Hello Oxygen

One look around my living room right now you can tell it is different. There is a big noisy thing in the corner and tubing running around. Yesterday Jilli had her feeding pump sitting and between the wires and tubes in my house I felt like I was in the spider web room at Leaps and Bounds (props to you if you just got my reference)

Tuesday mid day I got a call from Jillian's pulmonologist about the 24hr o2 test. She said that there were times that while Jillian was playing her numbers would drop lower then they should. She said there were times that she did not think the machine was picking it up correctly but needless to say it still happened enough that we need to do something (as the person watching the numbers and seeing what Jillian was doing there was a direct correlation to o2 numbers dropping and Jillian playing) She said that Jillian needs to be on oxygen while she is moving around. She also asked about what times Jillian was sleeping and said that she did not love some of her numbers then so she is sending Jillian for a sleep study as well.

 Wednesday morning I got a call from the pulmonology nurse with more of the details of the plan. I also got a call from the sleep lab and we scheduled her sleep study for the end of May (our May is already looking crazy and it has not started yet!)

In the afternoon I got a call from VNA saying they got the order and someone would call me to let me know they were on their way. A few hours later a man showed up at our house with all of the stuff. Jillian kept calling him "not daddy" every time she looked at him. He brought us one large oxygen concentrator, 4 small tanks and one large back up emergency tank. The back up emergency tank was broken when it got here so we are waiting for someone to bring a new one and the rest more little tanks for next week. He showed me how to set everything up (this part feels like a blur to me) and was on his way.
I then had the task of getting the oxygen cannula on my two year old. I put it and the tender grips (adhesive pads that hold the oxygen tubing to her face) on her. She looked at me like I had forsaken her and went on to scream for a while. We Facetimed with my mom and Jilli started to calm. Then we Facetimed with Mikaley and Jillian got to see her dogs (Jilli thinks one of their dogs is a kitty dog, lol). That was probably the closest (seeing it on iPad) Jillian has ever come to playing with a dog.
It took several hours to convince her to walk and play. She just wanted to sit next to me at first which I totally understand.
My mom came out and brought me dinner. I was so grateful! Brent has been out of town for work this week so it was nice to have a second set of hands to help me for a little bit (Brent flew back in this afternoon)

Now it is just figuring everything out... where to store the portable oxygen tanks, how to best tape it to her face (they gave us 4 sets of stickers to last the month... ie  8 stickers), how to use everything, remember all the steps the guy told me... ect

Today she went to day care. Since she only needs it when she is up and moving I did not put it on her until we were there. She pushed the portable tank (too heavy for her to carry) in a Little Tikes shopping cart which seamed to work well. Hopefully it continues to work. We are also trying to figure out all of the daycare logistics of her being on oxygen. I work in her daycare (Elkhorn has all of its 4K classrooms in daycares) and they have been great with all of Jillian's medical stuff but this is something new for all of us so we are trying to figure it out (Jillian is the first kiddo with this level of medical need that has attended this daycare).

Today she is doing a lot better with it. I am so proud of her. I know she does not love it but she is leaving it on her face which amazes me. I think she was equally mad at me today when she figured out Walgreen's forgot to flavor her mouth med and mommy did not realize it before giving it to her orally. The look I got for that one...

I want to say a HUGE shout out to the people who have gone out of their way to help us with this transition to oxygen. It is what she needs and is what is best for her and I am SO grateful that she is getting what she needs but it is still hard. There have been many emotions during this process and new things to learn, and I am grateful for the people who have stepped in and loved on us. I am grateful for the people who helped keep Jillian entertained on Facetime yesterday to help her with this transition. Thank you to the friends who have sent me text messages or facebook messages, they each touched my heart and made me smile. Each person who has rallied around us either physically or emotionally or spiritually has truly helped in this transition and I thank you!

