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tripete

Very helpful member
Joined
Dec 5, 2014
Messages
1,002
Reason
PALS
Diagnosis
12/2014
Country
US
State
PA
City
Lancaster
I am curious on how different PALS progression occurred?

My breathing muscles have been the first to go. At first I noticed difficulty breathing at times when I should not have had problems. For example I was an avid runner and I began to notice that at relatively short distances I was loosing my "wind" much quicker than seemed normal. I chalked it up to humidity, not enough sleep, a cold coming on, etc. Then I notice carrying equipment (20lbs case) across a parking lot that I could not breath. I went to the hospital thinking it was a heart attack only to be told I was fine and to follow up with other docs. Two years later my diagnosis.

My hands and forearms also began going early (all though I did not understand what was happening) particularly the left one. It started when I noticed intense muscle burning in the forearm and hand when driving. I wold hold a hot or ice cold cup of coffee to sooth the pain. Slowly I noticed the muscle in between the thumb and pointer finger begin to disappear. Two Fridays ago I fell and tried to catch myself with my left hand (and my face), and now my hand is clawing and difficult to use.

My legs have held out pretty good. I have visible wasting in both feet and around the knees, it is more pronounced on the left side. My balance has been very bad, stairs extremely difficult, extreme tightness, and the fatigue wipes me out very quickly. I have lost my balance and fell into walls, windows and cabinets (thankfully not to the floor, only the asphalt the one time and that was me being stupid). I get cramps around the hips (getting in and out of the car) and in the thighs and calves constantly. I am assuming it will not be long before I loose my legs but am curios as to how others progressed who did loose the use of their legs?

I am also loosing my ability to sit up, I slouch a lot and when I turn in bed I rely a lot on my arms and legs as my core is just shot. Bulbar wise, choking is increasing and face and neck cramps are on the rise.

I hope by sharing these progression stories that others will be helped by knowing what is happening and planning for what is to come.
 
Hi Pete. I can share with you my father's progression as he also had bulbar.

He kept most of it to himself for a long time before the physical changes prompted others' concern.

He had sleep apnea and used a CPAP but started having difficulty lying flat so he would elevate the pillows. He was choking on thin liquid and started changing his food choices. At the same time he developed head drop which he attributed to a back problem. He also lost a significant amount of weight.

Around this time he wasn't able to do things he normally did like mow the ditch with a push mower. He was taking more and more naps and falling asleep in strange places (CO2build up). Then his voice started changing and becoming raspy.

It took about 10 months to confirm ALS. The progression seemed to slow a bit. A few months later he had more difficulty doing daily things like shaving, showering, brushing his teeth, getting dressed.

Tripping became a concern and he got one brace, then two. Went from a cane to a walker but could still drive. Then turning the ignition became difficult as well as getting in and out of the car. His progression seemed to stabilize again for a short time.

About 3 months before he passed, he had to use a wheelchair to get around as his breathing worsened. His legs, arms and hands still functioned but he was weak. His voice was becoming softer and more difficult to understand. His hands began to stiffen and curl. He was still eating normal food with supplements of Ensure to maintain weight. Then one day he could no longer eat (he had a PEG inserted at diagnosis but had never used it).

From here he went to having air hunger and needing morphine, became bed bound and required 24/7 care. He lost his voice but could communicate via a whiteboard. He started sleeping longer. He was in this state for two months.
 
Pete, I will share my husband's story since he isn't on our forum...

July '14 - slurred speech began

January '15 - We thought slurred speech was from some bony growths he had under his tongue

May '15 - Bony growths removed from under his tongue

August '15 - Called a speech therapist to see if she could help him with his speech. She was concerned something else was going on and told us to see a neurologist

Sept '15 - Neurologist ordered brain scan, lot's of blood work and emg.

Oct '15 - Everything else ruled out - EMG showed abnormalities - Bulbar Onset ALS... - Weakness also showed up in his both ankle areas but he had not noticed

November '15 - It became harder for him to walk in his steel toed work boots - fatigue made it hard to work as well

Dec '15 - Second opinion - Bulbar Onset ALS - He quit work

May '16 - His speech is getting worse - I can understand him most of the time - Walking is more challenged all the time, each step looks like a struggle

June '16 - Speech getting worse - I have to ask him to repeat alot - his balance is affected now - he has noticed some numbness in his right index finger and thumb

July '16 - Speech is worse, most of the time I can't understand him - Walking is getting more challenged - steps are very difficult, he uses hand rails to pull himself up steps - balance is worse, he has to keep moving to keep his balance and/or hang on to something
 
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Probable first symptom: severe cramping in calves and upper rear leg muscles upon stretching in morning or sitting on hard chairs. Both legs.

