This odyssey all started in January 2015 when I unexpectedly found myself on a journey through hell on earth with a mysterious, severe and progressive disease, for the second time. During a very long and hard diagnostic process I have had to learn things I’d never thought I’d have to learn, seen things that have made me doubt humanity, and have endured atrocities no living thing; human or animal, should be expected to endure, and have done it mostly alone.

Some background; 12 years ago, in the summer of 2003 I became acutely ill and almost died of another mysterious disease which included 30 or more symptoms and about every 5 days ravaged a new system in my body. Later after many visits to the ER, and after seeing numerous doctors I was diagnosed with Sarcoidosis, a multi-system disease which has a lot on common with Lupus. The first symptom was an excessively dry mouth and then the rapid loss of bone in my upper jaw, creating blackened gums in both jaws and a big and widening gap between my teeth within just a period of 5 days.  I went to a doctor who after looking me over carefully determined that I had Staph or Strep, gave me Zithromycin and sent me on my way. It seemed to help at first but I noticed each night my symptoms still worsened and after the full round was finished I relapsed. By that time I’m sure it had infected the CNS because I had grown confused and was having a lot of trouble doing the simplest things, and I couldn’t comprehend the meaning of what others were saying to me on the phone. Mobility, writing, and even speech were compromised. It was posited by a home healthcare nurse who saw me about 5 weeks after onset that I may have had some strokes because I had facial palsy and significant change in mental status.

I’d been losing 6 Lbs. a week, had projectile acid reflux so severe that it spewed out onto my bedsheets each time I fell asleep, and this progressed to severe shortness of breath, cardiac arrhythmias, near syncope, cold body temperature alternating with low-grade fevers, facial palsy, pain in all my muscles, and much more. The drugs that were FDA approved for Sarcoidosis I found from my reading, were adding insult to injury and the risk or organ damage, fatal infection and avascular necrosis were things I wanted to avoid if I could. My current doctors didn’t know what else to treat it with so I went online to see if I could find a better option and finally found an off label protocol which used pulsed antibiotics and Benicar (an Angiotensin Receptor Blocker used to treat high blood pressure). It was new, safer, and I found, more effective than either steroids, biologics, or anti-cancer drugs such as Methotrexate often given to patients with that disease. I asked my new GP, Dr. B. at Emory to prescribe it and at first he was hesitant, but one day I came into his office so short of breath I was desperate, and he agreed to write the description. My improvement was dramatic, with significant reduction of pain and respiratory distress within just 2 weeks, so I knew I had made the right treatment decision.

After about 2 years I felt maybe I was ready to go back to school seeking a degree in psychology. It was not long afterwards that the Medicare Part D legislation created under former President George W. Bush forced all those dually eligible on Medicare/Medicaid currently receiving their prescriptions through Medicaid to be switched to the private contractor for all prescription coverage. Little did I know that now no off-label protocols would be covered by my insurer.  I tried to appeal with my doctor’s help but to no avail, and buying Benicar in cash was not an option, as 1 month’s supply would cost me more than my monthly income.

No sufficient prescription aid programs existed for this drug at the higher dosage indicated by the protocol, so sadly, I had to go off of it. For 10 years I maintained by reducing my exertion and eating a macrobiotic diet consisting mostly of vegetables and avoiding Vitamin D (because it’s the Vitamin D Receptor that is dysregulated and therefore the target in Sarcoidosis and most of the other chronic serious diseases). I thought I was succeeding at keeping the disease under control but I had no idea what was about to happen.

I walked into my GPs office initially because I felt more fatigued than was my usual baseline and thought that maybe if I asked for a physical therapy referral this might build up my stamina and that I could take on more during the day. Unfortunately not only did this not help, but actually seemed to exacerbate my fatigue and sore muscles that seemed to get tired easily. I had trigger points in my back and neck that were inflamed. Only massage seemed to help but Medicare won’t cover much of that even though it will cover some if given by a physical therapist.

Since I had clusters of episodes every few months of falling asleep during the day Dr. B. suggested I have a sleep study, which at the time I couldn’t imagine had any connection since it wasn’t constant but I agreed to give it a try to rule out those types of disorders, if nothing else.

I called Emory’s Sleep Medicine Department which only had two specialists in it and was scheduled with Dr. H. When I described how I’d been feeling he said he had a hunch that my case might be different from the majority of patients he sees most of whom have obstructive sleep apnea. We might be dealing with a more rare variety, he said. The sleep study was scheduled and that night I noticed lots of jerking when falling asleep but was unaware of anything else. I just chalked it up to a rough night in an unfamiliar place with all the wires probably interrupting my sleep. I totally expected the test to be negative and that soon my worst days would be behind me and that better health and a fuller, more functional life was on its way.

