I knew I would have a precious baby boy when I looked at a picture of my husband, John, at three years old. I saw the black and white picture soon after we were married, and I couldn’t take my eyes off of it. I just stared with amazement at my child. I knew I would I have a boy, and he would look just like his father. My in-laws gave a copy of the picture to us, and I would gaze at my Conner.
Becoming pregnant two and a half years after getting married was a dream come true for me. Being pregnant was everything I had ever imagined it would be. I loved everything about it-staring in the mirror at my beautiful, big body and being adored with such purity. With the exception of swelling at the end of the TEN months, everything was routine. There was a definite decrease in fetal movement but I had absolutely no way of knowing it was abnormal-it was my first child. Earlier in the pregnancy, I had been doing the “kick count”. It wasn’t adding up right, but I thought I was not perceiving and recording accurately.
More than spending my whole life wanting a child, I spent twenty years wanting a complete family. After my father died when I was eight, I aspired to have a complete family again someday with a mommy and a daddy and, one day, a child or two or three or more. John and I just bought a house before I became pregnant. We already knew when we moved in which room would be the baby’s. Soon I found out that I was pregnant and I knew that my lifelong dream to be an important member of a happy, complete family had been achieved. I was home free; I could see my perfect life just over the horizon. I would sit in the baby’s room and rock in the chair (I would eventually not be able to rock Conner in) and cry at the accomplishments and progress that had brought us to the happiest moment in my life.
On July 13, 1999, at 1:07 p.m. I had the most beautiful, huge baby boy. John Conner Riggs, Jr. was born weighing 9 lbs., 2 oz. with a hairy, black head. Conner scored high on the Apgar tests. He immediately breastfed. He was my perfect, big baby. I was and will always be so proud of him. And he did look just like his daddy. Conner cried but at first I could always soothe him. I stayed home with him for 11 weeks-as long as I could. I had to learn, but it didn’t take long for me to be Conner’s “Mommy.” I felt so special and proud. That was my moment and measure of success.
By the time I went back to work we all noticed, mostly unspoken, problems with Conner. We nicknamed him, “Floppy,” having no idea that his floppiness would be the cause of his death one day. While everyone else was concerned about Conner’s muscle control, I was stuck on his eating. I thought the transition to formula after a month of breastfeeding caused problems. I thought that we were going to be like so many other new parents who were having a hard time finding the right formula. What our problem was, which was different from others’, was that Conner wouldn’t even get the food into his stomach. He cried when I tried to feed him. So, if it were intolerance to the formula, it would have at least made it to his tummy to not be tolerated.
Well checks came and went, and I just figured if anything were really wrong, a doctor would have told us by now. By Conner’s second month well check, though, we were all deeply concerned. I, being really preoccupied with the poor eating habits, persisted in asking the doctor about the weak muscles and the eating problems. At the instant he put the two together, we were referred to a neurologist who would see us the following Tuesday.
It was the beginning of testing and waiting with sick, uneasy feelings in the pit of our stomachs. It was that day sometime in mid-September, I believe, that we first heard of the disease, SMA. Conner was being tested for muscular dystrophy and SMA type 1. I looked up both on the Internet and saw that SMA type1 children die around the age of two. So, it wasn’t that. It was some type of muscular dystrophy, right? Okay, I thought, my child won’t walk. We will enrich his life so that he does more than he would if he could walk. We’ll take him everywhere. We’ll teach him everything. He’ll have such a full life; it won’t matter that he can’t walk. I could deal with that if Conner could. We didn’t know anything about muscular dystrophy either. But what we learned a couple of months later was that he did, in fact, have Spinal Muscular Atrophy type 1. We learned that he would need delicate, specialized care if he were to live for any length of time. Even with all the special care that I needed to learn before we even left the Pediatric ICU that Christmas of 1999, he may not make it through a cold.