It will probably be a little bit of a transition period of us getting our wings to do things outside of the house. The guy set up the one portable tank for me yesterday but after her using it at daycare today it is almost used meaning I need to set up the next tank myself (we don't have school tomorrow so we are hanging out at home for the day) With time we will become pros at the oxygen too just like her feeding tube, but for right now it is still new. We will probably take things slow for a bit her until we build confidence at how this all works and what we need to do. I was laughing in the car today as I drove home from work trying to figure out some logistics out and thinking about how most parents pack their kid a lunch to go to daycare... I pack mine medical amino acid formula and oxygen. I ask for patience as we figure this all out and understanding that we probably will be home a little more for a while and that is nothing against the world, just us getting use to our new normal (come on over... just don't get tangled in wires or judge the crazy, lol)

She was pretending to call people last night once we finally convinced her it was ok to play with oxygen on

Still as cute as ever even with an oxygen cannula on

The look on her face after she calmed down yesterday

Oxygen concentrator, travel tank and backpack

Facetimeing with Mikaley and her stuffed animals

She played with my phone for a while a took pictures with different settings

This back and white one made her smile

Playing with a toy in my classroom before we left work. She did such a great job with it at school today!

The Jilli update (like my creative title)

This is another blog post with lots of info and updates. I will try to keep it well organized :)

1. Jillian had her 24hr pulse ox study starting at 3:30 on Wednesday and ended around noon on Thursday (yes I do get that it was not actually 24hr... close enough) Within the first hour I told Brent that the sound of the alarm was going to make me batty! We are use to beeping at our house... her feeding pump has a phd in beeping. But when it is a new beep sound it feels so much louder then the beeps you are use to. They set the pulse ox to alarm any time it went below 90 for the o2 levels. She had direct correspondence to when she was up walking and playing that it would drop to the low 90s and down into the 80s. She did do an amazing job at keeping it on. She was annoyed by it at first but within 5 minutes she was fine and went about her way. By the second day she was even trying to carry the pulse ox machine around herself. She was mad when I took the pulse ox off of her when the study was over. Silly girl! Now we wait to hear from the dr. They told us we would hear from them within 48 hours... but I don't know when the 48 hour timeline started (when the test was over, when they picked up the pulse ox, when the company that ran the test got the information to the doctor's office, when the doctor looked at the results... those all could have large gaps of time between them) I am hoping someone calls soon and that the dr office has received the test results and has a plan. Obviously she dropped too low too many times so we need to do something. We tested her pulse ox again today in therapy and once again it did the same thing. We have 4 weeks of therapy results and a day long study that shows something is not right.
2. Jillian had her echo on Thursday afternoon (hence why her other test was a little shorter then 24 hours) She was such a big girl for it. She laid on the bed, watched TV and did not make a sound or mess with the test at all. There was one odd thing with the test... they were struggling to see her heart when they went up from the bottom of her rib cage by her stomach. The tech tried and then a doctor came in and tried and they were not able to get the pictures they were trying to from that angle. The dr asked about her stomach surgeries and if there might be scar tissue up there from them. I said that all of her stomach surgeries were at the bottom of her stomach (feeding tube placement) and that I did not think there should be anything way up there causing a problem. The rest of the test when fine and they said that everything else looked good. Our follow up schedule with them will be based upon genetic testing results.
3. Jillian's ped called me this morning to check in. I was so grateful that she did because I had some questions for her and she had some questions for me. I asked her about the echo and the problem that they ran into. She said that she would like a CT done of Jillian's abdomen to make sure everything is in the correct place. This is not the only time there has been some questions about this area on Jillian. They first tried to place her feeding tube laproscopically however that did not work and they said that it might have been because her stomach was positioned high. Now with two different tests coming back with odd results in this area we need to look into it a little more. Jillian's ped asked me to contact GI to see if they would help coordinate doing a abdomen CT at the same time as her lung CT in July.
4. I contacted GI to see if they could help with the the CT. They said they would and they seamed interested as well. They said that if her stomach is not positioned properly that could be adding her her digestion issues. I am grateful for their help in this. They also said that they would like us to see one of the speech people at Children's again that works on swallowing/feeding issues to look into the aspiration stuff again before we were to do another gastric emptying study so we can all work together to minimize Jillian's aspiration risk in testing. 
5. Today we went to therapy and Jillian has a new PT. Last week we had both her old PT and the new PT and this week was just the new PT. I really like her and she was really good with Jillian. She also had Jillian's orthotics. Jillian wears the smallest size the company makes (her feet are really small, she wears a toddler size 4)! I am hopeful the orthotics help with Jillian's walking (she alternates toe walking and walking on the insides of her feet)
6. On Thursday the Children's financial department called (we were at Children's when they called and their voice mail scarred me because it just said it was the financial department calling about our account...) They were calling to let us know that Jillian's main insurance approved her genetic testing and her secondary state insurance denied it claiming it is not medically necessary. They said that with the primary approving it that we should do it now because we have the best chance of it being covered and we have already hit her deductible and out of pocket max for the year... they asked if from a financial standpoint if we were ok with doing it now... I said yes, lets get it going. They were going to let genetics know that the financial is ready to go. I am not sure if genetics is going to call me before they run the test or if my phone conversation with financial will start the test, either way hopefully it is getting closer to starting! Yeah!

