Like all mothers, all I hoped for while I was pregnant was to give birth to a happy, healthy baby. I am the mother of 4 children, 2 of which have been touched by Cystic Fibrosis (CF). Over the next few minutes, I would like to share with you the experiences I have encountered as I have watched 2 of my children, Rob & Karen battle this debilitating disease.
Rob’s life of battles began the day he was born with the discovery of a clubbed foot. Rob was the eldest of 4 children, with 2 younger brothers, James and Chris and sister, Karen.
His determination to overcome adversity began to emerge immediately. Even having surgery twice in his 1st year and a plaster cast from hip to toe didn’t stop him from crawling and walking within days of having the cast removed.
He began to define who he was at a tender age. Rob had a presence when he entered a room. He touched many lives and was adored by many for his compassion and generosity. A natural, spirited leader who was full of life, determination and mischievous charm.
As a youngster he underwent surgeries for tonsillitis, adenoids, and repeated tubes in his ears. Despite his ongoing illnesses, he loved and excelled at skate boarding, bike riding, and competitive hockey.
As Rob grew, his medical problems continued, but he never let them keep him down. Visits to the Pediatrician’s office became the norm. His Pediatrician diagnosed him with chronic asthma and his standard prescription was… “Take 2 puffs of this, and 2 puffs of that, then reduce it to 1 puff and come back in a week”.
At about the age of 12 when Rob’s “so called asthma” continued to worsen, I asked the Pediatrician if it was possible that Rob could have Cystic Fibrosis? I didn’t know much about CF – all I knew was that CF affects the lungs and causes difficulty breathing. The Pediatrician ordered a chest x-ray and said, “If you don’t hear back from me everything is okay”. You can only imagine how incredibly relieved we were when we didn’t hear back from the doctor because it meant that CF had been ruled out.
I continued researching and trying to find out what was causing Rob to be so sick. I asked for him to be hospitalized for testing and observation but my requests were dismissed and fell on deaf ears. Doctor’s visits for asthma, chest infections, and pneumonia became more and more frequent. By the time Rob was sixteen I was desperate for answers and had had it with the Pediatrician.
After much consideration, I took him to my family doctor who was a General Practitioner. Dr. Sharon Rubin was disturbed by Rob’s deteriorating condition and medical history. Rob was very skinny, pale, had a barrel chest and his fingers were clubbed. She ordered a battery of tests and referred him to a Respirologist. Six weeks later we were totally shocked and devastated to learn that Rob actually had Cystic Fibrosis! We truly thought CF had been ruled out several years earlier.
Most children with CF are diagnosed before their 1st birthday and without treatment the prognosis is not good. The damage to Rob’s lungs was already extensive – his lung function had already deteriorated to only 34%.
After Rob’s devastating diagnosis his 3 siblings were also tested. After 6 more agonizing weeks of waiting we received the news that Rob’s 9-year-old sister also had Cystic Fibrosis. For me, it was so unbelievably emotional and terrifying but I had to park my emotions to put on a brave, positive face for my children and family.
In Rob’s later teenage years and early 20’s, he endured numerous hospital stays.
Like most young men in their late teens and early 20s, Rob developed a love for cars - driving, racing, and mechanics. He was quite captivated by all things related to cars, really. His primary sources of entertainment became music, poker, movies, and cars.
As Rob’s condition worsened and his numerous hospital stays continued, it became evident that the one thing all of his interests had in common was that they were all activities he could enjoy without struggling to breathe.
For more than nine years, the CF medical team at St. Michael’s hospital treated Rob and worked to improve his quality of life. Then, at the age of 25 his lung function dropped to a dangerous low of 17%. His condition began to steadily decline and on February 25, 2005 Rob underwent a successful double lung transplant.
His recovery was remarkable and miraculous. Rob was elated to be able to breathe again.
As a mother, watching my son begin to experience and enjoy life was truly a miracle. I still struggle to put into words exactly what this felt like. Mothers rejoice when their babies take their 1st steps and youngsters ride a bike for the 1st time. I rejoiced when I saw Rob take a little jog down to the end of the driveway for the 1st time, just weeks after his transplant. It was so exciting to see him jump on a bike and go for a ride. All the simple things that people take for granted were so exciting to watch unfold after the transplant.
For the next five years he lived life to the fullest and made up for lost time. He loved life!!! He exercised vigorously, lifted weights, skated, socialized, laughed, danced, and did many of the things that Cystic Fibrosis had previously prevented him from doing.
In September 2009, after enjoying a weekend cruise on Lake Ontario with his friends, Rob began experiencing excruciating abdominal pain.