I thought I was going to have a family! I thought all my dreams were going to come true. WHY??!! Why was my child going to suffer and die before our very eyes? I don’t want to hear that everything happens for a reason because NO reason is worth the suffering of MY beautiful, innocent child. I would rather die or live without the lesson than watch my own precious baby live and die because of this disease. What is the lesson to Conner to have to suffer and die so young? That is the question. It is not about what I or John or anyone else is supposed to learn, it is about CONNER and his life and what he gets out of it.
That is my purpose for now. I am going to make Conner’s life as comfortable as I can while he is here. He may not go many places, and he may not see many things but he will be comfortable and he will be loved. So many people have helped us to make Conner more comfortable, and I can’t thank this community and our family (grandparents!) enough.
On top of the devastating news about Conner’s diagnosis, we further learned that if I became pregnant again there is a 25% chance of having another diseased child. Even worse, I don’t appear to be a normal carrier, so any pre-testing would not be helpful. Even if we could afford the process of pre-implantation diagnosis, my gene would appear to be normal and not signal a diseased embryo. The only hope John and I have of being certain that we would have a healthy child is to find the mutation(s) on my gene (through looking at Conner’s gene) and be able to pinpoint the very place on the gene where there is a “mess-up.” We are actually in the process of having that done. But we may never get results. I don’t know that the fertility specialists that do pre-implantation diagnosis would be satisfied with the uncertainty we offer. We are not that far. We can’t consider having any more children at this point for many reasons, which deeply hurts me inside. There is a shred of hope for children one day in the future-just not a thought for now.
I do not have any judgmental feelings at all for other families who proceed with pregnancies despite the 25% risk but we are not so courageous. This is just our decision, and I don’t think that means that everyone should feel this way. Thank goodness considering I have seen and heard of others having such beautiful, healthy children.
Because of the mutated gene issue, the diagnosis was a bit confusing to us in the beginning. Conner’s first hospitalization was prior to us receiving any test results. In fact, I believe we ended up in the hospital the day Conner went to draw blood for the DNA test. He wasn’t eating and they wanted to watch him. He did okay at the hospital. I don’t know why. He would hardly eat at all at home but at the hospital he seemed to get down enough. Conner didn’t gain any weight for a long time. We couldn’t break fourteen pounds for so many months, yet he was so big when he was born. The gastroenterologist and the neurologist kept suggesting SMA type 1 to us but we couldn’t believe that Conner had this disease. We needed proof. I didn’t want any contact with any other families until I had proof. I can remember feeling so sorry for the other families and thinking that wasn’t us and it would never be us.
When the neurologist delivered the news to us that Conner’s DNA tests and the Muscular Dystrophy tests came back normal, we then were going to await the true diagnosis. We knew that there was a chance that the DNA test could come back normal and Conner still have the disease but we felt that was too rare and not the case with us. So what could it be? We had to put Conner through the painful process of the EMG- which to this day I wish we would NOT have done. Why? Why did we do that stupid, painful test that did not tell us anything WORTH sticking needles into all of Conner’s muscles. At least someone should have put emla (caine medicine) on to numb his skin or something! With the muscle biopsy and further DNA testing, there was NO reason for the EMG. We shouldn’t have even done the muscle biopsy until we exhausted the DNA testing. I would have chosen to do neither and regret doing them both.
Anyway, Conner went to Egleston/Scottish Rite to get the muscle biopsy. He did so well after the surgery. Now looking back it was because he was intubated and it had cleared up his secretions. Of course, it only took a few hours for him to get clogged again and stop eating. Little did we know all the equipment that we would need just to keep him comfortable. We had asked the doctor in Atlanta repeatedly about the congestion in his throat and heard not one word about assistance or a suction or anything about making him more comfortable or that he could die in my care from choking to death if we didn’t know how to position him or clear him. I would intuitively put his head down and beat on his back and let the junk drip out but I didn’t know what I was doing or why.
On the way back from Monroe, GA to Statesboro, through tiny back road towns, Conner had increasingly become lethargic and eventually threw up when I took him out of his car seat. I didn’t know it but he was slowly strangling on his own spit and probably was for hours or days or weeks. I ran him through the emergency room doors in the tiny town hospital. I didn’t realize it at the time but all they did for him that night was give him motrin and suction him. That was enough to get him home and at 5:00 a.m. the next morning we were back at the emergency room in Bulloch County, GA. It took about three days before they finally contacted Savannah.
