My MDT meeting news

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Hi everyone,

So finally time for an update!

I had a MDT meeting last week.

The news was not ideal. I was told that my AVM is still a grade 4 and still the same size. What the embolisation did was suceed in reducing the flow considerably.

The AVM is still very complex, it has been glued in the centre and now has bits of AVM around that central part. Sort of looks as if I have 3 AVMs around the centre gluey part.

There are many feeders, too many to count. This includes two main arteries: Posterior-Cerebral and Middle-Cerebral.

In terms of treatment:

  • radio-surgery (Gamma Knife) is not an option. My AVM is far too diffuse in structure or “bitty” as they discribed it. My AVM also has lots of glue in it which reduces the effectiveness of this technique. It will not fully treat my AVM.
  • Standard Embolisation is not an option because my AVM has too many feeders.
  • Craniotomy is not an option. The fact that I would 100% have total right side visual field loss in both eyes is not the concern. The risk of a death is far too high to consider it.

The only potential option is trans-venous embolisation. This is a very new technique, only 100 or less recorded cases. The AVM is glued via the “drainage” veins side only. The leading neuro-radiologist in the UK in this area, Dr Pete Cowley, was at the MDT meeting. He does not feel comfortable with operating on me because the risks of this option is so far unknown. If it went wrong I would be highly likely to die or be considerably disabled. As far as he knows so far only AVMs with a single drainage vein have been treated this way (I have several) and my AVM is larger and more complex.

Dr Cowley said if it was him or his close family there are only two other people whose opinions he would trust. They are both at the forefront of research and experience in treating AVMs via trans venous embolisation: Prof Charbel Mounayer in France and Prof Rene Chapot in Germany. I am going to call in to request for both their opinions. No idea about costs abroad, I have no private health insurance and fully rely on the NHS. I have little expectation but atleast I will have peace of mind once I have exhausted all my options.

I will have to mention to these specialists though about my radiation exposure. During my embolisation I was exposed to 12 gray which is over 6 times the maximum standard for this procedure. I will have been exposed to slightly more than 12 gray when including angiograms. Above the 20 gray threshold and I have a high likelyhood of damage to vessels in my brain and potentially begnin tumours over time. Also for angiograms and embolisation higher radiation output will be required to see through the huge amount of glue. When considering trans-venous embolisation, radiation exposure will be particularly important.

Unless anything changes, I will be monitored annually from now on. I have been told I can carry children so far as I have a relaxed pregnancy and have a C-section. I am allowed to do all activities except from contact sports and heavy lifting. So I just carry on with life now I know where things stand!

It is disappointing but also somewhat a relief to know where I stand so I can make plans moving forward. I am going to see my neurologist, Dr Alexander Leff. He will help me with visual processing(his specialism) and concentration rehabilitation. I saw him earlier last year but he asked me to see him again once I had my angiogram and MDT meeting. He will also either help me with my chronic fatigue management or refer me to a occupational therapist in this area. Action is being done to help me start my career working to my best capability!

I also have non-AVM health stuff going on. I am seeing a endocrinologist on the 21st this month following multiple blood tests showing raised levels of prolactin (changed since this was last checked out 3 years ago). Since I have additional symptoms potentially linked to me having a Prolactinoma (begnin pituitary tumour) they want to have an indepth look. Hopefully I get the all clear though!

Other than that, my poor ankle is recovering after completely rupturing my ligaments back on NYE - started the new year on the wrong foot aparently :laughing:

Belated Happy New Year to all!

Corrine

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Hello!

Wow! Well, the main things I get from this are:

  • Its good to hear you sounding positive about life. You’ve not got the best deal but it is really good to maintain a positive view and be saying to yourself what you can do (e.g. have a family at some point) rather than be weighed down by what you might not do. I think it does you the greatest credit to be so positive. The other really positive person I’ve read in recent weeks is @LoganW. You guys are top of the class there!

