Friday, October 24, 2014

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!

1 comment:

Jamie Ollarsaba said...

I LOVEEEEEE this update Meg. I have "watched" (and recently been apart of) your journey with Cloey since her birth. As a mother whom faced some tough diagnosis for her first two daughters that eventually passed, I COMMEND YOU for all you do! Advocacy is so important but many times people rather "listen" to the professionals instead of thinking about the quality of life one has.

Cloey was gifted with an angel of her own, her mother! Congrats to you and your family.. Miracles do happen!

We love sweet Cloey!

Jamie