Getting Back to Normal

So we did end up flying home late night on Tuesday, September 8th. The go no-go decision was not clear cut. The x-rays of Lucas’ lungs we good enough for us to leave, but his oxygen (O2) sats were pretty low (about 92). The concern was that since high altitude in the plane (cabin pressure is about 8000 ft) lowers everyone’s oxygen even more, that Lucas might have a major issue if his O2 dipped into the 80s. In the end, we got a not-to-reassuring “I think you’ll be fine” from the doctor, and the ultimate decision was ours.

We decided to make the trip, primarily because at home we had access to Lucas’ airway clearance vest and nebulizer – two items we knew he needed, but we were having a difficult time locating as out-patient in Boston on a holiday weekend.

The flight started out OK. Lucas actually fell asleep as Jill and I monitored for grey-blue color and bluish fingertips. I watched the moving map on the JetBlue monitor in front of us making mental notes of the nearest children’s hospital we were flying over. The biggest issue of the flight came when Lucas woke up from reflux with uncontrolled coughing. At one point, Lucas gave me his terrified look. It’s the look in his eyes he gets when he can’t breathe, and scares me like I can’t explain. Normally, we only see that look after a surgery and an occasional post-surgery night terror. I remember telling Jill that the flight might be one of the dumbest decisions we had ever made, and I can attest to you that at that moment, I almost made them land the plane in Chicago!

Instead, we got a quick answer to our prayers and Lucas was able to calm down just enough to take some breathing meds. The rest of the flight had a couple more scary spells, and lots more coughing, but nothing like that first one. I continued to watch the map the entire flight and was so happy to finally start our descent into Phoenix. Ironically, when our plane pulled into the gate, the flight attendant asked everyone to remain seated because an emergency medical crew would be boarding the plane. Gigi asked me if someone had called for Lucas. They hadn’t, and we were very glad to be headed home and not straight to the Phoenix Children’s emergency room.

Lucas was so excited to get home. He immediately went for his toys and starting playing. Over the last week, the CPT vest and hypertonic saline have been a crucial part of Lucas’ recovery, and in the last few days, Lucas is eating again, starting to feel better, and getting back to normal. I’m still not sure if we made the right decision to fly home, but it sure is good to be here. We even went swimming for a few minutes today.

Below is a picture of Lucas from June while he was at our Nanny’s house. Does he have the best smile or what! Love, love, love this photo of our little miracle boy!


Lucas Has The Best Smile Ever

Flying Home (maybe)

Respiratory distress in a kid like Lucas is pretty complicated. The last few days have been a roller coaster, but generally speaking, Lucas is doing a bit worse instead of better. It’s the opposite of what we were hoping and expecting. Now, the question is, is Lucas well enough to get on a plane and fly home, or put another way, can we be away from emergency access to a hospital for about 10 hours?

We are speaking with the doctors in a few minutes to talk about this very question. Lucas’ O2 sats are still much lower than normal. The good news is that whenever we do get home, we’ll have access to his nebulizer and CPT vest – two things we could really use right now.

Still resting comfortably

Lucas is doing well post-op. He is still resting comfortably in mom’s arms and hasn’t yet needed any narcotics or even oxygen. This is pretty rare for him. If his pain is under control when he wakes up, and he is willing to drink some fluids, we may not even have to spend the night in the hospital.





Another Successful Dilation

Dr. Manfredi was “very pleased” today with how Lucas’ esophagus looked. It’s always totally awesome when Dr. Manfredi uses the phrase “very pleased.” Today was no exception.

Lucas’ top stricture looked “a lot better than last time.” He said that he almost decided to just leave it alone, but then went ahead and did some work on the  shelf of scar tissue that we’ve been battling for a while now anyway. However, he didn’t need to do as much work as in May.

The lower stricture still looks great, and again, didn’t need any work at all. So awesome!

The best news is that Dr. Manfredi thinks we might be able to go all the way until January before doing a contrast study locally in Phoenix! In other words, this very well could be Lucas’ longest break between surgeries ever! It will also mark the first time in his life that his age in months will be greater than his number of surgeries. A silly milestone, I know, but one I’ve been looking forward to for a long time.