When Conner reached Memorial PICU we finally realized what we’d need to do for Conner to, at least, be comfortable and, at most, live a year or two longer. We learned, of course, that the DNA tests didn’t matter. Conner has all the characteristics of SMA1 and we better learn what we need to know if we want any more time with him. We did. Conner got his g-tube and nisson surgery with that stay. That was last Christmas. My full-time (and over-time) job became being a nurse to Conner. So many people were concerned and showed us how much Conner is loved. That was so inspiring to us. I can’t tell you how much it meant to us to know that we had so many thoughts and prayers upon us at that time and ever since.
It was then that I finally called the Swanson’s. I am thankful that they were so open to us. We actually took Conner to their house and met their twins one day. They looked so much alike-Conner and the girls; we must assume the Skylar and Cassidy look alike, right? Of course, it didn’t take the DNA test to convince me at that point that Conner has SMA. We did get further results after we were all tested which showed that John carries the deleted gene, and I carry the mutated gene. Conner received one deleted and one mutated, consequently, giving him the disease. No one in our families had any history of a muscle disease.
I can remember driving to the doctor’s office after getting the results from that first DNA test. I remember feeling sick to my stomach. John, his mom, my mom and I drove 50 miles to Savannah. No one really said anything the whole way. I just couldn’t believe what was happening and that the situation had become this serious-life or death. When we heard his test was normal I told the doctor I just wanted Conner to live. I didn’t care about anything else. When we got the results from the muscle biopsy, over the phone at first, I was told that Conner definitely did not have SMA. We spent that time with a false sense of assurance that Conner would at least live.
Everyone else, the doctors and nurses in PICU, may have known the truth but we had held on to every shred of hope until we couldn’t anymore as Conner lie in front of us so lifeless and sick. I guess that’s what you do when you’re moving through denial. I can’t explain how hard it was to come to the realization that our precious, perfect baby may not make it home, or live a month or a year. How could we hope for longer than that?
We didn’t even know our child anymore. The mommy I had become to Conner was now a mommy that couldn’t even soothe her baby. I was afraid to hold him, and every time I touched him it was to do something discomforting, like stick a catheter down his throat. I felt like we had to get to know a new child-this new serious child. Our Conner, the one we had dreamed of our whole lives, was already gone-the dream of the perfect child and loving, happy adult gone. We had to meet the new and needy, sick Conner. Little did we know how perfect he would be.
Conner is so precious to us. He has dark golden, curly hair and a mysterious color of green for his eyes. Conner is assertive in his desires. (Nice way of saying bratty!) No, he is sure of what he wants. He wants VIDEOS! He loves veggie tales and Elmo. He loves A Bug’s Life and A Toy Story. He likes to be massaged and rubbed. Conner loves to ride in the van. But he loves his daddy the best. When John walks into the room Conner’s eyes light up like the sun and a smile glides across his face. I think Conner has the severe masculine characteristics of his daddy and uncles so mommy just doesn’t cut it. Daddy, granddaddy and uncles seem to provide the best, most interesting conversation and entertainment.
Everyday I weigh Conner’s comfort with his suffering. I promised God and myself that when the suffering was overshadowing the comfort, that we would evaluate our extreme efforts to keep Conner here on earth. We do not want to keep him here for us. We want what is purely best for Conner’s soul. At this point, Conner’s will to live extends beyond any decisions that I need to make. He has deteriorated extensively throughout the past year and is using his breathing machine most of the time. I would be doing him a favor to keep it on all the time but, for my own sake, I like to see him off of it. That is what I said I wouldn’t do. So, everyday I wonder, how long? How long do we decide we want him to live? Or do we wait for him to get so bad he is suffering all the time?
