From the moment the line turned blue, I was a mother. It's funny how that works! I think for the fathers, it takes more-an ultrasound, the first sight of the baby, the first smile or coo-but for mothers, it is different. From the moment we know, we are hooked. From the very first day, I knew this was special, and I knew how important it was I not take a moment or a thing for granted. I was so grateful that, unlike many other couples in America, we didn't have any trouble with infertility. When I found out he was a boy, my first thought was "uh-oh, I don't know what to do with a boy!" But I figured I would be able to figure it out! We chose his name, which came out of the blue from Steve, as Devon Richard, after his Grandpa. I did a video tape for the baby, taping his room, his stuff, and me getting bigger every month, along with some things I hoped for him for his future. I started a journal for the baby, to give to him when he was a grown man, telling him what I was thinking and feeling and hoping. The pregnancy was so very easy! I didn't have morning sickness, I wasn't overly tired, I didn't get huge, and all in all, it was just a wonderful experience and one I truly enjoyed every day! I even continued flying up until 3 days before he was born, perfectly comfortably. The last month I noticed that he wasn't moving as much, and the doctor monitored him once a week until he was born to make sure that he was okay. He seemed to be fine, just "quiet".
The day Devon was born was one of the most memorable and happy days of my life. The labor was long and difficult, and after 20 hours they were preparing me for a C-section, when all of a sudden Devon decided to make his appearance the "normal" way after all! He was so tiny, 6 lbs. 6 oz., 19" long. He was perfect! His APGAR scores were 8 and 8, and he was the most beautiful baby I'd ever seen. :-) He had the best little personality, and never cried! He latched on to nurse like an old pro, almost attacking me, with suction so strong I couldn't disengage him without hurting myself! From the moment I looked into his eyes for the first time, I understood two things that I don't think anyone can understand until they have a child: 1). The true mother's protection instinct. I knew I would do anything, even die for this child, without a question. and 2). How much my own mother loved me.
When the nurses left him alone with us in my room, lying in his hospital gurney, I whispered to Steve "Can we pick him up?" I didn't even unwrap him at first-I simply didn't know what to do! The first diaper change was hysterical, because Steve and my mom put it on, but didn't get it tight enough, and as soon as he was picked up it drooped off and fell around his ankles! We still get a laugh out of that-he just had a tiny little bottom!
When we got home, I didn't take a moment for granted. I spent so much time just staring at his little face and features. I took pictures constantly, and had Sears portraits made of him every month to track this first year of life when they change so much! Steve (and others) made fun of me for how many photos I took, but I didn't care-this was my baby and I was going to document every smile and sweet sleeping pose that I could!
I read all of those "Baby Milestone" books. I kind of noticed he didn't move his legs much, but the books didn't say much about legs yet so I figured that must come later. I am an only child, and Devon was my first, so I simply didn't know. I noticed he wouldn't lift his head from his tummy yet, but at 2 months that still wasn't something that bothered me. I determined I was not going to be one of those mothers that hovered over everything and worried myself sick over things. So I chose not to.
At Devon's 2 month check-up, he saw a Nurse Practitioner instead of the doctor. After a cursory examination and his shots, she sent us on our way. He was gaining weight fine, and looked perfectly good, she said. But around 2 1/2 months I got this little worried feeling in the pit of my stomach. Why would he just cry when I put him on his stomach. Why didn't he try to lift his head? I waited another week, and finally called the doctor. The nurse on call asked me if he held his head up from my shoulder, which he did, and I said Yes. She told me not to worry about it then, he was just doing it on his own time schedule. That made me feel so much better-for about another week. Now Devon was 3 months old, and it was really bothering me that he wouldn't hold his head up from his stomach. At All. So I call the doctor again. This time they tell me I am being an over-worried mother, and that he would do it when he was ready-that he had a big head for his body and maybe it was just hard for him to lift right now. I didn't feel better at all. That feeling in the pit of my stomach wouldn't go away. I knew darned well I was not being an "hysterical" mother. Something was wrong. And finally I was ready to admit it to myself. I decided maybe he just had weak muscle tone and needed therapy to help. That's all it could be, right? So I call the doctor again, at 3 months and a week, and I tell them that Devon is ill and needs to be seen. By a doctor, not the nurse practitioner. Of course I am lying, but I don't think they will look at him unless he is ill, and I am right. They agree to see him the next day.
That next day was beautiful. It was one of those late summer/early fall days where the colors are just brilliant and the air is warm but not hot, the sun was out and everybody was in a cheerful mood. It was a day nothing really bad could happen. I decided that Devon really was fine and when they saw him today I would feel stupid when they said there was nothing wrong and he looked healthy to them. When the doctor came in, it was the first time I had seen him. He looked over Devon, checked for reflexes (I didn't see any reflexes and that feeling in my stomach got worse), pulled his arms up and dropped them, over and over, and same with the legs. They just fell to the table. He didn't try to pull away, he just looked up at the doctor with a pleasant look on his face. And then the doctor looked up at me with a frown, and it was the first of many times that my world was shattered. He said to me "Something is wrong. His muscle tone is ALL wrong. We need to run some tests." I said "For what?" And the doctor said "He probably has Muscular Dystrophy or some other form of neuromuscular disease. I will go schedule the tests." And then he left the room and I was left standing there, with my son, and my world had just shifted on its axis by about 20 degrees. I got home on autopilot.
And thus began the rounds of testing and doctors.
The first thing we had to do was a blood test for Muscular Dystrophy. It was a nightmare. He had very small veins and the nurses couldn't get the blood. We were there for 30 minutes while they poked and poked him, and this poor 3 month old baby was just crying his heart out. I was trying so hard to be strong, to not be one of those mothers that gets upset, but I couldn't help it, and my eyes filled with tears that I tried to hold back. I looked up, and the nurses were crying! Finally they said they were done, that the test results should take a couple of days. I go home, I look up MD on the internet. I see the twisted bodies and the diagnosis of death, and I am crushed. But, I think, many of them live to their 20's or 30's...perhaps that far in the future they will have a cure! We can care for our son in a chair and he will have an excellent life! The next day I get a phone call-they didn't get enough blood yesterday and need us to come back in for more. I do so, and again it is a nightmare. I tell the nurses to get whatever blood they wanted this time, because he was not coming back in again even if it meant he remained undiagnosed! This was their last chance to get his blood for this test! Finally it is over, and I carry him in my arms to the car. This time, as I am driving away from the hospital, I just lose it. Devon and I both wailed all the way home. I cried for pain he had felt, I cried for the fear of waiting for the diagnosis, I cried because I hadn't cried yet about any of it. I cried until I had a pounding migraine.
