No Fever!

Kaitlyn's fever went away! We are pretty sure it was just from them changing out the tube. Here is a picture of her new tube. It's a much better setup and way easier to use. The surgeon will check it again in 2 weeks to see how things look.

We also tried out the backpack that comes with the feeding pump and used that during this afternoon when she wasn't eating her bottle. It worked great! It will be so nice to have the backpack when we are on the go. It is very discreet too, not the actual backpack but the fact that there is a feeding pump inside.

 The front pocket unzips so you can access the pump's screen to turn it on and change settings.

In the back of the backpack, the feeding bag hangs and the tubing comes out of a small hole in the bottom of the backpack. You can see the pump is in the front.

For those families who are dealing with NPA, just to give you a rough estimate of what it costs for a G-Tube and Nissen surgery laproscopically with a hospital stay just under a week. The hospital billed our insurance $54,193.22. So depending on what your deductible is and coinsurance, etc. For instance, we have a deductible and then pay 20% of the total cost up to a certain amount. This gives you a good idea of what to expect. Geez, I wonder how much the surgeon gets?

Looking forward to a great weekend together and more healing for Kaitlyn.

G-Tube #2

Today we went to see Dr. Schulman, Kaitlyn's surgeon, to see how her G-Tube and incisions are healing. He didn't like the way the tube looked. It was moving around more than it should so he took it out and put in a different tube and had us get an xray of Kaitlyn's stomach to make sure the new tube is placed correctly and there isn't any leaking.

Kaitlyn with Dr. Schulman and Daddy

Kaitlyn still isn't eating much by mouth, we aren't quite sure why. This afternoon it seemed as though she has had some pain from changing out the tube. She also started to get a fever. A nasty virus has been going around and we really hope that she doesn't have it. Viruses cause her platelet count to drop dramatically and could cause her to end up in the hospital. We are hoping it is just from switching out the tubes. If she still has a fever tomorrow I will be calling the surgeon and then the hemotologist to have her counts checked.

G-Tube Madness

Kaitlyn is recovering at home really well. Her appetite is definitely less than it was before surgery and it has been a challenge to get her to eat. We have been using the feeding tube at night with the pump but have been trying to keep her on bottles during the day for as long as she can tolerate them. It is really important to us to have things be as normal for her for as long as possible since her life will be so short. But since she has shown a decreased interest in her bottles during the day, we are probably going to start adding in some tube feedings during the day too.

This is what the tube looks like without the dressing holding it in place. In about a month or two she will just have a button or portal instead of a tube hanging from her belly.

Her personality and spirit is really great for having had such an invasive surgery recently. She isn't 100% back to her normal self but we are getting there! 

I think it has been harder on me emotionally with all of the changes that have had to be made since we've come back from the hospital. I literally walked into her room, opened her top dresser drawer and scooped all of her tops out, mainly onesies, and put them in a box of things that won't fit her anymore. All the cute outfits that she'd been wearing these past few months are in a flash gone and I'm staring at an empty drawer. We now have to shop in the little girls section instead of the toddler section since her belly is so big and the need for us to quickly access to the tube. She's 20 months old and wearing anywhere from 3T to size 6 tops!

With the IV pole and feeding machine in her room (which we hear all night long on the monitor- I think it sounds like an automatic stapler) and the quick disappearance of her clothes, it has made the reality of her disease even more real and difficult to handle. 

The set up in her room

Kangaroo Joey Feeding Pump

Chip is much stronger than I am and I give him total credit for keeping me sane (or somewhat sane) during all of this. 

Books with Gigi and Cole!

With all this being said, I truly feel blessed that she is doing so well and that we are all at home together again. We have also been overwhelmed with the generosity of our family, friends and people we don't even know who have reached out to us, donated to Kaitlyn's Fund and have been there for us during this time. 

A HUGE THANK YOU!

Adjusting to our new life at home

Leaving the hospital after Kaitlyn's G-Tube and Nissen surgery was a great feeling and a huge sense of relief.

Little did we realize, the craziness was only beginning! Kaitlyn had a lot of difficulty sleeping and during the next day, she would only take naps if you held her and was fussy the majority of the day. Pretty difficult when you also have a 7 week old baby to take care of too. She also didn't have much of an appetite so we had to do some tube feedings to make up for the food she wasn't taking by bottle. She does have moments where she is happy and wants to play but they are short lived.

Gave a few smiles for Valentine's day.

