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Clare's Story

Clare's Story

A patient experience of A&E

For every major vascular event I have had, doctors have either tried to discharge me from A&E or have succeeded in doing so when I’ve been critically unwell. So, what is going wrong? I hope to answer this question by drawing on my experiences as someone living with Vascular Ehlers Danlos Syndrome (VEDS), so that people in my situation can be more prepared. Before my first major vascular episode, I believed that my greatest challenge in an acute medical event would be to make sure that the doctor knew I had a diagnosis of VASCULAR (not hypermobility) EDS, and therefore at risk of vascular complications and hollow organ rupture. I soon learned that it is far more complicated. Following several difficult experiences in A&E, I think I finally understand the main problems. I wish I had understood these specific issues before I had my first aneurysm, rather than just having a broader expectation that I would face problems because the condition is rare.

I do first want to mention the problem of VEDS being a very rare condition. It is very important be realistic about your A&E experience. As it is highly probable that your doctor or nurse has never heard of, let alone seen a real life patient present with VEDS. Whilst health professionals are always working hard to broaden their knowledge, it is not possible for them to know everything. A large proportion of a doctors’ knowledge will depend upon their clinical experience, which will be different for each individual. A doctor explained to me what he called the ‘unknown, unknowns’: There are things he knows that he knows, and there are things he knows he doesn’t know. But then there are the things he doesn’t know that he doesn’t know. VEDS frequently falls into this category. On the flip side, a doctor may believe they understand VEDS. However, they may not fully appreciate the potential complications, such as the subtle presentation and instability, especially if this is based on textbook knowledge.  

So other than VEDS being rare, what are the specific issues to be aware of? Firstly, Ehlers Danlos Syndrome (EDS) is more commonly associated with the hypermobility type. After my first aneurysm, I thought that my medical history would be a clear indicator to doctors that I have vascular EDS rather than hypermobility EDS. But I was wrong. Only a few months after the event, I was told by a doctor that she ‘wouldn’t expect me to have any more aneurysms because what we know about EDS is the hypermobility’. It is unrealistic to expect doctors to know about every type of EDS. However, the doctor’s comment did surprise me given that she was aware of my recent vascular event and diagnosis of VEDS. Despite knowing this, she confused VEDS with the hypermobility type. This contradicted my belief that if a doctor knew about both my diagnosis and medical history, there would be no confusion between vascular and hypermobility EDS.

Another issue is that we don’t look like we have anything wrong with us. When reflecting on a difficult experience with an A&E consultant, he told me: ‘You do not look like a ‘vascular’ patient’. Meaning that I do not present with the normal risk factors that would alert doctors to a potential vascular problem. I am younger than a typical ‘vascular’ patient, I am not overweight, I don’t smoke, and I don’t have high blood pressure. Therefore, on initial presentation a doctor may not consider vascular complications, especially if they have no experience of VEDS. I had always expected that looking ‘normal’ could be a problem if I was ever to experience a complication. But this was a complete revelation to me, because as someone with VEDS all I can think about is vascular problems. Whenever I present at A&E I emphasise that I have VASCULAR EDS, so I thought that doctors would investigate to rule out vascular complications. I found it a really useful insight into how clinicians might think.

I discovered this next problem when I attended A&E for severe pain in my left arm. Vascular complications in VEDS patients can be very subtle in presentation. On this occasion, minor changes showed up on the CT scan of my arm, so the vascular registrar did not think this was the cause of the pain. He discharged me from A&E in severe pain, without a diagnosis, and telling me that there was nothing on the scan to indicate that this was a vascular complication. I had still thought that having a significant vascular history would protect me, and the doctor would be more cautious in his clinical decision making. So, I was not prepared for his decision to discharge me. I also thought that because the CT did not show any changes (or so I was led to believe), and he was a vascular specialist, I must have been wrong in thinking the cause was vascular, even though the pain was similar to previous acute vascular complications. I went home only to return to A&E a few hours later because I couldn’t tolerate the pain, and my condition deteriorated. The outcome was that I went on to develop several aneurysms in the arm that required surgery. Aneurysms in the arm are very rare, so that complicated the situation. On reflection, I should have trusted my instincts, and asked for a second opinion at consultant level, and refused to leave A&E. After the event the vascular team agreed that I should have been admitted for further investigation.

