Hypoplastic Left Heart Syndrome: A Handbook for Parents

Alexander's Story
by: Anna Jaworski


Alexander Guy Jaworski was born in the heart of Texas on August 11, 1994. He came into the world weighing a healthy six and a half pounds and looked like a perfect baby. Well, almost perfect.

As Frank held his son for the first time he was overcome with emotion. He greeted his newborn son, calling him by name, and started counting fingers and toes. In an instant he voiced a concern to the doctor, “He’s breathing too fast!” The doctor smiled and informed Frank that he was fine and that he just had “newborn breathing.” A look of relief swept over Frank’s face as he handed me Alexander for the first time.

I was feeling relieved, too. This had been a difficult pregnancy. Hearing Alexander’s sweet baby cry filled my heart with happiness. We had done it! We made it to 37 weeks and Alexander was just fine.

But everything was not just fine. When the nurse brought Alexander to my room for me to breastfeed he wouldn’t nurse. After much coaxing and help Alexander finally latched on but seemed too weary to eat. “Oh, you have a sleepy-head,” the nurse teased me. She got a wet washcloth and put it on Alexander until he awoke and slowly began to suck. After about ten minutes he was asleep again. I couldn’t believe it!

I had nursed my first son, too. When he was a newborn he nursed for an hour every two hours. I only got an hour break between feedings. And now I had a sweet little lamb that I couldn’t even get to nurse for half that time! The next attempt was no different. The lactation consultant came to my room to talk to me. She repeated what the other nurse had said about him just being sleepy.

I was told by many people not to compare my two sons. For one thing, Joseph was a full-term baby. Alexander was three weeks early. That alone could account for many differences. And every baby is different anyway.

Alexander was jaundiced (yellow) but not jaundiced enough to be put under the bili lights. I had to take Alexander back to the hospital every other day for his first week of life to get the jaundice checked. Each time that Frank went with me he asked the nurse in the Follow-up Newborn office about the breathing. Each time we were told it was “newborn breathing.”

Alexander’s jaundice got a little better, but he was still noticeably yellow. I made an appointment with the lactation consultant. When I visited her I told her that every time I tried to feed the baby I had to change his diaper, remove his clothes and use a wet washcloth on his body in order to get him to latch on. Then I had to rewet the washcloth with cool water and rub his body with it for him to nurse. I went through this every three hours. He would only nurse for 20 minutes. I was told this was within the normal range and not to worry. She told me that he probably had “breastmilk jaundice” and that if I continued giving him sunbaths he would be pink (or tan!) in no time.

I started keeping track of how many wet and dirty diapers Alexander made and exactly how long he would nurse. I was worried that something was wrong. He rarely cried for me. I usually had to wake him to be fed. I also kept track of how many sunbaths I gave him.

We had to take Alexander back to the hospital before he was two weeks old. At that time he was weighed and measured. The nurse informed us that Alexander was a little small. But, after looking at my petite frame, the nurse just smiled and said that Alexander must take after my side of the family. Although he was still yellow, she didn’t seem concerned and told us we didn’t have to come back until he was one month old for his shots.

Frank asked her about the breathing. “He’s almost two weeks old now,” Frank reminded her. We got the same answer as before: “newborn breathing.” We went downstairs to the emergency room where Frank worked as a nurse. The nurses and doctors swarmed around Alexander. He looked so small and sweet in his carrier. Frank took him out and asked one of the doctors about Alexander’s breathing. The doctor asked him what we were told upstairs. After Frank told him, the doctor shrugged and nodded his head in agreement.

Days passed without incident. I still gave Alexander sunbaths two to three times a day but didn’t notice the jaundice passing. I still often had to wake him for his feedings. But he was staying awake more. My three-year old, Joey, and I started playing with him. I’d put the baby on his quilt and Joey would lay his quilt right next to Alexander on the carpet. Then we’d play Wheels on the Bus and Itsy Bitsy Spider. We sang all kinds of songs throughout the day and noticed Alexander perking up when he recognized them.

The first month check-up was upon us before we knew it. He was weighed and measured and again we were told he was small for his age. I seemed to recall Joey being the same way the first year, but, by age three he was in the 50th percentile. Frank asked about the breathing again. I was beginning to get tired of him always asking about that. The doctor reassured Frank that it was “newborn breathing.” Frank protested that the baby was not newborn anymore, he was four weeks old! Regardless, we were told, it was nothing to worry about.

