Genetics December 2014

Tonight I am exhaust, and drained... but hopeful!

Today was a busy day! We got to see both IR for a tube change and genetics. I was so nervous about the appointments today that my diet up until dinner consisted of two cups of coffee, a little chocolate, two potato chips, and 2 waffle sticks. Yup, I don't really eat on days like this!

We made it up to Children's in good time. The parking structure was packed but they are doing construction on it again so the packed part was not surprising. My mom met me up there today.
Our first stop was IR to get her GJ tube changed out. They have to be changed every 3 months. She was excited at first for her hospital bands because we let her choose where she wanted them and she decided that today they were her bracelets. She loved holding the "stickers" too (labels). I love how she loves the little things about life! 
They called us back to IR and different people came over to see us. When you are seen every 3 months they start to know you. We have been visiting the IR department since Jillian was 4 months old and when she had an NJ tube and PEG we never made it the 3 months without a problem so we were seen more frequently then.
I gowned up in the paper suit, hair net and led dress. She thought I looked funny. We went into the procedure room and she sat on the bed. She was not thrilled but did just fine. Then the radiologist came in and we got started. 3 month old GJ tubes look pretty yucky coming out. She cried but laid still for them. She is still at the point where I can district her from things. I think what she hates the most is being strapped to the table. It is pretty quick to change them out and we were headed back out of IR pretty quickly. I put the hair net on her as I was undressing and she thought that was pretty funny! Life is about taking advantage of the moment!
We then headed over to the lobby area so Jillian could look at the fish. Some people were handing out balloons and Jillian was really excited to get a star balloon (a mommy safe kind)
Then we walked over to the genetics office. Her weight and height are up a little however she always measures bigger on their stuff then anywhere else so I take their vitals with a grain of salt.
Our genetics counselor came in. She started talking about how with just state insurance there was not a lot more testing they could do that would be covered (state insurance does not cover a lot of genetics stuff) and then I told her that come the first of the year Jillian's primary insurance would be United Healthcare. You could see her expression change! She said that United is one of the BEST insurances for genetics stuff because they cover the most things. This is AMAZING news. One of the tests that genetics has talked about running for 9 months is a test that costs around $15,000! State insurance will pay about $1,000! Brent's old insurance made it clear that they would pay nothing for it, making the other $14,000 our responsibility. Now with the new insurance they are pretty sure that they will be able to get it covered. Until we submit for pre-approval we will not know for sure but they are very hopeful. They said that United understands how much money it can save them in the long run to run this test instead of hundreds of other "smaller" ones.
I am feeling relieved that we have a plan! It is going to take a while to do everything. Brent and I have to meet with a genetics counselor first (a 2 hour appointment) to talk about what we want to find out from this testing and then they submit for approval from the insurance company. That appointment is set for February. Then it will take around a month for insurance approval if all goes well. Next they will take blood from Brent and I. They already have Jillian's blood stored in the lab. Then they will run the testing on all three of our blood. This will take about 3 months to complete. After that we will meet with them again to talk about results. I am hopeful that we will have results by this by summer. There is no guarantee with this test, but there is hope.
We talked again about a muscle biopsy. That is still not off the table, however they would like to run this other test first because it is less invasive. (a muscle biopsy requires surgery, where this is a blood test) We might still end up that route at some point but not right now.
We talked a little about Jillian's odd symptoms.  She has a strange rash that started on her face today. No one can explain these rashes and they go away strangely just like they come. Genetics got to see it today. We talked about Jillian's endurance and speech, and coordination issues. We talked about how her GI system seams to just stop working for periods of time and there seams to be no rhyme or reason (they thought the story of her randomly puking while checking out a Target a few weeks ago was funny) We talked about how we can't seam to make it 48 hours this fall without a neb. We talked about her reaction to the shot (they agreed too that her reaction is not normal nor ok) They said they are not sure if it is one thing or multiple things happening at the same time but they hope this test will shed some light on that. 

I can't begin to explain how good it feels to have a plan. Good enough that I am less stressed and had a yummy meal at Cheesecake Factory and dessert!

I am seeing the pieces fall into place. This is one of those times were you can look back at the trials and see where God's hand was in all of it. It does not necessarily make you love the trails that you went through but they make a little more sense. I see why Brent needed to change jobs twice this fall and why  God made him uncomfortable enough to make 2 job changes in 6 months. Without it we would not be where we are now with a chance at running a test that could help Jillian. This fall has been really rough, but like always, God had a plan all along.

Thank you SO much for all of the prayers today! They were felt. I TRULY appreciate them. I am so grateful for the people who stand around us on this journey and love us. The people who hug us when we cry and dance with us for happy news (it was awesome today watching the texts come in after I told some people about genetics, and how you could just tell that they were celebrating with us and loving us in every season) Thank you to everyone who had to deal with a crabby Amanda this morning and loved me anyhow. I don't have the energy or time to really stress about appointments like this until the 24 hours before the appointment, and even though stressing about it does not help, it still hits hard right before a big appointment and I am grateful for the people who love me though the stress. Thank you!

Jillian walking around Children's Hospital of Wisconsin in her Super Tubie shirt and Tutu!

Imunology

We don't see enough specialists at Children's (hahaha) so yesterday we got to meet a new department...

When we were in Airo Digestive the ENT referred Jillian to immunology because of some suspected allergies,  she always seams to be sick with something and it takes her longer then normal to bounce back, and because she struggles so much with vaccines. So, I called and got us an appointment.

