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Sunday, November 4, 2012

5 Weeks Post Fontan

Since we last talked...Jonah has been making slow and steady progress. After that horrible Tuesday he spent in the tank, I called his Cardiologist to explain the series of events from the day. As I mentioned before, it was decided that Jonah's diuretics should be cut in half. This was a big move in our minds because it meant testing the waters...rocking the boat...playing with fire, if you will. We knew that we couldn't keep slamming him with the "big dogs" forever, and clearly his body wasn't happy with them, but it still meant opening the door of potential fluid accumulation on the right lung. I think that we were even more nervous because we saw first hand how small changes result in big problems. Here, we were making a pretty big move in our minds.

Turns was just the right move at the right time. Since then, it seems we have more and more of the old Jonah back. He has had more good days than bad and he seems to be a little more predictable. Last Friday we saw our Cardiology team again and Jonah had the full battery of tests. His EKG was good. His Echo reinforced that with good overall function. But...they could see that the pressures were still high in the new circulation supporting Jonah's heart. We were mostly reassured that this is actually pretty typical at this point after surgery. The biggest sigh of relief came when there was no evidence of fluid building anywhere near his lungs. Praise God!!! There are a number of concerns that play a part in the potential for chronic pleural effusions post-Fontan but probably the biggest culprit is the location of the Gortex conduit used to bring up the inferior vena cava to the pulmonary arteries. It runs next to the right side of the heart very close to the right lung. It is also semi-permeable. As pressures increase, resistance builds, the natural exit would be leakage out of that conduit as well as back up in the pulmonary arteries. We have heard the adjustment the body needs to rest with this new "improved" blood flow can take anywhere from 3-6 months, up to one year!

Jonah's meds were left alone until we meet again in December. A few other considerations at the moment are 1) Jonah's INR and 2) his electrolytes. Both clearly affected by his diet and intake. On that front, Jonah is also much improved. There are a couple of old stand-by's that Jonah is not fond of right now but overall he is eating pretty well. His oxygen saturations have been consistently 96-98% while asleep without any supplemental O2.

These last 5 weeks, even these last 5 days, have brought their own set of challenges and changes. There are no words to explain what this experience has been like just standing by Jonah's side, just holding his hand through it. For him, we have certainly seen the physical impact, but the emotional impact has taken a serious toll also. For every smile, every laugh...for each and every new word/s Jonah has said since coming home...we count our blessings and thank God for His victory!!

Thursday, October 25, 2012

One Wild and Crazy Ride

And so it goes with our dear Jonah:) We have said this before in the past...he goes from 0 to 60, he has no grey area or middle ground. His recovery after the Fontan has not been any different. Whether it is medications, not eating, no naps or a new drawn out bedtime routine, oxygen saturations, dehydration, temperament...Jonah's day-to-day seems like a play book of opposites.

Tuesday was kind of a train wreck. It started out with Jonah completely refusing anything to eat for breakfast which made sense because he quickly started to work on a poop that was not going to come easily. I let go of the idea of food to let Jonah work on a few things but we needed to get going on checking his INR for the week. It was the first trip to our local hospital's outpatient lab and I had a feeling it wasn't going to be quick. We had to register first, but overall, the whole process didn't take as long as I expected. This lab is able to check his INR by doing a finger stick similar to a diabetic checking their blood sugar. This did not make any difference for Jonah-traumatized...although I think this part will get easier. Any waaaay, his INR was 3.3. Too high for the target range of 1.8-2.2.

By now it was about 12:00. With nothing in his belly and all his energy spent freaking out in the lab, Jonah passed out in his car seat immediately. I just kept driving with the hopes he'd stay asleep long enough it could qualify as a nap and I ended up at my parents house. This is where we ended up on that teeter-totter-gone-bad again. Jonah started throwing up. He still hadn't eaten, he still hadn't pooped and now he couldn't even keep water down. Within a matter of hours, his eyes hollowed out, dark-ER circles appeared under those eyes, and he was so lethargic he couldn't hold his head up. He had been drinking well throughout the day until now but his fluid restriction/medication balance was clearly unbalanced!

