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konagirl

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Hi there. Figured it is about time I stop stalking this forum and finally make a post. You are all amazing and brave people, I am hoping for your insight. I am hoping you can provide me with some encouragement. I have come this >< close to posting at least 10 times in the past two months, and backed out every time, afraid of what I would hear. But I’m ready. I think.
I apologize in advance, I will probable type too much and ramble on. I am 35 years old. My husband of 11 years is 36. We have an 8 year old boy and a 2 year old girl. He is my soul mate, my best friend and the best father I have ever known. We truly have a happy life. Had? Have? I don’t know. This all started in July. My husband is an avid wakeboarder. He is extremely coordinated and balanced and can do pretty much anything. At 36 he can still shred on a skateboard better than most teenagers. He and our son ride 4 wheelers and dirtbikes daily. He is a very active man, we are a very active family. In July he started noticing some problems with his legs, he told me “they keep locking up on me, it’s weird, it’s like they get stiff and I feel like I am walking like a robot” This happened occasionally but it was not all the time or even every day. Around this same time he started having issues with his left hand. He was having a hard time doing small things like taking the cap off the toothpaste. He noticed it most in the morning and at night, he said it seemed to get better during the day. Unlike his legs though, this was every day. I told him he was probably just sleeping on it wrong. There was never any tingling, numbness or pain though, just a weakness. He went to our primary care Dr and they ordered an MRI, this was Mid-August. By then, his leg episodes were getting more frequent and he was losing his balance a lot. Dr ordered a lumbar and a cervical MRI. He had these done August 28th. It was around this time I noticed the twitching. Constant fasciculation’s that ran from his shoulders to his elbows in both arms….and never stopped. Early Sept we were out in the boat and while he was able to wakeboard fine, he was falling a lot because he just couldn’t land his jumps, the same jumps he never had a problem with before. I told him he’s just getting old and teased him about it. I didn’t understand what was happening. The MRI results came back and showed some mild cervical stenosis and we were referred to a neurosurgeon. Took a while to get that appointment and we had to wait until Oct 1st. The weekend before our appointment we took the kids camping at Disney and to the magic kingdom. He was able to walk the entire park that night but was slower, if we had to move quick he could not do it because his legs just didn’t want to work the way he told them to. He also fell twice that weekend at the campground. I told him he probably had too much to drink. October 1st rolled around and that is when things got real, our lives changed forever. The neurosurgeon looked at his MRI ‘s and said there is nothing there that would be causing his symptoms. This is not surgical. He said he wanted to do a myelogram, just to 100% rule out any compression on the spinal cord. And then he said it. He said “if that comes back clear, you need to see a neurologist that specializes in neuromuscular disorders like ALS”. I did not know what that meant but it sounded scary. Of course I googled it, that’s when I lost my shit. I thought no way, no how, that’s not it. He looked it up, he lost his shit too. I told him there is no way this is it, there are a million things that could cause his symptoms and that the Dr only used that as an example. From that day, I made him promise me not to look at the internet again. And he hasn’t. He’s not the over-obsessive researcher I am and just believes what I tell him. The more I read, the more I got scared. I never let him know this though. I put on a brave happy face for him and the kids every night and then cry all day at work. The myelogram happened Oct 8th, they came in pretty shortly after to tell us it was normal. They called for a neuro consult. She did an exam right there in the hospital and said we need to get see a neuromuscular specialist. They got us an appt for the next day. The neuro appointment was pretty useless and I knew from Day 1 that I did not like the Doctor. He ordered a round of testing….blood work, brain MRI and 2 EMG’s – one on the left arm/hand and 1 on both legs. The EMG’s were last, they happened on Oct 15. (this was all happening very fast!) By then I had found your forum. I was on it every second of every day. I put a passcode on my phone just in case my husband ever picked it up, I did not want him to see it. I didn’t want him to be scared, I was scared enough for both of us. I read story after story after story….looking for something, anything that would tell me I was wrong. Two things I was hanging onto were things I read in the stickys…true clinical weakness is like losing wifi signal, one minute you have it and then next its gone. That had not happened with any of his muscles. Nothing was completely useless. The second thing was the EMG’s. I read that a clean EMG means NO ALS. I clung to that and hoped and prayed for a clean EMG. I made a lot of promises to God for that clean EMG…and we got it. I had never felt happier about anything. I jumped up and literally almost knocked the neurologist over from hugging him when he told us there was nothing abnormal in either of them. But he was also stumped. He didn’t know where else to look. I never really thought this was his specialty but you can’t deny a clean EMG, right!?!? He referred us to Mayo but warned it may take months to get in. I called some contacts I have made through work and was able to get an appointment with Dr Boylan, the head of neurology, for just two and half weeks later. So that’s where we are today. We had our appointment with Dr Boylan yesterday and I left more confused than ever and all of my fear and panic is right back in place. While I trusted the clean EMG’s…the internet said to so I have to, right?! I still had/have doubt. Today his symptoms have doubled. The spasticity in his legs is a constant thing now(I know now that is what it is called). He cannot run or walk down stairs, he goes up them ok. He falls. A lot. He cannot even stand on a skateboard. His left hand is a constant problem. He drops things. A lot. He yells at his left hand. Literally yells at it. I know its frustration but I can’t help but wonder if he is losing his mind.
So back to our appointment yesterday…the entire reason I am writing to you. I’m confused. Dr Boylan did a physical exam and he was great. Everything my husband told him, he really seemed to “get it”, no one else had. He said a lot yesterday, but a few things really stuck out. He did an entire physical exam and did not find much clinical weakness, which the last neurologist said as well. It’s the spasticity that is doing the biggest number on his legs and making him stumble and fall so much. I think he said there was some actual weakness with his left hand? I was not clear on that. He wants to start him on Baclofen and see if it helps lesson the tightness. This is great right? No weakness. A medicine that can help. I was feeling pretty pumped. But then he said he feels confident that we are in fact dealing with a motor neuron disease. He mentioned PLS. I shut down right about then and I have to be honest, I did not hear much else he said. He ordered more blood work, which we did right after the appointment and then we have more EMG’s on 12/2 and a follow up appointment with him for right after the EMG’s. He said we should have all of our answers then. But PLS…? I am only semi familiar with the term. Its better, right? When I look it up, one of the first symptoms is also weakness. We were just told he had no weakness! So how is he getting to this? I am confused. After almost a month after getting a clean EMG and feeling like we can now conquer anything else, I am shocked and feeling very confused and lost. I purposely did not ask a lot of questions because I try to keep my husband in the dark somewhat. Maybe that’s horrible, I don’t know. But until we have definite answers, I don’t want him worrying. I feel like 12/2 is going to be a big day. I need to know what we could possibly be dealing with.
And I just realized I have just written a book. Again, I am sorry, I knew it would happen. I guess all I really needed to say was…what is the difference in PLS and ALS? What I am really hoping is that all of you experts tell me that he does not sound like he has PLS…or ALS…or any MND at all.
 

