Aug 5, 2005 – 26 Years Old
Ian, my oldest, had noticeably been dragging for a while. He had been excessively sleeping and complained of a bloated stomach. I must have been concerned because I remember approaching him only to find he pushed back by getting snarly and demanding to be left alone. It was the stomach issue that prompted him to go to our family doctor. Our doctor was the same doctor who had delivered Ian 26 years before. Ian’s birth had been a natural one with no meds, no IV and no complications. Dr. Keltz listened to Ian’s complaints, checked him over and took blood as part of the examination.
Ian may have put off this doctor’s appointment, however he had witnessed a neighbour complain of his own stomach ache, ignored it for a while only to be diagnosed with stomach cancer. In a few short months, this burly man lost 60% of his body weight and then passed away. It was the memory of a neighbour’s inattentiveness to his own symptoms which spurred Ian to get to the Doctor.
Our family doctor reached Ian at work the next morning and instructed him to go to the hospital emergency immediately. The only information we had at that point was that his blood counts were dangerously low.
Day 1: I met Ian at North York General Hospital in the emergency waiting area. They had taken his first round of information but hadn’t yet put him through the triage process. Once admitted into Emergency, he got a blood transfusion which perked him up for a short while. Our wait continued; Ian dozed on and off all day. By 11:00 pm, 13 hours later, we were still in emergency and with no diagnosis. The lights were being dimmed encouraging waiting patients to sleep. I went home to get some sleep myself. After I left, Ian was allowed to go to the toilet by himself, with his low blood counts he passed out on the way back and fell to the floor.
Day 2: Next morning I returned to the hospital and nothing seemed to have changed. Ian’s night had been restless. After his fall they were not letting him get up off the bed and had inserted a catheter. The energy boost from the blood transfusion had long worn off. He was tired, frustrated and not feeling well at all. I couldn’t understand why we were still in emergency. There was something obviously wrong, but what?
Mid-afternoon was when they started talking to us about TTP. By this time, Ian was floating in and out of consciousness. I was numb. Neither one of us grasped fully or understood the ramifications of what we were being told, nor had we ever heard of TTP. Thankfully, there was a plan: he would be sent down to Toronto General Hospital via ambulance and they would treat him there.
Funny how clearly you remember unimportant things and forget the important ones. In crisis your memory becomes fragmented. The body truly moves into shock. Things that mean nothing to you stick out. The ambulance team, who took Ian and me down to Toronto General, were a male-female team. He was wonderful. She was not. There was some disagreement that clearly had started before they picked us up. She said nothing to us at all and spoke in clipped tones to her partner. I was relieved she had taken the position of driver up front and was not in the back with us. I can’t remember how I got home that night. I can’t remember how I broke the news to Ian’s three siblings. I can’t remember the conversation with my own dear parents. Yet, I can still remember clearly this sour pinched miserable face of a stranger.
The ambulance ride down was thankfully uneventful. In the half-light, the paramedic and I spoke in hushed tones. Ian was quiet as he drifted in and out of consciousness with us on either side of his stretcher.
At Toronto General, the paperwork had already been filled out. Registration gave Ian a room number and up we went. By now it was after 8pm.
His assigned hospital room was a normal 2-bed one. Both beds were empty, and a large machine with dials and lots of tubes was between the beds. A nurse was sitting quietly in a chair reading. She stood up and greeted Ian with a welcoming smile and warmth in her voice. “Hello Ian, There you are. I’m Chelo. I’ve been waiting for you ALL day.” I started to feel lighter. It was the first time since all of this started that I had the sense that everything was going to be all right. We were not alone in this. Chelo hooked Ian up to the machine and started the first of so many lifesaving plasmapheresis treatments he was yet to have.
Chelo was our first contact with the Plasmapheresis Team. They are a group of dedicated passionate individuals who worked from a busy room on the 7th Floor of Princess Margaret. TTP is only one of the conditions that they treat.
While I felt nothing was happening in emergency at NYGH, lots had been going on. North York General Hospital had been in contact with the Plasmapheresis Team at Princess Margaret Hospital, who had been distance monitoring Ian all day. Some of the time delay had been that the team were insisting that he be admitted and given a hospital bed. Toronto General Hospital and Princess Margaret Hospital are across the road from each other on University Avenue. Connected by an underground tunnel, they are both part of the University Health Network.
At the time, I didn’t know how lucky we were. We had early diagnosis and access to treatment on our side. First, Ian had actually gone to the doctor. He was into treatment less than 3 days from his visit to the family doctor. Had he left it much longer, his platelets would have kept falling and his organs would have gone into distress. Second, the doctor on call was the wife of Dr. David Barth, Head of Hematology at Princess Margaret Hospital. She knew of TTP and had quickly zeroed in on identifying it. Possibly had she not been on call when we first went in, Ian may not have been given that first blood transfusion. Since TTP affects 3 people in one million, most doctors would not have seen TTP symptoms first hand. Like so many others, we could have gone through a number of alternative diagnoses before they arrived at TTP.
