Today is a special day, as well as yesterday even the day after tomorrow will be and so on for myself and others. March 21st marks 6 years since my second double lung transplant, followed by March 22nd making it 14 years from my first transplant. My family, friends and I acknowledge them as lung anniversaries just a little extra celebration that we are thankful that I am here today. Every day after are special, they are extra days, hours that I can tack onto those 14 years of being here enjoying life and making memories with all of you.
I count each day as a blessing and I am so grateful for my organ donors and their family’s for giving me the gift of life not once but twice. As well the hard work from the Doctors and nurses that have helped me along this journey. Last year was just a little reminder to me for how important life really is and how lucky I am to have received these gifts.
My last blog post was written last February 2015 “In for an Oil Change” talks about how my lungs were in rejection and the treatment that the transplant team wanted me to follow. Plasmapheresis, IVIG, and IV Pulse Steroids. I always planned on giving an update on how I was doing after this treatment, I just didn’t know how and when was the right time to post it. What better time than a day like today; so special but what can I say EVERYDAY IS SPECIAL.
February 13, 2015, I was discharged from Toronto General Hospital after my Plasmapheresis treatment was completed. I had received my first few courses of my new treatment called IVIG and would require another 6 to 12 depending on my body’s tolerance. It would mean coming down to the transplant day unit at TGH each month and spending a few hours hooked up to an IV.
The IVIG was set up to treat the antibodies that were found during my last lung bronchoscopy, you might have already looked up what IVIG is, if not let me give you a little information on the treatment.
IVIG is a blood product that is ministered through an IV. It is made up of immunoglobulins sort from IG, which is also called antibodies. These antibodies are clean antibodies that are to replace the ones that were attacking my transplanted lungs. Antibodies help protect the body from germs, such as viruses, or in my case, my body thought my transplanted lungs were viruses.
The treatment of IVIG is the IG that is prepared from the plasma collected from a large number of normal individuals, usually between 10,000-50,000, who have been carefully screened to make sure they are healthy and do not harbor certain infectious diseases. The plasma contains a broad range of specific antibodies to many different types of bacteria and viruses. Each plasma donor must be acceptable as a blood donor according to the strict rules enforced by the Canadian Association of Blood Banks. Donors are screened for travel or behavior that might increase the risk of acquiring an infectious disease. Only the IVIG is purified from the pooled plasma. To commercially prepare the IVIG for patients with primary immunodeficiency diseases, the immunoglobulin must first be purified (extracted) from the plasma.
IVIG replacement therapy is generally administered either intravenously (abbreviated IVIG), or subcutaneously (abbreviated SCIG). IVIG infusions are usually given every three or four weeks. SCIG infusions may be given as often as daily, weekly, or as infrequently as every three to four weeks (similar to IVIG), depending on the specific SCIG product that is being prescribed, the patient’s age and the preferences of the patient and the prescriber.
Before the treatment, I am given a dose of Benadryl in the case of any allergic reactions from the IVIG. My vitals are followed closely as some of the side effects include a headache, nausea, low-grade fever, chills, rash, neck/back stiffness, hair loss, and fatigue. Generally, these side effects are mild and tolerable and most often, they can be alleviated by decreasing the rate of infusion. More serious side effects, such as allergic reactions, are rare but have been reported.
Due to my small size, the treatment only takes a few hours as the dose is based on size and weight. I am also given a large pulse of prednisone to try and treat the rejection.
This becomes a monthly routine for about 3 months, at this time there wasn’t much improvement or results, in fact, I could say my PFT’s, (oxygen saturation) was getting worse. I was really tired; my weight was dropping. I didn’t have much energy throughout the day and started a bad cough, my muscles ached as did my joints to the point I could hardly move. I also started to notice some hair loss.
My cough was so bad it got to the point where I made an appointment with my transplant team. I went down the following day to Toronto General Hospital after calling them as the team were concerned with my condition and were following me closely. I did all the regular tests (blood, X-ray, PFTs as well a sputum culture) before the team saw me. Most of my test results hadn’t come back by the time I saw the team. They put me on a high dose of oral antibiotics and told me if I felt as if my condition was getting worse to call them and come back down.
