Life expectancy.

[LessThanRich]

So having read posts here over the last few weeks, I see stuff from people my age who have been suffering since their 20's and posts from people who started suffering in later life, but no posts from someone in their 50's who has been suffering since their 20's. Are these people all dead or now incapable of using a computer? I started suffering at 26, now 37, what is my life expectancy? Its quite incidental because if I died today, it would be no skin off my nose. If anything I wanna know how many more years am I going to have to suffer AMN. Sad eh?

[StevenSims]

Hi Rich,

It's such a varied disease so you can't compare yourself with others! I was 26 too but had Addisons only and my AMN didn't start till I was 33, I'm 43 now, the shorter life expectancy is due to Cerabral activity but not all of us get that?

You need to keep yourself positive! there are others worse off than us!

All the best

Steve

[LessThanRich in reply to StevenSims]

The thought of being a handicapped wheelchair user for another 30+ years is horrible.

[StevenSims in reply to LessThanRich]

You are lucky you don't have Addisons too! I've been ill since Sunday and I'm taking 4 X my dose of steroids! Keep positive you just got to learn to live with it!

[SongStream in reply to StevenSims]

Steve, I wish you well! I'm sure you're adjusting your electrolytes.

[LessThanRich in reply to SongStream]

After 10 years I'm still not dealing very well with it all. Aside amassing all the stuff to make my life easier, I am awful. Ever since getting diagnosed 2 years ago it has only been worse. And yes I am lucky not to have Addisons too.

[StevenSims in reply to LessThanRich]

Hi Rich,

IT is hard dealing with it at first! When I was diagnosed I was told I have ALD and I've about 5 years to live! That was a bit shocking when months earlier I was fit and active! Also having a lesion on my brain is a worry but it's been stable for a few years now, hopefully it will stay that way?

All the best

Steve

[Aaron98 in reply to StevenSims]

Steve, I was told the same when I was first diagnosed. Fortunately, this doctor was wrong. I will never see that neurologist again. My current neurologist attended Stanford with Keith Van Haren, and knows him personally, so while he is not a specialist on AMN/ALD, he's got a pretty firm grasp on it.

[LessThanRich in reply to Aaron98]

"at first" how long does this last? I have been suffering for 10 years already and been diagnosed 2 years. I think I am a terrible handicapped person and I don't deal with it very well at all. I am super surprised that everyone here is on about prolonging their lives if they can, I won;t be getting any invasive treatment to prolong my miserable existence. I am ready to die tomorrow, today if we can organise it...

[KazzyALD]

Hi Rich. My uncle would of been late 20’s when he was diagnosed. That was in 1987. He’s still with us. My cousin, however, was diagnosed in his early 30’s I think, he passed away at the age of 51.

Karen

[SongStream]

I'll be a young 57 years old this year and was diagnosed with AMN when I was 37; although, I've had Addison's since 17 (a symptom of AMN) when my brother had an Addisonian crisis at 19. I've had other symptoms like tripping and unable to lift up my feet since my early 30's. My brother had more noticeable symptoms like drop foot and he's now 58. Note that a "newborn male patient has a 35–40% risk to develop cerebral ALD between the ages of 3 and 18 years." Ref: adrenoleukodystrophy.info/c...

As for the risk of adult ALD, it is estimated that 20% will develop cerebral demyelination:

ncbi.nlm.nih.gov/pmc/articl...

It is most important that you have an annual MRI. There are now alternative treatments: stem cells; bone marrow, etc....

[LessThanRich in reply to SongStream]

I heard its more like 40% chance of cerebral. Not that stats matter, if its gonna happen, its gonna happen. Not sure I want any of those treatments. I'm not up for prolonging my life that much. I am over suffering already and I'm only 37.

[Aaron98 in reply to LessThanRich]

I've heard different percentages from different sources. An ALD specialist told me it could be closer to 50% of AMN men eventually developing cerebral disease. Bottom line is, we don't know.

If it was clear that I had developed cerebral ALD, I would absolutely go for the bone marrow transplant. I have an unaffected older brother who would probably be a good donor. Not to sound morbid, but I would much rather die from a failed transplant than from cerebral disease.

[LessThanRich in reply to Aaron98]

I find it crazy you would try and fight it to extend your life. I am ready to die already.

[easygoin1]

Hello Rich,

My Addison's was diagnosed when I was 23 and had been the cause of many serious sickness episodes when I was younger but not diagnosed properly. I have now developed the neurologic symptoms now that I am in my early 50s. My walking is getting more difficult all the time. We lost my brother, who also got the damaged X chromosome, when he was 49. It is not an easy thing to determine life expectancy with this disease as it has so many phenotypes. I wish you all the best and pray that they are able to come up with some help for us!

[LessThanRich in reply to easygoin1]

Ok, so the missing demographic of sufferers exists! I'm not sure if that's good news or not.

I had a vasectomy, so I did my bit towards killing this disease. I have no kids, so it will die with me now.

After that whatever. I am not against having an accelerated life and checking out early. I had enough of suffering already and I am not expecting a miracle cure in my lifetime.

[Hillary]

Hi Rich, my grandfather had AMN (we now know) and lived to 84. My mum, carrier had no symptoms and died 83. My brother had Shilders disease, back in 1953 prior to the diagnosis of ALD, and died age 7. This was before I was born, I’m 62 and have had AMN symptoms for 29 years, my son was diagnosed with ALD in 97 and died age 9. I now have a granddaughter who was born after her parents went through PGD. we have stopped ALD/AMN in our family. We have all had the difficulty in accepting our lives are changed, I personally take antidepressants and have a wheelchair which I love. This rare disease affects people in so many different ways. My sons consultant described the journey as “walking along a cliff path in dense fog, you never know when you will fall”.

