Welcome! Part I

Welcome to my blog! I am Jen, 36 years old...grateful for those years. My childhood years were uncomplicated, with occasional health problems such as colds. In hindsight, I did not pay much attention to other symptoms or issues that are so obvious now. Aches and pains in my joints with repeated dislocations of my my knees, starting at age 8. Symptoms of what I now know as Raynaud's disease as early as 10 or so. Even the nagging tightness of my chest with any form of running or heavy exertion. I brushed it off...I brushed all of them off. Little did I know just how significant all of this would be until later in my life.

I managed to get through high school, having two beautiful children all the while working and attending collage to become a nurse. I guess my health took a nose dive shortly after my children were born at the age of 23 or so. Prior to their births, I rarely saw a doctor. As a child I rarely saw a doctor unless it was urgent...my parents did not have health insurance and did not make much money. But, as my problems became unbearable, I finally started to seek regular care. My joints hurt badly, I had difficulty even moving around. It also seemed that I was catching just about every respiratory bug that came into contact with me. I had horrible palpitations, my heart felt like it was going to beat right out of my chest and run away!

My first visit was overwhelming...vials of blood taken for a multitude of tests, an EKG, xrays of various skeletal parts, referrals to other specialists. In a short time span of probably 6 months, I saw about 3 different doctors. An internist. A cardiologist and finally a rheumatologist. Oddly enough, my bloods came back negative for just about everything, no RA, no lupus, no collagen problems. The rheumy chalked my aches and pains up to fibromyalgia and put me on meds for that. The cardiologist believed that my palpitations were due to being overly anxious and stressed, being a relatively new mom as well as working and going to school. Okay..I bought some of that rationale but not all of it. I knew that anxiety was not the complete answer to feeling the way I did and I was not about to let a doctor tell me otherwise. Ultimately, I was right...I was finally diagnosed with SVT, supraventricular tachycardia, after having a run of palpitations while doing my clinical in the ER. How convenient that was! The ER doctor hooked me up to the monitors and voila! The rest is history. The internist diagnosed asthma as far as my lungs were concerned. Wheezing, coughing...okay...so I bought that diagnosis too..at the time.

As time marched on, my health became more complex. I began getting mind splitting migraines that literally incapacitated me for months at times. A handful of doctors (a rheumy and 2 neurologists) said that the migraines were "psychogenic" or in my head. This was determined after a series of tests: MRI's, spinal taps to rule out neuro Lyme disease and MS and several more vials worth of blood tests. One doctor said it was vasculitis and put me on massive doses of steroids. They did help my headaches but this was not vasculitis I learned. A doctor even thought that I had a condition called pseudomotor cerebri, which essentially is too much cerebral spinal fluid causing too much pressure in my head and therefore, headaches. So, yet another med was tried on me.

I was feeling like my whole world was spinning out of control at this point. I was about 26 at the time, had just graduated nursing school and was embarking on my first job in the medical surgical unit. I could barely function with these headaches and was even forced to take a medical leave. The side effects were horrendous with the steroids. I swelled up like a balloon, my feet actually jiggled as I walked due to the severe fluid retention. I felt horrible and weak. My body finally rejected the steroids, I went into flash pulmonary edema (fluid in the lungs) and had to be hospitalized. I was given IV diuretics to try and get rid of the excess fluids in my body. After 5 days in, I came out 20 lbs lighter! It was decided that although steroids helped my headaches, it was time to come off of them as they were doing more harm than good. A slow taper was initiated. Steroid cannot just be discontinued all at once, it has to be gradually decreased. If it is stopped abruptly, the body can go into shock and result in death. As badly as I wanted off of them, I had to be patient.