  • I love the fact that Dr Leff wants to see you. For all of the difficulty that some people have in getting interest from doctors, it sounds like you’ve got a great team there and they are out to do the best for you that they can. Again, really positive to my eyes. It probably means they hope to learn more from you than a dozen other people (which is never quite a positive thing!) but it does mean they sound like they are really interested and will do their best. Love it.

  • Sorry to hear you’ve had such a rubbish start otherwise to 2019. I can only hope it all gets better from here on.

Really good to hear from you and really good to read your positive tone!

Lots of love,

Richard

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@corrine I am so sorry that about your complex AVM. Did you ever send your scans to Barrow in Arizona for a second opinion there?
I know its rare but between 2012 and 2015 while I waited for my brain to heal ( had a CVST stroke in 2011 ) Had my 1st embolism in Jan 2011 and then had another stroke - my AVM /DAVF was complex and they wanted to go right back in …but I felt terrible and needed to go back to work in order to keep my insurance.
So when they went back in some of my DAVF had sealed itself. now the part that is still open they cant reach without possibly paralysis so we just keep an eye on it for now.
So I pray the same happens for you. I do know another person on here this also happened to and I do think it was also a DAVF.

I am so sorry about your ankle!

Hopefully this year turns out to be better!

Happy New year!
Hugs
Angela

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Hi Corrine:
You have had a tough go of it, but you are one of the most positive people I’ve had the pleasure of corresponding with! Your positivity propels me and your fellow AVM-ers forward. If you can handle all your stuff, I know I can try to handle mine. Wishing you and yours a Very Happy New Year, and may God continue to Bless you.

Sharon D…

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Hi all!

Sorry for such a delay in response. Been a bit overwhelmed with job applications! Hoping to get one as soon as possible - very ready to fly the nest :baby_chick:

@DickD for sure - I feel very relieved to finally know where I stand regarding my AVM going forward! I feel very lucky to apear to be top priority to all the medics (some of which aren’t even related to AVM stuff). It is good that now I have an answer, all priority is being taken for getting me as good as I possibly can be rehab wise.

My main hope is to help manage my chronic fatigue which apears to be my main issue. I can’t be going for nap breaks when I have a 9-5 :joy: I feel positive that we will make progress with that.

@Angela4 I am hopeful that in future, once experience of trans-venous embolisation has been more extensively used that I could have treatment this way. There are only two leading specialists in that technique so I need to get round to sending my scans to them. Hopefully they might keep me in mind when their ready. For now, it’s a matter of being smart with my health and getting on with life :raised_hands:

It would be amazing if it did close up but since it’s an AVM covering the entire lobe… :frowning_face:

I had an appointment with a endocrinologist regarding possible issues with my pituitary glad and thankfully was given the all clear!

I have had several tests and visits to the Gastro team regarding constipation struggles (TMI sorry). Turns out I have a slow digestion transit and muscle issues causing it. I’ve been put on a limited diet to try to fix this but struggling to commit (gluten free? Really)

I have also seen a Rheumatologist to talk about my joint issues. I have officially been diagnosed with Joint Hypermobility Syndrome. I have literally every symtom - some of which would have never come to mind. This includes slow digestion transit! My Rheumatologist is a little concerned and is going to discuss with my neurosurgeon team about whether I need DNA tests. Apparently there is a gene that can be a link between vascular issues and hypermobility. He thinks it’s unlikely though and that I won’t need to be tested :crossed_fingers: I am being sent to a training cause and then into physiotherapy to help with my joints.

Completed therapy (yup third therapist) week before last. Had alot of family drama but hopeful that once I move out, some space will help my relationships with them.

Definently feeling positive. Just feeling super keen to finally get a job! Been having loads of interviews - time to find one I feel is right for me :crossed_fingers:

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Morning! Its great to hear you!

The one thing I know connected to hypermobility is Ehlers Danlos Syndrome and @Angela4 and @Kirsty79 will be your friends there.