Yup, thats a summery of the past few days. Thank you for all of the prayers, they have really been felt in this all and I have been encouraged greatly by some amazing people the past few days and I am so grateful! Please continue to pray that pulmonology comes up with a good plan for Jillian and that is communicated with us quickly and that we continue to move with the genetic testing and that it points us to a cause.

She had danced a little bit

She was interested in the pulse ox machine.

Playtime should not cause this...

Jillian pushing her pulse ox at school

during the echo

Therapy wore her out! She is still sleeping in her chair over an hour later

I have been quite for a little while

Typically if I go quite on here for a while it is because of one a couple of reasons:
1. Life has just been status quo for a while and there just has not been much to talk about (not often)
2. Life has been busy
3. Life has gotten crazy with Jillian stuff and time is on short supply
4. I don't think I can type what I want nicely

Right now you can combine 3 & 4.

Jilli curled up on Saturday saying "tummy hurt"

 This has been my motto the past few weeks:
'But those who wait on the LORD Shall renew their strength; They shall mount up with wings like eagles, they shall run and not be weary, they shall walk and not faint.' (Isaiah 40:31) (NKJV). Ie: There is a plan for all of what you are about to read... even if it does not seam clear to me right now.

I mentioned last time I wrote about Jillian's pulse ox dipping. When Jillian is walking around and in PT her pulse ox is dropping into the 80s. THIS IS A PROBLEM! I called her peds office the other day to ask for help about this all and when I said pulse ox in the 80's they talked about ER right then, however this is a long term problem not acute so the ER is not going to be able to solve the problem.

