Trach-ties
My Story - Updates
This web site was begun in 1997. A lot happens and changes as time goes on. Conditions improve, some deteriorate, others remain unchanged.
This page will keep you updated on my progress, whether for the better or not.
After my trach was placed I did improve for a little bit. It was such a relief just not to have to fight for every breath. My energy improved, I was getting back,
or so I thought, to how things were before the tracheostomy. I was able to maintain a light exercise regiment, take care of my family again. No more steroids! Yippee! We did notice
that I was still very susceptible to infections and more things set off the reactions. The only way to control my environment to keep the reactions to a dull roar
was to stay at home where we knew that my environment was controllable.
We soon suspected something wrong with my immune system. It became more evident with the reactions increasing in frequency and from more precipitating elements, along with repeated episodes
of tracheobronchitis that took long term (months), high dose antibiotics to clear. In 1997 there were not too many tests common for testing the immune system. More
indirect hunches than concrete facts. The one prevention was avoidance of precipitating factors and to stay in an easily controlled environment.
In July 1998 I noticed that climbing stairs was getting increasingly difficult and fatigued easily. Reaching over my head and holding on to objects over my head were also getting difficult. My kids would laugh
at the pots and pans hanging in my kitchen with one side flattened from being dropped as they had been taken off their hooks. The muscle weakness seemed to progress over months, to the point
that I was unable to walk up stairs, unable to reach over my head or hold my arms out straight for any amount of time. By January 1999 I was a sight to see try to stand from a sitting position. I would have
to "walk" up myself or furniture to stand. The usual testing was done as far as checking blood for the usual culprits, all of which came back 'normal'.
This continued to progress. My proximal muscles (ones closest to my trunk) were weaker than the distal (farther away from the trunk). Between the muscles that were weak not being able to hold me up, and those
that weren't weak went into spasm to compensate for those that were weak. Standing or being on my feet for any length of time was getting more and more difficult. My gait got wider and more unsteady, I started losing my balance. Falling became a daily affair. I would spend most of the day in a recliner for comfort in breathing and to prevent falls, getting up for necessities holding on to walls and furniture by spring of 2000.
In November 1999 I once again had gotten tracheobronchitis. This time I felt like I was pooping out. It was getting harder to breathe, and my color was getting worse. My level of alertness was open to opinion. Sometimes
I thought I was ok, but the family said I wasn't always making sense and confused. I had been treated for the tracheobronchitis for 3 weeks when I called my doctor, basically asking for the ventilatory assistance again to get over the hump. He knew things were pretty bad for me to call and make that request.
Finger pulse oximeters indicated that my oxygen levels were too low. That day Oxygen was delivered to our home. It certainly helped. I was still easily fatigued, and congested, but didn't feel as air starved. My family says I
started to perk up some, not continuously in a daze or sleeping.
I have been on it since. We have tried to wean the oxygen without success. My oxygen saturation drops, I get somnolent and my color deteriorates. Not a pretty sight I assure you. I have an oxygen concentrator in our home,
and tanks called E cylinders that I have for use when I am away from home or in the event of a power failure. The concentrator has been nicknamed "the bomb", given to it by my husband and it stuck. The family joke is if you
want to find me, follow the green tubing from the concentrator and you will find me at the other end (its 50 feet long). This positive, light hearted humor has been helpful in our adjustment to all the changes we have encountered.
In May 2000 I had a Physical Therapy consult. They came to my home, originally to strengthen my legs so that I could walk up the stairs. They were a bit surprised to see the state of all my muscles. It went from leg strengthening to
a regiment to strengthen my lumbar back and legs and arms. Occupational Therapy was brought in also to assist me in different ways to dress and other everyday activities. They said their job was easy since I had already devised some compensatory
maneuvers of my own. They were a bit unorthodox, but they worked. After weeks of trying to strengthen muscles and stabilize my lumbar spine the decision was made that for safe mobility and due to my bad respiratory status, that I was a candidate for
a power wheelchair.