Today is another huge bunch of answered prayers for our little miracle. So thankful that Lucas keeps defying the odds.

EA/TEF – An Overview

Esophageal Atresia and Tracheoesophageal Fistula. Today those words just roll off my tongue, but two and a half years ago, I had never heard of it. Jill and I found out at our 20-week ultrasound that Lucas “lacked a stomach bubble,” and that fact combined with Jill’s high fluid level meant that Esophageal Atresia was suspected. Ultrasounds are really good at seeing fluid – amniotic fluid and blood. They are really bad at seeing just about everything else. You can see the outline of the baby on ultrasound because the baby is floating in fluid. You can see the heart well on ultrasound because of the blood flow. On ultrasound, you can see the stomach fill and empty with fluid as the baby swallows, unless of course, your baby can’t swallow.

The below video is a good overview of Lucas’ original and longest running battle. It shows the exact type of EA/TEF Lucas was born with. The surgery they describe in this video was performed when our tiny pre-mature Lucas was just 26 hours old. What a fighter and little miracle he has always been!

Prayers Answered Again

It’s hard to describe the emotions when you have a day like today. If I think at all about how things went, tears quickly fill up my eyes. Tears of joy.

Lucas was a little trooper yesterday not being able to eat the entire day. We gave him Senna Syrup twice to produce bowel movements and an enema in the evening. We didn’t get to bed until after 1 am, and then we had to wake at 4 am for another enema and our eventual arrival at the hospital by 5:30 am. For what its worth, waking up at 4 am to give an enema to a 16 month old will not make my “Best Ways to Start the Day” list any time soon.

The rest of the day, however, could not have gone any better. As Jill mentioned in a previous post, there were really three procedures today:

  1. An esophageal dilation
  2. A colonoscopy
  3. Lucas’ second Fecal Microbiota Transplant

Dr. Manfredi was “really pleased” with how Lucas’ esophagus looked. The top stricture, at the anastomosis site, is still wide open and looking great. He didn’t do any work at that site today. The bottom congenital stricture is also healing up really nicely after the extensive work done last week. The even better news was that Dr. Manfredi was able to pass a 12 mm  balloon back-and-forth through the stricture without resistance. This was awesome news. It is exactly where we want his lower esophagus to be. He also cleared us to eat food again, which we are sure Lucas will be very excited about. The only thing Dr. Manfredi did at the lower site today was inject some steroids to promote healing – no dilatation. He will look at the congenital stricture again in a week or two to see how it continues to heal. My impression from Dr. Manfredi was that it looks so great, that if we can get this to heal well now, that we will be able to go a long time before our next dilation.

The biggest news of the day, however, was the colonoscopy results. Both Dr. Russell and Dr. Manfredi said that Lucas’ colon looked great. They were also able to drive up into the ileum, the final part of the small intestine, and that also looked great. They did take biopsies, but visually, there was no sign of Crohn’s Disease. This awesome news was so shocking to me, that it almost seems unreal to type. Lucas’ symptoms matched Crohn’s so well, but God has a different plan and keeps teaching us to expect miracles from the power of your prayers.

Lucas’ also had his second Fecal Microbiota Transplant (FMT) today. You may recall that a few months ago, Lucas became the first child at Boston Children’s Hospital, and one of (if not the) youngest children to ever have an FMT. Unfortunately, and despite the incredibly high success rates for FMTs, it did not succeed. Today, we did a few things differently. The biggest change was that today we used stool from the “poop bank” provided by OpenBiome. Dr. Russell’s early Crohn’s research suggests that using stool from an unrelated donor might actually yield better results. For that reason, and a few other reasons, we made the difficult decision to go with the bank this time. Like any donor product, such as blood, it is not without risk, but we felt the benefits outweighed those risks. Also, since this FMT was administered via colonoscopy instead of NJ-tube, we gave Lucas some Imodium AD and had him lie in a head down position for a while in an attempt to keep as much of the transplant intact for as long as possible.

Our mega-prayer now is that this second FMT will be completely successful, clear Lucas once-and-for-all of C. Diff, and that with C. Diff gone Lucas will make quick big strides against our two biggest remaining hurdles: rickets and immune deficiency. Yes, it’s possible. God still makes miracles happen every day.