This problem was discovered during therapy because Jillian has such a short stamina and we were trying to figure out why... now mind you the therapist and I talked testing her pulse ox during therapy on 3/30. We tested it during therapy on 4/6. I contacted pulmonology that day. A nurse called me that day to say Jillian's dr was out of town but a fellow would be contacting me that day. By Thursday that week I still had not heard anything so I called them. A nurse apologized and said someone would contact me that day. Friday morning I called because the fellow still had not contacted me. Friday morning I got a message on-line to give her albuteral before therapy. This response made me mad. I called Jillian's ped's office and they refereed us to cardiology and told us to contact pulmonology again on Monday.
Monday 4/13 pulmonology contacted me and said to give her albuteral before therapy and if that did not work to contact them and we would do a pulse ox study. Surprise surprise the albuteral did not fix the problem. I contact pulmonology again on 4/13 and then did not hear from them. I called on Thursday that week because I had not heard back and they said they were ordering a 24hr pulse ox study to be done at home and that they were contacting VNA to provide the pulse ox. It had been a few hrs and I had not heard from VNA so I contacted them. They called me back while I was at my allergy appointment and VNA told me that they are not licensed to do the test pulmonology ordered for multiple different reasons that all made sense. I contacted pulmonology and they told me VNA just needed to do the test. This went back and forth all of Thursday and Friday with countless phone calls that VNA told me they could not do it and pulmonology told me that VNA had to. I was stuck in the middle between the two. It was not pleasant and I could feel my blood boiling. It was not a good situation.
This now brought us into the weekend where I knew once again I would not hear anything til Monday. Dan's mom called me and she helped me vent and process through what the best next steps would be. Jilli also took a walk on Sunday outside. By the time she was done her heart rate was 125 and her pulse ox was 81! Saturday night during dinner the skin around her lip looked blue. I was feeling beat up and helpless because I knew my kid needed help but not the kind of help an ER would provide... long term help.
Monday morning (4/20) I called Jillian's ped's office and expressed my frustrations with them.  They told me that since we were seeing cardiology today to wait and see what cardiology had to say and if they would not get us a long pulse ox study then her ped would help us. Monday afternoon while we were at therapy pulmonology called and said they found someone else who is licensed to do the test and that company would be contacting me. Thank you! This whole pulse ox dropping convo has been going on for almost a month... a month in which my anxiety has been high as well as my blood pressure... and oh yeah we find it very likely that our child is not getting enough oxygen.

Today we went up to Children's. We had the cardiology appointment scheduled but we ended up needing to see GI first today. Jillian's tube site has been weepy/bloody since Sunday. It is not granulation tissue but like the skin around the tube has just burst open. Her GI motility has been yucky the past few days and we have had issues with popping med ports and hard flushed because of bile wanting to flow up the j port. I gave it a few days to try to fix itself but when it still looked yucky this morning I called our awesome nurse and she told us to come up early and the nurse that was in charge of feeding tubes for the day would help us out. We went in and they used silver nitrate to "burn" the area to stop the bleed and this special stringy stuff to put over it to help it heal. This is odd because normally her site looks amazing. It is not infecting and she has only complained a few times that it hurt but it should not be bleeding. She did great with the silver nitrate treatment. She said "ow" once, otherwise she held up her belt for them and was such a big girl. I was amazed.
We then headed over to cardiology. They did vitals and then we went into a room. A nurse came in and went over history with us. Then the dr came in and we talked a little bit more of Jillian's history and what is going on. She said it could be that there is a hole in the top of Jillian's heart, or it could be a few other structure issues, however she thinks it is most likely that the blood is not coming from her lungs well oxygenated. She said that if it is one of the things that she listed that most of them are not things we can do anything about (that hit my heart like a bag of bricks). They tried to get us in for an echo today however the lab was booked so we are going in Thursday afternoon to do the echo. The doctor agreed that Jillian's stuff does look like mito. Everywhere we turn mito looks more and more like the reason for Jillian's stuff.
While we were with the dr the company that is doing the pulse ox test called and left me a voice mail. I called them back and they are bringing the pulse ox over tomorrow at 3:30 and they will come pick it up on Thursday. I am glad to finally have that scheduled after all of the fighting, although I will believe the test is actually happening once it started...
 We did get some great news this weekend! We got a letter in the mail from the insurance company that looks like they have pre-apporived her genetic testing. This is HUGE! Now we are just waiting for a call from genetics so we can give them the final ok to run the test.
We have also been messaging back and forth with the GI motility dr. She is great! We are working together to figure out the best plan for Jillian GI wise. I like that she is trying to look at as many options as possible and talking to other doctors so we can make the most informed choices. I really appreciate how hard she is working for Jillian.

