Upon arrival the spasticity in my legs was unbearable from being in one position on the gurney. I asked them to please loosen the straps so that I could shift onto my side.
They wheeled me into Piedmont’s ER and the female paramedic listened as I told her how long and hard the past year had been and that I feared for the future. She held my hand until the staff was ready to bring me into one of the ER rooms. I was shaking a little, not knowing what would become of me but knowing it was serious.
The waiting seemed interminable. The ER doctor was a young slim man with a long neck and dark curly hair and a rather loud voice. Nurses and various other staff came in and out of the room asking me questions, checking my legs to look at the weakness in them, pushing, pulling, the usual routine. There were so many I couldn’t keep track, but they were on high alert.
My whole body had become hyperflexive; not just my limbs anymore. When they checked reflexes with the hammer even the opposite leg or arm jumped!
When they called in the neurologist on call and she and her PA both did the same exam I could tell she had not seen anything like it before. She was fairly young with medium length brown hair parted in the middle and introduced herself as Dr. Heidi Woessner. She was easy to talk to and non-assuming and told me she’d run some tests and be back in awhile to give me some recommendations.
She returned sometime later and despite her admission that she wasn’t sure what was going on I felt as though she would do her best to find out. She and her young PA told me they were going to try to speed the process along of finding out what was wrong with me. They decided to admit me on their neuro unit there at Piedmont. I breathed a sigh of relief that finally there would be some forward movement in the process that had up until then been stuck.
The challenge was that the hospital was full and so I had to wait in the ER until a bed opened up. I waited from about 5 AM until 4 PM to get a room on the 6th floor. Medications had been documented in my chart but had not shown up yet in the system and I was getting into quite alot of pain due to all the spasticity.
They told me that my doctors at Emory wouldn’t know I’d been diverted. I had my laptop with me but felt so awful it was awhile before I felt up to plugging it in to start notifying people. I needed to tell my son and a few others first; those who would be most able to help and those in the autoimmune community who cared how I was doing. I knew I needed to get someone to go by the house and take care of my pets and possibly bring me some things like clean underwear.
The man who does home repairs on my house had told me once if I ever needed anything to call him but as it turned out he could do nothing when the time came, as his own father was in the same shape.
My son had to take a bus up from Athens, GA. to Atlanta one day (similar to Greyhound because there is no linkage with the public transit system) and he spent the last $20.00 he had. None of his girlfriends’ family would bring him in their car. My son suffers from a disability himself. He cannot drive now because of a benign brain tumor called a D-Net made of excess neural tissue that presses on areas of the brain causing seizures on a daily basis; mostly Complex Partial but he has some Grand Mal seizures too.
His phone is often cut off for non-payment and I have my suspicions that he is just too generous with his money, although her income is higher than his. He always seems to be the one with the short end of the stick in that relationship. I hate to see him in that situation but can only wait for it to become intolerable enough for him to draw his own conclusion however long it takes.
They did one whopping MRI of the brain and spinal cord, and a spinal tap, numerous blood tests, etc.
The day of the MRI I suddenly had to pee not knowing it was going to take nearly 2 hours because they were actually doing 3 or 4 scans at once. They got a bedpan and lifted me up off the table and somehow that position with their hands on my spine caused me to jerk spastically backwards. It was kind of comical, and I couldn’t help but laugh saying one day this would make quite a story to tell my grandchildren. The whole thing was rather funny and I had to try hard to keep from laughing while I was in the machine, as they wouldn’t pull my pants back up after my using the bedpan for fear I might have to pee again. They just put a pad under me and covered me with a blanket.
I joked that I hoped I would not break their expensive machine, as the thing seemed to be overheating with the length of time I was in there. I think except for one area the pictures for the most part came out pretty clear.
I found that I got rather tickled after that at random times, even when I felt irritated, and did not know why. It was as if something was broken in my brain that I couldn’t quite explain and my emotions were all over the place. I alternated with periods of crying, but mostly laughing. It might be psuedobulbar affect, but I’m not entirely sure.
Then there was the time the aid was supposed to help me take a sponge bath at the sink and left me there and never came back. I tried to figure out a way to wash my feet, as I was having trouble reaching down. I finally just grabbed hold of my legs and lifted them into the sink. When the nurse came in she looked at me as if I were a naughty child, looking at me with a mixture of fear and disdain. “What are you doing!” she admonished as though I’d committed some awful faux pas . “I’m washing my feet”, I replied matter-of-factly. “I couldn’t reach them on the floor so I had to just be creative” I said, laughing. Something had to be done to break the tension around there, as you could have cut it with a knife.
“That’s not creative!” she said shooting me that look of disapproval again.
“Yes it is” I replied. “Come on, don’t you ever have days when you’re in a silly mood and joke around with your friends? You have to have fun sometimes”. The nurse remained stone-faced. “No”, she responded, “I don’t. You’re not right” she said shaking her head gravely. “You’re just not right.”
