A lot has happened recently, I am going to catch you up on everything Tubie and hopefully later this week find time to catch you up on Tutu. In September, Tubie got more tubes. This time in her ears. She was seen in August with hearing loss and in September, ENT went in and found her ears pretty plugged up. After cleaning out her ears and putting in tubes, she had almost perfect hearing!!!
About 3 weeks ago, Tubie started crying almost anytime her tube was touched. After a quick call to her complex care pediatrician, I took her to urgent care.
The doctor at urgent care was very unhelpful. I don’t think it helped that we had to sit there for over 3 hours and Tubie was falling asleep. Luckily we had a GI appointment that week. Her GI doctor thought she had an ulcer in the stoma (gtube track) so we started treating it with medication to help. A week later and there was no improvement. We changed her to a longer button. And gave it another week.
After a week with a new tube size and treating her 2-5 times a day, she was still in pain. We went back and made the decision to do a scope. While waiting for the scope we had some fun with Tutu and went on a field trip. Where they had a handicap accessible wagon and Tubie got to enjoy herself.
Bright and early the next morning(ok it was just early, the sun definitely was not up) we were at the hospital for her scope.
The doctor came out after she was done and she has a bad ulcer in the stoma track. It is full of puss and very red. The doctor does not know why the medication we had been using hadn’t worked yet.
Being the amazing doctor he is, he had us continue doing what we were doing to try and help some while he worked on a better plan. It has been a week and he has been in touch every weekday trying to figure out the best course of action. Not wanting to do something that could cause more harm then good. In the meantime, we switched Tubie’s button type from a Mic-key to a Mini. This button has a different ballon shape, is lighter and has a lower profile. While it won’t help with the current ulcer, I think overall it will be a better fit for her.
Today I realized how much all of this sucks. Tubie is in a lot of pain. And it makes me want to cry and the worst part is the only way we have at this moment to try and make it even a little better is to hold her down(Tutu is way better at helping then any 4 year old should be) or basically tie her down(I don’t always have help) in order to do what is best. I know while it hurt she is more pissed off she can’t stop me from holding her down to do it. I know that even if she doesn’t actually understand, I am doing what is best. But I also know that sometimes this life just sucks.
I am going to end this blog on a positive note though. Look at Tubie sitting in the shopping cart. 💓💜💓💜 She was so excited she kept shaking her head so getting a good picture was impossible.
This week I needed to go into Hobby Lobby for one thing. It should have been fairly easy. Tubie was hot so I chose the GoTo seat over wearing her. Since I just needed one thing and it was too big for her stroller basket, we needed to use a cart. I love craft store shopping and have nothing against the store. I love my GoTo seat because it allows Tubie to shop with us. However due to the size of the carts, it was a total failure. By the time we left, I was stressed, Tubie was crying and Tutu was upset because I was just trying to get her moving through the parking lot. There is something everyone can do to try and help. First, we are blessed to have a GoTo Seat. It is not possible for all families. Second, sometimes as special needs moms, we just need to get a few things and don’t want to take equipment out to get it. So if you can take a copy of this pamphlet for the GoTo Shop and share it with all your stores. Let them know how much it will help families like ours. The Caroline Carts are amazing but designed for bigger children. The age requirement is 48 months and I am not even sure Tutu would fit at that age. So if you can please share with all your stores and also with all your friends. The more companies see the need. The more likely it will happen!!
What she looked like by the end of our shopping trip.
This week Tutu started preschool. This in and of itself is a huge milestone. But it was very bittersweet for me. While I am extremely proud of her, this was never apart of our plan.
Before we started planning our family, really before we were even engaged, we planned to homeschool our children. I wanted them at home with me. I did so much research and was so excited for homeschooling. Then Tubie came along and time to homeschool wasn’t there. I wasn’t getting one on one time with Tutu who was craving learning more things and with Tubie’s therapy and appointments putting Tutu in school only made sense. Once again our plan has shifted(you seeing my new life motto yet) and things have definitely change. Tutu is loving school and her teachers truly care about her. We currently have a plan for the future as far as schooling goes but let’s face it, nothing ever goes as planed.
