Big week ahead

Jerry and I are walking around the house holding our breath hoping nothing goes wrong as we wait for Zachary to be decannulated, medical-speak for getting the trach out. We think it may be very soon. We hope it is soon. It is like waiting for Christmas, but better, only with a fear that someone may decide to cancel Christmas at any moment.

I am trying not to get excited about the possibility of decannulation. I fear that if I get excited I will be faced with a huge disappointment if it does not happen. I am trying to breathe, trying to focus on the day in front of me, trying to not sit around and wait for Christmas, whenever that my be. It is hard not to think about it though.

From the moment Zachary received his trach we've been working toward getting rid of it, weaning the vent, first during the day, then at night.  Then weaning the oxygen, wearing speaking valves and now capping. The trach was supposed to help Zachary breathe and give him a chance to grow and develop. I believe the trach achieved those goals. Zachary is growing and he is learning. Zachary has come a long way and I am so proud of him.

I imagine a trach-free future for Zachary, full of swimming lessons, trips to the beach, splashing in the pool, and all the other things kids with trachs can't do.  I look forward getting out of the house without lugging emergency bags, suction machines, pulse oximeters, and oxygen tanks. I imagine a life that is a tad bit simpler and with a lot more closet space once the trach supplies are gone.

Getting the trach out will be a major accomplishment. We will keep you posted on Zachary's progress. I hope to have big news to report soon, very soon.  It's going to be a big week, maybe.

Too many appointments!

It is me again, Erin, Jerry's wife. Today I am feeling a bit sad about how lonely our lives are. It is hard to explain what it is like to have a child so fragile he can only be cared for by one of us or a nurse AND is difficult to get out of the house. Because of that we hardly ever go anywhere together. 

Our favorite nurse had volunteered to work Saturday night this past week and we were looking forward to a night out. It was disappointing that something came up and she had to leave early. I think this would be a disappointment to anyone with children, but the thing is - we can't reschedule. We cant just get a baby sitter for next weekend. Under normal circumstances we may have grabbed our kid and gone out for a family dinner. But it is flu season. We do not want to risk the flu. So we stayed home and consoled ourselves by letting the dishes sit in the sink while we watched TV - which we hardly ever have time do. It is 3:00pm the next day and the dishes are still there.

Zachary had five appointments last week. He had physical therapy, speech therapy, a meeting with respiratory therapy, nutrition appointment, and we had an appointment with one more gastroenterologist.

Zachary works hard in PT. Lucky for my the PT does not let me participate in the sessions any more. I am too much of a distraction for Zachary. I spend five minutes with her at the beginning of the appointment and five minutes at the end getting my homework assignments. The PT and nurse handle the session. Don't worry, I do not mind having an extra 50 minutes to myself! 

I have to be involved in ST. This week Zachary bit an apple. Like I said last time, progress is slow. We gave Zachary lots of praise for using his teeth. I feel ridiculous sometimes, but progress is progress no matter how slow.  The nutritionist was amazed with Zachary's weight gain. He is catching up! Despite a great weight gain, I did get more homework. Puree meat. Sigh.

The GI appointment was the most interesting. I've been to a lot of GI's. Zachary's other specialists wanted us to see a GI. So I've been searching for one that was willing to work with us to help Zachary become an eater, better manage the reflux, and get rid of the g-tube. I ran through Zachary's medical history up to present day and she was wondering why I was there (since he is eating and not vomiting). I did not let her know that I was there because I felt forced to be but simply that he's had GI issues and that I thought we needed someone to monitor him.  

I was very honest though and let her know that I've met with MANY GIs and could not find one who wanted to help Zachary learn to eat and was interested in controlling the vomiting. It must be a very small field because she knew everyone we met with. It was strange for me to discuss my disappointment of her colleagues with her. I liked her a lot though. 

She examined Zachary and told me he looked great! What did I need a GI for? She said I must be doing a great job. She apologized on behalf of her colleagues simply stating that GI is such an art form and sometimes doctors get stuck with their way of doing things. She also noted that she will appear to be the hero because all that is left to do is take out the G-tube. I wasn't ready to do that just yet, but when the time is right and our nutritionist and pediatrician are supportive we will go back to her and she will take it out. I do find comfort in knowing we have a GI who I can call with any questions, in a particular one who thinks we are doing something right. I was pleased to get some positive feedback.