Two days later I call for the results, and I am told "Well, this test takes 2 weeks!" Oh, I almost died. I had barely eaten or slept for the last 2 days and now I'm being told I have at least 12 more days of this! A week goes by that I don't even remember. Then they schedule a CT-Scan of his head-they want to test for Cerebral Palsy. They give me medication to paralyze him so he won't move during the test. I find a sick irony in the fact that they are making me drug my son so he won't move so they can run a test to see why he isn't moving. That test is also bad. He fights off the medication, turns his head back and forth, and cries and cries; until his Daddy stands by his head and sings him to sleep. Finally he is asleep, and they run their test. I ask for the results. I know they can see right there whether he has brain damage of any kind or not! But you know what they tell me? "Sorry, we have to tell your doctor first, then he can contact you." I was furious! They know what the results are and it is MY son and I am paying for this test and I want to know the results right NOW! I wait another day and call the doctor's office and am told they hadn't looked at his case yet. So I told them I would wait, and to go look. They weren't thrilled, but I didn't care. It was my first lesson in not just being complacent with physicians. Finally she comes back on the phone and says the Cat Scan was negative for any Cerebral Palsy or brain damage. I get on the phone, call everyone I know, and say "It's Not Brain damage!" and we all laugh and cry and feel light as a feather.
The next obstacle is this MD test (Muscular Dystrophy). Finally two weeks have gone by and I call. They say they don't have the results from the lab back yet. Again I turn into Demanding Mommy Mode and I said to this poor lady "Listen, I am waiting to find out a test result on my son that will tell me whether he lives or dies! It is CRUEL to make me wait this long on something this critical!" She called the lab, they were done with the test they just hadn't sent it on to the doctor yet. So in 5 minutes she called me back-the test for MD was also negative. I started jumping up and down and screaming for joy. I called everybody who was praying and told them the good news. I said "Thank GOD. Now that we know what it isn't, let's find out what it IS!" I figured now that we knew it wasn't MD, we could handle Anything Else it could possibly be.
I sat down at the computer and searched for "Neuromuscular Disease" and "Hypotonia" and "floppy" since that was what the doctor said was wrong with Devon. I made a chart of every neuromuscular disease that had floppy as a symptom, along with all of its symptoms, how it is diagnosed, and what the prognosis is. And then I began eliminating based on what I knew. For example, I eliminated any diseases with mental retardation because I could tell that his mental capacity was fine. I got down to a few diseases, but what was most encouraging to me was that almost all of them meant a life spent in a wheelchair, but a life none-the-less. There was one on my list called "Werdnig-Hoffman Disease", and it had the worst prognosis of any of the others-death by age 2. I read it and when it said weak neck muscles, weak cry, weak suck, I thought "Thank God it can't be that-Devon's head is strong except from his stomach, and his cry and suck are VERY strong. I couldn't handle that!" Steve and I discussed how it was fine that our son would probably be in a chair-that he would have every opportunity and would be able to do anything in life he chose. We were prepared-we thought.
My doctor next set up an appointment with a neurologist, and wasn't in any hurry. That encouraged me so much-I figured if it was that critical, they'd set up an appointment immediately. But they didn't. I figured it must just be a developmental delay after all, or one of the more manageable neuromuscular diseases on my list that seemed to fit the symptoms Devon had. I also figured when I went to the appointment, HE would want to schedule tests too and I wouldn't find out anything of importance at that first meeting-so I went alone with Devon. I was wrong.
The neurologist was a kindly man. He took off Devon's clothes, and played with him and talked to him for just a moment. He tried to pick him up with his arms from lying flat, and I felt myself defending why Devon's head didn't come up with his body. Devon just grinned up at him, loving the attention. Finally he walked away, and Devon started crying for attention. I went to get him, but the doctor wanted him to cry for awhile until he got really mad. I didn't understand then, though I do now. Finally he sat down with a sigh and asked if there was anybody he could call for me. I wanted to know why. He didn't want to tell me. I told him I'd made up a chart of all the possible neuromuscular diseases and he asked to see it. He could see what I had eliminated and why. He asked me if I had ever heard of Werdnig-Hoffman disease. I said Yes, but after looking at all of them I couldn't remember which that was. His face was so sad, and I just kept feeling sicker and sicker. I had to drag it out of him. I said "is it bad?" and he said "Yes." I said "Is it fatal?" He just looked at me and again asked if there was anybody he could call. I told him that I wouldn't have asked the question if I couldn't hear the answer, and for him to please stop dancing around and TELL ME WHAT WAS GOING ON. He said "yes, it is fatal." I felt numb, but just wanted to get the facts out, so I said "How Long?" When he looked back up at me he had tears in his eyes and he said "Less than 2 years, most likely less than one." I just nodded. I didn't cry, I didn't scream. I just looked over at my son, lying on that table. When I'd walked in here I was holding a perfectly healthy boy with maybe a muscle problem. When I left I would be holding a child with less than a year to live. My mind couldn't wrap around it. He said he was sorry, he said he needed to run tests to be sure. I told him "But Devon has a very strong suck and cry and his neck control is good except from his tummy!" He gently told me to go home and research it myself and see what I thought and that in the meantime he would schedule an EMG to test for Werdnig-Hoffman. He was very compassionate and very kind, and I will always remember him kindly for that.
I don't remember leaving his office, but when I got back on the road for the hour and a half drive home, I called Steve at the office, told him what he said, and he looked up Werdnig-Hoffman on the internet. The first site he found was then the only personal site we found on the disease-the first time we heard the letters "SMA". It was Michelle Irving's site, www.smaangels.com, and at the time it was a site about her two children who had died of SMA. I cried all the way home as he read the words of her story to me.
