Stephen
 Stephen was born by Cesarean Section on January 11, 1993, because of mild fetal distress. His blood oxygen saturation level was low after birth, and he was put in an oxygen tent. A pediatrician ruled out fluid in the lungs and didn't think it was an infection, so he was transferred to a pediatric hospital (about an hour away) for diagnosis. Around 2:30 am, a cardiologist called and wouldn't tell me anything more than that my husband had to meet with the chief of Cardiology as soon as possible. Now I was really worried.
The next day, my husband had to tell me that my baby had Hypoplastic Left Heart Syndrome. This is a heart defect where the left ventricle, the part that pumps the oxygenated blood through the body, is undersized. We had three choices. One was to make him comfortable and let him die. One was to transfer him to another hospital and undergo a series of three or four surgeries that would basically make the right side of the heart do the work of the left side. Each surgery carries a risk of death, and some children who do survive the series don't have sufficient cardiac function to lead a normal life. Before my husband even mentioned the possibility, I knew that my baby needed a heart transplant. For us, it was an all-or-nothing choice. Either he would die, or he would lead a normal life after the transplant. Three days after his birth, Stephen was placed on the waiting list for a heart transplant.
The first week after his birth was probably the worst. Stephen and I were in different hospitals, an hour apart, and my poor husband was torn between the two of us. He was also in the difficult position of having to tell me the bad news and explain to me the defect and the options. I had to spend three miserable days in the hospital, knowing that the rooms around me held happy parents and healthy babies while my arms were empty. Even after I was released, the entire family wanted to see Stephen that first weekend and spend time holding him. Only two visitors were allowed in the NICU at a time, so we all had to take turns. With three grandparents, his aunt, his great-uncle, and two great-grandparents wanting to see him, I didn't have nearly enough time with my baby. Finally, when he was eight days old, I was able to hold him for hours and finally feel that this was my baby.
Stephen was in the Neonatal Intensive Care Unit (NICU). He was given IV prostaglandin to keep his ductus arteriosus open and keep him alive. He was fed through a nasogastric tube to conserve his strength and fed small amounts of high-calorie formula, and given Lasix to help prevent fluid from building up in his lungs and body. After a week, he moved into the step-down room (for less critical infants) and I was able to give him a bottle. We were lucky that Stephen's vital signs were good enough that we could feed him one (sometimes two) bottles a day, that way we avoided feeding difficulties later. He is still a good eater. Stephen actually gained a pound his first month (normal weight gain is about two pounds). Everyone was thrilled. They didn't expect any weight gain because he needed so much more energy just to stay alive.
The first month was fairly uneventful. We waited, but Stephen was doing so well that we knew we could afford to wait. I think it was easier for me than for my husband. I put the minimum of necessary work into the household and devoted the rest of my time to Stephen. I visited him practically every day and could stay for hours. My grandmother came to stay for three weeks and cleaned my house from top to bottom, so I didn't have to worry about that. All my energy was involved with Stephen. My husband still had to work full-time, and he was taking two computer courses at night at a local college. We both agreed that these courses were an investment in our future so he shouldn't drop them. However, that meant that he could only visit Stephen two nights a week and on the weekends (We kept Thursday nights as our evening home together). It was difficult for him to balance the demands of work, school and family. Emotionally, life was as smooth as it could be, we just waited and loved Stephen.
On Sunday, February 7, the hospital chaplain christened Stephen in an empty room in the NICU. We couldn't risk bringing him to the chapel. On February 9, Stephen's ductus arteriosus started to shut down and we nearly lost him. It was really scary; the staff kicked me out of the NICU while they worked on him and I couldn't reach my husband who was working late. Within a few hours he was doing much better. He improved over the next few days, but was feverish and irritable. We didn't know if it was an infection or the higher dose of prostaglandin. Eventually, the dose of prostaglandin was reduced, and Stephen was clearly feeling better. Now the need for a new heart was more urgent. I waited and waited, and started second-guessing myself. Should we have decided on the surgery? Is it too late to change our minds and do the surgery? What will we do if he dies? Also, about now, I became more conscious of the fact that getting a heart meant that some other family's baby was dead. This was a more difficult time for
me. Stephen wasn't growing anymore, and his oxygen saturation was down. We knew that soon his condition would worsen. We waited and hoped for a heart while knowing that another baby must die for our baby to live.
On Friday, February 26, it snowed and I was really tired, so I stayed home and napped. My husband went to see Stephen, now six weeks old, after work. At 5:30 I got the call from the transplant coordinator that we had a heart for Stephen. My husband didn't want to put Stephen down long enough to call me himself. I rushed to the hospital, and we just held him and loved him until it was time. We carried him to the operating room about 11:15. At 1:30 we were told that the heart was there. At 5:00 I was told that Stephen was off the bypass machine but still unstable. At 5:45 he was stable, and at 7:45 the surgeon came to talk to us. The heart (from a 10 week old boy) was a good match and everything went very well. At 8:45 Saturday morning, we went in to see Stephen. He had a ventilator tube in one nostril and a nasogastric tube in the other to suction out his stomach. He had three small IV lines in his right atrium, putting medication directly into the heart and measuring the central venous blood pressure. He h
ad pacemaker wires attached to all four chambers of the heart. He had two chest tubes draining fluid and blood from the chest cavity. He had a central catheter in his right arm and a peripheral IV in his left arm. He had an arterial line in his left groin for medication and measuring the arterial blood pressure. Despite all of this, he looked good. We couldn't stay with him for long or touch him because he would start to wake up and his blood pressure would rise.
