Paul brought me into Ava’s room to give me a brief explanation of why the doctors weren’t able to attempt Ava’s surgery and why she wasn’t going to make it. I called Kristin Spyker and asked her to help me get in contact with Stanford or Boston for a transfer. I had already called her from Kaiser while I was waiting on an update and she already knew something was going wrong. Shortly after an older CHLA cardiology doctor came in and gave me yet another brief explanation of why Ava couldn’t be helped. I immediately demanded to have Ava transferred to Stanford. He looked me in the eye and said that no surgeon on earth could successfully repair Ava’s defect. I simply replied with, “I need you to request a transfer now”; and the doctor walked away rolling his eyes.
My family had decided it was time for them to leave. Some thought I had lost it, others wanted to give me time and space. From the bathroom I could hear someone whisper, “She’s having a mental breakdown.” At times when I tried to contact Kristen I was treated like I was making a mistake. When I went to look at a text from Kristen Paul even said, “Is that your little Kristen” and another family member literally forced me off a phone call with her. My sole intent in these moments was to try any avenue I could to save my daughter. Sadly, this is a common occurrence in situations such as this. As emotions are strained, stress is high and confusion is everywhere it’s common for family to misunderstand and get angry with each other. Some family members were nothing but supportive while others had moments when they weren’t. I’ve spoken with multiple women who’ve had similar reactions from their spouses and family.
Hours passed before I was finally able to ask the charge nurse if the transfer request was sent. Paul asked where she was multiple times and we received no feedback. Ava’s surgeon was also not able to come talk to me on the night of the first. Despite the comment Paul had made about Kristen he was very determined to try to get a transfer if that meant saving Ava. When the charge nurse finally returned she said they spoke directly to Dr. Hanely at Stanford and that Dr. Hanely himself thought Ava was not stable enough for transfer but would not comment on whether or not he was capable of performing the surgery needed. I contacted Kristen again to give her the bad news. She had already been cycling through some connections to see what other options we had for Ava. Next we would try getting her to Boston. This was our last and only hope.
Kristen tried her hardest to get me in contact with Boston and although Paul had faxed Ava’s records we simply didn’t have enough time to wait for a response. Ava’s health was deteriorating quickly because of her pulmonary veins. When I first arrived at the hospital on the night of the first she was able to turn her head and look up at me with her big brown eyes. But by the morning of the second she was unresponsive in every way and frequently gasping for air, although she had a machine breathing for her. Her breathing machine sounded an alarm about every 15 minutes because her lungs continuously filled with fluid. It was very painful to watch but we held onto the hope of hearing from Boston Children’s Hospital.
Then 2:00 PM came and the machines were not enough to keep Ava alive any longer. Ava had already been resuscitated once during her transport from Kaiser to CHLA and we decided we wouldn’t have it done a second time. A Chaplin was provided and we were able to get locks of Ava’s hair, footprints, and hand prints. We called family to let them know they could come say goodbye. Ava’s health began to worsen very quickly. As we waiting for signs that she wasn’t going to stabilize we continued to kiss her forehead, play with her hair, and hold her little hands and feet. Once our family arrived it was apparent that we had only minutes, she wasn’t recovering and the doctors were not going to try anymore than they had. Ava was removed from life support and I held her in my arms as she passed. This took only moments.
This post has taken me such a long time to write because for the longest time I felt like I had failed and I was ashamed to admit it. I had one opportunity to make a good choice as Ava’s mom and that was when I chose the hospital Ava was to be delivered at. I know now that once a Heterotaxy child is born into a hospital the fate of that child lies in the hands of the doctors. Although there are instances in which parents have more time to fight for transfers this is often not a possibility. What else could I have done but chosen the best hospital for her? That was really the only way I could have given her the best care because I wasn’t the one who was capable of providing her needed care. In earlier blogs I mention that CHLA and Kaiser aren’t necessarily bad hospitals but that they weren’t right for Ava. This fact is still true but I’m going to explain how ugly this fact can be for Heterotaxy patients.
