NHS: Antiphospholipid Syndrome (APS)
(9 years, 9 months ago)
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Baroness Drake
To ask Her Majesty’s Government what actions they are taking to raise awareness of the autoimmune condition Antiphospholipid Syndrome (APS) amongst general practitioners and throughout the National Health Service.
Baroness Drake (Lab)
My Lords, I am keen to understand what the Government are doing to raise awareness of the autoimmune condition antiphospholipid syndrome—APS—among GPs and throughout the NHS, and to promote greater doctor recognition of the symptoms and earlier diagnosis.
APS, also referred to as Hughes syndrome, is an acquired autoimmune condition. The clinical features include thrombosis—venous, arterial and microvascular —and/or pregnancy complications and failure. It is important to recognise APS in the context of these problems and the need for appropriate treatment to reduce the risk of recurrence. In APS, the immune system produces abnormal antiphospholipid antibodies that target proteins attached to fat molecules called phospholipids, which makes the blood more likely to clot. There is currently no cure for APS but, if diagnosed, the risk of developing blood clots can be greatly reduced.
APS can affect people of all ages but it most usually affects adults aged 20 to 50, and affects more women than men. The exact prevalence of APS in the population is not known, but it is estimated that APS is present in around one in six of patients who have thrombosis or pregnancy loss, and present in 13.5% of strokes, 11% of heart attacks and 9.5% of DVTs. There is a need for a high-quality study of how common diagnosed APS is in the UK. Simple blood tests that identify APS, available in every district general hospital in the country, can often explain the underlying causes of strokes, heart attacks, pulmonary embolism and recurrent miscarriages. However, the condition is still poorly recognised in the UK. These simple blood tests can achieve earlier diagnosis and treatment for many patients, but there is an issue not only with testing, as some clinicians do not think of the diagnosis in the first place.
It is essential to raise awareness of APS among GPs and throughout the NHS. In my personal experience GPs and dentists, even if aware, are often not confident of treating patients who have it. This resonates with the findings of the charity the Hughes Syndrome Foundation, which ran a poll on the HealthUnlocked website. It found that 40% of GPs were aware of the condition but hardly any were confident in diagnosing or treating it. When the issue of making doctors more aware of APS was raised previously with the Department of Health, the response was that there are hundreds of different medical conditions and there is no particular reason why more attention should be drawn to this one—an opinion that I believe needs to be challenged.
There has been a significant increase in the level of research into APS, expanding the evidence base which continues to confirm that APS contributes significantly to the incidence of strokes, heart attacks, recurrent miscarriages and DVT. This is increasing awareness of the syndrome and the need for long-term treatment but, notwithstanding the growing body of evidence, while there are NICE guidelines for prevention and treatment of venous thrombosis there is none for APS.
The world of obstetrics has been one of the first to pick up on the syndrome. There are guidelines published by the Royal College of Obstetricians and Gynaecologists on the investigation of recurrent miscarriage, one of which is that women who have three consecutive miscarriages before the 10th week of gestation should be tested for APS. As a woman, one inevitably asks why it is necessary to suffer the deep distress of three failed pregnancies before blood tests are undertaken. Why can it not be done sooner? There are many other causes of miscarriage but that is not an argument against early testing of APS as, if its presence is confirmed, it is often treatable. The recognition and treatment of APL-positive pregnancies has undoubtedly delivered an improvement in pregnancy figures, but some hospitals do not include APL in their pregnancy screening. Yet APL-positive pregnancies increase the odds of stillbirth by between threefold and fivefold.
Guidelines on the Investigation and Management of Antiphospholipid Syndrome, published by the British Committee for Standards in Haematology, recommends that anyone under the age of 50 who has a stroke should be tested for APS. However, empirical research, again carried out by the Hughes Syndrome Foundation, found that not all the tests which look for APS are included in thrombophilia screens around the UK. The UK’s National Screening Committee’s guide to such screens states that the typical test includes lupus anticoagulant, but there is no mention of anticardiolipin or anti-beta-2 glycoprotein 1 tests. The Hughes foundation research reveals that not every hospital even tested for the lupus anticoagulant. If someone under 50 has a stroke, it is not certain that guidance will be followed or key tests carried out.
