New Developments in My Disease Process; The Next Leg of the Race

The oxygen I've been on at night has reduced the overt apneas quite a bit and seems to have improved the daytime dyspnea, but after my last post I was still having quite a time with those narcolepsy type symptoms and the jerking when trying to fall asleep. The pulmonologist set up another sleep study, this time both night and daytime which he said if it came out negative (and he didn't think it would), would prove that the jerking was not breathing-related and would indicate something else. He thought that if it came out positive then that would cancel everything out; meaning that either it would get a false positive or a positive and it would be impossible to know which it was. The one caviot is that I would have to sleep at least 6 hours during the night for the daytime study to tell us anything because if I didn't then I could possibly fall asleep more than normal simply from sleep deprivation. If narcolepsy were present in addition to the sleep deprivation we'd never know. I knew going into it that this would be a feat akin to driving your car between two toothpicks laid almost side-by-side, but if that was the only way to possibly get some information which could lead to more complete treatment then I'd give it a try.

As time went on the jerking at night continued to get worse and the sudden sleep attacks subsided (they come and go in clusters usually every few months). I think maybe I had one sometime during the week before I was to have the sleep study.

Then sometime in the same week (one evening between July 19th and 25th) I was going down my driveway to get the mail and fell really hard on the concrete. My feet twisted and bent back under me and my knees skidded about a foot downhill. It hurt terribly but after I caught my breath I could tell that although I was skinned pretty badly nothing was sprained or broken. I just assumed I must have slipped on something or put my foot down in a strange way and that that had caused it, so I got up and went into the house and washed off the scrapes, thinking nothing of it. It took off quite a bit of skin on my ankle in the front and strangely turned blackish purple after the scab healed and came off. This also happened to the scrapes on the knee even long after applying vitamin E and even CBD salve and time for healing.

Then a few days later came the sleep study on July 29th. I didn't have my usual tech because she didn't work on Wednesdays and only worked at night so I wouldn't have her for the daytime study either. I did make her a pair of earrings though to drop off for her when I was there; little dream cloud stud earrings with rainbow moonstone cabs set in them and a brushed finish. The stones had more flash than you can see in this picture. The sterling silver sheet I cut them out of is quite thick, so getting this much detail into the crevices between bumps wasn't easy!

Just as I'd feared the technician I was assigned that night was not my cup of tea; a 60 something Jamaiican woman with a starchy demeanor, pretty much all business. I had the TV on to distract myself, being slightly nervous, and the first thing she did was ask me to turn it down when she returned to put on my electrodes. (I'd had to turn it up because it seemed like the air conditioning was making it hard to hear). Then, she asked me to move the ice I brought out of the way, and when I told her I get dehydrated easily due to my disease and need to have it next to the bed in case I wake up and need it in the middle of the night or I can't get back to sleep, she started to challenge me. Apparently the pre-registration person I'd informed of my special needs had not passed this information onto her. (In contrast, Gana, the one I usually was assigned to had been nice enough on my previous sleep study to come into the room and ask if I needed some ice water without my having to ask). That kindness had really made an impression on my heart.

This other tech seemed unaware of the fact that my doctor had ordered several other leads to measure the jerking on arms and legs, looked at me as if she thought I was crazy when I asked her about which they were, but then found out as she was applying them that they had in fact been specified in the order, she just didn't recognize them as such. After that she started telling me each thing the others measured. While that was interesting from a scientific standpoint, I didn't have the sense she was giving this explanation for my benefit, but to cover her own ass in case she flubbed it up. Her social stiffness only intensified my uneasiness. Both the skill and the bedside manner of the technician you're assigned can make all the difference in what kind of experience you have during one of these things.

This one must have asked me two or three times if I was on nighttime oxygen as if she couldn't believe it or thought it was strange. She mentioned they didn't get many people in there who are on oxygen. I found that hard to believe! Although my breathing pattern is rare I'm sure that others with underlying conditions also probably are on oxygen. Maybe just not on her shifts. The whole time I just wanted to press a buzzer and have her removed like on the "Gong show", LOL. This was not going well.

She noticed the healing scabs on my leg and asked if I had fallen and I told her I had.

I was deathly cold and even after turning the thermostat on the wall to heat I still couldn't get warmth back into my body once I'd lost all heat. It's some sort of autonomic problem I have had for many years, and unlike most people I have to thermoregulate from external sources much like a reptile.

