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duplinwino

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I haven't been on in a while and I hope everyone is doing well. I appreciate the messages!

I'll try to make this short but my head is spinning after a new visit to a new ALS Clinic yesterday. A little background, DH was diagnosed with MND/atypical ALS early 2007. He has had a fairly slow progression, he is total care now as he is unable to move his limbs. We've been following with a local well known neurologist that I've known for over 12 years, works down the hall from me, it has just worked out for us to stay with him.

Well, it has gotten to the point that resources are hard to come by and we wanted to give the multidisciplinary team a try. Why we haven't before now is hard to explain but I digress.

After reviewing DH's records and examining him, he doesn't think he has ALS. His EMG in 2006 showed conduction block in two nerves which is atypical for ALS. He had brisk reflexes about a year ago but now has little to no reflex at all, which we know, ALS patients have brisk reflexes. He was 30 when diagnosed, not unheard of but very young. And he has little to no bulbar involvement at this point. He feels his picture better fits MMN.

This blood test was done back in 2006 and came back negative. This new doctor wants to send it off again, to a lab that specializes in this antibody (?) and it will be back in four weeks. He also wants to do another EMG to look for conduction block, though his nerves may be damaged at this point. We discussed IVIg.

He did a pulmonary function test with the nurse at check in and he wanted the respiratory therapist to redo it because it was so low. The RT comes in and yep, sure enough, his FVC is 40. His NIF is -58 (negative), normal 100-110 so he is now going to be placed on BiPAP.

My question is, does anyone have experience with this? His limbs are so wasted after almost 4 years of progression and his diaphragm weakened, if he does indeed have MMN, is IVIg going to benefit him? Other than maybe stop his progression?

I'm really looking for any and all insight here. Thanks so much!

Ashley
 

rose

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Ashley,

Laurel's husband was originally diagnosed with ALS, and it changed to a rare version of CIDP. She, herself has a nursing background, so might also have a better handle on some aspects of these similar conditions ,than others, to begin with. She's not been super active on here lately, but I'm sure if you sent her a message she'd get back with you.

Your news sure sounds like there's hope for treatment!
 

laurel

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I'm Rose! Ashley going the IVIG route is well worth trying. My husband had two to three years of misdiagnosis and by time he was diagnosed correctly, he had wasting and loss of the right hand and forearm and drop foot in the right foot. He has been on IVIG since 2007 monthly. Of course the atrophy won't go away, but he is able to use his right hand now (before he couldn't use eating utensils, put a key in a lock, turn his ignition key in the car etc. Now he can do those things--albeit a bit clumsily. His mental concentration was poor and he had excess saliva and word seeking problems. Those things seem better too. If IVIG doesn't work there is plasmaphoresis and IV steroids. Hubby had the antibody tests through Athena Labs. and those were negative. He had slightly elevated protein when they did the lumbar puncture. I believe he does have a conduction block as well. Push, push, push for some treatment ASAP. There is no reason the neurologist shouldn't give him a loading dose of IVIG right now while tests are pending. The loading dose is 2 Grams of IVIG per every kilogram of his weight. i.e. if he is 100 kilograms or 220 lbs in weight, he would get 200 Grams of IVIG. This dose would likely be spread out over three days as they only infuse so much in one day. If he does get IVIG ordered, ensure that he drinks lots and lots of water prior to the infusions and during as headaches are a huge problem for those receiving IVIG. And many people take a Bendryl and a couple of Naprosyn before the IVIG to ward off the headaches. These are things that we learned on the CIDP forum as the neurologists seem to omit these things. Also many neurologists seem to skimp on the IVIG (due to extreme cost which for hubby is about $10,000 monthly) and patients on the CIDP forum comment that the IVIG didn't work as they never received proper loading dose. I had to advocate like crazy for my husband to ensure that he got his IVIG promptly, and his neurologist still thinks I am a pain in the butt. Good luck and keep us updated please. Also, I think Peg has MMN and is member of this forum.
Laurel
 

laurel

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Sorry Rose--of course I'm not you <g>. Meant to say I'm here Rose. I'm rushing around multi-tasking--feeding horses, dogs, and getting ready to cross the border and shop in Lynden, Washington.
Laurel
 

duplinwino

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Thank you Laurel and Rose! Laurel, please tell me this is covered under your insurance some how. The doctor told us yesterday for the cost and my jaw dropped!
 