When I got pregnant with Tubie, there was no question as to how she would be fed. Tutu was still nursing and continued to nurse up until a few hours before Tubie was born. When she was born and I woke up, we tried nursing right away. And at first she did really well.
When she was transferred to the NICU she struggled to latch. We both were struggling. My milk was coming in and she was pretty tired from all the testing that had been going on. We were trying to avoid nipple confusion and put in an NG Tube to see it some food in her belly would help her relax a bit. And it helped some. She finally nursed after 12 hours of refusing.
Then she stopped. There was no LC available on the weekend so after talking with the nurse, her doctor and my personal IBCLC, we decided to try a bottle. We mostly wanted to see if she would suck because at that point she was refusing my breast and a pacifier(given during transport to the hospital). She took the bottle great. We knew she could suck. So I started pumping like crazy and we started bottle feeding her.
The rest of her NICU stay was bottle feeding, pumping, her direct nursing two times a day and Tutu coming up and nursing once a day. My milk came in pretty fast so I was providing all of her nutrition.
After we got home we went to just nursing her but she started to lose weight again. We tried a few things starting with bottle feeding and at one point trying to fortify my milk with formula(my doctor tried to get us human milk fortifier but it could not be found). She threw up after any feed with any formula. We went back to just breastmilk bottles and she slowly gained some weight. Eventually she was clear for just nursing. We nursed for about 6 weeks when she stopped gaining again. This time however she refused a bottle. An Early Intervention feeding therapist came to the house and we couldn’t get her to take it. At that point she was well hydrated so we just kept nursing.
When she was 3 months old, she started having issues staying latched and that was when she screamed to even nurse. And when she did nurse it was enough to stop the hunger pains. We were admitted through the ER and after 9 days in the hospital she got an NG tube. We were discharged with the NG tube. She was getting 3oz of breastmilk through her tube every 3 hours around the clock. I wasn’t sleeping at all. We eventually switched to continuous feeds overnight so I could sleep some.
At some point during this time, Tutu stopped nursing. I don’t remember exactly when, I just remember, one day her not nursing anymore. She was around 2.5 years old.
She got her gtube at 5 months old when it became obvious that she was thriving with a feeding tube. She gained weight and was holding her head up better. Not having to work for her nutrition was what she needed. In addition the NG tube created an oral aversion so she refused to eat.
After her heart surgery, her dietitian and I sat down and had a talk. I was physically and mentally exhausted. With breastmilk in her feeds overnight, I had to get up every two hours to get more milk because it couldn’t sit out that long. I couldn’t pump enough in one session to make it safe to be out all night so we made the decision to use formula at night as long as we could find one that worked for her. We went straight to the most broken down formula. She kept it down but she was in horrible gas pain. So we backed off for a bit. Then the dietitian said she had luck with a different brand. Tubie did well on it. So we went to breastmilk all day and formula at night. She was still getting about 60% of her nutrition from breastmilk but my mental health improved so much and I was able to better care for her.
Sometime in May I realized we had enough breastmilk to get her to a year and I slowly stopped pumping.
After her first birthday, Tubie started a blended diet. That is just real food through her feeding tube. Currently we use a pouch of Real Food Blends and add some extras to give her more carbs.
I currently make 2 days worth of food at a time. To the pouches I add 1 cup of old fashioned oats, 1/2 cup of my homemade Greek Yogurt, 24 oz of whey(this is what strains off the yogurt and is full of carbs), 2 vitamins and 1/4 cup of honey. I do change things up sometimes using a banana instead of honey and almond milk instead of whey. To the last feed of the night she also gets 1 tablespoon of cornstarch to helps with her carbs over night. She get between 750 and 800 calories a day.
If Tubie gets too many calories she can become overweight easy. Because of her Noonan Syndrome she will be short stature and isn’t mobile yet. So we limit her to a healthy amount of calories that does not risk her weight. But we discovered after the growth hormones started she wanted more volume. So we supplement her volume with pedialyte. This keeps her weight healthy (although she is under weight for her age her weight to height is perfect) and full. We are working on getting her to eat orally.