That night I stayed up and researched everything I could find on SMA. It was almost all technical medical stuff, and the more I read the worse it got. For the next 3 days, I couldn't eat, sleep, or concentrate. While I was waiting for the tests for MD and CP, I felt like things could be okay. But now, waiting for this test for SMA, I knew. I knew in my heart that this is what my son had, and I couldn't stand the thought of having it confirmed. It was such a worse-case scenario. We couldn't even give him a good life in a chair-it was an almost immediate death sentence! All night every night I lay in bed and pounded my head in the pillow trying not to wake up Steve. I felt like I was dying. Finally the day came, and I INSISTED that the doctor tell me the results immediately instead of telling my doctor who could tell me. Both doctors agreed to this. Devon went through the EMG, with needles being put into the nerves in his legs while they measured electrical activity. He cried a little but it didn't seem to bother him as badly as I would have thought. I still hated it. The doctor had a student in with him, and I could tell they both thought that what they were looking at was fascinating. I knew then. If it were normal, it would not have been nearly that interesting. Sure enough, he turned to me and said that the test was consistent with SMA, and that he was sorry. I just dressed Devon, while this doctor kept saying he was sorry over and over, and Steve and Devon and I got out of there and drove home in silence.
That night I sent out the following e-mail to my family and friends:
And that night, now that things had been confirmed and I knew what we were facing, I could finally sleep.
The next few weeks went by in a blur. I literally could feel my heart shattering in my chest like so much glass when I would wake up and the first thought that hit me was "Devon is Dying." It seemed so unreal. He was no different than the day before, but nothing would ever be the same again. I didn't know where to go or what to do. I got through my days by keeping to our normal routine, and by spending all my time on the internet looking for information and help. I found the Families of SMA guestbook, and instantly became a member and frequent writer. The people I met there were what saved me. It was there I learned that we weren't the only ones going through this; that other parents were handling this same diagnosis with fight and determination and grace and courage. I knew if they could do it, I could do it too. I decided I wasn't going to let him go without a fight, and I began looking into every possible treatment out there from massage and acupuncture to test-drugs to Dr. Bach's protocol to natural substances.
Meanwhile, the neurologist wanted to do a muscle biopsy too, to make the diagnosis 100%. He said that this would remove any hint of a doubt. At the time, I did not know a blood test was available-and neither did he. That is the one thing I was and still am furious about-that I had to put my son through a test where they shoved needles in his legs, AND a surgery, on a ventilator, giving him a scar he had for the rest of his life-when all he needed all along was a blood test! I didn't need the muscle biopsy, I already knew. But we wanted to be 100% medically sure, so we said okay on the off hope that maybe we were all wrong.
The wait for the surgery was terrible. He was so tiny, just 4 months old when they had him up on their shoulder and walking down the hall towards the surgery room, and he was just peeking over their shoulder at us, happy as a clam. He had no idea what was coming and I just felt sick to my stomach. Afterwards they brought us to a recovery room and carried him in, wrapped in a thick blanket. He was crying hoarsely and hysterically and I couldn't calm him down. Finally I nursed him and that did it. His whole upper leg was bandaged, and I felt like the worst mother in the whole world. He was fine by the next day, but I have yet to recover from it!
I was at the GI doctor's office when the test results for the muscle biopsy came back, and she hesitatingly told me it was confirmed to be SMA. I didn't even blink. I told her I'd known that for a long time now. The GI doctor wanted to put a g-tube in Devon right away, at 4 months, even though he had no trouble sucking, swallowing, or digesting. I said no. I wasn't going to put him through anything until he needed it just because she said he would need it some day. IF he needed it, THEN we would get it. And I was getting the idea that I needed to have a more active role in deciding what my child needed instead of just taking doctors at their word. Because it seemed like she wanted me to get the g-tube now more for HER convenience, than for Devon's health. She thought that just because he'd need it sometime in the next year we might as well do it now. I, on the other hand, as his mother, wanted him to have as normal a life for as long as possible, and a surgery and g-tube did not fit that bill. I left there confused and worried that I was making a bad decision, afraid that I would wait too long and it would be too late, per her dire predictions. But we stuck to that decision, and I am grateful that we did, as he went 8 more months without having to have it!
The next day my neurologist called and said now that it was confirmed, I would have to make two decisions right away. One, would we put him on a ventilator when the time came that he couldn't breathe on his own anymore. Two, would we get him a g-tube to help feed him when he couldn't suck or swallow anymore. I asked if the ventilator would save him, and he said to me "It would prolong his life, but he would be utterly paralyzed and on life support until he did die." That sounded horrible to me, cruel. (I now feel that should have been handled differently by the doctor!) So I told him no, we wouldn't ventilate, but yes, we would have to get the g-tube because I couldn't imagine letting my son starve to death. He said that these were the same choices he would make if it was his child facing this. For some reason I didn't feel better.
And thus began our lives with SMA. Our family and true friends were wonderful. They were the ones who didn't run away, who weren't afraid to be around us. They were the ones who treated Devon exactly like they would any other child. They would play with him and talk to him and love him unconditionally. They followed our example and followed our request, to only be happy around him. Devon's life may have to be shorter than normal, but it was also going to be happier than normal. Nobody cried around Devon, not me, not anybody. We traveled with him, we took him on airplane rides and boat rides and on vacations to the beach and all around. We took him to see family and loved ones and let them get to know and love him. And in the meantime I looked up anything I could find to save him. I would have donated my own spinal cord if it would have saved him. Everybody we knew prayed for a miracle for him. I know of many of his family who prayed that their life would be taken in his place. But SMA gradually had it's say.
The story of life with SMA is a book in and of itself. I honestly didn't really realize how hard it was until it was over and I could look back and see it outside of the fog I lived in for so long. Devon just got stronger and stronger until he hit about 7 months. He could lift his legs, move his upper arms, hold his head up by himself when you sat him up. He could grab my fingers and pull himself up into a sitting position in my lap and keep his head up, and he would smile so big and be so proud.
Around 7 months of age, we got a Bi-Pap and decided to try to get him used to it before he really needed it. It was horrible. He hated it. He screamed himself to sleep and I would lay there on the floor and hold his hand until he cried himself to sleep, and then I would cry when he couldn't see me. I felt like I was causing him more pain than help. I felt like a terrible mother as he'd look at me and just cry and cry and you could hear him yelling "I HATE THIS, GET IT OFF OF ME!" He didn't even need it yet, but we wanted him to be used to it before it became critical. And eventually he would go to bed at night with it on. But when he woke up in the morning, he wanted it off ASAP. This continued the rest of his life. And to this day, I hate that machine, and if I never have to look at that mask again in my life, I will be happy.