He was hospitalized but no official diagnosis was made. The agonizing pain persisted and he made several visits to walk in clinics, family doctor etc.… to no avail. Unfortunately, most doctors don’t know what to do with such a complex patient. The transplant centre only treats transplant related symptoms. With nowhere else to turn, Rob called the transplant centre and spoke to his transplant coordinator who initially determined his symptoms were not transplant related.
Several months later, in March 2010, we took Rob to St. Mike’s emerge to explore whether or not the escalating symptoms were CF related.
It was five years post transplant when devastation struck again.
Pancreatitis had been ruled out but Rob’s abdominal pain did not subside. It became apparent that his kidneys were shutting down. That was when he was transferred to Toronto General to further explore the symptoms from the transplant perspective.
He was put on dialysis while tests continued to determine the cause of the abdominal abnormalities. After many weeks of not knowing, the unexpected diagnosis was Post-Transplant Lymphoproliferative Disease (PTLD), also known as a Hodgkin's Lymphoma. Rob had Cancer. PTLD is condition associated with Epstein-Barr virus (EBV) or Mononucleosis (Mono). Transplant doctors monitor patients closely during the 1st year post transplant because that’s when most cases of PTLD occur but Rob’s didn’t surface until five years after his double lung transplant. Needless to say, we were all shocked by the latest heart wrenching diagnosis but knew we could face the struggle as a family.
To treat the Hodgkin's Lymphoma Cancer, Rob was taken off all of his anti-rejection medications and aggressive chemotherapy was started. Although Rob’s condition was rare, the doctor’s at Toronto General Hospital told us it was well documented that anti-rejection medications could not be taken while undergoing Chemo Therapy.
Rob was fearful of giving up his anti-rejection drugs but put his trust in the doctors who assured him that organ rejection wouldn’t happen because the Chemo would keep his immune system suppressed.
Rob endured the rigors of chemotherapy and ultimately fought and conquered cancer. While Rob was able to survive the cancer, we soon learned that his new lungs took a terrible beating. His lungs started rejecting – rapidly.
In September 2010 Rob was hospitalized as it was becoming apparent that his lungs had been compromised from harsh chemotherapy treatments. He was treated with IV meds for a few weeks but there didn’t seem to be any improvement. By October, Rob was fearful of what was happening to him. He spent most of days resting in his room, trying to reserve his strength and coming out only occasionally.
Rob had become bed-ridden and over the next several months it became very clear that he would need a second double lung transplant. If this wasn’t daunting enough, one of the conditions of but being put back on the transplant list was that Rob had to be cancer free for one full year.
In April 2011, Rob was hospitalized again for several weeks. Family members took turns and shifts staying overnight in the hospital with him. He was completely helpless, scared and could not be left alone. We were his advocates. We were his voice. By May and June 2011 he would only come out of his room only to use the bathroom which was a huge, exhausting ordeal for him.
Every day of his last year was a struggle both physically and emotionally. His lungs continued to deteriorate, but he never gave up and never complained. Despite his incredibly weak body, Rob was still determined to get through another transplant. After his first transplant he got a taste of the good life. He wanted this chance again so he could start living and embracing life again. My son wanted nothing more than to do what we all take for granted… he simply wanted to be able to breathe.
Although, he was getting weaker he continued to focus on plans for his future. He saw himself working with young people as a counsellor. He wanted to give back by providing support to others in need, such as the sick or troubled youth.
On Labour Day Monday of 2011, Rob had a very simple meal request. He wanted oatmeal. Even eating was exhausting and difficult for him. This time was especially difficult. Within minutes of eating his breakfast, he started desperately calling to have his oxygen turned up to the highest level. We quickly made the adjustment, but he was still in distress and panicking – no amount of oxygen was enough. In the seconds and moments to follow, we watched helplessly as the oxygen was not enough and Rob appeared to be suffocating.
I then did what any parent in my situation would do. As my hands and voice quivered, I called 911 and the Paramedics arrived. They worked on him in his room and eventually convinced him that he needed to go to the hospital. We asked the Paramedics to transport him to Toronto General where organ transplant experts are, but they had to take him to the nearest hospital – St. Catharines General.
Dr. Vasan in Intensive Care collaborated by phone with Dr. Singer - Respirologist at Toronto General Hospital and it was decided that Rob would need to be sedated and placed on a mechanical ventilator until he could be transplanted. He would be put into a medically induced coma. Dr. Vasan was brutally honest and direct. He explained to Rob that without the assistance of a respirator he could just close his eyes, go to sleep and never wake up. He also cautioned that even on a ventilator, there was still a chance he might never wake up.
