Sticky Blood-Hughes Syndrome Support
8,251 members8,450 posts

blood clots lungs

Hey everybody. I joined this site a while back as my boyfriend has Hughes Syndrome. He was diagnosed with it 1 year ago and has blood clots on the lungs. He gets very out of breath and his heart beats very fast to compensate for his lungs not absorbing enough oxygen. He has been on Warferine to treat this. but sadly after one year the blood clots have not reduced or gone away. He has recently had the news that his brain is getting less oxygen and causing damage to brain cells. He has been asked to sign a form allowing them to prescribe dangerously high levels of warferine now to battle this.

Please has anyone been in a similar situation to offer advice as my boyfriend is at the point of feeling like he is not getting any better and is really down..

40 Replies
oldestnewest

Hello.

Where are you from and who is managing his APS?

It might be worth suggesting that they try him on Fragmin (low molecular weight Heparin) injections instead of Warfarin to see if that makes a difference. It did with me and I am now on Fragmin for lief and I feel much better than I did on Warfarin. I had clotting incidents on Warfarin but I have not had any on Fragmin.

Dave

1 like
Reply

My boyfriend lives in Bournemouth (and myself Exeter) and I do not know who is his APS specialist.. I will ask him. Thanks for your information; I will pass this on.

Reply

Update. Further to my reply last night thank you Manofmedip, I have passed this information on to Kevin. He has replied below :)

Reply

Hi it is so important that your boyfriend has a designated Hughes Syndrome/APS Specialist to manage his condition and help his GP look after him.

Hide South East Specialists »

