5th Edition - EMMA HOPE NEWSLETTER
This is the first Emma Hope Newsletter since the one I wrote right after Emma was born. I never planned on writing another because I was confident that the perpetual drama and excitement we experienced while she was in utero wasn't going to be repeated. Emma was going to be a perfectly normal, boring child, worthy of just the run-of-the-mill Christmas letter and nothing exciting enough to warrant another Newsletter was going to happen until she was in high school and making headlines as the star quarterback of the football team. Oh well.
2005 started off with more drama and excitement than we were prepared for. Emma's legs seemed to be getting even weaker, and although most children her age were running around the house and learning how to climb, she was still struggling with crawling. We expressed our concerns to her therapists and eventually to her neurosurgeon. He ordered a CT scan and a series of X-rays to check and make sure that her shunt was still functioning properly and not in the wrong place and interfering with a part of her brain controlling motor skills. On Friday, February 11th, we had a meeting with the neurosurgeon to go over the results. Everything looked perfect, he said, and there was no evidence that anything was wrong. He had another possible explanation for her condition - "tethered cord syndrome." He told us that sometimes some scar tissue forms near the base of the spine and it grabs hold of the spinal cord. As the child grows, the spinal cord gets stretched faster than it can grow and eventually gets damaged. Symptoms worsen as the child grows, and complete paralysis is the end result. The condition can be corrected relatively easily with surgery, but the surgery itself was going to mean a week or so with Emma in a hospital bed unable to move. We were seriously conflicted about this. It was certainly nice to have an explanation and a cure for her leg weakness, but we were absolutely dreading the possibility of more surgery and a traumatic and painful recovery.
My mother had flown out from California and had been visiting with us for a couple of weeks, and we had brought her to the doctor's appointment in Hartford so that my father wouldn't have to travel all of the way to Bristol to pick her up. He was going to bring her back to his place in Salem where she would stay until her flight out of Boston would bring her back home. We said our good-byes at the doctor's office, my parents headed north and Amy, Emma, and I headed home to Bristol. Shortly before we arrived home, Amy noticed that something was wrong with Emma. They had been talking and playing games just moments earlier, and suddenly Emma's demeanor had changed. There was panic in Amy's voice as she tried to describe what was happening as she was climbing into the back seat to attend to Emma. Seconds later, Amy screamed for me to pull over - she thought that Emma was choking on something and not breathing. I gunned the engine, turned left onto a side street just two streets before ours, pulled over to the side of the road, and got out. I unbuckled Emma from her car seat and pulled her out of the car faster than anyone had ever done so with a child before. Emma was still breathing, but something was seriously wrong and we didn't know what it was. I opened her mouth and found nothing. As I looked at her, I realized that she was completely unresponsive. Her body was limp and her eyes were glassy and focused on nothing. Amy found her cell phone and called 911 within a few seconds, but we still didn't know what to say when the operator answered. Amy started to describe the situation, but little useful information was conveyed with all of the panic-ridden screaming, so I took the phone from her. Emma's breathing was becoming labored, and it sounded like there was a lot of fluid in her throat. I talked to the 911 operator with the phone on one ear and Emma's chest held up to my other ear. I gave her our location and told her that something was seriously wrong with our baby. Even though it seemed like far too much time, within a minute or so we heard a siren. Amy ran to the main street to flag down the police officer that was on his way. He arrived a few seconds later and jumped out of his car. After a quick visual examination of Emma, he said that she was still breathing and he asked us what was wrong. I told them that her breathing was labored, but more importantly, her body was limp and that she was completely unresponsive. He still didn't seem to see any problem and he asked me to put Emma down on the seat of the car so that he could look at her. I did as he asked, and he moved in for a closer look. Just as he did so, the most frightening thing I have ever seen in my 35 years on this earth happened. Without moving a muscle and still staring off into space, Emma vomited a disturbingly large amount of white foam out of her nose. Amy started screaming again, shouting "she's dead, she's dead!" and the police officer immediately spoke into his radio. I don't remember exactly what he said, but I remember the panic in his voice. I told him that if the ambulance didn't arrive in the next few seconds, I was going to bring her to the hospital myself - we were only three or four minutes away. He told me that it would be here soon, and he put the oxygen bottle that he had carried with him up to Emma's face.
