Glimmer of hope

[pmbenb83]

Hello everyone,
I'm not sure if this qualifies as a "story of hope", but for our family it is a "glimmer of hope." After being bounced around to different doctors for 11 months, many EMGs, etc., my husband was told in early October that they were leaning toward atypical ALS. Thankfully my husband, being pigheaded, spoke up and said that he would be a guinea pig and would like to try the IVIg treatment for MMN, which was the other diagnosis they thought he might have, but his GM1 didn't support it. That kind of scared me for him to say that to doctors at a "teaching hospital". Their eyes really lit up. lol After the doctors consulted each other, they said that he could give it a shot and explained everything in detail.

Over the past 2 weeks, my husband had the IVIg infusion, which made him severely sick, but he now has more energy and he said, "I feel like my old self." His right arm was his weakest extremity and he could barely lift it up to feed himself. Without thinking, he raised his right arm up and put it behind his head to pull up his pillow during the infusion. We both looked at each other with shock and amazement, because he hasn't been able to do this for over a year. Little moments like this have been happening this week, which gives us our "glimmer of hope" that this might work. We are still very cautiously optimistic, but are on the verge of shouting from the rooftop!! He has also gone up on his dumbbell weight from 2.5 lbs to 5 lbs, which to him is nothing since he was used to benching 300 lbs. He knows that he has to take baby steps, as to preserve his muscle that is left.

Tonight was our measuring night, which we have been doing weekly and he has gained 1/4" in his right bicep, left bicep, neck, and chest. Another "glimmer of hope" that we hope continues. :) It is so hard not to get excited and I have butterflies just typing this, but I am also feeling the need to protect ourselves from a possible let down. What an emotional roller coaster!

As we have more "glimmers of hope", I will be sure to post again in this new section. This is a great addition!!

I also want to personally say thanks to Jamie, Peg, and Patty for all of your PMs and suggestions of what to ask the doctors. The MMN might have been THE question. Thank God for this forum or we might not have seen this glimmer at all.

Sending you all my best and hope you all have stories of hope to share real soon!
Take care,
Pam B in Va

[Jamiet]

We are all pullling for your hubby....i am so excited to hear this!!


take care,

jamie

[Icanmanz]

Hi Pam. Great story of hope. Will keep you guys in my prayers. I have a question though. What is IVI infusion? Can you explain that to me, please? I'm a little naive sometimes, but I can be nosey!!!! LOOL

Irma

[freddiesnetty]

Hi,
I hope the progress continues. I pray for a miracle everyday for all of us...I will keep your family in my prayers. God Bless!! I too am wondering if this is like kelation?? Not sure if spelled right...
Annette

[pmbenb83]

Wow, You guys are quick!! Thanks for the prayers and good wishes!! We appreciate all of them!

Irma ~ You're not being nosey... if you don't ask questions, you won't know the answers. :) I found this website from GOOGLE that explained it as Intravenous immunoglobulin (IVIG) which is a blood product administered intravenously. ... IVIG is given as a plasma protein replacement therapy.

We would ask questions, and the nurse said that they really don't know how or why it works, but only that it works on some patients so they keep doing it. Kind of scary to me, but my husband wanted to try anything to try to extend his life.... well within reason. From what I understand MMN is where the immune system starts attacking your own motor neurons and this treatment is going to suppress his immune system. They also told him not to have the flu shot, which I believe would boost your immune system. This is all new to us, so if I am wrong hopefully someone will post and let us both know. ;)

So far something positive is happening for which we are very thankful, and even if this keeps him in his current condition, we will be forever thankful. Some people have been doing this treatment for 10-15 years and have slowly progressed into other diseases, which is why he wanted to give it a try. They truly inspired him.

Irma, I wanted to tell you that your posts have been a huge inspiration to me too. My husband is still capable of caring for himself, but I was drawing strength from you through your posts to become prepared to do whatever I need to care for him if ever I am needed. I just hope I can do as good and caring job as you did for your son. I know he is very proud of you! Please take care of yourself and know that I am thinking of you and your family during this holiday season.

Take care.
Pam B in Va

[pmbenb83]

Annette ~ Thank you also for the well wishes and prayers!! As to your question about kelation, I think Kelation is a therapy for heavy metal poisoning. I just looked it up online, because you peeked my curiosity. :) So far, the doctors said my husband tested negative to heavy metals. His problem may be related to his immune system getting off track and attacking him instead of an illness. The IVIg is actually a trial to see if it works. The doctors are still perplexed by him..... he's another medical mystery.

