Archives For lung transplant

Eric+Marissa - Breathe Deeply Sign

Marissa’s Perspective:

CFers are an incredible bunch, and Eric is no exception. Having a close view of his courage, strength, and determination has changed me for the better…and the not-so-easy times we’ve encountered have helped me grow as a person. I’ve become more thankful for the many positive things in my life, more content to enjoy simple pleasures like a night at home with my favorite person in the world. I’ve evolved into a creative problem-solver, having become a master organizer of medical supplies and sneaked cream cheese into Kraft Easy Mac to add much-needed calories. I stay cool in a crisis and am always prepared (just ask the EMTs and ER staff), thanks to some epic hemorrhages, a cancer diagnosis, and other emergencies. 

Mostly, I’m stronger. As a spoonie myself, I never would have believed I could make through what we dealt with this past fall. I was getting 2-3 hours of sleep a night as I held on to my full-time job and made it to the hospital 5+ days a week to talk to the doctors and attend transplant caregiver classes. On top of that, I was fundraising for transplant expenses and doing my own medical research in my “free” time, all while the love of my life, my whole world, fought for his life and we anxiously waited to hear if he would be accepted as a transplant candidate. 

And most importantly, I’ve become a believer. A believer in miracles, God, hope, love, and a small, small world. My new take on belief started the day after Eric was finally listed for a lung transplant after an emergency inpatient evaluation. It followed an episode of massive hemoptysis, two hospital transfers, a BAE, many days in the ICU, and a call from an ICU doctor saying that they would be forced to intubate Eric due to the massive strain his heart was under trying to compensate for his broken lungs—the call that left me sobbing hysterically at my desk before racing to the hospital while making impossibly difficult calls to our families. 

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I headed to the hospital that morning feeling relieved that Eric had finally been placed on the transplant waiting list. The relief didn’t last long: the hemoptysis, which had been held at bay for over a week, returned and grew more severe throughout the day. Not long after I arrived, Eric got a call from an out-of-state number on his cell phone, which he ignored. A few seconds later, I got a call from a number I didn’t recognize. It was a transplant coordinator, calling to inform us that a pair of donor lungs had been located and were a match for Eric, he was at the top of the list. We were in complete disbelief. Getting “THE CALL” less than 24 hours after joining the wait list? Certain this would turn out to be a “dry run”, we kept the news to ourselves and tried to enjoy the day together, keeping the news to ourselves but growing more and more worried as the hemoptysis got worse and the doctors told us nothing more could be done to stop the bleeding. I helped Eric wash his hair and we talked about the transplant education classes he’d missed as an inpatient. 

As the day progressed, we continued to wait for updates on the status, expecting each call to end in disappointment. Eric, exhausted from coughing, fighting for oxygen, and the flurry of surgical preparations, thankfully fell asleep. That left me alone in the dark hospital room, worrying about the rising level of blood in his culture cup and awaiting more news from the transplant coordinator. We finally got the call around midnight—the lungs were a go! Less than 36 hours after being listed, Eric was in surgery, and not a moment too soon. When I spoke with his surgeon the next day (after 12 hours in surgery), he told me that Eric’s lungs were in horrible shape, with a number of blood vessels ready to burst

Add to that all of the amazing things that I witnessed as Eric recovered post-transplant—breathing room air and walking as soon as he was weaned off sedation and walking nearly a mile just a couple of days after surgery. Then, a few weeks later, we were contacted by the donor’s family following a news story…and now the donor’s mother calls Eric her “bonus son.” 

More and more, I think CFers get a few more miracles than the average person; maybe it’s so they can share their unique stories, inspire the people who know and love them and leave their incredible marks on the world. 

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“More and more, I think CFers get a few more miracles than the average person; maybe it’s so they can share their unique stories, inspire the people who know and love them and leave their incredible marks on the world.”  – Marissa

CAN YOU SEE ME NOW

May 16, 2017 — 1 Comment

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You didn’t cause it. You can’t cure it. You can’t avoid it. You can’t ignore it. You can’t control it. You try to manage it while concealing it from the “normal world”.

Cystic Fibrosis (CF) is often called the invisible disease. Invisible illnesses are conditions patients have that are not obvious when looking at them. People with invisible illnesses face a lot of prejudices. Accused of faking, lying or exaggerating they are often misunderstood.

“Just because you cannot see a person’s illness doesn’t mean they don’t have one. Someone may look “OK” when they really are not. Invisible illnesses often have no cure with patients having to take medication for the rest of their lives to help control and manage their symptoms.”

Take a look at me for instance, coming from an outsider’s point of view. If you saw me walking down the street:

He was walking with a group of friends when I spied him. I noticed him immediately because of his short stature. At first, I thought what is this young boy doing hanging out with these older kids. The closer he came I realized that he wasn’t as young as I thought, in fact, he was mature and probably the same age as his friends.

