Even during the first sleep study I noticed alot of jerking in my limbs and sometimes my trunk as I started to drift off to sleep. This increased over time and became so severe that it often went on for 6-7 hours. In sheer exhaustion I finally fell asleep during the day in order to sleep at all only once it had worn itself out around 7 AM.
During the summer I was at my whit’s end as this was interrupting my sleep so badly that it was beginning to really irritate me.
The Biot’s Breathing is more a matter of low respiratory drive; not associated with air hunger generally so I usually don’t notice that I’m under-breathing. Trying to purposely breathe continuously like most people do completely exhausts me, so I don’t do that anymore.
It was the jerking at that point that was the most bothersome to me. I spoke to Dr. Hoff who was still in wait and see mode, but things started to become more urgent for me, as I didn’t know how much longer I could go on 2-4 hours of sleep a night. I knew that this jerking would have to be brought under control if I were ever to have any peace and quality of life.
Clearly some neurologist was going to have to handle this since neither Dr. Hoff nor Dr. Baird knew what to do to make it stop. I haven’t had much luck with neurologists so I figured that I needed to choose wisely or it would just end up going nowhere and I’d be stuck with the problem. Most neurologists I’d seen at various times of my life knew something was wrong but couldn’t put their finger on it, nor did they follow the thread for long before dropping it and shrugging their shoulders, leaving me to suffer in silence with a variety of presenting problems.
In August 2015 I began falling for little or no reason, about 4 times in just 1 week. My legs would just fail and I’d fall on my hands and knees, teetering on my big toe bent back under me. My left leg had started getting stiff and I had fasciculations in the foot and toes.
When I told Dr. Hoff about it he had me push up my left leg against his hand pushing down and my leg started jerking so badly I almost injured myself. I later found out this was hypereflexia, an upper motor neuron sign. He also documented weakness in that leg.
That day I found by the time I was ready to go downstairs to wait for my transportation home my muscles felt so tired and overworked just from a simple exam of muscle testing I could hardly walk that far. Every muscle in my body was spent, literally and I felt systemically ill. I knew something was seriously wrong.
Dr. Hoff was now sure I needed to see a neurologist because now although he had his suspicions of what was going on in my body be knew it would require an expert to nail down what was wrong, but he did not know who to recommend.
In the meantime he scheduled another sleep study including a few more leads that weren’t included before in previous ones, some to measure activity in my limbs, and he was going to also have me stay for a daytime one to evaluate me for narcolepsy. He explained that there were so many confounding variables that it might be hard to tell if it was narcolepsy that was hitting me unexpectedly or just sheer sleep deprivation because of all the jerking keeping me from falling asleep at night (I had been having some episodes at home and even out shopping where I literally fell asleep on my feet, in front of the computer while typing, and during several other activities). If I was able to get 6 hours of sleep that night then we might be able to know which of these it was, but if not the daytime sleep study wouldn’t really answer the question. Hopefully this would provide some useful information to the neurologist I was going to see next.
Unfortunately the combination of severe jerking and the various techs coming in to adjust the equipment kept me from getting the required 6 hours of sleep, so the daytime study was called off. The nighttime study did, however document the jerking on video which was quite overt and frequent. The leads were non-specific although the did document the bursts of activity. Not being a true EMG that was the best that could be done to document what was going on in my muscles in a sleep setting.
Dr. Marie Trotti and Dr. Rye both worked in the Sleep Center, so I read up on both of them, and since Dr. Hoff was considering that I might have a movement disorder I settled on Dr. Trotti since she had a background in movement disorders.
I scheduled an appointment with Dr. Trotti and had to wait about 2-3 months to get in. This seemed like the longest several months of my life but I stuck it out, as I really believed she could help me if I could hang in there long enough.
The appointment time came and went and try as I might to stay on top of my medical transportation they failed to show up and I had to wait yet another 2 months to see her. I was literally in tears when I realized there was no way to get an urgent appointment any earlier. Emory was just too backlogged with patients. On top of the jerking keeping me from getting quality sleep, and the sudden sleep attacks at odd times, I was rapidly losing my ability to walk. This was very scary as I was becoming very disabled very quickly and it was becoming difficult to do normal activities around the house.