Relaxing against a chair

Here is what we could use prayer for right now:
1. For the pulse ox test to show what we are seeing at home. Jillian has had so many tests that come out leaving everyone scratching their heads. We have seen her pulse ox drop every week at therapy for the past 3 weeks. We have also seen it at home. I am just praying that the pulse ox tomorrow picks it up because we can only help her with this issue if we they see it during the test. I don't wish for my kid's pulse ox to be dropping but it is so we need to get it accurately documented to best help her.
2. Pray that once the dr gets the test results that we come up with a good plan quickly.
3. Pray that the echo shows us what we need to see. Pray that Jillian lays still for the entire 30-40min procedure (she will not be sedated) so that she is able to get all areas looked at.
4. Pray that if there is a heart problem that we are able to work with the dr to best help her.
5. Pray that genetics contacts us soon so the test can start. We will not get results back for at least 3 months from the time the test is started.
6. Pray that when the bill comes the insurance company still decides to pay it. Pre-approval does not mean payment, it just means that they likely will pay, until the bill is submitted we will not know for sure.
7. Pray for results for the genetic testing. They are looking for a needle in a haystack (multiple drs have said that obviously something is wrong... its just seeing if science is smart enough right now to be able to pinpoint it). There is about a 30% chance of them finding out what is going on. If it is mito, it takes many families YEARS to get the correct diagnosis. Please pray that they are able to figure out what it is sooner then that. They are also looking at some other stuff then just the exome sequencing, please pray that helps us in figuring out what is going on with Jilli. We have hit the point where we understand fully that knowing what it is will likely not bring a cure but knowing what we are battling would be nice.
8. Pray for healing of the tissue around her tube site.
9. Pray for a situation with her orthodics, we are working on getting Jillian fitted for othodics but have hit a few bumps in the road. They look like they should hopefully be solved Monday but we will see.
10. Join us in thanking God for all of the amazing things we have in our life. We have an amazing little girl. We have some great people around us who have let us vent, cry, and rant these past few weeks. I know I have been crabby during this pulse ox situation and I have been a pain to be around but I am so thankful for everyone who has loved me despite. God has brought some amazing people in our lives.

Thanks for reading my long blog post. Thanks for praying. I know its a lot. Feel free to ask questions as I am sure this tired brain has left things out.  Thanks!

This look is more and more common at our house(yes she is cuddling with an empty water jug... I don't know why...)

She was Mickey/Minnie head to toes on Saturday and she lloved it!

Her at Uncle Seth's Birthday dinner. she was kinda like a wet noodle. This was after the area under her lips went blueish

Then she went to sleep on daddy

Cuddling with daddy's leg on Saturday morning

looking at books with Grandma

I think some boys in her life have had an influence on her... We stopped at the mall this afternoon so I could walk (good stress relief and it was too cold to be outside) She just wanted to go to the Lego store. She was excited to get out of her stroller and look at Duplos 

Easter Pics round two

So these pics are a little late getting online because we forgot multiple things at my parents house last weekend between me being sick with bronchitis and my dad taking the staircase on his back with enough silverware for 20 people flying though the air, we forgot the camera and Brent's wedding ring (he washed the silverware after it flew).
So here are pictures from Easter :)

She got a motorized Toby. She has had a lot of fun playing with it

My tubie girl smiling

She is getting so old :)

That is quite the look there Jillian

She decided that george needed to massage his legs

Yea thats a more normal pic of us

I love Jillian hugs!

Kisses for our special girl!

peek-

a-boo!

Grandma and Bumpa

playing with GG

quite the look Jillian

collecting Easter eggs on the deck

Opening her eggs to find quarters! She was excited to put them in her piggy bank

In other Jillian news, we heard back from her pulmonologist today. She wanted us to try albuteral before therapy to see if that helped. Her pulse ox before therapy was 98. They checked it multiple times during therapy and she ranged from 86-94 depending on what she was doing. It should not be dipping that low. I have sent messages to her doctors letting them know and we will see what is next. I am glad we figured out to look at this and am I hopeful that we can figure out something to help her. Please pray for good communication between all of her doctors and us.