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AVM Survivors Network

Me and my Left Arm - Considering New Treatments After Years of Wait and See

#1

I am re-posting this here to the specific arm AVM location - Apologies if you already read this in the new member intros:

Hi, my name is Kristin and I am 44 years old with diffuse AVM from the mid upper left arm down to my thumb. I have had what I now understand must have been AVM in my left arm since childhood. When I was in grammar school I developed two tumors on my tendon sheaths of my wrist that were removed, but had a high recoccurance rate. I started feeling pain in my arm and my veins started to show abnormality around the 6th grade. Around this time, I started developing limited mobility in my joints in my left arm which has gotten progressivily worse. Now my elbow is locked basically at 90 degrees.

The root of all these problems was always couched to me as lymphatic malformation. I was a patient of a well known orthopedic/oncololgy specialist in Boston, but we adopted a wait and see approach. I think this was actually good as I would consider the vein problem as a level 2 for many, many years.

I am now in LA and in the last couple of years I started noticing the veins becoming worse, so I have engaged a new orthopedic/oncology specialist (they deal with other soft tissue abnomalities - not just cancer). He has sent me to a bunch of specialists, including an ortho elbow specialist, vascular, and now an oncologist. I am a little freaked out because there is discussion of using a targeted cancer drug called votrient, as well as embolization. This drug seems very harsh and I am inclined not to do it. That said, I have developed a vascular bruit in my wrist over the last year and a half and I am freaked out that the vasular problem seems to now be getting worse for the first time in years.

This is not even getting into the ossious deformity in the elbow socket. They are looking at the bones and there is as possibly of doing an elbow surgury to fix this. I am concerned though that the surgery could make the AVM worse. Has anybody had any kind of lathroscopic surgery on a limb with AVM?

So my questions:

  1. Has anybody had targeted cancer therapy drugs to treat AVM - if so which ones? I was orgiinally sent to the oncologist to find out about MEK inhibators, but the oncologist said that was only an option for a cranial AVM because that is life threatening, now he is talking about Votrient.
  2. Has anybody had a surgery for a joint on a limb with AVM? What was the result?
  3. Is anybody else being treated by ortho/oncology specialists.
  4. Does anybody have a recommendation of an AVM centric specialist in Los Angeles?

Thanks!

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#2

Hi Kristin,
My son has an AVM on his right thumb/wrist/palm. We have met with many doctors and I hope my answers below help.

  1. A few doctors have recommended trying Sirolimus. My son does not do well with getting blood taken, so we have said no for now. He is only four years old and they would have to continually draw blood to monitor him on the drug.

  2. We have taken the cautious approach and have not done surgery. Dr. Waner in NYC was the only doctor who suggested immediate surgery.

  3. We saw the oncologist when discussing Sirolimus.

  4. We do not know a specialist in LA. We live in Philadelphia and have been to CHOP, NYC, and Nemours in Delaware.

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#3

Hi, I have an extensive high flow AVM in my left arm spanning from my thumb to my shoulder and upper back left side. A small lump on my thumb was noticed at birth. I am just reengaging with treatment after many years of no treatment. I am 41 and haven’t had any embolisations since I was 15. I am currently under the Royal Free in London UK. There is a lot of your story which is similar to how my arm has developed and the problems I am experiencing.

  1. I am on various medication including thalidomide which is now used for the treatment of some cancers as it reduces the growth hormone. (You have to get over the history of this drug as it is weird to think of being on it). I have only been on it for a couple of months and I am being monitored closely.

  2. Sorry not had joint surgery yet, but all of my bones and joints are affected and have been pulled into my AVM and so are deformed. I can not rotate my wrist and I have lesions throughout my bones.

  3. I am not under an oncologist. The vascular team are leading my treatment but the palliative care team who also work in oncology are leading my pain treatment.

  4. I’m in the UK so can’t help with the last one.

I am not sure if this helps at all.

