be a part of what we do. Setting up this amazing group has definitely been worth it.

If you would like to find support and connect with other people in your local area through an SRUK support group, please visit sruk.co.uk/find-support/support-groups or call Scleroderma & Raynaud’s UK on 020 3893 5998 to get started. Other RAIRDA member charities – LUPUS UK, Vasculitis UK and the British Sjögren’s Syndrome Association – also help support groups for people with other rare autoimmune rheumatic diseases conditions.

 

Sara was diagnosed with scleroderma in February 2020. She had been constantly ill with severe infections for two years, as well as symptoms of Raynaud’s, and suspected an autoimmune condition due to lupus being in the family. In January 2020, her GP sent off bloods, which came back positive for scleroderma and she saw a rheumatologist a week later.

My diagnosis came at a very difficult time; I was signed off work with fatigue, chronic insomnia and anxiety, my grandmother was diagnosed with terminal cancer and I had suspected COVID-19. It was a really overwhelming experience, especially with the pandemic and lockdown. After a difficult few months, things started improving and I was able to work from home.

I recovered relatively quickly from COVID-19, but unfortunately developed ‘long COVID’, it is thought that the scleroderma significantly delayed my recovery from the virus. My treatments and ability to see specialists have also been delayed and it was daunting to have to self-manage without much information so early in the scleroderma journey. Physiotherapy hasn’t yet been possible, and I had to wait to see a dermatologist, although I have quite significant skin issues. I could only get a video appointment, and was given a ‘diagnosis’ of possible psoriasis linked to my scleroderma, but this was without an examination.

Discovering the SRUK (Scleroderma and Raynaud’s UK) website was an absolute lifeline, and it was great to know that I wasn’t alone. I have been watching their various webinars and reading the myriad of information on their website and social media pages. This was often my only source of information, so to try and give something back, I have been actively supporting them by raising awareness – I was even featured on BBC News 24 and Radio 4 – which was nerve-wracking, but great to feel I was helping others. I have also fundraised through a sponsored walk and hope to be able to do more fundraising once lockdown has ended.

The lack of control that comes with my diagnosis scares me, but I try not to let it overwhelm me. I’ve been seeing a psychologist for several months, which has really helped my mental wellbeing, and helped me come to terms with my condition, which I knew nothing about before the diagnosis. However, it was a struggle to get access to formal health support to begin with.

Whilst I have started to feel more in control of my condition, there have been ongoing issues with my health that were made more difficult with COVID – I needed urgent gallbladder surgery last year but had to wait months; and then it was postponed due to lockdown. Luckily, I have had the surgery now, but it was a horrible experience having to wait for nearly six months whilst in pain…. a hardship I know so many people are currently facing. My grandmother also passed away before Christmas, and I had some challenges at work due to my condition, which just increased my stress level after an already difficult year.

I am determined to keep as informed and in control of my condition as possible. I’m also due to get married this year, so I’m hoping that restrictions might be lifted, and that this year will be much better than 2020. All you can do is try and focus on the positive things in life, and all the many opportunities we are lucky to have. Then, we can get through whatever life throws at us.

RAIRDA surveyed 1,300 people with rare autoimmune rheumatic diseases about their experiences during the COVID-19 pandemic, including asking about how their care had changed. We found that even as early as April and May 2020, 80% of people had experienced some kind of change or disruption to their care. Read the full report here.