A home health nurse came to teach us how to use the feeding pump, which will be used overnight while Kaitlyn is asleep. It is pretty easy to use but the IV pole with the machine and hanging feeding bag is difficult to get used to in her room. Although her disease is real and we are aware of it all the time, seeing her room look more "medical" is very difficult emotionally.

Before we left the hospital, they changed her dressing around the G-Tube with a different type of adhesive to keep the tube in place and restrict the movement and pulling at the actual site on the skin. She will have these dressings changed every week for about 2 months or so and then a "button" will be put in where there is just a port and no hanging tube.

We are so glad to have Kaitlyn and Cole back together again. They are so cute when they interact with each other. Because Niemann-Pick disease is genetic, Cole had a 1 in 4 chance of having the disease as well. We are so blessed that he does not have the disease but is a carrier, like Chip and I are. It won't effect him until he is ready to start a family of his own. His wife will have to be tested to see if she is a carrier. Niemann-Pick disease is a double recessive genetic disorder, meaning that both parents have to be carriers and each child born would have a 1 in 4 chance of having it and a 50% chance of being a carrier. I will post more about that later in our resource section.

We are going home!

Got the great news that we get to go home from the hospital today! Kaitlyn is doing great, we are just continuing to manage her pain and making sure she keeps up her feedings. We are so excited to get back home!

A home health nurse is going to come out tomorrow to bring us a feeding pump for overnight feeds and show us how to use it. We were taught here at the hospital how to use the G-Tube for feedings where you push the food in the tube using a large syringe but not how to use and program the actual pump. We will also need to get all of the other equipment like drip bags, syringes, and coverings figured out. We are still going to feed her bottles as well since she can still tolerate them. This way we will transition her to just G-Tube feedings over time.

 

 Being silly.

We decorated her room. Here is her wall of cards.

Playing a lot more now.

Hospital Day 5

Kaitlyn is doing really well. I feel that we have her pain controlled better today than yesterday. She has had another IV line taken out so now we only have 1 out of 4 still in! She is looking better each day and being at the hospital hasn't seemed to cause any anxiety for her as it has in the past. The pain meds might be helping with that though, since they make her more relaxed.

She has been playing more and rolls to each of her sides without any obvious signs of discomfort. We are really surprised at how quickly she has wanted to do this.

It's funny, the green thing that is covering her G-tube is a Soothie pacifier! Our surgeon cut a hole in the top of the pacifier to run the tube through and secured it with a special tape. This allows for the tube to be held in place on the body so it can heal better and when the tube does move, it doesn't disturb the skin that is healing around the tube. The pacifier will come off before we leave the hospital and it will just be the long tube for about 2 months as everything heals. After that, they put a "button" in that is a port that you connect the tubing to and it is flat on the skin. That way there won't be a tube hanging from her all the time.

We were told today that as long as we know how to adequately use the G-tube when Kaitlyn needs it, we can still give her bottles and gradually transition to G-tube feeding when she truly needs it. Right now she is doing okay on the bottles since we have thickened them a little more than what she was getting before. This allows her to swallow easier. So all in all we will be doing a little of both once we get home.

Kaitlyn loves her Muppet! Chip had it made for her at the Muppet Workshop at FAO Schwartz in NYC last year. It has been one of the best distractions ever for when she is fussy. Her name is Princess.

Another of Kaitlyn's favorite things to do is to read books! We have been trying to make life at the hospital more like home and do more of the fun things we do at home, like reading together. This book was a gift from her Aunt Jess about rainbows. Kaitlyn wanted to grab and touch the ribbons on each page. A good sign that she is feeling more like her normal self.

It has been so thoughtful of our friends and family to send and bring Kaitlyn gifts while she's been in the hospital. We have been able to decorate her room with flowers, balloons, cards and stuffed animals. It makes it look more like a fun place to be for sure! Thank you!

Having some fun playtime together. She is getting more interactive- another great sign that she is feeling better!

She's been able to get some good rest, the pain medicine certainly helps with that! Now if only we can get some rest too.