Unfortunately, migraines weren't the only problem at this time. My asthma was also acting up. Any exposure to noxious chemicals, such as paint thinner, waxes, even paint, would send my lungs into a severe coughing fit complete with wheezing. I also could not be around anyone who smoked, which is a good thing I guess. I was hospitalized frequently for exacerbations which were triggered often by respiratory infections such as bronchitis and pneumonia. I thought it was odd that I was getting so many lung infections, I blamed my asthma...my doctor blamed my asthma. So did the allergist. I believe that I was hospitalized on average about 3 times a year just for "asthma" related issues. At the time, I did not question why this was the case...not until a few years ago. I will explain this a little later...like a stone rolling down a hill, it moves slowly at first and then gathers speed rapidly. This is what happened to my health...first a few illnesses spring up, one here..another there. Then rapidly, everything gains momentum..one illness turns into another, what was a symptom years ago becomes very relevant later...all of this happens rapidly, all within a few years.

About 5 years ago, I had to stop working..not because I wanted to, my health would not allow it. My asthma was out of control, I was in the hospital more than I was out. In a period of just one month, I believe that I was hospitalized 3 times. 3 times...but, at that time I knew that it was much more than asthma causing this. I felt different, not like I did with an asthma flare up. I was short of breath all of the time, even worse whenever I laid down. Ultimately, I had to prop myself up on several pillows to sleep comfortably. My oxygen levels dropped very quickly with even the shortest jaunts. I was scared out of my mind as the doctors raced to control my symptoms, pumping me full of all of the typical asthma medications: steroids, theophylline, albuterol, advair...everything they could think of but nothing was helping. In fact, I was getting worse by the day. I was hospitalized again, but this time, I demanded to be transferred to another facility that could run more tests on me. I live in a very rural area, our local hospital is a 80 bed facility, so being transferred to a university hospital was in my best interest.

The university pulmonologists accepted me as their patient under the label as severely asthmatic. They assumed that it was asthma, but that diagnosis changed in lieu of my lung function tests (PFT's). They were shocked, as was I, that the results did not indicate asthma as being the greatest component to my problems. My muscles were the problem. The values pointed to a neuromuscular cause to my extreme shortness of breath. I nearly fell out of my bed...During that hospital stay, I was tested for everything under the sun including pulmonary hypertension, lupus, alpha 1, and finally mitochondrial diseases (affects muscles). The tests for some of these disorders were uncomfortable to say the least, a heart cath, a very involved EMG (electromyelograph) to test my nerve conduction as well as my muscles, and a muscle biopsy to boot. Unfortunately, nothing was diagnostic at this time, so I was sent home but with a new buddy: oxygen. That's right...even though the doctor's couldn't figure out why this was happening to me, I needed oxygen because my blood gases showed low oxygen levels.

Over a period of just months, my condition continued to deteriorate much to the frustration of everyone involved in my care, me included. I was hospitalized for pneumonia as well, during that time, the nurses observed that I was apneic at when I slept which drove my oxygen levels down even more. I was prescribed BiPAP at that time to alleviate my desaturations. BiPAP is a machine that delivers continuous air at two pressure settings. The inspiratory pressure kicks in when a breath is drawn, it is the higher of the two settings. The expiratory pressure is triggered upon exhaling, it is much lower than the inspiratory pressure. This is designed to take away some of the work from my tired muscles, to keep my airways open.

BiPAP proved to be a lifesaver on many occasions. As mentioned earlier, my muscles were weakening at an alarming rate, especially my diaphragm. The doctors still did not know why this was happening. I was spending more and more time on the BiPAP machine. Initially, I was using it only during sleep, but as I weakened, my time on BiPAP included daytime hours. In just two years, I became totally dependent on BiPAP. I was on BiPAP and O2 24/7. My lung function tests were abysmal at only 14% predicted for someone my age and height. I could not go to the bathroom without turning blue. I became a prisoner of my home on so many levels.

I contracted a viral infection which landed me in the hospital yet again. I was deteriorating rapidly. I could not eat, I could not tolerate coming off BiPAP long enough to swallow even a few morsels. IV's were essentially keeping me hydrated. After another multitude of tests, it was determined that my condition was not reversible and the decision to place me on the ventilator was made. My blood gases were also horrible. On November 1st, 2004 I was taken to the OR where the ENT surgeon performed a tracheostomy and I was placed on a vent permanently. Nothing prepared me for what lay ahead for me. I was going into this blind. Sure, I had taken care of many people with tracheostomies but I had no idea what it felt like to be such a patient. I only could offer support and a shoulder to lean on.