On days were we meet I new doctor I have butterflies in my tummy! I told my coworker that it is like going on a blind date. You have no idea if you are going to get along with the person, if they will listen to you or if you will have the same plan. We have had (and still have) some amazing doctors on this journey who have helped Jillian and always wanted the best. They have listened and worked with us. There have been some that stand in the middle that do their job and while I am sure they care, they are not always the easiest to work with or the most open or have the best communication. Then there are the doctors that I would really like to never talk to again.... EVER.  There are three specifically that are on the Amanda Crap List. Luckily the likely hood of us seeing one of them is next to none because he started his own practice however at anytime we might have to deal with the other two. It always scares me when we meet a new doctor that they are not going to listen to me or understand what I am talking about. Jillian has no overarching diagnosis. She has a lot of strange symptoms that have yet to make sense to anyone.

So I did what I normally do before we meet a doctor... I look them up on-line to find out about this guy. Everything about him that I read was glowing. He was voted one of the nations top doctors for 2013-2014. He has done a lot of great stuff in medicine. Honestly this made me feel a mix of hopeful that he was as good as the internet made him out to be,  and a littler nervous that he was book smart and not talk to parent smart.

I was blown away! He is one of the most child and family centered doctors we have met. The whole clinic was great. The person weighing Jillian understood the need for us to get a good weight on her and they were fine with us undressing her first so we were not weighing her clothes (some clinics get really annoyed by this). Her weight and height are the exact same as they have been for months. She is back under the 5th percentile for both weight and height. She still sits at at 50th percentile for wight for height. Jillian hates the pulse ox and blood pressure and the lady doing those was great (her pulse ox was low for her yesterday... not sure why). The nurse that came in first was really good. Then the fellow came in. She took the time BEFORE she walked in the room to go over Jillian's chart. To me that is huge. I can quickly give someone the highlights of the last 22mo however hashing through it all takes a long time and I end up forgetting parts. There is too much to remember. The fellow talked with us for a while. She looked at Jillian and said that we would probably be doing blood work and that for one of the tests she would need to have blood drawn, get a vaccine, and then have more blood drawn 4-6 weeks later. This I was not expecting (actually this kinda hit me like a train and I did not know I was standing on the tracks). She said we would talk about in more when the doctor came in.

The doctor came in and we talked. He really wanted to hear what we had to say. He said that he agreed with the fellow that we needed to do blood tests and the testing with the vaccine. I loved that he had a conversation with us about the vaccine issues and did not just look down on us for not having her shots up to date. Many medical professionals have judged us for that and she is not up to date on her shots for medical reasons. With him it was a conversation about how he could best help in that area and what was most important. The decision was to give her the MMR shot.

We went down to the lab. They needed a lot of blood. They had to calculate it out to make sure they could take as much blood out of her in one day as they needed. Luckily they could. Jillian was not happy about it all but she was a trooper about it.

Now we wait. Ever feel like you are sitting on a bomb and waiting for it to go off? That is what I feel like right now. We are waiting for this shot to take its affects. It always takes a few days and then by 10 days out she is so sick she can't handle it and we need to see a doctor. So now we wait. We joked with them that they should just schedule us a room for 10 days from the shot. Hoping we don't need it, but reality and the past is very vivid in our minds.

The doctor said the he likely does not hold the magic piece to all of Jillian's problems. He doubts he will be able to solve the big picture stuff for us. We kinda stumped him. He said he has never seen a kid that gets respiratory symptoms from a shot. He told us his plan was to talk about her case with other doctors in the department to see if anyone else had an idea. I am thankful that he is not too prideful to collaborate with others to try to get my child the best care that he can. That means a lot to me. It is always a strange feeling when you stump a really smart doctor, but I feel like he is going to do the best he can for Jillian. Like we told them yesterday, we have a lot of tests that come back "normal," we don't want something to be wrong with our daughter however something is wrong with our daughter and we would love some answers. We always say we want them to tell us what is wrong... just don't let it be something too bad! We watch our daughter's body fail her daily (the fellow touched Jillian's legs yesterday and without us telling her she asked about Jillian's low tone because she could feel it in just touching her legs) and we want some reason why. We want something to point our finger at and say "this is the problem." The older she gets the less likely that I feel that the problem will be easily fixable but we will keep looking. Our goal is to give her the best life possible, God gave her to us here on this earth and that is the least we can do for her, to try to give her the best!

Waiting for labs. I was trying to distract her

Jillian sleeping on my legs Tuesday night. She fell over with exhaustion

 

What a week

Ever had one of those weeks that you are glad is almost over? That would be this week.

Last week we got to be a part of my cousin Jessica's beautiful wedding. Jilli loved seeing all of the people and it was nice to see family. Welcome to the family Ryan!

A weekish ago I had anaphylactic reaction to latex. It landed my butt in the ER for several hours. I have not been in the hospital for asthma since a couple of months before my wedding (5 years ago) so I am not use to is anymore (there was a 6 year time frame where I frequented the hospital for asthma issues). I had never had a full anaphylactic reaction before. I am allergic to apples and it cause my throat/lips to tingle however it has never sent me to the ER. It has been a while since I have seen an ER move that fast. My respiratory rate was so low it was making things flash and beep. Within 15min of entering the ER I had an IV started, shot of epi, muscle relaxer and predisone in the IV, start of an hour long run of a run bag of IV fluids, and a neb treatment. Before I went into the ER I did a neb treatment and took 2 Benadryl. Within an hour and a half time frame I had 3 nebs. The doctor said when I arrived I was not moving air however after all of those drugs within a couple of hours I was able to function. I would bet that it I was not a moderate asthmatic that knew what they were doing they would have admitted me however they let me go home. I have been on nebs all week and predisone. Today I am starting to really start to be me again. It has made the week long.