Lots of love and very best wishes with the job hunting!

Richard

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Hi Corrine. Myself and my daughter are waiting for a diagnosis for a connective tissue disorder. Initially the genetisist believed that it was ehlers-danlos, possibly vascular or classical. My daughters results have come back as a mutated smad3 gene which indicates loeys dietz which is very similar to vascular ehlers-danlos. My genes are being tested now to see is it can come upp with any answers as my daughter has symptoms thay aren’t associated with loeys dietz. I know what you mean about having symptoms thst you would not think would be associated with the disorder. I hope you get some joy with a second opinion.
Kirsty

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Hello @corrine Sorry for my delayed response I was in the hospital trying a Ketamine IV infusion for pain. More on that later…
As Dick has mentioned I do have Ehlers Danlos which is a hypermobility issue it’s a genetic disorder- I have type 3 which they have not found the genetic marker for it.

If you are Face Book there are some good groups. If not

https://www.ehlers-danlos.com/patient-support/

Slow motility is super common with us EDs
So is having vitamin deficiencies
Usually taking magnesium at night and coconut oil either in pill form or just adding 1 - 2tsp a day to your coffee or tea will get things moving naturally.

Many people do feel better doing gluten free but some don’t.

Many people have also POTs and Mast Cell issues as well they call this the trifecta.
They feel better going on a low histamine diet and taking Zyrtec and Zantac
You can find videos by Dr Anne Maitland who is the number one dr on Mast Cell and EDs.
Building our core is key , so stuff like Pilates and doing therapy in a warm pool.
Never push yourself.
Never yoga.
Regardless if you don’t have the vascular EDs every type of EDs has risk of tearing veins and arteries . Also ligament and tendons . We tend to fracture more instead of breaks.
We are not supposed to have steroid shots.
We are not supposed to have have antibiotics in the cipro family.
Lidocaine does not usually work on us.
We tend to be more sensitive to drugs.
Please feel free to ping me about anything.
Hugs Angela

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Hi Angela,

Amazing to hear from someone with the same condition! I’m not sure whether my condition is hypermobile EDS or joint hypermobility syndrome, my memory is a bit poor of the day! I’m not sure what the differance is between the two? I am waiting on getting a letter back from the Rheumatologist which will give me the formal diagnosis name. I have every single symptom for the hypermobile EDS except I don’t have any prolapsed organs or bladder weakness.

I may get tested for the vascular form, but hopefully it won’t be necessary. Since I only have one AVM apparently very unlikely.

I take magnesium already - do the tablets even absorb? Or should I try to find another form of suppliment?

I was using MCT oil (the active component of coconut oil I beleive) which did actually help with concentration and energy. I need to get back to using it - I stopped because I found it so disgusting to take :mask:

I’m hoping the physio will help strengthen the muscles around my joints. Spraining all the time is a real pain. Definently think I am more sensitive to medications which is annoying.

The thing that bothers me most of all is my digestion. My digestive tract is really slow and my rectal muscles don’t function well. Constipation is awful. I can see my food sitting there when I’ve eaten, it’s not gassy bloating, just my food not being absorbed at a normal pace. It is difficult getting the right balance with fibre, either too much or too little. The only time I get anxious about my AVM popping is all the straining :sweat:

Have you got issues with this too? Have you found ways to resolve this?

Really appreciate your response and advice!

Hugs,

Corrine

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Kirsty,

Thank you for sharing! I’ve been referred to a gastro consultant at UCLH who is particularly specialises in EDS, hoping to get advice from her and see if she confirms things. My main concern is my digestion really.

It is interesting looking into potential genetic links! My Rheumatologist is considering testing but don’t know the decision on whether or not he and neurosurgeon think it’s necessary. Honestly… I don’t know whether I want to know. I know it would be good to know to take into account when planning for pregnancy.