They seemed very comfortable with one another laughing and joking as they sauntered along. He had a presence and seemed to have no problems keeping his stride even though his legs were much shorter than his friends. Little did I know at the time that he was a warrior; born with cystic fibrosis he challenged life on many levels.

Other than his size I had no idea that he was struggling just to breathe. Living with an invisible disease you quickly learn how to compensate. No one wants their disease to own them. As I watched them disappear down the road an old lyric came to mind.

You can’t judge one by looking at the other.

You can’t judge a book by looking at the cover.

Oh can’t you see, oh well you misjudged me.

I look like a boy, but I have C.F. undercover.

You can’t judge a book by looking at the cover.

There’s a story behind every person, a reason why they are the way they are. Think about that before you judge someone. There have been times that people have doubted me when I was not well. I’ve been told, “you look alright to me, what’s wrong with you? What’s your problem?” What they don’t understand is that they’ve got it all backward; I am not well, rather I am faking being well.

Before my lung transplant, I frequently had chronic chest pain caused by excessive coughing. Coughing was my body’s natural mechanism to try and rid my clogged lungs and airways of thick sticky mucus. Coughing helped to relieve the tightness living inside my chest. At times it felt like as if a slippery serpent was wrapping its scaly body around my lungs, strangulating and constricting my breaths. The wheezing that would escape from my lungs would be a hiss of a rattle.

The cough woke a fiery dragon, burning the lining of my lungs. If that wasn’t enough to suffer through, my stomach muscles would feel like a punching bag. The sheer pressure of coughing weighted my shoulders down and caused a deep stabbing ache between my shoulder blades. This is how I would start my day, in constant pain. I hid in the pain, showing no outward signs of the torture I was battling. The physical pain was hardwired into my system; I simply faked it pretending to be well all the time.

I appear as I want you to see me. I paint on my ‘I’m OK’ face every morning before my day and walk around with a smile, but the truth is that I am in chronic pain, always struggling, but you will never see that because I do not want to appear in that light. You may believe I am faking being sick, but I am actually faking being well, and people need to not be as quick to judge.

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The only person who could detect I was getting an infection was my Mom. She could see through my smoke screen. She by-passed the “I am fine mom, don’t worry” or “No, we don’t need to waste our time going into clinic the team isn’t going to find anything.” Her radar was incredibly accurate; before I knew it I would be in the car heading for the hospital to be admitted for a two-week course of heavy duty intravenous antibiotics.

With breathing restrictions, physical activities weren’t always easy for me.

There have been times when I needed oxygen 24/7. The sheer act of walking was a major task. I used my disability parking permit when I was out. Several times I returned to find handwritten notes tucked into my windshield stating that I abused the system. Nasty little sentences slamming me for misusing a disabled parking space. Why would someone as young and healthy as me require a disabled parking pass? In fact, I remember once coming back to my car only to find a note which read: “Someone in their prime age, has no use to abuse a benefit like that. You should be ashamed of yourself”.

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Many of those living with an invisible disease’s can relate with this experience;  even though you know you’re sick, you know as soon as you step out that people will see your lack of a wheelchair or crutches and do a double take of the handicapped spot you’ve parked in.

Yet the truth is that about 96% of disabled people have what are called an invisible illness. Because they’re invisible, people assume we’re faking all the time. The scariest thing is that many painful chronic illnesses go without acknowledgment or recognition from society, even disability circles. It can even get to the point where the illness controls that person’s life. When it’s such a major thing to that person, it’s not helpful when people dismiss it as a fabrication.

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“I didn’t realize disability had an age limit… someone should really tell my body that.”

Thinking on this topic, yet another painful memory floats up inside my head. I was learning how to drive. It was a hot humid summer night, my older brother Chris had taken me out for a lesson. We had stopped at a McDonald’s for ice-cream. Returning to my car I saw a woman make a gesture to her younger daughter regarding my size and the fact that I was driving. She openly pointed to me, motioning her hands to emphasize my small stature. While she was mocking me behind the wheel she was laughing. Not only did this make me feel disrespected it also made me mad. I secretly wanted to go over to the woman and tell her how insensitive she was. I wanted to educate her about CF and tell her how fortunate her daughter was to be healthy. But instead I got into my car and drove away. This memory sits with me. The worst thing you can do to a person with an invisible illness is, have them feel like they need to prove how sick they are. 

Depending on the mutation of the gene, Cystic Fibrosis can play a large part in children’s growth. Ciliated epithelial cells in the person that have a mutated protein lead to abnormally viscous mucus production. The poor growth in children typically presents as an inability to gain weight or height at the same rate as their peers and is occasionally not diagnosed until investigation is initiated for poor growth. The causes of growth failure are multifactorial and include lung infection, poor absorption of nutrients through the gastrointestinal tract, and increased metabolic demand due to chronic illness.