Joined after a year by foot drop of left foot.

Diagnosed after ~18 months of the above.

Right leg started going, pretty well along after another ~12 months.

Started hand and neck cramps next, another ~12 months.

Breathing issues started slowly and sped up.

To now, another ~18 months, full time in PWC and 24/7 BiPap.
 
Probably: Twisted ankle December 2012 for no apparent reason. Stayed weak but recovered enough to resume walking several miles a day

October 2013 - Fell while "speed walking" Did not break fall with arms. Got TBI and had some cognitive issues

Summer 2014 - got cramps and twitches in left foot. Never went away. By November they were up my left leg into my glutes.

October 2014: Dirty EMG by local neuro

November 2014: Abnormal PFT; cramps in intercostal muscles

December 2014: Mayo (Dirty EMG/FVC reduced) "possible dx"

Jan 2015 - August 2015 - Harder to walk, cramps spread, twitches all over body, left hand atrophy; both calves atrophy

Went to three other ALS Clinics for opinion

Dx August 2015

Today: Harder to type; lots of atrophy in hands, arms, feet, legs. Hard to walk 1/4 mile; balance worse; voice raspy (have no idea if this is the disease, GERD, or thyroid issues.) I can still eat and drink liquids. Loss of coordination. Just got Trilogy. Cramps all over, pain, numbness/tingling in left leg from nerve irritation.

Gained 20 pounds since diagnosis. No muscle, just fat.
 
I can't edit my post to add timing which I overlooked.

From what we pieced together, my dad's symptoms probably started around January 2014. The weight loss, choking, CO2 build up, voice change and head drop were going on up until October. He had seen his PCP, an ENT, and a chiropractor but no one put all the pieces together.

In October he went to his office after breakfast and my mother had a feeling something was wrong. She checked on him and found him asleep in a chair (he had just gotten up less than hour ago). She tried waking him and it took three attempts so she called an ambulance.

He was hospitalized while they ran tests. They came up with a heart attack and pneumonia (he didn't have either). On the second day a neuro came in, suspected ALS and referred him to Chapel Hill. We thought they were nuts.

Two days later after the EMG, MRI, etc. he was confirmed. He passed away 13 months later in November 2015.
 
June 2014- notice pointer finger& thumbs both hands not working right. I.e. Can't tie knot in balloons.
Sept 2014- muscle atropy between thumb& finger both hands emg rule out carpal tunnel, Mri shows herniated disk between 5-6-7. Go see Neruo- surgeon First time hearing possible ALS
See muscle Neruologist refer to UVA clinic
Feb 2015 confirmed dx ALS. Since then life has been real different for me. Steady down hill dance to now I am 95% pwc
Breathing compermised especially laying down. Waiting for dam VA to get trilogy to me. 1 month into lunasin ( no results yet!). Hands- arms- legs - feet not good
Toe drop started early on so had braces right away w/ cane then rollators then pwc.
Can now tell it has entered my diaframe muscles.
My swallowing is still functional but cautious.
Also mucus in throat alot now.
If not in pwc I wall crawl from bed to chair etc but gonna stop this as I have had legs crumple and down I go.
When this happens I just go with it ,don't try to block w/ hands arms cause they have no strength and don 't want to break something. Sorry so long but great thread. Love ya all Chally
My last 10 k run was in fall 2014. Worst time ever and legs so cramped, knew something was up then. Gave up driving early on and stairs are out of ques. Massage once a week& good food& water
 
I appreciate everyone's comments. The common thread for progression appears to be:

Fatigue
Cramps
Noticeable Muscle Wasting
Then Loss of Use

The fatigue and cramps continue through out the whole of the process until the muscle wasting is complete and there is no more muscle to cramp.

This, for me, would be an accurate road map of the progression for my muscle failures including the muscles for breathing. I hope this gives others who are thinking about this an idea of what to expect.

Time frames from fatigue to loss of use will very greatly by individual. I suspect the point of origin and the muscle condition will play a roll in how long muscles last. For example; As mine was respiratory onset (I believe respiratory and bulbar to be different, and the experts argue it as well) it has been slower to attack my limbs. Being a Triathlete my lungs and legs where in very good condition. So my progression in those areas may seem slower as I started at a higher physical condition. My first FVC, even when I knew there was a problem, was 170%. So a drop to under even 100% would be very noticeable to me, and large, but not bad to others. The same would be true with all muscle groups. Naturally if you start higher, it will take longer to get to full loss of use. Also if it starts in a particular place it would seem logical that that point of origin would see the quickest decline.

I hope this helps.
 
