Thanatophoric Dysplasia is a million dollar word. Like a lot of medical terms the word comes from the Greek language. The term Thanatophoric Dysplasia means literally "a death causing inability to form." It is an acurate description of Kolby and other TD kids. All forms of Dwarfism are essentially a skeletal inability to form in one way or another. There are inherent weaknesses in the form of the skeleton. One of the greatest weaknesses in children with Dwarfism is the neck.
As his parents, we decided on an operating principle that would guide us all in our decisions. The principle was this: we would do whatever we felt was best to give Kolby the best shot at a quality life. The weakenesses in his neck coupled with his respiratory deficiencies made the choice between a vaginal birth or a C-Section difficult. On the one hand, a C-Section would reduce the dangers of causing him physical harm during the birth process. On the other, a vaginal delivery could help clear his lungs and help him to breathe.
Our Doctor is wonderful. As an OBGYN who faced a rare and tragic pregnancy, he handled himself with poise and compassion. He gave us all the information we could ask for, and when we decided that for us a C-Section delivery was best according to our guiding principle, he supported us. We know that he was uncomfortable with our decision, and he had made some valid points to support his preference for a vaginal delivery, but our heart was telling us that we wanted to deliver Kolby injury free. We couldn't help him breathe if he had a broken neck. We thank our Doctor for his support and for empowering us to make that decision.
Our due date was January 2, 2004, but on Wednesday, November 26, 2003 Mom's amniotic fluids began to leak. Fortunately, we had a scheduled Doctor's vist that morning at 10:00 so we didn't panick. The Doctor confirmed that it was amniotic fluid, and we were scheduled to have a C-Section later that afternoon.
How we would have loved to have a few more weeks! Kolby was old enough to survive, but lung development is late in a pregnancy and his rib cage was so small that we feared his underdeveloped lungs simply wouldn't be able to provide him with enough air. But, there was no choice. He could no longer remain in the womb as it was no longer safe for him. The day of his birth/death had finally arrived.
When we arrived at the hospital and checked in, we were led through to the rear of labor and delivery through two sets of double doors into a wing that was dark and quiet. This was the section of the hospital where difficult pregnancies were placed. Mothers would be able to recover from delivery without hearing all of the happiness and commotion in labor and delivery and postpartum. We would be able to rest in silence after Kolby died.
When Mom's nurse entered the room she reminded us of a sweet grandmotherly type. She was nurturing and soft spoken, and shared that she too had lost babies in her past. She could sympathize with us and offered herself for whatever we needed.
After a short while, they took Mom away to prepare her for surgery. After Dad washed and changed into his scrubs, he looked out the window. He could see a park and some light traffic on the street below. He watched a nurse climb into her car, put on her seatbelt and leave. An elderly couple held hands as they tread slowly along the terrace. A bird clung to a powerline. It was clear that life was going on outside, but Dad felt like it had stopped from where he was standing. As a light rain kissed the window, a tear migrated down his cheek. He felt all alone.
Mom, meanwhile had been led into the surgery room. She was glad in a way that this was all finally coming to an end. She had felt miserable for several days with her excessive amniotic fluid, and the delivery of this child would mean physical comfort for her. Interestingly, she reports that she felt a great deal of peace in those moments. She was not all gloom and doom, and she actually experienced feelings of hopefulness. Perhaps, more than anything else, she simply realized that her days of worrying and suffering were over. Kolby's fate was now in the skilled hands of the doctors and in the tender embrace of God. She rested in His presence.
They called Dad and said that they were ready. When dad arrived they were just putting the spinal block in Mom, so Dad snapped a picture and then had to leave again for a few minutes. When they called him again Mom was surrounded by all the doctors and nurses. Dad sat next to Mom and asked if she was OK. She smiled and said she was. They talked a little as if nothing was going on behind the curtain erected just at her chest. Then everybody began to say, "There he is!" and "Hello Kolby!" Mom and Dad waited eagerly.
There was no cry, suddenly the Neonatal Doctor appeared from behind the curtain and we saw Kolby for the first time. What a beautiful child he was! We could immediately notice his features that were classic Dwarfism. He had short arms and legs, and a large head with frontal bossing on his forehead. His appearance was not unlike any other baby beyond these features.