My appointment with Dr. H. was still a month or so away, but I received a call asking me to come into the sleep lab for another sleep study for a CPAP Titration. I nearly thought they had the wrong patient because I didn’t snore and had no other indications I had sleep apnea. They assured me that I in fact did stop breathing during the night and said this could be a problem if not taken care of. It was only a week later, so I figured whatever type of sleep disorder I had that it must be important to do something about. The person who scheduled it wasn’t entirely sure of the details of my condition, but said she’d send me a copy of the report.

I went in and had the same technician assigned to me as the first time and although I didn’t really like the idea I figured “how hard could this be wearing a mask while sleeping”. I had no idea! After trying several masks I settled for one that at least didn’t blow out my eardrums. The nasal masks were intolerable right away. This one was like a bridal with all sorts of straps but it covered my face and didn’t put such pressure directly up my nose. Even though it was better than the nasal masks the straps in the back hurt my neck and caused my muscles to be miserable.

I tossed and turned trying to get comfortable but no position really worked. Then as I fell asleep there was a huge strong vacuum which seemed to reverse and actually sucked the air out of my longs. It reminded me of those commercials about Space Bags in which the vacuum cleaner sucked all the air out of a plastic bag and the bag crumpled in on itself. In addition I had bad smelling air inside my lungs that came back up.

The tech came in around 5 AM looking concerned and asked if I wanted to end it a little early and I said that I did and told her about how it seemed to do on me the opposite of what it was supposed to do. She looked almost as though she might cry and said she was so sorry, and took off the equipment as fast as she could, stating that she really hoped there would be something the doctor could do to help me.  I knew that there was more to this than met the eye but hadn’t received the report yet so I still didn’t know the half of it.

The next several weeks were a hellish roller-coaster ride each night with cliff-hanging stoppages in my breathing that now I was aware of and at times my heart actually paused beating for a few seconds. Then came the shortness of breath during the day too.

I saw Dr. H. in the office and he told me “ This isn’t the type of sleep study we like to see” and said “I haven’t been able to find another case like this in the literature since the early 1900s when some patient had been documented as having Tuberculosis of the brain.” He said I desaturated even while awake watching TV during the sleep study and that he was concerned about my hypoxic burden”.

A pattern similar to Cheynes-Stokes, Biot’s breathing (or Biot’s Respiration as it’s sometimes referred to) is so rarely seen that he gave some sort of presentation on it to other doctors. He kept saying “This is not good. This is not good.” He drew a picture for me of the brainstem and said the problem causing this was most likely either in the Pons or Medulla. He showed me a copy of the report and the chart from the first sleep study with flat lines interspersed with about 3 smaller breaths in-between.

The CPAP titration looked pretty much like a flat line all night. He explained that not only did the ventilation not help, but actually made it worse. At the time it almost didn’t feel real to me. I sort of made light of it and reassured him that somewhere there must be a silver lining in all of this. If there was one he couldn’t see it. He told me this would be challenging to treat.

I told him “Well, I’m probably the most damned determined woman you’re ever going to meet.” Although what I said was true, little did I know that this was just the first hurdle I would encounter. There would be more obstacles to overcome, some of them steel walls 10 feet thick.

He ordered a brainstem MRI which didn’t show anything, and not knowing much about the neurological ins and outs of this he wasn’t sure what to do at that point. Neither was I. This was new territory.

He and his nurse started working on having Medicare certify me for oxygen since I was unable to tolerate any pressure. It took quite awhile to get through the process of getting the oxygen approved and I was agonizingly short of breath with little and sometimes no exertion, especially when going out. First I had to have a “6 minute walk test” to see if I could qualify for portable oxygen which would allow me to get out more.

That didn’t work because the tech kept telling me to breath (which to me felt like hyperventilation since I don’t breathe regularly the way others do, and that raised my oxygen saturation above 88 % within that 6 minute walk back and forth down the hall. Next we had to get a nighttime oximetry test done at home for a nighttime Oxygen. That took several months alone, but it turned out I qualified. It went down as low as 80% for 5 minutes or more during the night. I qualified and was approved for an oxygen concentrator at home.

The first time I put on the oxygen I felt so much more alert, and much to my surprise my nail beds which I’d assumed were normal being a lavender color turned distinctly pink. I had no idea how long my breathing pattern had gone on before I’d stumbled upon it, but I felt more clearheaded than I had in years.

Though the oxygen provided relief for my breathing at night and eventually stabilized my shortness of breath during the day, the cause; the underlying condition remained elusive. I hoped nothing further would develop and remained hopeful that it was as bad as things were going to get, and I went about my life. For the time being Dr. H. and I could watch and wait, cautiously optimistic.  In the meantime this remained our little project, more of a puzzle to figure out in the abstract, and although the Biot’s breathing indicated that a neurologist be called in, the situation at the time appeared pretty stable, so neither of us was particularly alarmed.

Little did either of us know, trouble was brewing right around the corner.