Seriously, they keep telling us this is all typical of a post op Fontan but it in no way, shape, or form makes this any easier to accept or to handle. Our nerves are shot. Our patience lost. Our energy on "E".
We talked with our cardiologist and decided to cut Jonah's diuretics in half and are sticking to 0.5mg of Coumadin until the next INR check next Tuesday. This change in medication can be a good thing. A step in the right direction, but we are still nervous of a lurking pleural effusion. I guess we'll never know how he's really adjusting until we try. And the fact that he was so dry again tells Jonah's medical team it's time to cut back, an indicator his body may be acclimating to the new circulation.

Only time will tell. Hoping we can hang on...

Monday, October 22, 2012

What's Happening Now

Since Jonah's cardiology appointment last Friday, the only real change made was to give one less dose of diuretic and, as of this weekend...sort of unofficially...we are going to give it a go without oxygen overnight.

On Sunday morning we woke up to hear Jonah saying "owie owie" on the monitor. He wasn't crying, just sounded annoyed. I figured he was trying to pull the nasal cannula off his face which we taped to the side of his cheeks to keep in place. When I walked in I found this to be true but also discovered the tubing of the nasal cannula (which is all one piece) had wrapped multiple times around his neck and pulling tight from the machine delivering the oxygen next to his bed. Yikes-beyond scary!!!! That was all we needed to pull he plug. We had been afraid of this possibility the minute the equipment came in to our house...especially with the way Jonah sleeps...any way you rig it, the possibility is just always there.

So last night we decided to leave his oximeter probe and machine on all night to get an idea of Jonah's real oxygen levels while he's asleep. We haven't done this...ever, actually. But in order to feel comfortable without the oxygen we needed to know. While reading books in Rob's lap he was about 94-95%. When we peaked in his room before we went to bed we saw 97%!!! This was such a relief and was enough proof for us that Jonah does not need the supplemental oxygen at this time. Tonight, we set the monitor up again and are hoping for the same numbers!

Eating has definitely become more of a challenge as the days go on and we think the meds are playing the biggest part in this-for so so many reasons. Jonah appears to be much more gaggy with foods of a certain mushy, sticky texture. Right now, it's pretty likely we can relate that to the attempts we made to mix medications in things like his yogurt, applesauce, oatmeal, bananas...all his favorites pre-surgery :-( He definitely does not have a vigorous appetite either. Meds, change in blood flow to his gut, and maybe even bad timing on new molars equal Jonah barely picking at noodles, Cheerios, and chicken. We are 3 weeks from the Fontan and it has been 3 excruciatingly long weeks of watching this. It is truly wearing on us. Besides being dehydrated, he las lost at least 2lbs.

Tomorrow we will be checking out our local lab for Jonah's INR level. In two weeks we see cardiology again to fine tune things yet again and definitely do an Echo/EKG. In the meantime, we hope to have more days like this...

Friday, October 19, 2012

One Small Step

On Tuesday, we went to see the cardiovascular surgery team at Hope for our first post-Fontan follow up. Jonah had bloodwork and a chest X-ray done before seeing the nurses to help determine our plan of care going forward.
His lab results looked really nice with all if his electrolytes in normal range. His kidney function was good and those values (BUN) only showed mild dehydration. His INR was just a touch on the high side so we have ever so slightly tweaked his Comadin. The rest of his medications were unchanged at this point.
The biggest accomplishment during this visit was discontinuing Jonah's oxygen during the day. That's it. One change at a time. We collectively have determined that taking things slowly with Jonah seems to have the best results. We've learned a lot about how his little body works along this bumpy ride. With that, taking Jonah off oxygen feels like a giant leap in his recovery. In no way do we want to push his heart and lungs at this point, so we wait on pins and needles during this precarious time as his vital organs adjust to his new blood flow. We are still putting the O2 on him at night but the freedom of having our boy back untethered from all of the leads and cables and tubes has been so refreshing.
Tomorrow we see Jonah's cardiologist who will take the wheel from here on out. We aren't sure if we'll have an ECHO or EKG, we're just hoping we can get Jonah in the front door without a complete meltdown. Seems doubtful, but he's full of surprises;-) We will keep you posted and Thank You again for hanging in there with us!!!

Sunday, October 14, 2012

Take a deep breath

Our first 2 nights and full day home have been quite uneventful. A rainy fall Saturday easily leant itself to a day inside watching movies, playing with every toy Jonah owns, and taking a few naps. Really, for me, it seems the exhaustion has truly set in. In the hospital I got enough sleep to get by but the days were always full of ups & downs, changes here & there...I always had to have my head in the game. Yesterday in the comfort of our own home I finally felt like I could breath easier, let my guard down, and let go. Actually, this is true for all three of us!