Have you found out whether it is possible to isolate this gene it it does turn out to be inheritable for conception?

Have you had your results back?

Best wishes,

Corrine

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Hi Corrine. If it was me then I would always want to know if there are any genetic links. I hate waiting and not knowing or having a name for the condition. I haven’t had my results back yet, apparently there was a problem with the equipment. My daughter had an echocardiogram which shows she has aortopathy so will need regular scans. She was meant to have an angiogram that was organised by the neurologist but both myself and the genetisist had concerns because sbe hasn’t had an mra to see what her blood vessels are like. The neurologist finally agreed to postpone the angiogram until she has had a scan. It doesn’t give me much faith in the neurologist. I have asked her secretary to ask the neurologist to contact me to see whether my daughter could have myoclonic epilepsy but she hasn’t.

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Hi Richard,

Thankyou for linking me up! Looking forward to getting a letter from Rheumatology in regards to the appointment to see the conclusion. Will be good to have some answers. Will be seeing a gastro specialist in EDS so think that should be useful for advice and answers I hope!

Job hunting has been slow but have a promising interview for a job I actually want (unlike the others). Hopefully good news soon for me to update with.

Love,

Corrine

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Hi Kirsty,

I know deep down that you are right. I just feel alot happier with it being “just” a random mutation rather than something inheritable. Conceiving kids is a really important thing to me, something I always wanted. Living with the AVM for life is already a spanner in the works in terms of saftey for pregancy. Hopefully there is nothing I can pass on but I know you are right to make sure.

I’m sorry to hear about your daughters diagnosis. Definently seems irresponsible of your neurologist not showing extra precaution in terms of double checking especially since MRA’s are pretty accessible. Good your put your foot down. I have my fingers crossed that the MRA will be out of the way very soon and the results come back clear. Is it possible to be transferred to another neurologist if your current one doesn’t become more reassuring?

Very frustrating that you’re not hearing back from your neurologist about potential of myoclonic epilepsy. Hopefully a load of nagging will get her on it (sucks when we are forced to do that).

Hope they get the genetic testing done right this time, waiting really is awful. Please let me know how things go when you get updates.

Corrine

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Thanks Corrine. When it comes to starting a family with eds I think its important to know what you’re dealing with for yourself and any future children. The neurologist was told by the genetisist that it isn’t vascular eds so she felt ut was safe to proceed with the angiogram. I told her that it looks like its loeys dietz which is the equivalent of vascular eds but I don’t think she has heard of it before. At the multi department meeting the other consultants said we done the right thing with postponing it. The neurologist didn’t want mri/mra due to the radiation but that is the least of my worries when you look at the risks involved with angiograms

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Do you know whether hypermobile EDS is inheritable? Highly unlikely to have vascular EDS since I have none of the symptoms other than an AVM but worth a check. I am hoping it is joint hyper mobility syndrome instead :crossed_fingers:

I will request a genetic test for vEDS and HHT, regardless of likelihood, better to get it ticked off the list and out of mind. If I have either I will then look into genetic testing for pregnancy to prevent passing it on. I hate the idea of it but it’s one of those things I should not ignore if the opportunity to check is there!

Yeah all things considered, I would personally not be too concerned about radiation from an MRA. I agree that an Angiogram is something far more important to consider in terms of risks.

Hope they follow through with getting the MRA done and investigate the loeys dietz further!

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Yes it is hereditary but different types of eds can be within the same family. The genetisist is doing splice testing on my daughter next week, whatever that is lol

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Hello @corrine
A slow digestive System is sadly normal for us EDs regardless of type.
Along with SIBO & Gastroparesis.

We really aren’t supposed to have colonoscopies and scopes so if they can do a stool sample test first that is best.

If they are a motility dr they will probably start with the radiated egg test where you eat eggs that have radiation and then they scan you to see how fast / slow you are digesting them.

So things that help is coconut oil or MCT oil daily.

Also smaller meals more frequent meals.