Lots to catch up on

Jerry's been busy with work so I am going to take a moment to provide an update. A lot of great things are happening in the White House and I want to share all the good news. It is impossible to make a note of everything that has happened in the last few months but I'll provide the most notable highlights.

Jerry last reported that we were waiting for the results of Zachary's sleep study. We hoped the results would be good enough to allow us to start weaning Zachary from his ventilator. The results were great! We did not wean Zachary from his ventilator though, we stopped using it cold turkey. I was scared and nervous about taking such a big step. After all, Zachary had been using a ventilator the vast majority of his life (except for a few weeks on CPAP and a few days on just oxygen). On the other hand, I was so happy doctors had faith in Zachary's improvement that it was hard to keep back the tears and stop myself from doing the happy dance. No more vent as of November 28, 2012. Big stuff.

It is not all good though. Zachary has needed oxygen on occasion since we stopped using the vent. We don't think he has been sick so we are stumped by the need for O2. Zachary had an appointment with his pulmonologist last week and he suggested it could be an airway issue. I do not want to think about this because it could mean another surgery. Obviously we will be seeing Zachary's ENT (ear, nose, and throat doctor) to discuss.

At this time, cardiology and pulmonology have cleared Zachary for Trach removal. One more doctor to go, ENT. (Of course, a new specialist could be sprung on us any day). There will be lots of checks and double checks, plus at least one hospital stay, before the Trach is removed, so we have a few months to go, at least. Fingers crossed.

In other news, we stopped giving Zachary his high calorie formula. I have spent more time on weight/feeding/reflux issues than anything else, including respiratory. I took Zachary to many doctors regarding his weight/reflux/feeding issues and received all kinds of opinions from he's fine to he needs to be in the hospital. It has been tough trying to figure out what to do.

The fact was that Zachary was vomiting daily, sometimes up to six times daily on the formula. Medication was not helping. He was getting the formula mostly by drinking it from a bottle, but sometimes I did have to supplement his caloric intake and use the g-tube. Despite getting him the required amount of calories his weight gain was marginal. I decided I did not have much to lose. We had experience from our first attempt giving him real food, so we used that as a base and began. I stopped the formula and after a few days (a few very difficult days) Zachary got hungry enough and started to eat. I took Zachary to his nutritionist and pediatrician about week after I stopped the formula. Zachary was making progress, he was starting to eat. To my surprise, they were supportive. Despite a significant weight loss, but not a dangerous one, I was told to keep at it, and good job. Whew. I feel so much better about giving my son real food. And Zachary is gaining weight better than ever and the vomiting has stopped.

Zachary has been in feeding therapy since he was in the NICU. We were warned that many many preemies have oral aversions. Zachary is a part of this club. I thought eating issues would be an easy fix once we got through the first G-tube wean. I know now that there is no easy fix. Zachary will probably be in feeding therapy for a very long time - years possibly. Right now everyone's biggest concern is that Zachary does not bite or chew. He eats soft foods, like yogurt, purees, hummus, mashed potatoes. The speech therapist gives me lots of exercises and games to play with Zachary to get him to use is mouth in new ways. The nutritionist requests that I mash and puree this, that, and the other things, for better nutrition (and she requests that I add butter and cream to everything too - yummy). Zachary of course does not the like the games or foods at first, but I must do them multiple times each day. Exhausting! I know all this work will eventually pay off (we have seen progress), but it is tough. The progress is very very slow. I am tired of making mashed sweet potatoes (or whatever) every day for weeks only to have Zachary reject them every time, until that one magic day he eats a bite or two and FINALLY a whole bowl.

And don't get me started on what happens to all the food Zachary does not eat. My new jean size proves those yummy sweet potatoes loaded with butter and cream did get eaten.

Zachary also had a follow up appointment to check his fistula repair. It was a very long and frustrating appointment that deserves a post all its own, but the news is that things are healing OK. The healing isn't the best case scenario, but good enough that he won't have any problems long term. The doctor seemed satisfied with the results. Zachary will have to be followed for a few more years (yikes) just on this one issue alone.

Zachary is cuter than ever. He is walking, running, and climbing all over the place. He's not talking yet, but using more and more sign language everyday. Just yesterday he signed Mama - twice. I would have rewarded him with ice cream if he'd eat it.

