The Value Of A Life

The Value Of A Life

Krystal L. Smith

I found myself sitting terrified in a perinatologist's office, waiting to determine if the blood screen for trisomy 18 was truly positive. After a level II ultrasound, the doctor said he did not see any markers of T18. I was offered a CVS, and opted to have it. As we chatted about the testing procedure, he pointed to the door and said, “If you look on the bulletin board in the hallway there are women on it who had a trisomy diagnosis, terminated, then had a healthy pregnancy and baby the very next try. If you wanted to be more discreet we can send you to another city. If you choose to carry, we will support you, but your baby will suffer when it is born. He will suffocate. They can give him morphine, but it will be very painful.

In medicine, I have heard it a thousand times. “I took an oath to first do no harm.” What I have found in my time spent in the hospital, is that what is ethical is extremely subjective. I have many thoughts on what the value of life is, how human beings should be treated, and ultimately the role of healthcare professionals in deciding the treatment of any person in need. My goal is to express the view point of a mother with a medically complex child on matters such as ethics, treatments, and research.

When I look back on the conversations I had with a variety of doctors during my pregnancy, I cannot remember a single time hope was offered to me. I was never told there are living trisomy 18 children. The terms used most frequently were “not compatible with life,” and “not a viable pregnancy.” I chose to carry my son to term, though I was told he would likely pass in utero. There were many days in that first trimester I wondered how I could carry on. My heart so full of love for a baby who wasn't going to be here very long. How will I do this? I promised him from the time I knew he existed, I will give you your best chance. I decided that wasn't going to change because of a diagnoses. From then on, I did everything in my power to help him. I ate well, I exercised, I took supplements and vitamins, I drank water until I would nearly float away. Anything I could do to help him, I did. As time went on, I began to have a little hope; cautious hope.

Calloway Augustus, “Gus” as we lovingly call him, had to be induced at just over 41 weeks gestation. The baby who wasn't supposed to make it to a live birth was going to be born. I had met with a team of doctors and nurses who said they would make sure my birth plan was understood and followed. They promised Gus would receive the interventions I asked for. Everything was planned. NICU was supposed to be there for his birth, everyone knew I wanted full interventions unless otherwise stated. I was assured that we would be taken care of.

December 6, 2016 a beautiful baby boy entered this world. He was born without a doctor present, without the NICU team we were promised, and ultimately lived in spite of the lack of care. We had been told he could be monitored in the delivery room to remain close to us during our prenatal care conference. All of a sudden this was not true, and we were told we could just hold him and spend time with him alone. If he turned blue, they said they could be back in 10 minutes. My family insisted they take him to the NICU since they refused to monitor him in the delivery room. Away little Gus went, crying and breathing on his own.

The care we received in the NICU was poor. While they did give him oxygen as needed and a feeding tube, they refused to do anything else. My mother and sister had done extensive research on living trisomy 18 children and knew the common problems associated with the chromosome abnormality. They knew what tests we needed and why we needed them. We were refused all testing we requested with the exception of an echocardiogram. At one point I was told by a doctor that nobody sees the economic value in trisomy children and that it is considered silly to intervene. He told me he had a child with a chromosome abnormality, and that there was no way he was going to give up his lifestyle to deal with that. He and his wife terminated the pregnancy. He told me people will be cruel, say horrible things to me, and that my marriage will crumble. He said my life would be over, and that I am a much better person that he is. While he stated he was a Christian, and his wife is a saint... I am certain he is very confused about what that means. We were sent home after two weeks and remained there about ten days before sweet Gus started having O2 desaturations to the point we had to take a helicopter ride to a children's hospital. We never retuned to the hospital I gave birth in.

Upon arrival, we were told Gus likely had a respiratory virus. With Gus's diagnosis, he knew that we wanted as much time with our son as possible and they hoped to give me that time. I looked at him and told him I know his diagnosis, and that our goal is to get well and go home. He stated it was clear that I understood what Gus has, and he will put a note for other doctors not to give me “the talk” again. He was treated for respiratory distress and recovered quickly. While we were there originally for respiratory distress and apnea, other problems were discovered and discussions about treatments and the degree of interventions began to be discussed. I was told repeatedly there was no reason to do a sleep study, that apnea was apnea, and what difference was it really going to make? I was told that a tracheostomy would not be helpful to my son because he had central apnea, and he would be vent dependent with no quality of life.

