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My journey into the realm of transplant actually began in July 1999, but I didn't know it then. I was in the hospital and Dr. James Tomlinson came to see me. He is a pulmonary and critical care specialist. He was (and still is) a real nice guy. It was then that he told me his opinion was I needed to consider getting listed for a double lung transplant. I was stunned. I was in denial. I didn't want to be told this was my next step in living with cystic fibrosis (CF). I brushed him off as a yahoo at first. An alarmist, I thought. (I was wrong). I put his offer aside and vowed to take better care of myself, just to prove him wrong. Jump ahead to April 2000 I had jumped ship from being a 36 year old adult and still in the pediatric CF clinic, to a new adult pulmonary practice in Norfolk, Virginia. It was one of the best decisions I have made. Fortunately for me, Dr. AJ Quaranta took over my care and he was very aggressive in treating me (which I really needed!) I continued to work full time and put CF on the back burner. I was hanging in there and enjoying life. Married life to Brian was awesome. I was going to show them all that transplant was not an option for me, that I would take better care of myself and make it all go away. Winter 2001 The past few months were rough. I think I was on antibiotics more than I was off them. Not a good trend. Brian and I will celebrate our 3rd wedding anniversary this Spring and what a real blessing he has been in my life. Brian is always there and so very supportive. We have talked about transplant, but not really personalizing it to my circumstances. I created an artificial construct which was a paradigm of failure. It went like this: If I can work 40+ hours a week, then I will be normal like everyone else. No room for self loathing or pity here. But wait....it gets better. Since I will pretend to be normal now, I can slack off on the treatment a little. Wrong-o! Try as my warped mind did, it does not work that way. So the more I worked to prove myself normal, the more I slipped into a downward spiral of failure and sickness. It is a full time job in itself--living with CF! November 2001 I was intubated and ventilated for respiratory failure and after 10 days, I was finally weaned from the ventilator. Another scary ordeal to go through.... again. I clearly remember Dr. AJ Quaranta asking me..."Lori...how many more times are we going to do this [being put on the ventilator to breathe]?" I told him..."I want new lungs!" My Pulmonary Function Tests revealed a slow and predictable decline in my FEV1 (Forced Expiratory Volume in 1 second) where my lung volume was at 32% of predicted for normal. At about a 2-3% drop per year in CF patients, the writing was on the wall. I began to seriously consider all my options. When you can't breathe anymore, it is either new lungs or death. "I have changed my mind." Sitting in the chair next to my bed in the ICU at Norfolk General, that phrase seemed to just float casually into my mind. It was like some message from above, out of the blue, but it hit me like a ton of bricks. Suddenly I wanted a lung transplant. That was probably the biggest surprise to admit to myself, that after so many years, in just one moment my mind could change so quickly and so definitively. There is a big fork in the road up ahead. Up until now, I thought maybe I had my CF life all planned out. I thought I had my CF death all figured out as well. After all, I have been blessed to experience so many things despite having CF, that a shortened life seemed a small price to pay. In my mind, when the time came, I would die with the help of a Propofol & Fentanyl IV drip with family and friends at my side. What a selfish thought. I am certain they will have me visit a shrink to discuss these feelings, but for now, I will simply say I have changed my mind. However, I do not want to rush the process. I know the upside: that people who get new lungs can breathe easily, for the first time in their lives or for the first time in a very long time. Some can dance, ski and roller blade. Others, who couldn't walk five feet without gasping for air before, gradually are able to run. Others can now go to college full-time and look forward to working. But I also know the odds that come with transplant. One in six aren't alive a year after the transplant. Half don't make it five years. One in 15 don't even make it out of the hospital (like my 2 CF pals Jeff and Eileen). And those that can breathe after the transplant are breathing in a new world, one that comes with complications and debilitating side effects. These people can have diabetes and kidney failure, insomnia and tremors, numbness and depression, hair loss and hair growth. There is lower back pain, hypertension, heartburn, fluid retention, acne and hormonal problems. Lung transplant recipients can go blind. And then there are medications, sometimes as many as 15 a day, just to deal with these side effects. Meanwhile, at any moment, the body may reject the donated lungs, acting on instinct to reject that which is foreign. And so people who receive new lungs must also take a battery of drugs to suppress their immune system. The idea is: trick the body into believing that these strange organs belong where they are. They never know how long the trick will work. They never know how long they'll be healthy, before infection returns or rejection sets in. Knowing these possibilities, I decided to try and get on the lung transplant list at Norfolk General Hospital. For me, it is a win-win situation. I either get new lungs, feel better, have a better quality of life, and return to the job I love. Perhaps I get the new lungs but die or I do not get any lungs at all and still die. Either way, I will be with God. It is still a win-win situation for me no matter what the outcome is, but of course, I would prefer the chance to breathe normally again and not die from respiratory failure. January 2002 I began the evaluation to see if I would