On Friday, January 30, 1987, at 12:34pm a little boy was born to Connie and Joe
Quinn. He weighed in at 6 pounds 11&1/2 ounces and was 19 inches long. Quite a
nice size for a baby who was born 7 weeks prematurely, I think. I cannot remember seeing Jonathan until he was 6 days old. Immediately after his birth, he was rushed to a neo-natal ICU because he was in distress. Jon was born with premature lungs, thus was in respiratory distress. Joe said that they brought Jonathan in so that I could see him, but I cannot remember too many things about that day. I delivered Jonathan by c-section, so I was still quite drowsy when they brought him in to me.
Jonathan was in the NIC unit for 9 days and then brought back to the local hospital
for 7 more days. He had to stay in the local hospital, because he was losing weight
and they wanted to make sure he was not having any other problems. After 9 days,
I begged them to let me bring Jonathan home. It was very draining having to find
rides to and from the hospital for feedings. Also, I had a 2 year old son, Anthony,
at home and he didn't understand why his mommy was always dropping him off somewhere
and leaving him there. Anthony couldn't figure out why his baby brother wasn't
coming home with me. I would go out to see his brother and he would always ask
me if he was coming home to see him. I finally convinced the nurses to let me
bring Tony in to see his baby brother. How exciting, but at the same time it was
very emotional. Tony was so proud of Jonathan that we came home and he called
his aunts and his grandparents and told them all about his visit! He asked so
many questions about Jonathan's hospital stay that I couldn't find the answers
anymore.Finally, the big day came.....Jonathan came home to join our family and we could begin our life as a "real family".
Jonathan was born as the result of an amniocentisis in my 7th month of pregnancy. I was very frightened when the labor started and the contractions were coming harder and lasting longer. I knew that this was very abnormal, especially since I had already delivered another baby. The nurses and the Dr. were running all around the unit and I was getting more nervous and upset by the minute. Of course, they all tried to assure me that everything would be fine, but I knew that the reason for the amnio was to see if Jonathan's lungs would be mature enough for delivery.
I cannot remember too many things about the events that led up to the delivery of Jonathan, nor can I remember seeing Jonathan after his birth. Joe said that the Doctor had brought Jon in for me to see, but I cannot remember that. The only thing that I remember was waking up and the nurses telling me that everything was going to be okay. What did that mean, okay? I just gave birth to a baby....was there something that I should know? She told me that Joe would be in shortly to see me. Joe came in and all I can remember his telling me was that our baby was in a lot of distress and was rushed to the NIC Unit of the area hospital that could care for him. Joe said that Jonathan had premature lungs and they had to take care of him in the NIC Unit because that is where he would get the best care.
Joe had a very good employer at the time, and since Jonathan was in a hospital near his place of employment, he got to visit Jonathan everyday, sometimes two or three times a day. I, on the other hand, did not get to see Jonathan until he was 6 days old. I can remember that it was a very emotional visit. Jonathan was hooked up to all kind of tubes and monitors. Actually, Joe said that there were a lot more tubes before I got to visit. I couldn't wait to hold him, he was so beautiful, so precious and so tiny. I remember looking at his ears, they were so little that I thought he wouldn't be able to hear. His fingers were so tiny and thin, that I thought they would break if I touched him. I cried for such a long time before I could see my son, and now I was there, I was within an arms length of him, and I couldn't stop crying. I don't know whether it was joy or sadness that I felt, but I do know that it was a moment that I will never forget. The moment the nurse handed me my son for the first time.
It was scary to hold Jonathan with all the tubes and monitors hooked up to him,
but I got over it quickly. I waited 6 days for this moment and I was not about
to lose it. How very precious this life is.....how very dependent on everyone
around him. When I held him for the first time, he was being tube fed, because
he had no "sucking" ability. The nurses were trying to teach him with a "preemie"
bottle nipple and he was having a rough time of it! It took quite a while to teach
him to use a bottle. When Jonathan finally came to the hospital where he was born,
it took me two hours to feed him 2 ounces of formula. It was hard to try to find
rides for all the feedings, because I would no sooner get home, and it would be
time to go back for the next feeding. I persisted because I wanted Jon to gain
weight so I could get him home. Finally after 7 days, the pediatrician saw how
dedicated I was to having Jon survive, and he released Jonathan to the outside
world. 