While feeding Tubie definitely didn’t go as planned, it is what I know now. It has become a part of my daily life and it is a lot easier then I ever imagined.
*Disclaimer: This post is going to be long. Please stick with me to the end!*
When I got pregnant with Tubie, we were a bit surprised. It had taken us two years plus fertility treatments to get pregnant with Tutu so when the word “Pregnant” pop up on the test, two months after we started trying again, we were shocked.
My pregnancy with Tubie was pretty uneventful. Well as uneventful as a summer pregnancy with a still nursing toddler can be, so hot and exhausting. We had one ultrasound that had soft markers for some genetic issues but both were unlikely due to all the other information we had and she doesn’t have either condition. On the night of August 2, 2016, I started having contractions. Things started happening fast and scary. I ended up with a stat csection under general anesthesia with my husband out of town (I will share my entire birth story one day but it is long). Tubie was born at 1:18am on August 3rd and I met her when I woke up a few hours later.
Her first 30 hours of life we thought we had a healthy baby. But when I went to take a shower and the nurse was taking her down to the nursery, she turned blue. During an exam immediately after getting her breathing again, they found a heart murmur. Tubie was transfer to the Special Care Nursery at the hospital. Tutu was able to come in and meet her.
After a few hours and some conversations with the pediatrician we decided to transfer Tubie to the closest children’s hospital. She had been rolling her eyes which made the doctor concerned about seizures, the heart issues and some other physical issues. She left the hospital without us (I couldn’t drive or ride in the ambulance due to surgery) and we followed after I was discharged about an hour later.
In the NICU she had a ton of tests run. We found out she was not having seizures but she did have 3 heart defects. A bicuspid aortic valve, pulmonary valve stenosis and a tiny hole in her heart. None of these were life threatening and the plan was to just follow up. Genetics came up to visit and thought she had something called Noonan Syndrome. That was the first time I heard the name of a condition that would change my life. Blood work was sent off and we were told it would be 3-6 weeks before we heard anything.
After 9 days in the NICU, Tubie was discharged. She was taking a bottle, finally gaining weight and her billi levels were in the normal range. We had a bunch of follow ups scheduled and a pending genetics test.
Through all of this, I have to say that Tutu was probably the strongest. She was still nursing when I went into labor and was suddenly without me for 10 days except for visits. She was a good as a 2 year old with her entire life turned upside could be, including being shuffled around a bit.
We got home and Tubie started nursing but we had to start giving some fortified bottles because she just wouldn’t gain and even lost some weight. The problem was every formula we tried using for her to add calories to my milk, she threw up. Her pediatrician and I decided to just have her nurse and to set up an appointment with GI.
Her first GI appointment was September 16, 2016. I remember this because it was also the day we got a phone call with confirmation that she had tested positive for Noonan Syndrome with a NF1 mutation. I promise that I will do a post on what all her conditions are in more detail but I want to keep this post as short as possible.
Over the next 7 weeks we did what we could to get Tubie to grow and thrive. Doctors appointments for adjustments and weight checks which all helped her for a bit but not for more then a week. One day I realized she was barely nursing and when she did nurse it was a fight. I decided to take her to children’s. After an entire afternoon in the emergency department we were admitted. It was a Friday night and if you or you child have ever been in the hospital, you will know, nothing happens on the weekend that is not an emergency.
On Monday, an echo was done and pulmonary valve stenosis had gotten worse. A condition we had been told that may need intervention at some point needed intervention ASAP. She was scheduled into the cath lab on Thursday morning. We were hoping that getting her heart working properly would cause her to eat better. On Thursday the cath was done but unfortunately failed. We found out she would need open heart surgery in the coming months.
We decided to let her rest for the night and we would put in an NG tube the next day. She needed nutrition and to gain weight before heart surgery.
By the time we got the NG tube in, it was too late for training through the hospital so we had to wait until Monday afternoon. Sunday night she spiked a fever. She tested positive for rhinovirus but was still cleared to go home the next day. As the doctors were preparing her discharge papers, she started choking and breathing funny. I ran to get the nurse but ran into her doctor. They decided we were not going home that day. She later tested positive for Group B Strep. We are still unsure if this was late onset from me having it in pregnancy(she was tested at birth) or from the cath lab. She had to stay for 10 more days to receive IV antibiotics. During her time being on IV antibiotics and feeling horrible, she completely stopped nursing. After 3 weeks she finally came home for her first Thanksgiving.