As clinical recognition has increased, so has the spectrum of signs and symptoms of APS to include features such as balance problems and gastrointestinal pains—symptoms which, when looked at holistically, can be indicative of APS. By not achieving greater awareness, pulmonary embolisms, strokes, heart attacks and miscarriages which are due to APS and often preventable will continue to occur, exposing individuals to risk and the NHS to much greater costs.
What action are the Government taking to raise awareness of antiphospholipid syndrome among general practitioners and throughout the NHS? What are they doing to promote greater doctor recognition and diagnosis of APS? What action are they taking to promote earlier and more routine blood testing to identify the condition? Will the Government promote APL testing in pregnancy screening throughout the NHS and review the policy whereby women have to have the distress of several miscarriages before they are tested for APS?
People with undiagnosed and untreated APS face being unwell over long periods, often for the want of simple blood tests. They can despair of ever finding a confirmed diagnosis or course of treatment, yet the treatment of APS can be life-changing. I conclude with a real-life clinical case study. Over a 30-year period Mrs A, now 50, suffered two DVTs, one pulmonary embolism, angina, transient ischaemic attacks, three miscarriages and, finally, a slight stroke. Eventually she was diagnosed with APS. I had my own journey, but eventually I met the wonderful doctors at Guy’s and St Thomas’ Hospital.
Baroness Morris of Yardley (Lab)
My Lords, I am delighted to be able to contribute to this short debate on getting greater awareness of antiphospholipid syndrome in the NHS, particularly among GPs. I declare an interest: I am chairman of the Hughes Syndrome Foundation, and I am very pleased to be so. I test positive for antiphospholipid but have had no more than minor symptoms. Where I am unusual is that I had a GP who spotted the signs early on, for which I am grateful. We need to get to a position where that story of early identification, and therefore no great illness other than minor inconvenience and minor treatment, becomes the norm, rather than stories such as the one that my noble friend Lady Drake finished her speech with.
The first point to make is that this is not a rare condition; few people have heard of it—it has a very complicated title, which does not help—but it is not rare. One in six miscarriages and instances of thrombosis, and probably most heart attacks, DVTs and strokes in people under 50, can be put down to antiphospholipid syndrome. Converting that percentage into cases, if you say that 10% of DVT clots can be attributed to APS, gives a figure of 3,000 events in the UK a year. So although this has only relatively recently been identified, it is not a rare condition. I bet I can say that almost all of us know someone who is APS-positive but we do not know it. The problem is that they will not know it either.
APS is essentially a success story. It was identified in 1983, and since then remarkable progress has been made in clinical and scientific research. PubMed is an international directory of worldwide scientific research. When we at the foundation looked at the publications in 2006, relatively recently, there were 20 published research papers. By 2014, that had grown to 602 published research papers each year. There are international collaborations under the banner of APS research, and recently at UCL here in London the UK Antiphospholipid Syndrome Society was formed, bringing together haematologists and rheumatologists—APS specialists are usually found in those departments—to collaborate and continue with research.
About 18 months ago we at the Hughes Syndrome Foundation realised that one of the things our patients were saying was that they did not know where the consultants were. We put together a directory of consultants in the UK and found that there are 100 consultants in hospitals in this country. So there is a growing body of evidence about antiphospholipid syndrome, and it has worldwide recognition.
It is not just that we know more about APS; the treatment is successful as well, as my noble friend Lady Drake indicated. There is no cure, but it is treated with anticoagulant medicines. The effect of that on women who have had previous miscarriages is that the success rate of 20% before 1990 among people with APS is now over 80% today. I think noble Lords can imagine what that means to women who have had three or more miscarriages, are treated and then have over an 80% chance of delivering safely.