We
didn't tell anyone that we were bringing Jonathan home from the hospital, but
you would have thought that the newspaper headlines said that Jonathan Quinn was
released from the hospital. We brought Jon in and introduced him to Tony, closed
the blinds, and locked the doors. We thought that since it was such a long and
tiring 16 days, we would bond as a family and shut out the outside world. Tony
was excited that finally all of us were together and he could hold his baby brother.
The peace
and quiet lasted about 2 hours. The phone kept ringing, there were neighbors at
the door and Jonathan was crying. The family members and the neighbors kept coming
by leaps and bounds. How tiring, but I could understand how they felt, too. A
new baby in the family and he is over 2 weeks old, and no one got to see him.
We finally got to sit down and eat dinner that night at 9:00PM....A nice quiet
homecoming! The pediatrician told me that it wouldn't be easy but he had faith in me. He knew that I could handle the feedings and Anthony and being a wife. I knew that I could handle it too, but it wasn't easy. Jonathan would have slept his life away if I didn't wake him up. This I was not used to. When Tony was a baby, I did not have a schedule, I left him make his own schedule. I had to wake Jonathan every three hours and feed him three ounces. Not an easy task, especially when it would take him two hours to drink three ounces. Then I could go back to sleep for an hour and wake him up again. This got old pretty fast, but when you love someone and they depend on you for everything, you do whatever you have to do to assure them of their survival. Joe tried to help me as much as he could, but he did have a job and I knew that he had to be there or else there would be no job. I tried not to let my weariness get the best of me, but my lack of sleep soon won out. I was like a "bear"....at least that is what my friends told me later. No one wanted to hurt my feelings, after all I was trying to do what was best for all of us.
I can't tell you how difficult those first few weeks were. But Jonathan began
to gain weight within a few weeks, so I could try to get some sleep. I began to
feel better and things were starting to settle down. Anthony was taking this big
brother role a bit too far. Jonathan was so tiny, that Tony thought he was a toy.
I will never forget the morning when I was in the kitchen getting Tony his breakfast
and Jon a bottle, and when I returned to the living room, there was Tony sitting
on the sofa holding Jon. The grin on his face said it all. I was angry but was
chuckling under my breath at the same time, since Tony had no idea of what could
have happened had he dropped Jonathan. Over the course of the next few months everything started to fall into place. Jonathan was sleeping better, he was gaining weight and we were adjusting to all the changes that were taking place for last four months. We were so exhausted that we decided that we deserved a vacation. We took the boys to Ocracoke Island, North Carolina. We decided that since Jonathan was doing so well and we really needed some well deserved family time, that we would take a week and go to the beach. All was well.....right? Wrong!
The drive
itself was tiring, two babies in car seats and lots and lots of traffic. Jonathan,
we found out, didn't like to travel. He screamed the whole ride to North Carolina,
he wouldn't take his bottle and he didn't sleep for the whole trip. We decided
that he must have had motion sickness and thought that we should call the pediatrician.
He agreed with what we thought and told us to give a small dose of benadryl for
the ride home. First of all we had to survive the vacation before we were going
home. Afterall we were on a nice relaxing vacation at the beach. The only time
Jonathan would not cry was when Joe or I would hold him. Our vacation became a
nightmare. We tried to show Anthony how beautiful this island was, but it just
was not working. We managed to stay for the week, but Jonathan was the center
of all we did. The ride home was equally as bad as the drive there, even with
the benadryl. Our vacation was in July 1987, and things began to get steadily worse from there. Jonathan could not even go in a car to the market about one-half mile away. The only conclusion that anyone could come up with was that he suffered from motion sickness. By the time Christmas 1987 came, we were beginning to become as exhausted as we were when Jonathan was born.