Thank you for reading!

what the hell is going on?

I am not angry.  Just thought some of you may have been wondering what the heck we've been doing since we warned about the approaching storm in the last post. 

We made it through Hurricane Sandy unscathed.  We lost power for about 24 hours but were able to keep Zachary's emergency backup batteries charged by plugging them into the charger in the van, though that required quite a bit of driving around.  It got cold in the house (59 degrees) but the power came back on shortly before we were ready to abandon ship.

A lot has happened since then. 

Probably the biggest news is that Zachary had what we think was a successful sleep study.  We don't have the official results back yet but he went all night without oxygen or needing to be put on the vent, so those are really good signs.  His carbon dioxide got a little high but not so much so that he needed to be vented.  This means we may soon be able to start weaning him from the vent when he's sleeping, which is another prerequisite to getting rid of the trach.

We've also started weaning him from his bottles of formula, which means starving him until he's so hungry he'll eat stuff like yogurt and baby food and rice cereal.  We've made some progress, though not as much or as fast as we'd like.  And he's lost some weight, dropping below 20 pounds again.  But we're seeing a dietitian at Georgetown University Hospital who seems supportive of our desire to get him eating real food.   It's real tough on the days he's cranky, crying, and signing for his bottle, but we've held firm so far.

He's had a bunch of other doctor's appointments too in last month.  He's seen the pediatrician multiple times, the pulmonologist, the cardiologist, and the dietitian, not to mention his physical therapist and speech and occupational therapists.  All have gone reasonably well.  Still getting better, just more slowly than we'd like.  But he's headed in the right direction.

And we've entered the dreaded RSV season, the time of year when kids get this respiratory infection that really puts those with chronic lung disease like Zachary into a tailspin.  The doctors have told us to put him on lockdown and avoid other children or enclosed places where it could be transmitted. 

We're haggling with the insurance company, Medicaid, and the pediatrician's office to get him a synthetic vaccine called Synagis that's supposed to help, but the health care and insurance bureaucracies are beating our butts right now.  He's technically outside the guidelines for getting this very costly series of shots (one per month from October to March) but all the doctors seem to think he needs it.  We're going to prevail eventually, though hopefully before he gets RSV.

Mom and dad are TIRED.  The nursing hasn't gotten any better.  But I think we've gotten better at dealing with the disappointment of unmet expectations.  We could use a break though. We each stay up two nights a week to cover unfilled shifts.  I am surprised at how efficient one can become on an hour or two of sleep per night, though it is no way to live, and as one of my friends says, is unsustainable.  That's for sure.  But it's better than the alternative, sadly. 

Hope to start posting more regularly again.  Thanks for checking in on us!

calm before the storm

So I'm sure you've heard, there's a storm a coming. 

All the smarty weather people say it is going to be bad.  Word is that the worst will be from about 8:00 a.m. Monday to 8:00 p.m. Tuesday, when we're supposed to get really heavy wind (up to 80 miles an hour) and rain (5 to 10 inches), which if accurate, will almost certainly mean we'll lose power.

Washington has officially shut down.  No government.  No transportation.  No schools.  Those of us who live here are used to the over-hyped prognostications about winter storms that never seem to be as serious as they are made out to be.  Hope that's the case this time.

The power company called a couple days ago to tell us to expect to lose power for multiple days.  Guess that's what passes for customer service these days.

The medical supply called to see if we needed anything, and when we said we needed some oxygen, said they'd put in an order, which means we'll get it who knows when. 

And the hospital called to see if we had any alternate plans other than shelter-in-place, which, not to their surprise, will be to go to the hospital if we lose power for too long so we can keep the ventilator operational.  We're hoping though that we can sufficiently charge the equipment in the van and avoid any hospital visits. 

We've prepared about as much as we can.  All the back-up power for Zachary's medical equipment is charged.  We've got lots of canned food and water to ride out the storm and its aftermath.  Fresh batteries for flashlights abound.  All the cars are filled with gas. 

So we're just sitting and waiting.  Just a cold light rain right now.  Soon to change though. 

wedding weekend

We went to Richmond this past weekend to watch one of Erin's cousins get married.  Zachary went with us too, though he skipped the wedding itself, which was lots of fun. 