Given this success story—success in identifying and researching the condition, relative success in being able to treat it—it is incomprehensible that there is such poor awareness of the condition elsewhere in the National Health Service and among GPs. Quite frankly, it is a lottery as to whether a diagnosis is ever made. Good-quality international work on this shows that on average patients wait three years for a diagnosis. We at the foundation put out a questionnaire to our members and found that a third had waited over five years for a diagnosis, and if you read their stories of going from consultant to consultant and from doctor to doctor, with no one recognising that this might be the cause, it is easy to see how early identification would make a real difference to them.
It matters that there is early identification, not only because treatment can be preventive—it might not cure the condition but it can prevent the worst effects of antiphospholipid syndrome being experienced by patients—but because once it was identified, more appropriate treatment could also be given. This is part of the problem: GPs or experts sometimes say, “Ah well, we’ll spot the thrombosis anyway. We don’t need to test for APS. Once the thrombosis happens, we’ll treat it as well as we can”. The point is that the treatment for thrombosis, for example, is different for those who have APS and those who do not. If you have thrombosis, you are likely to be put on warfarin for a period of three to six months. If you have thrombosis and APS, you need much longer-term treatment than that and will probably be on an anticoagulant for the rest of your life. Identifying APS as a cause of other conditions matters because it is the right thing to do, but also because it might determine the type of treatment that is given.
I suspect that the Minister’s experience in debates such as this is that people come forward and say that they want to bring the benefits of research and clinical progress to as many people as possible, and the debate then centres on the cost of drugs or the availability of specialised equipment. We all understand the difficulty that the National Health Service experiences in trying to afford bringing all the advances in medicine to patients who need them, but that is not the issue that we bring to this debate today. APS testing is relatively inexpensive: £25 for the two tests that are needed. As my noble friend Lady Drake said, the two simple blood tests can be requested by a GP. The barrier to testing is not a lack of resources or expensive equipment; the issue is that we have a disjuncture between what is happening at the clinical research level and how it is being acted on, particularly among GPs. I do not blame GPs for this. It is not surprising, given that there is so little planned structure for making GPs aware of the signs of the condition or its treatment. There is nothing in the NICE guidelines. It is not included in GP training, and even GPs who are aware of APS lack confidence in knowing how to treat it and to take it forward.
As a non-medical person, I think I understand how this has grown up over the years. APS affects all parts of the body, so every expert, every clinician, needs to be aware that APS might be a cause or a contributing factor, but because of this it has too often been seen as a second-order condition and has not been the first thing that specialists look at. Given the growing body of knowledge and evidence about APS and its treatment, it should now, 30 years after it was first identified, be seen as a condition in its own right. It needs to come in from the edge of medical awareness and be seen as part of mainstream diagnosis and treatment.
Some years ago, the Hughes Syndrome Foundation received a grant from the National Lottery and was able to send leaflets to GPs to try to raise awareness of the condition. It has just got a new leaflet and intends to do that again, but frankly that is not what should be happening. We are very happy to send out leaflet after leaflet, but we need a bit of help from the established part of the National Health Service that is meant to raise awareness among GPs. I therefore join my noble friend Lady Drake in her request to the Minister to say what else can be done to raise awareness among GPs.
I do not know what the Minister is about to say, but I suspect he will tell us about the things that already happen and that have been put in place to raise awareness about APS among GPs. All I can say is that it is not enough. It is not working. Too many people who have APS are still not having it identified as quickly and early as possible. They are not having the treatment that they need and the preventive treatment that can keep them well. All of us would want to overcome this problem with a £25 blood test. I am grateful to the Minister for his courtesy in asking us in advance about the issues that we wanted to raise, and I look forward to his response.