We began to notice that Jonathan was having some motor difficulties about this time and when we mentioned this to the pediatrician, he assured us that Jonathan was born premature and he had a rough start, so these difficulties were to be expected. We were noticing that Jonathan tremored in his one arm when he would reach for his bottle or his cup. He was sitting up, but still needed to have pillows surrounding him, because he could not sit for a long period of time. However, he was trying to pull himself up and toddle around the sofa and chairs, so we didn't give a lot of thought to the matters at hand. Only when we were visiting friends for fellowship during the holidays, did we become increasingly alarmed.
We left the boys to play in the living room with Sally's son Jacob, and when Sally
went to check on them, she came into the kitchen in a frenzy. "Something is very
wrong with Jonathan", she said, "come quickly". Joe and I, and Sally and Butch
went into the room and there we found Jonathan lying on one side and his both
arms were "shaking", not only when he was reaching for something, but even as
he lay on the floor. Of course, I thought that since he was not sleeping well,
he was probably tired. We soon left their home and returned home and put the boys
to bed. When Jonathan fell asleep, the "shaking" seemed to stop. Again a rough
night sleeping. I think I had forgotten what it was like to get a good nights
sleep. Jonathan seemed to only sleep if he was being held in an upright position.
Neither Joe or I thought very much of this, we just thought that it was all related
to his being a "preemie". Everything went down hill from there. Within a two week period Jonathan's both arms would tremble when he would be doing anything, playing blocks, sitting on my lap and even holding his bottle. I decided tht it was time to be assertive and tell the Dr. that I thought something more was going on here. By the time I got the appointment, Jonathan was in bad shape. He couldn't sit, stand, walk or even pull himself up. His whole body became involved, his legs were trembling, his arms, his body and his head, and his eyes were rolling in his head and when they weren't rolling, they were "jerking" back and forth. He was in a vegative state. He would only lay in his crib and scream.
When I called the Dr., the nurse said that the only appointments for that day were well baby appointments. You can only imagine what thoughts were going through my head when she said that. I immediately said that there would be a room waiting for Jonathan and myself and we would be seen by the Dr., no matter what! We went to the office, and I went in and registered Jon and they again said that he would not be seen, I think she was afraid that Jonathan was contagious and would give all the well babies a disease. I said that we would stay in the hall, and they should have us enter from the back. She had no choice, we were there and I would not leave!
Before too long, there was a room waiting for us, and one look from the pediatrician and by the look on his face, I knew that we were in trouble! He said that he had no idea what was going on, but made an immediate phone call to the Geisinger Hospital in Danville. Jonathan was scheduled for an appointment within the next week.
We met with Dr. Pileggi at Geisinger and he told us right up front that he was not sure what was wrong with Jon, but he would admit him and we would stay for a while. We admitted Jonathan to the hospital and when they were doing all their preliminary workups, I was sent to register at the Ronald McDonald House. The first real break I had since July 1987 and this was March 1988.
Jonathan was kept in the hospital for one week. They ran so many tests on him and took so many tubes of blood that I was becoming increasingly worried. They would take a series of blood draws and then be back in a few hours and draw more. Everytime I would ask about it, they said, "oh it is routine when you have a baby in the hospital for a diagnosis".
Geisinger is a teaching hospital, so there were always people in and out of Jonathan's room. They were videotaping him, taking pictures of him and even Dr.'s who had nothing to do with us were in looking at Jonathan and making comments about what they thought was wrong with him.
Finally, one of the Dr.'s who was part of the team, came into my room and said that she had to talk to me and needed to have my husband present. That did it for me. I knew it had to be bad news. Joe was at work, so he would be at least 6 hours before he could get to the hospital, so she said to leave a message and she would be back and talk to me. When she returned, she said that she had good and bad news and I should go with her to the family room. I picked up Jonathan and off we went. When we got there, she said that I should just lay Jonathan on the floor and sit down and talk with her.....no way, I was not laying this child down! She was very upset with my not following her orders. I politely asked her to state the findings and I would be go back to my room to wait for Joe to call. She said, "the good news is he doesn't have a brain tumor." I can remember waiting for the bad news, I began to cry and she just sat there looking at me and said that maybe we should wait until Joe got there. There was no way that I was going to wait. "The bad news is that the myelin sheath on his brain is deteriorating", she said. What does that mean, would he die, was he going to be like this for the rest of his life...so many questions....and she couldn't answer me. She said, "we will talk later when Joe gets here", and she got up and left me there. She left me sitting in the family room with my baby and in a terrible emotional state.
