This quote has quickly become my favorite. My sister-in-law gave me this quote etched in stone just after our daughter Jamie died. It sits next to our fireplace below her pictures where I can read it whenever I look at her. It is so true, we only got to hold her in our arms for 4 months and 25 days.
Spinal Muscular Atrophy (SMA) took our daughter, robbed her of a life, and stole our dreams.
It was September 6, 2003, and finally, after 4 ½ years of trying to get pregnant our daughter was going to be born. She was almost 1 week late, but then again, she knew where she had it good. Everything went so well , the pregnancy was perfect, no problems at all. We had been waiting for this moment for so long. We were excited and scared, after all our whole lives were going to change forever. We just didn’t know how true this would be.
We decided to leave for the hospital at 2pm. I figured that, if I were over reacting, that I came in too soon that, they would just send us home. The contractions were about 5 minutes apart when we left. We got to the hospital went to the desk to sign in. The nurse told me I couldn’t possibly be in labor, I wasn’t miserable enough. I was checked – only 2 cm! Ug! It was our choice we could stay or we could go home and come back in a few hours. We stayed, what was I going to do at home anyways? I was checked at 5:30 – still only 2 cm!! What was going on? Frustrating! Well, the Dr helped me along and got me to a 3. I started to get nauseous and threw-up. The nurse came in and offered me a shot of gravol for the nausea and a shot of Demerol for the pain – sure – right in the butt. I went to the (awesome) whirlpool tub, If it wasn’t for the contractions, I would have fallen asleep. It obviously did its job because at 6:10 I got out of the tub and they check me again 6 cm!! Yay! Time to call the anesthesiologist to give me the epidural!
Well, the anesthesiologist, was still in the OR., just relax, I could tell by looking at the nurses that he may be there for a while, they kept on called, he just wasn’t coming. By 7pm the nurses were really looking worried, I wasn’t all that worried, I had my nitrous – and I was quite happy with it. Then the big gush came – 7 cm, nurses still nervously looking at the clock, 8cm and boy did I want to push, 9 cm, who cares where the anesthesiologist was, as long as I had my gas, ok - lets PUSH! No problem ,just let me keep my gas. Then the nurses told me that I didn’t need my gas anymore – I thought they were wrong. We just wanted to be together, then they took it away from me. Alright, time to get this over with, apparently I pushed for only 15 minutes and she popped out at 7:42 pm.
IT’S A GIRL!! All my mom could say was how perfect she was! Of course she is perfect! One of the prettiest newborns you’d ever like to see. Even with a natural delivery.
Life was perfect. Perfect little baby, she latched on right away, everything was just perfect. Even though everything was going so well, I will always be haunted, call it mother’s intuition or fatalism or what you will, I said to my mother, (and I don’t know where this came from) “Things are going too well, something bad is going to happen.”
Then around 3-4 weeks of age, we started noticing that Jamie had become a bit “lazy”. Janne liked to dress her because she used to help him, by pushing her arm and legs into her clothes, but she didn’t do that anymore. She just lay there. Ahh but she must just be so content and laid back! Nothing could be wrong with our daughter. Something though kept nagging at me. Something was wrong, but I couldn’t put my finger on it. When she was 1month old my mom had been holding on to her and commented on how “floppy” she was. This is the beginning where other people close to us could tell there was something wrong. At Jamie’s 2 months visit (a Wednesday), she had been feeding well, sleeping well, and gaining weight, but she was floppy. Our family doctor noticed her decrease in muscle tome and thought it might be best for us to see a pediatrician – just to be on the safe side. I started to get an uneasy feeling when, by Monday (Nov 10), we had an appointment to see the pediatrician. At that appointment he looked at her, especially at her tongue, she had a tongue tremor called fasciculation, which I would later find out is present in almost no other disease that SMA, and at that, not all SMA children have this tremor. I think he was extremely concerned, because he tried to get us into Sick Kids right away.
