On August 7, 2011, a little before noon, I took H-Bear to the E.R. at St. Francis Children's Hospital in Tulsa, Oklahoma, after having difficulty keeping her conscious in the morning, repeated vomiting, and the worst night's sleep we'd ever had. Her breathing was labored, and I was scared but as my memories attest, fully unprepared for what was to come. She had just celebrated her 2nd birthday two weeks before and the day prior we'd had a fun time around Tulsa visiting Uncle Hef and Aunt Katie:
|Flying a plane at Tulsa's Air Museum
The E.R. staff got us back pretty quickly, and the first sign that something was very wrong was that her oxygen saturations on the fingertip pulse ox were 70%. I saw a worried look pass between the two male nurses on intake, and they whisked us back to an E.R. bed where she got hooked up to oxygen pretty quickly! I'd never really taken anyone to the E.R. other than M-Cat for some stitches when he was nearly 2, so it seemed surreal when they wanted to admit her right away. She was put on the floor and began treatments for asthma. This seemed to make sense. We were in a new environment where the high temperature the day before had been 115 degrees Fahrenheit. There was even an Ozone Warning. Josh decided by evening to leave his first "alone weekend" in years and drive the 8+ hours to Tulsa to be with his baby girl.
Overnight we had some good, smiling moments, but she didn't seem to be responding to treatments as the doctors had anticipated. She had been admitted on a Sunday afternoon, so the best and most staff were not on duty, and we didn't really see a doctor until Monday. That doctor came in on rounds mid-morning and ordered H-Bear's first VBG (venous blood gas). Her CO2 level came back at 110. Deadly high, though we didn't understand that at the time. He told us we were going right to the PICU and that H-Bear needed to be intubated.
I have never been so glad to have my husband by my side. Over the next few hours, H-Bear was transferred to intensive care, where her new doctor let us wait a bit longer and test her again. When her CO2 remained high, indicative of acute respiratory failure, he told us we really must intubate. We left her in the hands of doctors and nurses and respiratory therapists and held each other in the bay window of the family waiting room, where we could see her doorway across the courtyard.
When we returned, our baby girl was knocked out on Fentanyl and Versed, a long breathing tube out of her mouth and tape across her face. She also got a feeding tube through her nose. Then, the long waiting and confusion began. For 12 days in Tulsa, she remained intubated with no clear explanation for her respiratory failure. A special sensor in her tube monitored her diaphragm pulses and doctors were mystified at the low level of muscle activity that would generate breathing. In hindsight, the oxygen they were administering and the painkiller/sedative cocktail that coursed through her veins were both suppressing her body's will to breathe, but they were keeping her alive and reasonably comfortable.
I slept very little during those weeks, lost some weight in addition to most of August, and prayed continually for my girl to be restored. I distinctly remember and will not forget...
- My brother sitting with us for an entire week, loving us
- My dad flying to Tulsa and my parents keeping M-Cat entertained all week
- Taking M-Cat to see Winnie-the-Pooh at the theater before he flew home with Grandma to start school
- Our church sending a dear friend, Uncle Jon Jon to sit by our side for days after my brother returned to work and our family back home, and to run errands for us like picking up a DVD of ...
- Shaun the Sheep, the only thing that seemed to consistently bring a smile to our girl's face
- Denise's sister and brother-in-law (whom I'd never met) visiting us in the hospital and just hugging me while I cried and gushed
- Cards and photographs and child-draw pictures and gifts and wads of cash that arrived in the mail from our MOMS friends
- Sitting in the "solarium" with our doctor enumerating some of the awful things that could be causing H-Bear's distress (including one at which he paused and looked at us with the kindest eyes and said, "Oh, I really really hope she doesn't have that...that would be awful," -- and she didn't, thank God)
- The on call PICU doctor we saw for one night who laughingly said, "Looks like Ondine's curse to me," explaining the mythical story of Ondine more than the CCHS that causes this condition
- Many sleepless nights researching what could be wrong with her, including the night Ondine's Curse was mentioned, when I found the CCHS Family Network, which had a study of less than 200 families with CCHS -- so rare, I thought, "It can't be that. How do these families live with this?"
