Princess Cloey: Life after Hospice

Sunday, June 24, 2012

Life after Hospice

Cloey did it again! against all odds, she was discharged from hospice in May after a year without using ventilator support and having minimal complications. Obviously we are amazed and so happy to still have our sweet Cloey with us.

So what does this mean? Well, Cloey was due for several maintenance consultations and procedures 2 years ago when we started the whole Hospice journey, so now all the things we put off need to be addressed. We also need to determine if she is truly "all better", whether by miracle, medicine or simply outgrowing her breathing (or lack of breathing) condition. Or, if she is just declining more slowly than we had anticipated. Figuring this out is VERY important. It's the difference between taking her off of her AND (allow natural death, formally DNR do not resuscitate) order and putting our efforts into teaching her life skills for her *gasp* future, or continuing to focus primarily on her quality of life and making her comfortable. Her doctors and specialists in Phoenix are not sure what to do with her. They are good doctor's but are simply not equipped with the multidisciplinary model of care or knowledge and medical advancements to manage a disorder never seen before in the world. To complicate matters, our sweet girl is also becoming a young lady. This means changes in her dietary needs for her feeding tube, and her hormone levels need to be evaluated. Not to mention, no one expected her to reach this point, so no one knows what these changes will mean for her or how they will affect her delicate system.

First, we need to determine if Cloey is a "Chiari kid". Basically, Chiari malformation is a congenital condition where part of your brain protrudes into the spinal column at the base of your skull, this condition is often seen in Spina Bifida. When Cloey was 2 1/2, on our first trip to Mayo, it was discovered that she had some stenosis (pinching) in her spinal cord at the base of her skull just under her brain stem. She had a decompression surgery done, where they took away some bone at the base of her skull and the first 2 vertebrae to relieve the pressure on her spinal cord. She did great and everything looked fine. The neurosurgeon explained that the procedure was the same they do for Chiari malformation, but that she did not have it. 2 years later on a follow up scan, it looked as though her spinal cord was at risk for damage again, especially because she was now walking and more active. Because her entire spinal cord has low fluid the only option was to fuse her entire cervical spine and eliminate all movement in her neck, otherwise further damage would leave her paralized from the neck down. The fusion was done at Barrow by one of the best surgeons in the nation and went very well, with no complications. Fast forward to July 2010, we took her to Cincinnati Children's hospital to sort out her breathing issues. The doctor reviewed her history and evaluated her. He said he felt very strongly that she had had Chiari, all of her symptoms align with damage to her brain stem where her autonomic functions are controlled. Things such as bladder control, breathing and heart rate regulation, eye dilation, digestion, everything your body is supposed to do automatically without thinking about it. She lines up almost perfectly to a "Chiari kid" symptom wise. Unfortunately, the fate that comes with this particular malformation is an inevitable pacemaker, colostomy bag and 24 vent dependence, among other things. I called her neurosurgeon that did the original decompression and basically said "but she doesn't have Chiari, right?" his heart was obviously heavy as he shared with me that he technically couldn't say she had Chiari because it is medically defined as brain tissue protruding more than 5mm, and hers was not quite 5. He went on to share with me that, when he did the surgery her brain (at the brain stem) looked like a bruised peach. He said he believed that damage lined up with her symptoms as well, making her unofficially have Chiari malformation. This is when and why we decided to put her on Hospice. But the decline didn't happen the way it should have if she had had a true Chiari malformation at birth, so we need to determine if the brain damage has repaired itself, basically if she "outgrew" the autonomic problems, or if we are still looking at Chiari, she is just stronger than most ;)

We will also be seeing-

Dietitian and GI to determine adequite nutrition and the possibility of future g-tube removal.
Urology to discuss managing her incontinence.
Orthopedic surgeon, to evaluate her growth and abnormal bone structure.
ENT to check her ears and evaluate her Tracheostomy
Sleep disorder specialist to determine if she is still not breathing on her own when she sleeps
Endocrine to evaluate her growth and hormone levels
Neurology to determine the extent of her brain damage

This doctors will all work together as a team to determine the best plan for Cloey from here. It is going to be a very busy week full of decision making, but we are cautiously optimistic, maybe even hopeful. Could all the fighting for her finally be paying off? Could we really have a future WITH her and WITHOUT a battle to save her life every six months? Could she finally experience life with fighting for it? We can't really believe any of this yet, but the spark is there, and no matter what we are grateful for every extra day we spend with her sweet spirit. Being here again, using donated funds, seeing Ronald McDonald House and all of the volunteers ready to jump in and help any way they can, all of it reminds us how blessed we truly are. This world IS still full of good people and kindness, and we are lucky enough to experience true love and charity once again. It really does take a village, and we are forever grateful to all of you who have contributed to Cloey's care in ways big and small.