Nutritional failure in cystic fibrosis is multifactorial. Malabsorption of fat, protein, and fat-soluble vitamins is a result of insufficient production of pancreatic enzymes, which can be exacerbated by bile salt abnormalities in the presence of concurrent liver disease. Progressive pulmonary infection can lead to increased work of breathing, reduced appetite, and increased caloric needs from inflammatory catabolism. Other factors that affect nutrition include cystic fibrosis–related diabetes mellitus, altered motility of the gastrointestinal tract, and small bowel bacterial overgrowth.

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Pancreatic insufficiency results in malabsorption and maldigestion of nutrients and fat-soluble vitamins. In fact, cystic fibrosis derives its name from the cysts and fibrosis noted in the pancreas of patients with the disease. Pancreatic enzyme replacement therapy (PERT) and optimization of nutritional deficiencies can prevent growth failure and improve other outcomes in patients with Cystic Fibrosis, including quality of life, resistance to infection, and chronic lung disease, which can lead to longer life expectancy. The type of the genetic mutation causing cystic fibrosis determines whether a patient is pancreatic-sufficient or pancreatic-insufficient, although approximately 85% of patients are pancreatic-insufficient by the age of 1 to 2 years. Pancreatic sufficiency in the setting of Cystic Fibrosis is a risk factor for recurrent pancreatitis, and recurrent pancreatitis can often be a presentation for the diagnosis of cystic fibrosis

CF has me forever ensnared in a childlike body. Short stature, thin, I will be the eternal kid due to the mutation that prevented my growth. My body wasn’t getting enough nutrients at the time I hit my growth spurts and puberty. Being in my body isn’t always easy. Even though I am a grown man I struggle to live life as “normal” as possible. It’s made daily tasks a challenge as well I feel like I’ve missed out on parts of life. As I journey throughout life I’ve learned to adapt. Blend in and even though what seems like a simple task I have taken on the challenge to overcome it.

Living with an invisible illness, it’s difficult and often pointless to try to explain to someone who has no idea of the daily struggles you face. Feeling the pain, feeling unwell, feeling awful on the inside whilst looking perfectly fine on the outside is a hard act to keep up. Putting on a brave face for everyone wears you down. There are many forms of invisible illness other than CF. The invisible sufferers include Anxiety, Alzheimer’s, Chron’s, Depression, Diabetes, Epilepsy, Fibromyalgia, PTSD and many different kinds of Cancers.

Just because you can’t see it, doesn’t mean it doesn’t exist.

We learn not to talk about it! We’ve learned not to talk about our illnesses. Out fear out of disbelief, patronizing phrases or yet another unhelpful answer, and because we’re sick of being made to feel over-dramatic as if it’s all in our head.

We need someone who’s willing to listen. When I say listen, I mean really listen. Not give us answers to make us feel better at the time or daft answers that, while I’m sure are said to be helpful, only make the situation worse. We just want a friend who’s going to say ‘I’m here for you’ when we need them most

“Ask questions before you judge me. I’m happy to share my story and want to educate others. My challenges/pain is real so if you don’t understand, get to know me.”

The best thing you could say to someone with an Invisible illness is to tell them “you believe in them”. Believing is acknowledging you understand what they are going through. Today we have no reason to judge anyone as we don’t know what their story in life is. Judging a person does not define who they are. It defines who you are. So remember to keep an open mind.

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We may not look sick, but turn our bodies inside out and they would tell different stories.

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Life is A Card Game

March 22, 2016 — 2 Comments

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.

Recovering in ICU from second double lung transplant

Recovering in ICU from the second double lung transplant.

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. 

Sheba and I

A recent photo of Sheba and I out on the trail

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.

Life is a card game

Playing cards with Tanis in ICU

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.

Become an organ donor 

National Organ and Tissue Donation Awareness Week

Live Fearlessly

March 22, 2015 — 1 Comment

I am luckier than most people, even the ones who have won the lottery!

 

How many people can say they have received two double lung transplants?

 

My first transplant (March, 22nd 2002) and 8 years and a day earlier (March, 21st, 2010received my second transplantWhat are the odds of receiving two gifts of life a mere day apart?

 

I always celebrate both special days.  We call them “Lung Anniversaries”; however I tend to celebrate my first transplant in a more significant way. Without it, I would never have had the chance for a second transplant. Thirteen years ago my family and I were introduced to the world of organ transplantation.  

