Sticky Blood-Hughes Syndrome Support

Summary from Kings Hospital

I've had my appointment at Kings re my low heart rate. They were fantastic!!!

I've to have loads of tests to cross other things off the list but the current thinking is that either APLS or Lupus is affecting my heart

On paper my lupus is stable.

As my INR is still wildly unstable I'm back under hospital care instead of community care. In order to try to bring me back into range. Over the last 2 years that's only been 52%.

Has anyone else with APLS found their heart affected? I'd like to hear from you. 💋

22 Replies
oldestnewest

The INR and anticoagulation is very important when we have APS.

Before I was anticoagulated I had very high bloodpressure and also chestpain. Most probably PEs but as I have micro-emboli they are not seen on a Scan of today as they are too tiny but still make damage to organs.

They found I had Pulmonary Hypertension and Tricuspid- and mitral-leaking heartvalves.

After anticoagulation with a stable and rather high INR of 4.0 I am stable and feel ok. No high bloodpressure but still on bloodpressuredrugs (those I had before I was anticoagulated).

That is why I am nagging about the anticoagulation as I know how important it can be with our illness.

If i were you I would read "Hughes Syndrome: Highways and Byways" by prof Graham Hughes and M Khamashta.

Also "Sticky Blood Explained" by Kay Thackray. I know she has had heart-issues and I think she writes a bit about it in her books. The second book is "More Sticky Blood"

Take care!

1 like
Reply

I'm sorry u have had so much to contend with.

My poor pharmacist & I have been battling alone with my INR for 2 years. The haematologist I was under was not understanding and dismissed my one positive lupus anticoagulant antibody result and list of symptoms saying she didn't believe in zero negative APLS.

Now I have a different one who obviously sees I'm in trouble!!!

My blood pressure is the lower end of normal.

The thinking atm is that the APLS or SLE has affected the natural pacing of the heart

Many thanks for ur reply - very useful. I believe I have micro-embolisms in my head/brain 💋

1 like
Reply

I am glad you do not have high bloodpressure. I just wanted to tell you that heart-issues can sometimes be connected with our illness.

Kerstin

Reply

Thank u. Yes they think lupus or APLS has caused mine 💋

Reply

Yes, I have had my heart effected...they found “Libman Sacks” growth ( known it can be a problem with APS) on my mitral valve and did a mitral valve repair to fix it. Went to top notch Hospital in NY with Cardiothoracic Surgeon to have it fixed.

Problem was I had so many medical people on my team they forgot to anticoagulate me on discharge, then not even 16 hours later I had a second stroke leaving me paralyzed on left side.

I’m assuming that they all thought the other had taken care of the anticoagulation- hence it wasn’t done😖 beware everyone.

Always make sure they have anticoagulation planned and how they are going to accomplish this before agreeing to any surgeries! Good luck!Cindy in NJ

2 likes
Reply

How terrible I'm so sorry that one cute had such devistsorffects on u. I hope u r doing well now 💋

1 like
Reply

Yay!!!!!!!!im so glad you finally have the help you deserve and desperately need!

This makes me VERY happy!

1 like
Reply

( but not to hear of the heart problems, obviously... but now you are in good hands. )

1 like
Reply

I am glad your appointment went well.Obviously, they need to determine what is happening to your heart.

I wish you well.

Ros

1 like
Reply

Thank u Ros x

Reply

Yes, WendyWoo50, have heart problems. Cardiomyopathy (heart failure) from microvascular clotting. No major coronary arteries blocked, only tiny ones.

Treated with lots of meds and Syncronizing defibrillator pacemaker. It practically came on overnight.

I don’t have high blood pressure either. I did have a very fast heart rate-120 if walking across room.

If you want a list of my cardiac meds, let me know.

Nancy in West Virginia

1 like
Reply

Thanks for sharing. It's amazing how quickly it came on.

I was fine with a resting pulse of 56-60 bpm then suddenly ...

29/12 pulse 49 bpm

4/1 pulse 42 bpm

9/1 pulse 34-36 bpm

10/1 some beats 19bpm mainly 34-36bpm

Not POTS as pulse has been dropping never increasing

Now a steady 46-48 bpm

Reply

WendyWoo50,

I'm still waiting for a definitive APS diagnosis but have had multiple clotting events, PEs, dvts, TIAs and CVEs. I was diagnosed with Ehlers Danlos Syndrome prior to having any clotting events but now there is a possibility that I have systemic lupus too but I'm awaiting multiple tests for this.

I have excessively high heart rates on all minor exertion with acute breathlessness and have had a yo-yo BP with this. I used to have a low BP of around 90/70 but since all the heart rate issues it can be 158/90 for example but then low the next time, my highest was 202/120.

I too experienced and still experience multiple sprays of smaller clots like Lure2 and also like Lure2 my London based respiratory doctor thinks that I may have pulmonary hypertension again, possibly the exercise induced type, not that I can actually exercise at the moment! I have ambulatory oxygen now which is really helping my breathlessness but my heart rates are still going much too high and disproportionate to what I'm doing, like 168bpm after putting my coat and boots on.