Between the usual wires and the oxygen canula I had a facefull of stuff on me. The oxygen they used was an old-fashioned bottle on wheels. I hoped that she knew how to use the thing because I sure didn't. She connected all my wires to a boxy-looking machine with read-outs on a screen on the bedside table that I hadn't seen in my previous sleep studies (which she didn't give much explanation about, just said it was the type of machine my doctor had specified to be used for the test). It had what looked like a floodlight attached to it on the side and the face of the square box screen had some lines (which I think corresponded to the different leads). This time in addition to the EMG leads to measure the jerking they also had one that went on near my clavicle on the left side measuring Co2.

After that I was glad to be left alone. I was getting pretty sleepy when she had been hooking me up, but once I lay down to try to sleep then the jerking started and just made me more alert with each time it happened. If any of you have had this for any length of time you will know how exhausting it is, both systemically and to the muscles themselves. I've been experiencing it at least since last January if not before and it's gradually been getting worse and more frequent ever since. Knowing that I needed 6 hours of sleep in order to make a daytime study valid this constant interruption was really frustrating!

The tech came in a few times to fiddle with equipment because her monitors weren't picking up what was on the machine in my room, and that interruption didn't help me be able to sleep either. Then after several hours of violent jerking that left me even more exhausted and exasperated I finally fell asleep. I was awoken by the door opening and a guy walking in to fiddle with the box connected to the leads. I told him that I had finally fallen asleep after jerking and jerking which I thought would never stop, and asked him how long I had slept. He told me about 3 hours and that I was doing well. He and a young woman had just come on for the change of shifts, and although they had woken me up I was relieved that I had some nicer techs now for the next part of the study. The trouble was, then I couldn't get the 3 more hours needed, and next thing I knew the young woman was back in there telling me that the doctor had called off the daytime study since it didn't look like I would get 3 hours more in in enough time. I told her I could keep trying if that would do any good, and that my transportation wasn't scheduled to pick me up until 6 PM that night and I wasn't sure if they'd come earlier or not, but she said that the doctor had already canceled it. I told her I didn't know how many of these Medicare would cover, so I was concerned we'd never know about the daytime sleep attacks; whether they're actually narcolepsy or not. Luckily my transportation service agreed to pick me up early. The broker had called a taxi, so that was more flexible than if they'd scheduled a van service. I showered to get all the glue off, got dressed, gathered my belongings, and went out into the hall where I handed the package containing the earrings to the young woman and ask her to give it to Gana when she came in and she said that she would give it to her. I went down to the lobby to wait for my ride knowing I'd probably fall asleep for the other 3 hours shortly after returning home. I was feeling pretty disgusted as the taxi pulled off with me headed back to my house.

The jerking continued night after night and it got to the point that it was worse to try to go to bed at a reasonable hour because all I did was lie there and jerk until around 4-7 AM, and as time went on it got more and more like 7 than 4. Three hours a night turned into two hours a night and I was getting really worn down after several more weeks of this.

Then within a period of 3 or 4 days during the week of August 10th - 15th I fell 3 times in one week. It was strange because I was tripping on nothing in some instances and in one of them my toe barely nicked the corner of a handbag in my bedroom and it seemed that instead of my foot kicking it out of the way as would have normally happened and my other leg widening my stance to brace myself it was as if my legs did nothing to protect me at all and I went down full force on my hands and knees. This caused another skinned area on my knee because of rug burn on my carpet. It has healed considerably now and doesn't seem to be leaving the big blackish purple coloring behind that my first fall a few weeks ago did, but I knew something wasn't right because I've never been accident prone and 3 falls in one week is unlikely to be a coincidence!

One day after the last fall I began feeling a perceptible weakness in all my muscles (all except in my face). There started to develop not only a weakness but a feeling as if my muscles were over-worked. I was "feeling the burn" so to speak all the time as if I were constantly exercising. I had no idea what on earth was going on but I didn't have a good feeling about it. Then stiffness started to set in. It did not seem related to the last fall because that one only injured my right shin, not the left, and the left leg was where I noticed the stiffness and more of the weakness.

On my return visit to the pulmonologist this past Monday, August 17th I told him about the falling. He seemed totally surprised although he admitted that my sleep study was quite abnormal; that although the oxygen was improving the overall AHI (Apnea/Hypopnea Index), that the Biot's Breathing is still there, and that he saw some other abnormalities he had not seen in the other two studies (evidence that something's progressing with the underlying condition). He told me that the EMG leads picked up the jerking clearly, that it was not happening at exact intervals but with random time in-between jerks, and happening pretty frequently. He also told me that I was technically awake (probably a split second before dropping off to sleep), and that there were transient Delta waves picked up in my EEG in that waking state. (Delta waves are only supposed to happen in the deepest stage of sleep) so this is very strange. The last irregularity he mentioned is that the percentage of the last stage of deep sleep once I got into it was larger than the other stages. He didn't know what these abnormalities meant but definitely thought it was odd.