Jilli has been taking nebs this week too. She had a broch on the 23rd. They say that recovery from those are quick however with Jillian it takes a long time to bounce back.

Tuesday I had a follow up with my doctor about my trip the the ER. We decided that it was a good idea that I now carry an epi pen and stay far away from latex (I really want fries and campfire sauce from Red Robbin right now but they have balloons there). After my appointment Jilli and I went and spent time with my grandparents. It was good to catch up with them. Tuesday night was spent trying to get my epi pen. Apparently our new insurance covers only a couple of dollars of the over $300 pen. I found a coupon online for $100 off however it was still a crazy price. I kind of needed to pick it up that night though because I was taking a bunch of 4K kids to an apple barn on Wednesday and wanted to be safe.

Wednesday we went to the apple barn. The parents of the kids in my class probably think I was crazy because I did not touch anything while I was there but the field trip went well. Before I left for the apple barn Jillian's pump decided that it did not want to work. It just kept erroring over and over. We had a problem with it last weekend with her pump turning off in the middle of the night for several hours so she missed several hours of feed. By mid day Wednesday I was ready for her pump to take a flight (this is the one that they switched out for a month ago and honestly I have disliked this new one for a while, it runs slow and the battery sucks and it does weird things) I called med supply as I left work and they said they could switch out the pump. I was on my way to Milwaukee to get one of Jillian's meds anyhow so we went to their main office. We were there for a while. Jilli and I then stopped at the zoo for about an hour to see the elephants, giraffes and to ride the train. We LOVE to go to the zoo and I love that we have a zoo pass and are able to just stop by for an hour and see a couple of things, ride the train and not feel bad about only being there for a short time. We then ran over to Children's and picked up her med. Then we went to the Mayfair Collection. They have a Carter's store. I was looking for a jean jacket for Jillian. We bought her a peach jean jacket on clearance a few weekends ago and she is in love with it. She LOVES jackets right now (almost as much as shoes) Carters had the same jacket in blue on clearance and the kenosha store was out of it. The one there had it in an 18mo so I picked it up however it is still way too big for her right now. Then we went to Mayfair and met Dan and Brent to make Build-a-Bears for Tubie Friends. Then we went to dinner at PF Changs (my meal sucked... don't get their Pad Thai!) Then we went home :)

Thursday night we had dinner with Bren't parents in Lake Geneva and then came home and watched the Packers kick butt!

We also got a call back from the nurse in charge of the aero digestive clinic on Wednesday. She gave us results back from her surgery:

ENT: There is no clef, just that weird thing where there does not look like there is muscle but there is. She said to follow up as needed.

Pulmonology: Her lungs have bacteria and such in her lungs that show that she is aspirating regularly on her own secretions (this is the same as her last bronch last Christmas) They said to stay on our normal follow-up schedule with them.

GI: THEY WANTED TO SEND US BACK TO FEEDING CLINIC. I will fully admit that there was NO nice word in my head when she said that. I then questioned this and kinda said no. Why would we go back to feeding clinic? She is not medically cleared to eat. You just told me that her lungs show chronic aspiration! The nurse said that she would talk to the speech path that works in feeding clinic and get back to me. She called back a little while later to say that the speech path said she does not belong in feeding clinic (THANK YOU!) She said the speech path said to send us back to Dr. Kahn (THANK YOU!) So the moral is, after me throwing a fit, we are going back to the GI dr that I like! Yeah! Sad news... we could not get back in until November.

Jillian's new pump is runs fast. She spent most of Thursday miserable. Her first pump ran fast and we could only get her as high as 58, her second pump ran slow and we got her up to 61. Her new pump is running about 7% faster then it should. We have bumped her back down to 58 and she is spending less time in pain. We are working with her dietitian to work on getting her to grow (her pants from last year are falling off of her)

So this is a catch up on this week :) the best part... Lots of cuddles from my little girl! I love jilli cuddles!!!!!!

Jilli and I at the reception

Jilli dancing with Jess

double nebs!

Jilli and daddy sitting at the top of the stairs

Jilli cuddles

Jilli taking her meds

On the train

She had such a big grin!

Waving with two hands

Jilli stuffing a bear at Build-A-Bear

Jilli took a picture of grandpa during dinner

I also have TONS of these photos on my phone

Jilli watching the Packers

This is a picture from her surgery. This is her throat. This picture is sideways. It shows the funny part if you know what you are looking at... if not... her is a picture of how just above the vocal box should not quite look

August genetics appointment

I tired emotionally today. There has been a blessing in being crazy busy with work stuff the past few days... It has made it so I have not stressed out about seeing genetics. Funny after genetics today I am more stressed about the genetics appointment then before..

Here is the genetics plan right now:

1. Head CT. We have to sedate her for this so we are trying to get that figured out. Based on that will determine if we go to see Nero again. We are trying to be able to do her tube change and CT at the same time. I am really hoping for something amazing with scheduling. Finding me a Friday afternoon where we can do both of those things... I know I am asking for a lot but it would be best for her.

2. State insurance will not pay for genetic testing. They said she is a candidate for genetic sequencing. Paying out if pocket will be in the $12,000 range. For now her blood is sitting frozen in case we decide to do that test later. 