Hi. My mom has ALS. She was found unresponsive almost 2 years ago. Before that she was having a hard time eating or drinking without choking. Her voice was as if she had a bad sore throat, it got so bad that over the phone I couldn't understand her yet she kept putting off seeing a doctor. She finally did and he gave her spray for it ! Ugh well she was found unresponsive and rushed to the ER it took a day or 2 for her to come to and the main thing was getting the breathing tube out . They tried few times and said she needed a track. She was in ICU for 2 months and Nobody had a clue what was wrong . We were then told she needed to go to a nursing home with 24hr care . Well it was the worst day of my life . This august will make 2 years. She is only in her 50s . She got to the nursing home and about a week later they started therapy she was doing great . Sitting up do all exercises but trouble standing on her legs. Well after 6 months they said could no longer do therapy. 2 months later she had foot drop and then her legs wouldn't move then few months later her arms didn't move now she can't move any body parts and she has had a feeding tube since this first happened from the hospital and kept failing swollen test . So now she lays in the bed , can't talk, can't eat or drink, can't move any part of her body YET has her mind 100% . :(
 
Chris had almost no cramping - only when he would over extend his arm as they were wasting he would get a cramp in a single muscle that was stretched more than it could handle.

Being bulbar he started with slurred speech and swallowing issues.

Then fasciculations in his upper arms, and rapidly lost fine motor skills in his hands. He was losing arm strength as the muscles were visibly wasting.

He had a lot of spasticity, so was very rigid and jerky in his movements.

As a cafe owner he used his mouth and hands the most, and that is what he lost first. He was left handed and his left arm and hand wasted and became useless much faster than his right even though it had been his stronger hand and arm. Until the fall when he ruptured the tendons in his right shoulder, then suddenly his left arm was the better one.

Chris experienced overall fatigue, due to breathing issues, but he did not experience fatigue in actual muscles as they were losing ability.

I think that progression is different depending on the degree of upper and lower motor neuron involvement as they both cause different types of symptoms.

Thanks for an interesting thread Pete!
 
This is something I often wonder about Pete. My husband Dave is my PALS; diagnosed 12/2015.

Summer of 2014, I noticed that my husband seemed to be going thru an accelerated aging process. He was 67 then, so I attributed this to age, however, he was tiring more quickly than usual and had lost his usual energy and look of vitality he always had. He frequently looked drained after doing usual activities & started to take naps, something he had never done. That Fall he strained his back stacking a lot of firewood, and then again in December shovelilng snow. We thought he had injured his lumbar spine or discs, but over the course of the next 6 months he saw 2 orthopedic surgeons (one who thought he probably had a pinched nerve somewhere that was not visible by MRI; the other surgeon said no way). His low back continued to hurt him, and he had cramps in his upper traps/top of shoulders, especially when driving. He also had these odd "spasms" in his diaphragm that would come and go. He had mentioned this to his PCP in the past and the PCP labeled them as spasms.

2015 came and he continued to lose strength and endurance but nothing alarming yet. His back continued to hurt & he thought maybe the decreased stamina was because he had cut back on many activities due to continued back pain & right leg pain. He continued to be able to hike with me 4-5 miles but with complaints of this pain, plus what he now referred to as right leg "weakness". This continued to get worse & he started to have decreased strength in his right knee extension and dorsi-flexion (bringing toes up) on the right foot. He had begun losing weight in Spring 2015, and this continued throughout the Summer & Fall, along with severe fatigue. He complained that his legs "feel like I ran a marathon", they were that profoundly tired. He incidentally also contracted Lyme's disease in July, and was treated for a month with the antibiotic and felt improved strength and endurance while on the antibiotic...... In early September I noted nearly constant little twithces in his right lateral forearm and right lateral calf. I was alarmed and watched them constantly. I didn't remember what they were called, but I knew if they continued, this was definitely a nerve disruption, and is not seen with too many conditions, one being the dreaded ALS. This prompted us to see his PCP in late December, who referred us to neurology. Dave still had no UMN symptoms, but the muscle loss continued and now the fasciculations were "florid" and throughout most of his body. We still had hopes this was a disc problem, (our PCP never mentioned ALS), so we consulted a highly regarded neurosurgeon as well. I had been in denial about the loss of muscle Dave had; didn't really see it as "muscle wasting", especially since he was still so functional and could still mow our 2.5 acres of hilly land with a self-propelled walk behind mower. When we saw the neurosurgeon, he also said he did not see enough evidence on Dave's MRI to diagnose a localized problem such as a disc/nerve injury; he also noted muscle loss between the finger/thumb of Dave's right hand. I had never noticed it, although Dave had mentioned some decreased grip strength in his right hand and some "arthritis" in his right thumb.