 

I will always remember the night I received my first set of lungs. I had been admitted to Sick Kids Hospital and wasn’t doing well; in fact, I was in what they call “end stage CF” (cystic fibrosis).  My lungs were so damaged my body was operating on a mere 10% lung function.  All I did was sleep and eat. It wasn’t much of a life; in fact, it wasn’t life at all. My family and friends would visit, I would struggle to stay awake; all I wanted to do was sleep to help keep my dwindling energy level up.

 

The night of March 21st, 2002 there was a very bad winter stormI wanted to see my family, but my sister Amy, my brother Matt and Dad couldn’t make it because of the weather. My Mom (was staying with me); my brother Chris and his girlfriend Kim (now wife) were visiting before the storm started. I still remember ordering a delicious salmon dinner in from a restaurant down the street. After we ate Chris and Kim headed back to Stouffville as the storm was intensifying.

 

Once they left my mom and I got ready for bed. My mom fell asleep on the couch and I in my hospital bed. Several hours later I heard someone talking outsides my hospital door. Thinking it was one of the nurses chatting with another I kind of ignored it and let my head sink back into my pillow.  Suddenly the orange glow of the hallway light grew brighter as the door opened.  It was like there was a large candle in the room.  I lay there thinking the nurses were coming in, to do their rounds. I quickly learned this wasn’t the case.

 

Two figures walked in, I could see their silhouettes through the hospital curtains.  They approached my mom who was sleeping on the couch. I could hear the soft voice of my transplant doctor Melinda Solomonas she gently woke my Mom up, “Tanis we have lungs for Dan”!

I remember my mom saying, Dan did you hear that, I answered Yes.

Instantly my brain was in overload, what would the next few hours bring, was I strong enough to survive the operation, would I still be alive tomorrow?

The doctor and nurses left the room to give my mom and I some time to talk as well to call our family. I couldn’t stop the fear building up inside me. A voice in my head was shouting that I couldn’t do this and I wasn’t going to survive. My mind was feeding my body with fear.Struggling to get hold of my fear I told my mom I didn’t think I was strong enough to survive the transplant and we should let the doctors give the lungs to someone else.  My mom looked me right into my eyes and said, “Dan you are going to be fine, I will be waiting for you on the other side of the operation”. It was her reassurance that gave me the strength to continue. There is a more in-depth story on my blog.  https://darkandstormyblog.wordpress.com/my-story/

 

 

At this point in my life, I had learned to become fearless. Shutting down that part of my mind where fears were created was now essential to my survival. I was facing real fear, fear that I had never met before. My choices were to panic or accept the situation and try to stay calm.  

 

Fear is contagious, it feeds our minds allowing our imagination to take over and play tricks to us.  Fear left to grow distorts the situation clouding our minds with negativity. It is this fear that prevents people from fulfilling their dreams and goals.  I looked through the fear that was building inside me that night and told myself I wanted to LIVE, I wanted to ENJOY LIFE, I wanted to SEE THE WORLD!

 

Without this operation I didn’t have a future, this was the closest chance I would ever have to continue living. I filled my fear with places I wanted to see; things I wanted to do, I wasn’t ready to give up. I became fearless allowing my life to become limitless as I believed in myself I knew I could do anything at that point.

 

When we were first looking into the possibilities of transplantation, the team of doctors and nurses told me they didn’t have a crystal ball, they couldn’t guarantee what would lay ahead for me. However, because of that team, I am alive thirteen years later!

 

In high school, I remember reading a textbook in science class about cystic fibrosis. My twin sister Amy was beside me as I pointed to the statistic that the average life expectancy of someone with cystic fibrosis was 16. I guess you could say I had turned a blind eye to that part of my disease. I always tried to live a normal (normal as possible) teenage life doing my treatments and taking my meds. In fact, I would impress my friends as to how many pills I could swallow at one time.

 

I knew we can’t live forever, as the band Cake so elegantly puts it, “As soon you’re born you start dying”. However at that age, you’re not thinking about death, you’re having fun with friends, making mistakes and learning from them.

A few weeks after reading that page from the textbook, I realized things could always be worse. This is when I came up with the hourglass theory. My theory is simple, at birth we are given an hourglass filled with sand, pebbles, some rocks as well a little hammer. It’s up to you to break down those pebbles and rocks into sand if you want to live longer. Even if it is challenging it is through your experiences that you’re making choices, creating memories, you’re living your life!

 

So I decided to live fearlessly. I could have just put the hammer down and let the top of that hourglass empty to the bottom. However, I chose to keep hammering away! Yes, I have had some bumps in the road that I have recovered from and still, am recovering from. In my journey, I have learned a lot about myself. Believing in myself has allowed me to be fearless, letting the world become limitless!

 

 

At Sick Kids ICU, my first transplant March, 22. 2002

National Organ and Tissue Donation Awareness Week