Eight Years Ago

Alright, I have to steal the keyboard back for one post...

It was 8 years ago today that Jason and I walked down the aisle to vow before God our commitment to each other. I remember people asking me if I was nervous. And I remember very vividly being very calm, as I knew this was the man God brought for me to spend the rest of my life with. I'm certain that Jason felt the same.

Over the past 8 years, we have been through lots of things. Jason worked hard while I finished school. He supported me through my first years of teaching. When I had the spare time, he encouraged me to volunteer and work at the zoo. Now he works so hard to allow me to stay home with our kids. It's been a huge sacrifice for the entire family, but so worth it in the end.

Jason is the best daddy I could ever imagine to Paige and Eli. He loves to play with them, love on them, and grow them to know and serve God. There is no way these kids will grow up not knowing their Daddy loves them very much. As a mom and friend, it is amazing to watch Jason with his kids. I love to watch him play with Paige.

In this most recent storm we have endured, I can't imagine going through it with anyone else. Jason has been a rock and huge support for me through it all. As we have to make decisions and as we learn more about Eli's condition, I'm so glad we are in it together. Jason and I see so many things the same, we rarely have to discuss them in length to come to a conclusion and decision.

Reflecting on the past 8 years and what God has done in our lives, I know His work is in the middle of our marriage. Everything we have and will endure is to shape us to what God can use for His glory. Jason and I are in for the long haul. We have a vision for our marriage. We want to be 80 years old, walking on the beach holding hands, madly in love with eachother. The only way we can do that is by enduring the times we are in now. For better or worse.

Jason, I love you more today than I did 8 years ago. Thanks for being there to accept me as your wife as I walked down the aisle to you. I look forward to 50 more years with you, so we can be 80 and walking on the beach together.



Okay, you can have the keyboard back now...

Getting dressed and drinking milk

Eli is doing very well, lately. He's been moved to the "less-intense" side of the ICU. His old room was in a set of rooms designated specifically for high-needs cardiac patients. Eli had to give up his room for someone with higher needs. He continues to build up his breathing strength, but he's been extubated for almost a week now. "He's on his own," as the nurses say.

He's also starting to take milk from a bottle. He's not great at it, but he's getting used to it. They give him as much as he'll take in 15 minutes, then feed him the rest through his feeding tube. The bottom end of the feeding tube, incidentally, has been raised from his intestines all the way up into his stomach. While he used to have a direct shot into his "gut," all his nutrition now comes through his stomach like the rest of us.

We've had the opportunity to change a few diapers. What an unusual blessing this is, to finally get to change a dirty diaper ourselves. Today, we even put some clothes on him!

The medical staff has always refused to give us times and dates, because Eli dictates them, they don't. So while we don't have actual numbers, it seems very fair that we should expect Eli home before Easter. This will raise a few new concerns we hadn't considered. Eli has to stay healthy. He has another surgery some time this summer, and to get sick before then could be a major set back.

This has led us to an unusual realization. We've tried very hard to keep everyone as updated as possible, through this blog. There are so many who are reading, and trying to stay informed, to let everyone know individually (letters, phone, whatever) would have been impossible. This blog has served as a wonderful piece of therapy for us, as well. The transparency we've been afforded has been great. In fact, we hadn't realized how tuned in most of you were until recently, when we started talking about holding Eli. We've been put into a really hard spot. We now recognize how closely you've all been following, and how attached you've become. So many have become excited about finally getting to meet Eli and hold him, and all those little things that make babies so great.

Having pulled you all in so closely, we're now forced to ask everyone to keep a bit of a distance, in a manner of speaking. There are two reasons behind this. First and foremost is Eli's continued health. The more who hold him, and love on him and everything else... the more chance he has of getting sick. In addition, he's still a little overwhelmed by human interaction. It doesn't take much to get him worked up. Then his heart rate increases, his breathing accelerates, and his blood/oxygen saturation begins to drop. You and we have red and blue blood, Eli has all purple. We're used to 97% oxygen saturation, he's used to 85%. When he gets worked up, it drops into the 60% area, and we've even seen it drop into the 40% when he's extremely worked up. When we get home, we're not going to have all these special monitors any more, and we're going to have to learn how to read him ourselves.