Late November/2015 Dave had an thorough physical and muscle grading test performed by a neuro-muscualr disease specialist neuro, f/b and EMG. Based upon a history of weight loss, extreme fatigue, complaints of weakness, noticeable muscle loss, fasciculations and the EMG results, we were told "this is most likely ALS"......

As of this date, Dave still has no UMN signs. He has continued to be eaten alive by the disease, and his trunk looks skeletal & his arms are very small. He can still drive and until recently could walk 1/2 mile. About a month ago he began to have shortness of breath with light exertion, which has now progressed to SOB even when eating. We had a Trilogy delivered this past Tuesday; he is having a hard time adjusting to it, and cannot fall asleep with it on. He has just started having some swallowing problems, particularly dry or firm foods (steak and pizza are now out). He is still quite functional with his "ADL's" (activities of daily living) and can shower, dress, button his shirts and tie shoes, albeit with difficulty.

So, long story, but his progression was: Slow loss of strength/stamina, started losing weight, extreme fatigue happening with more regularity & with lesser & lesser activity, (pain - just incidental and most likely another co-morbidity/problem), fasciculations began in limbs & progressed thru trunk and entire body, muscle loss became apparent, able to walk only about 1/4 mile due to back pain and quadricep pain, inability to walk up any incline due to lack of extensor muscles in spine and back/leg pain, slowly losing ability to walk up any stairs, SOB progression, and recently trouble swallowing firm foods.

I hate this disease!
 
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My husbands progression thus far:

For several years he has had stiffness and tightness in back and arms but has a job that requires him holding equipment and thought it was from that.

Spring2015: cramps and fasics in arms and legs, attributed to his physical job/dehydration/lack of vitamins

Sept2015: fall at work led to pain in hip (sciatica type pain). Didn't get better. Some tripping/clumsiness

Jan2016: hip pain worse. Affecting gait. tripping and falling.

Feb2016: difficulty starting with going up stairs. Can't go up in toes.

March2016: stairs worse. Fatigue. Neuro starts testing.

April2016: dirty emg.

Today: fatigue, difficulty walking longer distances, stairs are hard. Cramps, fasics continue. Still working.
 
Well I wrote it all out and then power failure which kicked me off. Here is the short version

Steve presented in May 2013 which mon stop fasciculations in his right bicep, very bad calf cramps at night, and difficulty holding writing tools, typing, and a golf club. Strength and ,endurance decreased, fatigue, frustration, and difficulty breathing increased.

Summer 2013 steve stopped playing golf due to cramps in abdomen, legs and arms. Said he couldnt breath out there. Starts with sleeping difficulty. Sporadic at best. Has anxiety and panic attacks

Fall 2013 cramps and difficulty writing increase, ability to breathe decreases

Christmas 2013 face plants rwice due to all over body cramps at once

Spring 2014 visits to pcp for allergy treatment, dx with double pneumon8s, has full cardio and pulmo workup. Dx...fast heart rate and beta blocker, pft 40s. Diaphragm paralyzed. Diagnosed in May with als

Summer 2014 thru christmas 2014 decline is occurring but subtle. Slurring when tired, sleeping longer, difficulty grasping and opening things. Head drop so severe he can no lonfer pick it up to look forward.

Jan to june 2015 a lot of muscle wasting happened during this time, His hands and feet became tendons and bones, his shoulders, belly,chest, leg muscles shrunk before our eyes. His bones became more prominent. He could still type but very slowly.

June 2015 to March 2016 bipap usage increase to 24/7. Diet changed from soft food to smoothies to softer foods. Choking increased, tomgue fasics started, slurring increased. Ability to care for himself mostly gone. The weeks just before he died, he choked several times, had been declared acute respiratory failure, became badly jaundiced , and developed an odor. He died from a fall from which he couldnt get up
 
I havent had cramping... Yet.
Possibly April 15 fall on stairs, surgery on left wrist
Sept 15 intermittent slurred speech and deteriorating hand writing and fine motor in right hand.
Nov 15 general fatigue
Dec 15 prob dx, constant slur
Jan 16 dx, and visible wasting in right hand
March 16 left hand starts to go, managing food textures to avoid choking
April-June 16 balance issues increasing and falls
June 16 fall, surgery to fix right wrist
Now pwc outside house, balance terrible, speech monotone, hands are weak and twitchy, arms can't reach up or out, help needed showering

I feel like I will look back at this and miss the function I have now, but it still feels so quick! 9mths ago there was almost no sign...
 
"I feel like I will look back at this and miss the function I have now, but it still feels so quick! 9mths ago there was almost no sign... "

It is amazing what we come to "accept" as the new norm or standard. I look back at a last year and almost long for the functionality I had then, even as depleted as it was.

There are days when I just wish this disease would hurry up and get it over with.
 
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