But, every time the boys and I took Frank dinner in the emergency room, Frank would pull aside a doctor and ask him about Alexander’s breathing. It finally got to the point that I couldn’t stand to hear it anymore. “He’s different than Joey! Just leave him alone! That’s just the way Alexander breathes,” I remember exclaiming after the eighth or ninth doctor told Frank it was “newborn breathing.”

But his concern became my concern. When Alexander was about six weeks old I noticed that he didn’t wake up in the middle of the night for his feedings. Instead I would awaken from my sleep to the sound of him making noise. I woke Frank up one night and told him to listen. It sounded like Alexander was grunting. I got up as usual and changed him and fed him. Frank didn’t seem concerned so I just made a mental note to ask the doctor during his second month check-up.

Three days before Alexander’s check-up we packed a dinner for Frank and took it to the ER. Again Frank pulled aside a doctor. Frank was called away suddenly so it was just the boys, the doctor and me in the room. I told him about Frank’s concern over the breathing and my concern about the jaundice. The doctor looked at Alexander intently. “When is your next appointment?” he inquired. When I told him it was three days away he nodded and assured me that he should be fine until then.

But, Alexander wasn’t fine. That night after we got home I tried to nurse him. As usual I changed him, stripped him down to his diaper and put the cool washcloth on him. This time he latched on greedily, sucked two or three times vigorously, then pulled himself off by arching his back and screaming. This scared me terribly. I pulled Alexander close to me holding him gently and patting him on the back. “It’s alright,” I said softly to soothe him. The same thing happened again and again for the next two days. By the time we drove Alexander to the pediatrician for his appointment I was exhausted.

Alexander was weighed and measured, and I could tell the doctor was not happy. She asked how he was eating. I told her that he seemed to actually be nursing a little more, but I was concerned. As I told her about him screaming after taking a few sucks I watched her examine Alexander. She nodded as she listened. Then I told her about the grunting.

She laid Alexander on the examination table and listened intently to his heart. She shook her head like she couldn’t believe what she heard. Then she probed all over his body and took his diaper off. She felt all around and double-checked herself. Slowly she reattached the diaper and said, “I’m admitting him.”

I couldn’t believe my ears. “What!” I exclaimed, “How can you admit him? What’s wrong?”

“I’m admitting him as Failure to Thrive,” she said calmly looking me straight in the eye.

“But, that’s not my baby,” I blurted out, “Those are babies who aren’t loved!”

“Yes,” she admitted evenly, “usually Failure to Thrive happens for psychosocial reasons, and I think I can rule that out,” she added hastily. “But, Failure to Thrive also occurs with critically ill infants.”

“Critically ill? Alexander’s never had a fever, a cold or an ear infection! How can you say he’s critically ill? He’s jaundiced and a little small, but he’s not critically ill!” I exclaimed feeling like my whole world was crashing at my feet. This was just supposed to be a routine visit. My baby was not supposed to be admitted to the hospital!

In spite of my protests we were whisked upstairs to the pediatric ward. The computers were down so everything seemed to be moving in slow motion. Frank was supposed to go to work after our doctor’s visit. He and Joey went to the ER to tell his boss what was happening. Meanwhile I held Alexander and tried to stay calm. I was scheduled to meet my sister for lunch so I called her. “You probably just need to change from breastmilk to formula,” she said reassuringly. “Remember how Megan wasn’t gaining enough weight either until I went to formula?”

I remembered. But I had this feeling in the pit of my stomach that it was more than that. There was a nursing change of shift and a very efficient-looking nurse came in and introduced herself to me. She stood back and watched me patting Alexander on the back. “Does his head always move back and forth like that?” she asked me. I nodded and added that it seemed to be worse today than before. “Has a chest x-ray been ordered?” she asked. I told her that they had only ordered blood work and a sweat test. “That baby has a heart or lung problem,” she stated simply. When the resident came in she voiced her opinion, receiving almost no recognition for it. Within ten minutes, however, the doctor came back and told me that he had ordered a chest x-ray for Alexander.

