But truth of the matter is, Ridge has many problems, and we are finally on track to getting him fixed, hopefully.
Let me start at the beginning...
Over the past several months, Ridge had been fed via G-tube at 28 k/cal (normal is 20) per ml, getting 35 ml/hour for continuous feeds 24/7. He has struggled to gain weight, even with the increased k/cals. We had been working with GI and dietitians to try to get weight on him. In the meantime, we also had a test done and found a new recurring fistula has appeared. Because of this, he needed more surgeries. More surgeries led to more anesthesia, more anesthesia led to more lung problems than he already had. During this time, using a hospital grade suction machine, I was pulling up white, frothy, bubbly stuff. We did not know if this was coming from his lungs or via reflux from his stomach. This became a back and forth battle of what it was and who could fix it.
So, that being said, March 29 he had his first attempt to close the fistula. They cauterized in the pouch and where the hole area was. We had to stay overnight as he required oxygen afterwards. We were sent home the next day. The symptoms continued with no true improvement. To us, he actually seemed worse. We went in for another test to see if the hole closed. Unfortunately, it was more open than before, which meant another surgery.
We had the second attempt done April 19. We had planned to stay this time, and we ended up staying two nights. Our surgeon pulled pulmonary in to get a game plan. This didn't get us very far at the time (we didn't get our normal pulmonary doc) so our surgeon made the decision to put a TPT tube (transpyloric tube) into his button down into his intestines. This would prevent the reflux from happening so much, and allow the hole to close without being irritated by reflux and stomach acids, as well as help heal his lungs. They were beginning to sound more and more coarse. Three weeks passed, another test was done. HOLE. IS. STILL. THERE!! The hole wasn't any bigger, but it also wasn't any smaller. Our frustrations began to settle in. The surgeons discussed heavily of whether we try the cauterization procedure a third time, or if we do a full thoracotomy. For those that don't know, the cauterization is less invasive and they perform this via a bronchoscopy. A thoracotomy is going in through his side, through the rib cage, collapsing a lung, and doing what they needed to this way. Ridge's lungs were in no condition for this surgery. Also, because he has tracheomalacia so bad, this procedure can just make it worse, making our respiratory issues even worse than where we currently stood. In the meantime, he has been getting fed via TPT for a month at this point, keeping reflux down and hopefully brining on some weight. Unfortunately, he was going the wrong direction and losing weight. This was becoming another problem to figure out.
Wednesday, May 16, we came down for appointments with Pulmonary and GI and at that point decided we needed to figure out:
- Why his lungs were having issues and get the documentation we needed for home oxygen.
- Why he isn't gaining weight.
- What in the world is this white, frothy, bubbly stuff and where is it coming from!!!
This is what I told them I wanted figured out while we were here this time. We knew we were having surgery May 17, so GI and Pulmonary teamed with our surgeon and came up with a plan. The plan was, have the surgery Thursday morning, stay and monitor oxygen, put on TPN and Lipids to clear the GI tract and determine where the stuff was coming from, see if he could gain, and give his lungs some support, and by Monday, go home with oxygen and potentially TPN/Lipids.
During surgery, the surgeon cauterized the hole and in the pouch, and also used glue to hopefully help hold it. However, the surgeon also saw this white substance I have been talking about and it came up from his lungs into his bronchial tubes and trachea during surgery. Ah ha! We had an answer before it even started! The junk is coming from the lungs. Unfortunately, that is a problem. Pneumonia it is! After surgery he did well, but as usual, required O2. We got settled in our room, talked to pulmonary and GI, and the ball was rolling to get the O2 and hopefully go home Saturday or Monday. Friday morning the PICC line was placed for the TPN and Lipids feeds. By Saturday, he had fever and was going downhill and fast.
From Saturday to Monday his fever only came down with Ibuprofen or Tylenol and we were giving it around the clock every 3 hours. He was restless, breathing fast, and struggling. Monday, he tested positive for a virus. So now we have a virus and pneumonia to fight, all while trying to heal the hole. This is slowly becoming a perfect storm for not healing. Pneumonia was settled in deep in both lungs, and by Monday night his fever was not coming down with the medicines. Respirations were in the 80s and his O2 level jumped from needing 6 L to needing 10 in just a couple hours. Our sweet nurse called the MET team and after coming in twice to observe, decided once we hit 10 L and over 102 fever we needed to transfer to the IMU (Intermediate Care Unit) for closer observation. [Disclaimer: Ridge loves his girlfriends in 3D - Surgery Unit.] Over the next two days, his O2 levels increased to 12 L. At this point, his stomach is bulging from so much air. We removed the TPT tube placed after the last surgery and hooked him to a vent bag to help relieve some pain and discomfort. Over the next several days, O2 was weened very slowly until we got down to 6 L. We were in IMU from May 21 - May 29. Finally, we were transferring out of IMU to ITU (Infant Toddler Unit...but we love our IMU team) and getting a bigger room and hopefully would be recovering soon. He was getting over the pneumonia and virus and starting to feel more lively and like my Ridge.
