Miracle's do happen!

It has been so busy I can't possibly catch you up chronologically, but we are coming home! Here is the final verdict-

Cloey does not have, or did not have Chiari. She has some old brain damage to her brain stem from the congenital malformation of her skull and spine, but the damage is not signifigant to her airway issues and is rebuilding neuro pathways at this point.  This is HUGE because it means she is NOT on an inevitable decline to a pacemaker, colostomy bag, 24hr ventilator, etc.

The next big issue was her autonomic dysfunction.  The sleep disorder specialist/neurologist, autonomic dysfunction specialist (also a neurologist), the endocrinologist, the pulmonologist and the POTS specialist, worked hard together all week to sort out her symptoms.  I mean, besides visits, they had several meetings together.  Based on testing and a recent study out of Stanford University (the sleep doctor consulted his colleague over there) It was determined that the majority of her problems are a result of her spinal fusion. Cloey's entire cervical spine was fused at age 5 to prevent paralysis.  It was necessary and she would be paralyzed from the beck down if we hadn't done it.  When they fuse the neck they don't do it completely straight, they fuse it a little bit forward to make life skills such as eating and using a computer more possible, unfortunately this study out of Stanford has shown that in some cases (particularly where the patients anatomy is narrow, like Cloey's) This increases pressure on the airway, and putting a trach in and using a vent, take away even more of the space making things worse.  Let me clairify, even though we didn't know this when the Trach was placed or when we placed her on the vent, it wouldn't have mattered. She needed those things because she was not able to breath adequately on her own, and as it turned out, They bought her 6 years to grow and open up more space in her airway.  Her sleep study from 6 years ago before trach and vent showed 70 apneas in an hour. This week, with the trach capped off and no vent or oxygen itshowed 7. Miracle. The patients in this study behave like quadrapaligics, meaning while they may have avoided complete paralysis, some side effects are still evident.  For Cloey, the fusion combined with her genetically small airway are causing her lack of bowel and bladder control, her weak intestines, her central apnea (brain not telling you to breath) and her irregular heart rate.  The good thing is this will stay the same or get better as she grows.  It is also the missing piece of the puzzle as to why she got BETTER on hospice without the vent.  It also explains some of her symptoms that they thought might be POTS.  The conclusion is that she does have some issues with her autonomic system, specifically orthostatic tachycardia (basically low blood pressure) but does not have POTS.  We were given recommendations to add water and salt to her diet, and also begin an exercise routine.  Again, these symptoms are also expected to stay the same or get better as she grows.

Than was ENT, we had a scope done on Wed, and everything looked good, not great but good enough to remove her Trach! It was done in the hospital and she stayed overnight in the PICU for observation and did great.  The plan is to use prilosec, flonase and singular to protect her airway from irritation and inflammation, also to watch her weight diligently because she can not afford anything else to cause airway obstruction.  Then we can use oxygen as needed.  There is a chance this won't work and she will have to be re-trached.  We won't really know for six months or so and will be doing scopes and sleep studies at home to monitor her during this time.  If it doesn't work, we may be looking at a trip back up here in a few months to re-evaluate.  But, she is doing really well and we are all hopeful this will "stick"

We also completely redid her diet for adequate adolescent nutrition, and only 1/2 time tube feedings! There is whisperings that the reflux medication and removing her trach could increase her appetite too, meaning a possibility of no g-tube in the future. Guess we are so used to tings snowballing in the negative direction it is hard to comprehend that they can snowball the other way too :)

Endocrine evaluated her as well, and ran some precautionary tests that we will get, and could be managed at home.  She mostly looked and things to rule anything else out and agreed with the yeam on the source of her symptoms.

I think that is everything...

