Our family's story began two weeks after our son's first birthday on April 20, 1998. He had not been feeling well ~ severe stomach pains, high fever, throwing up constantly. This was the first time he was ever sick at all. By the second trip in three days to the doctor's office, the doctor said it was not just a stomach bug as he initially thought, but something more serious and we needed to bring Joey to the hospital immediately. Thankfully the hospital was right down the street. The doctor there said that if it were something very serious he would send us directly to Children's Hospital in Boston. They did some initial tests and an x-ray and thought that it might be an appendix, but that's not common at such a young age. So our son was transported to Children's by an ambulance. My husband and I had to leave Joey in the hands of strangers, doctors and nurses, yet strangers just the same. We were to meet them at the hospital.
We got to the ER at Children's where it was eventually determined that Joey had an intussusception. This is when the intestine telescopes up inside itself and causes severe blockage, and it is fairly common among children his age. First they try an air enema, but after about three attempts it doesn't work, and they don't want to blowout his intestine, so he would have to have surgery. During the operation they may or may not have to remove some intestine, and he may or may not have to have a colostomy bag. They wouldn't know until they opened him up. We waited, and waited, and waited for what seemed like forever, especially since it's after 3 am. Nothing good happens at three am. By about 4 am or so, about three or four hours after the surgery started, the doctors told us that the surgery went well. The intestine was slightly red and raw, but they didn't have to remove any of it and he didn't have a colostomy. Thank God, this was the best outcome we could have asked for, or so we thought.
A few hours after the operation Joey woke up for a little while and was being goofy with me, being silly with his binky and blanket ~ that was the last I would see of my son for a very long time ~ the silly little boy that we knew, anyway. Over the next few days we watched our son slip further and further away from us, although he was supposed to be recovering. We watched his little 20-21lb body swell up like a balloon. His skin got so tight that it looked as if you could pop it with a pin and he would go flying around the room. The doctors believed he was feeling worse because the meds were wearing off and the body does tend to swell after a trauma, such as an invasive surgery like his. They said he should be fine by the weekend and we could go home then. "Get him up and moving around," they said. We tried, but he couldn't move. He was too swollen. On one of these "walks" around the floor, we carried him just to get him out of his room. I had an overwhelming feeling that there was something wrong with his liver. I knew a friend of a friend when I was younger and she would swell up or get puffy after a treatment, but I didn't know if it was from her meds or the problem with her liver.
I told my husband this story as we "walked" with our son, and now believe it was God's way of telling me or preparing me for what was happening to my son. It was an intuition like nothing I had ever experienced before. We continued to question Joseph's condition, but we were always given an answer that seemed logical. After all, they are the doctors and nurses and we are just the parents. I would later come to realize that yes doctors and nurses are very good at what they do, but there is nothing like a parent's instincts.
Through help of other parents we learned never to doubt those feelings again. Doctors know symptoms, and medications, and a lot more, but only you know your child and better than anyone. Never dismiss that.
Joey's condition worsened to the point that we could not get him to focus on my husband's finger, his eyes were all over the place. He was hallucinating, trying to drink from an imaginary bottle. He eventually started turning red, getting stiff, shaking and screaming. He was convulsing in a seizure. The doctors tried to get blood samples to tell what was going on inside him, but he was so dehydrated that they couldn't get any. They asked permission to try a vein in Joey's head. We said do whatever you have to, just save our baby. No luck there. They finally went to the groin area where you can usually always get blood. After pulling back and empty syringe for the third time the frantic look on everyone's faces was obvious. By this time Joey had stopped crying and fussing. He was just lying there. This was more disturbing than the constant crying and screaming. I do not know if he was clinically in a coma, but that's how I would describe him. He was breathing, but he was asleep with no response to verbal or other stimuli.
The head surgeon did finally manage to get the blood. They rushed Joey to the MICU (multidisciplinary intensive care unit) and were trying to get him ready for another surgery. The doctors came and talked to us. He had gone into liver failure, kidney failure, and pancreas failure, too, and they had no clue why. They needed to go back in to see if he had intussuscepted again and possibly do a liver biopsy, but they needed to stabilize him first. They told us he may not make it, they were going to do everything they could, and you know they meant it, but we needed to be prepared. The hardest part was putting him under. He was so unstable that putting him under the anesthesia alone could kill him. What choice did we have?