The Physical Therapist was very knowledgeable. She knew which type of chair would work best for me. The decision was made for a Jazzy wheelchair by
Pride Mobility. It took a while for all the proper documentation and paperwork to be done, and time to
order and assemble the chair. In the mean time we managed as we had the months before, between toddling around or a manual wheelchair. The Jazzy chair was delivered mid October. It took a little getting used to 'driving' the chair. I never had been any good with joysticks on video games. I had to learn how to maneuver
in the house, especially doorways. Going through them rather than into them was the hardest trick. The doors and door jams would have some stories to tell if they were capable of talking.
Images Courtesy of PrideMobility
The summer of 2000 we moved once again. This time it was my husband's retirement from the military. Relocating was more interesting this time. With my condition we could not travel far, had to make arrangements to have oxygen at our destination, and find temporary lodging and a home that I could survive in.
Fortunately we were able to find a home quickly and got moved in. I was more of a hinderance than a help physically, so was delegated a supervisory role. The chair was delivered to the new home not long after we moved in. My biggest fear was running into walls.
But as you can tell the walls and I have survived.
October 2000 also was the month of meeting all the new doctors. My doctor who had seen me through the worst of all this was meticulous as to whom he referred me to, that they understood how tenuous the situation was. Moving close to a large university hospital was
probably a blessing. They have the specialists I need all in one place. One, and most important, an excellent Immunologist. Our first appointment was basically one getting records and talking about how things have progressed. He seemed so fascinated. Things that
others had sat back and scratched their heads at, he was taking it all in and assimilating all the information. After blood tests, some of which are very new, we had the documentation of what had been suspected since 1995. My immune system was not working correctly.
I have Variable Immune Deficiency. My immune system had basically shut down for some reason. Probably starting prior to the start of all this in 1995, but that is when it snowballed. My body had compensated by going into what's called 'Anaphylactic Syndrome'
which explains the reactions. Rather than a functioning immune system telling it what to do to everyday elements, it would see every affront as an insult and go into overdrive, where a normal immune system would recognize it as normal and not react. I don't have "allergies" like everyone
else. I make little to no IgE, the immune globulin that causes allergies. I make no antibodies. Hence the negative antibody screens for things that should have been positive. The immune globulins that act as the 'generals' to orchestrate a response to infection were little to none, hence
the intractable infections, no normal white blood cell response to fight the infection. My reactions are from massive histamine release.
Once we had the 'why' it was time to come up with a treatment plan. The one definitive treatment for immune deficiency is intravenous immune globulin. Better known as IVIG. It is the immune globulins pooled from many blood donors, washed, reconstituted and given through an intravenous.
They replace the immune globulins that aren't there or that aren't functioning. But these were no miracle cure. Basically a treatment regiment to replace some gamma globulin. Once a month I started getting the infusions. They are not an over night remedy either. It can take up to
six months before you begin to have signs that they are working. After a few months I began to notice less frequency and severity in the reactions which had gotten to be almost daily. The first couple weeks after the infusions I no longer had the low grade fever that had become commonplace the last couple of
years, and I noticed some improvements in my asthma, which can happen with IVIG. One day of inconvenience and my life would start to turn around. As of July 2002 I now receive my infusions twice a month to maintain a level that I can live at home (still homebound, but status quo). We also found out why all my blood studies came back "normal". I don't make antibodies. Blood tests for most diseases check for the presence of antibodies your body makes. Without the ability to make these antibodies my blood studies will always be negative or normal, despite having a diease process or not. We have learned through this journey that "Negative/Normal" isn't all its cracked up to be.
Being a Medicare recipient meant that I had to go to the infusion center for it to be a covered service. Despite the underlying condition, they would not consider it as a home infusion, which private insurances allow. In doing so I am exposed to whatever pathogens or precipitating elements in that environment.
After the first infusion, I had been my usual status. Over one night I went from usual to extremely sick, with difficulty breathing, high fever and redness over the implanted IV access port that had been implanted a few weeks before. The ambulance transported me to the local hospital who in turn sent me on to
the University Hospital where my Immunologist practices. A chest x-ray showed a pneumonia, possibly caught from the day at the infusion center. I had life threatening sepsis. The infection had spread into my blood stream. With a fever of 105 degrees they decided it best to remove the port and started appropriate
antibiotics by PICC line (a long intravenous into my arm)(Peripherally Inserted Central Catheter). I was fortunate and after the worst was over was able to return home with a long regiment of the Intravenous antibiotics to look forward to. Extra IVIG was given during my hospital stay to help me fight the infection.