Every time I tried to put Alexander down he cried. So I held him. And I talked to him and sang to him and tried to pretend that everything was normal. My sister came and brought me dinner. She looked at Alexander tenderly and reassured me that everything was going to be fine. She made me promise to call her the minute I found out anything.

I called my mother in San Antonio. She sounded so calm and reassuring that I began to think I was silly for worrying so much. “The same thing happened to you,” she told me. “When you were about six weeks old they admitted you into the hospital because they said you were too small. They ran all kinds of tests on you and everything was fine. You were just small. I changed formulas and you started gaining weight and everything was fine.” I tried to cheer myself up with that information, but I had a feeling it had nothing to do with my breastmilk.

Finally around 10:00 PM I asked the nurse about the x-ray. “What! They haven’t come and done that yet!” she exclaimed. She told me she would check on it. Around 11:00 PM I asked the nurse again about the x-ray. She got angry and called down again. Within minutes an x-ray tech was guiding me to the x-ray room.

The next morning the resident came in. He told me all of the blood work came back negative. “That means it’s nothing simple, right?” I asked. He nodded and then said, “But the x-ray was conclusive.”

“You mean that was normal, too?” I asked him.

“No, it was conclusive.” He held up the x-ray so I could see it. I’m no doctor, but I could see it looked odd. “Is that his heart?” I asked pointing to a huge blob on the film.

“Yes.”

“It’s not supposed to be that big, is it?” I hardly dared ask the question.

“No. It’s supposed to be about this big,” he said showing me with his hand how big the heart should be.

He let the words sink in and looked at me expectantly. “What do we do now?” I asked somehow keeping my voice steady even though I felt like bursting into tears.

“Now we’ll run some more tests, but we don’t have a pediatric cardiologist here,” he informed me. “You’ll have to decide where you want to go, Austin, Dallas or Houston.”

My head was spinning. I called Frank. I called my sister. I called my mother. Each of them were calm and assured me everything would work out fine. Frank was on his way to the hospital. I looked down at Alexander and kissed him. “We’ll get everything all worked out,” I told him with more enthusiasm than I really felt.

The rest of the morning was a whirlwind of activity. Two female technicians came in with an ultrasound machine. “We’re going to do an ultrasound of his heart,” they informed me. They wanted me to hold him still while they did this so they wouldn’t have to sedate him. Naturally he did not want to cooperate.

“Would it be alright if I nursed him while you did it?” I suggested. I hopped up on the bed and began nursing Alexander. He nuzzled close to me and they began the test. After about 15 or 20 minutes he fell asleep. I was afraid to move lest I wake him him so I kept as still as I could. The test lasted about an hour.

After the ultrasound (also known as an echocardiogram) things really started to move fast. I was told that my son’s heart condition was too serious to be treated in that hospital so we would have to choose to go to a different city for treatment. Even though Dallas, Houston and Austin were offered as choices to me I chose San Antonio. I had gone to college in San Antonio and was familiar with their excellent medical complex and I told the doctor in charge of my choice. After some consultation I was told that there was an excellent cardiologist there and as soon as he accepted Alexander we would be sent there by ambulance.

There was a buzz of activity over Alexander. They had to start IVs in him and he cried in protest. Sometimes it took them two or three attempts before they could get the needle in, and I felt sick. Finally they had two IVs in, but they wanted a third one in case they needed it. I didn’t know what to do. I was afraid of bursting into tears in front of all of those nurses and doctors so I excused myself to the restroom. I finally allowed myself to cry a little. Then I splashed cold water on my face, dried my face and hands and walked back into the room. they still hadn’t gotten the last IV in his arm. I sat down by Alexander’s head and stroked his hair telling him everything would be fine. Thankfully they got the last IV in and we were rushing down to the ambulance.

The ride to San Antonio was uneventful. No lights. No sirens. When we arrived at the hospital things moved into high gear. We practically ran down the corridors until we got to the Pediatric Intensive Care Unit. We were “greeted” by a group of people who took Alexander and told me to stand back. I watched in disbelief as they took him off the gurney, put him in a bed and began hooking him up to machines. The ambulance nurse stayed by my side and explained what they were doing.