He had a weekend full of firsts, such as his first time staying in a hotel, which he thought was wonderful since it had so many doors he could open and close, and open and close, and open and close.  He also was quite fascinated by the elevators, in which he could see his reflection. 

He also got to meet some extended family for the first time, which gave him an opportunity to show everyone how well he waves hello and goodbye.  He also visited Virginia's capitol and had his first  full-blown public meltdown in the hotel lobby.  He also got to see grandma, Uncle Matt and Aunt Jill and cousins Jake and Grace, who visited him in his room while we were at the wedding and reception.  (We brought a nurse with us too).

It wasn't all fun and games, though.  He got sick on his second night there despite our best efforts, probably because of the germy floors and air.  Not sure just yet whether it's just a little cold or something more serious, like a respiratory infection.  Time will tell.  Right now he has a runny nose, dry cough, diminished appetite, low energy and is needing way more oxygen that he usually requires.

So sad to see him feel so crummy.  His coughing fits have gotten so bad and he cries so much that tears run down his little cheeks.  We're giving him more frequent and varied nebulized breathing medicines so hopefully that will start helping soon.

But the good news is that at least his feeding tube gives us the ability to give him food and fluids even though he's not hungry, so he can stay hydrated and nourished.  He needs to get better quick so we don't have to cancel the sleep study he has on Halloween. 

The sleep study, which requires a night in the hospital, will help determine whether he's retaining carbon dioxide when he sleeps, which is why he's still on the vent at night.  If he's not, then we can start weaning the vent at night over the course of a couple months, which is a necessary step before we can start the process of decannulation, or getting the trach out. 

If the sleep test goes badly, he'll need to stay on the vent at night through the end of the year and into next year while his lungs continue to heal from the battering they took when he was first born and they were paper thin and were brutalized by the force required to properly ventilate him.  Wouldn't be the end of the world, but would be a disappointment for sure since the trach would have to be in longer than we would have hoped.

We have had some positive news on other medical fronts recently.  He reached 20 pounds today so he's finally on the growth chart and trending up.  And his pulmonary hypertension hasn't gotten any worse, so we've decided to go back to seeing his first cardiologist who we really liked before it was recommended that we see a PH specialist. 

So all-in-all it was a good weekend.  Could have done without the sickness, but he had some new experiences and got to meet some people who care about him very much, which made it worth it, as did seeing Erin's cousin Marc and his family so happy at their wonderful wedding weekend.  

turning two

My baby boy turns two tomorrow!

We celebrated yesterday with a small party at our house joined by some family and a few close friends.  Aunt Judy made this amazing cake, which tasted as good as it looked.

I can't believe it has been two years since Zachary's remarkable birth.  They have been years filled with ups and downs and hopes and fears.  But most of all the days between then and now have been filled with love for this unbearably adorable little boy and amazement at how much he seems like any other toddler entering his terrible twos.  

I think one of the things that struck me most about his birthday celebration was how little I thought about the special circumstances that surrounded his birth and the challenges of the resulting rehabilitation, but instead was focused more on matters such as making sure there was enough food and drink and the score of the Redskins game. 

I wasn't thinking about lung disease, mucous plugs, feeding therapies, or any of the other unresolved medical and developmental issues, of which there certainly are many.  But yesterday I felt like I was able to put those very important issues into perspective, at least more so than I have in the past.  One of my greatest worries is that I have become so invested in being the parent of a preemie that I'm missing out on just being a parent. 

So my pledge to my son for his third year is to be a little less protective so he can do what little boys do while I work on being a little more father and a little less caregiver. 

Happy birthday, buddy.

happy anniversary

Today is my sixth wedding anniversary.  Hard to believe it has been six years since we stood in the shadows of the Jefferson Memorial before friends and family and promised all those things. 

We're not really the same people we were back then.  We're older, hopefully wiser, and definitely more weary given the challenges of the past two years.  We used to be those people who had cool and exciting experiences like exploring Ecuador, visiting London, enjoying Rome, and cruising the Italian Coast.  We used to camp, kayak, run, and in retrospect, waste a lot of time too.  Not so much anymore.

Some of the lifestyle changes are simply from having a child, sick or otherwise.  But some are unique to caring for Zachary, which we've done as best we can.  We've been forced to band together to make life-altering decisions, both for him and us, and modify our lives as needed, like becoming a one-income family so one of us can be here to care for him the way he should be cared for all the time.  