The only consolation was that once I finally was an established patient of Dr. Trotti’s maybe she could do all the necessary tests to solve this thing and maybe there would be some treatment that could help me. One could only hope. I felt that maybe finally my plight would be over once I got in to see her, but that was not to be.
Instead of taking on this challenge she explained that she was “this sleep person” her main interest currently being in Idiopathic Hypersomnia, and I found that her zest for new and unique cases seemed to have given way to the day to day narrow focus of routine and maybe less interesting ones. This was not the person I’d read about on the internet who looked at sleep as just a part of the big picture. She had become someone different now, content to look at the sleep aspect only. It was a shame because while there is a need for people in that sub-specialty I felt she could have aimed higher and maybe gone on to find cures for some really major and complex diseases if she’d wanted to. She definitely had the capacity. I liked her but was disappointed and not looking forward to being thrown back to some other neurologist to finish a job long overdue.
She documented hypereflexia and weakness in my left arm (so now between her record and the pulmonologist’s record of same in left leg I now had some idea of what was really going on and I could tell so did she, yet she was declining to take me on as a permanent patient and was going to do two tests (a cervical spine MRI and two blood tests to rule out Myasthenia Gravis) both things she thought least likely to be the problem.
To her credit; she did take the time to look at the films of my sleep study and give me the diagnosis of Sleep Myoclonus, and prescribed Keppra, an anti-convulsive.
She looked over my recent blood pressure readings at various Emory appointments and questioned why my GP wasn’t treating the intermittent high readings. Some were high altogether and some just one number (systolic or diastolic high). I told her he probably just figured it was due to the underlying condition and once she figured it out maybe it would be treated by treating the cause.
The two or 3 tests she ran was where she got off the bus so to speak, leaving a referral to Emory’s General Neurology Dept. (which was going to take a minimum of 2 months to get into. By this time I didn’t have two months to wait.
I called back and asked that the referral be sent to the Neuromuscular clinic directly since that was where General neurology would end up referring me anyway, and I knew that would take at least 4 months if I took the added step going to General neurology first. She did do that much and then my GP also sent a referral to Neuromuscular, but the two referrals sat there for about 2 weeks with no reply and I was not put on the schedule.
The man who runs that clinic is Dr. Glass, one of the top ALS specialists in the country. I read up on him and I thought if anyone had a chance of helping me it would be him. I figured why not be evaluated by the best and know for sure whether this was what I had, (or whether to head in another direction).
My GP tried finding out what the hold-up was and was told he was out of town and a general scheduler told me that he was “really particular” and had to review a patients’ chart and then give only his own nurse permission to put a new patient on his schedule.
She told me his nurse, Holly Doe would be calling me. I waited patiently but no call came. I continued to get more disabled and had increasing spasticity for which I had to be prescribed Tizanidine. That helped until it wore off and I had to take it again, but at first I could only tolerate a half dose because full dose made my neck and vocal cords too weak and it ached when I spoke.
I had to ramp up from a half dose, to 3/4 dose as the spasticity increased, and then to a full dose.
As we awaited word from Dr. Glass’s clinic I started having some episodes of choking on saliva and phlegm when lying down or sleeping. I urged my GP at Emory to please be more assertive and ask Dr. Glass to take the referral and also to get Dr. Trotti the last neurologist on record to step up and do something to help me. She couldn’t in all conscience just do nothing when she had the skills and ability to help me.
There was going to be awhile before another neurologist would pick up my case and since she didn’t finish the testing necessary to be accepted into Dr. Glass’s clinic, when I then followed up and my GP followed up shortly thereafter, I was then denied entry.
This was mightily unfair and cost me precious time which I considered may in the end cost me my life, and it gets worse from there.