All the best

Mary

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#4

Mary,

Welj, they came back to me with a treatment plan this week which just involves embolizaion. I think they have determined that doing the capsule release surgery on the elbow would be too dangerous right now with the current state of the malformations. Right now it is from the mid upper arm down to my thumb which has been the the location since puberty. I would say the malformation has been stable for years without any intervention. My elbow and my wrist joints are not fully functional but my fingers are and I get by. The oncologist did offer me Votrient as an anti-angiogenis drug to try, but honestly I felt like the side effects outweighed any potential benefit. I have an appt. with the Vascular Anomalies team at USC/Children’s hospital to get a second opinion as I reached out and they said they do see adults every once in awhile. I was a little bit shocked to find out that my doc at Cedars knows about this team and has worked with them on cases, but never mentioned them as a resource. I found them through internet searching. He says he didn’t know they see adults though.

I think my number one fear is the avm getting worse and progressing up my arm. I would say it has gotten worse this year, but progressing downward and more pronounced around my wrist. At what age did yours start becoming more pronounced up your arm? I am trying to understand what can cause it to change and possibly what to avoid. I was also interested to read about your joint problems. I can’t tell you how many doctors have asked ME why my joints are the way they are. This is the first year a doctor has told me that the AVM could cause the damage, but even then he was only hypothosizing that I started favoring the joints with the AVM pain as a child and then with immobilizaiton the joints were on their way to becoming contracted and stiff. Now I have bone deformity from this.

I did read that puberty, pregnancy, etc can aggravate the situation. Reading this, this was the first time that I put together that my major vein problems seemed to come on around the beginning of puberty around the 6th grade. I am realizing now that I am a woman of a certain age at 44 and wondering if I am perimenopausal and that’s what’s causeing me to have the new vein symptoms. This is on my list of questions for the USC team on the 11th.

Anyway, I am rambling - thank you so much for your replies. Believe it or not this is the first year I have been given AVM as a name to what I have and this is the first time I have been on a page like this and able to reach out to folks with the same thing.

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#5

I know this is not the same but I have calf VM. I had joint surgery on my ankle with associated. My ankle was quite unstable after years of embolization and favoring. It twisted quite easily and was a generally unstable joint. I had an ortho do reconstruction on my ligaments which went well. I had an embolization one week prior to the ortho surgery. This meant my VM was as small as possible before the procedure to fix the ankle. It also meant having my ankle flexed for 2 months in a cast wouldn’t be as painful as it is when my VM is active. Also I had reached my out of pocket max for the year so it was a good time financially to stabilize my joint and reduce additional osteoarthritis. My bones were not deformed by my VM. My VM was not in the exact location which was operated on as my VM is deep in muscle only.
This is clearly not the same situation as what you describe but just to say I’ve been treated by both my IR and an ortho at around the same time.
I hope you find relief!

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#6

Thank you for replying! So, you did not have any adverse effects to your VM after having the surgery? I am still hoping that the Ortho guys at Cedars and Kerlan Jobe will still do the capsule release surgery eventually. Either that, or I am hoping one day I will get my Luke Skywalker Jedi robot forearm, LOL! One of my doctors replaced half a dude’s pelvis recently with a prosthesis and I am thinking seriously, my elbow can’t be operated on and you did that!?

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#7

Not so far as I can tell. Again they aren’t in exactly the same place and I had quite a bit of PT afterward to strengthen the area. So far I still have the arthritis I had before but it is WAY more stable. My VM still hurts now and again so I know its not gone but it isn’t to the point of daily pain which is my marker for seeking treatment. VMs like mine are low flow and so the major issues are blood pooling/getting stuck in an area causing swelling and pain. I would guess with AVM the ortho would be worried about you bleeding out rapidly if they accidentally nicked a vessel during the surgery. My hope is that once your AVM is more under control they might be willing to reconsider. As with all vascular malformations amputation is always an option, though not without its own set of complications. It was something I chatted about with my ortho to which he suggested I see an ortho oncologist as they are more versed in limb and muscle group removal. Best of luck finding the right treatment plan!

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#8

My current doc overseeing the case and my doc growing up who monitored it are both Ortho/Oncology. I like how broad their knowledge is, but I am realizing some more specialized AVM experience is maybe necessary. I do have a vascular specialist now that he referred me to, but getting second opinions in that realm.