CAHPAH

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My wife used to go to a neuro at Washintion University in St. Louis. They do some pretty good research on MND. Last Nov they told my wife they discovered a new biomarker indicative to MMN. The information had not been published then and I don't know if it has been as yet. You might have your ALS clinic get in touch with them. If they are going to retest DH's blood they might want to add that to the test. His name is Pestronk (314-362-6981).

My wife did come back weakly positive for antibodies to this biomarker. They did one IVIg treatment which made here very sick for a week. Her response to the treatment was slight at best and they declined to do any more IVIg treatments for fear it might do her in.

From what research I did on MMN it looks like they have pretty good results in treating it with the IVIg treatments.

Best of luck to you.
Jim
 

Peg B

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Hi Ashley,

I was first DX with ALS but then MMN as my progression is super slow. I hope my story helps some:

My right hand cramping started early in 2002, and my finger began to bend. It stopped moving completely in 2006. From 2002 - 2006 I fell many times, became increasingly tired and had continuous trouble writing a check as my right hand was so weak. On Oct 31, 2006 I was given an ALS DX. This was done by a neurologist who then sent me to the U of Michigan - ALS clinic Dec, 2006. On the EMG I was positive for 3 limbs for ALS. But I haD no upper neuron (brisk movements) and yet no antigens in my blood work and only a partial conduction block in my right arm. But because my progression was so slow she Dx me with "Atypical ALS or MMN." she put me on IVIG beginning in Jan 2007 in order to help with the diagnosis. The reasoning went - If I have MMN, I will respond to the IVIG, if I have ALS, I won't respond to the treatment. I have responded well.

I began with a two day dose in my home. I experienced severe flu like symptoms - headache etc. at that time. When I called the clinic I was given compozine for the headache and nausea and it worked. Since then I always get, just before treatment, - compozine, 2 Benadryl, IV prednisone, and two acetaminophen (Tylenol -sp?). I have not had any side effects since that second dose. For about two years I had treatment every two weeks. We went to 4 weeks for a few months but I got too tired and more cramping. For the last year or so I have had treatments every 3 weeks. I still get the treatments at home with the same nurse who is now a friend. I sleep much of the time during the 5-6 hours it takes to give me the treatment. I cannot get it too fast as I react somehow but I forget how - itches or something. Anyway - I saw my neurologist last Thursday and I don't have to go back for six months. A second finger is starting to bend but I have responded well to the IVIG and my Dx has been MMN for over a year.

Regarding insurance: I do not pay anything for the treatment. My insurance covered it and then Medicare when I became officially disabled. I worked for 36 + years in education. I hope that gives you hope.

Other issues that come up for me are cramps in my legs. I drink tonic water with quinine - about 1 1/2 liters a day. The quinine pills are not available in the US but Faygo Diet tonic tastes great (to me:razz:) and only costs 99 cents compared to the others. Kroger brand is cheaper and OK. I also have restless leg syndrome and 1/2 vicodine takes care of that at night.

Please note I had a neurologist back in 2002 tell me I had a pinched nerve even when I suggested the possibility of MS or ALS. I also had one in 2003 tell me I needed to exercise more. YIKES! When I could not move my right pinky finger at all my regular Dr. sent me to a hand surgeon, we thought I might have broken it. That Dr. sent me to the neurologist who diagnosed me with ALS on my lunch break. So keep fighting and be a big pain for your husband's sake and yours too. My thoughts and prayers are with you. Keep us posted. Sincerely, Peg
 
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