NHS Specialists

Professor KevinDavies

Consultant Rheumatologist

Department of Rheumatology

Royal Sussex County Hospital

Eastern Road, Brighton

East SussexBN2 5BE

Telephone: 01273 696955

Dr Christopher Edwards

Consultant Rheumatologist

Department of Rheumatology

University Hospitals Southampton NHS Trust

Tremona Road, Southampton

Hampshire SO16 6YD

Telephone: 02380 798723

DrGillianEvans

Consultant Haematologist

East Kent Haemophilia Centre

Kent and Canterbury Hospital

Ethelbert Road, Canterbury

KentCT1 3NG

Telephone: 01227 783157

Dr Yousuf Karim

Consultant Immunologist

Department of Immunology

Frimley Park Hospital

Portsmouth Road, Frimley

SurreyGU16 7UJ

Telephone: 01276 604604

Dr David Keeling

Consultant Haematologist

Department of Haematology

The Churchill Hospital

Old Road, Headington

Oxford OX3 7LE

Telephone: 01865 225318

DrDenisede Lord

Consultant Rheumatologist

Department of Rheumatology

Kent and Canterbury Hospital

Ethelbert Road, Canterbury

KentCT1 3NG

Telephone: 01227 766877

DrDenisede Lord

Consultant Rheumatologist

Department of Rheumatology

Victoria Hospital

London Road,Deal

KentCT14 9UA

Telephone: 01304 865406

DrDenisede Lord

Consultant Rheumatologist

Department of Rheumatology

Queen Elizabeth the Queen Mother Hospital

St Peters Road, Margate

KentCT9 4AN

Telephone: 01843 225544

Dr Sam Pantakalam

Consultant Rheumatologist

Department of Rheumatology

Eastbourne Hospital

Kings Drive, Eastbourne

East Sussex BN21 2UD

Telephone: 01323 417400

Dr Sue Pavord

Consultant Haematologist

Department of Haematology

The Churchill Hospital

Old Road, Headington

Oxford OX3 7LE

Telephone: 01865 225318

DrAnnScott-Russell

Consultant Rheumatologist

Department of Rheumatology

William Harvey Hospital

Kennington Road,Willesborough

Ashford,KentTN24 0LZ

Telephone: 01233 616267

DrAnnScott-Russell

Consultant Rheumatologist

Department of Rheumatology

Royal Victoria Hospital

Radnor Park Avenue, Folkestone

KentCT19 5BN

Telephone: 01303 850202

DrAnnScott-Russell

Consultant Rheumatologist

Department of Rheumatology

Buckland Hospital

Coombe Valley Road, Dover

Kent, CT17 0HD

Telephone: 01304 201624

Dr Hassan Shehata

Consultant Obstetrician

St Helier Hospital

Wrythe Lane, Carshalton

Surrey, SM5 1AA

Telephone: 020 8296 2542

DrRobinWithrington

Consultant Rheumatologist

Department of Rheumatology

William Harvey Hospital

Kennington Road,Willesborough

Ashford,KentTN24 0LZ

Telephone: 01233 616221

DrRobinWithrington

Consultant Rheumatologist

Department of Rheumatology

Kent and Canterbury Hospital

Ethelbert Road, Canterbury

KentCT1 3NG

Telephone: 01227 864233

Dr Sarah Woodham

Consultant Rheumatologist

Department of Rheumatology

Hillingdon Hospital

Pield Heath Road, Uxbridge

Middlesex UX8 3NN

Telephone: 01895 279682

Hide South West Specialists »

NHS Specialists

Dr Sarah Allford

Consultant Haematologist

Department of Haematology

Musgrove Park Hospital

Parkfield Drive, Taunton

SomersetTA1 5DA

Telephone: 01823 333444

DrSarahBartram

Consultant Rheumatologist

Department of Rheumatology

Salisbury District Hospital

Odstock RoadSalisbury

WiltshireSP2 8BJ

Telephone: 01722 429217

DrAndrew JohnBell

Consultant Haematologist

Department of Haematology

Poole General Hospital

Longfleet Road, Poole

DorsetBH15 2JB

Telephone: 01202 665511

Dr Frank Booth

Consultant Haematologist

Department of Haematology

Weston General Hospital

Grange Road, Weston-super-Mare

Somerset BS23 4TQ

Telephone: 01934 636363

Dr Desmond Creagh

Consultant Haematologist

Department of Haematology

Duchy Hospital

Penventinnie Lane, Truro

Cornwall TR1 3UP

Telephone: 01872 252501

Dr Paul Creamer

Consultant Rheumatologist

Department of Rheumatology

Southmead Hospital

Westbury-on-Trym

Bristol BS10 5NB

Telephone: 01174 141614

DrJeremyHobart

Consultant Neurologist

Department of Neurology

Derriford Hospital

Derriford Road, Crownhill

Plymouth, DevonPL6 8DH

Telephone: 01752 202082

Dr Sally Knights

Consultant Rheumatologist

Department of Rheumatology

Yeovil District Hospital

Higher Kingston, Yeovil

Somerset BA21 4AT

Telephone: 01935 384955

Dr Ellie Korendowych

Consultant Rheumatologist

Department of Rheumatology

Royal National Hospital for Rheumatic Diseases

Upper Borough Walls, Bath

Bath BA1 1RL

Telephone: 01225 465941

Dr Jason Mainwaring

Consultant Haematologist

Bournemouth and Poole Haemophilia Centre

Royal Bournemouth Hospital

Castle Lane East

Bournemouth, Dorset, BH7 7DW

Telephone: 01202 704786

Professor Neil McHugh

Head of Connective Tissue Diseases

Department of Rheumatology

Royal National Hospital for Rheumatic Diseases

Upper Borough Walls, Bath

Bath BA1 1RL

Telephone: 01225 46594

DrTimNokes

Consultant Haematologist

Department of Haematology

Derriford Hospital

Derriford Road, Crownhill

Plymouth, DevonPL6 8DH

Telephone: 01752 202082

Dr Samir Patel

Consultant Rheumatologist

Department of Rhematology

Southmead Hospital

Westbury-on-Trym

Bristol BS10 5NB

Telephone: 01174 141140

Dr Wayne Thomas

Consultant Haematologist

Department of Haematology

Derriford Hospital

Derriford Road, Crownhill

Plymouth, Devon PL6 8DH

Telephone: 01752 437041

Dr Alistair Whiteways

Consultant Haematologist

Department of Haematology

Southmead Hospital

Westbury-on-Trym

Bristol BS10 5NB

Telephone: 01179 505050

MaryF

1 like
Reply

hi, I tried to get hold of doctor bell in poole hospital. they said he wasn't there anymore. he was also listed at a private hospital, but he is not their either

Reply

Please try another from the list, that is your best bet. MaryF

Reply

Hi Mary, please can you tell me who is a specialist in the Leicester area, having difficulty getting INR right and been pretty poorly over last few weeks. Been working but a real struggle.

