Category Archives: Thalassemia

In Memoriam…

I remember how I felt when they told me that I was pregnant. My first reaction was shock. I had been through a year and a half of pure hell: the gut wrenching pain, the rollercoaster of emotions, the bad reactions to the super hormone injections, the tearful taxi rides to the hospital and then back to work as if nothing had happened. My body reacted horribly to the treatments each time, and each time a treatment failed, I swore it would be the last time I tried. I was beyond my breaking point many, many times, at times even suicidal. The added stress of being off my Thalassemia meds and knowing that my iron levels were rising while I continued to receive blood in order to stay alive was enough to make sleepless, tearful nights the norm.

My husband Jim stood by my decision to try for a baby, even though it was painful for him to see me like that. I knew that I would always regret it if I did not try to achieve something that I wanted so badly. I had grown up believing that I could do anything that I wanted to do. And I did. I went to university, I got married, I worked hard at following the medical treatments, sometimes painful and exhausting, that were necessary to keep me alive and healthy.

Of course, living with a chronic illness, Thalassemia Major, makes having children extremely complicated. With the current generation of Thalassemia patients, women are actually living long enough to get married and have children for the first time. This was never the case before, and so this is all new territory. Some women have attempted it and have succeeded, but of course, every person is different, and the level of reproductive tissue damage varies from patient to patient.

Jim kept telling me that it was a miracle that I was even alive, and that he would be thrilled if he could just have me for life. The thought that the medical treatments and potential pregnancy could cause my health to fail, and that he could eventually lose me, terrified him.

I couldn’t even allow myself to hope that any of this would work. I knew it would crush me if it didn’t, and I was trying to prepare myself for the worst. And then, one evening at work last Fall, the nurse called and told me that my blood test was positive. I was pregnant.

Jim and I were shocked. The odds that this would actually work had been so slight. We went out to supper that night and were in such utter disbelief that we mostly just stared at each other, speechless.

I told a few close friends and family the news, and they were thrilled. I was cautious. I was extremely nervous about the upcoming ultrasound, though I told myself that I was probably overreacting, that the biggest hurdle had been overcome. Sometime within that two week waiting period, I actually let myself feel a bit hopeful and excited about what was to come.

At the ultrasound appointment, the technologist bluntly announced to Jim and I that the growth of the embryo had stopped, and so I was given a prescription to induce a miscarriage. We weren’t at all prepared to hear that, and were both shocked at the news. I cried so hard that I could barely see. We saw our doctor afterward and he told us to wait an extra week, just in case.

A week later, the situation was the same. We were then told to wait yet another week. It was like an act of deliberate cruelty. We both felt that we were going to go insane waiting. I scheduled an extra blood transfusion to boost my hemoglobin level, continued on my pregnancy diet, and continued taking the hormones that made me feel physically ill and depressed. I knew there was very little chance that the pregnancy was viable, but even if there was only a 1% chance, I had to do everything that I could.

Finally, after the third ultrasound, we were told that the pregnancy, at two months, was definitely non-viable. I could stop the hormones and wait to have the miscarriage naturally.

I was living a nightmare. One evening at work, my body started hurting all over. I felt extremely sore and weak, and my best friend, who luckily works with me, sent me home in a taxi and I went straight to bed. The miscarriage started the next day and continued for a few days after that. And then it was over.

Except that it isn’t. Six months later, the thought of what could have been still hurts. Six months later, I still love the potential life that could have been our son or our daughter.

My Hands

wedding_henna_designMy hands create, comfort, arrange, wash, wave, steer, nurture, type, play the zils. They fall into stylistic form when dancing bellydance or indian fusion. They allow me to navigate the Internet, click on what I like, and serve coffee to my friends.

I often fantasize about having hands with no scars.

The veins on my hands are pierced every three weeks so that I can receive blood. These veins have been used since I was six months old and they have been tired for a long time. The surfaces of my hands are scarred as are the veins themselves.

There are days when my veins are impenetrable, when the nurses have to push the needle against my skin so hard and it is so painful that I feel like I am screaming inside. And then there are days when the needle slides in easily, as if my skin is made of butter. There are days where I feel like a human pincushion, where two or three nurses take turns trying to get my vein, and I just want to cry, give up and go home. On other days I laugh and tell the nurses stories even as they start my IV…those are the good days.

Sometimes the needle goes in too deep, puncturing the vein on both sides, and the vein is blown.

When I was a teenager, I realized how ugly the scars on the backs of my hands were and took great pains to hide them. Today, I show my scars to good friends.