Drinking lots of waster .

I take a slow release magnesium

Some others take a lot of supplements
There is a link I am blanking on now but will get back to you.
But only start one new thing at a time and do it for two weeks so you know what is helping and what is not.

We tend to need electrolytes as well since we don’t absorb nutrients which has to do with our small intestines.

I like NUNN non caffeinated, they come in a tab form so I can travel with them . But you can find so many off Amazon.

We also tend to have more food sensitivities which many not be true allergy-

I had a food sensitivities test years ago and that showed like 20 foods that were making sick that once I stopped eating for a few years some I knew where true allergies like shellfish, milk, wheat , but some my dr said were cause I was just eating the same foods every day.

Here is the company that did my test but I am sure now there are more

Also if you are on Facebook there are Ehlers Danlos groups on there and some are very supportive.

All types of EDs are inherited and they will tell you it’s 50/50 that it will be passed down.
Personally I think it’s just how strong it is in the family. In my family it’s strong. But with proper management you can still live a full life.
My sister who is my half sister both have it but she has it way worse than me and since she was young. I thought she was lazy and more of princess type .
We are 15 years different and I we grew up totally different where I grew up in San Francisco walking the hills and playing sports , had twin step brothers and was a Tom boy. I played soft ball on a coed team until I was 38 when I got married.

My sister was always sick and always complaining of body pain.

She was the first to get diagnosed then my mom then me, then my Aunt andall 5 ofher kids.
Then we thought clearly our grandma had it but never complained about it .

My sister had a son who clearly has it he was born with a hernia and hip issue.

But kids are born with issues every day and the earlier we know that our kids have EDs the better we can help them.

Oh another thing that can be impacting your digestive system is your blood pressure. Is yours low?

Angela

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Hi Angela,

I’m so sorry for such a late reply. Not been meaning on neglecting activity on here, I’ve had a pretty difficult year (not health, family stuff) but things are finally settled and I’m back!

Yes, low blood pressure. The nurses / doctors always look worried when they check it and I reassure them that it’s always like that.

Way back when I used to have terrible migraines pre-embolisation, I tried propanalol (beta blocker) which lowers your blood pressure. They failed to notice how it was affecting my blood pressure and as I increased the dose I became faint and woozy. Vertigo and zombie like.

As for tummy…

I’m trying out putting a little more salt in my food. I usually put very little. Might make some differance to my tummy!

I’ve tried electrolyte sachets and drinks and I absolutely hate them! I also hate stool softer sachets - also grossly salty tasting.

So… I’ve been trying to follow my dietricians advice. I’m not having fibre rich cerial (despite loving it) and having rice crispies instead. Seems to be making a differance. I stay well away from brown bread/rice/pasta but never liked those in the first place. I use fruits + veggies as my source of fibre mainly. It is tricky with fibre, not having too much but also not too little.

I’ve been trying to drink more water too! Apparently soups are also recommended but it’s not something I’d have regularly. I’m having smaller portions as you say (mainly trying to lose weight lol).

I was born with a loose hip - maybe EDS related?

Similar to your sister. Not sure whether it is EDS or just being a lazy person. Wasn’t a very active child. Hated all things sports.

I wouldn’t say my EDS is bad, bad. I know of people wheel chair bound or using a walking stick due to EDS.

Mainly achy muscles, dodgy ankles, very slow digestive system. Fatigue is debatable - cause of AVM? EDS? Simply a lazy person?

I’m being seen by a specialist to look into potential vascular EDS to test for it. I hate the idea of it but she is encouraging me to do it. I can accept hypermobile joint EDS more readily. Mine isn’t all that bad so I can just raise my kids with suitable diet and excersizes to strengthen their joints from a young age. but I really really don’t want to have the vascular type. They can’t test for it in pregancy so it’d just a nice additional thing to make me feel guilt about wanting bio-kids. Going through pregnancy whilst still having an AVM is enough risk for me to want to deal with already. Huff.