I have found this next issue to be problem with EVERY vascular event. Vascular complications in VEDs patients can be VERY unstable. For example, when I had my first major vascular event, I presented at A&E with vomiting and severe pain. The doctors acted appropriately and sent me for an CT which showed that I had multiple aneurysms. I had never had an CT scan before this, so the doctors didn’t know how long the aneurysms had been there. And because they were considered small, the doctors advised they would not operate due to the risks. The decision not to operate seemed sensible to me, as I am aware that the threshold for surgical intervention in VEDS patients should be higher. The problem was that I was discharged despite still being in severe pain and feeling extremely unwell. The doctors did not realise at that time how unstable the aneurysms were.  

I remained in this condition for nearly a week; abdominal pain, severe nausea (I couldn’t even smell food without feeling sick), fever, and distended abdomen. After visiting the GP countless times, and being diagnosed with acid reflux, I returned to A&E when the abdominal pain became even more severe. By this stage I was desperate, and kept telling the doctors that something was seriously wrong. I was diagnosed with a urinary tract infection (UTI). The severity of the pain was explained away as me having, a ‘low pain threshold’. The doctor wanted to send me home, but with support from my family I was admitted. A repeat CT showed that one of the aneurysms had significantly grown in size, and I underwent emergency surgery.  

My experience suggests that doctors think aneurysms will behave in VEDS patients as they do in the ‘normal’ population, and are not prepared for the unstable nature of the condition. Whilst the decision not to operate initially was appropriate due to risk, it would have been beneficial to monitor my condition and repeat the scan when I did not improve. However, I acknowledge that it is very difficult for doctors to know how frequently to scan VEDS patients.  

The final issue to mention relates to pain. Pain in the event of a vascular complication is very unpleasant, but it is also an invaluable indicator when you have VEDS. I assumed that if I told the doctor how severe the pain was, or they realised that my level of pain was inconsistent with their diagnosis, they would consider admitting me for further investigations. But getting others to understand how much pain you are in is very difficult. Feeling like the doctors didn’t believe me was really upsetting and frightening, but the rational part of me understands that pain is very difficult to assess in another person. Especially in the case of rare conditions, and VEDS pain presents itself differently because symptoms are more unusual. 

In my experience, raising concerns about pain levels has NEVER changed a doctor’s decision, or concerned them enough to admit me, and on occasion it has been attributed to my pain threshold. An A&E consultant explained to me that “the challenge relates to pain that is out of proportion with vital signs or examination findings. If the underlying diagnosis is serious, a normal examination can provide false reassurance to the clinician trying to make the diagnosis. Especially if they are not aware of all possible/rarer diagnoses, which is when a clinician might try to attribute the mismatch to a patient’s pain threshold”.

As a patient, my advice is never to rely solely on the expectation that your symptoms will be investigated if you feel in severe pain. But I have learned that for me, severe pain (however it is perceived by a doctor) has always been a crucial indicator of a serious vascular complication. Experience has made one thing very clear to me, and that is that a VEDS patient in severe pain should not be discharged from A&E. Even if investigations indicate that there is no major vascular event occurring, because presentation of complications in VEDS patients can be subtle and unusual. Senior and specialist advice must be sought under these circumstances. 

I hope that sharing my understanding of these problems helps you in getting the care you need when you face complications relating to VEDS, as well as giving health professionals a deeper understanding of the potential complications associated with this rare condition. Life with VEDS is hard. You often don’t feel safe in your own body, or in the care of health professionals. The last thing you want to do when you are unwell is challenge a doctor’s decision. But as people living with a rare condition we often have no choice, and need to work alongside health professionals in the management of our condition. Regardless of the challenges I have faced, I have huge respect for the health professionals involved in my care. I owe them my life, and will never be able to thank them enough for what they have done for me.

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