Thanks Dean.

Reply

NHS Specialists

Dr Khalid Ayes

Consultant Stroke Physician and Lead Clinician

Kettering General Hospital

Kettering

Northamptonshire NN16 8UZ

Telephone: 01536 492000

Dr Richard Gooding

Consultant Haematologist

Leicester Royal Infirmary

Infirmary Square

Leicester LE1 5WW

Telephone: 0116 2586534

Dr Rachel Jeffery

Consultant Rheumatologist

Department of Rheumatology

Northampton General Hospital

Cliftonville, Northampton

NorthamptonshireNN1 5BD

Telephone: 01604 545654

Dr Karyn Longmuir

Consultant Haematologist

Kettering Haemophilia Centre

Kettering General Hospital

Rothwell Road, Kettering

NorthamptonshireNN16 8UZ

Telephone: 01536 492000

Dr Bethan Myers

Consultant Haematologist

Department of Haematology

Lincoln County Hospital

Greetwell Road, Lincoln

Lincolnshire LN2 5QY

Telephone: 01522 512512

Dr Bethan Myers

Consultant Haematologist

Department of Haematology

Leicester Royal Infirmary

Infirmary Square

Leicestershire LE1 5WW

Telephone: 0116 2586534

Dr Marian Regan

Consultant Rheumatologist

Department of Rheumatology

Royal Derby Hospital

Uttoxeter Road

DerbyshireDE22 3NE

Telephone: 01332 788380

Dr Marian Regan

Consultant Rheumatologist

Department of Rheumatology

London Road Community Hospital

Devonshire House, London Road

DerbyshireDE1 2QY

Telephone: 01332 347141

Dr Gill Swallow

Consultant Haematologist

Department of Haematology

Queens Medical Centre

Derby Road

NottinghamNG7 2UH

Telephone: 01159 249924

Dr Sonia Swart

Consultant Haematologist

Department of Haematology

Northampton General Hospital

Cliftonville

Northamptonshire NN1 5BD

Telephone: 01604 545839

Dr James Taylor

Consultant Rheumatologist

Department of Rheumatology

Northampton General Hospital

Cliftonville

Northamptonshire NN1 5BD

Telephone: 01604 545647

Dr Yunus Tayob

Consultant Obstetrician

St Albans City Hospital

Waverley Road, St Albans

Hertfordshire AL3 5PN

Telephone: 01727 866122

Dr Christopher Tew

Consultant Haematologistt

Department of Haematology

Lister Hospital

Coreys Mill Lane, Stevenage

Hertfordshire SG1 4AB

Telephone: 01438 781149

MaryF

Reply

Oh brilliant, my specialist is Dr Gooding and I'm seeing him at the end of this month.

Many thanks Mary

Ps my new employer was asking if my condition is covered under the DDA can you advise what I say please?

Thanks again

Dean

Reply

I think if you are disabled, for whatever reason it will be taken into account! MaryF

Reply

My dear, I am so so sad to read your message - you must be feeling helpless. I have no words of help, beyond that you must talk to a phlebotemist consultant without delay. Ring your hospital & get reception to out you through to the right dept, & get the help you need. It truly is a case of fighting to speak to the right people - it can be difficult, I do know, but wish I had done this one thing years ago. I too had clots on the lungs, & have been on 5mg Warfarin for nearly 5 yrs, now stable. I have lupus, Hughes' syndrome (a surviving patient from Graham Hughes' original research programme from the early '80s), am also a coealiac! Have chronic osteoporosis, hyperthyrodism, & have had a lifetime of chronic bowel problems having just had Delorme's operation in June which was really awful - I am nearly 85 but think it really helps to be positive & forget

1 like
Reply

thank you for your reply Claire.. it is good to know that you have been on 5mg Warfarine and it has helped you remain stable x

Reply

Hi purplestars,

Your boyfriend can be happy to have you to help him!