Also around 7 months of age, he started crying and screaming when he ate. He would suck and suck and then pull away and just cry. I didn't understand what was happening, I didn't know what was wrong. I called a lactation consultant and they told me to pump and see what I had. I rented a professional-quality pump, and at the time for the next meal I was going to pump it and feed it to him in a bottle so I'd know how much there was. I got an ounce and a half. My milk supply had just gone down and down, because he was no longer able to suck hard enough to keep up the supply. Within days I had to switch to all bottle feeding, and I was crushed. It was like SMA won a round in the war. Devon loved to nurse-he absolutely loved to cuddle and nurse and it was so comforting to him, and we had just lost that. I felt like I had just lost another piece of him to this stupid disease. For several months he did fine on bottle feedings, but then he got pickier and pickier on what he would eat. He started only eating certain solids. He wouldn't drink the bottle if it wasn't warm-to the exact right temperature. He wouldn't drink if it was anything but a particular brand of formula. A couple of times I decided I wasn't going to let him be that picky, so I tried to out-wait him. But he won, because I couldn't let him go for days without food-which he would gladly do if it wasn't done his way. He started losing weight.
So from 7 months on, we got on an eating cycle that continued until we had to get him the g-tube one week before his first birthday. He would eat great, no problem. Then he would go a day and not eat much at all. Then he would eat fine for 2 weeks, then spend 2 days not eating well. This kept going until finally he had more days he wouldn't eat than he did days he would. By 10 months or so I was spending most of my day on the couch with a bottle in his mouth trying to get enough nutrition in him to keep him gaining weight. I had a baby scale in our kitchen and I weighed him every morning to see how he was doing. By 11 months his secretions had gotten very bad. He was teething a bunch of teeth at once, and his saliva was so thick and so copious that I couldn't get food past it. So on the bad days I would sit on the couch with a bulb syringe and suck out the mucus in his mouth, then squirt formula in his mouth with a medicine dropper, then suck out the mucus again. It would take me all day to get enough in him. But he never fought me. He would open his mouth to take the formula, and he seemed grateful for it. Then two days later he would be able to eat on his own again. Finally just before his 1st birthday, there were no good days. I was on the couch all day every day with him trying to keep him hydrated. I called the GI doctor and said "NOW we need the tube." She wouldn't return my calls for a week. I called every day, getting more and more panicked. Devon was dropping weight like crazy, he was getting pale and listless and lethargic. He was starving to death in front of my eyes and she was not returning my calls. I knew I had waited too long, and I knew why. I didn't want this to be happening so I tried to pretend it wasn't. I figured he would be okay. He had to be. He would start eating again. He would fight this off. He would beat SMA. He wouldn't need a surgery. By the time I figured out that he was going to DIE if I didn't get him help on this; by the time I figured out that the only way SMA was going to win was if I DIDN'T FIGHT IT, it was almost too late.
I was a bit more knowledgeable by the time we had this surgery. I told them I didn't want him put on a ventilator unless it was absolutely necessary. She said that probably wasn't possible, that SMA I kids weren't strong enough to undergo a surgery with general anesthesia off of a ventilator. I told her to try. This time they let us change him into hospital clothes, and they let us go into the room with him. They took pictures of us with him before the surgery, and they explained precisely what they were going to do. They let us stay until they put the mask on his face with strawberry-scented gas to knock him out, and then they had us leave. 15 minutes later he was being wheeled down the hall to his room and we were with him. 20 minutes later he was awake, and after a dose of Tylenol (nothing stronger!) he was fine! And the doctor said to me, in total amazement, "I can't believe he didn't need to be put on the vent! He is so strong! Your other doctor didn't believe me when I told him that Devon didn't need to be on the vent for this surgery." I wanted to cheer-that's my boy! He wasn't bothered by that tube at all, and suddenly our lives were much easier. I could give him the vitamins he needed and he would get them rather than spitting them out because they didn't taste good! I could still feed him normal food first, and then on those bad days, make sure he got enough through his tube! It was wonderful, and I wish we had done it months sooner. Fear cost us precious time with him, time that I didn't have to spend on the couch with a bulb syringe and a medicine dropper. I wasted so much time fighting something that only helped, and that is one of my regrets.
Around 7 months we also got Devon a pulse oximeter, so that we could monitor him at night. His levels were always around 98-100%, even off of the Bi-Pap. Gradually those started getting lower and lower, even ON the Bi-Pap. We couldn't figure out what was wrong. One night the levels wouldn't stay above 94%, so we stayed up all night, and pushed on his belly when he breathed, and that kept the numbers up. Steve did it until he was exhausted, and I passed out too. Finally around 6 am we took Devon to the emergency room because we were too tired to continue to do it and we were afraid something was really wrong. After blood work was done, we found out the cause...we had a loose probe on his toe!! He was fine! We felt like morons, and exhausted, and upset. And grateful. So from then on, that was the first thing we checked when the numbers got low! We noticed that his levels were lower on our machine than in the hospital ones, so we figured the machine must just be off and that was why the lower Sats than he had months earlier. This downward trend in Oxygen levels continued up through the end.
Also at 7 months, Devon lost his ability to hold his head up, and pull himself up into a sitting position in my lap. He would try and try and I could see the effort in his face...and then he would flop back with this sad and confused look on his face. His body was betraying him, and it hurt me so much to see that look on his face that I never tried to help him sit up like that again. He would try to lift his head like he used to, and would be unable. He would try to lift his arm, and be unable. He would try to turn over and be unable. It was crushing to me. I tried to ignore it, to stick my fingers in my ears and squeeze my eyes shut and say "Nah nah nah I can't see or hear you so it's not real" just like a child. I went around smiling brightly and talking cheerfully and doing everything for him as if nothing was wrong because I never wanted him to know, and I didn't want him to be sad. As you can see, 7 months was a big important mark in Devon's life, the time when, for him, things started unraveling. I think by the time he was 11 months old, I wasn't fooling him anymore. I think he could sense my desperation and sadness, because he lost some of his sparkle. His smiles weren't as wide and his eyes always held a tinge of sadness too-and I think it was partly for me. That will always be another of my regrets, because it felt like I failed at the one thing I was trying to control--him only knowing happiness.