I went over to the bed and looked at Alexander. All of a sudden he looked so tiny and helpless. I struggled for some way to make this seem normal. I leaned my head down on the rails until my cheek rested on the cold steel. Ever so softly I started to sing one of Alexander’s favorite songs. But when I got to “Please don’t take my sunshine away” the words stuck in my throat and tears slipped onto the bed.

Within a short period of time my parents were by my side and looking down at Alexander. He looked around curiously and every once in a while even managed a feeble smile. By evening my sister was also by Alexander’s side. She talked playfully with him and joked with my parents and me. She somehow brought a little sunshine to that horribly dark day.

That night my sister and I slept in the same bed together. It seemed like we were transported back to another day and time. We whispered to each other in the dark just like when we were little girls. When I confessed how afraid I was of losing Alex, Chrissy rolled over and held me. She told me, with a catch in her voice, that the only consolation she could find in that thought was that her twins would have a cousin to play with. We both fell asleep with that vision in our minds and tears on our pillows.

The next day more tests were conducted. Then there were emergency meetings. After a cardiac cath was done the surgeon and the cardiologist had a meeting with me, Frank and my parents. It was there that we sat at desks and listened to the test results. The doctors drew pictures on the blackboard of a normal heart’s anatomy and physiology. I was fairly familiar with that from my anatomy classes in college. Then they drew a picture of Alex’s heart.

I remember staring at that blackboard dumbfounded. From all of the anatomy and biology classes I had had, I knew Alexander shouldn’t have been alive. I kept looking at it in disbelief thinking they must be mistaken. All the while they talked. Oh, I’m sure I hear them, but my mind kept asking itself how he could be alive.

It was in that meeting that the doctors told us Alex had Hypoplastic Left Heart Syndrome (HLHS). I had never heard of that syndrome before. I asked what could be done... a heart transplant? the doctors told us we had two options: the Norwood Procedure or do nothing. If we did nothing, Alex would die. There was no hesitation on our part; we chose the Norwood Procedure.

The doctors stabilized Alexander for the weekend and he was the first surgical case Monday morning. The doctors had made it absolutely clear that most babies pass away before even making it to surgery. The next highest mortality rate was within the first 24 hours following surgery. Is it any wonder that I was terrified at the thought of leaving the hospital? I was afraid to leave Alex; it might be the last time I would see him alive.

The night before the surgery Frank and I stayed at Alex’s bedside. I spoke with the nurse on duty, J.R. We held a practically normal conversation, but most of the time I talked, I only used part of my brain. The other very conscious part of my brain was staring at Alex and praying that he would survive. I prayed for him to be strong and to live. I prayed for God to give me the strength to somehow get through this.

At one point in our conversation I told J.R. that the hardest part of all this was not being able to hold Alex. I told him how my arms ached for him. I hated that he was hooked up to all kinds of machines, and I could only touch his hand and stroke his hair. I don’t remember J.R. making any kind of response, but I remember feeling a little better for telling somebody the anguish I was feeling.

Before long it was midnight. Frank and J.R. had been trying to persuade me to go home and get some rest. I was so afraid to leave Alex. I just couldn’t bring myself to leave him. What if he needed me? Finally J.R. ordered Frank to take me home and to come back at 6:00 AM. He promised he would call if Alex’s condition changed at all. Frank finally dragged me away at 1:00 AM although I don’t even remember walking to the car.

Sleep came and left instantly. In no time I was back at the hospital. I was practically running down the hall, my heart beating fast. Was my baby alright? J.R. greeted me with a smile which was quickly replaced with a serious look. I tried to look past him at Alex, but I couldn’t see him. J.R. was talking, “I have some good news and some bad news. The good news is that Alex had a good night. The bad news is that I didn’t have time to bathe him. Do you want to do it?

Did I want to do it?! I felt my eyes fill with tears. J.R. gave me a basin and I filled it with warm water. Then he gave me a washcloth and I set to work. Alex seemed so tiny and frail. I must have bathed him dozens of times before, but this time felt so different. I carefully washed him and talked to him just like I did at home. I know he was sedated, but I like to think he heard me anyway.