They came back to us just after the surgery started. He had gone under and was doing all right ~ so far so good. A few hours later, our families by our sides again at 3 am, we saw them wheel his bed back into the MICU. The child I saw was now bloated to 30lbs. That's 10lbs of fluid under the skin on the body of a 13-month-old baby. He had tubes in his nose and down his throat, he was on a ventilator, breathing for him, and he had monitors hooked up all over his body. My son had become this unrecognizable, swollen, creature on a breathing machine with blood on his face and tubes coming out from all over his body, and beeps going off, and I couldn't handle it. For the first time since this whole ordeal started I had this anxiety attack and I ran from the room. That couldn't be my little boy lying there, it just couldn't be. I had cried before, I had felt hopeless, I had prayed constantly, in fact I never felt closer to God, but in that moment I felt complete despair and disbelief. Nothing could have prepared me for what I saw ~ nothing could ever prepare a parent to see their child like that.
But do you know what the real kicker is? We now know all of this could possibly have been prevented. If there was infant screening we may have known Joey had an FOD and his care for the intussusception could have been modified to compensate for his condition. Joey hadn't intussuscepted again, but they did manage to get a liver biopsy. Joey did eventually get better. He began breathing on his own and got moved out of isolation. We started to see glimpses of the little boy we'd known, again. But he also went through bumps along the way ~ fevers that they could only get down with cooling blankets. Because of the damage done to his liver, it was only 30 percent functioning at one point. They could not give him Tylenol. They put him on an experimental drug that they sometimes give to those who have overdosed on Tylenol, but it gave him a rash all over his body and it had to be stopped. He got a few doses in before the side affects showed, so it could have helped. His heart failed at one point. He had a rapid heart rate and there was fluid around it. It was as if the toxins were making their way through his whole entire body, and they were. We just prayed it wouldn't go to his brain next.
The first time he opened his beautiful blue eyes again I was asking him where Brach was. Brach is our beloved chocolate lab. From that point on I knew if he remembered Brach he would remember us. I just wouldn't allow myself to think otherwise. The doctors were not so sure, he had toxins in his brain, that is why he had those seizures, and no one was certain what damage had been done. The only way to assess the damage was to wait and see how he reacted to stimuli, mental and physical. My Mother's Day gift that year was that we got to leave the MICU, and go back to the 8th floor. It was the best present I will ever get (besides going home).
A month after we had come to Children's we were finally going home. And two months later the metabolic doctor called to say that the skin biopsy they did on Joseph when he was in the hospital came back positive for an FOD. It was a sad moment, but it was also a relief because it explained a lot about what had happened to Joey. It gave us much anticipated answers. The reason Joey had gone into multiple organ failure was that when his body ran out of energy it went to his food stores, but because he lacks the proper enzyme to break fat down and turn it into energy it caused a toxic effect and those toxins then went through out his body. It only takes a child 4 to 6 hours without an outside energy supply to go to his food stores, in Joey's case he hadn't been able to keep anything down for about 48 hours before he was brought to the hospital. When he was in the hospital he was given IV fluids, but not at the proper dextrose level for a child with an FOD. That IV, however, is what kept him alive, but at the same time it wasn't enough dextrose and that is why he kept getting sicker but stayed alive. He was allowed only a minimal amount of oral liquids because of the type of surgery he had gone through. It was almost a week after he first got sick that they took him off the IV and that is when he spiraled down and started the seizures.
Although we still don't have a complete diagnosis, we know he has an FOD and are still working with doctors to label his exact disorder. The anesthesiologist, that kept Joey alive during the second operation, told us that he had never seen a child as sick as Joseph who survived, and on the rare occasion a child had survived, he hadn't seen one survive without any residual side affects. We credit God, all the many doctors and nurses that worked and continue to work with us, and the many people who prayed for Joseph, many of whom we don't even know, for his survival. We also thank our families and friends for always being there ~ you all made a very difficult time a little more manageable.
Today Joseph is a healthy, happy, beautiful, and silly 3-yr-old boy, whom you'd never know anything had ever happened, except for the large scar across his abdomen just below his belly button.
He is living proof that there is power in prayer and a living reminder, for which there are many not living, that infant screening is a necessity. No parent should ever have to go through what we went through or have to lose a child to these treatable disorders. No child should have to die or almost die to be diagnosed. Our sincere hope is that no other child ever will.
Stacey and Paul Webber (Joseph, too!)