Is my course rapid?

[KarenNWendyn]

Hi everyone. I'm recently diagnosed with "a definite motor neuron disease, probably ALS" per my neurologist. Cramps initially in legs started sometime in 2016. In October '16, I had a strange sensation in my left ankle and thought I had a tendon injury. That sensation lasted 3 months and then improved. Leg cramps worsened and became nightly, involving upper and lower legs. Later I developed chest wall cramps with bending forward. I first noted some difficulty walking by February '17 and my gait was markedly abnormal by May '17 with bilateral foot drops and a steppage gait. Widespread fasiculations started in May. I noticed weakness and cramping in both hands by early July. A couple weeks ago, I finally saw neurologist who noted profound weakness in most of the small muscles of both hands and both feet and with evidence of atrophy. I am now noting some spasticity in my hands. I am hyerreflexic in knees and ankles. Today I had difficulty stepping up on a small ledge, suggesting some quadriceps weakness. My gait is becoming more labored, particularly due to the left foot, where it all started.
This seems like awfully fast progression
to me. I'm worried I may need a wheel chair in the next few months. I'm curious to hear if others had similar time frames of progression or if mine is unusually aggressive. It's so hard to make plans.
Karen

 

[Nikki J]

Hi Karen

Sorry you have to join us but welcome.

We would all love a crystal ball so we could plan! Failing that it is good to try to stay ahead of things. So if you are thinking mobility aid soon start working on that.

However, you say you saw " a neurologist". Are you working on a second opinion with a neuromuscular specialist? We always recommend that but especially as your dx seems slightly wishywashy????

Progression varies tremendously. I have a friend who after 10 years has one leg only involved. There was someone here from a FALS family where everyone died in 2-3 months from first tiny symptom. Most are somewhere between the extremes. Although there is a saying start fast finish fast progression is not linear and plateaus are quite common. All we can do is prepare early yet try to enjoy each day

 

[KimT]

If you have not done so, please get a second opinion from a neuromuscular specialist. My local doctor (neuro) did the first EMG and he was having trouble with the interpretation. I went to Mayo and they repeated the EMG. It was different and was interpreted differently. I went to Johns Hopkins six months later and their EMG results and interpretation agreed with Mayo Clinic.

Sorry you have to be here but we are a pretty good bunch and do all we can to help.

 

[soonerwife]

So sorry to hear about your dx and that we have to welcome you here. This forum is a great place for information and support.

I think everyone should get a second opinion for sure.

The power wheelchair process took about 4 months for us. If you think you are going to need one soon, I wouldn't wait too long. Although you can possibly get one from a loaner closet if you need it sooner?!

 

[KarenNWendyn]

Nikki and Kim, thank you for your responses. To clarify, the neurologist I saw locally is a neuromuscular disease specialist who runs an ALSA-certified ALS clinic. I have confidence in him. I do have an appointment in Portland, OR at OHSU for a second opinion. My local neurologist didn't want to definitively diagnose ALS because he felt he would need to see evidence of involvement spreading to either the bulbar or thoracic segment (I already have it in the cervical and lumbar segments with definite lower motor neuron findings of weakness, atrophy, and fasciculations but with hyperreflexia as the main upper motor neuron sign). Since I saw him, I've noted an increase in spasticity mainly in the hands-- another upper motor neuron sign. Tests done to rule out other ALS mimics were also negative.
So it really is pointing to an ALS diagnosis, but yes, I'll get another opinion. Given that diagnosis, I'm still curious to hear from others what they think of the time course of my symptom progression and how that compares with their own experiences and with that of other PALS.
Thanks again,
Karen

 

[Lkaibel]

Agree 112% on the specialist. I would go so far as to say I call it ALS when it's Diagnosed at a Certified ALS Center.
You sure can tell that ALS is a rare neurological disease because when it's even suspected, many a medical professional starts sounds very wishy washy and confused.

It's very hard to say on progression. Honestly it seemed on the speedier side at first with my husband, he was diagnosed just just 3.5 months from first symptom. However, it has been slower than average one year out. For one thing his progression halted for about six months.

Plan for tomorrow, take all the pleasure you can from today and see that specialist I say.

 

[Lkaibel]

Our posts crossed paths. Glad you saw a specialist but second opinions are good too.

 

[Nikki J]

Thank you for clarifying.

My sister went from first hand weakness ( couldn't do a button) to severe atrophy of that hand and loss of function in 9 months. At that point she had some issues in the other arm and beginning leg issues. In other 9 months she had little use of upper extremities and needed help to walk and started bulbar issues. However she lived 4.5 years from onset. By contrast I took more than 3 years to have it go beyond my first affected limb ( leg) and I can still walk with an afo. Are you taking riluzole? I believe getting it early helped me but maybe I am just lucky.