When he was 11 months old, I had to take him into the ER at midnight. His sats were low, his heart rate was sky high, he hadn't eaten but 7 ounces of food all day, and he had a slight temperature. I was afraid for him. They took him in, and tried to put in an IV to hydrate him. It took them an hour and a half of sticking to get an IV in. He was held down to the table, with rubber bands all over his arms and legs, and bruises all up and down his arms, hands, and feet. Finally they got one in. It was a terrible experience for all of us, and I couldn't even look at him after awhile, because I didn't want him to see me there, not helping him with all these people hurting him. But I still had to be there. I couldn't leave, because if he had to suffer, so did I. That's just the way it is. Finally he was rehydrated, in a room by himself. They took him in for X-rays, and they sat him alone in this plastic bubble that was so tight it held him upright with his arms over his head. He looked tiny and terrified in there, and you could see the curvature in his spine and his little shrunken chest. It was just very upsetting. They told me to leave the bars on his bed up (it was a cage-all the way to the ceiling). I refused. They would come in and put them up and I would take them down as soon as they left. I was not going to leave him in a cage. The nurse said if he rolled off he could get a serious head injury. I told her he would have to roll over 3 times to fall out of this bed and if he did that I would scream hallelujah, not be mad! They told me not to feed him anything but water since he had an IV. I disagreed with that so sent my father-in-law home to get some food for Devon. I had just had it by now, and I knew that if anyone was going to care about the best for my son, it would be us, and we would have to stick up for him. Two days later we got to take him home, and he seemed fine.
Devon's first birthday was May 16th. He had just had his g-tube for a week, and was tired but seemed okay. He was a little uncertain about all the hoop-la for his birthday, but he stuck it out fairly well. A month later we were traveling to Missouri to visit my parents, when I was feeding Devon through his g-tube, and suddenly he started vomiting out his mouth and nose, very violently. We were at a gas station, so I pulled him out and over and hit him in the back and tried to clean up his nose and mouth right away so he wouldn't inhale any of it. This was the second time this had happened, and I was terrified he was going to get aspiration pneumonia. He seemed okay, and we went on for the weekend. That whole weekend he was a bit "off", not quite as happy or smiley. We figured the trip had been too much for him. On the way home, Steve noticed that Devon seemed a bit lethargic and uncomfortable, but didn't think much of it. I didn't even notice. We put him to bed that Sunday night, but I left him off of the Bi-Pap because I didn't want to give him any harder of a time after the weekend. I did hook him up to the oximeter though. Around 6:30 that morning, his oximeter kept going off, with the levels below 94%. I figured the probe was loose again, because he was sound asleep and looked completely fine. After my third trip across the house just to have the levels go back up when I messed with the probe, I just shut the machine off and went back to sleep. Yet another one of my regrets.
Devon normally woke up, quite cheerfully, around 8 a.m. Sometimes he'd sleep in until 9 am, but that was more rare. So when 10 a.m. rolled around and Devon still wasn't in there quietly and happily chatting to himself, I went in to wake him up. When I got in there I said "Hey, buddy, what are you doing lazing the morning away in bed like that?" As I got closer, I noticed he was breathing very rapidly and shallow. I got right up to him, and his skin was a funny grayish color. I grabbed him and said "Devon! Wake up baby!" and rolled him over onto his back. He opened his eyes, and when he did that's when I knew we were really in trouble. His eyes were glazed, unfocused, bleary, and he wouldn't stop breathing funny even awake. I grabbed him, yelled for Steve to get his Bi-Pap and oximeter, and put him on our living room floor. We hooked him up to the oximeter first, and his levels were reading in the 70's. My heart was racing a mile a minute, and as we got the Bi-Pap on, it wasn't helping! His levels stayed low, and his color was getting worse. When his sats dropped to the low 70's, we unhooked everything, threw ourselves into the car, and speeded to the emergency room. I just held him in my lap-a car accident was the least of our worries at that moment!-and begged him to keep breathing, to hang in there. When we arrived, they knew who we were as we had prepared them for what we wanted if this ever happened-they had a copy of Dr. Bach's protocol on file.
They took us immediately back to the ER. His levels would not come up, so they told us per Dr. Bach's protocol, they would intubate rather than just provide oxygen. We agreed. And this is the one that hurts me the most, to this day. Had we simply provided oxygen, he may have been okay. But that one decision set off a chain reaction of events that eventually led to his death.
As we were waiting to intubate, suddenly Devon vomited blood. It was dark red blood, like it was old. And there was blood in his g-tube too, also dark red. The doctors had no idea what is was or why it was there, and to this day we have no idea what that was from. But it scared the beejeebers out of us all. The doctor tried for half an hour to intubate him, and couldn't do it. He kept pushing these metal things down Devon's throat, and his gums were bleeding from being scraped and he was crying and it was more than I could handle. I started crying and I said "Oh, Devon, I'm so sorry baby". Steve grabbed my arm and he said "Laura, Devon needs you right now, you stop crying." And I did. Finally they called another doctor in, who got the tube in right away, the first try. I could have killed that first doctor with my bare hands.
They did x-rays on Devon, but we all assumed it was pneumonia since it is so common in SMA children. Our local hospital also called Riley Hospital for Children as it is our area expert children's hospital, and they sent a team up from Indianapolis an hour and some away to get Devon and take them back to their hospital. As soon as they walked in the door, they took over. They took Devon in a moving hospital back down to Riley, but they would not let us go with him or even follow behind them. So we had to go home, pack and then head down there. Finally we get there and they let us in, and he is on a ventilator, on oxygen, and sleeping.