The following Wednesday (Nov 12) we went to our local hospital for a few preliminary tests (ECG, O2 saturation and a blood test). That was the first time I had heard those three words that would soon send our world spinning out of control. I had never heard of Spinal Muscular Atrophy, but I knew that Spinal and Muscular followed by Atrophy couldn’t be good. There just is nothing good about the word “atrophy”. I went home and looked up SMA on the computer. I was in shock, she couldn’t have this disease that would mean, if she did have this, that she wouldn’t live to see 2 years old. Breathing problems, swallowing problems, feeding problems, ..are not able to lift head, won’t walk, …half of SMA I children die before their first birthday, …most never make it to 2 years…It was all too much, where did our perfect life go? It wasn’t supposed to go this way.
The next Monday (Nov 17) we had an appointment at HSC (Hospital for Sick Children) we got a bed and they did a few tests. It didn’t take them long, (neurological exam) to suspect with a high degree of certainty that she had SMA. The tongue tremor, the lack of reflexes and history all were consistent (except at this time she had no problems with eating or breathing). To be sure it was SMA, we had a DNA test done.
Christmas came and went, it was festive and joyful but at the same time sad and dark, always overshadowed with the though that this was possibly my baby’s first and last Christmas.
Jamie, around this time seemed to get a bit of a cold, she was a little congested, but for the first while, it wasn’t much to worry about. Then the congestion wouldn’t go away. I knew these babies were at risk for colds (because of their lack of chest strength and ability to cough) and it worried me. I went to an after-hours clinic, our family doctor and her pediatrician. They all said it was just a virus and it would go away. Again my instincts told me this was not just a cold, it was lasting too long, we were into January, and it had been about 5-6 weeks. Then she seemed to get stuffier and had problems with , what seemed like regurgitation or reflux when actually she was losing her swallow and couldn’t get rid of the saliva in her mouth. On one Friday evening she was choking on saliva, it was horrible. The next day we made the decision to get a suction machine. To me this was becoming all too clear, too real, it was starting to go the same way as other childrens’ stories that I head read about. I realized that this was the beginning of the end. It was a very scary piece of machinery. It’s loud and you have to stick it in your baby’s throat. It worked well and she learned that when this loud machine was turned on that she was going to feel better.
I had always wanted a family portrait. I kept waiting until Jamie was a bit older to have it done. One Sunday I had said to Janne that maybe after Church we should go and see if we could get some pictures done. I felt a sense of urgency about the whole situation. We went and there was no opening until later that day. We couldn’t wait around, I had to feed her, get her home, change her etc… We went home. I sat down, turned around and said to Janne. ”We have to get these done today, If we don’t I feel as if I may regret it.” This was exactly 2 weeks before her death.
Because this was such a stressful time for us, I had problems sleeping and we had problems dealing with all that was happening. We went to see our family Dr. I had slept only about 5 hours in 2 nights. I kept waking up. I was scared that she was going to stop breathing in the middle of the night. While we were at the appointment he decided to weigh Jamie. To my horror she had lost 10 ½ ounces since the beginning of December. I had thought she was a little thinner but it’s hard to know. The Dr. told me to supplement her with formula, because she was also a stubborn baby and wouldn’t take a bottle, I would use a syringe and get the formula into her that way. In the course of a week I had gotten and extra 52 oz. of formula into her.
The next Monday (Jan 26) we had an appointment at HSC to finally get our test results. It was no surprise what the results were. We knew. I think we always knew. “Homozygous deletion of SMN gene” is what the official results read. This meant the gene responsible for making survival motor neuron (SMN ) protein was absent on both chromosomes 5. Our daughter undeniably had SMA.
The following Wednesday we had another weigh in and after all that formula, Jamie had lost another 2 ½ ounces. On top of all that had happened that day, on our way home, my Jeep broke down. It was bitterly cold out. But we were close enough to my Nan’s apartment that I took Jamie and went there while Janne got the Jeep towed. That evening I made a conscious decision to call her pediatrician in the morning and arrange to have a feeding tube put in her.