Good luck to you too!
Take care.
Pam B in Va

[hopingforthebest]

:-D

Hi Pam

You have alot to be happy about! They way it was explained to us, is that IVIG that works point to MMN! As my husband's neuro said, MMN is a treatable disease.

Hope your husband keeps up the good work!

God bless you both!!

Patty

[Gina]

Hi Pam, if thier ever was a glimmer of hope i think you have found it!!!!!I so hope that it works out for your hubby but i know the emotional roller coaster that you are talking about.My husband also has als....or should i say probable als. which is all that the doctors would commit too.His respiratory system is the main problem and they could not figure out why that was shutting down so quickly while the rest of his body seemed to be affected less severly.Do you know if this treatment or does anybody know if this treatment is available in Canada?We have stopped going to the als clinic because it was an hour drive and my husband could not handle another visit just to be treated like a gineau pig!I have never heard of this treatment but i am sure that he would be willing to try it.!Maybe we didnt ask the right questions like you did!I am very excited for you and hope the improvement continues ......I am hoping their is a light at the end of the tunnel....thanks for sharing this news.....Gina

[Icanmanz]

Thank you very much, Pam. It is so nice to hear my posts are helpful. Sometimes I worry that I am sounding too morbid, and I am trying my best not to. I only hope that my posts that I am putting out are about my son, and not about me. You see, my pain is immensek(using right word??LOL), when I am posting I have to let it out. To me it is not a good thing to keep pain bottled up inside of you, let it out, share it with other folks that are going through the same ordeal. Somewhere along the line someone will come along and help you how to deal with it. I have shred posts with folks on this forum that are godsent angels. In other words, I get a good night's sleep after I leave this forum. Am I making any sense? LOL God bless you folks! Good luck with your husband, and I hope everything works well with God's help!

Irma

[kathmc]

This is wonderful news. I hope your husbands progress continues to wellness.

I put MMN in the search section here & it said no matches. what is MMN?

kathy
lexington

[KateO]

Pam -

I too had IVIG with the assumption that I had MMN. My first infusion was last April. The results were amazing - I walked for two entire weeks without my canes, I wasn't as tired, I just felt great. THen, I started feeling worse again. My next two IVIGs (spaced 2 months apart) yielded no results.

When I asked the ALS Clinic at JHopkins about it (they were not the ones who ordered the IVIGs), the explained that it was not that uncommon to see results after first IVIG even if it is ALS - and, that typically there are no results with future IVIGS.

I don't want to rain on your parade - I just know that I was so discouraged after the 2nd and 3rd IVIGs didn't work. I was diagnosed with ALS by Hopkins just a month after my 3rd IVIG. You didn't mention (or, I missed it) what ALS CLinic you are working with. You may want to ask them about my experience.

Take care.

[pmbenb83]

Hi Patty ~ Thanks for letting us know what your doctor said about IVIg. We are still hopeful, but staying very guarded until we hear the magic words from the doctors. I hope you and your husband are doing well. :) Take care. Pam

Gina ~ I'm not sure if Canada has IVIg treatments there. You need to ask doctors in your area. It made my husband very sick with SEVERE flu symptoms, which took 3 days to get over. The treatment is like a blood transfusion of immunoglobin from blood donors. This is supposed to suppress his immune system IF it is MMN, multifocal motor neuropathy. Good luck with your husband too! Pam

Kathy ~ From the NIH website the definition of MMN is multifocal motor neuropathy which is a progressive muscle disorder characterized by muscle weakness in the hands, with differences from one side of the body to the other in the specific muscles involved. It affects men much more than women. Symptoms also include muscle wasting, cramping, and involuntary contractions or twitching of the leg muscles. The disorder is sometimes mistaken for amyotrophic laterial sclerosis (ALS, or Lou Gehrig's disease) but unlike ALS, it is treatable. An early and accurate diagnosis allows patients to recover quickly. Good luck. Pam

[pmbenb83]

KateO ~ I'm so sorry to hear that the treatment was not successful for you. But please do not to worry about raining on our parade, because as I said to Patty, we are being very guarded with our feelings. We too are concerned that this could be a fleeting moment. We will see the doctor again in December and she will be able to tell us if it worked or not. For now, we are thankful for our glimmer of hope and will keep praying that it continues. :)

I noticed that you live in Montana, is this correct? Is there another Johns Hopkins other than the one in Maryland? We are in Northern Virginia and thought of going to Johns Hopkins for a second opinion. Right now he is being seen at UVA and everyone there have been wonderful. We are prepared to get a second opinion just in case he is given the ALS diagnosis, but not because we aren't happy with their staff.