The problem is that he needs a SPECIALIST of APS. That is a must! Sometimes we need high levels of Warfarin as we have so thick blood. I guess they have suggested an INR around 4.0. I have that. With the right Specialists (even an lung/heart Doctor) around him he will manage as he also is young and has had it only one year.

I have Pulmonary Hypertension and leaking heartvalves and do Ecocardiographies every year. The Doctors are nowadays so skilled on lung and heart-issues so with the best Doctors he will be fine I am sure.

Very good advices from Mary and Dave also and a list of different Specialists. When you ask about the name of the Doctor who is with your boyfriend at present. also look at the list if he is on it perhaps.

Best wishes from Kerstin in Stockholm

1 like
Reply

Thank you for the reply Lure2

Reply

Twelve years ago I had a stroke after experiencing shortness of breath, undiagnosed by my GP at the time. Whilst under the care of a London hospital,many clots were discovered on my lungs and I was subsequently diagnosed with APS.I made a good recovery from the stroke and I am now taking warfarin which is very well managed by my present GP and Dr.H.Cohen at U.C.H. Fragmin was necessary for me recently whilst undergoing minor surgery. Warfarin has to be taken with careful management and understanding on behalf of the patient.It seems to me that your boyfriend has not received the correct management and guidance necessary for living with APS and the necessary treatment.Hope all goes well.

1 like
Reply

thank you for your supportive message jagessar.. glad you are doing well.

Reply

Thank you so much for all of your replies.. I have only just seen the replies..... my boyfriend is also a member here and reading the messages gratefully... hopefully he will find the emotional strength to open up on here too soon xx

This afternoon I will take time to read all and answer properly .. take care all and have a good day...

Reply

When I asked b/f this morning as to who his APS specialist is the answer I have just got (via skype message) and its easier just to paste this rather than rewrite: (and he doesnt mind me doing this btw)and please bear in mind that he gets brain fog sometimes :) :

APS SPECIALIST

"I don't have one- i was supposed to see doctor Mainwaring, who is a haematoligist, but instead i got the same lung specialist i have had all along, the one who has got so much wrong. he says he talks to dr Mainwaring about me but he never asks about anything but my lungs, and seems uniterested in the side effects bear in mind he was the one that had me on rivaoxaban for 8 months, when it wont work with my illness - not even cleared for treating aps ( i didn't know that then)- I think my specialist is doctor hughes, not sure though..."

When asking about Warferine the answer is:

"they are aiming for an INR of 3- 3.5. however after the lung perfusion test they have asked for it to be upped a lot. the amount i take varies week to week, but usually about 4mg"

He is only on Warferine for treatment at present and painkillers.

I have suggested he ask about oxygen bottle as treatment due to his low brain oxygen levels.. he was offered this a year ago but declined, but I think it would be a good idea. so he is going to ask doctor about this too..

Reply

Hi. I am purplestars boyfriend. My name is kevin. I have looked on posts here before, infact a lot of what I have learned about aps has come from here.

My lung clots were first diagnosed October last year. That was after a long period were my doctors said my problems were stress and anxiety. I pushed and pushed, and oddly enough the day they said I might have clots was they day after I collapsed on a short walk to my local shop.

purple has told you my medications. Too say I am frustrated about my recovery would be an underestimation.

saw a doctor a couple of months ago that said unless they could control of my clots I would have the hear and lungs of a 70 year old in 2 years. not a nice though (unless you are 70)

running out of options and ideas. thanks purple for posting

2 likes
Reply

Hi Kevin

It might be worth you going to see Prof Hughes or one of his team, privately, at The London Lupus Centre, London Bridge Hospital.

I stick with my earlier suggestion that you ask for a trial of Fragmin, as an alternative to Warfarin.

Dave

1 like
Reply

I called them, their fees are out of my range unfortunatly

Reply

Ah, ok. well, you could still ask whoever is managing your APS if they would put you on a trial of Fragmin, perhaps for six weeks.

Dave

Reply

You will get good advice from the administrators on this site. They are VERY knowledgeable and helpful.