The challenges have brought us closer at some times, and driven us apart at others.  Sometimes it's not so hard.  Sometimes it's really difficult.  But we have been really lucky to have such great friends and wonderful family to help us through the rough spots.

I have no idea whether these continued tests will eventually break us or bond us more tightly.  But I am certain that Zachary is lucky to have the best mommy a little boy could ever hope for, and I couldn't think of a more dedicated and committed partner fighting with me for his future.

Happy Anniversary, Erin.

TODAY show story about the Zaky

http://video.today.msnbc.msn.com/today/49286455#49286455

The TODAY show ran a story this morning on the creator of the Zaky, which is used to comfort preemies in the NICU when their parents can't be there.  We were fortunate that Aunt Judy found out about this and got one for Zachary while he was in the hospital.  We still use it today!

BIG decision

We've made a big decision, or at least an important one that's going to have a major impact on life around here in the not too distant future.  That decision is to forgo nursing starting at the beginning of next year. 

In some ways it won't be too different than now, where we have week after week with multiple days and nights without nurses because there aren't any available who have experience working with kids with trachs who use ventilators.  But in others it will be a significant change, especially when we really need a respite from the physical and mental rigors of caring for a medically-fragile kiddo like Zachary.

Don't get me wrong; we have been really, really fortunate to have had the help and benefit of some very caring and well-intentioned nurses over the past 15 months since Zachary came home.  Might be a stretch to say we couldn't have done it without them, as we've been continually surprised by our capacity for adapting to each new challenge and learning how to care for such a labor-intensive little boy.  I wouldn't have thought I'd ever be able to change a ventilator circuit, replace a trach, or put a dislodged feeding back into place, but such tasks have become routine.

But we still haven't become fully accustomed to private duty nursing.  I still find it a little uncomfortable, if not downright intrusive, to have virtual strangers in my house day and night, each of whom has their own idiosyncrasies, like the one who used to throw bags of trash down the stairs, marking up the wall.  Or the one who wouldn't clean up.  Or the one who took their socks off in the middle of the night.  Or the one who almost always showed up late.

But there have been some very serious issues too.  Like the ones we found sleeping.  Or the ones who didn't know how to operate the medical equipment.  Or the ones who gave the wrong medication.  Or the ones who lied about doing treatments or taking safety precautions.  Or the ones who didn't know what to do in case of emergency.  Or the one who left someone else's pill on the floor of the playroom.  Or the one who left Zachary unattended.  You get the picture.

I think most of the nurses we have met are in the business for the right reasons.  Those that weren't were pretty easy to spot.  But good intentions just aren't good enough.   And all that caring and all those good intentions haven't always translated into competent care.  And it pisses me off, because I've entrusted these people with my son's life.  I don't think our experience is all that unique, unfortunately.  Other parents have shared similar stories.  I wish I knew why this seems to be an industry-wide problem and how to fix it.  That's another post for another day.

For now, we're planning and preparing to adjust to a nurseless world next year.  We figure it will take a couple months to get the house in order and establish routines for doing everything ourselves.  But we have so many open shifts now as it is, especially at night, that we're getting a lot of on-the-job training.  And we would be happy to train-up any friends and family who'd like to help out every now and then.  Just ask.

This may seem like a dumb decision to make, and who knows, maybe we'll come to regret it.   But it will be a big step toward normalizing life around here and further healing from the wounds inflicted by our tiny tot's early birth.

pandas, penguins, and people

http://www.washingtonpost.com/local/newborn-giant-panda-cub-dies-at-national-zoo/2012/09/23/e58c19f8-019b-11e2-9367-4e1bafb958db_story.html?hpid=z1

You may have heard that the giant baby panda cub that was born at the National Zoo in Washington died Sunday, just a week after its birth.  (I've included a link to the story from the Washington Post).

To my surprise, the news made me sad, really sad.  It's not that I'm a huge panda fan, though I was disappointed we didn't get to see them during our visit last month.  I think it made such an impression because it got me thinking about the universality of parenthood, the fragility of life, and the profound sorrow that any parent, human or animal, feels at the loss of a child.   