be an acceptable candidate for a double lung transplant. I did the testing as an outpatient and took my time doing it. I did not feel there was any great urgency, after all, I was taking better care of myself now and felt that had to pay off some how. I was still able to work at the dream job I love doing, as a Death Investigator and with a lot of planning, I could still travel. It meant lugging oxygen, nebulizers and the ThAIRapy vest but I managed. Brian and I were both hoping the transplant team would come back to me and tell me I was too healthy to be listed for transplant. My lung function tests now showed my lungs were only working at 29% of predicted. Hmmm.... Our Insurance Criteria for Approving a Double Lung Transplant Disease-Specific Selection Criteria: Lung transplant for cystic fibrosis is considered medically necessary for persons who exhibit two or more of the following signs and symptoms of clinical deterioration: · Initiation of supplemental enteral feeding by percutaneous endoscopic gastrostomy or parenteral nutrition · Cycling intravenous antibiotic therapy · Non-invasive nocturnal mechanical ventilation · Increasing frequency of hospital admission · Increasing severe exacerbations of cystic fibrosis - especially an episode requiring hospital admission · Recurrent massive hemoptysis · Development of CO2 retention (pCO2 > 50 mm Hg) · Worsening arterial-alveolar (A-a) gradient requiring increasing concentrations of inspired oxygen (FiO2) · Decreasing FEV1 · FEV1 < 30 % April 6, 2002 Official letter arrives from Norfolk General congratulating me on being a good candidate for a potential double lung transplant. That means I am listed nationally with the United Network for Organ Sharing (UNOS) and waiting for someone to die, so that I could live. My transplant center thought I'd be a good candidate and I now shifted my focus to being ready for transplant. However...some where along the way, I got lost. What do I mean? Well, I did not follow the doctors recommendations to their satisfaction. I picked what worked for me and ignored the rest of their suggestions. I self medicated and did not call the Transplant clinic when I was getting in pulmonary trouble. All cystics do this: they learn from an early age how to take pills and make decisions. Not feeling well? Add albuterol treatments. Short of breath? Increase your Prednisone dose. Touch of pulmonary edema? Take 20 mg of Lasix. The Transplant Team thought I worked at my job more than they approved of, and I did it just so I could feel in control and be useful. I slacked off on nebulizer treatments and burned the candle at both ends. Bad Lori! This is called "noncompliance". In came the Shrink. "Why wasn't I more dependent on the doctors to run my life?", the Shrink inquired. "Well, living with CF has taught me how to best cope and I have learned what works for me. I am independent as much as possible and I don't run to the doctor for the slightest cough, hemoptysis or wheeze. I tough it out." The bottom line? I was made inactive (called Status 7) for new lungs due to my so called "control issues". So even if the perfect set of lungs were to match me, I would not get them until the Transplant Team had me back on track (their track). What a kick to my self-esteem, ego and psyche. However, even in this predicament, the Lord had everything in control. May 2002 My husband and I planned a vacation to Walt Disney World and invited my family to join us. There were 10 of us and what was to be a fun time, ended up quite the opposite for Brian and I. When I arrived at Disney, the fevers began. I took some Tylenol and went out to dinner. The next day, I spent all day in our hotel suite at the Grand Floridian, tossing my cookies nonstop. But this was Disney, the Happiest Place on Earth. I thought I had the flu and with a few days rest, it would go away. Nope. On day 2, my family insisted I go to the hospital, and at that point, I was feeling quite sick, so I agreed. I was in the ER of Florida Celebration Hospital at 10 PM with my husband Brian at my side. I did not present to the ER with complaints related to my CF, rather it was my mottled, purple legs and fluid in my lungs which concerned me the most. By 2 AM, I was in respiratory failure and put on the ventilator to breathe. My husband Brian was scared. My family had to return to Canada, leaving Brian all alone in Florida. We were in unfamiliar territory, had no friends nearby, and he was anxious and depressed. Yet even in our deepest despair, God intervened. My boss in Norfolk, Donna Price, flew down to sit by me and comfort Brian. I was gorked out and have no memory of this at all, but it was like a burden had been lifted off his heavy heart when Donna arrived. Our friend Lori Jernigan, a Duke CF double lung transplant miracle, also flew in from Norfolk to lend her emotional support to Brian. (Dr. Randy Garnett would later caution her never to expose herself to my CF germs which could compromise HER transplant. She has not listened and feels CF will not dictate who we can or can not visit or be friends with). My sister Noreen, flew in from Canada to be at my side, to uplift the medical team in prayer and to minister to Brian and his spiritual needs. Amen for sisters! So even when Brian felt most alone, God sent much needed support our way who quite literally, cheered Brian up, allowed him to go back to the hotel to rest and to shower, and still know that people who loved us both were there for us. There was a point in my hospitalization when Brian was told I was very critical and it was doubtful I would survive. After 28 days of this, with dimming prospects for recovery and fear I would die in Florida, Brian arranged for an air ambulance medical team to fly me home to Norfolk General Hospital on a Lear Jet where my doctors in the Pulmonary and Critical Care Division at Sentara Norfolk General Hospital took over my care. Dr. William Brock agreed to accept me as a ICU patient and finally, a few days later, I was stable enough to be flown home with a medical team, hooked up to a ventilator and at that point, coming home to doctors who knew me and had cared for me before. Great flight home, too bad I was gorked out and do not recall any of it, even the welcoming party at Norfolk Airport (thanks Donna & Jim Price). Fast Forward---> 4 months later. I had survived my bout with multi-system organ failure, sepsis, pulmonary edema, respiratory failure, pneumonia, renal failure, thrombocytopenia and bacteremia. 