Between these needs, and his need to stay healthy, we're going to keep him a little isolated. We still intend to be in public, we're not turning into hermits. You should expect to see us at church every week, and at the grocery store, and so on. We don't want people to avoid us, by any means. We just hope we don't offend anyone when they ask to hold him. And this brings us to our second reason for some space... we need to figure out how to be a family, finally. What's going to happen with Paige, who's been a role model child with constant attention? What are sleeping arrangements going to be like? What will his needs be, and will Eli have special requirements?

To make a long story short (I'm not so good at that, am I?), we still need all of you. Please continue to call, write, and even visit us. But also please also give us some time to understand how to care for a very fragile little baby before we're as reckless with him as we were with Paige (I still laugh when I think of how much we used to scare some of our friends with how daring we were with her). There are always adjustments to be made with any new baby, his will just require more attention.

The pictures above are from today, when mommy was bottle feeding him.

Hold him in our arms

Eli was extubated again today. I think we're going to stick with it, this time. He still has an IV in his left foot, but there's nothing tied into it right now. He still has the feeding tube, and an oxygen feed to his nostrils.

The only drugs still being pumped into him are methadone and valium. These drugs are used for weening him off the Fentanyl and Ketamine he's been on for the last 5 weeks. The latter drugs are opiates, and create a dependency. Without a gentle ween from them, he will go through severe withdrawals. Because he was on those drugs for such a long time, the weening process will take a little longer than usual. We've not been given any timelines, but my understanding is that we should expect roughly a week of diminshing doses before they're gone. The potential upside is that we might have the opportunity to begin teaching Eli how to eat while we go through this, rather than wait for him to head down into regular care. Today, he had good control over sucking on a pacifier, already.

We held him today, finally. He stayed in Tanya's arms for a couple hours while I made the drive from work in Bakersfield today. Then, he slept for over an hour in my arms. We finally have a little baby. A real, live, red-blooded (ok, purple blooded at this stage in his life) human being. More than ever, now we want to take him home and begin showing him the world.

Possible Extubation: Round 2

Eli's been doing very well on his lung exercises this week. We were just told that if tonight goes well, he'll be extubated again tomorrow. Tanya and I are a little gun shy about this, because we've seen him working so hard during his sprints. We're going to have to trust the doctors, but our fears of him not being ready continue to dominate our thoughts.

Please pray that Eli is ready and does well.
Please pray that Tanya and I get decent rest. We're both so constantly exhausted.
Please praise God with us that He has chosen to use Eli to bring glory to His name already.

A whole month

Can you believe Eli came into the world just over a month ago, already? Today marks one month since his first surgery, when he was only 2 days old.

After having to be re-intubated, Dr. MacDonald warned us that this week would be a long, difficult week because we wouldn't see any changes. All of Eli's changes would be happening on the inside. His lungs would be strengthening, his heart would be healing, and so on. However, we're not going to see any changes, so it's going to feel like we're stuck for a short bit. Tanya and I had already realized this ourselves, so we've sort of hunkered down to prepared to see... nothing.

Luckily, Dr. MacDonald was wrong! This week, Eli made some of the biggest visible changes yet! Sure, he's still intubated, but he's lost so much fluid and has become so much smaller! His head is finally larger than his chest again. We can see ribs when he breathes deeply. He has little arms and legs. His legs even fold in now, so he looks like a baby when he's sleeping, rather than being spread eagle because he was so bloated. He looks like a little baby! Before, many of the nurses would stop in and comment on how cute he was. Yesterday, the custodial staff was in there looking at him, making the same comments. We're awfully proud of our handsome "little man."