Lord Hunt of Kings Heath (Lab)
My Lords, I am very grateful to my noble friend Lady Drake for securing this debate and for her forceful speech. My noble friend Lady Morris also made a very powerful case, asking the Government to look at how they will support the improvements that have been spoken about so eloquently tonight.
I acknowledge the work of the Hughes Syndrome Foundation. Its fact sheets were excellent in getting across to me the scale of the issue that we face. I am very glad to hear that it will be circulating more leaflets to GPs in the near future.
We have heard that, if untreated, APS can lead to permanent disability, severe maternal or perinatal morbidity or even death; that symptoms can occur in virtually all organ systems; that venous thrombosis and stroke are the most common thrombotic manifestations; and that in pregnancy the syndrome is associated with adverse maternal and foetal outcomes. The problem is that the syndrome is underrecognised and underdiagnosed and can have, as we have heard, devastating consequences if untreated, mainly because of uncontrolled thrombosis.
Just to go over the ground already covered, these difficulties in diagnosis are compounded by a lack of standardisation of diagnostic tests. As we have heard, early recognition is crucial because treatment can reduce mortality or morbidity in relatively young people who often present with diseases such as stroke, myocardial infarction and deep-vein thrombosis. The example that my noble friend Lady Drake cited, of Mrs A, was very telling about some of the issues that can arise if there is not early diagnosis.
The first issue is, as my noble friend said, the need for a high-quality study of prevalence. My noble friend Lady Morris said that this is not a rare condition, but at the moment there is insufficient information to quantify just how common it is. It would be right for the Government to ensure that a robust study is now carried out to look at the prevalence of this disease.
I come to the simple blood test. What is so striking is that if it is simply left to individual hospitals or clinical commissioning groups—if they ever touch this area, which I doubt—we will not get consistency of approach and the uniform use of blood tests, which the evidence clearly points out that we need. If that is the case—if the noble Earl agrees with me—it is down to the Government or NHS England to start to signal to the NHS that we need to see a uniform policy on these tests.
I do not know whether the noble Earl read the piece in the Telegraph a couple of weeks ago in which there was an interview with Professor Hughes, the pioneering doctor who, with his team, found the evidence for Hughes syndrome many years ago. In that interview, he spoke about areas such as neurology and cardiovascular medicine, where there was less awareness among doctors of APS. He was quoted as saying:
“We know that it is present in one in five men aged under 45 who have a stroke, and one in five women aged under 35 who have a heart attack. Yet, it is simply not being picked up early enough to prevent these illnesses. Because the symptoms can be subtle, patients do get sent round the houses”.
The question therefore arises: given that the cost of the blood test would be a matter of a few pounds, why do we not ensure that those tests take place uniformly within the National Health Service?
We then come to general practitioners and the issue of training and raising awareness among them. In this Parliament, the noble Earl has debated many specific disease areas where issues have been raised about GPs recognising and diagnosing conditions that may not be rare but which they are not used to or do not recognise. One could approach that in a piecemeal way. However, given the pressure on GPs, and given that we know that the curriculum at medical school is very crowded, some other mechanism is needed to help general practitioners understand and recognise illnesses that are not common in the accepted sense of the term but which can have a major impact on many patients. The Government need to think very carefully with the profession, and in particular with GPs, about how we can improve their diagnosis success.
My noble friend said that there are no NICE guidelines and no national service framework. It would be very helpful if the noble Earl agreed that either his department or NHS England would set out what they consider to be the appropriate response of the NHS, as regards diagnosis of testing and treatment, so that we get a much more consistent approach. My noble friend Lady Morris said that investing in such a set of guidelines, including the testing, would be cost-effective. Of course, this syndrome was discovered in the UK by a fantastic British team. It would be ironic if this country, of all countries, was not then able to take advantage of the work that was developed in the UK. The noble Earl will know that, unfortunately, this is not unique in that respect. However, I hope he might be able to agree that something more needs to be done to get a cohesive national response.