When I had finished the bath J.R. said he wanted me to do one more thing. Then he told me where to stand, and he handed Alex to me. He told me he wanted to give me a chance to hold Alex before his surgery. Tears still fall thinking about it. We both knew it might be the last time I would ever hold him. I held Alex and told him I loved him, and I turned to Frank. I asked him if he wanted to hold Alex. He shook his head and told me I could hold him for both of us. I’ll never forget that moment. I’ll never be able to thank J.R. enough for what he did for me.

After bathing and holding Alex, I had to go and pump my milk. Before we knew it Alex was being wheeled down for surgery. Frank, Chrissy, my parents and I went to the waiting room with heavy hearts. The doctors had not given Alexander very good odds of surviving the surgery. We had to pray for a miracle.

It was not such a shock to see Alex after the surgery even though he was puffed up and had tubes and wires attached to him because I had met another family who let me see their son. Seeing Joshua prepared me for what Alex would look like coming back from surgery. He had a big bandage down the center of his chest covered with iodine. He was sedated and paralyzed, but he looked beautiful to me.

Sometimes being in denial is a good thing. Being in denial allows you the luxury of thinking like a normal person. It’s because I was in denial that I was able to function throughout most of Alexander’s ordeal.

When Alex was brought back from surgery he was put in a little room all his own. It was then and there that I brought out his quilt. He still had IVs in him and he was bloody, but I didn’t care. In that sterile environment so void of feeling I had to introduce something familiar to him, something that smelled and felt like home, like us, like love. It was one way for me to have a little control over this experience. There were so few things I had control of while he was in the hospital.

Alexander’s quilt and the nurses playing audio tapes of music we listened to at home gave Alexander a sense of being loved even when I wasn’t there telling him so. Perhaps the most important thing I did with Alexander, though, was to spend hour after hour with him taking care of him as much as possible just like I had done at home for the previous two months.

Bathing Alex after his surgery was important to me so I informed the night nurse I would be back by 6:00 AM the next morning so she wouldn’t do it. Another thing that was very important to me was to do everything I could to help Alex get better as fast as possible. I knew the longer he was in the hospital the greater the chance he had of developing infections.

The RTs were very helpful in assisting me accomplish that goal. At one point Alex was getting breathing treatments every three hours (Q 3). they had a tube they put near Alex’s face, like the one I held in the ambulance, that produced a cloudy mist. Some of the RTs encouraged me to direct the tube for them. They would then simultaneously beat on Alex’s chest with a dark blue object (clapper) turning him first one way and then the other. They explained that by beating on his lungs they encouraged the junky stuff to be flushed out of the body.

At one point I remember saying, “I’m so glad Alex has you, I could never do that.” I must have looked doubtful. The RT smiled and told me that he would teach me how. He also went on to tell me that Alex didn’t have to have a breathing treatment in order to have the percussions (beating with the clapper) done. He told me that Alex could have percussions done every hour - and it would help the atelectasis resolve faster. The next hour my mother, the nurse and I were turning Alex and doing the beating as a team. After that, Mom and I took turns. I’m convinced that is how Alex was able to be discharged from the hospital so quickly.

Our discharge was not quite as soon as I would have liked it to be. I was originally told that Alexander could go home on Sunday if his x-rays looked better. The residents told me that Alex could leave so I took Alex off the pulse oximeter, had his clothes on him and had car seat ready when I was informed that the surgeon wanted Alexander to stay one more day. I was devastated by the setback.

The night before Alex was discharged the PSCU was abuzz with activity. Dr. Calhoon was going to do a baby heart transplant. It just so happened that the baby was the son of a teacher who worked in the same school district as my father. After I finished singing Alexander his good-night song, my mother and I headed for the waiting room.

The waiting room was full of people. There were coolers of drinks and even pizza! I got the feeling that these people were having a party - yet a cloud of concern enshrouded the room. I introduced my mother and myself to David and Shelly LeCroy.

We struck up an instant friendship. We had so much in common. We both had two sons about the same age. Nicholas had also had a Norwood- but, his had not “taken.” Now they were waiting as the doctor was going to transplant another heart into their baby. I was filled with compassion for these brave parents. And I was touched by their sadness over another child’s death being their child’s chance for life.

Every time I went back to the hospital, I went back to see Nick. He was another source of inspiration for me. To see Nicholas and Alexander, you would never guess all the surgeries they have been through.