Just got back from my first neuro appt since my results.
Apparently here in UK the Docs do not offer ANY off label medicines until they have passed all clinical trials. They NEVER stray from this under any circumstances (i wonder what would happen if any of the neurologists here had HD?)

Basically im years from getting any memantine which I really think may help my brain fog reading about you guys over the water who feel your brains "unfog" when you take it.(I have not found any UK based HD+ person who takes it)

I understand they have to stick within the set guidlines and protocol...

I naively assumed asking for a very low dose of a chemical that is FDA approved for the elderly and frail would be assumed very low risk for me.

I'm annoyed because the mutant HD protein is causing me more bloody harm than what im asking for!

Anyway my next course of action is back to my GP (with whom i have a really good relationship) and she has already said she would consider "off label" use of drugs in certain circumstances.

After that I will be trying to source it without a script.

Barb, if you read this could you tell me the name/e-mail address of your neuro who you say has been really impressed with the results of your use of this drug.

Oh yeah I got the keys to my new shop last week... and im all move in and it was a good move... cheaper warmer nicer than the stall I had before. Will put up a link so you can see some photos soon

regards

Lorraine

Lorraine, i'm glad you got your new shop. sorry to hear about the frusttration about getting the memantine.

So frustrating Lorraine! I don't understand that logic but you find it all over - here too... and even at an HD clinic in NYC! Good luck with your new stall and good luck getting the memantine... (if you do start memantine please post how you like it)

Barb goes to the clinic headed by Dr. Hayden.

[www.orderofbc.gov.bc.ca]

Hey Lorraine, yes, memantine is off-label, but it is completely approved and passed all trials for alzheimers. Actually, any and every med that is prescribed for hd, are all off-label. Try telling your neuro that one But, i'm glad your family doctor may be willing to work with you on this, it's at least worth giving it a try. Here is a link to my clinic, with a phone number that your doctor could call, good luck

[www.cmmt.ubc.ca]

Thanks for info Eric & Barb..

Barb, my neuro told me the only thing he could offer me for this disease is
tetrabenazine... after making me hold my arms out straight and watching me walk across the room he declared me to be many years away from needing this drug but that was all that was on offer.

I laughed at him and said I was there to try to protect what neurons i have left and learn more about what i could do to hold this illness back until a cure is found. He looked surprised as if i was being unreasonable.

All he talked about was chorea... as if that defined the disease and all of the invisible subtleties where nothing... he glanced at the list of suppliments I had carefully written out and then ignored them (like i was declaring an apple a day) really on 20mg creatine i would have expected him to be worried about my kidneys.

Nothing... ears deaf, no knowledge.

You know what he said?.....

"oh your CAG is 3 less than your mothers.. that is odd because they usually only go up or very occassionally stay the same"

Even my HD fuddled brain immediately thought.. well if that was true this desease would burn itself out as the CAGs would get so high that eventually in a family line the last people would have severe JHD and they would be too young to be parents.

He is supposed to be a highly qualfied expert in nuerodegenerative illness!!! Glad hes not my mechanic!!

I am supposed to be someone whos IQ is racing down hill.

I am secretly thrilled to find myself feeling superior to this man..
"use it or lose it" they say... well i am constantly stretching myself physically and mentally and I will never roll over play dead and wait for the chorea to start and go cap in hand for the tetrab.

Sorry to rant again but this is still really peeing me off!

L x

Thanks for info Eric & Barb..

Barb, my neuro told me the only thing he could offer me for this disease is
tetrabenazine... after making me hold my arms out straight and watching me walk across the room he declared me to be many years away from needing this drug but that was all that was on offer.

I laughed at him and said I was there to try to protect what neurons i have left and learn more about what i could do to hold this illness back until a cure is found. He looked surprised as if i was being unreasonable.

All he talked about was chorea... as if that defined the disease and all of the invisible subtleties where nothing... he glanced at the list of suppliments I had carefully written out and then ignored them (like i was declaring an apple a day) really on 20mg creatine i would have expected him to be worried about my kidneys.

Nothing... ears deaf, no knowledge.

You know what he said?.....

"oh your CAG is 3 less than your mothers.. that is odd because they usually only go up or very occassionally stay the same"

Even my HD fuddled brain immediately thought.. well if that was true this desease would burn itself out as the CAGs would get so high that eventually in a family line the last people would have severe JHD and they would be too young to be parents.

He is supposed to be a highly qualfied expert in nuerodegenerative illness!!! Glad hes not my mechanic!!

I am supposed to be someone whos IQ is racing down hill.

I am secretly thrilled to find myself feeling superior to this man..
"use it or lose it" they say... well i am constantly stretching myself physically and mentally and I will never roll over play dead and wait for the chorea to start and go cap in hand for the tetrab.

Sorry to rant again but this is still really peeing me off!