The fear and emotion was unbearable for all of us. Rob asked why he couldn't just continue to try and breathe on his own. I gently re-iterated what the doctor had said, there was no other choice really. He couldn’t breathe on his own anymore and he needed the assistance of a ventilator to breathe for him. The Doctor asked Rob if he understood. Rob nodded and hung his head. I tried my best to remain composed and show that we believed there was still hope. I was Rob's lifeline. If I were to fall apart he might think it was over and I couldn’t let that happen.
He was so fragile. I tried my best to reassure him that he would wake up in a couple of months with brand new lungs. I softly squeezed him and told him, I loved him. He told me and my husband Gary that he loved us both and reluctantly agreed to go to sleep and be ventilated.
Before being intubated, he looked up at me and firmly said “I’m doing this for you”.
I turned away from Rob and towards Gary as the tears started to gush. Gary held me and quietly said, “stop crying. Rob can see you”.
I stopped and Rob said, “Say it mom”.
I knew what he wanted but I said, “Say what Rob?”
“You know mom, say it! What you said to me when I was going for my first transplant”.
“You mean what I used to say to you when you played hockey?”
“Yeah mom, say it”.
“Ok Rob… Knock em dead”.
Dr. Vasan asked us to leave the room while Rob was being intubated.
Rob had told me many, many times over the years not to cry because I had to be strong for him. It was a very tall order to ask of a mom. It was impossible not to cry, but over time I learned to do most of my crying alone and out of sight. I had to stay strong for Rob and the rest of the family.
That week in St. Catharines intensive care unit, was a tumultuous emotional roller coaster ride. Finally a bed became available at the Toronto General Transplant Centre and arrangements were made for Rob to be air lifted. His vitals kept crashing whenever hospital staff and Paramedics tried to move him. The Air Ambulance Paramedics were afraid to transport him because they were fearful that he might not survive the trip. To assist, Intensive Care Doctor, Melanie Hollidge, went above and beyond her duties as a medical doctor. She took the unheard of and unusual step to board the helicopter and fly alongside Rob on the journey to Toronto. (To this day, we are eternally grateful to Dr. Melanie Hollidge. I still don't know how she got back to her post in St. Catharines.)
Gary, my daughter Karen, and I drove the one-and-a-half-hour trip and watched while the helicopter landed on the roof at Toronto General Hospital. Rob not only survived the flight, but his vitals had actually improved.
Rob had always been somewhat of a thrill seeker so we wondered if he was aware that he was taking his first helicopter ride and that’s why his condition somewhat improved.
The TGH transplant team, who are used to dealing with the worst of the worse case scenarios, gave us renewed hope. They seemed very confident and optimistic that Rob would pull through, however his carbon dioxide levels continued to be a major concern. It was decided that Rob would undergo surgery for an artificial lung (Novalung) to act as a bridge to get him through to the transplant.
Before undergoing surgery for the artificial lung, it was imperative that Rob was still cancer free. If there were any indication of cancer at all what so ever, he would not receive the Novalung. A CT scan was done and we were more than relieved when results confirmed there was no cancer. Rob was on his way to a rare surgery to receive the artificial lung. We had renewed hope again. Immediately after the surgery, he was officially put back on the transplant list for a 2nd double lung transplant. Hope was returning and growing.
The plan was for him to remain sedated on the ventilator, with the artificial Novalung and then be weaned off so he would be awake before the transplant. He was still considered extremely critical and keeping him stable was an on going process.
To our dismay, after the Novalung surgery, Rob started bleeding internally from the groin and abdomen area. Now he would need further surgery to stop the bleeding and to surgically remove the Novalung from the groin area and reinsert it into the neck area. Sadly, even after the 2nd surgery the bleeding would not stop. A team of specialists discussed every possible scenario to get him back for a 3rd surgery to stop the bleeding but every attempt to move him caused his vitals to crash to extremely dangerous levels.
What Matters in the End
An emergency transplant team meeting was held on the morning of September 16, 2011 to discuss all possible options to save Rob's young life.
I was in my car, on my way to Toronto General hospital, when I received the call on my cell phone from a hospital board room. I pulled over and listened quietly and intently. They put me on speaker phone so I could listen to a team of doctors who explained every possible course of action:
1) Take Rob to the OR to operate to stop the internal bleeding. However because his vitals crashed every time they tried to move him they were sure he wouldn’t make it to the operating room. He would surely die on his way to the operating room.
2) Operate on him in his ICU room. They explained this couldn’t be done because they wouldn’t have access to vital equipment which is only available in the Operating room
3) Wait to see if the bleeding would stop on its own
They kept asking if I understood. I asked if they expected me to choose from the options presented. They said no. They would never expect me to make such a difficult decision.