This doctor with another backing him up, went as far to tell me he hoped I would be reasonable and not do “too much.” He claimed we do too much for these kids in this country. He also stated that if he was in a car accident, he would come back to haunt his wife if she ever got him a tracheostomy and a vent. He said he told her to let him die, because he would be miserable. One of the reasons he said it would be unfair, is that my son would not be able to swim under water and I would never hear his voice again. His idea of quality of life is apparently going under water in a swimming pool. According to him, once you are trached you are always trached and nobody ever gets off of a vent. He pressed me to answer him about whether or not I would consider a trach for Gus, and I mumbled well no, I would never want to hurt him. During this time, I knew little about trachs or vents. I only had the information he wanted me to have. Unfortunately, I was only being told one extreme in a large spectrum and being fed a lie that suited this particular doctor's “ethics”. Later, because of a very special pulmonologist who has living t18 patients, we were offered the sleep study that I had desperately wanted. It was determined that Gus did not have central apnea, he in fact had the most severe case of obstructive apnea this hospital has ever seen. After doing my own research and speaking with the tracheostomy nurse, I learned children get decannulated all the time and vent weening is standard procedure. Yes it is possible you always have a trach or always need a vent, but I was told nobody ever gets rid of either. The trach nurse and I talked about what life looks like with a trach, and she explained Gus could have a great quality of life! Even if he needed a vent, it would not keep him from a great life. She explained the trisomy children are her favorite, and they definatly have a place in this world. Her view was quite different from the other two doctors who continuously told me a trach was a bad idea. As I look back, I remember thinking, isn't not being able to breathe a worse idea?

One of the repeated arguments I hear is how uneconomical it is to intervene for trisomy children when they have such a limited life expectancy. The question is often why would we do anything, go to all the trouble and spend all this money on a child who will be a vegetable. There are several points to be make in opposition to this argument. First, trying to base the value of a human life in monetary terms is absurd. Ask anyone who has lost someone they love what they would give for one more day, 5 more minutes, or one last hug. The answer is usually a simple one. “Anything.” Have you ever heard someone say, “Well, I would give 200 dollars, but any more than that would be uneconomical.”

It seems discriminatory that there are some chronic illnesses that are deemed worth treating, even if the outcome is dark, but others are deemed not worth the effort. I have always compared it to things such as cancer or cystic fibrosis. We don't just give up on giving those kids their best chance, so why is it that something like an extra chromosome devalues your right to treatments and your best chance at life?

There is a huge misconception when it comes to trisomy 18 in particular. There are living children who are thriving. They are far from the vegetables that their parents were told they would be. These children are full of personality. They smile, laugh, cry, and communicate. I am puzzled as to why these stories and the wide range of outcomes is not explained to the parents by physicians. Why are doctors not reaching out to groups such as Rare Trisomy Parents on facebook to see the wide range of trisomy outcomes and all the things these children can do. At what point will physicians start looking at the dates of the research they are reading and realize this is a different day and age. The majority of studies conducted were in the 1960's. Medicine has come a long way since then, the problem now is an ethical one.

Another argument I often hear from the medical field, is that these children are burdens to caregivers and ultimately destroy family relationships. I would argue that trisomy 18 children are some of the most loved in the world. I do not know one parent of a living or deceased trisomy 18 child who regretted their decision to carry their baby and do all they could to help them live. My child is not a burden to me or to my family. We love and cherish every moment, every single day. No one feels burdened. The biggest burden we carry is the fight for equal rights to medical care. The stress from this aspect is the most unbearable at times. Our children teach us lessons others may never know and fill our hearts with the most joy imaginable. To call them a burden is insulting.

Every child is an individual. The range of outcomes goes from mild to severe with all trisomies. Not everyone fits into one box. How can we limit interventions when we cannot say with certainty how a child will be effected? Is it not worth trying simply because there is an unknown factor? Would we do that to a child who does not have an extra chromosome? What is it that determines who lives and who dies, who gets treatment and who doesn't, who is worth the effort and who is not? A diagnosis is not a prognosis. To deny a child care, who cannot speak for themselves is unethical. Doctors have a right to deny care if they feel it is unethical to intervene, but they should refer parents to someone who will treat the child if they see it as the best option. Often people think they know what they would do if it was their child. But truth be told, you don't know until it is your child. What is the value of a life? It is invaluable. You can't put a price tag on love.

Against All Odds

The story of Gus's birth is a hard one to tell. It is such an important piece of the his story, and it needs to be told. While the pain of that day will always haunt me, the miracles that followed from his first breath and every day after are a testimony to the power of Jesus.