Then he tested my muscle strength in my arms and legs, asking me to press back against his hand while he was pressing one way. I had to try with all my might to push up with my left leg and after awhile it trembled in straining to do so, but then something really bizarre happened. It suddenly started jerking wildly. It kind of scared me. I told him that nothing like that had ever happened before. He didn't make much of a comment but let go of my leg and said something about the weakness in the left leg which I couldn't quite catch, and then said it was a good thing I was going to see a neurologist, and that she might even want me to see another specialist.

He asked if I'd been having the daytime sleep attacks lately and I told him that no not for a few weeks, and he told me to come back in December, further out than he normally had scheduled me before, since that was the first symptom I had been referred for. He made a comment that for the life of me I can't figure out the meaning of; "This is good for me, but bad for you." Not sure if it was the longer wait until the next appointment with him he was referring to or something else. As we walked out into the hall I told him that I'd pretty much given up on neurologists because as a general rule they gave up on me, and I had tried for years to live with my neuro problems and compensate as best as I could, but now that things are getting worse I cannot afford to let it go any longer, to which he agreed, but then said he wasn't sure what he's done would be of much help to the neurologist and made another cryptic statement; "You don't want a diagnosis, you want answers".  Since we were already headed our separate ways in the hallway I didn't have time to ask him what he meant. I couldn't tell if he was asking me (supporting me in) what I wanted, or telling me what I should want. Realistically I know that in this system of medical care you pretty much have to have a diagnosis for insurance to pay for treatment (and he's well aware of that too), but bottom line, I want it treated and I want to not have to suffer anymore. As I was leaving I said, "Well...It's there whether it has a name or not." He agreed. If I could wave a magic wand I would like to have never had it in the first place and started out whole, but I never had that choice. Not now, and not when I was a child going through other neuro stuff that try as I might, kept interfering with my constant attempts to live a normal life. Quite frankly I'm tired of having to swim upstream, but that's what I'm handed so all I know to do is do my best to enjoy my life and hope that medical science has the technology to fix it after a number of years have now passed, or in the near future.

I left that day feeling increasingly stiff with tired muscles and managed to make it down to where I had to be picked up, but barely. The driver was a jerk and took almost 2 hours to pick me up, then went into a long list of self-justifications as to why and no real apology. All I wanted was to go home as soon as possible and go to bed. My body was aching and I was so tired I couldn't stand one more minute of consciousness.

Thursday was supposed to be the day I saw the new neurologist but another van service failed to pick me up even though I'd arranged transportation to get me there a half-hour earlier than my appointment time. Kimberly, the woman who does quality assurance for the transportation broker system in Dekalb County was flabbergasted that they kept saying they were 20 minutes away, yet never got here at all. Each day it has become more difficult for me to walk and I feel this neurologist might not be like the others I've seen who've done a few tests and looked baffled at the results, shrugged their shoulders and sent me on blankly. I figure if anyone in Atlanta can figure this out and connect all the dots it would be her, but as it got later and later and no driver showed up the clinic told me that I'd have to reschedule. First they told me the first available appointment was "next year", and I told them that was ludicrous for a patient with these symptoms. The receptionist told me she'd contact the doctor and see if she could somehow fit me in sooner and the next time she came up with was October 8th (I believe). I told them this is still not soon enough, and this could be something in which early enough detection is key and I don't want to be ruined for life because I wasn't seen in a timely manner. I asked if this was really OK with her (the neurologist) that it wait that long, given the symptoms I've been having recently. She then told me she'd contact her again, say that date's not acceptable, and mark the message "high priority", and that most likely I wouldn't get an answer until Monday when a certain woman would be following up on it. I hope the neurologist is not put off by my assertiveness but given the fact that I have no caregiver or help at home to help advocate on my behalf I have to make absolutely sure that things go as they should. I just worry about what will happen if I get to the point that I can't do that for myself anymore.

I had hoped that my testing would be out of the way by the end of September and that I'd be on some sort of treatment by then because my son has extended EEG monitoring coming up at Emory then and I want to be there for him during that time (at least during the day). They require that somebody stay with him to keep him awake when he has sleep deprivation. This is in preparation to his having laser surgery on his brain for a benign tumor which causes him to have seizures. He has the chance for a normal life if he can get through that and possibly some other tests. They need to pin down exactly where the seizures are coming from and he has to have a certain number of seizures on videotape in order to meet the qualifications for the surgery. This surgery is still very new technology, so the screening process is rigorous, and a board meets at the hospital to make the final decision as to which people get it and which don't.