The noble Earl has been very generous with his time, but perhaps he might be prepared to meet my noble friend, and perhaps the Hughes Syndrome Foundation, in the very short time remaining before we prorogue, to discuss whether the Government will be able to help in the way my noble friends have suggested.
The Parliamentary Under-Secretary of State, Department of Health (Earl Howe) (Con)
My Lords, I begin by congratulating the noble Baroness, Lady Drake, on securing this debate about a condition that, it is fair to say, receives little parliamentary attention either in this House or the other place. I also thank the noble Baroness, Lady Morris, and the noble Lord, Lord Hunt, for their excellent and powerful contributions. I listened with care to all that they have said.
Many of us—other than perhaps the two noble Baronesses—will be unfamiliar with antiphospholipid syndrome, or APS, so at the risk of repeating the information so usefully set out by the noble Baroness, Lady Drake, it may be helpful if I begin by explaining a little more about the condition.
APS, also known by some as Hughes syndrome, is a disorder of the immune system which causes an increased risk of blood clots. APS can affect people of all ages, including children and babies. However, it usually affects adults aged 20 to 50, and affects three to five times as many women as men. It is not clear how many people in the UK have the condition, although some estimates say that it affects at least 1% of the population.
People with APS may experience myriad symptoms. These can include headaches and fatigue as well as problems with mobility, vision, speech and memory. People with APS are also at risk of developing potentially life-threatening conditions, such as deep vein thrombosis—a blood clot that usually develops in the leg—and arterial thrombosis, a clot in an artery, which can cause a stroke or heart attack. It is estimated that APS is responsible for one in every six cases of deep vein thrombosis, strokes and heart attacks in people under 50. As has been said, pregnant women with APS also have an increased risk of having a miscarriage, although the exact reasons for this are uncertain. Some women, sadly, suffer recurrent miscarriages, defined as three or more miscarriages, and APS is thought to be the cause of this in around one out of every six cases.
Diagnosing APS can be difficult as some of the symptoms are similar to those of multiple sclerosis. Therefore, blood tests designed to help identify the antibodies responsible for APS are essential in diagnosing the condition. When a blood test is done, APS is easily distinguishable from MS at that point. On the difficulty around diagnosis generally, certainly in terms of how easily it can be picked up by a GP, my advice from NHS England is that, if a patient went to see their GP complaining of some of the low-grade symptoms of headache and fatigue, it is unlikely that APS would be something that the GP would immediately suspect and test for.
There is currently no cure for APS, as has been pointed out. However, if it is correctly diagnosed, the risk of developing blood clots can be greatly reduced. Anticoagulant medicines such as warfarin, or an antiplatelet such as low-dose aspirin, are usually prescribed. Treatment with these medications can also help pregnant women improve their chances of having a successful pregnancy. With treatment, it is estimated that there is about an 80% chance of a woman with APS having a successful pregnancy.
I turn to the specific issue the noble Baroness has raised—ensuring that the medical profession is sufficiently aware of APS. As I am sure noble Lords know, the content of GP training is decided by the medical royal colleges and approved by the General Medical Council. The noble Baroness suggested that GPs are not confident enough to recognise APS. I do not doubt that there have been cases where diagnosis of APS has been too slow, but I hope that she will agree that it is not possible to generalise in this area about the number of doctors who are alert to the condition.
The noble Baroness, Lady Morris, said that APS is not included in GP curricula. I understand that the Royal College of General Practitioners curriculum covers thromboembolic disease, which would include the understanding of predisposing factors for autoimmune conditions such as APS, so GPs should be sufficiently informed about the condition. NHS England assures me that APS is also widely recognised in general medicine, neurology, haematology and obstetrics. I also understand that the British Society for Haematology, the Royal College of Physicians and the Royal College of Obstetricians, provide guidance on how to manage APS.