We pulled into a gas station to fill up before the 37 mile drive to the hospital in North Austin.  It was induction night. I had several bags packed, pillows, my diffuser and lavender oil, and keepsakes for Gus. I started crying as I waited for my husband to fill up the tank. I cried the whole way to the hospital and balled when we parked. It was cold, it was dark, and it was time to have my baby. I didn't want to go. My family helped me drag all my things into the hospital. We had to wait an extra 30 minutes for our room to be cleaned, more time to worry and more time to be frightened.

I got to my room, put on my hospital gown and waited quietly for the nurse. My first nurse was amazing. I will never forget her. She had a cousin who had a T18 baby, and she was very sweet and uplifting. She took excellent care of me and monitored Gus so carefully. She made sure she slowed down the pitocin if Gus was stressed. I adored her. I will always wish she had been there the next day when it was time to push. But she wasn't. Another nurse came on duty at 7 that morning. This is when it all started to go wrong.

By 1 pm it was time to push. This new nurse told me to bear down and push as hard as I could and to take a breath and push again. It didn't take too many pushes until Gus crowned. There was no doctor, but the nurse said she had called him and he was on his way. Instead of waiting for him, she continued to order me to push, bear down, as hard as I could. I told her I felt like he was going to come out.... and he did. Gus's head was hanging for minutes while this nurse frantically yelled at my sister to pull the emergency cord. Nobody knew where it was, there was no label that said "EMERGENCY CORD".  After finally finding this cord and pulling it, "the stork" was called over the loud speaker repeatedly. Nobody told me who the "stork" was or why he was being called. Actually nobody told me anything.

Next thing I know there is a man with glasses and a surgical hat and scrubs on staring at me from below my legs still tucked into the stirrups. He says nothing to me. Gus is taken to the warmer and the NICU team comes rushing in asking what is going on. The Stork, is talking to the nurse saying there is a lot of bleeding, and the cord is too fragile, therefore delivering the placenta is going to be difficult and risky. Again, he says nothing to me, just looks at me with ghoul looking eyes. Nobody reassures me, nobody tells me if Gus is ok. My mom and sister just keep saying Gus is perfect, he is perfect. I see my husband crying. I am in a fog. I don't know what is happening. Chaos ensues.

My OB shows up, says nothing to me, just barks, "Somebody turn on the lights in here". Mumbles something about me having several tears, and starts sewing me up. At no point did anyone ever tell me how Gus was, or say I am so sorry I wasn't here. There was no explanation for how or why things were so out of control. 

I hear the NICU nurse asking what we want to do with Gus. She has the birth plan in her hands, I have met with her previously, I have had several email correspondences about what I wanted for Gus, yet here she is, questioning repeatedly what my wishes are. My sister asks if we can have a pulse ox to monitor him, we are denied a pulse ox. They tell her that they cannot leave one in the room unless a nurse is present the whole time. My sister says take him back to NICU to be monitored, and again they ask if we just want to hold him and keep him with us. They say if he turns blue they can be back in 10 minutes. I cannot understand why this is happening. We met with everyone before Gus was born. Everyone knew the plan. We were told he could be monitored close to me in the room unless he needed extra support. Why are we now being told that wasn't possible, and why is everyone acting like it is ok to just leave us alone with him? The trauma of the whole thing is not something I will likely ever be able to express in words.

I can't really say what happened in between all of this. I know at some point my husbands family was allowed to come in, and Gus was in my arms for a little while. There are pictures of this, though it is so foggy in my mind. Gus and my husband are taken to NICU, where Gus did turn blue and his oxygen saturation plummeted. Daddy was holding him when it happened. What if we had just kept him with us? Would he be here right now? NICU could be there in 10 minutes? Gus would be dead in 10 minutes. Looking back, I recognize that Gus was indeed supposed to be here. He survived what I would call a traumatic and horrible birth into this world. I wish I could say it got better, but it really didn't.

Gus was given a feeding tube through his nose, and placed on oxygen. He was given formula without my knowledge, and did not tolerate it. He was then given IV fluids. By the time I was released to the antepartum wing, it had been several hours. Eventually I was able to be wheeled over the the NICU to see him. He had the deepest eyes, so blue, like pools of water. I could see that Gus had knowledge beyond his age, as if he was a very old soul. By far, he was and is the most beautiful thing I have ever laid eyes on. There is something about him that draws you in. He is mesmerizing.