I'm trying to rest as much as I can and not walk around too much, trying to save my strength until something can be done. Out of necessity I've had to cut back on the number of hours I'm spending on my Etsy shops because I'm absolutely exhausted. I find that I jerk less when I try to sleep mostly in the daytime rather than at night, so I'm listening to my body until there is something that will get it to stop. I don't have much of an appetite but I notice I am a little more hungry after I sleep for a few hours during the day.

Chronic Disease Strikes Again

I guess some of my readers have wondered where I am lately. It's been awhile since I've really written in my blog, and alot has been happening.

For those who don't know the background on this; 12 years ago just about every system in my body went haywire and I almost died and was finally diagnosed with Sarcoidosis. I got on a cutting edge off-label protocol called The Marshall Protocol just in time, and was on it and feeling improvement for 2 years when Medicare Part D came along and once my drug coverage was transferred from Medicaid they would not cover the medications. The cost out of pocket was too much on my very small income, and I was unable to qualify for the few medication assistance programs that existed at the time, so I had to go off it.

I tried controlling it by taking it easy, eating a mostly macrobiotic diet and limiting light exposure and for awhile that kept things to a low roar, but gradually my pain level increased and more symptoms started returning. I tried to push it out of my mind. I really didn't want to believe I was having disease progression because I felt I hadn't had a chance yet to really succeed in life and was tired of all this. I just wanted to put it behind me and get on with and enjoy life.

Meanwhile sales had not been good in my Etsy shops and I thought if I could just build up my stamina maybe I could try to do some things to increase my income and hopefully give my business the shot in the arm it needed to really make it profitable.

My plan was to go to physical therapy (hopefully pool exercise and massage), reduce my pain level and then possibly take some part time work to pay off debt and reinvest more capitol in my business so that I could buy some tools like a rolling mill, kiln, and electroforming equipment, and maybe take some more specialty classes to add some new techniques to my arsenal. I've wanted to be able to make my budding vine bracelets in other metals for awhile so that a larger number of people can afford them, as I think that really is one of my signature pieces.

During physical therapy I had a near fainting spell, and began noticing for several weeks that my breathing was difficult and had some motor slowing that would last several hours at a time on and off.

My primary care doctor had suggested I have a Sleep Study too because I was also having sudden episodes of really strong sleepiness. I figured if it was narcolepsy then I could just take a pill for that and move on to my goals. I actually thought that test would be negative, so went into it just wanting to get it over with.

Well I went in and they hooked me up to an EEG and all these other sensors to monitor my breathing and heart rate, etc. I was attended to by a nurse named Gana who was very nice and that night there were just a few other patients, so it was pretty quiet. The TV just so happened to be tuned to a show called Prospectors and they were pulling big honking aquamarine crystals out of the ground. We talked a little bit about jewelry and gemstones before they started monitoring.

An infrared camera filmed from across the room my every movement as I slept (or tried to anyway).

I have sinus problems, so having something sticking up my nostril to sense my breathing was kind of difficult. I don't think I slept very well and kept waking up needing to drink ice water. Even then I thought the test would come out negative and that it was just my sinuses that were irritated and maybe all that stuff hooked up to me that was interrupting my sleep.

Little did I know it went alot deeper than that. About a week or two later I got a phone call from the sleep lab saying they wanted to do another sleep study. I asked why and the woman on the phone told me that they wanted to do a CPAP titration. Mind you I had not even seen the doctor for a full report yet and I was shocked. I told them this and said "Are you sure? I was having lots of sinus inflammation. Couldn't that be it?" A man got on the phone and said I had stopped breathing. Then I spoke with a nurse who confirmed that was correct.

I made the appointment thinking Oh God what a can of worms have I opened up now? I didn't know the half of it; not  yet. I asked the nurse from the doctor's office to send me the full report.

When I went back to the sleep lab they hooked everything up to me except the nose sensors and then the same nurse I'd had the previous time came in to fit a mask on me. The first one was a nasal mask and when it was hooked up to the machine it shot air with such force up my nose I thought my eardrums would burst. It seemed to be hitting an obstruction somewhere in my sinuses. Then I tried another one that was similar and that didn't work either. Then I tried a full face mask. That wasn't as bad since the air was more indirect, but the straps were pushing my head down into my neck and increasing my pain after awhile, so I had to try one with just one strap around the back of my head. That shifted a bit but it was the lesser of several evils. I thought at first that maybe I could tolerate it, but then the minute I started falling asleep it would suck air out of my lungs rather than blowing wringing them out like one of those space bags you see advertised on TV. The air from inside my lungs smelled like some sort of awful athlete's foot fungus.