- The doctor who told us the morning we left Tulsa that the next course of action there was a tracheotomy and a permanent feeding tube
There is so much still so vivid, I could write a book of my memories of that short time. I carry so many beautiful memories, too, especially when H-Bear was finally approved for a life flight back to Nashville:
- Flying on the tiny plane over Nashville, I thought my heart would burst for fulfilled desire
- The friends and family who came to Vandy to see us after so many messages and prayers from afar
- The Resident who ordered the test for CCHS the very first day we were there
- The doctor who listened to my research that Fentanyl and Versed could be causing her apnea, and took her off these standard PICU drugs
- H-Bear successfully extubated 2 days after returning from Tulsa
- A sweet friend coming to visit her and play on the regular floor, even though she couldn't walk or barely even sit up on her own yet
I remind myself of these beautiful memories, the things that went right. I remind myself that we took her home for two whole weeks before we got the CCHS results back, and even after that she wasn't ventilated for another week and a half while we observed her at home on her pulse oximeter. That other CCHS parents gasp now as they read this because they know their child, like mine, could have died during that time, and our child didn't by the grace of God. That our child lived for two years with a condition that usually presents at birth, a blessed time filled with happy memories, a time to celebrate my brother's wedding (5 weeks after she was born), mourn the loss of Josh's grandfather (6 days after she was born), and acclimate a self-centered older brother to a new sibling. How much we would have missed if her CCHS had presented typically at birth! I recognize the blessing that her road has been much easier than most CCHS children, as has her family's.
At the same time, I feel loss and fear because of her unique trajectory. When I go to Heaven and seek answers for things that have not made sense in my life, right up there will be, "How could my child live for two years with this genetic condition with no signs? What caused it to suddenly catch up with her?" I had a normal child through her second birthday, and I feel a sense of loss that I would not had she stopped breathing in the hospital right after her birth. Instead of the joy of release from an extended NICU stay, the steps of weaning off the ventilator during the day, and learning to eat without a feeding tube, H-Bear suffered a sharp decline, and it has made me perhaps more fearful and less hopeful than many CCHS parents.
The memory that haunts me is how quickly she went from happy and well to nearly dead.
|The day before her hospitalization in Tulsa.
I had PTSD before, but I'm not sure there's a category for our current experience. Since last August, H-Bear has been in the PICU twice and the ER another time for respiratory distress. Of the four rapid hospitalizations, three happened with little advance warning. A happy girl was playing on morning, and could barely breathe in the evening, even with her ventilator's assistance more recently. On each of those sudden occasions, she had been out in the heat preceding, so we have stayed inside most of the summer, incredibly dispiriting to our nature loving family. I can't get past the memory, though, that keeps recurring. I quietly wait for it to happen again, because I know it will. It's nearly impossible to predict the timing. The best I can do is be prepared and look for the warning signs. We are getting better at that -- we kept her out of the PICU the last round and treated her at home, after given that choice by the ER.
Shortly after H-Bear came home on the ventilator, I drove past a park with my mom on a beautiful fall day and I said, "I don't think I'll ever feel carefree again." A year out, I have learned to enjoy my children more and many simple pleasures I previously took for granted. Nonetheless, the memories haunt me, and they are a constant reminder that I must be vigilant with this one, this precious life before me. I try to remember the love and goodness, the uncommon generosity that we've received in the year past.
Tonight, one year, later, August 7th, 2012, H-Bear's sleeping with her BiPAP after a busy day. First, we had a good visit to the pulmonologist ("I'm happy about this visit!" he declared.) Then, we had some lunch and nap time at home. We rounded off the day with a trip to our favorite bounce place, Monkey Joe's, where my little gymnast was on top of the world, later eating her biggest dinner in quite a long time. We played dolls and watched U.S. women advance to the beach volleyball Olympic final (I think she'll make a great volleyball player someday with her 6ft+ projected height). One of the best days we've had in a while, I'll hold on to this memory.