3. Jillian got bloodwork drawn today to check her carnatine levels. They submitted a script for a supplement in case it is low. They will let us know next week if we need to start it or not. That med will need to be taken 3 times a day...

4. The muscle biopsy is still on hold. This frustrates me. Do I want to send my kid back in the OR... No. Do I feel like we are going to end up doing the muscle biopsy eventually...yes. So I would really like to stop putting off the inevitable. I know they want to exhaust all of the least invasive options first but isn't there a point where the least invasive option is doing the "more invasive" option because the help that it will give the child will be the best thing?
5. The doctor told us that if Jilli gets any bad colds/viruses or high fevers this winter to call their office and they will most likely preemptively admit her for IV fluids and such. I am hoping to not need it at all but it feels good to have a plan in place for the inevitable.  It is frustrating to take Jilli to the walk-in clinic sometimes because Jillian does not present a typical case and sometimes I bring her in before all of the wheels fall off the wagon so they do not do much for her and we just have to sit at home and wait until she gets sick enough to be rushed in. He agreed that we should not take her to local hospitals but instead get an ambulance transport up to Children's. As I sit here and write this it kinda hits me. I have a kid that is in a health situation that justifies us making plans like this with doctors and making prearrangement was not our idea, but theirs. It is rather a humbling thought. We kinda just live the day to day and do. We don't stop often to look at all of it or the intensity. Honestly, we can't. But when we do it kinda hits you.
6. We see genetics again in December. This is where I started to get crabby. I want this process to move faster. By the time we see them again she will be 2. That will mark two years of this crazy without a lot of answers to the why. What is the underlying cause. We have a lot of "band-aids" holding it all together... kinda. She is not in immediate danger like she was younger, but the balancing act of her body functioning is held together with a lot of patches that kinda get us by day to day. It would be great to have a treatment plan for a condition instead of treating the symptoms. I want to get this moving so we can do the best things for Jilli. I feel like I am on a slow boat that I could row faster. Jilli is making progress developmentally but it is at a very slow rate. She was officially diagnosed with apraxia this week. That kinda spells out that it is going to be a long slow road for speech. The geneticist was impressed that she has made some gains so he is not as rushed about that but I don't think he understood the amount of work that it is taking to make each little tiny gain.

So that puts me at the point where I am glad we have some plan, because no plan would have really made me mad, however I don't love the speed of the plan.

On a side note, we got to meet a med student today. He was shadowing in the genetics clinic. The poor guy was so scared. The doctor asked him if he would be the one to look at her ears and the poor guy looked so frightened. I told him that Jilli was a good test person for looking in ears because she thinks it is funny. Hopefully doing an ear exam on Jilli makes him more willing to try more... however we kinda spoiled him with an easy one today.

Today my mom and I went shopping for dressed for my cousins wedding. I finally found one after looking at many stores. We found Jillian one too at an amazing clearance at Jaine and Jack.

Now I think I am ready for bed. This next week for me is the equivalent of a retail workers week before Christmas... the most busiest time of the year for teachers. School starts in a little over a week!

Ch ch ch changes...

I feel like we are in a time of a lot of transitions right now. Corney songs about changes and different seasons have been running though my head.

So what is all the change?

As I type my baby brother is working on moving into his apartment in Ohio. This will be a big transition for our family. See I went away to collage... an hour away. Seth stayed home and went to a school in town. He now has an amazing opportunity to work on his PhD at Kent State. He will be there for the next 5 to 7 years... 7 hours away! While in many families the big sister would either shrug off her brother leaving or be happy for his departure, my brother and I have never really fought. We get annoyed with each other from time to time but growing up at our house meant that fighting with your sibling was not an option. While we were not as close while I was in high school and collage, over the past couple of years we have been getting closer. He was the one I called when our heat went out and Jillian was only around 6 weeks old and Brent was out of state. He sat here all day with me while repair men marched in and out of our cold house in February. He was someone I could always call to bail me out. While my brother will always be here for me, it will just be different now. He wont be here to do crazy things like spend his birthday in a hospital room with Jillian (his 21st, none the less) or go to moves I dont want to see at late hours with Brent. It will be an adjustment. I am so happy and excited for him. This will be such a good opportunity and he is SO smart and I am SO proud of him.

In the spirit of moving, last weekend a moving truck showed up at our house. No, we are not crazy enough to move in the middle of our crazy (although Brent has suggested it and I give him a crazy look), Dan moved out last weekend. Dan moved in last June, for a month of two. He was in the process of finding a new job and his lease was up on his apartment and he did not want to sign a new lease if he was not sure where his new job would be. His job hunt took longer then he planned and in April of this year he got a new job in Milwaukee. He has since been commuting an hour each way to work. He found a place in Milwaukee much closer to his work (and close to Children's) that he will be sharing with his sister. It was hard to watch that moving truck leave our house though. Over the last 14 months we became an odd little family. He moved in when Jillian was 6 months old and had a feeding tube down her nose (yes, we do measure time at our house by what type of feeding tube Jillian had). A lot had changed over that time. He lived with us for the majority of Jillian's life so far. He could have just kept himself hidden downstairs (the first time Dan lived with us, in collage, Dan and I did not really talk or get to know each other until right before he moved out, in fact I did not even have his phone number most of the time he lived with us the first time), however he dove head first into our crazy and if Brent was not home he would help me with Jillian stuff. He made formula a few times and knew how to get her meds ready. It did not creep him out that we leave drainage bags to dry from our cabinets (although his rule was that he would not wash Jillian dishes... I don't blame him, they all smell like Elecare Jr or are syringes or bile drainage bags). He fell in love with our little girl in a way that I am sure he did not think he would. Those two have a special bond. Some nights she would just want him instead of her boring old parents. It is strange now just texting Brent on the way home from work to see what time I should plan dinner. It is an adjustment. I am sure most people would see having an extra person moving out of your house as getting things back to normal, but having him here became our normal, and now this is odd.