Jonah woke up twice the first night home. After a diaper change and something to drink we all went back to sleep pretty quickly. Last night he slept through the night and woke up in a much more pleasant mood.
The medication schedule for Jonah is the heaviest in the morning and evening as most of them are once or twice a day right now. Going forward I will try to crush any pills the night before to cut down on the prep time in the morning and lessen the anxious cry for something to drink as Jonah waits for everything to get ready. So far he's been really good with his new regimen, as in no puking and not fighting too bad. As soon as we cut back on some of his electrolyte supplements the total # of meds should be more manageable.
Oxygen at home has been going alright. Jonah is very active and can easily get up & quickly take off in one direction while we try to react fast enough to bring his O2 tank along. He is very good about keeping the cannula in his nose and doesn't seem to mind the tubing trailing behind him, that is not the hard part. Keeping up with him has been a two person job for sure so it will be interesting when Rob goes back to work this week.
We gave Jonah a real bath for the first time in practically two weeks. We washed him up in the hospital but it's not the same. We were able to get all the adhesive off his body, cleaned up the "gunshot wounds" that are his chest tube sites, trimmed his nails and washed his hair (thank goodness we cut that mop before we went in!)

Jonah goes back on Tuesday for a full days appointment including a chest X-ray, blood work, and a full exam by the surgical nurses. Please please pray that Jonah's lungs continue to be free of fluid and that we may discontinue the oxygen during the day!! We know that each and every one of your prayers have been heard and that He has answered back in His own way, in His own time. God Is With Us! God Is Able! We have been abundantly blessed and know our Lord will continue to strengthen us!

Jonah's "coffee"...milk in a Caribou cup...

Friday, October 12, 2012


We received the good news this morning; Jonah's fluid around his lung was gone and his blood work was acceptable enough (low INR, low potassium) to go home! From then on, the day was a whirlwind of medication orders, pulling of Jonah's last IV, flu shot, packing, and checking out of the RMDH.

Jonah was happy to be in his car seat and immediately started pointing out all the trucks and buses he could find. But the excitement got the best of him and he was quickly asleep and slept the rest of the way home. Taking advantage of Jonah's nap, Stacie unpacked a bit and got organized once we got home while Rob stayed with a sleeping Jonah in the car running in the driveway. When we finally woke him up to tell him where he was, Jonah was all smiles. We sat him on the couch, turned on a favorite movie and let him wake up before having to cram the next round of meds down him. After a good snack, Jonah was ready to get down and play on the floor with his cars. It takes two people to get anything done now that he is connected to a the oxygen tank. Speaking of oxygen, Home Care dropped off more oxygen equipment and now we have half a dozen oxygen tanks and a giant electric beast that is the size of a mini-fridge hooked up in his bedroom.

After visits from our wonderful neighbors and Gaga and Papa, it was once again time for another round of meds, and before we knew it, bed time. He fell asleep in record time back in his old bed. Hopefully we all can get a full night's sleep. We have grown so accustomed to the 12pm, 2am, 4am, 5am, 6am vitals, labs, X-rays, meds, etc that we don't remember what a uninterrupted night of sleep is.

Check out the bag full of meds we had to pick up in the pic below.

Thursday, October 11, 2012

So close, yet so far away

On Tues we were given the option to go home Tues night or Wed. We chose to play it safe and wait until Wed, and boy did this turn out to be a good decision. The docs had noticed a slight sliver of fluid around his right lung but at the time they were not concerned. However Wed's X-ray showed the fluid had dramatically increased, and therefore the dream of going home was crushed. But on the bright side, if we had not chosen to wait until Wed, we would have gone home without the morning X-ray and would have been oblivious to the growing fluid around his lungs. By the time we would have caught it (at next week's follow-up), it would have been a bad situation; he would have been re-admitted and had the chest tube put back in.

So yesterday they loaded up the diuretics once again in hope of drying up the fluid around Jonah's right lung. If that didn't work, they warned us that they would have to put a chest tube back in. Luckily the diuretics worked! The X-ray this morning showed good progress. Although there was still some fluid, we are hopeful that more of the same diuretics today will do the trick. The cardiologist won't commit to a discharge date yet, but hopefully it will be tomorrow or Saturday. It all depends on how his X-ray and blood work checks out.