I had no idea about how much I could love and how deeply attached I could become to someone I hardly even knew, even before he was born.  Zachary's birth was so improbable anyway, with years of trying turning into IUI and then IVF, each costly cycle raising our hopes then dashing them days later until we finally got picture proof. 

We suddenly joined this club that carries with it both unmatched joys and enormous responsibility.  His early birth and multiplicity of problems prompted me into bargaining for his survival.  Though my prayers would acknowledge that it was selfish to question God's plan for him, I would beg for that plan to include many years of health and happiness, with or without me.  I realized from that initial prayer, which was my first in some time, that I would be willing to do anything for him.

I remembered the scene from the movie March of the Penguins where the poppa penguins risked their lives braving icy winds to keep the just-layed eggs warm while the momma birds trekked many miles back to the fishing grounds to fatten up so they could nurse the baby birds when they hatched.  The movie showed one father whose efforts failed and the way in which he expressed his sorrow, much like the mama panda, making distress calls and cradling a toy much as she had been cradling her newborn cub.

I guess the news just hit home because I know we are blessed that we still have our little guy.  We thought on more than one occasion that we wouldn't have him for long, going so far as to have a nurse take one first -- and we thought last -- family picture of the three of us.  I cry every time I look at that picture, sometimes even when I think about it, like now.  But I can't bring myself to erase it, maybe because it reminds me how lucky we are and not to take a single second with him for granted.

I might just be feeling overly emotional because I'm tired, it's late, and I'm spending another lonely nurseless night watching my boy sleep and wondering what he'll be like when he grows up, praying that he's not taken from us too soon by accident or sickness, and wondering how any parent could ever bear that burden. 

So I guess in at least one way pandas, penguins, and people aren't all that different. 

boys weekend

It's just the boys this weekend. 

Mommy went on an out-of-town paddling trip yesterday, leaving us behind to get into all manner of trouble. 

So far we've watched some TV, which Zachary doesn't normally get to do.  He didn't so much watch as just grab the remote and change channels.  I think he was enamored with the power it gave him.  Hope that doesn't go to his head.

We also tried to stay up late last night, but the little guy tuckered out at 8:00 p.m., which is earlier than he normally goes to bed.  He made up for it by getting up around 6:00 a.m. this morning though. 

We've got a big day planned today.  Going to go for a walk this afternoon, then after playing for a while, are going to watch Florida State and Clemson, if he can stay up that long.  Then tomorrow we're going to watch the Redskins. 

Erin and I have been trying to get away for a day or two here and there to mentally decompress, get a little exercise, and recharge our batteries.  One of these days we may even get away together for a few days, or at least that's the hope.

So anyone have any ideas what me and the boy can do while mommy's gone?

what's been going on?

I've been off the grid for a while and neglected my posting duties, so here's what's been going on the past several weeks for those of you who are interested, which I guess is you since you are reading this right now.

Zachary visited his cousins Grace and Jake a couple weeks ago in Cleveland.  He LOVED being around kids his size, well, almost his size.  It was interesting to see him interact with them.  He was fascinated by Jake, who's 3, and followed him around trying to pat him on the head.  Jake was a good sport, but could have done without the irritation.  Grace just wanted to hug and cuddle with Zachary, which was really cute. 

I was worried about him getting knocked down and around, but on the few occasions he took a tumble, he popped up smiling and laughing.  He did do a header onto the hardwood floor while running around the house, resulting in a nasty-looking knot and bruise on his forehead.  I think it hurt me more than him.  I'm looking forward to him spending more time with them and other kids his age and ability so he can start getting used to interacting and socializing with someone other than us and his doctors and nurses.

He also had an ultrasound last week to check on the artery and vein that were fixed a month or so ago.  There was still some residual blood flowing where it shouldn't, but it wasn't too much and the doctor thinks things looked pretty good.  Has to check again in about six months but it looks like we'll be able to avoid a second surgery, which is a relief. 

But for whatever reason, his heart rate has been creeping up to pre-surgery levels, about 15 to 20 beats per minute higher than it was before he started having problems.  He's not showing any signs of being sick, like increased secretions or a fever or anything like that, so we're miffed.  His pediatrician doesn't seem too concerned, but can't explain it either.  We've reached out to his cardiologist and pulmonologist to get their thoughts, so stay tuned.