A week passes and I still did not feel well, in fact, I felt like death, my breathing worsened my body was stiff, overall things just sucked. I heard back from the hospital and they said my sputum culture came back growing Pseudomonas.
Pseudomonas is a bacteria/fungus that can be found grown in the blood or lungs causing infections. They wanted me to start IV antibiotics. The team felt that I could just do home IV’s all I needed was to get a pic line put in. The team was going to arrange the pic line to be put the following day as well set up home care.
A pic line is a peripherally inserted central catheter; a form of intravenous access, it extends to the superior vena cava that can be used for a prolonged periods of time. I returned the following day to get my pic line put in and my first treatment of antibiotics at the transplant day unit. This was followed by a 2-month course of home IV treatments.
During this time I was still getting treated with my monthly IVIG. I was into my 5th treatment and the side effects were getting worse to the point where I could barely move. Walking up stairs took so much effort as the pain would shoot throughout my body with each step I made. Little tasks such as climbing into bed would take 5-10 minutes – so much effort and pain. Trying to open the jeep doors and climb in was physically impossible; the pain that radiated through my body was unbelievable. I felt like I was being electrocuted. There were times I wanted to move my hands or legs mentally yet physically nothing happened. I felt crippled and depressed, my life was being tested. I spent most of my days just sitting on the couch trying to sleep as I wasn’t sleeping well at night.
During this time I remember writing in one of my journals,
“The world you’re used to is getting smaller, my limitations are becoming a sad reality. The easiest task turns into a day’s project, making me feel useless. I watch my body detransform into a broken down operating system. It mentally irritates my mind erasing all the goals that had been chosen for that day. The lack of oxygen running throughout my body and the pain that has staked its claim make simples tasks so challenging. I need to rest and prepare for the next day, another long day in hell.”
We often make tomorrow the busiest days of our lives as we feel we can just push whatever we don’t accomplish today into another tomorrow. Not many of us live in the moment. The past is behind us, the future ahead but each moment is only lived in the now.
My motto is to live for today. I have learned to fill my days to the fullest. Go see a friend across town or go out and see a family member or better yet go travel. Take that walk or do that drive you been thinking of, just try and stay busy. Live every day as if it’s your last, the little things do count.
What would you do if I told you tomorrow would be your last day alive?
I bet most of you wouldn’t know how to respond. You may say one thing but wouldn’t follow through.
Why is that?
During my “difficult times” last year I wasn’t living much of my life. I was simply existing; I would watch the days and nights go by slowly without being able to do the things I loved, nothing brought me joy. My mom saw a big change and was worried. I was tumbling into depression and had no energy to change it. I felt useless, withering away, couldn’t do much about it, shortness of breath plagued each moment, I couldn’t get anything done.
As June approached Tanis was definitely worried. She decided that she would take matters into her own hands. She contacted the hospital and told them she was worried for my well being and thought maybe it was time to have another clinic appointment. She wanted me to go on home oxygen so my body wouldn’t have to work so hard. My body at this time was using so much energy towards breathing that it was breaking down muscle and body weight to be used as energy.
I head back down for a follow-up to the hospital this time I did all the usual tests that the team looks at, before seeing me. I had an extra test the team wanted me to do called a 6-minute walk. It’s a test to see how much oxygen I was using while exercising. This test is performed to see if you require the use of added oxygen. The way this test is performed is by two 6 minute walking tests. You are constantly monitored to a desaturation device that alerts the technicians when your oxygen levels drop below 85%. The first test you walk without the aid of oxygen and use room air. The second test is done with oxygen to see if your body benefits from the use of oxygen. You aren’t told what test you are performing as it’s all done randomly because they want it to be a clean test. If your oxygen saturation drops down to 85% during the test without oxygen you may apply for the use of home oxygen.