They've also taken out all catheters! Both of the last two chest tubes and the bladder catheter are gone. He's using a diaper, just like a real baby. And we don't have to change it, they do! The only tubes still in him are IVs in each wrist, and the IVC (IV line, direct to heart) in his belly button. The number one concern on his care sheet right now is the IVC, and the fact that it's been in there 30 days. Typical longetivity for that line is about 10 days. Being in there an entire month now, it's a very serious threat for infection. They would have removed it already, but they're having trouble getting a good "central line" for Eli. He's a hard poke. The doctors are starting to chatter about the possibility of putting in a Broviac line. It's a little invasive, and requires a quick trip to the OR, but it's a lot safer and affords better care for Eli.

Update: The IVC was removed late last night, and they were able to establish a couple other IVs. The Broviac may still be in his future, but we'll cross that bridge when we get there.

For today, we're very excited about his progress. Here's the little guy.

Re-Intubation

Eli fought and fought, but breathing on his own was just more than he could handle today. He's been re-intubated, and they'll try again next week, probably. In the meantime, they'll start running him through some strengthening routines. His lungs and all the muscles needed for breathing just aren't strong enough yet. The reality is that he's been intubated his whole life, so he's never had to use those muscles. The exercises they'll run him through will help build those back up.

He was off the ventilator for 4 hours, breathing on his own. Although it was very labored, he was maintaining proper blood-gas levels on his own. He was just exahusted... (literally?)

The photo was taken during that time. We've all commented on how much he resembles Paige, but it's even more pronounced without the medical tape moustache, don't you think?

Extubation

Eli was taken off the respirator this afternoon. That was about an hour and a half ago. He's struggling quite a bit, so there's very real concern that he'll have to be re-intubated. His voice doesn't work yet, but he's constantly crying... a silent cry, but his face still shows the struggle all too well.

This is pretty hard to endure. I just want to do something, but I can't. A lot of you have made comments to us about our strength. It's now that we realize our strength was based around how well he has been doing. Now, with struggle in our face, it's not so easy to be upbeat. This isn't a life threatening situation. Worst case, they'll just have to put the respirator back in and try again another time. Still, it's agonizing to watch him struggle so much.

As I've been writing this, they took another blood-gas test, and his numbers aren't as good as they were an hour ago. They're checking oxygen levels in the blood, among other things. They also gave him some sedatives to calm him a little, and they're going to re-test his blood-gas in another 15 minutes to monitor the trend.

Please pray that God would allow Eli the strength to continue breathing on his own. Please also pray that our desires don't clash with God's plan.

3 Week Update: Big Progress!

We have a lot to be excited about this week. Eli has finally started making significant healing progress! So many positive things have happened this week. Last week, his white blood count had increased, presumably due to some infection. He received antibiotics, and is doing well now. The primary focus for this week was to battle his edema problems, the fluid swelling. All his kidney numbers are finally down to normal, and the kidneys are finally starting to process fluids like they're supposed to. The largest of his chest tubes was removed this week, as was the drainage catheter from his abdomen. He is now left with only the two small tubes in his chest. Yesterday, he had a net loss of 150ml of fluid, and the day before was over 360ml of fluid. In the last two days, he's lost over a half a liter fluid more than he's taken in! Not bad for such a little guy! He's also gained significant weight since he entered the hospital. He came in at 7.9lbs (3.6kg) and was measured at 12.7lbs (5.8kg) this week. Keep it up, Eli.

Today, he also started receiving mommy's milk! They're starting slowly, 2ml/hour, and it's fed through the yellow tube in his nose. Once he's off the respirator, we'll begin teaching him how to eat. This will include teaching how to latch to a bottle, how to coordinate the suction action with the swallow reflex, and so on. He'll have to be able to accomplish that before we can take him home.

They relocated the IV in his thigh to his left hand. This is slightly saddening, because now both hands have IVs, are all taped up, and we can't hold either one. The fenaldopam was stopped this morning, as well. As we understand it, this drug was used to assist kidney function, but he no longer needs it. In fact, his furosemide (diuretic to encourage elimination of fluids) has been re-started now.