I have been put on Apixaban but I know that I'm not therapeutic on it because I'm still producing clots in my legs that are passing onto my lungs and TIA type symptoms. I want to go back to warfarin as I used to have to maintain an INR of 3-4, preferably above 3.5 and apixaban only gives the equivalent of 2-2.5 but I'm being told that I have to take Apixaban!

Like you I have a haematologist who has ignored two positive lupus anticoagulant blood tests and ignored all of my multiple clinical features of both APS and lupus and has instead based my diagnosis solely on blood tests taken while I was on anticoagulants. I was told that I don't have APS and also told that I don't have micro clots and that I've never had micro clots!

My brain MRI from when I had my first clots on the brain says I had "ischaemia consistent with 'micro clots' and possible vasculitis" and my local respiratory doctor in her letter regarding my more recent PEs, says that I have experienced repeated sprays of small emboli. I don't understand why a haematologist would fail to acknowledge my medical history and current symptoms and I feel a bit like a time bomb just waiting to go off with either a full blown stroke, or a fatal size PE.

Do you mind me asking who your haematologist was and who you now have at Kings that is helping you, you can PM me if you would prefer. Thanks so much, Claire

Lure2,

We are so similar with the micro clots, pulmonary hypertension etc. If you're happy to share then I would be very interested to know more about your diagnosis, how did they discover the micro clots you have? I've been told that micro clots in the lungs don't show up even on a VQ scan. Please would you PM me as I don't want to hijack WendyWoo50s post. Thanks, Claire

Nancy in West Virginia,

A cardiac nurse I recently spoke to, mentioned micro vessel blockages to the heart, when we were discussing my excessively high heart rates. I've been having multiple smaller clots passing to my lungs but she said that they could also pass to the heart and then block the smaller blood vessels feeding oxygen to my heart. She said this might explain why my heart continues to be pump faster even though my finger oxygen saturation is 98%, it's because my heart still isn't getting enough oxygen.

Is this what happened to you, re your heart micro vessels? Please PM me if you would prefer to keep the info private. Thanks so much, Claire

Reply

Claire, I first had microvascular clotting in jaw bone causing trigeminal neuralgia. A biopsy of jaw bone showed avascular necrosis due to blockages in tiny jaw arterioles. I was put on Coumadin which seemed to dissolve blockages in jaw bone. Jaw pain is 90% improved on Coumadin.

My heart rate was super fast so the heart failure doctor put me on a huge dose of metropolol (200 mg) even though I do not have hypertension. A side effect of this dose is it slows heart rate and I now run 80-90 bpm mostly. I am still in heart failure but it is improved with meds, pacemaker, INR of 3-4, and thyroid meds(I think), and biventricular pacemaker.

I am in heart failure. In 2015 my ejection fraction was 20-25. That is really bad. Now it has improved to 45-50. Normal is about 70.

Have a tiny bit of pulmonary hypertension.

Also, oxygen saturation’s get low mostly at night so I use oxygen with my Bipap.

I also have vasculitis.

For 18 months I was on oxygen 24/7. My vasculitis was really bad then and I was receiving Rituxan for vasculitis. The Rituxan seemed to help my lungs also and I as able to discontinue oxygen supplementation during day.

Good luck.

You need a thorough heart workup. I hope your doctors do that.

Nancy in West Virginia

2 likes
Reply

I think this microvascular clotting in heart is called ‘Syndrome x.’

1 like
Reply

Nancy in West Virginia,

Thanks for responding, you have really been through the wars and are definitely much worse off than I am. I have had some heart tests already which apart from my small pfo hole and my high heart rates they have told me that my heart is essentially ok but I haven't actually seen a cardiologis yet. The tests were done just to make sure that I was safe to undergo a general anaesthetic for a recent operation. They hoped that the operation would resolve my oxygen problems, acute breathlessness and high heart rates but it hasn't so now I'm facing more tests. I really thought my health was going to improve significantly post op, so it's very disappointing to still feel the same. For those of us with clotting issues, it seems that we always have to face a very long journey in order to even get a diagnosis, let alone treatment and then improvements to our health.

They queried vasculitis when I had micro clots on my brain in 2007 but I don't really know what it is if I'm honest, I just know that I have sticky blood and must be on anticoagulants for life.

I think I need to see a rheumatologist who specialises in clotting and connective tissue disorders and also a cardiologist to check for any blockages that may have been caused by micro clots. Just got to figure out who are the best specialists to see. 😵

Claire

1 like
Reply

My heart rate dropped from 56-60 bpm to as low as 19 bpm never high x

Reply

Wendy, I have POTS. It is “ early and mild.” Diagnosed via tilt table testing last March. Heart issue with APS. It’s. Dysautonomia- also from brain to bowels.

1 like
Reply

I hope ur doing well and that u can be helped x

Reply

Also Wendy, my brothers heart rate was very slow and he had an ablation at Cleveland Clinic which fixed it.

Nancy in West Virginia

1 like
Reply

Wow that sounds like a quick solution. I hope he is well now. 💋

Reply

He is

1 like
Reply

You may also like...