The docs also have recommended that Jonah stay on 1 liter of oxygen 24/7 when we go home. At least for the first week and it can be reevaluated at the follow up appointments. It will be challenging to say the least to have a 2 year old hooked up to a big oxygen tank, but we are willing to do anything to help in Jonah's recovery. We are quickly realizing how long and treacherous the Fontan recovery can be. It will take months for Jonah's body to adjust to the insane re-plumbing of his heart. So if a week or so of oxygen will help, so be it.

As for Jonah himself, he is being a very good and patient little boy. He is playing well, and we are doing our best to keep him occupied and entertained. The last 2 days we have been getting out of the room and walking down the hallway to the play room. Today he took a wagon ride down to the main lobby to look at the fish tank, took a short stroll outside and stopped up on the 3rd floor to say hi to all his friends that took care of him on the PSHU.

Hopefully we can keep his spirits high, along with our own, so we can get out of here in the next day or so without too many more challenges.

Please pray for a good X-ray and blood work in the morning!!

Wednesday, October 10, 2012

No Discharge

More fluid built up in Jonah's right lung. We have been advised to stay for at LEAST 2 more days.

So hard. So sad.


October 10th. 9 days after Jonah's 3rd open heart surgery. We are going home. We have been on quite the journey, even since getting to the 2nd floor.

Jonah's INR was right in the range they were looking for yesterday so we have the appropriate Coumadin dose planned for now. The team has found a local clinic that will continue to check his INR as instructed. The nice thing is that the clinic only does a finger prick similar to a blood glucose check to get the sample they need. We report the results they advise us on the dose.

Jonah received a blood transfusion yesterday with the hopes that it would help his oxygen levels. It was only slightly lower than where Jonah was pre op, but any attempt to optimize Jonah's oxygen carrying cells the better.
The plan as it stands has Jonah going home on a little bit of oxygen overnight. His doctors and nurses are confident this is only short term while Jonah's body heals and his lungs relax and adjust to the new blood flow. They are also very conscious of promoting a good quality life and given Jonah's age and activity level, wearing oxygen all day long isn't needed especially because it doesn't necessarily have a significant effect. As we've been on the step down unit, Jonah's team tried another medication indicated for the use of pulmonary hypertension. The idea was to give it to him and watch for any increase in his O2. If he did respond, the school of thought was that the pressure in his lungs was still too high, resulting in lower sats. Jonah's oxygen levels did not change. It was no better, no worse. A good thing actually! With that, having Jonah wear supplemental oxygen for a while as he recovers will help to shed extra fluid in his lungs. Over time, his sats may improve. They may not. If not, they likely contribute the lower oxygen levels to collateral vessels stealing blood flow from the lungs. Just to clarify, Jonah has not decompensated in any way; his sats are 86-92%, just not a consistent 94-97% like some other Fontan's. Jonah's team is comfortable with that going forward but want to help him heal without any rebound in pleural effusions.

We still need to go through rounds and the specific details of discharge. We could still have a long day ahead of us but the sooner we get Jonah home the better. He is frustrated. Angry. Wants nothing to do with being comforted by us. He's slowed down on the eating front. He's bored. He's tired of all the cords, cables, and tubes connected to him. And obviously, he's thirsty. Home will bring more distractions and hopefully a little more fun.

Keep you posted on our exit plan!

Monday, October 8, 2012

Day 7: The Good and The Bad

Good news: we are on the step down floor and are one step closer to going home.

Bad news: on the step down floor we are much more on our own and don't have the same care/help as we did on the PSHU. Mom and Dad are running on fumes and had the worst night's sleep yet of the week.

Good news: yesterday Jonah ate good bfast, lunch and dinner.

Bad news: today he has yet to eat as of 5pm.

Good news: echo and EKG looked good this morning.

Bad news: O2 is low (90) and is still on oxygen. Don't have a good explanation yet. Sats aren't much better with O2 and an experiment with Viagra didn't do much either. It is unknown if we will have to go home on oxygen.

Good news: he is generally feeling well and is interested in playing. Even took him to a play room where he took his first steps walking again.

Bad news: ALL he wants MILK. The fluid restriction is tougher than we thought. Only 27oz a day doesn't last long when he is so thirsty from all the diuretics. Last night was difficult for us to watch him incessantly cry for milk and there was nothing we could do.