Brent is in the full swing of his new job. He is loving it and is much less stressed at night, for that I am grateful! He also gets home at the same time each night and does not have to work nearly as much at home. He is also adjusting to a new med he is on for debilitating head aches. His doctor was trying to do nerve blocks in his neck to help with these awful head aches and hand tremors that started about 6 months ago however the shots were making him very sick. The new med is helping a lot more with the head aches however it has side affects itself (but not nearly as bad as the shots). Brent has never been a person to sleep much and normally would go to bed sometime after midnight, however on this new med his body needs more sleep. For him that is a lifestyle change that he is still adapting to.

I am in full swing of getting ready to start my new position teaching 4K in the fall. I am really excited for this new opportunity. With starting anything new, there is a lot to do. As per normal me, I have a to-do list running that I keep remembering little things that still need to get done. Being a teacher, the time leading up to a new school year is always the busiest. My type A personality kicks in and I drive to do my best. I am excited to get my classroom set up and meet my kids. I am excited for the opportunities that this new position will give our family (I will be working 5 half days). Hopefully I get most of my to-do list done in the next two weeks. School starts Sept. 2nd, ready or not! :)

Jillian is about to have a month with A LOT of appointments. I was working on organizing calendars today and she (along with me) is going to be one busy girl! We are moving her PT and speech time in September to right after I am done teaching. We are still trying to find time to do her OT eval and will probably be adding OT to the therapy mix in the fall (at least that is what her PT has hinted at). Next week we go for her genetics follow up. I am trying to remain neutral about the appointment. I am hopeful that all of her test results are back and that we can continue to move forward with our plan. That has kinda been stagnant for months again and I just can't think about that or I get annoyed. In September we also meet with the aerodigestive clinic for the first time. I am excited to see how that all works and hopeful for some better coordination between clinics. Jillian also sees audiology to have her hearing tested again. It was tested last year and her speech path would like it tested again so we know how her hearing is functioning now. I am assuming it is close to last year. She still does not react to low tones (bass guitar, fireworks, ect). September also brings a tube change. Her last one went so smoothly and I am hoping for that again. It also brings the Children's Hospital walk/run and my cousin's wedding. Hopefully Jillian will have as much fun dancing at this cousin's wedding as she did my other cousin's wedding last weekend (I still need to get to blogging about going to the wedding and all the fun Jilli had... soon hopefully!). September will be busy for our little girl but hopefully it will be a good month for her. She has still been struggling a lot with poop and that makes challenges for the rest of her system. We are seeing gains in her speech and that makes me so happy. It brought tears to my eyes the first time she signed "book" a week ago. She normally just signs more and we have to guess out of everything around her what she wants more of so for her to sign for an actual object was huge. We are working hard on the sign for "help" so she has a way of communicating that she needs help other then screaming. She is really struggling with it because her hands do not cooperate with her and most of the time she will just start waving her hands around knowing she needs to do something with them however she struggles with making a fist. It is rather cute actually... until she starts screaming... lol. We are blessed with this little girl and her amazing smile and great attitude. I love her so much and am so proud of her.
Jillian's feeding pump also got changed out last week. I had heard that some med supply companies change out feeding pumps once a year for service however we had never heard anything from our med supply company about this. That was until I got a call Friday morning that they were coming Friday afternoon to switch out her pump. This caused a little bit of panic in me because we were going out of town on Saturday and what if the new pump did not work right? They also told me they would not be delivering it to me until after 4pm on a Friday... A Friday that I also had a ton of things to get done and had the internet guy coming over in the morning for several hours to fix our satellite and I NEEDED to go to the store before we could go out of town, thus making it an already crazy Friday. On top of that med supply was suppose to have delivered this month's supplies on Wednesday however they did not have bags for the pump in and had to wait until they got their delivery before they could bring us ours however this cut it very close to when we were about to run out of formula... that they don't sell in stores. So when they called saying they were switching out her pump it almost put me over the top, however I pulled myself together, took a 19mo old to the store during her nap time, and made it all work! So, we have a new pump (same brand). It has a few quirks but thankfully none of them caused for any more headache then getting the pump exchanged was.    

So, those are our changes right now. Rather overwhelming some days, but I am doing my best to keep it all together. For every season...

This was the last night that we all spent under the same roof while Dan lived here. We ate yummy food and played video games. See Jilli joined in on the fun too!

Uncle Seth putting his sock on Jilli. I don't think that is the fashionable shoe she is looking for

Dan's moving truck

Jilli had to check out the bathrooms at Dan's new place. She loved the big bathtubs so much she did not want to leave.

The family at Kopps. Mikaley taught Jillian how to put spoons on her nose. After she apologized for teaching her how to use a spoon the wrong way... I said it was just fine, she does not need a spoon to put things in her mouth so why not put them on her nose!

This picture means a lot to me. I know it means nothing to most of the rest of the world, but is truly special to me.