On the human side of the equation, we were excited to see Eli's eyes wide open today. They were bigger than we've ever seen them. It also appears he's beginning to focus and track. Even as sedated as he is, he would turn to look at my fingers when I used them to get his attention. He also made long, consistent eye contact with both Tanya and I today, as seen in the photo above.

I've been back to work, and it's a little different now. I find I have more patience and less of my intolerance weaknesses. I tend to be a perfectionist, and push very hard on my field crew for the same. A shift in priorities has made that less important to me than it used to be. Not every hill is worth dying on, after all. So many things aren't nearly the big deal they used to seem.

We're back home now, as well. Overall, I think it's a better situation. It's nice being so close to Eli, certainly. At the same time, living out of a suitcase in a hotel for weeks at a time can really drag you down. And we still have Paige to take care of. She's been so awesome throughout this whole ordeal. We couldn't have possibly asked for a more perfect little kid to go through this with us. Every night, she prays "Jesus, please make Eli's heart much better. Much better. Much better. Amen."

2 Week Anniversary

Today marks two weeks since Eli's first surgery. He's finally starting to make some progress, and that's a huge relief. His numbers continue to improve every day. However, there was a noted increase in his white blood cell count today. That's typically a sign of infection, so they've started him on some general antibiotics until the lab results tell them exactly what they're looking at. They've removed the catheter from his abdomen. Today also marked the first time we got to see his naked chest, and surgery scar. It looks really good! Tonight's nurse, Tracy, took the liberty of styling his hair again. We like her a lot. She clearly cares about Eli, not just her patient. She calls him "her little blue friend."

We got some visitors from church today. We started the morning with breakfast with the Redferns. Later, pastor Jim made his way up. Paige sure loves pastor Jim, and wouldn't stop telling him all about the Ronald McDonald house. She became tongue-tied as she didn't stop for air, she was so excited. The Doolittles also came by and said hi, since they were in the neighborhood.

Tonight, Tanya returned to her first Arbonne presentation since Eli's birth. She's very excited, and had a great time being "back in the saddle" of something slightly normal. Paige and I walked down to the cafeteria (free meals beat good meals, these days, and we didn't have a car anyway) and had a father/daughter date in the fish room. For those who have never seen CHCC's cafeteria decor, it's pretty awesome, especially for us coral reef nerds. Then we came back and watched a Winnie the Pooh flick at "Ronald McDonald's house."

Tomorrow (Saturday), the three of us are headed back home. I'm going back to work on Monday, and being in Kingsburg enables so many more babysitting options, so Tanya will have more opportunity to see Eli by herself. It will be nice to sleep in our own beds and be home again, but we will miss the convenience of walking to Eli's room whenever we have the whim. I can already feel a sense of absence, as I mentally prepare to go back to work and be as far as 130 miles away from Eli on any given day.

Finally, it's also important to us that we continue to thank you all for such loving, faithful support. Every day, we hear about someone else (often people we've never met) being encouraged by our story. We can only attribute our peace and comfort to Jesus. He's bringing that to us through all of you, and that's what being part of his body is about.

Swooning Nurses

Eli has the nurses wrapped around his little fingers. We've heard them arguing over who gets to care for him, some have offered to adopt him, and all have made comments on how cute he is. One of his daytime nurses styled his hair a while back, very nice and conservatively. Last night, a new nurse (to Eli) gave him a bath and styled his hair again. He's only two weeks old and already turning into a punk!

His numbers continue to improve. Today, his creatinine was 0.44, down from last night's 0.50. Dr. MacDonald told us today that they'd love to restart the diuretics, but Eli's already taught them to be more patient than usual. He's going to be here a while, and we just need to wait.

