Eye report

                

            

I got to cuddle this little bug this morning as we waited to meet all the Dr.'s for surgery. It always kills me to send her back with the nurse just as she's so happy and doing well. I just want to run away, and say "we changed our mind! She's too happy right now." But I know I have to do all of this while she's well. We are still sitting and waiting for her cleft surgery, but the eye Dr. Has come and gone. He said her right eye looks good. There is a little cloudy tissue covering part of the pupil but they can take that out and her vision with glasses will be about normal. Hearing that was like angels singing. To know that she'll have great vision in that eye after wondering for so long is HUGE. The left eye, however, he said was a different story. He still needs to go in to try to clear some cloudiness, but it's more to preserve her peripheral vision, so that she doesn't go blind completely in that eye. A little harder to choke down. We don't know how much she can see and my concern has grown in the last few months. Sometimes it's like Ruby can't see me at all and looks past me. Sometimes I KNOW she can see me. It's something that I really haven't wanted to talk about because I didn't want it to be true. It's a scary thought to think that your baby looks right through you while you are holding her and trying to get her to laugh. So to know one eye is going to be great is a huge blessing. I still think there is lots of hope for her left eye and vision. I am not going to give up on that. 

I am glad I pushed for this exam. I have felt with all the glaucoma pressure concern that her overall vision was getting pushed to the way side. I do wish I would have pushed earlier, but I can't live thinking it would have changed things. I have to trust Heavenly Father and know that he KNOWS Ruby and is aware of her and her vision. He's going to make this ok and Ruby will do the best she can in life. She's proven that already.

1 week until surgery

We have exactly one week until Ruby's big surgery that we have planned on for over a year.  I am nervous.  I shouldn't be.  I know exactly what they are doing, but sending my daughter in to be put to sleep for 4 hours is nerve wracking.  We have lots backing us.  An awesome Dr. that we have complete confidence in.  And I got to request our anesthesiologist.  A friend of a good friend. He happens to also be a cardio anesthesiologist, which Ruby needs after the pulmonary hypertension scare when we were admitted in April.  This anesthesiologist wasn't scheduled that day, but didn't hesitate in the slightest when I asked him if he'd do it.  He said, "if it were my daughter, I'd not want them to say no."  So nice.  Both Dr.'s are LDS and that means a lot to us too.  Knowing they have the priesthood and they are probably going into the surgery with prayer just like we are.  I appreciate that.  The more attention I can get from Heavenly Father, the better!   Our faith is our life and I appreciate when it intersects with the things that have to be done.  I have talked a lot about tender mercies, and that is another tender mercy.  The Lord knows I need lots of them.

We have had a lot of decisions to make regarding this surgery.  Whether to do an endoscopic surgery again (for the 3rd time) or do an open surgery, which will also enable the Dr. to take cartilage from her rib to build up the cleft and give it more support.  He couldn't do that by going through her mouth. Also, whether to ventilate her for a couple of weeks or to give her a trache.  I have cried and cried and prayed and prayed about this.  I cry again as I write this.  I DO NOT want this.  The last thing I would have expected was that my daughter would get a trache.  I remember someone telling me at church right after she was born that their friend had to get a trache and you can't even tell now.  I wondered why she was telling me that and said, "oh, she'll never have to get a trache."  Never say never.  We know it's the right thing to do in the long run and ventilating her would cause irritation on the sutures, possible causing them to come undone, let alone the weaning off the drugs she'd be on to keep her sedated.  Oh, that was terrible in April.  I DO NOT want to go through that again. One Dr. also reminded me that this IS NOT a surgery for a trache.  This is a surgery for her cleft and the trache simply keeps the airway open for a few months until the swelling goes down and her throat can heal.  That statement helped a lot.  We can do anything for a temporary time.  I keep telling myself that.
 She now has a level 3 cleft as opposed to what we thought was a level 2. This is the reason for the cartilage being built up. He said it was a higher level 3, but it still breaks all the way through, like the picture shows.  So grateful it's not a level 4.  I wish this surgery was a surgery that we just go in and "fix it" and it was successful.  It's may not be.  So many times it is months and months before seeing any success rate.  And I have inquired about a few level 3's on a FB page I am on and a lot of times the surgeries are getting them to a level 2 and then they may need to go in again to have it fixed again.  I am hopeful though, that as this being an open surgery, there is a higher chance of that not having to happen.