Nikki J

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I am so terribly sorry for all you are going through. You are in good hands with Dr Boylan. Yes if this turns out to be PLS it is a better answer than ALS. To be honest it sounds like you are in for a stressful diagnostic process

PLS is a disease that only affects the upper motor neurons. Upper motor neuron does not mean it affects only the upper part of the body. Our muscles work because of upper and lower motor neurons. ALS affects the upper and lower motor neurons.
Upper motor neuron disease is found on physical exam. Spasticity is an upper motor neuron finding. The EMG does not show upper motor neuron problems. It only shows lower motor disease. So someone with ALS will have an abnormal exam by the doctor ( spasticity, abnormal reflexes etc) along with weakness and an abnormal EMG showing changes consistent with ALS
Someone with PLS has the abnormal exam but a normal EMG. The thing is Umn disease that looks like PLS turns out to be ALS when LMN findings develop later. Most people with PLS are told the diagnosis is provisional unless they have gone several years ( up to 5) without LMN disease. PLS is something people live with for 20 plus years so if you must have MND it is a better answer than ALS but it is only good news if your choices are PLS or ALS.
I wish I could say oh it does not sound like it but I can't Again I am sorry
 

konagirl

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CALS
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City
Jacksonville
Thank you Nikki. Thank you for your honestly. I just don't understand. This can't be happening to him. This should not happen to anybody! I am so sad. And angry. We have not told anybody what we are going through. We have both lived in Jacksonville our entire lives and will have a huge support system when the time comes, but I have not wanted to worry anyone because I have been so positive this was going to turn out good. I am losing my hope. He still doesn't understand what is happening. Am I doing him an injustice by keeping him so hopeful that this is all going to turn out ok? At this point I feel like I am lying. He has done what I asked and is staying off the internet, all because I told him I am doing all the research and to just ask me. But I am lying to his face. I tell myself I am protecting him, but is it going to make it worse? I just don't want to see his heart broken yet....
 