It was my dad's birthday on Monday. We went to dinner at Fred's. His kids got him "Its a Small World" dolls. He LOVES that ride!

Jilli loves the dolls too!

She put on Brent's slippers. She has a thing for shoes! She looks so tall in this picture. It is funny that the night before she was told that she could not go on ANY of the rides (with me) at the carnival in Lake Geneva. Apparently you have to be at least 36inches to ride the merry go round or small train with a parent and she is not even close to that! 

This is a waffle. This is a waffle my husband made for me. This is a waffle that my husband made for me using the recipe they use for waffles at Disney World (what I eat for breakfast daily there) because he knew how happy Disney World makes me and he knows we probably can't afford to go for several more years and he wanted me to have some Disney magic at home! 

  

Feeding Team Eval

Friday was Jillian's eval with the feeding team. If you have not been following for a while here is the back story:
Head of GI decided that we needed to go to feeding team despite our thoughts on the situation. Our other appointments with GI were all cancelled (NOT by us) and we were told we had to see Feeding Team before we did anything else. To say I was annoyed was an understatement. Feeding Team's goal is to get kids that are SAFE to eat off of their feeding tube and eating normal food. They do this with a multidisciplinary approach and it is a great tool for kids that are at that point. We are no were near ready for feeding team with Jilli. She is still not safe to eat. For the past few months I have been stressed about this appointment.

My mom came with Jilli and I to the appointment. We got up there and I have to fill out more paperwork. They brought us back and measured Jillian. We we visited with GI back in May Jillian was
21 lb 1.7 oz and 75.4 cm. Friday she was 21 lb 1.6oz and 76.6 cm. (ie, no real growth in 3 months...) Jilli is looking more mature in the face so people keep telling us she looks like she is growing however she really has not.
We then went back to the feeding team room. It is a large room with a kid sized round table and an adult sized round table. It also has a lot of high chairs and cabinets. The first round of people came in. First we meet with a Speech and language path (same one who did Jillian's swallow study in June), an nutritionist, a psychologist, and a nurse.
Their first question was "What is the goal in bringing Jillian to feeding clinic?" My first response wanted to be "to jump through this stupid hoop that your boss is making us jump through so I can get the best care for my child because she really does not belong in this clinic..." but I took a deep breath, looked at my mom and said "Our goal is always that someday Jillian will be able to eat by mouth but what is most important to us is that she is SAFE to eat by mouth before we push it." They found that an acceptable goal...
We then discussed Jillian's case in detail. At the end of our discussion everyone made their recommendations.
SLP: You can try to do tastes (ie, dip a spoon in baby food and shake all of the food off so only the taste is left on but she can't get any volume) but only when it is safe and at our speed. She said Jilli is not safe to eat volume orally.
Dietician: Jillian has not been handling the goal of bumping her up to 61ml/hr. Since that is not working we are going to try mixing her formula to 22cal per oz instead of 20cal. We are hoping that she tolerates this change. Other then cal changes being hard on her pooping, she normally reacts to them well.
Psychologist: We are doing exactly what we should be. We are making the experiences with Jillian's oral med possessive and we have worked hard to keep her from developing an oral aversion. She said we are right that keeping her airway protected is most important right now. She said that she is there if we need her someday but right now is not the time for this.

Then everyone left to go meet with the Dr. (head of GI guy). Once they were done meeting they brought us to an exam room across the hall and we meet just with the dr. He shook my hand and said that he did not think we have ever met before. (insert a ton of emotions here that I kept inside my head). I reminded him that Jilli is the reason he had to listen to the theme song of Daniel Tiger's Neighborhood for over 30min one day. Then he remembered us.
He said with the conversation about muscle disorders and that she is not safe to eat that this was not the clinic for her (insert a momma who had to just smile or the sarcastic comments about this situation were about to go flying out of her mouth). He also said that some kids with muscle stuff only get worse with their GI stuff and that this clinic is here if we need it someday but that we might not. As hard as that is to hear it is a realization that we have already come to, it is just nice to see that he has finally gotten there too. We want our kid to eat but we have seen what has happened to her body since she has been born so we need to be realistic in order to give her what she needs.
He said they are transferring us to different clinic. The airodigestive clinic. It is a multidisciplinary clinic were an ENT, pulomonologist, GI and SLP work TOGETHER for kids with GI and lung problems! This sounds like where we should have been for months. In the end jumping through this hoop is going to get us better care for Jillian and in the end that was my goal.
They decided that we would be seen in the airodigestive clinic once every 6months (starting in September) and then see our NORMAL GI dr each month in-between! To me this sounds like the best of both worlds! We get to go back to the dr we like and get to work with a team that specializes in kids like Jilli. The only snag in this plan was that a dietician does not work in the airo clinic and a dietician needs to see Jilli next month because we are making the changes to her cal count.
The nurse came into the room and said that mom and Jilli should stay in the room and wait for all of the paperwork that they needed to give us and that I should go up front to make a separate appointment. As I was walking up front our favorite nurse was about to enter someone else's room. She and I stood and talked for a minute and then she wanted to see Jilli. We then went and found Jilli. She talked to her for a little bit and then decided that we needed to go see everyone else. See Jilli is well known in the GI department. A lot of different people have worked with us and she steals their hearts. Our nurse took off with Jillian in her stroller down the hall. We left my poor mom sitting in a room alone waiting for paperwork. Jilli and I got to go in the offices to see different people that we knew. Then someone found us with the paperwork. We talked for a minute and they said we could go make the appointment. My mom found us and we headed to the front desk. There was a new receptionist and she was having a hard time making the appointment with the dietician so our dietician ended up coming up to help.
We left there and headed down the elevator. As we got out of the elevator on the second floor there stood our old dietician who we have a great relationship with. We stood at the elevator and talked for a while and then a different GI nurse came by and we all stood there and talked. It was just one of those days were we ran into all of our favorite (except our normal dr and favorite receptionist) around GI. The GI department LOVES our little girl. Many of them would bend over backwards for her and many have. From personally carrying Jillian into the OR so she is not scared to taking time to really listen to what we are saying, the support staff in GI is amazing. They are the ones that have made Children's feel more like home to us and they truly care (to the point where I wrote many of them thank you cards and Jilli is known as the little girl's picture who sits on desks all over GI).