                             Levels of Laryngeal Clefts


We have had a few appts. before surgery, a couple with GI, to check on Ruby's nutrition.  She has done well and is on the lower end of what the Dr. wants her to be gaining each day.  But she's gaining and that's huge and the Dr. is happy with that.  Ruby is so amazing.  She is the HAPPIEST baby.  She has such a sweet, tender nature and doesn't cry often.  I always say, I feel so bad that she has to go through this because she is so sweet.  But she is probably so sweet because she goes through this!  Everyday stuff is miniscule compared to what she has to go through in the hospital.  Why cry about it?  She teaches us so much. 

We also just met with the eye Dr. I swear the eyes are such a slow complicated process.  I get so frustrated that we can't just get her the glasses I know she needs.  I know she doesn't have great vision.  The left eye is still cloudy.  The Dr. asked if it was getting any better, and I said, "I really, really want to say yes".  But my heart knows it's not and it's hard to say out loud.  I know it can, but it's not yet.  He tried to get a prescription for me and I was so hopeful, but he said he couldn't see into the back of the right eye (THE GOOD EYE!).  There is something blocking it, whether it's scar tissue or (he didn't give me any other options).  I don't know what that means.  They aren't good at explaining future.  So they have to put her under and look that way.  I started crying.  I don't want to schedule her AGAIN to go under anesthesia to get it checked.  Then if there is something, they don't touch it and schedule AGAIN to do the work.  Did I say slow process?  I meant SLOW.  It's like anesthesia left and right for them and no big deal.  It drives me nuts. 
After I told him when her surgery was, to see if they could do it then, he told me he was scheduled for surgery at another facility.  I asked if any of his partners would be at PCH and if they could look since they weren't doing anything.  Our first eye Dr. we found out would be there.  I got a call from his assistant today saying he would do it!  YAHOO!  I said I was so thankful, and she informed me that she was surprised.  He NEVER says yes to doing things like that, but when she went in and asked him, he didn't skip a beat when he answered that he would.  I said, THANK YOU, THANK YOU, THANK YOU!!  I thought my prayer wasn't answered when we couldn't get that prescription in the office yesterday, but it was.  And obviously there is a benefit from seeing what is going on in Ruby's eyes in more depth.

Back to surgery.  Because that is mostly what I think about right now.  I told Nate they should put a movie theater in the hospital.  He said I wouldn't be able to concentrate on the movie anyway.  He's right, but it would be nice to atleast try. What do you do for 4 hours when you can't concentrate on anything else?  I guess I'll let you know in a week. 

Meanwhile, Ruby is doing GREAT!  She is sitting up better...unassisted for a few seconds until she timbers over.  She's still on 1/2 l of O2 at night and the vest therapy 2ce a day.  I can really see a difference in those adding to her growth.  I don't know how that will all change after surgery, but I am sure it will!  Every time we go in, we are released with a "new" normal! 

Update

It looks like we'll be getting out of the hospital soon...it's looking like tomorrow (fingers crossed).  Ruby has held her o2 level and progressed so amazingly well this last week. I have had a few nurses/residents come check on her and they are all so amazed at her progression. Such a blessing. I can't express how grateful we are for the prayers and well wishes. We know they make a difference! I know I say it over and over again, but it's true. 

                       

The concern about her heart is gone. Amazing. We did an echo the day after Dr. Rao came and talked to us, he came back and said her echo showed no sign of pulmonary hypertension! I was so happy. I spent the night before devastated because I thought her heart was now going to be such an issue and affect so many things and I am so grateful I don't have to worry about it at this point. 

She got her GJ tube replaced twice since it was first put in last Friday. We had such a nightmare with two of them that we had to go up a size on the 3rd go round and it's worked. It does leak a bit when both the g tube and the j tube are clamped, but nothing compared to the green bilious that was pouring out before. 