Reply

thank you Baba

Reply

I have left a message for doctor Mainwaring. I know his name and he is on the list posted. hopefully he will call back

and thanks everyone for your replies, and thanks purplestars for finally making me do this :-)

1 like
Reply

totalus if you don't get a response from the secretary of Dr Mainwaring then you should urgently consider another name on the list regardless of if it means you having to travel, as your condition urgently needs to be reviewed and if it were me I would not be hanging about. I'm not impressed frankly with this Dr for not wanting to see somebody in your condition so I'd feel happier if you did see somebody who agreed to see you more urgently and who does NOT want to give you any oral anticoagulants as you do not fit the criteria for the only one that has been tested thus far for APS.

Personally I think you should be under Prof Hunt at St Thomas and you should ask your GP for an urgent referral there tomorrow given your condition!

Please keep us updated and do ask for help if you are getting nowhere.

2 likes
Reply

Thank you APsnotFab - your reply was valuable

Reply

😇

Reply

Ask and acquire Fragmin shots immediately unless you have some health condition that would not allow it.

1 like
Reply

thanks for all the advice. I have tried some other doctors today, but none have comeback. I wont give up though :-)

regards the hughes hospital, is the treatment that much better?

will ask about fragmin when I finally get a doctor that will help

Reply

UPDATE - they are increasing my wharafrin to try and get INR of between 3.5 and 4.5 -target 4. It was previously 2.5 to 3.5. They also want to check me twice a week.

I have made contact with the Nuffield private hospital with a view to getting a second opinion.

Thank you all for your support and advice

Reply

Do push for a Fragmin trial please, Professor Hughes himself advocates this when Warfarin is not suiting an individual patient. MaryF

Reply

Hi, I wonder if you have tried to get a referral to Prof Hunt as your Specialist APS-Doctor?

Yes, some Specialists are better than others and prof Hunt is one of them. APsnotFab suggests this doctor and an urgent appointment. I would definitely follow her advice. You should have the best - it is your life.

Why I say this is that I have been on this site for at least 4 years and for one year I was also an Admin and I trust the Admins 100 %. We both know that we need a Specialist for our illness.

As to the Warfarin I have today an INR between 3.5 - 4.0. I get a Fragminshot if the INR goes to low. I have Pulmonary Hypertension from this illness.

I get frightened when you do not take your situation seriously. I know what I am talking of!

Kerstin in Stockholm

Reply

Yes totalus this would be a very very good idea. MaryF

Reply

thank you I will pass this on to Kevin

Reply

Good news guys - Kevin has received a call from Dr Mainwairing and he is taking over the case and proceeding to do other tests.. all is going in the right direct it seems.. I, in the meantime, will remind Kevin of the helpful suggestions you have all put above and ask him to consider this.

2 likes
Reply

That's great news. All I would say is that if Kevin was prepared to go private then I would go to the best and that would be Prof Hunt. If you want her private contact details just in case to make an appointment, then please let me know, you can always PM me.

1 like
Reply

UPDATE

Dr Mainwaring has been great. He has asked for my vit D and calcium levels to be checked. Already requested an appointment with a rheumatologist, and also things they need to investigate Fibro Myalgia. Even got a personal letter from him the post today.

All of a sudden I am feeling much more positive !

I want to give hima try before I look at going private.

Reply

Thats great and I hope it works out... but please be aware that Prof Hughes often talks about Fibro being either Sjogrens or Thyroid problems or even both. These two conditions often run alongside APS but the tests in particular for thyroid are bad and you would need in-depth ones done probably by an Endocrinologist to be sure nothing is going on. If you suffer dry mouth/eyes the sjogrens is a likely cause.

Reply

PTE Surgery

Dear purplestars - my 26 y/o daughter had this same described condition until 3 months ago when she had surgery to remove PE (blood clots in her lungs). She was mostly being seen prior to then only by Rheumatology specialists at 6 top medical institutions across the country over an 18-month period, who all unfortunately completely missed what was actually going on. I only say this to exemplify how elusive what I am writing to you about, is.