Conner’s care is certainly a labor of love. Before he awakes in the morning, I have turned him several times throughout the night very gently so I don’t wake him and hooked up some food from his pump to his g-tube. When he wakes we start his nebulizer albuterol mist then beat on his chest on all four lobes with a face mask taped to a couple of tongue depressors, courtesy of Memorial PICU. He takes part in the treatment by holding the nebulizer and every morning we say, “Conner does it all by himself!” He likes that.
This process loosens the junk in his lungs and helps mobilize the secretions. Next, we use the inexsufflator to inspire about 40 lbs. of pressure into his lungs and expire the same. This is a cough for Conner. I suction whatever is pulled up. We do this until Conner is clear in his lungs and throat. We can tell if he’s clear by listening with a stethoscope and just looking and listening to him externally.
When Conner is sleeping or if he appears to be getting exhausted from breathing on his own (increased pulse rate, low O2, crying, paleness can be indicators) we put on his bi-pap (breathing machine-inspiratory air pressure and a little expiratory help). This fits on with a mask and a spandex, superhero-looking cap. Conner used to hate all that we did to him. He now expects it and shows his dislike to putting the cap on but once he’s set, he’s so content and comfortable. We don’t know that we have all our machine settings completely correct or his diet just right but we look for Conner’s comfort level. I feel confident that we have all we need in this house to make Conner as comfortable as possible.
He is on a pretty regular schedule, which helps a little to measure comfort. He wakes, treatment, food, videos, and nap. Wake up, move to the front of the house, play, read or videos, food. Visit with granddaddy. Treatment, food, video, bi-pap, sleep. Same day over and over with the exception of nurse visits, therapy, or doctor’s appointments. This is a good day, though. Bad days are exhausting on Conner, me and his daddy. I am tested to the point of wanting to hit my head against a wall or pull my hair out. I don’t understand what to do sometimes, and I know there is no one on earth that can answer my questions because there are no answers. The answer is that life isn’t fair. That can be a very frustrating pill to swallow at four in the morning when Conner is crying for reasons that all of my sma experience can’t figure out only to have a cycle of poor breathing and no sleep and, of course, crying continue on for hours or days until an angel reaches down to give you a rest.
What hurts is to see Conner struggle to breathe or get clogged and turn blue. We try to catch him before he chokes or clogs and gets pale but it happens so fast sometimes. As the year has passed, he is no longer able to be inclined for just about any time at all. His head must always be to the side or he chokes. He is either on his back or to the side or just a little in between. Holding him is hard on him but every once in a while we try to do it. I snuggle up to him or give him a squeeze to make up for the inability to hold him.
Conner is now 17 months (as of Dec. 2000). He moves his fingers enough to sign, “I love you” and that he is one year old. He can wave “bye, bye” with the wrist and fingers. Other than this movement with his forearms and wrists and fingers, he moves nothing else but his eyes. He can speak enough to say, “Dadadada” or “Naaaaaaaaaaaahhhh” or “AAAuuuuuuuhhh.” He uses his eyes to communicate most of the time. He speaks best on the bi-pap (breathing machine). We think we get an “Uh-huh” for a “yes” sometimes. If he could get old enough to really communicate his needs, we’d feel much more at ease in caring for him. For now, we will continue to find the smiles that brighten our days. We’ll listen to him talk and react to his silly videos. We’ll turn on his disco light and watch his satisfaction. And we’ll take the best care possible until God steals him back from us.
Of course the obvious lesson of Conner’s life would be to appreciate everyone and everything. The problem is that we are human beings trying to satisfy ourselves as well as the ones we love, and it automatically means that we behave in ways that appear unappreciative. The less obvious lesson is to see your days as part of a much larger picture. In order to handle losing Conner from this earth, I have to imagine that I won’t really lose him at all. I have the opportunity, since I know he is going to die soon, to make such a deep connection with his soul so I know we will always be together. I may not do the right thing or I may fuss with John. I may say something snotty or not work as hard as I should. BUT I can tell you that everyday is a day that I get spiritually closer with Conner and all that ensures that he and I will meet again at the end of my life. That is my lesson, but I will never be convinced that the means will be justified by whatever “end” develops further in our lifetime.