Ruby was also projectile vomiting this green bilious. It was SLIGHTLY alarming. It turns out that her body produces too much right now and doesn't seem to empty completely. So while her tummy was filling with food (when she was g tube fed) it was formula coming up, but now that her food is going into the small intestine, it's filling with just bilious, which is still upsetting to her system. Therefore, she'd have massive spit ups. I was so paranoid that she'd aspirated and we'd get bacterial pneumonia again before even getting out of the hospital. 

We started to "vent" her tube or drain it. So she is being fed with the j tube, the g tube, from the same port is hooked up to a different tube that drains into a diaper that is tightly wrapped around it. It's a little crazy how much drainage is in there, that has nothing to do with the food she's getting. She seems to be putting out too much, therefore her weight and nutrition is a concern.  The other night her heart rate went up quite a bit (170's. She's normally around 115) and she got a slight fever. I panicked. I thought," please don't let this whole process start over again." That was why we went to the hospital before...because of her high heart rate (200's) and her little body gave out. I had horrible flashbacks and couldn't get take my eyes off the heart rate monitor. Turns out she was dehydrated because she's draining too much from her tummy.  They don't know why this happens but give the answer that every child has a different reaction to the GJ tube. So they gave her IV fluids to conpensate, which immediately brought her fever down and her heart rate down. They also discovered that if they compensate her nutrition by adding pediatyle in the amount she outputs from her tummy, she'll stay hydrated without relying on IV's of fluids. Which means we can do all of this at home. Not that I want to.  The thought of having a drainage tube hanging from her all the time, is less than ideal.  I had a whole pity party last night and was bawling that I can't possibly take on any more when I get home. But just like everything else...it's learning step by step. It's not going to be easy to take her anywhere with a feeding pump and a drainage tube and 3 other kids, one of which doesn't want to hold your hand in the street or parking lot because he thinks he is big. But I can do hard things and I can figure it out...maybe crying intermittently, but I can do it. 

I watched this talk and loved it, but honestly thought, "why the heck does my burden have to be SO heavy?" I know, lots of people have it harder. But I sometimes don't think I can possibly handle any more.

https://www.lds.org/general-conference/2014/04/bear-up-their-burdens-with-ease?lang=eng#watch=video

But as my Dad reminded me...3 weeks ago I was pleading for her life. Begging and pleading. Prayers have been answered and now she's back and happy. I am thankful she's back. I can and will do anything for my dear Ruby girl...even if it's hard and I am feeling weak. 

                               

                               

                               

                               

Easter Sunday

We are still here in the hospital, and although it was Easter, it really doesn't feel like it is when your family is split in different directions. We have been blessed with so many prayers, by those we know ans those we don't know. Ruby has progressed so much in the last two weeks! She is off her oxygen now, and IR put in her GJ tube, which I honestly haven't fallen in love with. It's bulkier, she's got major gas and she hasn't stopped spitting up (which is the entire reason we did it) and it's leaking like crazy. She's happy regardless, but tomorrow we have to send her to IR again to have it replaced because it looks like there is a small leak in the balloon somewhere. And we have to talk to GI to figure out the spit up. So until those are figured out we probably won't leave the ICU.

We spent her 1st birthday at her bedside! She couldn't leave, so we brought the party to her! We ordered cupcakes for the nurses and brought in balloons and presents! She wasn't super happy that day, unfortunately, so smiles were scarce, but she's so content, and such a love.

                     

There is still some concern about her heart. The dr.'s called in a specialist from cardiology on pulmonary hypertension. I made the mistake of googling that. It's a bit scary. Dr. Rao (our pulmonologists husband) is so nice and came to chat with us for a while about it. He said IF she has it, it's type 3, which is temporary, which means it's going on while she is sick. He said the ASD she has is working to her benefit because it's pushing more blood through. Pulmonary hypertension prevents the right side of the heart from working well. It narrows the arteries in the lungs causing high blood pressure and less blood flow. It's not great. He said he wants to do another echocardiogram while we are here and then he wants us to get one in Prescott when she is well and because of the elevation change. He then will see exactly what's going on and determine if we need to be part of his pulmonary hypertension clinic. As much as I liked him, we are hoping for a big fat NO on that. 