My sister always said that I was lucky to have hands like a piano player, long and slender. When I would come home from transfusion, she would love that my hands were so warm. My husband endlessly compliments my hands and says that they are elegant and beautiful. When I come home from transfusion, he asks where the infusion took place and  kisses my sore hands and wrists.

There’s a comfort in using the same veins for every blood transfusion, just as there is a comfort in receiving your transfusions at the same hospital and having the same nurses start your IV. It’s not a pleasant experience, but every bit helps.

I often try to imagine what my hands would look like with no scars, no history, smooth and even. They would be unrecognizable, not my own.

 

Five Hundred and Thirty One

I remember the first time I came face to face with the people who were literally keeping me alive.

I’ve had the opportunity to speak at two Héma-Québec engagements in the last three years. The first was at a thank-you dinner for the volunteers who set up and run the blood drives that are held around the city of Montreal year-round. The second speaking engagement was a dinner to thank blood donors who had donated twenty-five, fifty, and even a hundred times or more.

The sense of gratitude I felt at these events was overwhelming. After all, when I receive blood every three weeks, all I see is a unit of blood with a tracking number on it. Obviously, I know where the blood comes from, but actually seeing the faces of these donors really brought the point home. Meeting the volunteers who devote their time and energy was humbling. I was given the opportunity to thank each one of them in person on both occasions.

Here’s the speech I gave on both occasions:

My first transfusion was at the age of six months, shortly after being diagnosed with Thalassemia at the Montreal Children’s Hospital.

Having Thalassemia makes it impossible to take life for granted. I have Thalassemia major, the most severe form of Thalassemia. It is an inherited blood disorder where your body has difficulty producing hemoglobin, which is the protein in red blood cells that carries oxygen throughout your body. When your blood does not carry enough oxygen to the rest of your body, you have anemia. This type of severe thalassemia is treated with blood transfusions and treatment to remove excess iron in the blood. The blood keeps me alive, but because of the amount of blood I need to receive, there is a risk of extra iron accumulating on my vital organs, which could be fatal. So, I also have to use a chelating agent to remove this dangerous iron from my body.

The blood transfusions have always been a constant in my life. I still go to the same hospital that I always have. It is the only hospital in Montreal that has a dedicated Thalassemia clinic. One nice thing about always going to this hospital is that there are other patients there who I have known my whole life. The fact that there is a group of people who have the same medical condition as me makes me feel that I am not alone. Talking with others while receiving blood is a nice distraction.

When I was five years old, I started receiving a drug called Desferal to chelate the extra iron from my blood. The treatment consisted of daily injections. At least 5-6 evenings a week, my mother would gather together all the elements that were needed to mix this medicine. We needed a syringe, venotubing, alcohol, gauze, sterile water, and a needle. Once she had mixed this medicine, tapping the syringe very carefully to ensure that there were no air bubbles present, it was time to find a spot to administer the medication. You can imagine that this was my least favourite part. I would make a million excuses for why I was not ready for the needle. I would ask if I could have 5 more minutes, 10 more minutes… We would examine my stomach, my arms, my legs for a spot that was not too bruised, not too sore. Then my mom would poke me with the needle, something she must have hated to do.

As I mentioned, this treatment began when I was five years old. I had already been transfused 52 times.

The Desferal was delivered with an infusion pump that I would be connected to all night. The pump itself was heavy and cumbersome and restricted my movement quite a bit. Sometimes I felt as though I were attached to a leash. I learned not to move too much at night. Some days I woke up to find the needle yanked out of my arm and the Desferal leaked all over my sheets. Or sometimes the needle had not been inserted deep enough, and I would wake up in the morning with a huge swelling on my leg. Those were the worst days. It meant that all the pain I had gone through the night before had been for nothing. Desferal was a very cumbersome treatment, yet very effective at reducing iron in the body. My parents instilled into me the importance of using this treatment very early on. From the age of 12, I began administering the needle on my own. I also knew that several patients had not used Desferal often enough, and the excess iron had accumulated in their tissues and vital organs, leading to their death. I knew I did not want to end up like them.

When I was in elementary school, I did not have the ability to describe my condition to my peers. They knew that I missed a day of school every month, and somehow sensed that I was different from them. That, plus the fact that I was very quiet, prompted them to be quite protective towards me. I was very shy about my nightly injections and did not often have sleepovers.

By the ninth grade, I had been transfused at least 175 times. I was able to explain my condition to my classmates to a certain extent, but I would only tell my very best friends. I was afraid that if I told everybody, I would be treated like I was made of glass. I was afraid that they would pity me, and the last thing I wanted to be was pitied.