The next 8 weeks were filled with doctors appointments and the decision to change Tubie to a gtube. We celebrated Tubie’s first Christmas at home and right after the New Year she was admitted to receive her peg tube.
The next few months were pretty quiet. Tubie was admitted once for what turned out to be a UTI. And we mostly spent our days at home trying to keep everyone healthy.
On March 16, 2017, Tubie went in for open heart surgery. The plan was to try and repair her pulmonary valve and repair the hole. The hole was expected to close on its own at some point but if they were in there it made sense to fix it. It turns out that the hole had already closed by itself. Her pulmonary valve was not repairable so she currently is living without a valve and will have surgery for a new one when she is older. After just a week in the hospital, she came home for daddy’s birthday.
Honestly most of the remainder of the year plus was uneventful. Tubie was admitted a couple times due to stomach viruses(which she can not handle at all ) and started physical therapy and occupational/feeding therapy. We tried and failed to potty train a very stubborn Tutu. Tutu turned 3 and Tubie turned 1. Tubie refused to eat the cake. We went to my mom’s house on vacation. And both girls grew and thrived. Tubie learned to roll from belly to back at 9 months and after being put on some maintenance pain meds (chronic pain is common in Noonan’s Syndrome) she learn to roll from back to belly and sit up in the same week at 15 months.
In January 2018, Tubie had a sleep study trying to figure out why she never slept. Turns out there is no medical reason and she just needs some help in that department. Also common with kids who are complex.
Moving on into 2018, we started patching Tubie’ s eye because it was having issues turning out and eventually put her in glasses. Tutu got her first hair cut and I survived life on coffee. We also celebrated Tubie’s 1 year heartaversy.
She continued to be admitted each time she got a stomach virus. We started noticing that while sick her sugar was low. In May 2018, we went to the endocrinologist. We decided to check her sugar when healthy. The night of her appointment she threw up a couple times but it was more not tolerating feeds then illness. She does not do well with heat at all and her room was a very stuffy 80 degrees that night. The next morning her sugar was low and we were sent to the hospital to be admitted. After a few days they were ready to send us home but I asked to stay one more night. The next morning her sugar was 33. A battery of tests revealed she does not make growth hormone on her own and this was causing super low blood sugar. And she doesn’t show signs when low. She had an MRI(this will be important in a bit) and was started on growth hormones. That stay was one of our hardest. When I was at the hospital with Olive, my mom was at the hospital with my grandmother, 6 hours away. It turns out my grandmother was sicker then any of us realized and she passed away before we could get down there to say good bye.
Tutu turned 4 and also got glasses in June and Tubie was admitted 2 more times with stomach bugs and low blood sugar in June and July. We went to my mom’s house for a much needed 2 week vacation and came back just before Tubie’s second birthday.
The week after Tubie turned 2, we had a ton of appointments. She had and failed a hearing test. We went to ENT and she goes in for surgery in September to see if tubes will help her hear better or if she needs hearing aids. We also got the news that a mass seen on her MRI in May had grown. It was previously was thought to have been a lipoma but now appears to be a teratoma. The only plan for it right now is to follow it. She will have another MRI in November.
This week Tubie learned to put herself into a sitting position which is a huge step forward.
A little over two years ago I knew a whole lot less about a lot of things. I was waiting for my second daughter to join us in this world and was expecting everything to be very similar to her sister. I had dreams of two toddlers playing and learning together. My dreams have shifted and I now have new ones and also live with a new view of the world. And honestly a whole new world. Because until you become the parent of a medically complex child, you do not realize there is an entirely different world out there where kids are sick and so much braver then adults. Where parents live off bad coffee and hospital food and sleep in chairs. This is my world now and I am going to try and show you a small part of it.
Please feel free to ask questions if anything seems unclear. And if you made it this far, welcome to my blog!