Also, while we're posting photos, here's a photo of his peak swelling, about a week ago. He was puffed up pretty nicely. He still has some decent swelling, you can especially see it around his jowls and legs. For a while, he was so swollen that his skin was stiff from all the fluid. Lately, everything's softening up and it feels much more normal.

We're so thankful for all your prayers. The support has come in from so many places, even people we've never met. We've been so humbled and appreciate you all more than we can put into words. Please maintain your faithful, diligent prayer.

Quick Kidney Update

Here's a quick addendum to the previous entry, regarding Eli's fluids and kidney function.

With a little aggressive prodding by me, buzz words like albumin (a blood component for moving fluids) and nephrotic syndrome (kidney die off that hinders their ability to produce albumin) started emerging. As I start doing a little research, I have a few questions that the nurse isn't able to answer. He calls in the shift intensivist (doctor). The quick-n-dirty version: his albumin levels are low, but that's expected after surgery. Not to worry. The numbers they've been using to measure his kidney function (BUN and creatinine) are getting better. With both of these numbers, lower is better, because that means the kidneys are getting the junk out. BUN should be in the low 20s, and over the first week after surgery, Eli's steadily climbed to about 52. It's been plateaued over the last couple of days. Tonight, we see it has finally started decending. Right now, it's at 48. The doctor says "52 to 48 doesn't sound like a lot, but little numbers make a big difference with these numbers. He's coming down the other side of the hill now."

Finally! Some sort of relief. We cautiously cheer that Eli appears to be coming "over the hump," so to speak.

Staying the course

The wide angle lens story is that Eli’s condition is still the same. We’re still just waiting for him to be weaned off some of his drugs, and rid is body of all the excess fluid.

This has been the primary concern as of late, his edema. His body isn’t transporting the fluids from out of the tissues, into the bloodstream, to be exported out. He was on diuretics, but those only work on kidneys, and this fluid isn’t being transported to the kidneys. As a result, his organs were “drying out” and his tissues weren’t. The diuretics have since been stopped.

Now there are new concerns of potential NEC. NEC is inflammation of the bowels, and can result in tissue die off in that area when the inflammation restricts blood flow. If caught early, simple proactive antibiotics can control any potential issues. In an effort to stay in front of any potential problems, Eli is being X-rayed every 6-8 hours so they can catch it before it starts. They’re also giving him more of the portion of the blood that is supposed to transport these excess fluids out of the body. The doctors’ concerns are not yet severe, just noted.
It has been expected that Eli would stay in the ICU for 2-4 weeks after surgery. It is becoming clear, and confirmed by the nursing staff, he’s going to be on the long end of this expectation. His fluids need to get out his body, then he can be fed milk (the feeding tube has already been inserted, they’re just waiting to be able to use it), then we can start cutting out some of his drugs, get him off the respirator, and start thinking about getting out of the ICU and into regular care, where we can begin teaching him how to eat (this will be a whole new battle, when that time comes), and eventually get him home.

We met Dr.Francis yesterday, another of the cardiology team. He went on to tell about his 8-year-old nephew who was born with HLHS. Dr MacDonald was his surgeon, as well. Dr. Francis went on to tell us how his nephew runs and plays with all the other kids, and you could never tell him apart from them. I asked him about the stat I saw a while back that said only 60% of HLHS kids make it to the age of 5. He said (paraphrased), “I would say it’s significantly better than that now. That’s 5 year old data, taken from kids who had surgery 5 years previous to that. So, effectively, you’re looking at 10 year old data on a procedure that’s only existed for 20 years. The advances we’ve made since then are huge. I remember, in my career, when we’d send these kids home and tell parents to love them while they still could, because there was nothing we could do to help them.”

Overall, we’re both discouraged and encouraged all at the same time. We’d like to see quicker healing progress, but it’s also encouraging to know that we’re in a constantly improving medical world, and HLHS isn’t nearly as gloomy as it used to be.

Please pray for Eli’s edema, that he begins eliminating his excess fluid.
Please pray for peace for Tanya and I. It’s difficult having no option but to wait and watch.