At this point nobody even knows what is wrong. They take about 8 rounds of x-rays, but none of the x-rays show a problem. So it's not pneumonia. They have him on antibiotics, but after 2 days and we know it's not pneumonia, I ask them why they are still pumping antibiotics into him if he doesn't have an infection anywhere. They stop the antibiotics. Every nursing shift that comes in has to be taught-by us-not to dump his food into his g-tube all at once-or he'll throw it up which would be deadly on that vent. If we weren't in the room when they came in the first time, they would try to dump the formula in. The doctors want to put Devon on Pediasure to help him gain weight. I lie and tell them he has a dairy intolerance because I do NOT want him on Pediasure after everything I had heard about heavy dairy and SMA children, and I knew they would argue and I wasn't in the mood. They said they didn't want him fed for 24 hours before trying to extubate him, but I didn't want him to go that long without food because of what we'd read/heard about SMA children and ketosis and burning their own muscles, etc. It was a daily fight to try to do what was best for him, and we NEVER wanted to leave him alone in there. Steve and I rent a hotel room and take shifts walking to the hospital and back. I take the day shift, he takes the night shift, so that Devon will never be alone in his room. The hotel is $90+ per night, so we try to get into the local Ronald McDonald house, and finally do. They were a lifesaver.
The doctors have been weaning Devon off of the vent, and after about 4 days he is on room air, no oxygen, and triggering each breath of the vent on his own. They sit down with us and tell us that if he doesn't come off the vent this time, the next time has an even worse chance that it will happen, because he will be even weaker. And they ask us what we want to do if that happens. The main doctor shows us pictures of a 16 year old boy with SMA 1, in his chair, and tells us how he can not even communicate. Then she takes us to the PICU ward and shows us children with a trach, what it looks like, how it works, etc. We tell her that we had decided we could provide a good life for Devon, on a vent or not, and that if he crashed we wanted him re-intubated and would try again, and if that failed, we would trach. She said okay. The next day was the big day. I went to McDonald's and got lunch. I came back in with the bag, and some doctor I hadn't seen before extubated Devon. He came off the vent coughing and crying, so weakly. I held him and tried to calm him down because I knew if he stayed hysterical he would only make himself weaker. I kept saying "He needs to go on Bi-Pap! He needs the Bi-Pap!" but they weren't listening to me and he was so scared and crying so hard I just worked on calming him. But then it happened-he started turning blue, his oxygen levels fell off, and he started crashing. I kept singing to him and talking to him and willed him with everything I had to start breathing deeper and stop crying, but even our "special" song "You Are My Sunshine" wouldn't calm him this time. They threw us out of the room, and the emergency team re-intubated him. I started bawling in the hallway, and Steve held me and let me cry. Some lady had the misfortune of walking by and asked if she could help and I'm afraid I was a bit rude as I just said "NO!" Finally they let us back in his room, and he was sleeping peacefully and back on oxygen.
I spoke to the doctor I knew, and she said that she would be doing the extubation this time, and that she would put him on Bi-Pap immediately. I have a feeling they are lucky we didn't sue for malpractice because Devon was strong enough that first time to survive off the vent and Bi-Pap for 20 minutes...had it been done right, he probably would have come home.
The next week they weaned him back down to room air and to where he was triggering the breaths on the vent again. The time we were there, it felt like we were in a whole other world. One of us always stayed with Devon, and we would watch TV or talk to him in there. I wish I had played with him more. He would wake up, look over, and make sure one of us was there, then he would close his eyes again and go back to sleep. When he was awake, he would wave, stare at his toys, and listen to us talk or read to him. He would smile around the tube in his throat and mouth, and he quickly became a favorite with the nursing staff because of his pleasant personality and bright eyes and cheerful temperament. They would debate who got to care for him that day, and one nurse, Paula, really fought to get him every shift.
The extubation was again scheduled for noon, and I didn't sleep a wink all night while Steve watched over Devon. That afternoon I took several pictures of Devon, because he was in such good spirits and waving and smiling around the tube and talking. I was so scared, because I knew in my heart this was it, that this was his last good chance. But he looked so strong and healthy, and was triggering every breath on his own, on room air, so we felt pretty optimistic. I had called Dr. Bach repeatedly, but nobody had returned my desperate calls. I later found out his wife was dying at that time, and that is why I didn't hear from him.
It is June 21, 1999, and my 13-month-old son is lying upstairs in the Pediatric Intensive Care Unit of Riley Hospital for Children in Indianapolis, Indiana. At the moment, all is quiet. It is late at night, the visitors are gone, the lights downstairs are off, and the hospital feels deserted. Steve is staying with Devon at the moment, so before I go to the hotel to rest for a few hours, I wander into the hospital chapel for the first time in the two weeks Iíve been there. Itís a dark room, and small, and the main focus of the room is a large book at the front of the room, on a pedestal, where people write their prayers for their desperately ill children. I read a few of them, kneeling by the book, and then decide to write my own. Tomorrow is the day. Tomorrow Devon will be taken off of the ventilator for the last time, and tomorrow will most likely be his last chance. Devon had been stronger than average until now, but he too was facing respiratory failure. I think of him, up in his room, hooked up to a ventilator, an IV, an oximeter, chest probes, feeding tube, and everything else he has been dealing with for these last few weeks. And I think of the smile he gave me right before he fell asleep. I write in the prayer book, asking God for mercy, to spare my son, to give us more time, to not let him be afraid or in pain. After spending some time in prayer, I go to the hotel to rest, knowing that when morning came, it would be the biggest day of our lives.
Meanwhile, that night, Steve also went the chapel to pray for Devon. He looked at the prayer book, wrote his own prayer, and knelt to pray for our son. When he got back up he noticed sitting right on the prayer book, a small, lightweight silver cross. The cross was not on the book when he wrote on it a moment earlier. The cross was stamped with ďGod Loves You" and a dove for "peace". He took it as the comforting sign that it was, and kept it for Devon.
Morning came far too quickly. When our doctor came in, I asked her "If he doesn't come off the vent this time, do you think he would be able to be successfully extubated next time?" She said they would try it as many times as we wanted until we knew for ourselves that it wouldn't work. I said "BUT DO YOU THINK HE CAN DO IT? What are his chances?" And she held her fingers very close together and said "The odds are very small that if this doesn't work, he will ever come off the vent."