He said either way, we are for sure to use only cardiac anesthesiologists to prevent any issues. It also looks like our cleft repair won't be happening for another 8-12 weeks because of her o2 issues. Which I am fine with. I don't want to take any chances either. 

It's so hard when things start to get better, having more problems in areas you didn't think were a problem, or they haven't been in the past even though the possibility could be there. I really just want her little body healed and for all of the parts to work together for good vs. bad. I look at my other kids and think of really how amazing our bodies are, for everything to work together so easily and to have never had to deal with any major health issues. 

I was reading a book by Maya Angelou, a friend brought me and this little poem hit a tender spot in my heart for my sweet Ruby:

The free bird leaps
on the back of the wind
and floats downstream
till the current ends
and dips his wings
in the orange sun rays
and dares to claim the sky.

I just want my baby to be the free bird we all are. I want her to run and play and jump and see everything she can see and do everything she can dream of doing. I want her to go to the movies with friends and eat popcorn out of her mouth and hike a mountain with a healthy pair of lungs.  Things that intake advantage of.  I want her to love life and understand heartbreak and joy. 

I know regardless, she'll love life. She already does. She'll teach the world of happiness and joy whatever her circumstances and she'll be loved by everyone she comes in contact with. 

Who can't love this beautiful, happy face?!?

                                 

                                 

Pneumonia and the rhinovirus

I was so happy that we had gone MONTHS without being sick in the hospital.  Last July, really.  Ruby has gotten sick, but we've been able to battle it at home since we have oxygen and an oximeter which monitors her oxygen and heart rate.
She even got pneumonia in February that we battled out over a week (which was fast for us!)

This last Sunday it all changed.  Ruby started to get grumpy Saturday night when I went to bathe her, which is unusual.  She is the happiest baby, so I knew something was coming.  Later she got a fever which usually always results in her having to have O2.  We ended up putting her on O2 and trying to get her to sleep.  She wouldn't stop screaming.  I knew it was pneumonia.  It is painful and she screams like that for hours vs. just weak and wanting to sleep like when she has a virus.  We tried all night to get her comfortable, but her heart rate wouldn't drop below 200 (normal is 120's), this was the kicker.  We knew we needed to get her into the hospital.  We took her to Prescott Valley (they treat more kids there), with the intent for them to get us to Phoenix Children's.  We went by ambulance, but looking back should have gone by helicopter.

We were met by my parents and brothers who stepped in to give Ruby and me a blessing.  So grateful they were willing to come down and do that for us.  Nate was with the kids in Prescott still packing them up to spend the weekend down here.

Shortly after getting there, her little body was giving out and couldn't take it anymore.  They whisked her off to hook her up to the ventilator to do all the breathing for her.  I was frantic.  Nate was driving the other kids down and hadn't gotten there yet.  Thank goodness my brother, Nick was still there so I could send him in to make sure she was ok, because I can't watch.  The hardest thing in the world is to watch your little lifeless babe in someone elses hands.  They saved her life.

We were moved into a room at the PICU and we were both so tired.  I thought for sure we would have a quiet night.  She was sedated and can't do much.  I woke up at 2 am to nurses and dr.'s surrounding her bedside.  I asked what was going on and her little body was giving out again.  She couldn't even handle the ventilator.  I had no idea there was another option, really.  They moved her to an oscillator, which not only breathes for you but pushes about 360 breaths/minute (they can adjust this #) into her lungs to keep the open so they don't have a chance of closing and giving up.

I again, couldn't watch all that was going on.  They had to do so much more to her to get this to work including put a catheter in her and other lines. This is much more complicated than a ventilator and there are so many more numbers to watch.

They escorted me out to the waiting room and I decided to read my scriptures to get my mind off of it.  I read Mosiah 3 and the two verses 4 and 5 were just what I needed to read at that moment.