Days passed and it didn't seem like Ridge was tolerating weening from the flow very well. He wasn't needing the FiO2 (oxygen) so much as he was needing the flow (liters per minute). The thoughts are, his tracheomalacia is causing him to need the flow to keep his trachea from collapsing. Hmm..what to do about that? I can't sit here on high flow until he gets bigger and grows out of his issue, because 1. remember, we aren't growing, and 2. some kids don't outgrow it so that would be a lot of time to risk the fact he may never outgrow this.
In the meantime, we are on TPN and Lipids and barely growing. I mean, 0.02 kg some days. Which is not much. Like an ounce. With the amount of sugar and fats, this isn't making sense. We are in the talks of having genetic testing to see if there are other reasons why this is, as well as continuously playing with his numbers to get us better results.
Last week, I decided I would finally raise the question: Do we need to get a second opinion and go to Boston. When one physician says, "If this were my son, I absolutely would" you jump on that option. We started working with insurance, requests, calling Boston, etc. Our case manager started the process and got everyone rolling. Why do we want to go to Boston you might ask? They have the best pediatric clinic for kids like Ridge with EA/TEF. If you're curious of Ridge's conditions or what we are going for, go check out their website (http://www.childrenshospital.org/centers-and-services/programs/a-_-e/esophageal-and-airway-treatment-program). It will give you some insight on what we are needing and what we are hopeful for! Dr. Jennings is a worldwide known surgeon that has developed life-changing procedures for kids like my Ridge. Did you read that right? LIFE-CHANGING! WE NEED TO GET TO BOSTON! The plan is for Dr. Jennings to check for any possible holes and to see if the surgery from 3 weeks ago worked. He will then perform a posterior tracheopexy at the same time, hopefully. Long story short, this will tack his trachea open by attaching it to his spine. This surgery could potentially lead him to eating soon, breathing better, and living a relatively normal (for him) life, and hopefully tubeless.
SO, our case manager reached out to Boston for us, and she said Dr. Jennings is going to take on Ridge's case and he can help us. Did you hear that? Those words have never sounded so sweet. Someone can fix my boy. The day I got the call from our case worker and she told me that, the tears flowed. I couldn't quit thinking about his life afterwards, seeing him run and play, seeing him eat, seeing him be a typical little boy with his big brother and sisters. I couldn't stop crying. I called and told mom, and we cried all over again. It has been an emotional week.
While we wait to go, we are still trying to work on Ridge's weight and respiratory state. He isn't gaining significantly on the TPN and Lipids and we aren't sure why. We are continuing to go up on numbers, and hopefully we will see more than just 0.02 kg in weight gain. It can be frustrating at times, but we are hopeful. Also, his gallbladder and liver numbers were out of whack in his labs, so an ultrasound was done, but praise the Lord it came back what they expected: its sluggish because he is only getting TPN/Lipids and there is nothing else wrong! Answered prayers there. We are on room level oxygen (21%) but he is staying between 3 and 4 L of flow to help with his trachea and the floppiness. Because of his gallbladder and liver and their condition, we are currently waiting to go to IR to get a new TPT line placed into his button down into his intestines. They are going to try to just push small amounts into his intestines a couple times a day to get his liver and gallbladder functioning correctly again. So while we wait for Boston, we will work on these improvements.
Phew. That was a lot. And I am sure I have forgotten some things. After 21 days locked in a room, he was set free to roam the halls. He has taken on his role as hall greeter very well. He gets called a pretty or beautiful girl very frequently, and we take it in stride and laugh, because 1. he is carrying a dinosaur blanket, wearing blue, and has a blue Paci with a monster Paci clip...and 2. we know he's beautiful.
As I wrap up this post, I just got the news that our insurance has approved the transfer and care in Boston! I can't quit shaking. I am so excited, we finally can get the help he needs! Now we just wait for a bed in Boston and wait for a flight!
Be praying for us as we make this transfer. As we've already been away from the other three for so long and their worlds have been turned upside down, we hate to leave them again. However, we are hopeful for the first time in a while that once we get back we will have a relatively normal life together as a complete family back together again, and that Ridge will be the boy we've prayed for him to be one day, and be completely healed. Also be praying for us that our insurance will provide the coverage we need as this is going to be a pricy transfer and visit and the bills will most likely add up. However, no price tag is going to be too big for my son to get a better life. We thank you all for continued love, prayers and support. We couldn't do it without our family and our village. We have people praying all over the country, and for that we are thankful.
On that note, I think I'll go to Boston...