Zachary is also very happy that football season has started.  He things Florida State could have a great year (national championship?) and that RGIII and the Redskins are going to be very exciting to watch, though probably won't win the NFC East or make the playoffs.  But what does he know, he's not even two years old, though he will be next month, believe it or not!

just because . . .

Welcome to Holland

http://www.youtube.com/watch?v=RqGQjoTn2xY&feature=shareis

I came across this poem many months ago.  It reminded me not to focus so much on what we've missed out on given the special circumstances of Zachary's birth and ongoing recovery, but instead to appreciate the things we otherwise may have never known or appreciated.  Worth the watch.

whew . . .

We're back (both from the hospital and to the blog).  We've actually been home from the hospital for a few days now but I've been too busy and too tired to post.

A lot has happened since last weekend.  Zachary was admitted to the hospital on Sunday after showing signs of congestive heart failure because of the flood of blood that was swamping his heart, a result of the artery that had connected to a vein near his tiny ticker. 

We didn't really know how sick he was until after surgery when the docs told us that his heart eventually would have stopped working from fatigue had they not fixed the problem.  One of the surgeons actually said "I'm not saying it would have happened in the next couple days, but it would have happened eventually."  Ok then.

The near four hour procedure was rough for us all.  They placed two metal coils and some metal wire (actually nickel and titanium to be exact) in the connection between the artery and vein to make the blood flow where it's supposed to.  Upon doing so they said his heart rate dropped back to near normal almost immediately and the blood that had been pooling under his eye as a result of the venous congestion started dissipating. 

The waiting was the hard part for us.  We got updates about every hour from one of the nurses who was assisting with the procedures.  It took so long because they also did a heart catheterization to measure the pressure in his ventricles (which was just slightly elevated) and checked to see whether he had any additional arteriovenous abnormalities in his lungs, which he thankfully did not.

He rested comfortably for the first few hours after surgery, thanks to some sedatives and pain-killers.  Then he woke up and was not too happy.  The docs thought it was a hangover from the anesthesia since he was under a long time for such a little guy. Had a very rough night that night with a really high heart rate and some vomiting, but he calmed down over the next couple days.

We're very happy obviously to have caught the problem in time.  But a test before he was discharged showed there was some residual flow of blood around the coils and wires that are supposed to stop that from happening.  Hoping that will resolve itself or he may need to go back in a month or so to have some more metal inserted to dam-up the vessels.

The other unfortunate occurrence is that he hasn't eaten much by mouth since the surgery.  Not sure why but we're going to be devastated if he stops eating orally after we spent to much time weaning him from his feeding tube, which his pulmonologist wants us to start using again, at least overnight.  He labels him as "failure to thrive," which is a sort of catch-all term for kids who aren't gaining weight and getting enough nutrition. 

We've worked hard getting him where he is, or was pre-surgery, and will do everything we can to avoid tube feeds with artificial formula, but we'll need to fatten him up soon or any weaning from the ventilator, and subsequent removal of the trach, will be delayed.  But the consensus seems to be that fixing the blood flow problem may help his overall health, allowing him to breathe easier, burn fewer calories doing so, and perhaps gain weight faster.  Let's hope!

Things went well, then, everything considered.  Whew . . .

NICU . . . PICU . . . CICU

We'll be hitting the trifecta, so to speak, on Sunday, though it's not one we are looking forward to winning.

The trio to which I refer is the third different ICU that Zachary soon will be visiting.  He was in the Neonatal ICU for his first eight months, then had several stints in the Pediatric ICU over the past year.  And now he'll be spending some time in the Cardiac ICU starting Sunday in preparation for surgery the following day to fix his AVM. 

We weren't expecting surgery so soon but his condition has worsened over the past week or so.  His heart rate is getting higher as his heart compensates for the short-circuited blood flow created by the tangled vessels.  He's also been breathing faster and the amount of oxygen in his blood has crept close to dropping below the level his cardiologist and pulmonologist are comfortable with.

The plan is to thread a catheter through his groin to his heart and inject contrast dye so they can see what exactly is going on with the vessels.  Then, absent any other problems, use a metal coil to divert the blood flow back where it's supposed to go, a procedure called an arteriogram with embolization.

Then they'll also perform a pulmonary angiograph, which is similar, but to check for vessel malformations in his lungs.  The lung doc wants to rule out an underlying genetic condition called Hereditary Hemorrhagic Telangiectasis (HHT) that could be causing abnormal blood vessel formation in his lungs.  Not going to worry about that now though.