Steve and I looked at each other, and suddenly we knew the answer. For us, for Devon. He had fought so hard, and hated the machines so much. Steve looked at me for help this time, because he couldn't say the words we both knew. So I took a deep breath, and with my next sentence I sealed our son's death. I told the doctors that if Devon crashes immediately, re-intubate him, because we can't just let him die like that on the table. But if he transfers to Bi-Pap, and can't make it then, we do not want to reintubate. Steve turned away and started crying, and I could hear the rush of air in the room as everybody realized what I'd said. You could have heard a pin drop. Finally the doctor said "okay", and they extubated to the Bi-Pap.
It was successful! He was off the vent, and on the Bi-Pap, and sleeping. I immediately called some people and told them the good news. I felt relief, that this crisis was going to be over, that we would be taking our son home soon, that we had more time with him after all. I was so happy, and tired, and relieved. He had always been so strong, and I knew of much weaker SMA I children who fought off a lot worse situations than this, and survived. I knew he was coming home!
Steve reached down and handed the cross that had appeared to him in the chapel to Devon, and Devon seemed to grab it with more strength than he had ever shown in his hands. It seemed to be formed for his hand-the perfect size so that his tiny hand could grasp it, and lightweight enough that he could hold it. Amazingly, he did not let go.
It was lunch, so Steve watched over Devon while I went to the cafeteria to get some food. I was 2 months pregnant at this time, but had never had any problems...until now. As I sat down to eat, I took my first bite of pizza, and was overwhelmed by nausea (which stayed with me for the rest of that pregnancy). 30 seconds later as I was struggling to eat something, the page came over the loudspeaker that every parent with a child in an intensive care unit dreads. "Will the mother of Devon Stants please come to the PICU immediately? Will the mother of Devon Stants Please come up to the PICU immediately." I left all my food there, barely grabbed my wallet, and raced to the elevator up to his room. When I got there, his heart rate was high, and he was distressed. Steve had thought I might be able to calm him down, but I couldn't. He wanted off of the Bi-Pap when he woke up, just like at home. It was obvious we had a serious problem. He fought against its breathing, and you could see him push his stomach against it. His heart rate started going up, and he was scared and upset. Some stupid nurse came in and whispered to another nurse how she thought the Bi-Pap was a cruel machine and how she never wanted to use it on a baby. If I could remember who she was, I would turn around and go tell her what a horrible thing that was to say in front of us.
We changed all the settings, many times, trying to find one he would work with. We sent someone home to get our Bi-Pap, thinking the familiarity of the machine might help. The doctor said we could try taking it off and see how he did without it. I thought it was worth a try because he obviously wanted it off. But the moment we took it off, his oxygen levels dropped to the 40's and he turned blue. She had to bag him and put the Bi-Pap on immediately to save him. It was terrible. All day long we fought. The doctor knew from the beginning that he wasn't going to make it, but it took us all day to figure it out. I sent out another desperate call to Dr. Bach. The day went by in a blur of beeping and whooshing noises of machines. The doctors offered to provide oxygen and morphine, but I refused because I wanted him to have every chance to live and I knew that once we administered the morphine, it would depress his respiratory system, and it would be over. I wanted to give him every fighting chance, I wanted him to live.
I had been begging to hold Devon from the day he was brought into the hospital 2 weeks ago, and they had always refused. Now the doctor told me I could hold Devon. I said I didn't want to because I didn't want to hurt him. She urged me to. That's when I knew secretly that she didn't think he was going to live. So Steve and I took turns holding him in our laps, just loving on him. Sometime that night I looked at Devon's eyes, trying to talk to him, and he wouldn't look at me. His eyes were glazed in pain and he wouldn't respond to me at all. I started saying "Devon, baby, look at Mommy! LOOK at me Devon! Please, sweetie, look at me!" But he wouldn't. I got the nurse in there and said "please give him something, he is hurting!" So they gave him the oxygen and pain killer, and he immediately started feeling better. I felt so horrible that in our attempts to give him a fighting chance, we caused him pain. As soon as we had given him the pain killer, I went out and made the calls to family, and told them if they wanted to see him they needed to come, now. Most were 7 hours away. My mom and her husband came, and sat in the room with us. Steve's mom & dad came in and talked to him too. Others tried but did not get there in time...We prayed for mercy, we prayed for healing, we prayed for Godís answers and for the faith to sustain us during this time.
It was late, about 11 p.m., and Devon's oxygen levels started dropping even though he was on oxygen and Bi-Pap. Steve gave him to me, and I held him, and as I had been doing all day, all his life, I memorized him. I memorized his scent, the feel of his silky and slightly sweaty hair, the shape of his ears and fingers, the peculiar color of his eyes, just everything about him. I knew that the next time I put him down would be the last, that this was the last time I was going to hold my son in my arms or see him alive. I held on to him like I could ward off death just by sheer willpower. I put him up on my shoulder, his favorite place to be, and I sang him his favorite song "You Are My Sunshine". The nurses took off all of his IV's and probes, and he just had the Bi-Pap on and an oximeter to read his oxygen levels and heart rate. His daddy kneeled next to him and looked him in the eye. Finally, his oxygen levels were very low, and Steve took off his Bi-Pap mask so that he wouldn't have to have that hated thing on when he died.
Inside me I felt a scream, I felt like shouting "PUT IT BACK ON! Somebody get in here and SAVE HIM!" But I didn't. I could feel him taking fewer and fewer breaths, and I could see the heart rate dropping lower and lower. I was so scared that he was hurting, I just kept begging God to take him now, don't let him hurt, just please take him. A few seconds would go by before he would breathe again, and I would just cringe, feeling the breath that I knew wasn't helping. I was just so terrified that he was suffocating to death in my arms and I couldn't save him and I just begged that he not be hurting. I could feel him getting heavier in my arms, and again I wanted for somebody to come in and save him. He would open his eyes, and make sure Steve was there, and then close his eyes again. I sang his favorite song to him, over and over, even though it was mostly humming by then because my throat had closed off. I whispered how much I loved him and how it was okay for him to go now. Then it was over; I knew the moment that it was, and I knew my son was gone and I was not holding him any longer. That's when I had to see his face, I had to know. So I brought him off of my shoulder, and looked into his face, and it wasn't scared or hurt-he only looked calm and peaceful.