All of this is an absolute miracle, one of her Doctor's said "No one expected her to be 11 and doing so well" I am sure this is the direct result of her super awesome medical team, hard work, service, sacrifice and prayer that she has received.  We could not be more full of Gratitude.  I was literally jumping and clapping my hands when we knew she would have her trach out. I can't wait to see her quality of life and independance blossom.  My brain looks at her and can't believe this is possible, but when my heart looks at her I see that baby in the NICU and say "I knew it"

Wow! Mayo has done it again

 Monday we were at the clinic all day, from 7am-5pm.  We saw the main doctor coordinating our care and spent about 2hrs with her.  We went over all of her symptoms and problems she is having. She agreed that Cloey has some sort of autonomic disorder that is causing her problems and set us up to test for one specifically called POTS that day.  The test was about an hour long and basically tested her nervous system and autonomic responses.  We discussed have a bronchoscopy on Wed to see what her airway looks like now that she hasn't been on ventilator support for a year.  We than had x-rays done of basically her whole skeletal system and saw the orthopedic surgeon who has been following her since she was 2.  For the first time he said her bone structure was "essentially normal", those two words never describe her so they were like music to our ears.  He said her spinal fusion looks great and her abnormalities look better than he expected them to look at this age. yay! Recommendations are to just to continue to monitor her about every 2 years, but to expect only minor fixable problems. double yay! This was one of our worries about her getting older, having to repeat the fusion or wear a brace or surgery on her elbows and hips.  So to hear that we are nearly out of the woods with that one is great.  We also saw pediatric surgery who is going to resize and replace her g-tube on wed while she is having the bronch done.

Today we saw the pulmonologist.  He had the test results from the test the day before and let us know that it did show she has autonomic dysfunction, it may be POTS or something similar.  We have not reviewed the results with the main doctor yet, so I won't go into specifics because we simply don't have them, but it means we have a disorder we can manage instead of putting her to sleep every night and wondering if she is going to wake up the next morning.  He went on to examine her and determined that from his stand point, she does not need the trach or the vent.  Whatever was causing her to need it before she has outgrown.  Probably fusion done 6 years ago has relieved the pressure on her brain and the damage from the "almost" Chiari, has repaired itself.  The neurons have had time to rebuild pathways.  Also, her facial structure that was preventing her airway has grown and opened up, allowing her to breath normally.  He shared his opinion with the sleep specialist who is also a neurologist and recommended a sleep study to determine if this is actually what is happening.  We see him in the morning.  He also is going to run a blood test to test for a specific gene for central hypoventalation syndrome (brain forgetting to breath) if she doesn't have it that is more evidence that she no longer needs the vent.  This is why we come here! more than an hour with each doc. Doc's working together and thinking outside of the box, or even just outside of their specialty. Combining studies and tests so she doesn't have to be under anesthesia more than once.  And most importantly, seeing her quality of life as a person and not just "one of these kids" and a chart to pass on. 

Than we saw the ENT.  She agreed with pulmonary, and from her stand point if the scope tomorrow and the sleep study come back clear.  SHE WILL TAKE THE TRACH OUT FRIDAY!!!! We were in complete shock, Ty literally asked her to clarify three times.  Decanulating (removing the trach) is usually a big deal involving reconstructive surgery, but in Cloey's case it wasn't placed because of her airway, it was placed because of abnormalities in her facial structure and brain, so her airy was is "essentially normal" meaning the hole will close on it's own, or at least mostly and may need only a minor surgery if it is not completely closed in 6 weeks. When it was placed we were told to consider it permanent, even if she got off the vent the chances of her facial structure and brain issues resolving were slim to none.  

Our heads have been spinning the rest of the day.  This changes everything! A year ago at this time we were preparing to say goodbye to her.  Maybe all the fighting for her for 10 years, giving her everything she needs no matter the cost, is finally going to pay off.  We are positively giddy at the idea of possibly seeing Cloey blossom without a trach or vent.  We also see what a miracle she continues to be, and the power of prayer.  eeeek!

Ok, back down to reality. We can't let our hopes get too high.  She still has to "pass" the sleep study and scope, and we don't know all that her autonomic disorder entails, but it's still exciting.

Ronald McDonald House was full, so we are on the wait list and are staying at a hotel at a discounted rate.  Everything else has gone amazingly smooth.  Of course, having both of us here helps a lot.  I am so grateful to everyone who has helped us financially and logistically so that we both can be here to share the burden and so we can make what has turned out to be some pretty BIG decisions as a team. It has really helped.

Tomorrow we will know lots more.....

Here is a picture of Cloey standing in front of a mirror that hangs in radiology.  when we first saw this mirror 9 years ago, it reminded us of her and we both got misty.  Ty says he is going to make one for her someday.  She has looked at her reflection in it every time we have come here since.

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.