L x

whoops pressed enter twice in my annoyance

Some doctors don't have a clue.

you go girl!!!

Kudos to you for having the confidence to KNOW you are more informed than this doc. Grrrrr makes me so mad! We were sent out of the HD clinic (after HD+ diagnosis from the clinic neuro and no "support" at all) told to take a multivitamin and eat a variety of fruits and vegetables... and the care went downhill from there... and that is NYC -I know that shouldn't make a difference I just had high expectations.

We all must follow our guts and be in charge of our own care. Good luck with your next steps!

[hdlighthouse.org]

He certainly doesn't know the facts about CAG counts as transmitted maternally. About one third of the time they remain the same, one third of the time they contract, and one third of the time they expand.

Marsha - do you have those stats in regard to paternal transmission?

Thanks!

Paternal expansions - 65 percent
paternal contractions - 16 percent
Paternal staying the same - 19 percent

Thank you Marsha!

DEAR LORRAINE,

SORRY MY DEAR, BUT THAT POST MADE ME ROTFLMAO!!!!

that was so funny, i would enjoy you as a writer, i could imagine the whole scenario performed on SATURDAY NIGHT LIVE...

a book with hilarious anecdotes, from people with life threatening diseases, about just how singularly under-informed. doctors can be....

Hi HDAC-

Marsha's data about paternal CAG transmission is correct, but I thought people might find some more context useful.

These data appear in a paper by Vanessa Wheeler (Wheeler et al. 2007, J.Med. Genet. 44, 695-701). Dr Wheeler told me "these data came from 184 paternal transmissions. For the 311 maternal transmissions in the same study we found 34.5% expansions, 34.5% contractions and 31% unchanged alleles. The numbers aligned well with previously published studies."

I also contacted Dr Jim Gusella. He told me "these numbers come from a particular population with a particular distribution of allele sizes. The actual propensity to expand will vary with at least two factors: 1) the initial CAG allele size and 2) genetic predisposition. Smaller CAG repeats are less prone to change and the fraction of the time that changes occur increases if the starting allele is a longer size. Similarly, some males show a much greater likelihood of transmitting increased alleles (which may be detectable in sperm DNA). There may also be other as yet unknown factors that can affect instability. The data from Vanessa's paper is a rough estimate across a typical range of allele sizes but the proportion of unstable transmissions would change if the population being studied had a different range of allele sizes (or a different genetic predisposition to instability)."

Robi

Robi Blumenstein
CHDI Management/CHDI Foundation
robi.blumenstein@chdifoundation.org
www.chdifoundation.org

Thanks Robi - very interesting.

Thanks Robi that is very clear and easy to understand.

My GP could not prescrible Memantine either (tightened up systems in UK mean any off label use of drugs must be "passed" by a board.. she says she cant do it within the current framework they just wont allow it)

I think I should be able to sign a disclaimer stating I have chosen this drug and any negative issues will be my responsibility.
Alas this is not the way it is.

She did however support my wishes and is going to see if she can refer me to someone in UCL queens square and see if they have any previous "off licence" use of this drug for HD. I doubt this will come to anything.

She also said she totally understood my frustration and would probably be doing something like this herself if she were in my shoes.

So I am going to be forced to buy this medication privately from a country with no prescription laws. Lundbeck make the original Ebixa and I have found a place in Thailand that supply the original blister packs... so I may try that.

I do not agree with this method of obtaining drugs which are probably here in the next street to where I live on the pharmacy shelf for a quarter of the price. But I am being left with to choice.

I am guessing if i go it alone like this then the neurologists may refuse to treat me. Cant say im that bothered. Think the US and Canada are so far ahead of the UK that this is where the final answers and any cures will come from.

Oh well maybe my Thai tablets will come with some free green curry paste

L x

Lorraine, as you have found out "expert" means different things to different people. Obviously you knew what ya knew and Marsha tossed a few figures to back up your point. I have looked at several studies that, within a curtain range, say roughly the same thing. Expansion is not the most likely outcome for offspring. Studies of course as small as these are going to vary. I bet there are four of them on this subject alone and none of the numbers match exactly. That's irrelevant to us out here. We just want to know if who we are talking to is an expert, and can at least get something as simple as this rightish. Your dr. sure didn't. Especially as it applies to you. A real expert would know what to say to you as opposed to a father to be with a CAG of 60 and JHD himself. I am fine with experts giving me knowledge.. I would rather speak to a person with less expertise if they are well rounded and can use wisdom with your personal circumstances in mind. So often I heard an expert say something... probably factually correct but had no idea what context to use the information. They know facts and not the audience.

The reason memantine in the UK won't get prescribed has nothing to do with you signing a paper. It's about cost to your system. Costly drugs used in an unproven way is a cost they can cut. It's about as simple as that.