Ian is one of the 45% of TTP patients with Idiopathic or Acquired TTP, where there is no defined cause. Since his original onset of TTP in 2005, he has had 4 relapses: April 2008, Two one on top of each other in February 2009 and another in March 2010.
By the 5th TTP bout, which would be Ian’s 4th relapse, we had the system down pat in terms of what needed to be done. When the early onset of symptoms would occur, Ian would go either to emergency at Toronto General or the plasmapheresis Unit at Princess Margaret Hospital to have his blood tested. The treatments would start and last about 2+ weeks in the hospital and he would then continue on as an outpatient, until his blood returned to normal and counts became stabilized. Treatment consisted of plasmapheresis, prednisone, Benadryl, calcium replacement and in one relapse, a chemotherapy drug called Rituximab.
The Fourth Relapse in 2010 was a disaster. It came on top of his third relapse. The outpatient plasmapheresis treatments weren’t holding; he would have a treatment, feel better for a bit, only to find his counts kept going down.
I would drive Ian to the Downsview subway station. From there he had a straight run right down to the hospital. Puffed up from the prednisone, depressed, listless and unkempt from lack of energy, bruises and needle marks on his arm from treatments and a white patch on his neck covering his central line, he looked awful. He looked so bad that on the subway people would change seats to get away from him.
Treatment wasn’t working this time. His counts weren’t holding. I dreaded the weekends because there were extra days between treatments, which meant Ian would sink even lower. It was the first time I had ever thought that we might lose him.
Dr. Barth recommended a splenectomy. Removal of a spleen is one of the treatments for severe blood disorders that aren’t responding to conventional treatment. He posed it first as if it were a choice. When Ian was still thinking about it, Dr. Barth pulled both Ian and I in for a serious talk about Ian’s TTP history and options. I am grateful for his truthfulness and attention to what was best for Ian.
The splenectomy, done laparoscopically, seemed to do the trick. Ian’s blood counts started to improve. The operation for Ian was a difficult one. He had a crisis episode several hours after the operation where he started to bleed internally. Removal of Ian’s spleen means there are some things that he needs to be careful about: Yearly flu shots are mandatory, he needs to watch his temperature and avoid catching bacterial infections.
Now 2017, It’s been 12 years since Ian’s first TTP onset, and 7 years since his last relapse. While he no longer has TTP, the possibility of a relapse hangs over him. Every bruise is a mini-crisis. If he can’t remember how he bruised himself, internal panic begins as he waits for other symptoms to show up.
With Ian’s first bout in 2005, we turned to the internet for answers. Information on TTP was limited. What came up was a UK site and Wikipedia that gave brief descriptions. Next we waded through scientific publications. Not much there was of value because samples were small and conclusions were vague.
Answering TTP Foundation is a godsend to those who are touched by TTP. I am appreciative of Sydney Kodatsky’s drive and the work she has done in creating Answering TTP. I marvel how some people face a crisis by standing up and making a difference while others fold. Sydney has made it easier for those now afflicted by TTP to get answers as well as educating those on the front lines about TTP.
Our family has upped their donations to the Canadian Blood Bank. Interestingly, Ian’s paternal grandmother Helen Wilson was an active volunteer for the Canadian Blood bank for many years. Her best friend Eileen Lewis, a small woman of 100 lbs reached the 100 donations mark. These blood donations are lifesaving for TTP patients. The patient’s blood is filtered; plasma is separated out and replaced by donated plasma. In the treatment, the patient is given Benadryl, hedging the possibility that there might be something in the donation plasma that would trigger an allergic reaction. In all of the treatments that Ian received, I remember only one that he had a severe allergic reaction to.
Ian is active with the Answering TTP website https://www.answeringttp.org/ in development and backend programming. Our family continues to attend Answering TTP fundraising events where possible.
Going through this experience of TTP with Ian has been a challenge for all of us – most of all for Ian. I don’t know what the future will bring for Ian. My hope is that TTP is now well behind him. So far, we are looking good.
Thoughts & Reflections:
TTP is life threatening. Early diagnosis is crucial. Early warning signs of TTP are varied for each person as well for their relapses. Not all symptoms present themselves; Ian’s trigger for his first onset was a bloated stomach and feeling tired.
For us there was no group support program for TTP. We managed to access the Healing Journey Cancer Support Program developed by Dr. Alastair Cunningham. While TTP is not Cancer, the program was very helpful. You can find recordings and the worksheets online at http://www.healingjourney.ca/. Dr. Cunningham stressed that if we got nothing else out of the program, meditation and journaling were key. I would also like to add cognitive behavioral therapy is a wonderful method of helping to normalize life and aid with coping skills. Answering TTP is a wonderful resource.