Keep posting, we are all here for you

[LessThanRich]

As it stands I am a really strong person, albeit my legs don;t work. So from what I have learnt it looks like I might have to suffer another 30+ years of this. In event of becoming a vegetable I will be going to Switzerland for euthanasia. I am not interested in treating brain lesions or prolonging my life. Luckily I have the best girlfriend who is ready to support me through everything, before Mika came along it was a really dark depression.

Its probably quite morbid, but I am happy to find some other wheelchair users. On the whole the AMN community seem to be quite against them, I am not sure why, as they definitely give you an independence which otherwise is lost with this illness.

[easygoin1]

I don't believe it is that we are against wheelchairs so much as most of us believe strongly in "use it or lose it" and are driven so we push ourselves to do as long as we can. I feel for you dealing with depression due to the illness, I am fortunate in that I haven't had to deal with that much. There are times that it comes though, especially when I am dealing with a bathroom issue when I am out, but try to be prepared with supplies at hand to take care of the problem. The big thing that I use to get by is looking around at other members of society and seeing that there is always "worse" so take what I have to deal with as not as bad as it could be.

[LessThanRich in reply to easygoin1]

My stance on "use it or lose it" is that I have already lost it and I am never gonna get better, so why struggle through life when there is no need? There is no way I could have lived on my own for 3 years without a wheelchair. Impossible.

[LessThanRich in reply to LessThanRich]

Another useful thing about a wheelchair is i have a bag behind me at all times with everything I need in it. Its always there, I don;t have to worry about carrying it or forgetting it anywhere.

[easygoin1 in reply to LessThanRich]

I think something you need to remember when someone says "use it or lose it" is that you are using it in the way that you are able, which most of us do in our own way!

[LessThanRich in reply to easygoin1]

I can still walk with crutches, but I have no idea why I would unless I had no other choice. I still stand up to cook and transfer to the shower/toilet/sofa/bed. But its beyond me why anyone would "crip" their way through life when tools exist to make you instantly "normal". I've said it 3 times already this week here, if anyone who suffers AMN hasn't even tested a wheelchair, you have no idea what you are missing out on.

[SongStream]

I can't say instantly "normal" but I appreciate that it is your new normal.

[LessThanRich in reply to SongStream]

Have you actually tested a chair? If you haven;t you are just speculating and I stand by what I say. With my chair I am not even handicapped, just little. You will have to relearn everything you know to accommodate the chair (this took me 2½ years), but at the end of the day you are essentially "normal" (hence the speech marks)

Its obviously no skin off my nose if you get a wheelchair or not. But I am trying to improve your lives. Please don;t speculate and at least go and try one. On the flat or downhill I move faster than someone walking. I can carry and move heavy stuff on my own. I can go as far as I want (especially if I get a push). You can get to the toilet VERY quickly and loads of other reasons to get one.

[Aaron98]

I have often thought along this same line of questioning, Rich. I have an extensive family history of ALD/AMN, and I have met a few friends through this forum, but I still feel that my understanding of AMN is nowhere near where I'd like it to be. Probably the biggest question I have is what eventually kills men with AMN. I realize that this sounds morbid, but it is an important element of understanding our disease.

My maternal grandfather was "diagnosed" with MS in his early 30s, correctly diagnosed with AMN in his 40s, and passed away when he was 67. It has never been clear to me exactly what he died from - he just grew weaker and weaker in the last year of his life, and then just died of "natural causes" (though I'm certain that it was complications due to AMN), almost as if he had aged rapidly. AMN does often feel like premature aging, doesn't it? My grandfather never developed clear signs of cerebral disease, and was not anywhere close to approaching dementia or a vegetative state.

Again, I don't mean to sound morbid, but I live my life with the expectation that I will probably not live to be 70. I developed some symptoms of AMN very early, probably a decade before my grandfather did. Does this necessarily mean that I will die ten years earlier than he did? Absolutely not. But I recognize that I could be halfway done with my life already, and that makes me want to make the most of it NOW.

[SongStream]

"Make the most of it NOW."

I heartening agree. The love of my life sent me a video message years before passing away that I recently discovered including the words: Love life. Live it. Love it.

None of us are guaranteed tomorrow. When you watch someone you love die from cancer, it puts things in perspective. Be grateful for your senses. See life. Smell life. Touch life. Hear life. Taste life. You get what I mean. Live mindfully. Be grateful. Adapt and overcome your challenges. AMN taught me so much. I am kinder, more grateful, have more empathy, humbled, etc....Look around. We are not alone with life's challenges.

[Hillary in reply to SongStream]

I’m with you on this, when you watch someone you love die, I lost my son aged 9, to ALD. it makes you a different person, more accepting of life, stronger and more able to adapt. It’s hard to keep positive at times so I find it so good to have this web site to vent, moan or post asking for support. We are definitely not alone!

[GeoffMahy Volunteer]

Hi, My name is Geoff. My AMN is very late onset, it was only diagnosed when I was about 50 and I don't have an adrenal problem I am now 74 and my only real problem is my walking. I manage so far pushing a four wheeled walker (rollator) but I am seriously thinking of getting a small electric mobility scooter.

I still drive a normal car but only with automatic gearbox. I hope this is helpful.