In my case, after about 3 minutes into the test without oxygen, my O2 sats were 85% and dropping making me eligible for oxygen. For those of you that do not know what O2 sats are it’s referring to the fraction of oxygen-saturated hemoglobin relative to total hemoglobin (unsaturated + saturated) in the blood. The human body requires and regulates a very precise and specific balance of oxygen in the blood. Normal blood oxygen levels in humans are considered 95-100 percent.
I head back upstairs with my mom and brother Matt for my transplant appointment and we meet with the team. They were glad to see that I qualified for oxygen and hoped it would help with the aid of adding weight and helping with giving me more energy. I brought up my concerns regarding the IVIG. I felt it wasn’t benefiting me as my PFTs were decreasing and my body was stiffening and my joint pain was getting worse. In fact, at this point in time, you could start to see what looked like my joints swelling up almost double in size in my hands. The Team decided that we could stop the IVIG treatments and scheduled me to see Dr. Rubin who is a Rheumatology Dr at St Michael’s. The team was interested to see if the IVIG had increased my arthritis.
There was one more topic the team wanted to discuss with me, quite a serious topic actually. Re-transplantation was said. Once the doctor said it out loud it was if a bomb went off, the room fell silent; heaviness fell silently into the stunned silence. I could see the concerned look on my mom and brothers faces. As if I was being taken away from them. The first thing that was said after the bomb went off, was
“You mean a third lung transplant”?
“Yes a third double lung transplant”, the doctor answered very calmly. I didn’t react the same way as my brother and mom. I remember looking the doctor straight in the face giving her the sign that she had my full attention, at the same time my brain was in overload.
I didn’t take this as a negative sign, yes the word re-transplant was just brought up but at least it is being brought up as a chance offered or at least being considered by the team.
With the topic being mentioned this brought up some serious questions to be asked.
Question one- do they really think I needed to be re-transplanted; their answer was it really is up to me as only I knew where my quality of life was heading.
Question two has a third lung transplant ever been done in the past. This question wasn’t answered right away, but with the blank look on the team’s faces, I took it as a “NO”. In fact, there has never been a third lung transplant performed anywhere.
With everything being said I still had a smile on my face, yes I’ve just been told in a way I am dying, but I have also been told there could be another chance at life. Why would I want to give up now? I’ve been here before it’s not the best feeling in the world but I enjoy Life and when given a chance to know there are options I’ll fight and do whatever it takes. I also know I have an amazing support team behind me to pick me up whenever I need it.
This brings me back to where I ask you if you knew today or tomorrow was your last day what would you?
One thing I can say is I am blessed with are, friends and family. My grandfather always told me when you are older and you can count on one hand the total of loyal friend you have at the time and fill a full hand then you have lived a successful life. Well, thanks to many of you I know I could use all my fingers and toes and more. Knowing this has always given me the feeling as if I have an army behind me as I am always preparing for war.
The Dr asked me how I felt regarding a third transplant. I told her it’s something, definitely something to think about and I wasn’t going to turn down as I love living and enjoy life. It was just a matter of when the right time would be. I was then told if I would like to start the process of being re-listed on to the transplant list. This involves many tests that would be reported back to the team, just because the team thinks I would benefit from a third transplant does not mean I qualify for one. A series of tests need to be performed and then evaluated by a board of doctors to see if my body is strong enough to accept another transplant.
Now, this wasn’t something I didn’t want to announce to the world yet. One I wasn’t sure if I would be put on the list as my case would have to be brought to the transplant team and from there they would decide whether or not I can be a candidate as well if they want to see what my test results looked like. A transplant is very hard on your body a third transplant is unimaginable.
Yes, I require new lungs to live but if my heart, kidneys, liver and other organs and body aren’t strong enough to survive the battle why give me new lungs when someone else would benefit from them more.
I plan to talk about this in another blog soon as I feel there has been a lot brought up already, I promise.
Two weeks pass by and I am on 24/7 use of oxygen. I had to go see Dr. Rubin at St Michaels hospital regarding my case with the pain throughout my bones and to see if he could answer why my joints were swollen. Once Dr. Rubin comes into the room and sees and feels my hands he instantly wanted to send me in for a few X-rays that day as well a few bone scans however the bone scans could not be performed that day and needed to be booked for the following weeks.