It’s not only the person in the hospital that is affected, family and friends are also impacted. I found people’s response’s interesting, difficult and also diverse. Ian’s siblings had different reactions. One jumped in and helped with his hospital needs, another couldn’t cope with going to the hospital, the third carried on as though nothing was going on. Relatives and friends, while expressing concern and sympathy didn’t know what to say or do. A dear friend when I shared with him the hopelessness I was feeling on Ian’s 4th relapse, told me to suck it up and change my attitude. My sister and my mother, both quietly wrote generous checks to help with extra expenses. My other sister and her husband stepped in and took over the care of Ian’s younger sister.
Each of us copes differently with crisis. I’ve learned not to judge the behaviour or statements of others. The most inane things come out of people’s mouths, which can throw you off. Yet some of the most heartfelt caring support comes forward in times of crisis. These messages from the heart are what I choose to remember.
By 2005, Ian’s first bout with TTP, I had been a single mother of four for twelve years. Their father was MIA, by his own choice for whatever reasons. He did visit Ian a few times. Regretfully, his firstborn son being seriously ill was not a game changer. A parent’s lack of attention to his sick child or any child no matter what age is hurtful and unkind. Ultimately everybody loses. If you are reading this and are not harmoniously getting along your child’s other parent, or other family members, please stop for a minute and think, what really matters? What kind of legacy do you want to leave behind? When in doubt, take the high road. It’s not always the easy path, but most always is the one with greater return. No matter how old we are, we still need our mom, dad and family to be there for us. Life is far too short to be not getting along. Crisis can be a chance to bring a family closer together to support each other in ways that strengthen each and all of us.
There is a balance between giving the TTP patient space and being involved. There were things going on with Ian that I wasn’t aware of. He was trying to navigate his own troubled waters and hid things from us. I wasn’t aware of how concerned he was of the aphasia he was experiencing because of the TTP. I wasn’t aware that he was worried that he felt there was a point of no return where they would give up and refuse treatment. Practical, sensible things that I would suggest to him, he would say no to. Later he would speak up and say he should have done that. He hated being the “Sick Guy”. People would focus on his illness in conversation.
It’s important to connect with the public support system that is available as soon as possible. Get a social worker lined up at the hospital to make sure you can access all the support social programs, financial, emotional and other options that are available to you. These people know what is available and want to help. Make the move before you feel you need anything.
TTP is a relatively unknown condition and doesn’t fit the normal system of illness. The ‘support systems’ and those who work them understand chronic illness. Someone gets sick and they stay sick. TTP doesn’t fall under that category. You have TTP, then you don’t. The fallout effects of TTP or possible relapses aren’t considered. As such, you may have to work a little harder to get what you need – or fill out more forms. Better to do it earlier than later. We lagged on this. The first time, by the time we started to access resources, Ian’s blood was normal.
Once the blood is back to normal doesn’t mean the TTP patient is. The mental anguish doesn’t go away. Each bruise, red spot, tiredness and bad dream brings the possibility of relapse to the forefront and new worries begin. Baring complications, it was a good year before Ian had his strength back.
Make sure your TTP patient has an advocate. This could be you or someone else. Check to see what treatments are being given. Speak up if you think something is wrong. If you are overwhelmed, get someone else to share the load or take the lead. Encourage the patient to pay attention to the people who are administering care and what is going on. By the 2nd relapse, Ian had become his own advocate. During Ian’s hospital stays there were a number of crisis points (calcium falling to low levels in his system during a plasmapheresis treatment, internal bleeding after surgery, a nurse starting to hang a 2nd drip bag when it wasn’t ordered).
Overall, I have nothing but praise for the health care system here in Toronto. Doctors, nurses and support staff were attentive, supportive, professional and communicative. I feel lucky that these amazing people in the UHN system were there for Ian when he most needed them.
Communicate to family and friends about visitation and phone calls. At the beginning of treatments and low counts Ian would want to sleep. A constantly ringing phone by his bed was not welcome. When he started to feel better, he welcomed visitors. The visits helped cheer him up and let him know that people were there for him. It helped to have a bulk email going out on a regular basis to update people on Ian’s condition.
Family is wonderful. One of many funny stories was, his Uncle Rob gave him a book on his first bout with TTP. It was Bill Bryson’s book, A Short History of Nearly Everything. With enthusiasm, Uncle Rob told Ian he had heard it was a good book and he had plans to read it himself. Déjà vu, 5 years later, doesn’t Uncle Rob show up with the same book and the same explanation. He had heard the book was good and had plans to read it himself. Ian and Rob both had a good laugh. Humour is a wonderful gift to bring to those who are affected by TTP.
Before I close off, I want to mention Hope. Hope is not to be ignored and discounted. Being hopeful can enhance your mood, lessen pain and lighten the load. It is a vital tool for healing. Hope costs nothing. Best of all, hope has no side effects. Like prayer, hope helps set intention and intention becomes the precursor to action and then results. Hope is the solid ground to stand on in the moments when you have nothing else. Hope generates a light that shines to lead you through the darkness.