Best Mayo Trip so Far!

We will be coming home on Sat! It was a very productive and positive visit, confirming why we sacrifice and come here.  The newer doctors who haven't been around for her entire journey, remarked at how loved she is, and that some of the other doctors would relay her story in the care conferences with pure amazement and love.  I am beyond grateful for the care she has received here over the years. One particular doctor, who works in another specialty now, went out of her way to come see us.  I wish I had taken a picture of her with Cloey,  because she really went above and beyond when Cloey was struggling the most. Lainey was a newborn at the time and she would help me,  push the stroller, carry Cloey's machines and hold the baby all in addition to providing excellent care for Cloey. She treated Cloey like a person, like a whole person, The way medicine should be practiced.  I made sure to tell her how grateful I am, because we may never be back, and Cloey is still asking why I made Shelly cry and why I was crying! It was a very touching moment. We have literally been busy the whole two weeks, I brought things to do in case she had a major surgery or something and I had a lot of down time.  That was certainly not the case! I didn't do any of it, we didn't do any of Cloey's school work either, but we got all the important things done and then some.  Listing everything we did would be long and pretty boring, so instead I am just going to give you the exciting results of our marathon 2 weeks! Just know Cloey went through the ringer of tests, she was poked and proded every which way and didnt complain once! We were told the for some of the things they have had grown men cry, or big football players pass out.  Not my Cloey, She was Brave like Merida!

(Thanks Lobdells)

GI (gastrointestinal)- Cloey is doing great off of her G-tube! She had some irritation in her esophagus so she will be going back on Prilosec for reflux.  Things were also slow moving because of her past intestinal surgeries so she will be adding a daily stool softener, no biggie!

Pulmonary (lungs)- We were told previously at home that Cloey had a 30% lung function, and that this was typical of kids who were on breathing machines for long periods of time.  The pulmonologist here was not convinced because she does not have any kind of lung disease and can walk long distances on occasion.  He did some more accurate testing and biopsies and determined that while she does not have full function, it's not that low. Her unique anatomy makes it so she takes really shallow breaths, she cant really take a deep breath and fill her lungs like you and I do, but the actual function of her lungs is good!  This is great news and means she is not in danger if she pushes herself.  He gave us the go ahead to increase exercise, and that means fun too! He said she should not be written off as a "vent kid",  "She's Cloey, lets see what she can do" Seriously, this is why we come here.

ENT (ear, nose & throat)-Cloey had a scope done and the good news is after 10 sets of ear tubes her ears are doing great! The not so great news is her airway is still abnormal, and basically just too small.  Both the pulmonologist and and ENT said that if they saw her airway today,  knowing nothing of her history, they would say she needs a trach.  However, because they take the whole person and quality of life into consideration, they agree that doing that again would not be beneficial.  We will just have to be aware of it and work around it.  The other not great news is that her left vocal cord is completely paralyzed.  Which means we can stop trying to get her to improve her volume in speech therapy, its just not gonna happen! This is probably due to the injury on her spine either before or after her spinal surgery.  It also explains why its so difficult to hear her sometimes.  We may look into getting her some kind of microphone or something.

Urology (bladder)- This is probably the most exciting news, it appears her bladder is now functioning normally! It's just that it is a muscle that hasn't been used in 13 years so we will need to "train" it with timed voiding.  Cloey will have a watch that will alarm every 2 hours and she will have to "go" whether she needs to or not.  After about 6 months she should be able to be dry, at least in the day.  We are so excited for her! After 3 bladder surgeries, spinal cord damage and neurological deficits, this is really one thing we thought would never happen.  Not only does this increase her Independence, it also open up all kinds of new wardrobe possibilities!

Autonomic dysfuntion (system that controls automatic things like blood pressure and blinking)- While Cloey does not have full on POTS she does still have autonomic dysfuntion. This presents in things like her eyes dilating randomly, even when its bright outside, her blood pressure being low, not being able to store iron well, not breathing enough when shes sleeping, and a low heart rate.  There is no real treatment for this, it is just how her body works. Keeping her healthy, exercising and adding salt to her diet all help.

Genetics (chromosomes and genes)- There is still no reported cases of anyone like Cloey.  There is, however, more kids similar with one part or another of her disorder.  They are going to do more research and send me a letter if there is any new information discovered on the specific genes she is missing/has extras of.  They also said that Cloey is now the case used to care for other children that are similar, since she is the oldest living at 13.

Sleep medicine- This is the worst news we received, but still not terrible. Cloey still has severe sleep apnea (mostly because of her abnormal airway) and will need to be back on a breathing machine at night.  The tricky part is that they are not sure this will work, just hopeful.  We will stay in contact with them to make sure. The good side is that this should help her to not tire so easily and be so sleepy all the time.

Nutrition (diet)- Cloey's iron levels were so severely low that she had to get an iron infusion through an IV in the hospital.  We will have to check again in a few weeks and maybe receive another infusion while at home.  She will probably also have to take oral supplements.  A lot of factors contributed to this- her autonomic dysfunction, heavy periods, poor sleep and also not as much iron intake since stopping her g-tube feeds of fortified formula.  Cloey's diet will need to be monitored to be sure she is eating enough good foods and she will need to drink formula in the morning with her breakfast, something like ensure.

Orthopedics (bones)- All of Cloey's joints are abnormal, but not fixable and don't cause any major problems. That is all the same, not worse, which is good.  Her Spinal fusion is still solid (good) but her scoliosis has increased from very mild 10% to 20%, still mild but because of the increase we will need to repeat an x-ray in about 9 months and make sure it is not worse.  The standard practice is to wear a brace to correct it at 25%. One thing we have going for us is that it appears Cloey is just about done growing. Most of her growth plates are closed.  Scoliosis gets worse as you grow, so hopefully we've seen the worst of the curvature. We've always known Cloey would be smaller. There was a
question as to whether she had a form of dwarfism at one time, so we are very happy that she has gained enough height to see over counters and otherwise get around in the world fairly easily.  It's also easier to care for her if she's not taller then me so it all works out!