Good news: electrolytes are improving and Jonah doesn't have to take as many meds.

Bad news: first trial of Coumadin overshot the INR target range of 1.8 - 2.2. INR was 4 this morning. Coumadin on hold until next INR test tomorrow. It could take a couple days to find the right dosage before we can go home.

Good news: Auntie 'Aga' Allison came by to save the day to keep Jonah, Mom and Dad company. Halloween arts and crafts were a big hit!

Sunday, October 7, 2012

Stepping down and Stepping Out

What a difference a day makes!! Part of Jonah's plan of care today was to take a wagon ride. And that wagon escorted him to his new room on the 2nd floor step down unit.

Today, Jonah's chest X-ray looked crystal clear after having his chest tubes pulled yesterday. His electrolytes stabilized somewhat and we were able to discontinue one completely. His diuretics were changed to oral form and the frequency decreased. His Arterial line was pulled from his leg and he's been a pooping machine. His first dose of Coumadin was started last night. Tonight's dose will be a little less and they will check his first INR tomorrow morning. We will spend the next few days here getting this in an acceptable range as his Coumadin will likely get tweaked. He is still on a little bit of oxygen but they expect this at this point and as Jonah really expands his lungs it should no longer be needed either. Jonah very shakily stood up today for the first time in 6 days!!!! He couldn't do it on his own but he is otherwise moving around playing in bed as if his chest was never cut open! He will get there, he is determined to check out his new surroundings:)

Take a look at our beautiful home away from home...

Saturday, October 6, 2012

Round 4-Total Knock Out

Things have been happening so fast around here, I forget where I left off and have no idea where to begin. We absolutely cannot believe its almost Sunday!! Sorry for the missed update.

As part of this process, Jonah's medical team aggressively tries to remove excess fluid from his body with the ultimate goal of protecting his heart and lung function and allow time for the Fontan circulation to adjust. Fluid around Jonah's lungs can and potentially always can be a problem. This isn't true in every case but it is something the team takes very seriously. After surgery, Jonah's body begins shifting fluid, the diuresis process starts roughly 48 hours later and it slowly gets ramped up to push the limits on every organ. Jonah did not respond to these medications as quickly/effectively as they'd like so they took it to the next level and the next, etc. Finally, the floodgates opened and he began to dump the extra fluid. While Jonah was so waterlogged one of his nurses explained... "He's not eating because his liver and intestines are floating". As he continued to shed the retained fluids Jonah's electrolytes were depleted. Calcium. Potassium. Magnesium. Sodium. We began quickly to replace these both IV and orally. The intravenous route only bumped them up to get him to the next scheduled diuretic where he would lose them again making it a wash. We tried every trick in the book to get these oral meds to stay in his belly. Jonah put the clamp down anytime yogurt, applesauce and a spoon came near him. The push of a syringe wasn't any better because either way he was gagging & puking it up. This went on for too long yesterday and Jonah didn't meet his liquid oral intake for the second day. There we were...what felt like standing on a beach getting knocked down by wave after wave-just getting pummeled. We'd ask all day, every day-"is this normal?" The answer to that turned out to be Yes and No. Jonah's eyes were dark, hollow, black holes. His shoulders sharp, his spine and ribs prominent. He was dehydrated. Extremely dehydrated. Not dangerously though as his kidney function was still good. Mission accomplished!! He was utterly dried out. This is what they wanted for his heart but admitted it was too much for the rest of his body. Rob and I were totally defeated. Desperate. To look at Jonah like that scared us more than looking at him fresh out of the OR 3 times.

Step in---divine intervention. A veteran nurse we never met before came in to change Jonah's sheets after throwing up. She was quiet. We were crying. Then it seemed we both started talking at once. Before the hour was over we had Jonah started on IV fluids with most of his big electrolytes in it and another cut back in his diuretics.

Fast forward to today...Jonah's eaten 3 meals, kept it down, pooped twice, is narrowing the gap between his input and output, his chest tubes were pulled, and oxygen weaned. Literally a night and day difference.

This surgery definitely has our vote for being the most taxing, frustrating, exhausting, confusing, and intense surgery of all 3 repairs. We felt broken last night but we picked up the pieces today to hopefully take more steps forward than we do back.

Take a look at the before and after...