They rushed him to the warmer and began to suction his nasal passages and mouth. Kolby grimaced and snorted, coughed once, but was otherwise pretty inactive. Usually babies flail and wail at this point, but Kolby appeared lifeless.
Quickly, some pictures were taken. Mom and Dad holding him, the Neonatal Doctor. Kolby in the warmers. Dad said goodbye to Mom and they carried Kolby into the Neonatal Intensive Care Unit. (NICU)
After conversations with the many doctors involved, and the research that they'd conducted earlier in the pregnancy, we had decided to give Kolby comfort measures only. We wanted to grant Kolby as safe a birth as possible with the C-Section, but after that we wanted to put him into God's hands. Let God's will be done. We had considered putting him on a ventilator, but didn't want to set ourselves up for the day when we'd have to decide to take him off.
Knowing these things, the Neonatal people had a room prepared for us within the NICU unit itself. Soft classical music played on a stereo, and the lights were down low. They placed Kolby into the warmer in that little room and connected him to a monitor. Dad cannot remember the exact numbers, but they were low. His heartrate was around 100 and his respirations were below 20. Shortly afterwards, the nurse asked Kolby's Dad if he wanted to be alone with him. When dad was alone, he scooted a chair right up next to him, touched him all over and spoke to him. Rubbing his hands and his heads, Dad told Kolby what a special little boy he was, and that his Mom and Dad loved him very much. He said that God loved him too. Dad told him that he was there for him, to help him die, or to help him live. Whatever Kolby could do he would do.
Dad cried. It wasn't the sort of cry that is soft and gentle as tears slip down your face and rest on your lips. It was the type of cry that takes all the air out of you. The type of Cry that makes you heave and make all kinds of embarassing noises. It makes your nose run too. When Dad opened his eyes he saw that his tears had landed on Kolby's head, and it reminded him of something that he had wanted to do earlier. Kolby'd Dad had been a minister at one time, and there wasn't time to call in a priest or pastor from the outside, so with Kolby's wet head, his Dad said softly, "Kolby, I baptize you in the name of the Father, and the Son, and the Holy Spirit."
Kolby was essentially lifeless in that room. His color was deep purple, and he kept making a clicking sound with his tongue. One of the nurses poked her head in to ask dad if he was Ok, and dad asked what that sound was. She said that he was gasping for air. It was like watching a fish lying on the bank of a river, slowly dying with his gills and mouth opening and closing.
"Can we give him some oxygen to make him more comfortable?" Dad asked. The nurse seemed to have been thinking that herself, so we put it on him immediately. He responded to the oxygen very quickly. His heartrate began to climb, he stopped making the clicking sound. He seemed to relax a little. Dad took a picture of the monitor (see the Miracle in Pictures page).
A short while later they rolled Mom into the NICU. The wonderfully compassionate staff of Home Hospital in Lafayette Indiana decided to allow her to recover from surgery with her dying son. Mom's hands were shaking from the spinal tap, but she turned and reached for her son. She placed her finger into Kolby's hand, and when he sqeezed back she was awestruck! His eyes opened and he seemed to be aware that we were there. His color had changed into pink by the time Mom came in, and he appeared to be stabilizing!
An epiphany is a manifestation of God- a moment in time that brings the Divine next to the human, the Eternal enters the temporal. That moment was the culmination of months of worries, of fears and anxieties, of prayers and supplications. For Mom and Dad, it was an epiphany. It was as if God had Himself entered the room and given life to Kolby.
If this was the only life that Kolby would have, even if he were to die within a few breaths or heartbeats, that moment was what it was all about. In our society people have the right to choose to terminate pregnancies for all types of reasons, including medical imperfections. Some of the doctors that we experienced through Kolby were of that opinion, too. Others were not. We can absolutely sympathize with parents who are making that decision, or who have made it. The idea of giving birth to a child who will die within hours is such a painful thing to face. It seems much easier to do a simple medical procedure and be done with it. We also admit the validity to the idea that early termination can be an act of love as it hopes to eliminate needless suffering for the child. But we would like to say to parents who are considering it, that we will cherish that epiphany moment for the rest of our lives. Again, the pain is intense, but there is also an intense joy. Weeping and celebration are concomitent events in a situation like this, they happen at the same time. With an early termination, it seems to us, there is only weeping.