I’ve tried coconut oil /
MTC oil in my tea and it is disgusting to me. Does cooking with coconut oil count? I do wonder… does EDS raise chances of arthritis?

Thanks for all of your advice / information!

Corrine

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Hello @corrine Please do not beat yourself up about having children. The odds are you probably have HEDS and not VEDs . But its good that your dr is testing you to be sure what type you have.
Dont call yourself lazy either . Its funny how we do thinks subconsciously to protect our bodies.
The Ehlers Danlos Society just had a conference and soon they will be making their slides available for free like they always do here is the link to watch out for when they do https://www.ehlers-danlos.com/2019-nashville/ - Now one easy thing to do that one of the doctors said ( they streamed it this year ) was that those of low on vit D should be taking 50,000 units a week which I believe is script so I need to get script from my doctor.
Achy muscles, dodgy ankles slow digestive system is all EDS no matter the type . Also Chronic Fatigue as well. which is usually tied to POTs or another form of Dysautonomia - being on Midodrine(script) can help the low blood pressure. Yes EDs does seem to raise the chance of arthritis and also other auto immune issues and inflammatory issues. If you dont like the taste of coconut oil, yes cooking with it counts and you can also buy mct pills so you dont have to taste them. Maybe you are a super taster? I have found another electrolyte powder that I like better to drink when I am home that comes in a few different flavors https://smile.amazon.com/Ultima-Hydrating-Electrolyte-Raspberry-Gluten-Free/dp/B01IIGNPK4/ref=sr_1_4?crid=L47IBF5D7TJR&keywords=ultima%2Belectrolytes&qid=1565668843&s=gateway&sprefix=ultima%2Belectrolytes%2Caps%2C184&sr=8-4&th=1 If you just dont like them make sure you are putting a bit of extra salt in your diet drinking extra water you really need more salt. I prefer celtic sea salt esp cause I am allergic to Iodine. Which I did not know until later in life and explains why I never liked salt but I love celtic sea salt. Also Yes being born with a loose hip is very classic EDs sign - my nephew and my little cousin were born with that too! Of course EDs is strong in our family no 50/50 on this side! My genetic Dr left UCSF and joined this group and they actually have an EDS genetic panel I heard its $250 but I have not verified it https://www.invitae.com/en/physician/tests/02313/ At the EDs Conference the tops EDs genetic Dr said she felt that HEDS was actually more that one thing and that once they found the gene it would be more than one gene and more than one type which is what my genetic dr said as well he had diagnosed myself , my mom and my sister as 3+ which basically was more hip stuff. All the drs recommended taking muscle relaxers at night to give our muscles a break -
Our muscles are tight and overworked - even if we are not really working out. The best work outs for us is swimming in a warm pool, pilates and walking. There is a special PT book for us Ehlers Danlos people - https://smile.amazon.com/Living-Life-Fullest-Ehlers-Danlos-Syndrome/dp/1478758880/ref=sr_1_1?crid=2D653L0N86RHS&keywords=ehlers+danlos+physical+therapy&qid=1565669840&s=gateway&sprefix=physical+therapy+eh%2Caps%2C204&sr=8-1 Its supposed to be done in the order on the book but you only add one new exercise at a time so you know if its causing you an issue or not. Usually our IT bands are a hot mess. Oh and before I forgot the Cusack Family Protocol which is supplemental program. I have not tried it. But some people swear by it and its basically an anti inflammatory . I knows its on Facebook under Ehlers Danlos Syndrome and The Cusack Protocol. Not sure if its on the web too. I think you have the right idea with your children with correct diet and building a strong core. The earlier they are diagnosed and can get into PT to protect their joints the better. I did not have any real issues compared to my sister since I was so active and had a strong core until I had my stroke and AVM. I think I am still healthier than her now. She is 15 years younger than me and has to take a nap every day. Hugs
Angela