Sunday, Stephen was awake a bit but still dopey. He received platelets and his peripheral IV was removed from the left arm. On Tuesday he was taken off the ventilator and put on oxygen. He had no voice because of irritation from the tube. Wednesday was a big day, the arterial line in the groin, the pacemaker wires and one line into the atrium were removed. On Thursday one chest tube was removed, and a nurse introduced Stephen to television. On Friday the two remaining lines into the atrium were removed and the second chest tube. On Saturday we finally got to hold Stephen again. On Monday he was off the oxygen and on Tuesday I gave him a bottle. He finally could eat as much as he liked, without fluid restrictions. Ten short days after the transplant, he was looking and acting like a normal newborn. He looked and obviously felt much better than before the transplant.
This time immediately after the transplant was not a time of celebration, but a serious time. There were still many things that could go wrong for Stephen. We knew that he might still die, and we were very conscious that the donors family was mourning the loss of their 10 week old boy. As each day went by, Stephen grew stronger and we became more relieved. He looked so much healthier. I kept reminding myself that the donor would have died anyway, and I was so grateful that his family, in the midst of their grief and pain, were considerate and caring enough to consent to donating his organs.
He wasn't ready to go home until 10 weeks post transplant, but nothing was a serious threat to his life. First, he developed an infection, treated with antibiotics. Then he had a bout of mild rejection, treated with stronger immunosuppressives. Then Stephen started having premature ventricular contractions, a type of arrhythmia that his doctors had not seen previously in a transplant patient. The arrhythmias got worse, and he was treated with medication. He was on this for about a year, and the arrhythmia hasn't returned. Then Stephen developed cytomegalovirus (CMV), most likely from the donors heart. He had to be treated with an IV antiviral drug for 14 days. We finally brought Stephen home from the hospital 16 weeks after his birth.
These last weeks were especially hard on me. Stephen was visibly healthy and I just wanted to get him home. Every time we discussed going home, something would happen. We were scheduled to bring him home on Good Friday, but his arrhythmias got worse. The transplant coordinator had to call us at home at 8:00 Thursday night and tell us that he wasn't coming home. I cried myself to sleep, and spent about four hours the next morning just lying in bed and staring at the ceiling before I could find the energy to drag myself out of bed. This was my lowest point in the whole ordeal. We stopped mentioning the h-word in Stephens presence. I finally packed a bag and left it in his room and told the transplant coordinator that didn't I want her to get my hopes up again. I told her to just walk in one day and tell me Youre going home today. I didn't want to have my hopes crushed again. I was also concerned because of developmental delays. Since he was confined to a crib or held all the time, Stephen had no incentive to
start exploring his world. He wasn't reaching for toys or opening his fists. We tried hanging toys over his crib and playing with him, but he was still delayed when he came home. The first week he was home, I watched about two months of development happen in one week. Ever since then, he has reached all milestones in the normal time frame.
It was wonderful to do normal things with Stephen. We were somewhat concerned with infection, but we weren't fanatical about it. I took Stephen to the drugstore and grocery store soon after he came home. We splashed in a wading pool that summer, and I took him on plane trips to Arizona and Florida to see his great-great-grandparents. Our lives quickly became normal and boring.
Stephen has only been hospitalized twice since then. At about 16 months he was admitted overnight for a blood transfusion and tests to determine why his bone marrow had stopped producing red blood cells (aplastic anemia). Apparently he had contracted parvovirus (Fifth Disease), and in patients with a compromised immune system parvovirus can suppress the bone marrow. Fortunately, he recovered on his own, before another transfusion was necessary.
At 31/2 he was hospitalized for 10 days to treat mild rejection. Another virus probably triggered that bout of rejection, which slowly worsened over six months. His medications were changed, but he had to be hospitalized for treatment with stronger immunosuppressives. This rejection was only visible on his electrocardiogram (EKG); he felt and acted fine. He spent 10 days in an isolation room in the CCU, only able to go from bed to chair. Since he is very active, this was quite difficult. When he went home, we still had to do 7 more days of IV infusions, but our lives were much easier at home. He recovered nicely and went back to his normal life.
Stephen's life today is very normal. He attends preschool two days a week, and takes swimming lessons at the YMCA. He runs everywhere and takes three mile walks with his uncle. He takes medications three times a day, but he has only four prescriptions. He needs to be pre-medicated before dental work, like any other child with a heart condition. His scar is barely visible. Stephen visits his cardiologist about four times a year for echocardiograms, EKG, and bloodwork to measure the amount of cyclosporine in his blood and monitor for kidney impairment. As an infant, his cardiology visits were more frequent because his medications had to keep up with his growth. Twice a year, he has cardiac catheterizations and biopsies, where his cardiologist looks directly at the heart and takes a tissue sample to look for signs of rejection.
Stephens transplant really has little impact on our daily lives. This impact was much more significant during his first year when he had more medications and more doctor visits. We are concerned with infections, but not overly so. We all practice good handwashing. If Stephen runs a fever, we visit the pediatrician to determine if it is viral or bacterial. If we find an ear infection, he gets antibiotics. If it is viral, I just watch him, and he recovers, just like any other child. He doesn't get sick more often than other children. We all get flu shots each winter to protect him. Stephen cannot have live virus vaccines such as the measles-mumps-rubella or chicken pox vaccine. We have to depend on herd immunity -- if every child around Stephen is vaccinated, the effect is the same as if he were vaccinated. He (and his siblings) must have the inactivated polio shot, not the live oral vaccine.
We don't know what the future holds for Stephen. He may have a fatal bout of rejection or he may live to see his great grandchildren. We believe we have given Stephen his best chance for a normal and healthy life. We have had him for four precious years, and we will be grateful for each additional year. Each year brings new advancements in transplant medicine, and a better chance for a long and healthy life.
Chris Molnar
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