1 comment:

hey, jode said...: Beautiful blog. Our prayers are with you and Cloey!; June 25, 2012 at 10:18 AM

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Some issues Cloey has faced

POTS
Von Willebrand
Ventilator dependant (12 hours)
bone anomalies
numerous infections
hypoglycemic, anemia, malnutrition
excema
at risk melonoma "abnormal birthmark removed"
intestinal deformity
tracheostomy
sleep apnea
sub mucus cleft pallette
esophegial reflux
VSD (hole in her heart) & arythmia
gross & fine motor skills delay
"lazy" eye/ glasses
spinal stenosis/fusion
bladder surgeries
sensory integration disorder
speech/motor planning delay
tonsils and adnoids removed
10 sets of ear tubes
mental retardation
siezures
Failure to thrive

The birth of a miracle

A Mother’s Story
A tiny purple foot. That’s all I could see. There was no, “it’s a girl,” or no “oohs and aahs.” I wasn’t holding her. The room was too quiet. No baby crying – just fast, technical talk between the doctors and nurses. This was the first time I had given birth, maybe this was normal. I just wanted to hold her, but all I could see was a tiny purple foot – shaking. I remember thinking that she must be cold.
Finally a nurse was bringing her over to me – wrapped in a blanket. I thought she was going to hand her to me, but instead she just said, “Kiss her forehead. She has to go upstairs.” I looked at her new daddy and said, “Go with her.” I think he was relieved not to have to make the decision between wife and daughter himself.
As he left the room I realized the prompting I had received months before to be sealed in the LDS temple (married not only for time, but also for eternity, so that we and our children can be together forever) was in preparation for this moment. I found comfort in knowing that if she didn’t make it, I would have the opportunity to raise her after this life.
Eventually, I was taken upstairs to see her. Her dad was with her. He put his arm around me and I reached into the clear little box and touched her hand. She was covered in tape and wires. Her eyes were shut and she wasn’t moving. Ten seconds passed – maybe – and a helicopter team came rushing in, with all the technical talk again. I asked what was going on. Someone held a paper in front of me with a diagram of a heart on it and started to explain what was wrong with hers. They said she was being taken to Phoenix Children’s Hospital.
I wasn’t listening. I just watched them as they lifted her tiny body into another box and took off running. Her protective daddy asked if he could go with her and they said no, he would have to follow by car. He kissed me and off he went, along with his dad, to be with our new baby girl.
At Phoenix Children’s Hospital Neonatal Intensive Care Unit (NICU), her concerned father was told Cloey had a coarctation of the aorta. She was on a breathing machine and was scheduled to have a surgery the next morning. The doctors showed him the x-ray that showed a hole in her heart. Acting on faith, Cloey was given a blessing by her dad and his grandfather. He had never given a blessing before, and had only been ordained an Elder (received the power and authority to give such a blessing) a few months before. I was not there, but was later told by a nurse it was a very powerful experience. She said several nurses paused during the blessing – unheard of in the NICU. The words of the blessing were more than an new dad with a heavy heart could have uttered on his own. They had to have come from a loving Father in Heaven who was mindful of the new challenges our family was facing.
Cloey was born on March 25, 2001 at 3:13 p.m. at Desert Samaritan Hospital in Mesa, Arizona. Within hours, both she and her daddy had left the hospital. Very early that next morning – before the sun came up, I called my husband. I was crying – lonely, and unsure of what was happening with my new baby – so he came back to be with me for a short time. By 10:30 a.m. on March 26, I had checked out of Desert Samaritan Hospital and was on my way to Phoenix Children’s Hospital to see Cloey. When I arrived, she was the biggest baby there and was right by the window. For that I was thankful.
Sometime that day, they ran another echocardiogram of her heart before taking her to surgery. The hole that was there the day before was gone. Cloey would not need the surgery! It was a real life miracle. The only thing that had been done to Cloey between the x-ray with the hole and the one without, was the blessing she had been gien. I knew then that the Lord truly does answer prayers, and that the power of the priesthood is real.
Cloey spent the next 14 days in the NICU. We were right by her side every day, and would stay until 1 or 2 a.m. We would go home briefly, eat and sleep, and return by 7 a.m. each morning. We read to her, played musical toys for her, talked to her and dressed her in her own clothes.
At one point, when they were trying to wean her off the breathing machine, she crashed. Alarms went off and they told us to step back. We thought we were going to lose her. Three days later when she was successfully breathing on her own, one of the doctors told us, “She is the master of her own destiny.”
Around this time, the geneticist pulled us into a room off of the NICU to give us the results of some blood tests they had taken. We thought this was just procedure. We later learned the blood tests were ordered because of her “dysmorphic features”.
The geneticist asked us to sit down. We held hands. He told us Cloey had an unbalanced translocation duplication 16q22 monosomy 14q32, to be exact. He wasn’t sure what to classify it as because he had never seen it before. But he was sure she would be severely physically and mentally delayed. He told us that it would be significant. She may never walk or talk or even know who we were. We both cried.
Until that moment we thought that this NICU stay would be our trial. As soon as she was better we would go home and this would all become a distant memory. We really had no idea something was “wrong” with her. All our hopes and dreams were gone for her at that second. Then he said, “You can take your baby home, and I will see you in six months.”
We went out into the hall and told my grandmother what the doctor had told us, and we all cried again. Then we went back into the NICU. As soon as I saw her again I stopped grieving for what she wasn’t and started loving what she was. She was so beautiful and was fighting so hard. She had no idea what we had just heard or that she had limits. She was just trying to be a baby. So we took her home and knew we would do all we could for her.