Thank you for your words of caution. I do appreciate you sharing your experience. I think this forum is a great place for us to exchange notes, because doctors can only do so much and the rest is left up to us to deal with.

Take care and have a nice weekend.
Pam B in Va

[Al]

Hi Gina. They do the IVGG infusion up here. They tried it on me with no success just after diagnosis on tthe off chance that I had MMN. I also tried 6 months of chemo with Cytoxan. No luck there either. Apparently I have ALS rather than an auto immune disorder. I thought at the time it was worth the try. Glad to hear it is working for someone here.
AL.

[KateO]

Pam,

Good, I'm glad I didn't rain on your parade. Looking back, I just wish my doctor had told me that with ALS it isn't that uncommon for IVIG to work the first time.

I do live in Montana and no there is not a Johns Hopkins here. Wish there were. Would save me a LOT of money. We do not have an ALS Clinic here in Montana. I really do love going to the Hopkins ALS Clinic. As I always tell friends, "I expected them to be brilliant. I did not expect them to be so kind". I appreciate that the Clinic's focus is helping me LIVE with ALS. If you're considering going to Hopkins, I'd strongly recommend it.

Take care. As a woman with ALS, I thank you for taking care of your husband. It's not easy.

Warm regards.

[arlie]

could you explain exactly what this treatment is-i am in toronto and have never heard of it-arlene

[Al]

They hook you up to an IV of Gamma Globulin. Tthey do it if they think you may have an auto immune disorder such as Multi Focal Motor Neuropathy rather than ALS.
AL.

[patricia1]

Is that the same as IVIG AL

[pmbenb83]

Hi Patricia and Arlene
Yes it is the same as IVIg. (Intravenous immunoglobulin) It suppresses the immune system and multiple sclerosis and Guillain Barre patients seem to have a good response with it. The nurse and doctor said that they still don't know how or why this works. She did say that even if he has good results, it doesn't clear him from ALS, which we were hoping it would. The infusion center that he visits is extremely full of people having it done. I was very surprised by how many and with various different illnesses.

My husband is going next week for his second treatment. He met with his local neurologist this past Monday and she saw some improvements in a couple of areas and a decrease in other areas. His left hand is getting a little weaker now and his fingers on both hands cramp and curl under at times. His doctors are still confused because he has symptoms of both MMN and ALS. He will go back to UVA in February for another EMG to see if there are any improvements/changes. If he has improvement, then I guess he will have this treatment regularly.

For now, all we can do is wait and enjoy life to its fullest.... with 3 kids in college, it doesn't take much for it to be our fullest. LOL In the meantime, we're still hanging on to our "glimmer of hope."

Take care.
Pam B in Va

[crystalkk]

Pam b,
Hang onto that "glimmer of hope" and have a Happy Holiday.
Positive thoughts may bring positive things!

[AHands]

Where/how do they come by the donated Ig?
Do you get a relative to donate?

What symptoms differ between ALS and MMN?
How does one determine if one is a candidate for IVIg (besides physician's advice)?

Personally, I have a close relative anxious to donate bone-marrow for me, but I haven't found info on that--what I've seen with respect to bone marrow for ALS has to do with stem cells and they use the PALS own marrow, not a donor's. I think they get stem cells from the patient's marrow and inject them into the patient's CSF, but I might be wrong.

Regarding the immune system attacking the nerves: I think this is "normal", that is the immune system will destroy the nerves IF given a chance. That's why we have a "blood brain barrier" that allows blood, but prevents antibodies from getting to the brain and nerves. Thats also why infections in the brain and spine are so dangerous--no antibodies to fight them, and many antibiotics cannot get through the BBE either. Fortunately, most viruses cannot get through the BBE either.

I think an autoimmune attack on the nerves can be distinguished from ALS by the EMG/NCS that we all know and love :wink: I think if your nerves are being damaged by your immune system, it will be the myelin sheath that is damaged, and that will show up on these tests. If the myelin sheath is damaged, you probably have something like MS as opposed to ALS.

This is all a lot of "I think"s. I am NOT a health care professional, just a guy with motor nerve problems and Google. If my understanding differs from yours, please let me know!

[Al]

I think they get it from blood donations and spin it off. ?
AL.

[Thumbs Up]

Yes I know what you mean. When I read the posts I feel so bad for everyone because I've been there, I have held back answering some because I afraid I will say the wrong thing. I hope so far I haven't offended anyone. I really do want to help.