We then headed to see my cousin Luke. We ran into them on Monday when we were up at Children's and he was still stuck there. We visited with him and his mom for a while.

We then went to Cheesecake Factory for lunch and then stopped at the zoo to ride on the train (we promised Jilli we would take her on the train for how good she had been) We stayed at the zoo for a couple of hours and then headed to Target to pick a few things up. We then headed back to my parents.

Jilli and I have been staying at my parent's for a few days. Dan has C. Dif. It can be very contagious and  deadly for kids like Jilli. I have had C Dif in the past making me more likely to get it again (I got it from contact with someone who had C. Dif, I had not been on antibiotics or in a hospital in over a year when I got it.) I contacted my doctor and he said that once the house was scrubbed top to bottom with bleach that Jilli and I could go back home. Brent has been working since Thursday to get the house clean enough for us to go home. I am really hopeful to go home soon. Please pray that none of the rest of us get it and that Dan starts to feel better quickly.

I want to thank all of the people who were praying. I sent out a couple of texts Thursday night asking for prayers about the appointment and for the C. Dif situation and we were covered in prayers. I am so grateful for the people in our lives so stand by us and pray for us, offer encouraging words, or let us crash at their house randomly because we can't go home. I am so grateful and we are so fortunate that God has provided some amazing people in our lives! This is not an easy road but God is good and He provides what we need.

A BIG thanks! and New PT

We are hugely thankful for some awesome men this weekend! When we bought our house the inspector told us that the decks were in ok shape and could use a board here and there replaced. Fast forward two years and that was farther from the case. Our back deck is large and attached to our house 1/2 a story off the ground (we have a split level house). It had no supports in the middle on the deck and the posts of the deck were not put in well. This was causing the deck to sag and wobble. Our front deck was just a ticking time bomb for someone to fall threw. By the landing to get into our house they only put down a piece of plywood and covered it with indoor-outdoor carpeting. It was sagging and not in good shape. Additionally we had a chicken wire fence between us and our neighbors that was rather beat up from a tree falling on it. All of the equated to some large safety concerns around our home.
Friday night Dan's dad and younger brother came down and spent the night at our house. Early Saturday morning everyone got up and Brent, Dan, Dan's dad and brother, and my dad all got to work on all three projects! By Sunday afternoon everything was done and we now have two safe decks and a new fence. I am super grateful for everyone's hard work and for giving up their time to help us.
While they were working Saturday morning Jilli and I went to my cousin's bridal shower and once we came home all Jilli wanted to do was sit in a chair and watch them work. It was like she was the little foreman on the project. It was adorable.

The guys put in the second row of posts

New PT:
Monday was a busy day! In the morning Jilli and I worked on laundry. Jillian had an 11:45 PT eval at New Berlin Clinic. We talked with the therapists and they did an assessment of her gross motor skills. They diagnosed Jilli with low muscle tone in her legs. Her joints are also able to rotate and move more then they should. Jilli is going to start PT once a week for 45min. We are doing to do PT and speech back on Mondays so we don't have to drive to Elkhorn twice.

Yesterday it worked out for the eval though that we had to be at the clinic from 11:45-12:30 and 4:30 to 5:15. This left a big gap in the middle of the day that we were not sure what we were going to do. Seth said that we could come and visit them at Yerke's Observatory in Willem's Bay to see where he has been working this summer. Jilli and I got a personal tour of the building and got to see where they are living. It was REALLY cool! We were able to stand in a room Einstein once stood in. We got to see really interesting parts of an old building. I loved it. Jilli was more or less along for the ride but she loved listening to her voice echo in the domes. Thanks Seth and Mikaley for giving us a personal tour!

Jilli and Mikaley looking at the Big Telescope!

They still use the same control panel

Jillian walking around with her feeding tube backpack (I wonder how many people have tube fed in the observatory....). She liked to hear the echo 

We were looking at old books. Jilli's eyes were big at the sight of a room with so many books

A picture of Albert Einstein in the observatory.

 

Swallow Studdy

This post has been sitting open in Firefox for a week with just a title and a blank body. I have stared at the big blank white box multiple times however unable to write a word. It is not that I did not have something to say, it is that emotionally I could not do it. So here I sit, a week later, making this big box a little less white.