Thursday, October 4, 2012

Fontan Trials and Tribulations

This Fontan surgery has certainly proved to be every bit as difficult as Jonah's medical team has explained to us-over and over and over. With that, Rob and have broken it down in to several parts and we feel like we just stepped in to only round 4 of the boxing ring.

1. The emotional toll & anxiety the preparation for this surgery took. 2. Surgery itself. 3. The first 36 hours immediately post-op. 4. Striking the balance of the fluid restriction and diuresis, eating, and replacing his essential vitamins & minerals.

As we've moved into this next phase, we really feel the exhaustion setting in as we realize how long this healing process is going to take. Last night and today Jonah has become very wise to the trickery that has been played on him when it comes to his oral medications. Overnight alone they added at least 4-5 new medications-all having to do with diuresis and the catch up that must be done as a result. Added to that is Jonah's need for a good old fashioned poop:) He's had a boatload of medication causing things to get blocked up and he's not super interested in eating/drinking. The new circulation also causes congestion in his liver and arteries feeding the intestines, so again, a reason his nutritional drive is in the toilet.

We are attempting to control Jonah's discomfort with Tylenol today-yup, nothing fancy- and wake him up a bit. Crazy thing is, he's doesn't seem to be in a ton of pain at all!

We did achieve some success today with the disgusting 'salt lick' that is his sodium replacement. Also, his potassium almost went dangerously low so we laced that with a little grape juice and he actually took it ok. I think that will try to be our routine for his new (temporary) oral meds...for now, of course. I'm sure he'll spit it back in our faces soon enough;)

We did have A LOT of good that happened today!!!! Here is our Highlight Reel ...
(The minute-to-minute woman in Jonah's corner that's made such an impact on us this admission is in the first pic: Dr. Melissa Nater)

Wednesday, October 3, 2012

I and Love and You

Hump Day

It's Wednesday. A full 48 hours from Jonah's Fontan surgery and it seems we have gotten over a huge hump in his recovery!!

Jonah had a really good evening and night last night. After allowing him to eat a little bit of applesauce and getting a better combination of pain control/mild sedation Jonah has been sweetly sleeping. Actually, sleeping more than obsessing over eating and drinking. It is such a relief!!!! It seriously seemed like his body was possessed. He was already very agitated to say the least but it seemed when we increased or bolused his IV pain meds he really went to the next level of irritable. Something like this'll be considered a paradoxical reaction to to those IV meds but- who knows?! We are trying to recall his Glenn and if the same response happened but the details are muddled.

From here on out the goal is to get the excess fluid out of his body. He has had roughly 1 liter of fluid out of his tiny 2 year old lungs!!!! Can you believe that?? He is peeing but in comparison to how much extra water weight he's carrying he still has a long way to go. His electrolytes are running only slightly below normal but this is par for the course because the diuretics take them all with it as he goes pee. Once he eats a little more he should be able to account for all those electrolytes himself.

Jonah's heart continues to stay strong!! We haven't done an echo yet to look at detailed heart function but everything else they monitor does not indicate there is anything to worry about. An EKG was done last night just to check what looked like a funky rhythm but even that showed normal sinus rhythm. What they saw was probably a result of a whopping cocktail of medication;-)

To all the Jonah fans out there--thank you for your continued love and support!! We are so grateful for each of you!! To our Lord, our Healer--thank you for bringing Jonah out of the darkness of recovery, and above all else, thank you for holding Jonah's heart in the palm of your hands and loving him like only You can do!!

Tuesday, October 2, 2012

Day 1, Part 2

Wow! I mean Woooooowww!! Jonah is officially post-Fontan. Between the hours of 7:31am and 9:30am yesterday we did not hear anything from the OR. About 11:00 they had him off bypass, closed him up and felt comfortable getting him to the Peds Surgical Heart Unit. By 12:30 Jonah was all settled in. To say that the surgery went fast is an understatement, but since his coming to the ICU, it's been "all hands on deck!!!" We apologize to everyone that we have not been getting updates out more often!!

First, surgery went fantastic!!! Here's the down and dirty...
* the Fontan surgery connects his inferior vena cava directly to the pulmonary arteries via a Gortex conduit. The right heart is bypassed completely to preserve the heart muscle and maintain good rhythms.
* they were able to use a 20mm conduit. This is an appropriate adult size that should last him long into the future without having to replace. See picture below...
* minimal bleeding while cutting the chest. No complications going on or off bypass.
* no fenestration done. Rob mentioned this...a fenestration is a small hole made to connect the new conduit and the right side of the heart allowing for a small mixing of blood that acts as a pressure release in the lungs. If the flow is too high in the lungs and the conduit resulting in resistance, they would do this.