With God, all things are possible.  -Matthew 19:26

Sweet Home Arizona

576 days ago the date was Tuesday, February 5th, 2013. Jill was 20 weeks pregnant. We didn’t know yet if we were going to have a boy or a girl, but shortly after our 10 am appointment we expected to get the answer. It was the day of our 20 week ultrasound – the anatomy ultrasound. We found out we were having a boy and were overjoyed, but we also found out that not everything was quite right. It was a day of mixed emotions. Three ultrasounds over the ensuing three days would confirm the fears, our tiny baby boy’s tiny esophagus was not connected to his stomach. 

Indeed, for the last 576 days, we’ve been told that Lucas’ esophagus isn’t quite right. That changed today. 

Today’s surgery started out much like any other. We said a prayer. I carried Lucas back in my arms into the operating room. Lucas cried, aware enough to know what would happen next. The anesthesiologist injected a small amount of medicine into Lucas’ PICC line and within a few seconds Lucas’ entire body was limp. I gently laid him on the operating table, gave him a kiss, and left the room. I joined Jill in the hallway and we walked to the waiting room. I called my parents and Jill went to get us food. This is our routine. We’ve done it 19 times. 

Except there would be nothing routine about today.

Within a few seconds of me getting off the phone with my parents, and before I had the chance to eat the sandwich that Jill had just returned with, Dr. Manfredi walked in the waiting room. He had a grin on his face that Jill would later describe as a smirk. He was pleased. Very pleased.

He said, “Lucas’ esophagus looks good. I didn’t even dilate.” We were speechless. It was completely unexpected news. The doctor continued forward explaining how great things looked and showed us pictures and fluoroscopic images in which you’d be hard pressed to see there was ever a distal stricture. Then Dr Manfredi shocked us again by saying that Lucas wouldn’t need another dilation for 3-4 weeks. I immediately looked at Jill and tears of joy filled our eyes. 3-4 weeks was enough time to go home.

576 days. And each and every one of those days just make today, this day of blessings, that much sweeter. The miracles continue. The power of prayer is demonstrated again. And for the very first time, we were told that Lucas’ esophagus looks good.

Little Fire Fighter

We used to never let Lucas play with hospital toys, but there comes a time when you’re practically living in a hospital that you throw caution to the wind. Here are a few pics of Lucas in the pre-op check in area this morning.



Eminent Discharge

Lucas is doing so great with his recovery, that we’ve received news that we may be discharged tonight! In fact, we just received training on how to care for Lucas’ PICC line when out of the hospital. This means that we should get the much needed chance to get out and about in Boston and enjoy a few fun days with Papa and Nani before they have to leave. We still have several appointments to attend in the coming weeks (endocrinology, genetics, esophageal atresia clinic, pre-op, etc), and at least one more surgery this upcoming Friday, but life will be a lot better not confined by the hospital room – even though we are in a brand new super nice wing of Boston Children’s.   🙂

Surgery Update

In some ways, yesterday could have been called a non-surgery. Although Lucas was under anesthesia for almost 3 hours again, so I think we’ll go ahead and count it. Dr. Manfredi removed the stint and examined Lucas’ esophagus both visually and under fluoroscopy. He said that he was very pleased with what he saw. So pleased, in fact, that he choose to not even do any further cutting or dilating! In other words, the first surgery went so well and the condition of Lucas’ esophagus was so encouraging, that all Dr. Manfredi ended up doing yesterday was remove the stint and look around. Another reason he just just looked was because the esophagus was still pretty “raw” as he described it and he wants to see how it heals up before proceeding. Dr. Manfredi mentioned that there were a couple of small finishing items he thought about addressing, but that sometimes it’s better to just leave a good thing alone instead of risking inadvertently making it worse.

The surgery ended up taking so long because our little bundle of energy managed to pull on his PICC line enough over the past week to make it no longer central. They replaced it with a new line “just in case” that we’ll have all week, and the replacement didn’t go quite as smoothly or as quickly as it could have.

In any case, how these esophagus results hold up will be very telling for Lucas’ future. We hope to find out a decent answer to that question during the next surgery on Friday, August 1st, but as it stands right now, we are very, very encouraged and hopeful. In fact, Lucas may take his first bite of food very soon!