After a number of hours of this, and twisting and turning to get comfortable I had all I could take of that. The nurse, Gana came in around 5 AM and I told her I was having a problem. The look on her face said it all, although she wasn't allowed to tell me the details. She took off the horrible thing and all the wires and said she was so sorry, that it was hard to watch, and hoped the doctor would find something that would help me. I asked if this happened very often and she said it did to some but was not common.

The next night at home was rough and left me gasping for air numerous times. It was worse than it was in the sleep lab.

A few days later the report came in the mail and it was much worse than I thought. Apparently I had a raging case of central apnea (which differs from obstructive in that it's usually neurologically based if not caused by heart failure). The test did not detect any heart problems, so that left the brain stem etiology. My brain fails to send the signals to my lungs to breathe. I stopped breathing 30 times in each hour of sleep, and I was also breathing too shallowly at other times, resulting in oxygen desaturation. hey documented it while I was awake watching TV as well!. Various symptoms I'd had for years started to all make sense.

I thought back to some neuropsych testing I had at age 6 that suggested that I had some central nervous system dysfunction or damage that was something to do with the regulation of alertness, and thought back to various EEGs I'd had where they found slow waves where they shouldn't be. The headaches, the intense period of complex partial seizures I had for a few years around 1986, and one neurologist had told me something "wasn't hooked up quite right" in my brain. Nobody ever had an answer for these things. All they seemed to be able to do was document it while I struggled alone as best as I could to live a normal life.

An MRI was scheduled which yielded no further clues as to exactly where this is coming from.

Now I find myself at age 54 with a failing respiratory system and I still don't fully know why. Lots of circumstantial evidence is scattered around like shards of glass on the floor but no smoking gun. This elusive thing whatever it is comes and goes like a thief in the night, robbing me of my most basic functions switching them off and on at will. I have possibly lived with it all my life and in order to adapt to this state of being one can never trust tomorrow. It holds all the cards in its hand and I'm just its marionette. I've tried fighting it, ignoring it, reasoning with it, but in the end it has a mind of its own.

I have been unable to eat solid food now for over a week except for a few crackers or a little bread. Sometimes I have little appetite and other times I really miss good food, but when I try to eat a normal meal about 6-8 hours later I get terrible stomach cramps and then diarrhea.

I've had this intermittently for awhile but never was able to find out what it was. Since the CNS controls all automatic functions chances are this is all part of the same thing. Sometimes when I have a central apnea event I wake up feeling like I'm going to crap my pants. This was the first clue that it must be connected. At times it also increases my pain in all the places I usually have it about 100 fold, like a lighting bolt going down all the nerves in my body. luckily those really painful ones have subsided over the past few days.

Breathing during the day is difficult at times. Certain things seem to trigger it such as sitting upright too long or standing too long on my feet, a stuffy room or car, or too much exertion, even intense concentration for too long. It seems as though this is happening around the clock.

When I next saw the pulmonologist he told me that it was the worst results he'd ever seen and that when he checked the literature there hadn't been another case like mine since the early 1900s in which someone had Tuberculosis of the brain. Because of the rarity of it he gave some sort of presentation on my case to other doctors. He went on to say that this is very hard to treat, and acknowledged that CPAP wasn't a viable option since it made me worse. In a nutshell it was clear that my body needed oxygen, so that seemed the obvious solution, even if not a full cure so he put in an order for that but his nurse needs to get Medicare to approve it and that's not easy with their guidelines. They have to find a medical equipment company that accepts my insurance and then Medicare requires them to have me monitor my o2 saturation at home at night. The reason for this is because Medicare has some sort of bias against accepting data from a sleep lab. The standard is saturation must drop to 88% or lower for 5 minutes during sleep . In the sleep lab mine dropped to 81%.

All I can say is this thing better not betray me when we need for it to show itself most! Otherwise I could be sacrificing brain cells and my condition could end up even worse if I don't get oxygen. Having it at nighttime only is still leaving me vulnerable during the day, but it's a start, and once that's established then maybe they'll approve it during the day too. As long as this has probably gone untreated I can't afford to fall through the cracks of the medical system now.

                                                 STAY TUNED FOR PART 2