I couldn't move, I was shocked......somebody pinch me and wake me up from this terrible nightmare. Finally one of the other moms in the ward came to look for me and she couldn't believe what I was telling her. She carried Jonathan and we went back to our room.
It was a long afternoon. Joe finally arrived at the hospital about 6:30pm and we could not reach the Doctor. When I told him about the situation, he was also in shock. How could this be happening and why would God let this happen to us. Boy I was so mad.......I was mad at the world. Everyone who came in contact with me would have had their heads chewed off if they just looked at me in the wrong way. It was after 8:00PM and we still were unable to find the Doctor. Finally, the nurses paged her. She left the hospital for the day, so we perisited until we were put in touch with another member of the team. This time, Dr. Pileggi came to talk to us. He was a wonderful Doctor and a compassionate man. He told us that Jonathan had a rare disorder called Myoclonic-Encephalopathy. I asked him about what I was told earlier in the day, and he apologized. The Dr. who came to talk to me, overstepped the team, and went with her gut feelings. I do not need to tell you what I said to him about that! I was left sitting all day thinking that my baby was going to die. I couldn't talk to anyone, couldn't eat or drink a thing, and cried the whole time I held Jonathan. Dr. Pileggi apologized for the error, but I was not ready to hear that. I wanted to hear what they could do for Jonathan.
Dr. Pileggi explained that they still needed to get the results of other tests before Jonathan would be started on any treatments. "How long would this take," I asked. There were no answers. The only thing he could tell me was that we could finally go home, and return in two weeks for a follow-up. Jonathan and I returned home with Joe that evening for the start of what would turn out to be a nightmare.
I was mad at everybody. I was mad at Joe, Anthony and Jonathan, friends, family and even God. Why would he allow me to have a baby with all these problems? What did I ever do that would have given him the right to give me a baby with all these problems? Why would he give me such a beautiful little boy and then have him suffer as he was? Why? Why? Why? I couldn't even get myself to pray to him. I was feeling all the terrible feelings of anger, guilt and denial. I followed all the Dr.'s orders when I was pregnant. I do not smoke, drink and have never taken drugs. I never even took a tylenol unless I called the Dr. and asked if it was okay to do that. I walked everyday, I got enough rest and I ate properly. Why, then, would this happen to me. It was probably something I did in the past. I always obeyed my parents, I followed their rules, I had only the friends that my parents thought were good friends. I was good in school, I got excellent grades and I was a good church going girl. "That must be it", I thought. "I was not a good enough Christian." "I was failing in the way of the Lord." I was reaching above and beyond for answers. I had accepted the Lord as my personal savior by this time. I knew that I had turned things around in my life and I also knew that God does not allow things to happen for his own purposes. I knew deep down in my heart that God did not let this happen to me because of something I did to punish me, but I was still angry. I could not understand nor would I allow myself to understand. I needed to get past all these feelings, I needed to understand, but it was very difficult.
The days grew worse. Jonathan would not sleep, he would not eat, and he needed to be carried all the day and night long. I was drained. I was home with Jonathan and Anthony all day long by myself. I was burning pots on the stove. The house was becoming a disaster area, bottles laying all over the living room, dirty dishes piling up in the sink. I was worn out. There was nothing that anyone could say or do that would help me to feel better. I cried all the day long and all night long. I had no time for Tony. Here was a three year old little boy who I didn't even know existed. I made him to grow up very fast. I would fill his breakfast bowl with cereal, give him a glass of juice or milk and then tend to Jonathan's needs. When Tony would finish up, I would clean him up and sit him down to play or to watch a video, so I could tend to Jonathan's needs. This would go on for lunch and dinner, too, unless Joe got home from work early. Jonathan would cry all the time, not even a bottle would pacify him. He was averaging 2-3 hours of sleep in a 24 hour period and that meant that I was also sleeping very little.