After I had a few X-rays on my hands, hips, feet and spine I return to the room to see what Dr. Rubin had discovered. He was at a loss for words and couldn’t say much other than there wasn’t any extra arthritic activity going on in my body and that the X-rays showed that it wasn’t in my joints at all. The x-rays showed swelling just before and around the joints as a blurred picture on my bones. He couldn’t give me any results yet and would like to see what the bone scans showed as well send me for a few blood tests. Some of the tests included vitamin levels. He was interested to see mainly if my vitamin A level was increased as it is a cause for increased calcium build up.
A few weeks pass by and I get a call to come in for my bone scans as well the same day a message from Dr. Rubin that my vitamin A levels were quite high. This is odd as with having Cystic Fibrosis my body doesn’t absorb vitamins well at all and I am required to take a few supplements to help with this. With this being one of the findings he asks me to stop daily vitamins as well my vitamin A and have my blood work retested the next time I am down at TGH.
I have my bone scans done completely a full body CT scan, as well my blood work drawn the same week. A bone scan is a nuclear medicine imaging test that uses bone-seeking radioactive materials or tracers (radiopharmaceuticals) and a computer to create an image of the skeleton (bones). A bone scan looks at the bones to see if there are any abnormalities, such as a fracture, tumor or infection.
You may be asking what the difference between a bone scan and X-ray is
A bone scan is used to show bone density and is used to show more detail it’s a highly sensitive test it can also be used to determine if a compression fracture of the vertebral body is old or new, as an old fracture will not light up and a new one will. It can locate damage caused to bones by infection, breaks or traumatic injury, and is helpful in finding bone cancer too. Bone scans are sometimes performed to rule out an inflammatory process like a small tumor or infection or an occult fracture which is small fracture not seen on an x-ray.
Bone scans, however, cannot distinguish what a lesion represents, and therefore cannot differentiate between a tumor, an infection or a fracture. Therefore, this type of imaging study usually needs to be followed by a CT scan and/or MRI scan to better characterize the lesion.
The results of the test reveal ‘hot’ and ‘cold’ spots. Hot spots appear darker on the image and denote an area of high tracer uptake, possibly indicating an abnormality (e.g. infection). Cold spots appear light and indicate the bone absorbed less of the tracing element.
During the time I was waiting to hear back from Dr. Rubin, I got a call from my transplant coordinator telling me that my kidney and liver enzymes were very high and they were worried and wanted me to stop taking my drug called Voriconazole. I am treated for Voriconazole for the Aspergillus that has been growing on and off in my lungs and sinuses.
Aspergillus fumigatus is a ubiquitous fungus found worldwide in soil, decaying vegetation, foods, dust, and water. Normal hosts quickly clear a fumigatus upon exposure, whereas it often persists in the respiratory tracts of patients with CF. Reported prevalence rates for a fumigatus based on sputum cultures range from 10% to 57% in patients. Prevalence of infection may depend on clinic geography as well as individual clinics’ surveillance methods for endobronchial fungal infections. Culture positivity for A fumigatus has been associated with increasing age, antibiotic or steroid use, indwelling catheters, and Stenotrophomonas maltophilia colonization.
The regular treatment for Voriconazole is 3 months on 3 months off however whenever I would come off the Voriconazole after my second transplant the Aspergillus would flair up making me sick and usually needed to go in for a hospital admission for IV antibiotics for several weeks. So the team came up with the idea of a new treatment of staying on Voriconazole continuously.
Voriconazole is used to treat serious fungal infections such as invasive Aspergillosis (a fungal infection that begins in the lungs and spreads through the bloodstream to other organs). It is also used to treat esophageal candidiasis (a yeast [a type of fungus] infection that may cause white patching in the mouth and throat) and other yeast infections of the skin, stomach, kidney, bladder, and wounds. Voriconazole is in a class of antifungal medications called triazoles. It works by slowing the growth of the fungi that cause infection.