I think that it was the noble Baroness, Lady Drake, who asked about the absence of NICE guidance on APS. In fact, NICE’s guideline on hypertension in pregnancy covers APS. Its document on reducing hypertension in pregnancy, published in August 2010, advises clinicians that women at high risk of pre-eclampsia, including women with APS, should take 75 milligrams of aspirin daily from 12 weeks of pregnancy until the birth of the baby. When the NICE guidelines on acute stroke and transient ischaemic attacks were in development, the inclusion of a management of APS was considered, but it was not taken forward as no studies were found that could form the basis of recommendations at that time. However, the 2016 edition of the Intercollegiate Stroke Working Party guidelines are currently in development, and NHS England’s national clinical director for stroke will discuss with the relevant clinical leads whether APS can be included.
The noble Lord, Lord Hunt, suggested that this was a problem that was down to a commissioning issue and that it should be for NHS England to have a uniform policy on blood testing. I differ from him in that I do not see this as a commissioning issue. It relies above all on doctors treating patients following the guidance that they are given.
I know that there are calls for screening for APS. In fact, UK testing for thrombophilia is offered on a selective basis during early pregnancy based on clinical risk factors, including previous foetal loss, still-birth, a history of pre-eclampsia and severe intrauterine growth retardation. However, as noble Lords may know, the UK National Screening Committee advises Ministers and the NHS in all four countries about all aspects of screening policy and supports implementation. The UK NSC reviewed the evidence for antenatal, newborn and adult screening for thrombophilia, including APS, in March 2010 and recommended that population screening should not be offered. Also, the UK NSC did not recommend antenatal screening. However, the UK NSC is due to review its recommendations on antenatal, newborn and adult thrombophilia screening this year. A public consultation will be held on the review and any stakeholders or individuals who would like input into the consultation should contact the UK NSC for a copy of the review’s timetable.
I understand all that the noble Baronesses said about the need to support women with APS who suffer miscarriages. We know that pregnancy is a time of huge emotional significance. Sadly, some pregnancies end in miscarriages. Families who have to cope with the distressing event of miscarriage need sensitive support and care from services and the people around them. That is precisely why the Royal College of Obstetricians and Gynaecologists has set out clear standards for NHS service organisations and levels of care that should be provided to help patients who experience problems in early pregnancy, miscarriage, ectopic pregnancy and recurrent miscarriages.
We still need to understand more about how APS affects people and how best to treat it. I am pleased to say that there is some research under way to help do just that. The National Institute for Health Research Clinical Research Network recently hosted a trial comparing the anti-coagulant effects of rivaroxaban and warfarin in patients with thrombotic APS. The trial closed to recruitment in November last year and is now in follow-up. In addition, the network has a new study in set-up that will look at the impact of fatigue and physical inactivity in people with APS.
I close by assuring noble Lords that this Government are determined to ensure that people with long-term conditions such as APS receive the help and support they need. I understand that noble Lords feel that gaps exist which should be bridged. I would be very happy to talk to the noble Baronesses, Lady Drake and Lady Morris, and the noble Lord, Lord Hunt, if they would like to come and see me about this. It is for this reason that through the mandate we have asked NHS England to make measurable progress in supporting people with ongoing health problems to live healthily and independently, with much better control over the care they receive. NHS England is taking action to make sure progress is made. For example, its business plan Putting Patients First sets out key actions to improve services for people with long-term conditions over the next two years. It has also recently published three handbooks to provide practical support for good management of long-term conditions and guidance on population risk-stratification. The aim is to help commissioners identify those patients who will benefit most from a personalised care planning approach. This, to be sure, is generic but I hope that it indicates the stress that we are putting on the importance of supporting those with long-term conditions, particularly those with multiple conditions.
In tandem with this, the Five Year Forward View, developed by the partner organisations that deliver and oversee health and care services, sets out that long-term conditions are now a central task of the NHS. Once again, I thank the noble Baroness for tabling this debate, which will help raise the profile of APS in a helpful way.