5 weeks in rehab to learn how to walk, eat, toilet, bathe and dress helped me progress to the point where I was discharged home to my waiting kitties and husband. Stair climbing was the most difficult thing for me to do, and since our master bedroom was on the second floor, those 16 steps seemed impossible. It made me certain my next house ought to have an elevator! It was a very difficult emotional journey for my husband Brian, yet through it all, he never left my side. Some days I was very mean to him (it was the drugs!) and I was discouraged at my lack of progress, however, once again, the Lord was gracious to us and spared my life. A Bump in the Road... I was orally intubated and ventilated for so long in the Florida ICU that my trachea was damaged. I had to have a tracheostomy and a PEG (feeding tube). When I finally woke up 6 weeks later in the ICU in Norfolk, I was devastated to find out I'd been to the OR 4 times for surgery and was trached. I also had my Medi-port removed from my chest due to a staph infection. I cried, sobbed and weeped. I was angry at everyone and confused. Last I remembered, I was at Disney! What had happened? Why had this happened? I begged Dr.Randy Garnett to let me die. I was even mean and nasty to him at times (again, it was those drugs). I could not talk (due to the trach) so I scrawled notes on paper pleading with him to make me a no-code, a DNR, to please let me die. The pain was too great and I was mad I'd had no say or control over this situation. My legs would not function due to foot drop. I could not walk. I had a hole in my throat to breathe and another in my stomach to eat. I had hit rock bottom. Sign me up for AA meetings! I had lost all control and for a control freak, this was not a good thing. Dr. Randy Garnett (my family loved him) said they (The ICU Team) had worked too hard to get me this far, so there was no giving up allowed. I really began to dislike the doctors. (Send in the Shrink again...) At least I was alive
September 2002 I was only home 2 weeks since being admitted in May when I was admitted yet again for another pulmonary infection and trach problems. I was not doing well managing the trach. One of the many problems with having CF is that the sputum produced is usually very thick and viscous. This makes it very hard to move it along and cough it out. Hmmmm.....so my trach would often get clogged with thick secretions and it made breathing difficult. Not a good thing. Ohhh how I hate the trach. Once again, I was read the riot act by the Transplant Team for not being compliant and doing all I could to be in the best shape for transplant. This was the second time they put me on notice (most people do not get a second chance, I am told) The only thing I could do was to turn all my concerns, problems and anxieties over to the Lord. He has promised me He will always be there, never leaving my side. I could not go this route alone. So many friends, family and strangers were praying for me, and I had to trust the Lord to direct my path. As His child, as a Bible believing Christian who had accepted Christ into my life at the age of 11, I was His instrument. A piece of clay for Him to mold and shape my life according to the Potters plan. At the time I was most neediest, most down and out, most fragile, He was there! I just had to ask. He heard my cries and as I let Him take control, things fell into place. God was once again, piloting my life. December 2002 The sad news is that I have become trach dependent to breathe. No, I am not stuck on a ventilator at home to breathe, but I have to use oxygen 24 hours a day, every day. I still have "issues" with using oxygen out in public. It is embarrassing sometimes to have people gawk at me when I am in public, but slowly, and with some acceptance, I am more comfortable and acknowledge that this is just the way it is--until I get my new lungs. The trach poses a new problem: I am not going to be transplanted unless the trach can be removed and I can support my own airway in a functional capacity to allow me to proceed with transplant. It's a catch 22 situation: I need the trach to breathe, but having it means no lungs. So...again the Lord intervened. Via the Internet, using the Google search engine, I was directed to Dr. Hermes Grillo and Dr. Cameron Wright, both in Boston, both affiliated with Massachusetts General Hospital, who will consult on my tracheal problem in late January. I won't know until then if my tracheal problems can be fixed, but I'd appreciate your prayers. Christmas is just 10 days away and I am all ready for it. It's not the gift giving but the time with family that I look forward to. To open up the Bible and read from Luke chapter 2, of the birth of the Baby Jesus, and to give thanks for all that I have and to be hopeful the new year will continue to shower me with blessing from God. This was one Christmas I almost was not around to celebrate. I am counting my many blessings!
Here are the CT scan results that document my CF lung disease:
I also have had 3 sets of POSITIVE Sweat Chlorides, all done at different CF clinics and also CF Genotyping, done at both Yale and NIH. UNC and Lab Corp were unable to find my CF genotype as they both use very limited CF mutation panels. There are over 1500 CF causing mutations, and very few labs go through the painstaking task of finding those rare ones, those less than 1% of the population. My two CF mutations fall into this less than 1% category. Only 2 labs have been able to find them correctly.
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