There are many more medical details I could go into, but this story isn't really about the medical aspect. It is about the human aspect. I was assigned a primary doctor in NICU, who really didn't want to do anything for my son. They went through rotations, we would have a different doctor for a day or two, then the primary would return. I was refused an EEG and told Gus was not having seizures, I was refused an airway scope, but finally got one which I would deem as completely inaccurate. I was refused a sleep study and told Gus had central apnea. I was refused a visit from pulmonology or any other specialty for that matter, and told that I could see all of these people out patient. They did get Gus a cardiologist and that was how I learned Gus did have a VSD and a small PFO. I was told that room air was not an option at home, and that such a machine for home use did not exist. Someday, I will type up a list of what I asked and what I was told, because there were so many ball face lies, it is truly shocking. There was an article titled, "Is Your Doctor Lying To You?"... let me spare you reading that and just say from my own personal experience... YES, it is highly likely you are being sold a bill of goods that is no where near the truth.

It is hard to imagine that the people who swore to do no harm, are actually the ones inflicting harm on innocent babies by denying them the care they deserve. Yet, I promise you, this happens every single day, and if you are not educated on the issue at hand, you are at risk of becoming a victim. Thank God my sister and my mother spent my pregnancy researching. I wasn't able to do so while I was pregnant. All I could do was get Gus here. My family advocated for Gus, and while we were denied so many things he needed, we got enough to keep him alive, and that is what this next paragraph will be about... going home.

Two weeks of NICU and it was time to go home. We had fought for home equipment, and thankfully had blow by oxygen and a pulse ox to monitor Gus with. We had a feeding pump and NG tube for nutrition, and caffeine for his "central apnea".  Home was better than the hospital, but still horrible. The monitor would go off all the time, feeding was so hard because it had to be done every three hours over the course of an hour and then you had to clean the bag. By the time you checked tube placement, changed a pamper, ran the pump and cleaned it, plus jumping up every 5 minutes when the monitor dinged to make sure he didn't need blow by... it was time to do it all again. Nobody slept. It was a nightmare.

New Year's eve, at around 5:30 AM I had to call 911. Gus's oxygen saturations were dropping into the 40's and I was having trouble keeping him up. An ambulance picked us up and we went to McLane Children's where Gus was well taken care of and stabilized, then took a helicopter ride to Dell Children's. He was admitted and they believe he had a respiratory virus. It was truly a blessing to have to come here. This hospital has treated Gus a million times better, and the team that eventually was formed has gotten him the things he needed. It was found that Gus does have seizures, he does not have central apnea, it is obstructive. His airway is the worst obstruction they have ever seen, and it is miraculous that Gus survived at home. That little boy has the kind of fight in him that doesn't quit. Truly, he makes it look easy, though I know it was not easy at all.

While so often I am faced with medical professionals who insist that the worst case scenario is always the outcome, my little mister continues to prove this false. He brings so much joy to his family and his nurses. I take videos of him doing all the things a 2 month old is supposed to do and force doctors to watch them. I am in contact with other families who have living trisomy 18 children and I share their children with medical professionals as well. It is hard to understand how they can not know these children do exist, especially in an age where information is a click away. Yet here I sit daily, shocked at the misinformation and ignorance stated by doctors.

It is a fact that there have been very few studies on trisomy 18 since the 1960's. I find those studies to be ridiculously outdated and not something to put any stock in. The statistics are skewed, mostly because people believe what doctors tell them and assume their child has no chance of survival so they choose comfort care with no interventions, or they abort. How many kids would live and have a great quality of life if we gave them the opportunity? Well we don't know. I can't say for sure the numbers would change, but neither can doctors... why? because there isn't any research. There are many still births and miscarriages with trisomy 18. There are babies so severely effected that they probably are much better off just being in their mothers arms. A mother knows when it is time to let go. Every case of trisomy is different. Children are effected differently. Ultimately, the parents should be the ones making choices for their children, not a doctor. The push of medical "ethics" is actually unethical. If a medical professional is not educated in all possibilities, they should learn. If they know the possibilities and only give the worst case scenario, they should lose their license. A family needs all the information, not just the portion that suits the doctors beliefs. But that is for another post another time.

I was told to include my religious beliefs in my birth plan and if I wished for a Chaplin to be present. I wrote that I did not want a Chaplin, and that we believed Jesus himself would be there. It is clear to me that Jesus is always there. Even in the most heartbreaking situations, He remains. We have to actively reach for him because the chaos and pain in our lives will steal our awareness of Him. Miracles are not just the big things in our lives that cannot be explained. Miracles are in the every day, every moment things... like breathing. Gus was born alive against all odds. Gus went home against all odds. Gus lived with the most severe obstruction this hospital has ever seen, against all odds. Gus went through a large multi surgeon surgery and survived, against all odds. Gus went into recovery with no complications and is healing, against all odds. This child was meant to be here. There is a plan for every life. Gus has a purpose. He deserves his best chance. WE ALL DO.