I continue to fight the battle, despite it being measured in years now rather than months, to get my infusions done at home, so that I will not be exposed to the pathogens/precipitating factors present in a hospital environment or any other public environment. My letters to Medicare were answered with "good luck, for this to be a covered service you will have to contact your Congressmen." I have contacted my US Senators and Congressional Representative. The Senators have virtually patted me on the head and sent me away. One replied that Medicare states I need to go through the appeal process. I have gotten no reply from my Representative. There is no appealing a service they won't be able to deny because no one will offer the service pro bono until their decision can be made. For an appeal there has to be a claim for service and denial. With no service there can be no denial of
payment. So I continue to keep in contact with the Senators and Medicare trying to get this as a home infusion. With my homebound status one would imagine that it would be covered. Unfortunately no. In the mean time Medicare pays the extra expense of transportation to the hospital, a days admission to the hospital, and the infusion. If anything, being a home infusion
will be saving Medicare, and taxpayer, dollars. Medicare (actually our elected officials) would rather incur the costs of another extended hospital stay in Intensive Care with Sepsis again and more ER visits for reactions that I can't control with the medicines I have for them, rather than take the more cost effective route of home infusion in persons with profound VID.
You might be asking...this is a work related illness/injury, why not be covered under workmen's Compensation. My next battle once I survived the physical aspects was Workman’s Comp and the Commonwealth of Virginia. They denied all claims, despite it being work related, claiming that the Commonwealth does not cover events that are “diseases of ordinary life”. So they jumped through that large loophole and denied all workmen’s comp or other responsibility on the part of my former employer. I went to fight it, only to lose in the long run. According to the Hospital’s attorneys, I could not be represented by counsel alone I had to present myself in person in the courtroom, which would have caused an acute episode and I would arrest again. Having a spouse in the military who was deployed and having two small kids, I considered that too much of a risk.
After months of tests (EMG, Nerve Conduction studies, more blood tests and simple neurological exams)the concensus is my muscle weakness is Myopathy of Unknown Origin or a variant of Myasthenia. You can see more info on this on the Neuromuscular Page. I have been started on a drug that is used more for narcolepsy and MS, but has shown promise in treating the muscle weakness and fatigueability of MG, called Provigil (modafinil). I have noticed some improvement with it for the length of time it lasts per day. It doesn't give me back all my strength but I am able to do more with it. It doesn't get me out of the chair totally by any means, but I am able to get dressed and toddle in the house a few steps better early in the day, falling less. I take it in the morning before I attempt to get up. I then have some muscle use to dress and even knit a few rows. The one down fall is when it wears off, it sure wears off. My muscles are weaker and sore (probably from me taking advantage of them working) and I get very sleepy very rapidly. My family calls it my "crash" time. They can tell by looking at me when that threshold has been met.
In June 2002, one of my goals has been achieved! My first goal was to stay alive and see my son graduate from high school. Thanks to an extra IVIG infusion, hepamasks (one over my face and the other over my trach), and supportive family and doctors I was able to attend his outdoor graduation (with all my paraphenalia)!! (when I get a picture I'll add it) Now I have until June 2004 to get ready for the next one.
As time's gone on my muscles have continued to deteriorate and my respiratory status is doing the same. The Neurologist has discontinued the Provigil for now. I am now in the chair more, no more trying to toddle around holding on to things. I am also back on the ventilatory assist at night. It seems I don't breathe too well when I am asleep. With my larynx close as it is I don't have to change into a cuffed trach like most ventilated trached patients.
We have started a big landscaping project in our yard. Prior to this what was considered our yard was hills going down into a "V" that rain run off loved to make a river along, running right next to our foundation. So for safety measures and to make the yard more "accessible" we have started a leveling project, making a retaining wall along the back with wheelchair friendly slopes doing back to it. The project started in the summer of 2002. We hope to finish it with a deck construction once we make sure the footings won't wash away with the next rain.
continued on My Update 2
Trachties