Cardiology- Cloey's heart is awesome! She has an irregular and slow heart rate, but that is not new.  The excitng news is that over a 48hr monitor her heart did great and responded to exercise. So this is not the cause of her fatigue and she is cleared, and encouraged, to increase her activity level.

Gynecology- Cloey has been having very heavy irregular periods.  We had an ultrasound at home that determined she had a double uterus.  It was recommended by her pediatrician that we have it removed and solve two birds with one stone.  However, it is not legal in AZ to sterilize the mentally challenged, even if its for medical reasons.  This is also an emotional decision for us, and we were not sure what to do.  We went to the Temple (a sacred place for me as a Latter Day Saint,  where I can meditate and pray) before I left and I felt comforted that, as always, I would know the best route for Cloey's care.  God may have given me a child with more challenges then most, but he has not left me alone in them.  So.......one of the first things we did when we got here was a pelvic ultrasound.   The Gynecologist was not sure of what she saw and wanted a clearer picture, so we had an MRI of her abdomen done.  The results were that she does not have a double uterus, its actually one uterus with a "divider" of fibrous tissue (kind of like scar tissue or cartilage).  This explains her heavy irregular periods, her uterus can't clamp down or shed the lining properly.  It also means eventually the tissue can be cauterized and her uterus can function normally.  For now she can be treated with hormones to suppress her periods.  I am not super happy about putting her on hormones, but I am happy with the thorough job mayo has done and that it will make her more comfortable and keep her iron levels up.  It also buys us time and means we can manage the rest at home.

More then ever before we are given the green light with Cloey. We may have to come back in 2 years, but only time will tell, and if we do it will be a maintenance trip.  She is dang near having a "normal" life and we couldn't be happier or more excited for her future. We also know this is largely due to all the support and prayers we have received on her behalf and we thank you from the bottom of our hearts!

Sorry Not Sorry

I was going to apologize for not posting for 2 years, but as I looked back at old posts, I realized how thankful I am that I haven't posted for so long. This Blog is meant to keep everyone informed when Cloey is receiving life saving medical care. By the Grace of God that has not been required for the last 2 years, and for that I am not sorry! We are, however, headed back to Mayo Clinic in Rochester MN on Monday.  The purpose of this trip is to evaluate Cloey's health and make sure we have a proper maintenance plan as she enters adulthood (eek!) Because her disorder has never been seen before, and the documented cases most similar to hers only lived until 7 years of age, Adolescence and adulthood is uncharted territory.  We will be seeing her team of about 15 specialists that will all work together to make a plan for her future.  For once, we are more excited then scared. I am planning on accomplishing whatever needs to be done to *possibly* make this the last time we will have to go to Mayo in MN before she turns 18 and is able to transfer care to the Mayo Clinic here in AZ.  This may not be possible, or we may need to do one more small trip in a couple years, but this IS the last of her donated funds and she is doing so well that we are sure going to try! It's been just over 10 years since our first trip there and as grateful as we are for all they have done, it would be so nice to put that chapter of our lives behind us.  I will be updating this blog during our stay, hopefully with all good news about our sweet little miracle girl!

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. 

Weeks 9-15

So Sorry It has been so long since I have updated on Cloey's adventures. She has remained relatively healthy. The only decline we have seen is a decrease in endurance, but thankfully it has been a slow decline and Cloey is still able to do most things.

The stress of it all has finally taken it's toll on our marriage. Ty and I our both overwhelmed and obviously coping in very different ways. We are in counseling and working on strengthening our marriage.

In the meantime, Cloey (and Cal) completed buddy bowling and received a trophy! She also enjoyed a week at Ryan House where she got to be nurse for a day and take care of patients (teddy bears). She was able to participate in Camp Zoo and feed the Giraffes, her absolute favorite a animal. And the biggest change of all has been going back to Brisas! because of all that is going on with us, we had to re-prioritize and decided to put the kids back in public school. When we told Cloey she asked if she could go back to Brisas, I wasn't sure it would be possible but the kind & caring principle over there made it happen (Thank You!)

Please continue to pray for our family, your prayers are felt and much needed.

Cloey Adventures weeks 6, 7 & 8

Cloey has been on some hard core antibiotics for a skin infection on her leg. It looks like it is working and the infection is clearing up. The problem is the antibiotics have caused a major bout of diarrhea & yeast. Nothing we haven't dealt with before, but this time it's different. Poor girl couldn't sit down even after I called and got her prescription diaper cream. Than we had to fight with her to clean her up, and watch her cry. Seriously? I thought we were doing "comfort care" everything is so much harder now that our goals have changed. Speaking of hard things, I read this article recently and It captures the feelings and emotions of our situation Dead On. While I don't know what it is like to lose a child, everything up to that point is exactly what it's like for us. It was so hard for us to read. For all of you who are asking how we are, this article will give you an idea. My prayers are with Sophie's family.

http://tucsoncitizen.com/arizona-news/2011/06/26/a-young-girls-battle-with-cancer/#.TiJq1Rr3sxw.facebook

We also moved into our new place and are just getting settled. Cloey loves it because it has stairs. We watched fireworks and lit some sparklers on the fourth, but didn't go to Tempe Town Lake like we had planned because we were in the middle of unpacking. Cloey also got to run and play at Makutu's Island, something else she has wanted to do for the last five years. It is awfully dangerous with her trach, but she really enjoyed her freedom and we loved seeing her so happy & proud of herself. That's what it's about right?