Some of the side effects are sudden behavior changes, problems with thinking or speech; upper stomach pain, itching, loss of appetite, dark urine, clay-colored stools, jaundice (yellowing of the skin or eyes); urinating less than usual or not at all; bone pain, swelling; uneven heart rate, chest pain, general ill feeling; or severe skin reaction; fever, sore throat, swelling in your face or tongue, burning in your eyes, skin pain, followed by a red or purple skin rash that spreads (especially in the face or upper body) and causes blistering and peeling.
A few days after my bone scans were complete I received a voice message from Dr. Rubin himself, and he sounded quite excited to talk about the results he found. And wanted me to come into his clinic the following day.
Once again I head down to St Michaels hospital to see him at his clinic. He saw that I was in the waiting room and called me right into an exam room so we could talk. On the screen of the computer was my bone scan, as I looked at it I could see a skeleton. There were dark spots all over the areas where I was feeling pain or noticed large swollen areas on my body.
He instantly said,” bone growth”. I said “pardon”. “It’s not arthritis that you are experiencing its bone growth. Throughout my body, I was experiencing new bones growing on old bones. He said No wonder you have been in severe pain.”
“So am I like wolverine”, I said in a nervous voice. He instantly replies with no I sent your case out all over the health network to see if anyone has seen or heard anything regarding this issue before. It’s very rare however the cause is an increased vitamin A level making your body increase calcium levels that are trying to repair any bone injury or even the slightest hit to a body part causing bruising. My body would send out a chemical to try and repair the injury and with this was building the bone to protect my body from future injuries.
This was all due to the Voriconazole increasing my Vitamin A levels. Also a result of my hair loss I was experiencing. The levels of Voriconazole were so high my body was starting to have a form of toxic shock. So, in the end, the IVIG was not causing the negative side effects to my body.
A month passes of being off of the Voriconazole and I start to notice a big improvement throughout my body. The pain that I was having was slowly melting away as well the swelling in my hands and feet. Dr. Rubin told me I will be left with the extra bone deposits throughout my body. The growth of the bone will stop but he would like to follow my case as it is very unusual.
With the pain gone my body started to get back to a normal state. It allowed me to fall back into my routine of being able to work out. I slowly increased the intensity of these workouts, becoming more mobile and returning somewhat back to my “normal” life. I slowly started to need less oxygen to the point that now I only use it when I am working out.
I noticed a big difference in my life; I was able to see some light at the end of the tunnel. I felt almost whole again, I still knew I have a large journey to face.
Last week was my 6th-year lung assessment (March, 16. 2016), it’s where I go down to Toronto General Hospital to perform a bunch of tests that the doctors can review and compare how well my lungs are performing. The team seemed to be happy with what has gone on over the past year. There are some issues that I still need to work on such as weight gain and continuing to keep my blood sugar levels low. They were also concerned about my immune suppressed drug levels being too high. My PFTs levels have stopped dropping, in fact, they went up .5%. It’s not much as they can fluctuate up and down a little but it’s heading in the right direction. I do not have much room for any more decreases in my PFTs.
After all the results and test were reviewed with me my team asked how I was feeling towards being put on the transplant list. I answered that when my quality of life is no longer worth living I will consider it.
6+8=14. Fourteen years I have been able to add to my life solely because two families knew becoming organ donors was the right thing to do. This has enabled me to “Live my life as much as possible”. I can be that happy person who can go out and be the life of the party.
The other day my Mom posted, “Imagine you are in a card game. The stakes are incredibly high. If you play the wrong hand you’ll die. If it so happens that the cards fall in your favor – you get to live – there are no guarantees for how long or how well”.
Life is a card game not everyone is dealt the best hand to play. The outcome of you winning or folding is how you play the game. Some of us have learned the art of bluffing, having fun and making it a social event. Taking a bad hand and winning despite the odds, you never know what the person you’re playing with or against may have in their hand. My Mom says I’m a card shark – I think I am.