He was right, and the ambulance arrived a few moments later along with another police cruiser. Two paramedics got out and immediately attended to Emma. I'm having trouble remembering exactly what happened next, but we were in the ambulance and on our way to the hospital shortly thereafter. The two paramedics were in the back of the ambulance with Emma and me, and the first police officer was driving with Amy in the passenger seat. Emma was strapped to the stretcher and she had an oxygen mask on. I was giving Emma's hopelessly complex medical history to one paramedic while the other was on the phone with the ER doctor. I understand that a paramedic isn't going to have experience with every type of condition, but I was becoming increasingly frustrated at the fact that he had no idea what I was talking about. Thankfully, it turns out that the ER doctor on the phone was someone that Amy and I both knew and respected, and he got the paramedics on the right track. At some point around this time, I finally heard the word "seizure." Hearing that word, as terrible as it was, put me at ease because I had previously thought that I had just witnessed my child become a vegetable. As if to confirm the diagnosis, Emma's arms started flailing shortly before we arrived at the hospital. Once we got there, we were immediately ushered into a treatment room along with the ER doctor that was on the phone with the paramedic. In the ambulance, the paramedic had given Emma a shot of Ativan at the doctor's direction in an attempt to stop the seizure. She was still seizing by the time we got to the treatment room, so he gave her another one. It was hard to determine when the seizure stopped because Emma completely lost consciousness in response to the drug, but we were told that the seizure did stop shortly thereafter.
As soon as things calmed down, I called my parents and told them what had happened and asked if they could turn around and come back. They did, even though they were only a few minutes away from home and about two hours away from us. Emma's first pediatrician, a doctor that Amy and I adored, had left the practice a few months earlier to work for a new pediatric program at the very hospital we were at. Thankfully, she was there, and the ER doctor had her come see us within ten or fifteen minutes of our arrival. She knew Emma's history as well as anyone, and it was very comforting to have her once again in charge of Emma's care. Together, we all decided that it would be best to transfer Emma to the Children's Hospital in Hartford, the CCMC. One of the doctors summoned an ambulance from the Hartford area, and it arrived about a half-hour later with a doctor from the CCMC. We called Amy's cousin Shannon, who lived just down the street, to come take Amy to Hartford while I rode in the ambulance. This time, I rode in the passenger seat while the CCMC doctor and the two EMT's rode in the back with Emma. If I wasn’t turned to watch what was going on in the back of the ambulance, I was watching the road in front of us, completely horrified at the way other drivers act while an ambulance approaches them from behind. I had always thought that I would make a good ambulance driver because of my driving skills and my ability to go fast and still drive so smoothly, but I quickly realized that it wouldn't work out because I would get fired on the first day for ramming someone intentionally. It's as if peoples' brains shut down when they hear a siren and see flashing lights behind them. The worst incident happened as we neared the rt.72/I84 split on the highway. It's a fork in the highway, with two lanes for rt.72 going off to the left and three lanes for I84 going to the right. There was a driver in front of us that wanted to go on I84, the same way we did. As we neared him, he pulled to the left (heading for rt.72) in order to let us pass. There were few other cars on the road with us, and God knows why this idiot didn't pull to the right, as logic would dictate. He was still in front of us and to our left as we reached the split, and at the last possible moment, he swerved two lanes to the right, missing the front of the speeding ambulance by no more than a few feet. If I had a gun, I think I would have opened the window and shot him.
We all arrived at roughly the same time, and my parents were already there waiting for us. We had a very large treatment room all to ourselves, and for quite a while there were at least a half-dozen medical personnel attending to her along with the five of us. Amy's mom showed up shortly thereafter with a friend. Medically, Emma was doing all right, but emotionally it was a different story. She hadn't eaten or had anything to drink for many hours, and the nurses were having significant difficulty trying to insert an IV into her chubby little arms with no visible veins. Emma was in full-scream mode, the stress level was high, the medical staff didn't appear to be functioning at 100% of their ability, and Amy was unleashing one verbal bitch-slap after another as two young candy stripers huddled in the corner, too frightened to move. Our pediatrician had sent us to CCMC with the expectation that we would stay until we got an answer as to why Emma had a seizure. Unfortunately, that meant many tests, many more hours, and a great deal of sedation for Emma. We decided that was too much for her. Emma got another CT scan and a shunt series (X-rays) to make sure that nothing had moved since the last one, but that was it. An MRI and an EEG was desired, but we decided to bring Emma home and schedule those tests for early the next week.