There was a blond-haired physical therapist that for all the world reminded me of Stewart Smalley, the guy on Saturday Night Live who was in that skit where he was always looking in the mirror saying “I’m smart enough, I’m good enough, and doggoneit people like me!” He was a bit irritating so I sent him away as I was never really sure what he wanted me to do, but whatever it was it seemed he was looking for a bone of contention and that in itself was tiring.
Dr. Woessner, guardian angel that she was had left for the Thanksgiving holiday, and her colleague took over the following week, so despite her best intentions things went downhill from that point on. Luckily her order for Morphine and Ativan to get me through the EMG was honored, although the first guy who was scheduled to do it kept unscheduling it and reminding each day’s attending that I’d unscheduled it the first time, as if to make some oppositional statement.
I was relieved when it finally was set that it was the other guy (who was much kinder and more mature). If you’re going to have needles jabbed into your muscles and your nerves shocked it’s probably a good idea that the one doing it isn’t holding a grudge, LOL. This test is a bit too reminiscent of a Cuban prison as it is.
I remember that day the young tech who was supposed to be there to offer comfort sat in the chair across the room staring uncomfortably at the floor. He’d worked there 23 years although I don’t know how someone stands a job like that witnessing that which is done to patients day in and day out for that length of time without becoming either hardened or turning into a puddle of jello.
I called over to him “Hey dude! Please! Come on over here and hold my hand,” to which he sheepishly complied. At the worst of it I let out a rather guttural yelp followed by a few rapid breaths (I have never been able to scream normally), and I think his hand sweated worse than mine did. Oddly I found that comforting, as it let me know that he still had empathy even after working in a place like that all those years.
The man administering the test only did a few areas on the left side of the body and seemed a little sloppy about it. Although maybe at least in part he intended to spare me, he failed to document the rate, so I don’t know if the test was sufficient enough to be valid. Since I am unable to point my toes he had to do it for me. He quickly ruled out a bunch of stuff and it seemed it was over in about 20 minutes, tops. He saw something but said he didn’t know what it was (story of my life).
My vital signs were all over the map and I found that there was one particular male nurse who about 3 days prior to my discharge seemed to want to manipulate the readings until it was somewhat more normal before having an aid record it. When I realized what he was doing I called him on it and he stopped. It was strange to say the least!
On the day of my discharge the attending told me that Administration had been on his back for a week to discharge me because I needed a specialist in movement disorders and they felt ill-equipped to provide more than supportive care. He said I really needed to go somewhere like the Mayo Clinic, John’s Hopkins, etc. I don’t disagree, but I was by no means well enough to leave the hospital and he told me that they couldn’t do a direct transfer unless I were going to die within 72 hours. He told me he hadn’t had any luck with that for the past 13 years.
The conundrum about testing he said was that at Piedmont hospital if you have fasciculations in your tongue they give the ALS diagnosis hands down even without the tests but if they are in another body part (such as my foot as it was witnessed by the man who did my EMG before he even stuck the first needle in) they brush that aside, as he says they are not a hospital qualified really to diagnose such things. I don’t want to hate my body but now I do as it seems not to be cooperating in getting the necessary information so I can do something about these symptoms.
Since it was a Saturday and I had no way to get orders written by my Emory doctors until Monday I asked him if he’d write the Saline fluid order for home healthcare to start an IV with the next day. He said he would since he acknowledged they’d determined my dysautonomia to be chronic now and in need of continuous infusion. At least it was keeping it somewhat stable although it didn’t cure it.
When the time came and my paperwork had been processed he went back on his word and told the nurses a different story. People were rushing in and out of the room and just signing a release to get my records sent to my outpatient doctors was difficult. I had to call the social worker 3 times to prevent the nurse assigned that shift, Carolina from dropping the paper on the table and bolting from the room. There was some strange energy going on to say the least! I stared her in the eye and told her I knew and she knew this was wrong and that this should have been done the proper way so that I’d be safe once I got home over the remainder of the weekend. She averted her eyes, knowing in her heart I was right. They intended to send me back to be Emory’s problem. Little did I know that Emory had plans of their own to deflect responsibility. I was only just beginning to understand the political games big healthcare systems play using patients as pawns.
Transportation was on their way to come with stretcher in tow. On the dry erase board on the wall where all the days’ schedule was posted I scrawled a message to Dr. Woessner which read “Thank you, Dr. Woessner, my Angel in the Outfield. :-)” If I could I would have her as my neurologist on the outside, but she only works inpatient. I have never met a neurologist as kind and caring and doubt I ever will again. The attending who discharged me made a comment once that I held her in high regard (as if I shouldn’t), but then why wouldn’t I? She’d always treated me with the utmost respect, compassion, and dignity.
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.