We're really bummed about the coming hospitalization.  Best case is he'll be in for three or four days if all goes well.  Could be longer if it doesn't. 

It's going to be sad to see him poked and prodded again and not understand why we aren't stopping his pain.  And he'll probably be most troubled by being caged up in the hospital cribs, which are really like little jails, bars and all.

We'll see what happens.

can't make this stuff up

6:30 a.m. -- walked in on nurse about to give Zachary TOO MUCH of the WRONG medicine

7:15 a.m. -- needed to suction Zachary but nurse failed to replace suction catheter without telling me

1:00 p.m. -- nurse mistakenly turns off vent heater wire

4:15  p.m. -- pulmonologist calls to cancel sleep study because of AVM

5:30 p.m. -- nurse throws bag of garbage on porch (not in garbage can)

8:15 p.m. -- found a Viagra pill (not ours) on the floor in Zachary's play room

AVM

Finally found out what's been causing the blue arm and other assorted symptoms Zachary has been experiencing the past few weeks.  It's called an Arteriovenous Malformation, or AVM, for short.

It was hard to follow the explanation we got from the docs but here goes.  One of the arteries in his right upper chest sprang a leak sometime ago (probably because it was nicked by one of the central lines he had in the past) and established a connection, referred to as a shunt or fistula, with a nearby vein.  Then the capillaries in the area intertwined themselves around the whole thing creating a tangled mess of blood vessels.

The result:  oxygenated blood coming from his heart is not going to his arm like it is supposed to.  Instead it is taking a short cut through the connection between the artery and vein and is being shuttled right back to his heart, making his heart work harder than it should.  The concerns are twofold.  First, the vessels could weaken and start bleeding badly.  Second, his heart could be jeopardized because it is trying to compensate for the diverted blood flow.  Both would be life threatening. 

AVMs are more common in the brain, though they can appear anywhere in the organs, torso, or limbs.  Many people who have them are asymptomatic, in which case surgical intervention may be eschewed.  But action is indicated in most cases when symptoms are present, as in Zachary's case. 

Aside from his arm turning blue, it gets cold and swells, and his heart rate has increased by about 20 beats per minute, indicating increased strain on his heart.  And he's been a little uncoordinated the past week or so, stumbling around and struggling to keep his balance.   These symptoms are not unusual for people with AVMs, apparently.

His cardiologist has consulted with a vascular surgeon, who is devising a plan of attack, which will involve one or more procedures to alleviate the problem.  He'll have to be in the cardiac ICU as a precaution following the surgery, which I think is just hospital protocol and not because of specific concerns.

We're sad, of course, that our little guy has to go through more invasive tinkering with his insides.  And we're also frustrated that this might delay the mid-August sleep study that could allow us to start weaning the vent.  Seems like we keep taking two steps forward and one, or more, back. 

Should know in the next day or so what the plan will be.

bruit

Yeah -- we didn't know what that was either until recently.

We found out though after Zachary's right arm started turning blue.  Turns out a bruit is the sound  that blood makes when it rushes past an obstruction, like a blood clot.  Not sure that's what it is yet, but the pediatrician and cardiologist are sufficiently concerned that they've decided to do an ultrasound to find out.

His arm started turning purplish-blue a couple weeks ago.  Sometimes just for a few minutes.  Sometimes for half an hour or so.  The thought is that maybe some blood on the way back to the heart is being blocked from leaving his arm because of an obstruction somwehere.

He had an U/S a while back because he has some prominent veins in his neck and chest that his cardiologist thinks may be the result of previous clots, which are not uncommon in kiddos who have had risk factors like central lines, of which he had three for many, many months.  Too soon to speculate on what the path forward will be if clots are found, though the good news is that it appears not to be cardiac related. 

The coming month is going to be important because, in addition to the U/S, he has another sleep study scheduled mid-month.  The study will require an overnight in the hospital and help determine whether he still needs to be on he ventilator when sleeping. 

If not, we will probably be able to start weaning that in hopes of shedding it for good in the Fall.  If he still needs the vent, probably will be vent-dependent at night through year's end, when the sleep study will be repeated.

Keep your fingers crossed!