[Al]

Hi Thumbs. I used to work with a guy that always said if you never make a mistake, well you probably didn't do much. So don't worry about things. Some people will always have something to say. As long as we don't offend intentionally we're all OK.
AL.

[pmbenb83]

Hi Crystal ~ Thanks for the words of encouragement. You have a wonderful holiday too! ;)

Hi AHands ~ I think only the doctor can make the decision to give someone IVIg. I agree with Al, I think the Ig is just from donations. We never thought of donating for him. I will ask when he has his appointment next week and I will let you know what they say. Have a nice holiday!

Pam B in Va

[AHands]

okay...yeah...blood donations. i was wondering if it was the sort of thing where its best to get the donation from a sibling, or otherwise "match" the donor? i realize its not "whole" blood, but Ig is the antibody part, right? therefor there might be a concern about autoimmune response in a poor match? i don't know.

i don't get the part about IV-Ig suppressing the immune system? I thought Ig = antibodies, therefor strengthen the immune system? Maybe since they're introducing foreign Ig they also administer some drug in conjunction which suppresses the immune system?

when you said "but his GM1 didn't support it" i was thinking "GM1" referred to a person, but now I see "GM1" is a component of blood plasma, or a blood test, right? i guess the blood test is really a count of anti-GM1 antibodies. i gather that its when this test comes back outside normal that they consider IV-Ig treatment? Kudos to your husband for pushing for the IV-Ig treatment even without the abnormal GM1 test! it seems increasingly evident that, as one doctor put it, you won't get the kind of aggressive treatment a doctor would order for a member of there own family unless you research and push.

it seems to me that in general medical science is sort of reverse-engineering--we start with symptoms and work "backwards" for years, centuries, or longer to the cause(s). (maybe thats the difference between "science" and "engineering"?) so, we put a name on the symptoms, and later discover that there are truly several "diseases" that fall under that name. diabetes seems like a good example: the first noted symptom was high sugar in the urine. eventually it was discovered that the severe "type I", or "child-onset", diabetes is an autoimmune problem wherein the immune system destroys the pancreas, while "type II", or adult-onset, patients have a healthy pancreas, but some unknown something prevents the normal insulin effect from occurring--either cause results in the same symptoms. Hence, two diseases with one name.

ALS seems to be at LEAST two diseases--we can already distinguish "familial" vs "sporadic", and i bet it wont be long before sporadic als gets subdivided into multiple causes, and possibly distinct treatments. but for now "untreatable" seems to be a big part of the definition of the disease. So if IV-Ig, or any other treatment, improves a patient's situation, we conclude they must not have had "real ALS" to begin with. Here's to hoping it turns out that NOBODY has "the real" McCoy! :-D

[pmbenb83]

Hi Ahands,
I don't think anything else is added to the Ig, but I will ask. I wish I would have thought about all of your questions before his first treatment. I think we were both thinking "just do something to fix him" and did not really research questions for the docs. Thanks for your inquisitive mind. :) I think my mind is exhausted and it helps to have someone new with questions to get it jump started thinking again. lol

Take care.
Pam B in Va

[AHands]

Regarding getting Ig donations from family members: apparently IVIG treatments are manufactured from "human plasma derived from pools of 3000 to 10,000 donors".

Its apparently been FDA approved and used for a number of immune disorders for over thirty years. Its thought to have potential for Alzheimer's, MMN, MS and maybe ALS, but not studied enough in those applications for approval.

It's also very expensive.

Theres a recent article over at the-scientist.com about an IVIG supplier testing their product for West Nile Virus contamination (all clean) and discovering that preparations made from people exposed but immune to WNV may have potential for helping those who succumb to WNV.

(PS Pam, I went to jrhs in manassas and hs in dale city)

[donnah]

Dear Pam,
Hold on to hope, you did the right thing by diving into the treatment before everyone came up with "questions & concerns ". My Ben died 12/6/2006, he had ALS we knew in the end he had it because I fought tooth & nail for him to get the IVIG treatment. In the end we saw no improvement at all, but if we did not try I would have wondered the rest of my life "what if?" So my thoughts and prayers are with you and your husband I cheering you on ........................... So hold tight on your "HOPE"


Donna
Caregiver to Ben

[pmbenb83]

Hi Donna,
I am so sorry to hear about your husband. You sound a lot like me... trying to find some way to make this stop and not giving up without a fight. Thank you for your encouragement; we really appreciate it. This time around, the sickness from the treatment hasn't been as bad, but he isn't sure if he will try it again. I support whatever he wants to do or try... within reason. :)

Thanks again for your words of encouragement. Have a Happy New Year!
Pam B in Va

[pmbenb83]

Hi Ahands,
I did find out that it takes a huge amount of donations to make one treatment possible, so donating for one particular person is out of the question.