The doctor came in, listened for a heart beat, and noted the time of death. She said we could stay in there as long as we needed. I continued to hold him, because I wanted to be able to hold onto him for the rest of my life. I knew once I laid him down, it would be forever, and I wasn't ready for that. I held him for another half hour or so, and when I went to put him down for the last time, I noticed his fist tightly clenched. I had never seen him hold an object tightly in his life, so I looked closer, and saw the cross Steve had miraculously found. It almost isnít possible for an SMA baby (or any 13 month old!) to grab onto an object, and hold it tight for 10 hours or so and never let it go. But he did. Devon died with that cross tightly held in his fist. ďGod Loves You. Peace". Devon left this world in his mommy's arms looking into his father's eyes. He entered the next world in Jesusí arms looking into his heavenly father's eyes. I thank God for the peace I have knowing that.
I called the nurse in and said we were ready for them to take him. She told me just to lay him down in the bed and they would watch over him until the funeral director arrived. And I knew they would watch over him, because as we had all realized he wasn't going to make it, every nurse and doctor in the room was crying along with us. So I laid him in bed, and he was just so beautiful and peaceful, and I would swear he had a healthy golden glow in his skin. He looked like my precious son was just taking a nap. Steve and I then took each other's hands, and did one of the hardest things in our lives-we left the hospital room, leaving our son behind.
When we got to our Jeep, Devon's car seat was in the back seat, strapped in. But no Devon. It was 1 in the morning, and as we drove home, we were past the point of exhaustion and numbness. The next day family came, and Steve prepared Devon's body for the funeral. I didn't want to do that because I wanted my last memory of holding Devon to be the one I had. Steve wanted to because it provided peace and closure for him-something he could do for his son. We had an open casket service so that his family and friends could say goodbye, but the boy who was in that casket did not look like the boy I had laid in the hospital bed. He had lost that beautiful glow, and they had makeup on his face. It was just another piece of proof to me that he was not here, he had been in Heaven, whole and perfect, for days now. The service was perfect. I can remember everything that happened, but only about a third of the people who were there. I got up and read "I Promise", the song/poem by Cheri Potter, as those were the words that were running through my head as I held him in that rocking chair on my shoulder as he died. His cousins (ages 7 and 5 I think) sang "Jesus Loves the Little Children", and his uncle Lon gave the message. Afterwards everyone was handed a white balloon, and as we went to the graveside, after a prayer, we released our balloons into the heavens. It was our way of saluting Devon, and of releasing him too.
Afterwards we came back to our house, about 50 people were here. I got out my 5 photo albums full of pictures of him so people could look through them, and we watched video tape of his life. Laughing, playing, smiling, having a wonderful time. It was a celebration of his life. Now all those photos I took were appreciated. I just wish I had more, because soon photographs become your memories, and you can never have too many memories.
Then everybody went home. That's when the real heartache begins. There is no more cushion, there is no more acting sane for other people. It is just you, alone, with nothing left but memories, clothes, and toys. We put pictures up of Devon all over the house. I always wanted to see his face, to make sure that I kept him memorized, that I didn't forget a thing.
So how does this end? Well, it doesn't. It's a daily journey, and will be for the rest of our lives. We will always be missing a son, a member of our family, no matter how many decades pass. People will ask "how did you do it"? Well, you don't have a choice, that's how. That's not true, you do have a choice. You can choose to continue to live life, enjoy life; or you can choose to bury yourself with your child. We made a conscious choice that we would honor Devon's life and his memory with only happiness, not despair. We wanted to remember the 13 months of his life, not just the one day of his death. We wanted his memory to be a joy to us, not a tragedy, because we didn't think that would be fair to him. His life was full of joy and love. No matter how the above story sounds, he had many more good days than bad, and even the "bad" days weren't bad for him, mostly for us! I am thankful for that, too. He really did have a wonderful life, and he was a happy boy-almost 90% of his photos he is grinning at me! His whole life he was only surrounded by people who loved him, and he knew it! So for him, and for us all, we made the choice to continue to live, to help others if we could, to be joyful, and to never take life for granted. We know he is looking down on us from heaven, with his gray eyes smiling, happy and whole and healed. And we know that some day we will see him again.
the days passed, Steve and I realized that God had indeed answered our
prayers, even if they werenít answered in the way we wanted.
We prayed for Devon to be healed.
Now he's standing like a child should, and in awe at the beauty
of his surroundings.
He has been completely healed and has earned the crown without the
prayed that Devon would not suffer.
For most of the 13 months of his life, he was healthy and happy,
which is more than many SMA children get.
We prayed that when it was his time, Devon would go peacefully, with
At the very end when he was on my shoulder, looking at his father,
holding onto that cross, surrounded by his family and us all telling him
what he has meant to us, I can honestly say his face was extremely peaceful.
He did not look sick or sad or hurt any more. He looked like he was
totally contented, and when he fell asleep for the last time he went from
his favorite place on earth surrounded by his most loved ones to his own
place in heaven surrounded by the only One who could love him more than we.
Our prayers have been answered, our son had a remarkable life, our own lives are irrevocably changed for the better, and our faith by trial has been bolstered by the knowledge that God sent a sign of peace and promise to us and our son-in the form of a perfect cross that a weakened child grasped on to for the last 10 hours of his life. ďGod Loves You. Peace.Ē
One more thing I need to mention. This story is just how I felt, how I remember. But the two most important factors in all of this, even though I don't mention them nearly enough in here, the two things that kept me waking up in the morning, smiling for my son, enjoying the days even when they were hard, surviving the death of my child, these two things were faith in God, and the love and support of Steve. If I didn't believe in a God who loved me and my son, who grieved for our pain as well; if I didn't have faith that there was a heaven that Devon was headed to where he could be whole and healed and free; if I didn't have that, I could not have survived this. And secondly, Steve was a full and complete partner. He helped me make all the tough decisions, didn't judge me for any failures, helped me be strong when I had to be and let me be weak when I needed to be. He cared for his son as gently and lovingly as anyone can picture, and was about the best father and husband I have ever seen. The hard times I mention just using "I", were not just "I". They were "We". And I could not have and would not want to have tried to do this without him.
For now, I guess this is where this ends. We can see pieces of Devon in his little sister, Kaylee, and we enjoy the living reminders. And he will live on in our minds, our hearts, our family, and our daughter's eyes.