We did just that. The weekend was uneventful and on Monday we scheduled the MRI and the EEG for first thing on Tuesday. Everything went smoothly and Emma was her usual, semi-cooperative self. Believe it or not, but there is still some confusion as to exactly what tests Emma received that day. Ideally, she was going to have an EEG to find out if she had epilepsy and an MRI of both her head and her spine so that we could see if there was a cause for the seizure and see if she had tethered cord syndrome. That day, even though a doctor had previously ordered an MRI of her spine, we were told that she was only going to get an MRI of her head and that there wasn't time for both. We would have to come back another day for the MRI of her spine. She had the EEG first, and that went well. The MRI came next. We later found out that she ended up having an MRI of her spine and not her head, because her neurosurgeon and her neurologist both later told us that Emma did not have tethered cord syndrome. Of course, the neurosurgeon couldn't find the films when we asked, and when we went to pick them up at the radiology department of CCMC, we were told that the films had been sent to a general surgeon that we had never heard of. It's an issue that remains unresolved today and we hope to get it completely cleared up this week. The EEG results came back giving us no explanation for her seizure. Her neurologist told us that it was quite common for children with hydrocephalus to have seizures. The surface of the brain gets a little squished while it is developing because of the internal pressure on it, and that interferes with its development. The CT scan showed that the surface of her brain looked much like that of some other children that were prone to seizures, and also hypotonia, especially in the lower extremities. When we asked him what we could expect, he told us that it was impossible to predict. She may have another seizure tomorrow, or not until next year, or maybe never again. If she had seizures regularly, we would put her on some anti-seizure medicine. In the meantime, he gave us a prescription for some medicine that we were to give Emma rectally if she had another seizure.
Throughout all of this, we were also aggressively trying to get Emma some more therapy for her legs. We ended up signing her up for "aqua therapy" at the pool in a nearby rehabilitation hospital. There was no doubt that she would enjoy that since she liked her swimming lessons so much. Sure enough, during our initial consultation, we took a tour of the facility and Emma lunged for the pool when she first saw it. She complained when we left that first time, just as she's done for every other time we have gone. We also started investigating getting Emma a "stander." Since she wasn't standing or walking on her own, she wasn't putting any weight on her legs. She was in danger of losing bone density, just as astronauts do when they are in zero gravity for extended periods of time. A stander is a rather large device that we strap Emma to and try and keep her in for a few hours a day - about the same time as an average child spends on his feet. We talked about them with her therapists and made an appointment to look at some at the physical therapy department at CCMC.
On Wednesday, March 9th, Emma went with me to our friend Liz's school to judge a science fair with her husband Dave, which is something I've done for the last few years. The day went great, Emma behaved wonderfully, met lots of new people, and chowed down on cottage cheese and juice boxes. When it was over, Liz took us all out to lunch at the Old Saybrook Fish House on rt. 44. Dave's mom was already there with Dave and Liz's son, Christopher - Emma's best friend. Emma sat in a high chair across from me. Just as we got our drink order, I noticed something different about her. As if to confirm my suspicion, Emma immediately barfed up a good portion of the cottage cheese she had earlier. Her eyes took on that glassy appearance and she became unresponsive. It didn't appear as bad as the first time because her body didn't become completely limp. She was still able to sit up, and she actually moved around as if everything was O.K. There was no doubt for me that she was having a seizure because of the look in her eyes and the fact that she didn't respond to anything at all - her movements were completely random. I brought her over to the bench with me and prepared to give her the medicine while someone else called 911 and asked for an ambulance. By the time I had given her the medicine and finished pulling her clothes back on, an EMT was standing behind me. Later, I asked the EMT to read the log to me because I knew I would be asked a dozen times that day what time everything happened. The 911 call happened at 2:22. The ambulance arrived at the restaurant at 2:24. I went for yet another ride in the back of an ambulance with Emma, this time directly to CCMC. While en route, I called Amy and asked her to meet us there. We also met another ambulance on the way and picked up a paramedic in case Emma needed more intense care. Emma stopped seizing a few minutes into the ride. The EMT knew exactly when because he was continually trying to get her to reach for a small stuffed animal. He declared the seizure over when she focused on it and swatted it away. Emma did O.K. for most of the ambulance ride, but I remember the paramedic shouting to the driver to speed up when we were still a few minutes from the hospital and Emma barfed up the rest of her cottage cheese.