Hospital Day 4 - Saturday

Every day is getting better and better for Kaitlyn. We were finally moved out of the PICU and into a regular hospital room at 11:30pm last night. A little late but we were happy to move. Managing Kaitlyn's pain has been a challenge. The medicine she is on can only be taken every 4 hours and around hour 3, it starts to wear off and she is NOT happy. We are trying to add Motrin in addition to her strong pain killer to see if it will avoid the break through pain. It is still difficult to pick Kaitlyn up without feeling like we are hurting her. When she lays flat in the bed she rolls side to side which is a good sign that it doesn't seem to hurt too much. Today, Kaitlyn had her first feeding through the G-tube. It was only a partial feeding to test how her body would handle it and then we bottle fed her until she was full. She did great! It didn't seem to bother her at all. It actually had the opposite effect the first time we tried it. We fed her 2 oz of Pediasure through the tube and she fell asleep! Tonight though, we are doing a drip feed, which is where a machine regulates how much food is given over a set amount of time. The drip feed she is on is for 1oz of Pediasure to be gradually fed through the G-tube every hour for 8 hours. This should also help her sleep longer or at least that is the hope, since she wouldn't wake up hungry. The nice thing about the G-tube too is that the nurse comes in to give Kaitlyn her medicine and is able to put it into the tube and not even wake Kaitlyn up! So nice since when Kaitlyn sleeps, we can sleep. We spoke with the surgeon today and he thinks that if Kaitlyn continues to do well, we might be able to go home Monday or Tuesday but of course it all depends on how she does. Chip and I are very tired and the day seems to go by so quickly since there is so much to do to take care of Kaitlyn and make sure she is comfortable. We do a lot of distracting with balloons and toys when she starts to get some pain. Other than managing her pain, we are learning a lot about how to use the G-tube. It seems fairly easy but it is still a lot to learn and adjust to.

We've had some visitors come see us, which has been really nice. Sometimes in the hospital you are so focused on your situation that you forget what day it is or what else is going on outside of the hospital. We even got to see Cole today, which was much needed, especially for me. It is hard to believe that he will be 7 weeks old tomorrow! I can't wait for Kaitlyn and Cole to be back together again at home. Hopefully it will be sooner rather than later.

 

Hanging out with Cole in the car since we didn't want him to be brought into the hospital with all the nasty germs there.

 

Sweet smile before he went back home with Gigi (Deanna's Mom).

Hospital Day 3

Kaitlyn is doing really well today. She was taken off of oxygen and her IV. She still has monitors for her heart rate, breathing rate and oxygen concentration. But the tubes and cords are slowly disappearing! As far as the G-tube, we have been told that the surgeon doesn't want to start feeding her through it yet but we can administer medication through it. They just want it to heal up a little better before we start the feeds. She was able to drink some Pedialyte this morning through her bottle and later today she was drinking Pediasure, which is awesome! We were worried that there might be some pain from the Nissen with drinking bottles but so far so good. Since she isn't sedated anymore and her pain medicine has been slightly decreased, she is very aware of what's going on around her and lets us know when she's in pain. When she is on the medicine, she is very content and plays with toys and is interested in her books. Today has definitely been busier for us as parents since she is awake more and needs more attention from us. We are able to hold her now and are able to rock her in a rocking chair which is really nice for both her and us. We have to be extremely careful when we pick her up and move her since her belly is so tender. Just changing her diaper is difficult since you can't just lift her up. She also has a lot of swelling from all the IV fluids and blood products that were given to her. I'm sure the swelling also adds another layer of discomfort. Otherwise, she is doing really well. We couldn't be happier with how things have gone so far. We are still in the PICU, only because the hospital is so full that they don't have any regular rooms for her to be transferred to. We still anticipate being at the hospital for about a week. She still has a lot of recovering to do and we have a lot of learning to do on how to use the G-tube. It's a whole new way of life for her and us.

 Watching a video and playing with toys!

 So happy to be able to hold her again

Much happier parents now that Kaitlyn is feeling a little better

Kaitlyn's first bottle since Tuesday night

 

The best sight of the day

Waking up

Kaitlyn has been breathing well on her own and they have stopped her sedation medication. She was agitated at first coming off of the medicine as I'm sure she didn't know where she was or why she was hurting. Once they gave her some pain medication, she was much more comfortable and she was even awake for about 10 minutes. It was a really great sign that she was interested in seeing and interacting with her books and balloons. We are able to give her clear liquids using her bottle so hopefully that will also give her some comfort. We will also be using the G-tube for feedings but for right now we want to do a little of both to make it less traumatic of a transition for her. Here are some pictures from today.

 Balloons from Daddy!

Reading a book, one of her favorite things to do.

 Awake but still a little groggy.

Us.