 4 For the Lord hath heard thy prayers, and hath judged of thy^arighteousness, and hath sent me to declare unto thee that thou mayest rejoice; and that thou mayest declare unto thy people, that they may also be filled with joy.

 5 For behold, the time cometh, and is not far distant, that with power, the ^aLord ^bOmnipotent who ^creigneth, who was, and is from all ^deternity to all eternity, shall come down from heaven among the children of men, and shall dwell in a ^etabernacle of clay, and shall go forth amongst men, working mighty ^fmiracles, such as healing the sick, raising the dead, causing the lame to walk, the ^gblind to receive their sight, and the deaf to hear, and curing all manner of diseases.

It took so long...that I also watched this talk by Dieter F. Uchtdorf:

https://www.lds.org/general-conference/2014/04/grateful-in-any-circumstances?lang=eng

Both of these things could not be by chance.  I believe in tender mercies.  I had no idea what talk would come up from conference and this is just what I needed.  I came back into the room SO grateful for the dr.'s and nurses that were able to be there at the right moment to save my baby's life.  I was so grateful for the medical technology available to keep her here with us.  I have since looked at this stay at the hospital so differently and once again have had my testimony strengthened that the Lord does really know each of us individually.

Back to the oscillator:
Ruby also had to be paralyzed AND sedated on this machine.  She could not wake up...if she did, she would try to breathe and fight with the machine which could have shut it off because it wouldn't work right. That was scary to watch.  As much as I wanted her awake with me, I want her comfortable and she was anything but. Her coloring was also so pale. It was really hard to look at her without bawling my eyes out.

That morning the Dr. also came in saying she really needed a blood transfusion.  I was already scared, but this put me over the edge.  She said I had two hours to decide and if we decided against it, she wasn't really going to progress.  Wow.  So I guess it wasn't really a decision when it was put that way.  Scary as it was signing the form warning you against hepatitis and AIDS.  I immediately thought of that talk and thought..."why would I not do what it takes to save my baby's life?"  I take those chances or she goes downhill.  I am grateful for the blood transfusion, it really helped.  The dr.'s could see a difference immediately.  It's just scary as a mom, not living in the medical world having to make immediate decisions.  I am a researcher and I want to know all of my options.  But when you don't have any...you just pray! Thank goodness after the blood transfusion she really got her color back.  She has been really puffy and retaining fluid. It looks so painful in her legs and feet. I just try to rub them thinking that's what I would want.

I really can't believe how many IV lines she had running to her. There is a tower to hold 8 different meds or vitamins or whatevers and she was using all 8 in the beginning. Now she's down to 4. 

These are the machines she is hooked up to after the oscillator left the room. That machine was so loud. It was like having a big air conditioner in the room that was blowing to a beat of 360 breaths/min.
She was on the oscillator for 4 days.  It's a tricky machine.  There are so many different things to watch for and adjust...it's WAY over my "NICU experience" knowledge.  She just switched back to the ventilator...so we are making progress!  It is so amazing to see her little eyes open.  It has been a long week without seeing her big eyes and little legs kicking. 

You can see her eyes open for the first time after the paralysis med stopped. So sweet and so puffy. She even tried to smile, Little Love.

Here, the next morning her eyes are SO MUCH LESS PUFFY!!

She started respiratory treatment with "the vest" (I tried to do a link, but my computer was acting funny on that website).  Basically its a lung shaker.  It shakes the gunk out of the lungs and then they suction it all out.  Crazy as it sounds it totally relaxes her and she sleeps during it. 

She is also on antibiotics, albuterol and a salt water inhalent to moisten the secretions in the lungs.

Little Love loves her tummy!! But tried to turn her head with that intubation tube in her mouth and that CANT happen. Poor thing. I'd want to turn my head too!

While she has been making progress, her lungs are still really, really infected.  She gets an X-ray every morning and yesterday's was actually more clear than today's.  There isn't really a clear answer on that at all except that she was on the oscillator yesterday, so maybe it had to do with the machine keeping her lungs open.