At the hospital, the doctors ordered yet another CT scan and a shunt series. The X-rays were successful, but Emma would not cooperate for the CT scan. She needed to be relatively still for about a minute, and try as we all did, it just wasn't going to happen. She wasn't about to go to sleep on her own, and we weren't about to let them stick her arms with so many unsuccessful attempts just to give her yet another round of anesthesia. We opted for a mild, rectally-administered sedative, but she powered through that bout of drowsiness like a trucker on amphetamines. Shortly thereafter, we decided to take her home. We had all had enough of this place and these machines, and Emma's neurologist had given us a good explanation for her seizures, so we didn't see the need for any more testing. We got a prescription for an anti-seizure medicine, Trileptal, from her neurologist, and we started her on that a couple of days later. She has not had any more seizures since then, and we have not noticed any side effects with the drug.
The visit with the physical therapists at CCMC to look at standers on a Tuesday went very well. After a quick evaluation, we first put her in this giant wooden contraption that was about the size of a small table on its side. She liked it because it had a big wooden tray with a plastic bowl in it to put stuff. We didn't because it was the type of thing that one just sets up in a room and leaves there. Even though it had small wheels on the bottom, it was too big and bulky to move around much. The next stander we tried was a "dynamic stander." It was made of tubular steel and looked like a small wheelchair but with a platform to stand on and a pad to lean against instead of a seat. It even had the racing-style spoked wheels. Emma didn't seem to see any advantage with this one over the last one, but then we showed her how she could move it. At first, she put her hands on the wheels and moved them back and forth, not going anywhere. Then we showed her how to push forward and let go, push forward and let go... Within thirty seconds, she was wheeling herself all over the department. I don't think I've ever seen such pride. In a matter of seconds, Emma went from crawling to standing and moving. The therapist told us that insurance companies usually balk at the higher cost of the dynamic standers, but we didn't care. Seeing Emma smiling and beaming with pride made up our minds for us, this was the stander we were getting. We weren't willing to wait the normal two to three months to get one, either. I got to work on the phone and after a few days of going back and forth with the doctors, the therapists, and the insurance company, I discovered that there was a good chance that insurance would reimburse us if we went out and bought it ourselves. The following Monday I ordered it directly from the manufacturer and we received it that Wednesday. Yes, it cost more than the truck I just purchased. Most of the time Emma can tolerate it for only a half-hour or so, but sometimes, if one gives her something to do, she'll stay in it without complaining for over an hour at a time. We've already rearranged the house to give her some more room, now Emma just needs to learn how to steer a little better.
We have another MRI appointment coming up soon to check on Emma's legs again, and hopefully that will be her last one for a long time. At least Emma doesn't seem to mind any of this excitement. She enjoys her therapy visits and absolutely loves her aqua therapy sessions. She gets to play with her cousin Sophia and Christopher on a regular basis, and she gets to hang out at the dyno shop with all the guys. Her ever-improving language and cognitive skills amaze us every day - she even calls her mom with her dad's cell phone with the speed-dial. Her regularly used vocabulary includes about a dozen words and she has spoken dozens more, including a few she got from her dad that really shouldn't be repeated. She will eat just about everything, and she is getting to the point where she demands to feed herself for most things. Like her parents, her favorite foods are all in the macaroni-and-cheese food group.
As I wrap this up, I'm watching Emma in her stander, calmly and patiently flipping through the photo album her grandmother just made her. If only things can stay just like this for a while...
Jonathan