I mentioned earlier in this post that I was told Standford was contacted, specifically Dr. Hanely. After Ava’s death it took months and request letters from a lawyer to receive Ava’s medical records from CHLA. Once I received the records I was astonished to find that no transfer request was completed or recorded. Her records made no mention of my request to have Ava transferred to Stanford or the supposed phone conversation had with Dr. Hanely. Her records simply said that a transfer request for another hospital was made but due to Ava’s severe condition transfer was not a possibility. This isn’t entirely untrue, Ava wasn’t in good condition, but I know now that more could have been done for Ava.
CHLA made no mention of ECMO or other methods of stabilizing Ava for transfer. It wasn’t until after Ava died that I learned from other healthcare professionals that more could have been done. Not only could more have been done to stabilize Ava but the records lead me to believe that no actual request for transfer was made. The doctor I initially requested a transfer from rolled his eyes at me while I stood next to my dying daughter. Last but not least I was assigned a social worker who callously made jokes in my CICU room about how, “These things happen”. CHLA had given up on Ava before I even entered the building. The only thing I could have done differently in that situation was deliver at the most experienced hospital.
I share my egregious story in hopes that if you’re on the fence about which hospital to choose, like I was, that you’ll choose only the best available. Although delivering at the best hospital available wont ensure that your baby live a long life or an easy life. In those critical moments, minutes, or hours after a Heterotaxy baby is born family will not understand, you will be confused and given little information, and un-experienced doctors will be just as confused as everyone else. How scary is that? A doctor who knows little about this complex specialized diagnosis, who is learning as they go, using your baby as a test subject, and has fears(as Ava’s surgeon did) that this confusing baby will likely die under their knife. Despite the uncertainty of a Heterotaxy babies length of life or quality of life I would do this all over again to choose a better hospital for Ava. Even if that meant only a little more time with her.
In the morning of the second, the day Ava died, I was able to have a short meeting with her surgeon. His eyes were red and as he gave me the Heterotaxy 101 speech with what seemed to be tears in his eyes I realized he was scared and lost. He told me he wasn’t going to operate because he didn’t want her to die under his knife. It’s hard to have someone to “blame” for Ava’s death. Were the pediatric cardiologists, perinatologists, and surgeons negligent because they didn’t refer me to a more experienced hospital and team of doctors? This idea goes against most hospital policies and practices because hospitals are businesses. Hospitals punish doctors for outside referrals. Dr. Montgomery admitted to me that he was reprimanded for giving me an outside referral for something as simple as a fetal echo.
Does that mean hospitals are to blame for the ugly “business” aspect of how they run? Hospitals see Heterotaxy babies as dollar signs, bottom line. Or is it my fault for not being educated quickly enough to make the best choice for my daughter? My confusion came from lack of understanding. My choice was made because I didn’t know if traveling was safe and I didn’t know for sure if CHLA wasn’t capable. Now that I know I’ll never stop sharing in hopes that parents will be able to make more informed choices. At the end of the day I can replay Ava’s story over and over again in my head and I’ll never know “who’s to blame”. To this day I have unanswered questions and I’m forced to live with this unknown forever.
I would like to leave you with the few positives I experienced at CHLA. Once a child dies it’s important to find a silver lining, a cause to live for, and to let go of blame so that you can be at peace. For my own peace of mind I share what I know but I try not to dwell in these moments. I prefer to remember the nurse who watched over Ava on the night of the first. She was a young woman who cared. Her attentive, gentle work will never be forgotten. This young woman even helped me change Ava’s diaper a few times. The Chaplin was very kind and sympathetic. As Ava was passing the nurses in her room were gentle and caring towards my entire family. One female nurse sang a lullaby and another male nurse sympathetically pronounced her death. The male nurse who had been watching over Ava on the second cleared the room quickly so we could have time alone to say our goodbyes, bathe Ava, and dress her for burial. These are the sad but peaceful “positives” I was able to take away from my experience at CHLA.