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#9

I just want to invite @Melissa_Joyce_Lott_C into this conversation because I think she was asking about arthritis recently.

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#10

Kristin, I think it is important for you to see the vascular team. As Rachel has indicated they typically use embolisation or sclerotherapy prior to any surgery. This controls the AVM and risk of bleeding far more. The vascular team, even if they normally work with children will not only be very familiar with the latest techniques but also be able to advise you on any other people you should see. The problem with our condition is that it is so rare as everyone is different. There aren’t really specialists but there are experienced people who have worked on them. I have had surgeries as a child which have had a negative impact on my condition now. They know a lot more now and I am also very cautious. If I know more than the doctor I start to doubt their abilities and definitely won’t let them near me. I would recommend that you read up as much as you can so you are prepared.

As a woman with an extremity AVM we are very unlucky. You are right, puberty, pregnancy, menstration and menopause all have an impact. I have definitely noticed that. I have suffered with severe pain all of my life but it has been a lot worse over the past two years. My doctors currently have been much clearer about the longer term issue that the blood supply is ultimately being cut off from the end of the arm/hand. The nature of an AVM is that the nutrients don’t get into the tissue and the blood pools and doesn’t flow properly. Therefore longer longer term we are at risk. My wrist is about four times the size of my right wrist and my AVM fingers although they move they are much smaller than the other side. They have been described as being the fingers of someone who has been paralysed. I can’t turn my hand over at all as the wrist is so large and almost fused.

It isn’t right that the way we use it causes the bones to deform. My doctors have been clear that the bones don’t form due to the lack of nutrients and poor blood supply. You need that to grow the bones properly. (My doctors at Royal Free are the leaders in the UK now and I feel that they really do know what they are talking about). My AVM has pulled in the bones, soft tissue and muscles. Basically my whole arm is probably best described as diseased by it. This means that amputation has come up. In the end it probably is inevitable, as it is doing it to itself. Very scary but to be honest the pain is so awful. So the medication I have been put on is last chance and won’t reverse the damage but may slow it down. So much to think about as I do not want to be too old if it is going to happen and options etc. (Topic for another day!)

The reason you may have been stable for a while is due to what your body has been going through but as the arm gets older the problems build up so it was probably going to happen.

It is a flaw in the growth hormone that we produce when we are in the womb to grow our limbs. This same hormone grows us during puberty but in most people and other parts of our bodies this hormone stops. In an AVM effected area it carries on. Therefore our condition continues to grow and deteriorate.

I hope this helps with a bit more context. You have one chance with any treatment as the damage can be major if they do something and it can affect the next set of options so taking time to think it through and to speak to the most experienced doctors you can is essential.

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#11

Finally - it hasn’t held me back. I have a great life. Three amazing children, a supportive husband and great career. It probably has made me more determined. X

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#12

Thanks Mary - that’s very helpful. I am being seen by the Vascular Anomalies people on the 11th.

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#13

Mary,

Great information sharing! Honestly, really helpful.

Insofar as you are describing quite a lot of bones and soft tissue involvement, have you a diagnosis of Klippel Trenaunay Syndrome, rather than just an AVM? It is possible to have “just” an AVM but what you’re describing is nearer to KTS by my reading. It just might help Kristin or others.

Very best wishes,

Richard

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#14

So, I had my visit with the Vascular Anomalies team from USC who work with Children out of Children’s Hospital here in LA (but sometimes see adults). It was pretty interesting. They we saying they thought the older area of damage around my elbow was actually vascular malformation and the new puffier area around my wrist was AVM. They are advocating not doing anything right now, unless an arteriograph could help the orthopedic surgeons delineate the barriers of the AVM and make them feel more comfortable about doing the joint repair on my elbow. That said, my initial texts with the Cedars docs sounds like they are still reluctant to do the joint surgery even with this new opinion. The new word I heard yesterday was hemartherosis or bleeding into the joint. They said they thought this is what caused the initial joint damage around my elbow and to start to becoming locked. I am one last meeting with the main surgeon from Cedars soon, but looks like I am coming out of this latest doctor odyssey with just new information and orders to wear compression.

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