It looks like we have a bit of a road in front of us.  A week or so on the ventilator, then nasal cannula, then she'll move from the ICU to the floor and be monitored there.  It's tough trying to be a mom to all 4 kids, when the 3 year old doesn't understand, the 5 year old is in 1/2 day kindergarten and the 7 year old and dad are up in Prescott.  It's not easy living far away when something like this happens.  I am grateful for so much help here in the valley with family and friends, but all the factors make life a little complicated.

Ruby had another eye surgery last Thursday to trim the shunt, which was put in to drain the pressure. Glaucoma makes they eye bigger, so once the pressure drains it shrunk back down causing the shunt to be too long, irritating the cornea. I don't know if this helped cause the cloudiness to the cornea, but I can tell you that I remember watching the change. It was right after the first cataract surgery. The left eye was worse than the right and I remember talking to my brother on the phone and saying, "it's like I can't even see a pupil anymore." Weirdest and scariest thing to see knowing something's not right. That's when we found out about the glaucoma. 

Surgery Thursday went really well. Ruby bounced back and she's already opening her eye more...as if it was uncomfortable because the shunt was irritating. Poor thing. It's so hard to find things out that are going on and only to realize that, "well, duh, that's why she wasn't opening her eye very much!"

We are on two drops (4x/day) right now and have seen the dr. twice since surgery. He said everything looks good and he wanted to see us in two weeks. I asked him what we were looking at further out. I hate the wrap up with nothing for me to anticipate. I need time frames. He then said he wants to get an ultrasound (no anesthesia required) done from a specialist that does that right in his office. He wants to make sure there is no odd shaping going on in the back of the eye. Then we'll follow up in 4-6 weeks and if there is no clearing of the cornea go to a cornea specialist to talk about a cornea transplant. (Insert where I ask for faith and fasting and prayers) We have 4-6 weeks and I know anything can happen in such a short time frame. I do know she can see out of that eye. When we patched it, it's very obvious.  Thank heavens for tender mercies. Just the fact that I know she can see out if it, even if I don't know how much. And the fact that the low pressure and the shortened shunt make it totally possible to clear up the cornea. It's almost like her vision , even with the cloudy cornea has gotten better, though. She is the smiliest thing and I know it's because she can see who and what is going on more!  I am hopeful. I am scared if I go back and have him say the words referring us away, but it'll all be ok. And I have comfort in me knowing that.

Life is good.

I am constantly tripping over these two step stools and out if frustration putting them back into the rooms they belong in. It occurred to me today as I stubbed my left pinky toe, that I don't want it any other way.  These two step stools are pulled into the room at any given point  by one or two of three kids who just want to see and play with Ruby. It doesn't matter if she's sleeping or awake, this little household celebrity gets midget visitors all day long. As soon as she wakes up, I hear, "yay, Ruby!" and "can I hold her?" She's perfection in their eyes.

 I realized that although I will do whatever it takes to make her life easier, her life is perfect. She's my beautiful baby and she's perfect. I am constantly praying for her and for Heavenly Father to help heal her ailments, and know he can, but if it isn't supposed to happen in this life, that's ok. I wasn't ok with that before. While I can't wait for her to have perfect eyesight, I am in love with her little pirate eye and the way she has to turn extra hard while on her stomach to appease her curiosity of what's going on.

And while she has low upper tone and may not sit up just yet, I get to have a cuddly baby that snuggles into the crook of my neck for just a bit longer this time until she's ready to move around on her own. And as my last baby, I relish those cuddles as much as I want her to get strong and be more independent. I love her so much it hurts. And now that she has tubes in her ears, the instant she can hear you coming, HUGE smiles for days. I am in love with this smiley baby and want her to know, as hard as life has been, I wouldn't have it any other way and I know she doesn't know any different.

 She's made our family better. Our kids are different. I have seen a love and tenderness come out of each one of them, that I have never witnessed before Ruby. I am different. I have a deeper understanding of life and the joy it brings. Nathan has had a better understanding of all of this from day one. He has been my rock and I am starting to see his positive perspective. Its been so hard, but I am happy, and I have my baby that I get to cuddle, and she's perfect and I love her, no matter what.