Dale City, eh? I know that area quite well. I grew up in Springfield and my husband's only competition in the late 70s went to Woodbridge High School. (My husband and I dated all through high school) Things have really changed around here. If you haven't been back lately, you'll need to take a drive up, but bring a new map. :)

Happy New Year!
Pam B in Va

[Peg B]

Hi Pam,

Great thread. I somehow missed it until today. Happy New Year to you and your husband and may the treatment/test IVIG, continue to support positive changes for your husband.

For information to others. I get my IVIG every two weeks at home. I will have another EMG/NCV in April to "determine" the amount of effect of IVIG on my body. At this point I am still MMN or "Atypical ALS." My last EMG/NCV was Dec 7, 2006. Subjectively it is hard to tell if I am "getting better" or worse or staying the same. I am definitely less tired after the IVIG. At my visit to the ALS clinic in Aug 2006 it looked more like MMN, at Dec. 20 visit, it looked more like ALS, hence the need for another EMG. Whatever I have she said it is super slow and will take "decades" to do me in. My symptoms began in 2002, I am positive EMG in 3 limbs for ALS and have no GM1 issues. According to the internet 80% of the people with MNN have GM1 issues. That leaves 20% who don't. So I remain hopeful and grateful. Respectfully, Peg

[pmbenb83]

Hi Peg,
Happy New Year!

Eric is very similar to you in that he did not have the GM1, he had a positive EMG in all 4 limbs, no conduction block, no Babinski's sign, no brisk reflexes, so he is either MMN or atypical ALS. His doctors have also said that whatever this is it is slow moving, so that is a good thing. :) His doctor wants him to do the IVIg one more time in February and then go back to UVA in April to see if he has had any changes. This makes him so sick that he questions whether he should keep doing the treatments. Also, like you, he has more energy afterward. Again, it seems like his arm has grown a little bit, which really gets me excited, but then after a few weeks it goes back down in size. Kind of strange.

I want to wish everyone on the forum a healthy and happy New Year!
Pam B in Va

[AHands]

I go back in two weeks to get my blood, emg/ncs and ct scan results and am hoping the blood tests will show positive for the anti-gm antibodies. thanks to you all for giving me that hope! i asked the latest neurologist (#5) about ivig and he said yeah, go for it if you get the gm1. sure, i said, but what if the gm1 test does not indicate for it? oh, i seriousy doubt they'll let you have ivig without it.

it sounds like the two of y'all have gotten ivig without gm1 indication. whats the downside of ivig? i suppose expense is one--$1000/dose and doses are what...1 per month? if one sees improvement after the 1st administration, but not after subsequent...maybe it makes sense to stop and try again in 6mos or a year? insurance is covering my riluzole, maybe ivig too or instead of riluzole.

for those who experienced positive results after 1st treatment but not subsequent--does that mean after the 2nd treatment things remained the same or relapsed?

[Peg B]

Hi Ahands,

The cost is 10,000 dollars a dose. I get it every two weeks and my insurance pays for it. It must be like kidney transplants in that the gov or someone chips in. I do not pay out of pocket and I started last Jan (07). Wow! Tons of money. They told me at the ALS clinic that individuals do not pay for it. I don't ask too many questions re: cost. I do know that the person who schedules my treatment is in PA and I live in MI. I have been told there are only 4 nurses in Mich. who do home treatments and they travel all over. What a blessing to get it at home. The other downside is side effects. I got heavy flu symptoms the first time but a does of liquid prednosone, and a few other meds before treatment take care of it. I sleep for 6 hours with my dog at my feet and eat cookies whenever I wake up. For some reason I crave the indulgence:-D. So it is not much of a downside on that part.

As far as results I could not move my baby finger at all in Sept.. 06. After treatment I could move it to the right but not to the left. I am not as tired at all but get more tired as it gets closer to treatment time. However, another finger is beginning to curl and I still cannot write very well, as my strength has not returnede. We will see what the EMG says i April. It is true though that neither Eric nor I are positive for GM1. Most but not all, are positive for GM1, but some do not get IVIG as it does not help them, but they still have MMN and not ALS. MMN is also progressive but much much slower. IVIG does not stop the underlying progress but slows the process and treats the symptoms. At least that is my understanding. I wish you the best. Please keep us updated on what happens with you. Sincerely, Peg

[AHands]

OK, Thanks for the info...
Counting the days until, and gathering questions for, my next appointment...