There is a rare surgery being done called "PTE Surgery" which is short for Pulmonary Thromboendarterectomy. This is a lifesaving procedure that is only done at 3 or 4 hospitals here in the USA that REVERSES lung blockages due to blood clots (pulmonary embolisms, or "PE") by surgically removing them. When it gets to the point that your bf's lungs are not getting enough oxygen, that is seriously important. A year ago my 26 y/o daughter presented with exactly what you describe. Blood clots in both her lungs & pulmonary arteries were discovered to be blocking oxygenated blood from the heart. She was dizzy, even passing out sometimes as it became harder & harder for her to breathe, even 'on the flat': shortness of breath became the norm. Importantly, your bf must be seen asap to rule it in or out. Please see links below at the very end of this post for info.

* In the UK, Endarterectomy is being done at Papworth Hospital - a very good resource to call for help.

* Link: academic.oup.com/ejcts/arti...

Having just spent the past year as the mother of a daughter with PE blockages and finding a solution to save her life, I urge you to have him seen by a pulmonary SPECIALIST.

Also, for others reading this post, know that difficult breathing is often misdiagnosed as general "COPD" when instead it is actually blood clots in the lungs (see below links) that many of us are unaware of having. The trick is to see a pulmonary specialist who can determine through CT scan whether you or your loved one has PE (blood clots) & therefore CTEPH: (Chronic Thromboembolic Pulmonary Hypertension). This hypertension is deadly; it is the result of PE blockages in the lungs, eventually causing dizziness, passing out, etc. Symptoms are imperative to heed: the right side of the heart is trying to pump oxygenated blood to the lungs but encounters resistance from the blockages; the heart meanwhile enlarges. Eventually the patient suffers heart failure. Therefore, the clots must be removed asap. And if the patient is not yet on anticoagulant therapy, it is mandatory protocol to prevent new clots.

*A red flag: IF he possibly has CTEPH from blood clot blockages, higher doses of Warfarin is not the path. There are lifesaving meds called vasodilators that are instead used specifically. Please see: "ADEMPAS". Pulmonary specialists at Papworth will know this.

Sadly, some people (just like my daughter before we found all this out a year ago) don't even know that they have PE, or pulmonary blood clots.

The only hospital here in California that does this lifesaving surgery is at the University of San Diego Sulpizio Cardiovascular Center. It has become the go-to place in the country for PE, PTE, CTEPH (please google to learn about all this, see if it may be a fit).

Below is a link for more information about CTEPH ((Chronic Thromboembolic Pulmonary Hypertension) which, again: occurs as a result of blood clot blockages blocking blood flow from the heart. I'm repeating it because I know there's a lot in this post, I know it can be confusing.

UC San Diego Med Center is trying to get the message out to the world about CTEPH, as patients are often misdiagnosed with "COPD" because Xray will not usually pick up blood clots in lungs, and unfortunately most general family doctors stop there. However, the concern is that there are many people walking around with pulmonary blood clots that will never know it, and therefore never get better.

It is imperative for anyone reading this who has these symptoms to take it further yourselves and request a referral to a pulmonary specialist immediately. Again - I took my daughter all over the country for help before an astute doctor changed our course and urgently referred her to a pulmonary specialist, who immediately discovered her CTEPH. No one else had even considered it so until a year ago, we knew nothing about it either and had seen many doctors. She is now 3 months post-PTE surgery and can breathe again. It has completely changed her life. She is no longer bed-bound on oxygen, and no longer afraid. She's happy. She gratefully has her life back.

Please Google all of these terms for yourself to read more: CTEPH, Thromboendarterectomy, PTE

* Link follows validating all of the above - scroll down to "CTEPH - Getting The Right Diagnosis": tinyurl.com/mjzv84p

* Link follows re: UCSD, their attempt to get the word out, and the pioneering surgeon Dr. Madani who just gave my daughter's life back to her: tinyurl.com/y97aq6ev

* Wikipedia's definition: en.wikipedia.org/wiki/Pulmo...

I'm not saying this is what he has, I'm just saying this is how my daughter presented and I am trying to help. No matter what, your bf needs medical care from a pulmonary specialist familiar with CTEPH.

If we can help just one person from what we have learned & experienced, then this long post was well worth it.

Thank you for reading my post,

Helen

Reply

You may also like...