Last Thursday Jillian had her second swallow study done up at Children's. We had not ask for a swallow study, the head of GI decided she needed one because he wants to move forward with feeding clinic. A swallow study looks to see if a person can safely swallow when given food. It is done with X-ray and watches to see if food goes down the esophagus or the trachea. Jillian has never had a problem with swallowing the first time something goes down. Her problem comes in when she refluxes her food back up; then she aspirates on it while food is trying to go up and down at the same time.
I am pretty positive with this study the head of GI's goal was to prove that she is safe to eat. Despite what multiple other professionals at Children's and Aurora has told him, I think he still does not get what is going on with Jillian. This study for Jillian proves nothing. It is is only a couple of minutes long and only looks at the initial swallow.
Last Wednesday night I was filled with anxiety about the swallow study because:
1. I was afraid (and still am) that despite that her issues are with refluxing foods, that from a clean swallow study he would say she is safe to eat and try to push us to feed her orally even though that is not what is best for her and will lead to pneumonia. Multiple other professionals agree that she is not safe to eat.
2. For a swallow study Jillian has to drink barium. While she is fine with drinking it, I was afraid of what it would do later. Her getting sick from aspiration is hard on everyone, especially her. We try our hardest to avoid her getting things in her mouth and to think about giving her something on purpose is hard to wrap my head around.

My mom went with me up to Children's for the test. We got in the room and started talking about the test with the speech path. I liked her a lot. She agreed with us that Jillian's medical history proves that there is some underlying medical issue going on and that it would be super helpful if we could figure out what that was. We talked about the aspiration and how it happens and she gave us the option to opt out of the swallow study. After some discussion we decided to do that study but to only do a very small amount (the same amount we give her orally each day for her medications). We decided to give it to her in a syringe so we knew exactly how much we were giving her. Currently the only way she is getting anything orally (her meds) is through a syringe. We decided that since all this test would look at was the first swallow, that is did not make sense to give her a lot to just make her aspirate when the camera was not on.
We got the barium in the syringe and started the test. The test last just over a minute. Jillian did not aspirate during the test but we did not figure she would.
The speech path said after the test that she has an immature sucking pattern but with her history she would be surprised if she did not. She agreed though that we should not be giving her anything but her medication by mouth. She said that with Jilli it is not safe. The speech path understood Jilli and her feeding concerns.
As much as I had a lot of anxiety going into this test, it did bring a little bit of peace about the upcoming feeding team evaluation. The speech path that did her swallow study is the same speech path that is on that team. Hopefully since she has already met with us and knows us and Jillian's story, I am hopeful that she will be an advocate with us for what is safest for Jillian. I know that everyone that works with Jilli wants to do the best thing, I just feel like some people define what is best for Jillian differently then others.

Since the test... Saturday morning Jillian woke up coughing and needing a neb. Saturday afternoon she was doing better. Sunday morning she woke up with a temp of 99.4 and needing a neb. We did nebs every 4 hours Sunday. By Monday her temp was down and we did a neb in the morning and then she was good. Tuesday morning she started off ok and then part was into the morning her teacher came to me and said that she was really rattly and asked if she could do a neb with her. We then did nebs every 4 hours the rest of the day. Yesterday she was rattly too and did nebs all day. You can feel one spot in her lungs that has a definite rattle to it. She has had a green runny nose off and on too, but honestly this is not too bad for her having taken something by mouth. We did not have to put her on antibiotics or admit her to the hospital. We did only give her a little over 2ml though and she let a good amount of that roll back out of her mouth. I know it is crazy that such a little amount could do so much but for Jilli that is just how it is. Could you imagine what this week would have been like if she had more?

Monday Jillian had her 18 month check up. Her check ups mainly consist of me filling her dr in on everything going on and what is happening with each of the specialist. She goes over the basic things too. She looked at Jillian's bottom and where her pee comes out is fusing again. We are going to put the cream on it again. We talked about Jillian's speech and gross motor delays. She has referred us to an independent therapy place in Elkhorn. We have her speech eval set up for Monday and are working on a PT eval. Her doctor did not like the amount that speech and PT are coming from birth to 3. Now we are trying to figure out all of the insurance stuff for having her in b to 3 and a private company. I feel like a total jerk if I were to pull her from b to 3 after just having them come out and re-eval her.

Things for Team Jilli are in full swing. This week at work we started a penny drive for Team Jilli. It is so cool to see the kids get so excited about helping other kids! Our Team Jilli goal this year is $1,500. 

This Monday night our friends Brain and Lauren from collage joined our dinner with Jaime and Jason and Dan. It was a really good night catching up with old friends. We were able to meet Brian and Lauren's little boy Joshua. Joshua and  Jillian just kept staring at each other. Jilli did a good job sharing her toys with him. It was such an encouraging night for us and we were so blessed to spend time with friends.

Through this long week I have been so grateful for some amazing people in our lives. For Jaime, my Aunt Sandi and my mom for talking to me and helping to ease some of my nerves before Jillian's test. I am so grateful for these three amazing women in my life. They are such a blessing to us. I am thankful for all the people who have prayed for us. As my Aunt Sandi put it "you are the people who go to your knees for us while we fight the battles and when we feel to weak to keep going." Your prayers have really been felt this week.

Seth and MiKaley came over for dinner. Jillian liked playing with them

Grandpa reading Jilli a book

She is wearing the same clothes she did last summer. She is between 9mo and 12mo in clothes

Playing with Potato Heads. We have some of the coolest Potato Head parts from Disney World

She LOVE Gears

Working on walking

Curled up with a blanket

The post op shoe is off my foot! My toe is still sore but doing much better!

Jillian's Breakfast... acid reflux meds!

Helping daddy fix a problem at work

My rock star tubie girl!

I love those sun glasses! They only stay on for a minute or two at a time but they are so cute!

Reading Doc Mcstuffins with Uncle Dan

Getting comfy while taking a neb!