I fed Jamie that evening, gave her formula, burped her and put her to bed. Because she had vomited a bit I decided to lay her on her side. I kept waking up during the night at every little sound she made, worried about her. She slept about 8 hours and then woke up, I tried to feed her but she was soo stuffed up and irritable. I laid her down on the bed. As I watched her I noticed that her color started to go bad. I went and suctioned her and decided that maybe I should take her to the hospital. I called my mom, because I had no vehicle (Janne was working midnights and was working late this morning) and to take her to the hospital. My brother came to get us, it had snow so much during the night and we needed a 4-wheel drive. Matt came in and I looked at Jamie again. Her color had gotten worse I decided that I had to call an ambulance. While waiting, for what seemed like an eternity for the ambulance, Jamie stopped crying, her skin went blue then grey , I suctioned her again but there was nothing left to suction. My poor brother was screaming at her to wake up and to stay awake, she came around again. I really wish he didn’t have to see that. I’m sure that it killed him. The ambulance came and gave her oxygen and we bundled her up and left for the hospital. On the way she stopped breathing. We bagged her the rest of the ride in. At the hospital she was intubated. X-rayed, and had an IV put in. The attending doctor said she was a very sick little baby when she came in but after being intubated, she looked 100% better. They suspected RSV (virus). I suspected otherwise. Sick Kids sent a team up to get her and took her to Toronto. To make an already long story shorter, Jamie stayed in the CCU at HSC. Eventually all her blood tests were coming back negative for virus, and bacterial infection. It had become quite clear that the disease had weakened her to the point where her lungs had collapsed because of the lack of muscle needed to expand the diaphragm. We had a horrible decision to make.
We woke up the morning of Feb 1 and went to spend the last precious hours we had left with our daughter. My parents, Matt and Laura (brother and sister-in-law), Janne’s mom, my friend Monique and my friends and Jamie’s’ Godparents Julie and Bruce and Linda , all came to see her that day. Pastor Jack who had gone though all of this with us came down to be with us. It was such a hard day, but everyone had the opportunity to be with her and spend time with her and say goodbye. I gave her a bath, and held her amidst the tubes and machines while she slept. We took her to a special private room where we could be alone. We made casts of her feet and hands, played with her and told her how everything was going to be alright. We told her she fought the good fight and we were so proud of her, we told her how much we loved her and that where she was going was so much better than this life a place where she would be healthy and happy and free.
Just before 7 pm the doctor increased her morphine, sedated her and removed the breathing tube that was keeping her alive. She quietly died within 10 minutes. She was snuggled on my chest surrounded with those who loved her most. It was very peaceful.
After the fact
There are so many little things that I remember about the whole experience. I remember how hard mentally, emotionally and even physically this was. I remember the turmoil that was going on inside me. To know your child is going to die from a disease for which there is no treatment or cure, is so unbelievably hard, it’s so foreign and no words can describe it. There are no books to tell you how to deal with this. People ask us how we do it, how we go on ? All I know is that we didn’t have a choice, we had to go though this and we had to somehow survive. Every day is hard. Some are harder than others. We have our faith in God and that has helped us. For a while I felt abandoned by God. I felt like He had forgotten about us while she was sick. Just before and now after her death we have experienced God lifting us up and giving us His peace.
There are so many things that we are grateful for. Grateful for having her, spending time with her, listening to her little stories and, bathing her, grateful for the little things. We were glad to have been able to have had time to say goodbye and to be there for her when she died and to have had her die in our arms peacefully. Even though it happened, I wouldn’t have had it any other way.
She is and always will be our beautiful, perfect and intelligent sweet baby. She had many friends who loved her like their own. She was here for such a short time, but has taught us so much about life, death, happiness, grief, and about ourselves.
It’s a constant struggle and will be for as long as we are around. We have many friends who have gone above and beyond the call of duty, so many people have been so good to us. On the flipside there are those that don’t know what to say or what to do or how to act, it makes for and uncomfortable situation sometimes, but remember it’s ok to talk about her. We always do!