I woke up in recovery. I could not talk. I immediately touched where the trach was, felt the plastic hardware and all of the tubes that were connected to it. It seemed so foreign and it was. I looked to the vent that was now aiding my breathing. I watched my chest rise and fall with each whoosh of the machine. Again..odd. Surreal. My family filed in shortly after I woke up...I kept wondering what they would think of all of this. Would they be scared. My kids were at home, I worried if they were going to accept me this way as well.

The first thing that my sister said to me was "man, you are PINK!" I guess I didn't realize just how dusky I had become prior to being vented. I looked at my hands, turning them over and over and realized that they were indeed a pink hue! My mother, father , sister and my now ex husband approached my bed, gingerly and slowly. They probably thought that I was breakable, as fragile as a delicate glass figurine. But I wasn't...I was still me, just with a whole bunch of extra tubing. Maybe the ventilator intimidated them. Hell, it intimidated me, so I could understand if it was making them feel uneasy. I could not tell them that I loved them nor thank them for being in my corner. I am a jabber jaw by nature, I even learned how to talk even with the BiPAP mask on. For those who have this contraption, talking is difficult with air being constantly forced into you. Attempting to talk often results in a strikingly similar Darth Vadar tone. But, I mastered it to the surprise of my family and my doctors. But, now I couldn't talk, not as long as my balloon was inflated in the cuff of my trach. No air could make its way bake through the vocal cords to resonate them. I had only been awake for not even a hour yet I missed speech.

I spent a week in the ICU after the tracheotomy and vent. I developed pneumonia as well as severe bleeding which occurred from being suctioned too aggressively. Some nurses were gentle in their suctioning, but there were some that surely were aiming for my toes. I am serious about that one. I finally took the catheter and suctioned myself from that point on. I also inserted my own feeding tube after an intern made his attempt at canulating my stomach. He was ordered to place a naso gastric tube after doctors decided that I needed nutrition. I had not eaten in a month, my protein levels were very low. A swallowing evaluation determined that everything was being aspirated into my lungs, so feeding me by mouth was out of the question. The only viable answer: a NG tube. He gathered up his supplies, checking and rechecking to make sure that every piece of equipment was there and then made his way to my room. I knew that he was a newbie at this, he looked like he was going to jump out of his skin. He was visibly shaking as he grasped the NG tube, lubricated it and then proceeded to insert it into my nare. He advanced it ever so painstakingly slowly, I think glaciers moved at a faster rate! It was too slow and too painful for me to endure so I grabbed it and put it down myself all the while gagging and vomiting. The ventilator alarms were going off continuously, it sounded like an arcade room. Several nurses filed into my room in response to the cascade of alarms and upon seeing me insert my own tube, stopped dead in their tracks. The took notice of the now ashen intern, who had backed up nearly into the wall. He kept repeating over and over "she put it down! She put it down!" The nurses began to laugh and told him of my past employ as a nurse. He sighed, shook his head and left the room.

After a week in the hospital, I was transferred to a sub acute facility to learn the ins and outs of living at home with a ventilator. I had to learn how to do everything over again...from eating, to dressing, even showering. All required new skills and took an enormous amount of patience to master. I had to learn how to do trach care on myself using a mirror (these days, I don't use a mirror, I do it by feel. People often ask how I can change my canulas without the aid of a mirror, I tell them that it is similar to putting pierced ear rings in, the only difference is that the hole is much larger!) Speaking proved to be difficult, but in a few weeks time, I was able to do that as well. Doctors had always said that no matter what they did to me, nothing could shut me up and they were right! I loved speaking too much and was not about to let a trach stop me from talking. At first, my speech was very garbled, almost unintelligible but after repeated tries, I began to understand what I was saying. Now, people who talk to me on the phone would not even guess that I was attatched to a ventilator.

This is a good time to end part one...I will be sure to add part II soon so stay tuned :)
Thanks to those who have managed to make it this far into my blog. Hugs, Jenn :)