Finally, the Doctor called with "news". He said that Jonathan had polymyoclonia-opsoclonus. Whatever that meant. I asked him what it meant, how it happened, when it would go away, and of course the all too familiar, why.....there were very few answers. What he told me was that we must now start to look for a neuroblastoma. I was heartbroken. I looked at Jonathan and cried for what seemed hours. He said that we should have this done as quickly as possible and then we would start Jonathan on ACTH corticosteroid treatments. We wasted no time getting the test done and afterward we did not know whether to be happy or sad. They said that there were no tumors found. We had been told that if there was a tumor, he would have surgery and then chemotherapy and things should get better as time went on. Shall we be thankful that there is no tumor or be angry? We came to the conclusion that we would be happy and thankful, because we would not want to see our child go through chemotherapy. We were already seeing a friend and her child through this treatment so we knew what could happen. We started Jonathan on ACTH therapy the next week.
The ACTH turned out to be a great drug, powerful, but it did what we prayed it would do. It gave us our son back.....well, not exactly! Jonathan began to sit up again and tried to pull himself up at the furniture. It took a few weeks, but he managed to do these simple daily skills that we "healthy" people take for granted. However, the mood swings and behavior were horrible. He still did not sleep, he cried and sceamed and he would not eat! The Doctor said that this too would get better. I finally realized that Jonathan was miserable because of the steroids and the lack of sleep, complicated by this dreaded OMS. It took me 3 months after the diagnosis to realize this, but somebody had to start to do the homework on this one. Jonathan had very little sleep from February 1988 until October 30, 1988, when I called the neurologist and demanded something to put him to sleep. I told them, "either you prescribe something or I will bring him to you and leave him there until he was better". They were no fools, they knew that Jon would never be better, so I got a prescription for a sedative. It worked like magic......Jonathan was out like a light within 20 minutes. Of course, I was afraid to let him sleep, so I kept a vigil over him the first night I gave him the sedative. I could hardly believe what happened, he went to sleep and slept for 15 straight hours! Me, I slept none, but that only lasted the first night! The next day things were no better with Jon, but I had convinced myself, that the more rest he would get, the better things would be. I just kept my faith and I do believe that was what kept me going.
After a few weeks passed, Jon seemed to calm down a little more. Then they cut his dosage of ACTH. Within 3 days, we had a monster again....the tremors were more prevalent than the week before, the ataxia was worse than it had been and he no longer was sleeping. The only thing to do was to increase his ACTH, and everything started to skyrocket again. The behaviors kicked back in, the mood swings returned and the poor appetite returned. Of course, it was like Jonathan had only one parent, me.......he would not even acknowledge Joe........through the whole ordeal, it was just me! Jonathan would only get more defiant and nasty if Joe would try to soothe him.
It was Christmas 1988.......Jonathan was a tyrant. I was too tired to care, but had to be strong for Anthony. We tried to have a nice holiday and relax and enjoy family and friends, but they knew how I felt, they knew how Jonathan felt and they tried to be there for us. However, Jonathan still would not be satisfied with anyone but me. Anthony enjoyed the day and I am sure we all did, but I was drained......I thought I had reached the bottom of the barrel and could go no lower. I felt as though we were ruining the holiday for our families so we made our way from home to home rather quickly and got back to our home, so we could tend to Jonathan as we always did. I got Jonathan ready for bed and gave him his medicine and he was off to dreamland in less than 10 minutes. I took advantage of the situation and for the first time since November 16, we slept a full night.
Over the next few weeks, Jonathan grew stronger and more steady and the Doctor thought we should decrease the ACTH again. Reluctantly I did as I was told. This time, Jonathan seemed to stay stablized. Thank God! I was elated and at the same time afraid to look to the future. He was still sleeping through the night, and the behaviors were not getting any worse. Finally this dreaded illness is leaving Jon's body. I was so happy........I made phone calls to everybody I could think of! Family and friends were glad that it was over and Jonathan would be back to himself in a few more weeks.
Things seemed to be getting better. Jonathan was laughing and smiling and trying to talk again. We were told to decrease the ACTH again and this time did not even think twice about doing this. We did not get through the first week, Jon was regressing and things were almost as bad as they were when he was first diagnosed, only difference being, he was still on his feet.