It was then I realized I really should be in the hospital, as I couldn’t even tolerate being in an upright position long enough to go back and forth to the doctor with non-emergency transportation. Often it took 3-4 hours sitting upright in my wheelchair to be picked up to go home and by that time I was in excruciating pain due to the spasticity in my butt, legs, hips, and upper body because of gravity on the muscles.
Cold weather on my arms triggered myoclonus really badly even during waking hours in my shoulders and it was unbearable. I really needed to be in bed, and since my wheelchair is a hoveround it places all the weight solidly on my core and there’s no way to evenly distribute my weight so that my muscles can be properly supported. I need one with a head and neckrest and the ability to recline in order to make such trips out.
No matter how much I tried doing the usual things I was getting more and more weak and exhausted. Lifting pots in the kitchen to cook was exhausting and sometimes just lifting glasses was taxing so I had to devise creative ways to carry things, put them on my lap, the small footrest on my wheelchair, and finally had to use mostly Styrofoam cups.
Finally I found it nearly impossible to bathe any longer. I could no longer lift my legs high enough to step in to stand and take a shower and could tell that taking a regular bath was out of the question because my arms were no longer strong enough to pull me up and out.
The last bath I was able to take at home was to sit on the edge of the tub and splash and wipe soap and water on me with a washcloth. I didn’t have the strength to do it for long.
I asked at that point since my GP was talking to the last neurologist whether she could do a direct admission since the dysautonomia was getting really serious and my disease was progressing at a fast rate. Something changed at that point and the GP I’d seen and trusted for 12 years suddenly started becoming very evasive. He wouldn’t answer my question and his responses became guarded as though he was being coached what to say. It was very unsettling. I must have asked that question 6 times without getting a straight answer.
He never used to keep things from me before but it seemed as though something had definitely changed. We’d had a good working relationship prior to that.
Since he was my primary care physician I asked him to be assertive with her so that I wouldn’t be left without a neurologist at this crucial time. He seemed increasingly cagey as if we were no longer a team and he’d chosen other alliances, running counter to my best interest. As ill as I am that was the last thing in the world I needed.
He would only say “If you feel unsafe at home you should go to the ER. Call 911 if necessary.” I wondered why he was laying this solely at my feet and wasn’t advising me as my doctor or trying to help facilitate. Something just didn’t feel right.
He never did tell me (until much later on the phone) if Dr. Trotti had admitting privileges at Emory neuro unit, so one day I decided to Google it, and it appeared she in fact did have them! (Later Dr. Baird told me that Emory’s idiosyncratic system says everyone has admitting privileges when in fact they don’t. I don’t know why this is but nobody would automatically know this and it’s very misleading, causing lots of unnecessary upset to patients). All this time he’d let me go on thinking that she was just choosing not to help when in fact she couldn’t. To this day I still don’t understand why he just didn’t explain this in the first place.
Of course it would have been prudent for some doctor to have admitted me for tests and observation at Emory and fast-tracked the classical ALS evaluation process while they stabilized the dysautonomia. It seemed like the logical and safe thing to do.
I didn’t feel good about the deceptiveness and that I was not privy to all that was going on since afterall this is my body and I had a right to be a full partner in the process.
The bits and pieces of explanations I was given were not adding up and I was left to read between the lines, with a feeling of uneasiness.
Finally there was a new neurologist scheduled for that coming Friday, and then an EMG scheduled early in December. I was going to try to hang in there until I could get into the General neurology clinic, but try as I might that was not to be.
Each day the dysautonomia got worse and finally around 4 AM Wednesday morning I nearly fainted several times while lying flat on my back, having hot and cold spells, and sudden urge to defecate.
I knew at this point there was no way this was going to be possible on an outpatient basis, so finally called 911. The ambulance was on the way to Emory when base told the driver to divert because Emory was overcrowded.
The driver asked which other hospital I had a preference for and I told him Piedmont since my father who had worked as a cell biologist at Emory chose Piedmont in the later years of his life. My father never really took good care of me but never failed to procure the best care for himself.