I grew up in a very close-knit community, yet to this day they do not know about my condition. When I was growing up, my mom would not talk about it with anyone in our community. It sometimes made me feel as though perhaps it was something that I needed to keep hidden.

But at home, it was because of my mom and dad’s strength that I grew up to be who I am today. My mom and dad always made sure that I had as normal a childhood as possible. They raised me to believe that I could do anything I wanted to do and be anything I wanted to be.

In college and university, I became much more open about it. By this time, I had been transfused at least 240 times. I was happy to discover that, even though people were at first shocked to learn that I was dealing with thalassemia, they were quite understanding and didn’t change the way they behaved around me. I also discovered that most of us are dealing with some type of problem or another— be it a medical condition, a psychological condition, a financial problem — and that I was not alone.

When I was 22, I was asked if I wanted to be part of a study for a new oral chelator in pill form called Ferriprox. Of course, I jumped at the chance to get away from the needles and the infusion pump. I had been waiting for that moment all my life. I started taking the medication and have been on it ever since. It has improved my quality of life tremendously.

In the transfusion room, patients don’t often think of the fact that an actual person gave us this blood from their own body. All we see is the bag of blood and it feels a bit disconnected. It is overwhelming when you actually do think of all the people who have made a choice to give blood, and that in doing so, they are saving your life. Blood donation is necessarily anonymous, but I sometimes wish that I could meet those people who give so generously.

I have worked full time for the past eleven years and it has its challenges. I require a transfusion every three weeks, and I receive two units of blood each time. Altogether, my hospital visits take 5-6 hours and I am quite drained by the end. I have to miss an entire day of work every three weeks, but luckily, I am at a company where they understand this and I am able to make up the time that I miss throughout the week.

In the past, I have not been so lucky. One company tried to make me use my vacation days for my transfusions. When I proposed that I simply not be paid for those days that I missed because of transfusions, I was actually accused of asking for “preferential treatment”. I actually had to say very bluntly to this person that I would die without these treatments. Another company almost reversed their decision to hire me when they found out I had to miss work so often. These companies would not have objected to make arrangements if I needed a wheelchair. Perhaps it’s because Thalassemia is not something that you can see when you look at me.

Living with Thalassemia is something that I have always done. It is a part of me. My parents always tried hard to make sure that I had as normal a childhood as possible, and when I became more independent, I, too, made sure that I never let my Thalassemia stop me from doing what I wanted to do. I grew up, I went to university, I fell in love and got married. I won’t let my medical condition be an excuse for not doing what I want to do. The fact that I am stubborn by nature has helped me well in this regard.

I estimate that I have had, at minimum, 531 transfusions over my lifetime.

I feel almost defiant. Why should I hide such a thing? It is not my fault that I was born with Thalassemia and need blood to survive. And there is no shame in it. In fact, I am just starting to realize that if I tell others that I need blood, they will realize just how important giving blood is. Being here today, talking to all of you is a great way to start.

I’d like to thank each and every one of you for the hope you give to people like me every day. Without you, I wouldn’t be here.

What I didn’t expect was the reaction of the audience to my speech, both times. There were gasps of surprise, and, as my husband observed, many tears among the audience members.

After each speech, I was approached by many audience members who actually thanked me. They thanked me for sharing my experience and for reminding them why they were giving blood, why they were volunteering at these blood drives, and why they wanted to continue doing so.

Five Years

It’s been about five years since my last post and a lot has changed, as would anyone’s life in five years. My life, my opinions, what I observe, what I find important, how I process information. We change a little bit everyday, influenced by the people we surround ourselves with, altered by the experiences we go through, and by deliberately focusing on the parts of ourselves that we choose to develop.

I was born with Thalassemia Major, a genetic blood disorder. In order to stay alive, I have been receiving blood transfusions every three to four weeks since I was a six month old baby.

I always wanted to find a way to thank those who had allowed me to stay alive and in good health by donating blood. A few years ago, the opportunity presented itself, and I grabbed it with both hands. Since then, I’ve spent a good part of my time and energy trying to turn my illness into a powerful positive, by speaking about it in public.

Over time, I’ve slowly been able to lose some of my inhibitions. At one point, I just decided to say “yes” to every opportunity I was presented, and simply ignore my doubts. Besides, there was no way I could possibly say “no” to an opportunity to be an advocate for those who depend on the generosity of blood donors. How would I live with that? I needed to reach out to donors and tell them my story so that they could understand what a powerful thing they were doing. I also wanted to tell potential donors why the need for blood donors was so great. I even decided to be in a TV commercial.