Nikki J

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Only you know your relationship. But if he heard Dr Boylan? Plus even without that he probably knows something is terribly wrong. He may be trying to protect you by pretending not to know. If that is the case you are denying both of you the comfort of facing this together. I am only guessing here
 

Atsugi

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KONAGIRL, Boylan is the best. He will take the time to answer questions or call in other experts to answer them.

I understand protecting your spouse. I did it differently. My wife knew the details of her ALS diagnosis and prognosis. I helped relieve her stress by dealing with the planning, the visitors, the Social Security people, and the funeral home. and the lawyers.

But Kona, your once-in-a-lifetime, million-dollar opportunity to get Boylan's undivided attention is NOT the time to be protecting your husband. Next time, bring a notebook with questions and ask each one and write down the answers. Ask him bluntly: Is my husband going to die? What is he suffering from? How will this affect his day-to-day life? IS there a cure? What should we do next? Etc... Don't leave until you and your husband understand the answers. By the way, it is OK to cry in a doctor's office. Just don't leave without your answers.

Did your husband serve in the US military? -- Mike
 

lgelb

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Kona,
I would really be up front with your love before the appointment. You both need to hear the same things, even if it takes a while to process and you will do that differently. Your husband sounds like a doer and there will be much life to live, once he knows what he's dealing with. In fact, part of his frustration is probably not knowing.

Typically, if a leading clinician says s/he is "confident" you are dealing with MND, you can take that to the bank. I'm very sorry.

And I agree PLS is more likely, but as you have read, it cannot be reliably differentiated from ALS for years because "UMN-dominant ALS" presents similarly. In upper motor neuron disease, there is more spasticity than weakness. The EMG is normal or near normal. It's less "can't do" suddenly than "gradually do less" and sometimes certain conditions/activities affect what can be done, but there is a downslope as you've described.

I think we'd all agree that no one should have to deal with this, and yet everyone reading this has, and you will, if it comes to that, as well.

Bring your husband up to date, Kona. It's something you should face together, as you have everything else. We will support both of you however we can.

Best,
Laurie
 

konagirl

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Thank you for your replies. Really, thank you, I appreciate them more than you know. Your time and your words are not wasted either, they are sinking in. I know that I need to talk to him. Its just hard. He was afraid of ALS. He heard motor neuron disease. He has no clue ALS IS a MND. He thinks he is safe. I'm not ready to see his heart shattered.
When I said earlier that we have not talked to anyone, I meant to say we have not talked to many people. His parents and my mom know what is going on and I have talked to two of my very best friends. We still don't know exactly what we are dealing with yet though, right? Dr Boylan ordered a lot of blood work that we are waiting on results from. Chad is a printer and works with toxins every day....and bites his fingernails. I am trying really hard to keep hope alive.
Mike, no he has not served any military time. I do have a question for you, since I have read that you have actually seen Dr Boylan and know him. I do plan to follow your advice and ask all of my questions, no matter how ugly they are, at our appointment on 12/2. I would like for Chads mom to be there with us. Do you think Dr Boylan would mind an extra person in the room? I would really like both his mom and dad there, but that may be really pushing our luck?
I do know the likeliness of PLS progressing into ALS down the road, and I understand now how and why the diagnosis is initially PLS. I'm still a little confused on the differences between upper motor neurons and lower motor neurons. If a person with PLS ends up in a wheelchair because they have lost ability to use their legs, wouldn't that muscle degeneration show up on an EMG? I am actively trying to learn though and hope to understand it all better soon.
One more question. IF we get a PLS diagnosis...do I tell him or let him know of the high percentage of PLS turning into ALS? Or do we cope with the good, the bad and the ugly of PLS alone and not let him have that additional worry? I know you cant tell me what to do, as you don't know him, but did you want to know every detail of what the future might hold? I feel like I am protecting him and taking one day at a time but now know I might be doing this all wrong. Where's the damn handbook?!?!?

Thank you,
Kelly

Ps I should have thought better when choosing a screen name, Kona is our dog :/
 

Nikki J

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There are people on this forum with PLS who have a wheelchair. So the 2 are not mutually exclusive
As the only PALS on this thread I will try to answer wanting to know. I knew I was at high risk because of my family. Having the gene meant my chances of getting this approached 100 per cent. I chose to get tested and know. And it was better knowing than wondering. And once I had the abnormal EMG I knew the rest instantly. In that I had no choice. But I am quite certain that if my road had been different I would still have wanted to know. And quite honestly I would be angry and resentful if my spouse chose to hide this kind of information- more so when I learned my parents and my mother in law! Knew before me. If there are still other things on the table then he like you can cling to hope but not to tell him where he stands seems like a recipe for trouble. You can't dance around this and have any kind of useful dialogue with Dr Boylan and he deserves to have some lead time to get used to the possibilities. How would you feel if Dr Boylan brings it all out in the open as is likely and then he asks you if you knew/ suspected?
 