Holt-Oram Syndrome

First of all, Eli continues to heal. He's a little slow about it, but still within normal time lines. The biggest issue they've had is with getting all the fluids out of his body. He'd been on diuretics for a couple of days, but they were only drying out his blood, not his body. Due to surgery, Eli has pretty significant edema, or "fluid swelling." Typically, the body will lose this fluid into the blood, where it is processed by the kidneys. Eli's body isn't doing a great job of getting that moisture into the blood stream, so it's just sitting in his body. Diuretics only work on the kidneys, so since the fluid isnt' getting to them, the diuretics aren't helping any. All the doctors are fine with this, it just means Eli needs more time. "He'll pee when he's ready," they say. There was, however, a very significant amount of fluid in his abdomen. In fact, they think this might be enough fluid to put pressure on the organs, especially the kidneys, and hinder their performance. So today they put in another catheter, in his abdomen. In only a couple short hours, it had already drained 210mL of fluid. So this is definitely a positive step.

Yesterday, we met with Dr. Powell, a geneticist. Eli's blood tests have come back positive for Holt-Oram Syndrome (HOS). We have yet to find out of this is related, influencing, or completely independent of his HLHS. We do know, however, that HOS comes in a frequency of 0.95 out of 100,000 kids. HLHS, by comparison, is 1:15,000. We're thinking about playing the lottery now.

We've also learned that HOS can either be hereditary, or spontaneous (de novo, as they call it). It can also be due to either a mutation, or complete absence of a gene(s). Eli is missing genes. This is not better nor worse than a mutated gene. It's simply different. The genes he is missing include TBX5, which is associated with HOS. It all happens at conception, it appears. We have 23 pairs of chromosomes, 23 from dad and 23 from mom. When those join, they line up in their respective order, like two halves of a ladder coming together (or the double helix). Within this, there are some 40,000+ genes that weave together to complete the whole process (I'm obviously simplifying this WAY down). So as all these genes were matching up, a few of them down "the long arm of chromosome 12" failed to match up. Imagine a ladder that had a rung attached to only one side. That entire rung falls away.

As we understand it (and please excuse the fact that we've only been given a cursory explanation ourselves), this mismatch and deletion can happen either because of random error (they just didn't line up correctly) or because either Tanya or I is a carrier, and our #12 is out of alignment, stuck to either #11 or #13. It seems spontaneous appearance is more common than inherited, so the odds are in our favor for being negative. Also, you can be a carrier without having any symptoms. The next step is for Tanya and I to be tested (small blood sample and 5-7 working days). If either of us is a carrier, that means we have an exactly 50% chance of passing this on to our kids. Eli got it, and we'll test Paige, though she shows absolutely no signs. If neither of us is a carrier, this was dumb luck, and we have no reason to test Paige. Either way, should Eli ever have children, he will definitely carry an exactly 50% chance of passing this on, himself. He, obviously, is a carrier.

We have a more in-depth meeting with Dr. Powell on Monday, and Tanya and I will have blood drawn. Until then, we're left without a complete grasp of what we're dealing with.

Also, just a little clarification and rumor control. People have come back to us with an understanding that Eli will be in the hospital 4-5 months. This is not accurate. Our expectations are 4-6 weeks from surgery, and he'll come home. We are expecting another surgery in 3-6 months, and we start this healing process all over. In that surgery, they will take the superior vena cava (SVC)(Eli incidentally has two) and plumb them directly into the lungs for passive flow through the lungs.

Eli has all ten fingers and toes. The only potential abnormality is triphalangeal thumbs. Only X-rays will show for sure. Our fingers are all built with 2 joints and 3 segments. Our thumbs are 1 joint and 2 segments. Eli might have thumbs that also have 2 joints and 3 segments. However, they are still opposed. Visually, they appear normal, though possibly a little longer than usual. We didn't even notice it until the geneticist pointed it out.

We'll continue to keep everyone as updated as possible.