Hospital Day 2

 Kaitlyn did well overnight. She was on the ventilator the whole time and was heavily sedated. This morning they took the ventilator out and took her off sedation. She now just has a nasal cannula that is giving her some extra oxygen. She is still groggy but is doing well breathing on her own. They will monitor her for a while here in the PICU and hopefully if all is well, she will be transferred to a normal hospital room. We feel like we can now breathe a sigh of relief that the surgery went well and that she is off the ventilator. We know that once she is fully coherent, it will be difficult for her pain-wise and in understanding what is going on. We are so thankful for everyone who has left us messages and has been praying for Kaitlyn. It has meant so much to us and has made a difference. We are hopeful that her stay here will continue to go smoothly. She is definitely a trooper!

Recovering in the Pediatric Intensive Care Unit (PICU)

We are now with Kaitlyn in her room in the PICU. She is heavily sedated and on a ventilator to control her breathing. It seems like there are monitors and tubes everywhere. It was really upsetting seeing her for the first time connected to all the monitors but we are so glad that they did the surgery laproscopically and not a completely open procedure. We aren't able to pick her up or do anything other than talk to her, touch her and love on her. The nurse said that she can probably hear us but it's like she is in a dream state and not completely aware of what's going on, which is a good thing. She stirs occasionally and has fluttered her eyes a few times but is definitely not awake at all.   

You can see the small incisions where they inserted the laproscope and instruments. The green piece is where the G-Tube is.

Kaitlyn is out of surgery

The surgery is finally finished and went fairly well. It took about 4 hours as was expected. They were able to complete both the G-Tube and Nissen surgery laproscopically and didn't have to cut her open, which we are thrilled about! During the surgery they had to give her blood and platelets because her levels had started to drop. There was some bruising to the liver which may have caused some additional blood loss. They are putting her in the Pediatric ICU and are leaving the breathing tube in and are going to put her on a ventilator to manage her breathing. She will be sedated for awhile. She could be in the PICU and on the ventilator for 1-3 days, all depending on how well she is breathing on her own. Once they feel that she is breathing adequately on her own, they will take her off the ventilator and take the breathing tube out and see how she does. After that happens, it will take some time for her to regain her strength. She will probably be in the hospital for a week. We are so thankful that it went well but are obviously concerned about her being on a ventilator and her awareness of the situation. We haven't been able to see her yet and we know that it is going to be extremely difficult to see her on the ventilator. 

Please continue to pray for Kaitlyn and for us.

Kaitlyn is in surgery

Kaitlyn is officially in surgery right now. She did really well getting prepared for surgery. The staff brought her a special doll and a blanket for her which was a really nice unexpected touch. We got to spend some good time with her before surgery and got to take a bunch of pictures of her. We were also able to walk with her to the OR room and she was smiling the whole way. Here are some pictures of her from today.

The doll on the right and the blanket behind her were the ones given to her by the staff. It definitely helped to distract her to have new things to look at and play with. The teddy bear is very special to me (Deanna). My great grandmother gave it to me when I was born and I used to take this bear everywhere with me. I even have pictures of me out in the snow as a kid with it. I really wanted Kaitlyn to have it with her since it is from her great, great grandmother. I know her spirit is with her today.  

 Having fun with daddy and playing with her new doll.

 

Special time together. I love that she was so happy this morning. It meant so much to me.

Sharing a special moment with daddy just a few minutes before going to the OR. 

Persevere!

Surgery Tomorrow

Kaitlyn went to the doctor this morning and has gotten the okay for surgery tomorrow. Her ear infections are completely gone now and her congestion has cleared.

The surgery is scheduled to begin at 11am tomorrow. We were told that the surgery will take at least 4 hours if all things go smoothly. They are going to update us hourly on how things are going.

Please keep Kaitlyn in your thoughts and prayers. 

Thanks for the support

We are so thankful for all of the family and friends who have shown their support for Kaitlyn and us as a family. This has been the hardest thing we have ever had to deal with and so many of you have reached out to us. We have even had a few donations to Kaitlyn's Fund to help with our medical bills. Please know that we will be forever grateful for your kindness and generosity.  

Surgery next week

Kaitlyn's G-tube and Nissen surgery has been rescheduled for Wednesday, February 9th. It was a really good thing that we canceled her surgery for this week because we later found out that she has a double ear infection! She will be rechecked next week before surgery to make sure the infection is completely gone.