 

[azgirl]

Progression is so different for each of us.
Symptoms began summer 2013 with left foot
Diagnosed December
Walking with AFO and cane by march 2014, walker by Fall
Wheelchair by may 2015

Now I'm living one day at a time , still enjoying life even with the new reality.

For now AVOID FALLs, and we will help you get through this nightmare.

 

[KimT]

As far as progression, I discovered something was wrong very early. I've always been very tuned in to changes in my body because I was athletic, worked out daily. and loved sports. I fell running in October 2013 and suffered a TBI. After that my metabolism really surged but I still felt something else was wrong. The neurologist who was treating me for the TBI sent me to Mayo after I complained of cramps in my left foot during the Summer of 2014. I cancelled a few appointments and finally let Mayo do an EMG in December, 2014. It pointed to ALS but, at that point, I had no clinical weakness, according to Mayo's exam. However, I knew I was much weaker in my left foot, ankle, and my breathing didn't seem right. I also began having pains in the intercostal area and in my weak leg. It took until August, 2015 to get a diagnosis. My legs continued to weaken and I fell in September 2016 and suffered a very bad sprain to my "good ankle." After that I progressed to where I can only walk about 100 yards or so. I have lots of pain in my back, trunk, legs and hands. The clinical weakness started showing up just before my diagnosis. I had a nice plateau from May 2016 until I fell in September.

I can still drive and make a tight fist with both hands. I've lost fine motor skills in my hands but can still type.

My breathing is odd. It fluctuates. I have paralysis in my right vocal cord but the degree has not changed in nearly two years.

This is the most frustrating, scary, and mysterious disease I've ever encountered.

Please, protect yourself from falls. My first one was when I had no clue what I was dealing with but the second one was 100% my fault. I was practically running out of the grocery store, carrying four bags and twisted my ankle on the curb. I guess I'm fortunate it didn't break. Then I got back on it too soon and made it worse. My bad.

 

[KarenNWendyn]

Thanks to all who responded. I realize everyone's course is different. However, hearing other stories definitely gives me a perspective and lets me know what I might expect. It also makes this feel more real to me, as I still have a degree of denial mixed in with all the somber heavy thoughts of reality.
Bless you all.
Karen

 

[swalker]

Progression is a strange thing for us all. We know we will all have progression, but none of us can predict what it will look like!

It is hard to say when all this started, but I will use mid 2012 as the starting point. Looking back before that, I had issues, but none that could be traced to MND with certainty.

In about a 2 year period I had lost about 70 pounds and had significant impact to my breathing and swallowing. I remember doing a 1/2 mile walk that completely drained me. I was surprised I could walk that far. I was diagnosed at the 2 year mark and stopped working a few months later.

At about 2.5 years I started using a wheelchair, but could still walk 100 yards.

I am now at about the 5 year mark (3 years since diagnosis). I can walk 100 feet if I absolutely have to, but I should not.

My progression seems to come in spurts. It seems like I can go several months with no noticeable (to me) progression and then in one week I notice something(s) degrading.

For me, there are two key observations:

1. When I started using a wheelchair I virtually eliminated damaging falls. Damaging falls certainly seem to speed up my progression, so committing to the wheelchair was a significant component of managing progression.
2. Getting lots of rest not only makes me feel better, but also seems to help control progression.

We are all different and because those two factors seem to help me is no guarantee that they might help others.

Steve

 

[KarenNWendyn]

Steve, thanks for your detailed reply. Best of luck to you.
Karen

 

[Dianee]

Karen, my brother in law had an unusually rapid progression, according to what his doctors told my sister.
We buried my father a week before Christmas, 2016 and my BIL was fine, at least to us. In early February he started with "drop foot' problems
in one foot. By the end of February he couldn't walk or stand without a walker. In March, he was in a top Philadelphia
hospital trying to get a diagnosis. He could still breathe and talk at this point and had controlled upper body
movement. By late April he was vented and he was finally diagnosed in early May after being in ICU for almost two months.
After a three week stay at a long term care facility, it was determined that he couldn't be weaned off the vent and
he went to a nursing home for disabled people. This particular home has a wonderful vent care wing.
He passed away on July 11.
I mentioned my father's passing because friends from out of town who had known my brother in law
told me after hearing of his passing, that they noticed at Dad's funeral how sluggish BIL seemed to be and how he had noticeable
slurring in his speech. One of them thought he was recovering from a stoke! So it would seem the symptoms
started earlier, but we didn't notice them.
Don't know if it matters, but my BIL was a Vietnam Vet. Maybe a different strain of ALS?
I will keep you and all the other PALS and CALS in my prayers.

 

[KarenNWendyn]

Thanks for prayers. I'm so sorry to hear about your brother-in-law.
Karen