They decided on option 3. The only thing to do was wait and see if the bleeding would stop on its own. In my mind this meant that there was still a chance the bleeding would stop.
I had heard from others that people who are in a coma can hear all that is going on around them, so when I arrived at the hospital, I spoke to Rob and told him to hang in there while I prayed for the bleeding to stop.
Dr. Ceila Cheparo, who had overseen Rob since before his 1st transplant, was there in Rob's ICU room when I arrived that day. She and I hugged each other and cried. It wasn’t until Dr. Cheparo said, “I never thought this would happen” that reality started to sink in. Hope was quickly dissipating and I was starting to realize that option number 3 was their way of breaking it to me gently.
Dr. Cheparo urged me to call Karen to come to the hospital as soon as possible. I said that Karen would come after work but Dr. Cheparo insisted that I call her to come now. Dr. Cheparo and the other doctors knew the bleeding was not going to stop on its own. Rob was going to die.
James and many family members were already there. We called Chris, the rest of my sisters and Rob’s cousins to come too.
Another meeting was held with all of the many family that were there at the hospital that evening. Dr. Downer along side Dr. Cheparo explained that the bleeding still had not stopped. They encouraged us to talk and share memories about Rob. In a round about way they were asking us to decide on the best way for Rob to die. Now there were only two options. Gently remove him from life support, one machine at a time, to which they assured us he would not suffer. Or leave him on life support and wait for something catastrophic to happen. It all seemed so surreal. Surely, this couldn't really be happening.
After that meeting, we reluctantly called for the in-house Catholic Priest to administer the Last Rites and anointing of the sick. I say reluctantly because if Rob could in fact hear what was going on he would surely think I, the person who gave him life, the person he counted on for everything, his rock, his lifeline, had given up on him. It was shear torture. There are no words to express the depth of this kind of internal conflict and pain. Even the young priest shed tears as he administered Rob's last sacrament.
Rob’s brothers, sister, parents, and many other family members surrounded and held him as the hospital staff slowly turned off one machine at a time. The last gurgle in from ventilator tube was extremely disturbing – a sound I don’t think I will ever be able to erase from my memory.
After we left Rob’s room a doctor asked me if I wanted Rob to be autopsied. I said, “I think we know what he died from but if an autopsy will help save other lives by all means”. Slowly family members dissipated from the hospital. Rob’s youngest brother Chris waited for the nurses to clean and remove all the tubes and stayed behind with Rob's body. Gary, Karen and I solemnly left the hospital together.
Over the years Rob fought and won so many battles despite near impossible odds. Many had come to see Rob as a miracle, and never thought for a moment that his time to leave this earth would come anytime soon. Sadly, despite his determination and the best efforts of the world’s top doctors and specialists, Rob’s fragile body could no longer house his wonderful spirit.
Rob could no longer keep up the fight and his courageous battle came to an end in the late evening of September 16, 2011.
Rob loved his family deeply and our love for him kept him believing and hoping. What a GIFT Rob was to all of us, to everyone who had the honour of knowing him.
Rob’s great inspiration and courageous spirit lives forever in our hearts.
We are indebted and so very thankful for the immense help from our community, research teams and all the people who generously donate their time and money to the CF cause.
We will continue to fight the fight for my daughter Karen and all the other young people struggling with this disease.
In Karen’s words “Living in this world without my brother has been very scary. Death is real. It really happens. I suffered horrendous grief, depression, and anxiety which was only contributing more to my own illness... I needed to allow myself to pull myself together. Rob would have wanted that.”
“Knowing that I have the same disease that killed my brother, my rock, causes real fear and anxiety. I constantly worry. I'm scared that things may one day progress. I am extremely cautious and aware of my body and any changes. I am acutely aware of all who are sick around me. If a friend or family member is in hospital, I need to weigh the benefits and risks of visiting them. When I am feeling tired, tight chested and might be coming down with something I stress over whether or not I should disappoint and cancel plans with my friends. Should I push myself and risk developing a chest infection and a potential hospital stay? I have to make conscientious choices in terms of where I go and what I do. I can't just go with the flow and decide it's too late to drive home after visiting family/friends... I have to make sure I'm home to take my medications. I am a germophobe. I'm terrified of catching a bug from someone. I'm constantly washing my hands. I'm fanatical about living in a clean environment. We constantly clean to ensure my environment is absent of potential harmful bacteria and allergens. I learned this from an early age. All of this can become physically and emotionally exhausting. There are so many factors I need to consider - so much to remember and incorporate throughout my day.”
Until the day that a cure is found, Karen vows to fight her battle with Cystic Fibrosis not only for her, but also for her brother.
Karen’s mission… “I always say that Rob fought for the both of us... And I'm going to live for the both of us.”