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Atsugi

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Take it from an old guy: Trust is everything in a marriage; don't put a chink in it.
 

konagirl

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CALS
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You are right. You are both right. I need to talk to him, and I will. Trust has never been an issue with us, I don't want it to be now. I really just thought things would all be ok. I thought I was protecting him from the worry and stress that I am carrying. But then things got really real. I will talk to him.
 

LifeEnthusiast

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Somewhere In
"He heard motor neuron disease. He has no clue ALS IS a MND."

Just as a side note without going into a lot of detail, but my issues started at the same time as an undiagnosed unrelated condition. It was only happenstance that I was sent to a neurologist who then after exam felt there was more going on. In fact, otherwise, I wouldn't have even sought out neurological opinion until about a year ago.

During this time, my neurologist kept saying he thought it was MND. I was relieved. I thought great let's treat it. One day I was in his office, and he mentioned MND again. My response was the same. His reply was "when I say motor neuron disease I mean ALS." That is when the world under my feet felt like it opened up and the reality sank in. Originally being a Canuck, MND was a foreign term for me.

As devastating as the news of what his meaning of MND was, knowing the reality in a weird way made things better. I've come to the conclusion that false hope can be far worse than having no hope (in terms of a cure). At least then, when it all sinks in, one can have the ability to start getting on with the business of living. And by God, there is still much living to be done.

Cee
 

cheerleader

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Konagirl, as it sounds like you've figured out, this is a shared disease- you are in it together! Please don't underestimate your husband's ability to deal with whatever he has to because you will be there at his side helping. As we found, this disease strengthened our marriage because we did talk everything through and coped with it because we were a team. Good luck to you as you deal openly with your situation.
A thought- know you want to include people you love in the consultation to help you process information. Because it is an exhausting and overwhelming process, you might ask them to be observers rather than too vocal. Things can get sidetracked if several people are asking questions, talking. Maybe designating them as note takers would be best. Do remember, too, sometimes intimate issues will be discussed, ( i.e. Your sex life, bowel habits, etc.) so something to consider as you ask others to join you. Donna
 

lgelb

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Kelly,
OK, so he is misunderstanding a critical piece of terminology, and correcting that will be hard. But the longer you wait, the harder it will be and Mike et.al. are right that it is a matter of trust, that you'll need even more from here on.

So yeah, time to own that, and commit to him not to let him misunderstand in future.

And that points up the fallacy of "I'll do the research." If he turns out to be a PALS who is happy for you to sweat the details (my husband was one such), that's fine, but he has to choose; you can't force that choice. And you always have to be prepared to honestly and fully answer every question.

As Nikki says, PLS is about upper motor neuron disease only, and upper neuron damage doesn't show up on EMG very much or at all. But it is still serious. Do a search; we've had some recent threads on this in the PLS forum.
 

Green Queen

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Joined
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DX MND
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AUS
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Gday Kelly
I'm so sorry you find yourself here. I can feel your torment in your words.

I'm 43 (just don't tell anyone ). Wayne's 45 and our kids are 13 and 11.

In September I was diagnosed with PLS.

I could word the last two years of our lives in two ways.

It's been a spiraling train wreck. I had to give up work. I can no longer drive or cook a meal or iron clothes...I can't even hug my kids properly. Everything is hard. Everything.

On the other side of the coin, I cannot begin to tell you how close to my children I've become. I don't pack their lunch anymore, but I'm here after school and we chat and laugh and joke around. My daughter at 13 is not a stranger to me, she shares. They both understand the importance of family. They are awesome caregivers. I still hug them. Albeit clumsily. The hugs I get back I can really feel now. That's a plus. They don't see me as disabled. I'm still mum.

My relationship with my parents, although never bad, is awesome.

My husband. Well he struggles with hearing the ins and outs of everything. But very supportive. He's amazing. Still calls me beautiful even though I have half a face that doesn't smile when the rest of me smiles.

We have learnt, over a few years, to live today. To enjoy today. Tomorrow will be here soon enough.

Limbo is hard. Diagnosis is hard. Venting is fine. Crying is fine. Laughing is great too.

Whatever is around that corner, we are coming with you.

God bless you on this journey.

Love Janelle x
 

Gembead

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Well said JanellexxxxGem
 
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