As far as post-op recovery goes...there was no amount of forewarning that could have prepared us for this. It was looking like we needed a tranquilizer gun at one point. Jonah woke up with a froggy voice asking that I sing him the ABC's. Not bad, right?? He managed to stay calm & quiet for a quick visit from each of our friends and family that were here rooting for him all day. After dinner at the RMH and an ABC marathon, Jonah became like a little torpedo in his bed...and spiraling with him were his chest tubes, Foley catheter, transducers, IV and oxygen tubing EKG leads, etc... At one point he had himself in a standing position trying to get into my arms.

We were told that Jonah's body metabolizes sedation very quickly, which, means finding a safe dose to administer was a slippery slope. The team increased his drugs, tried new drugs, but this boy was not going down without a fight. I left at 4am, rob came over to break me until about 7:45 this morning. The entire night Jonah would struggle to find comfort only long enough to untangle everything before he was on all 4's again. It is truly a miracle that he did not pullout anything critical to his recovery.

This morning was only slightly better in that he didn't seem as restless for as long whenever he got up. Again we tried the medication route. By now Jonah sounded like a scratchy broken record... "Water! Milk! Cheese! Oodles (noodles)! Peanut butter! Until finally they allowed him to suck some water (melting ice cubes really) from a sponge. could hear the bells ringing- Halleluiah!!!! He loved this!!! And it was seriously the most adorable thing ever to watch him like a little bird with his mouth open waiting for more!!! After his tummy tolerated this they gave a very effective oral pain medication. He's been lights- -out ever since!!

A gigantic thanks us owed to our doctor and nurse today who were so amazing at helping us help Jonah!! We love you girls!!!!!

Monday, October 1, 2012

Day 1 in the books

The day flew by and we are glad it's almost over. It's a huge relief to have this day behind us.

He flew through surgery faster than we expected. Thankfully they didn't have to do the fenestration (sp). He looked great when we finally got to see him, a little dusky, but not nearly as swollen as the first two surgeries. Since then it's been a fight to keep him resting peacefully. Within an hour of being with him he was trying to get up on all 4s and, in his groggy voice, asking to play with cars and of course for us to hold him. The nurses are trying to give him the right balance of narcotics without compromising other aspects of his recovery. So it's trial and error at this point. Each time he wakes up its a chaotic scene trying to help him get relaxed and comfortable without pulling out the dozens of tubes and wired he needs to be connected to. It's heart wrenching to see him writhe around and get tangled in all the crap he is hooked up to. And the more you have to untangle him, the more you have to delicately move him, and the more pissed he gets. Then repeat. We wish they could just sedate him so he would sleep straight through the next week, but that can't happen. Stacie and I are going to try shifts, where we rotate sleep at RMDH for a couple hours and then relieve each other. Stace has been a champ so far, but we have still a long road to recovery ahead and sleep and rest is going to be hard to come by.

We will post more details tomorrow but before I sign off, I want to thank all our friends and family for their support today. All the kind messages and prayers were overwhelming. At one point while in the waiting room it felt like half the room was filled with Team Jonah supporters and it really helped to pass those anxious hours while he was under the knife. It means so much to us and we are blessed to have such caring and kind friends and family in our lives. Thank you, we couldn't do this without you guys.

Short Term Pains for Long Term Gains...

...That's what Rob has been saying for the past few days. In our stronger moments this made sense, seemed so reasonable. It put this whole surgery into perspective. But we are human, we are weak, and we spent the better half of our weekend down right scared. However, last night as we were saying our prayers before bed...and "LIGHT broke in and brought me to my feet" ((Avett Brother fans out there;-) )) There was a voice in my head literally saying to me "Be not afraid. Be not afraid". I know that God has picked me up by my bootstraps before and again, I felt that swift kick in the pants. We were able to clear our heads, get packed up, and get ourselves to Hope this morning....Not without a few-thousand-tears though.

The OR nurses took Jonah back at 7:30 on the dot. The pre-op process was pretty smooth. Quick. They gave Jonah Versed about 10-15 min before going back. He seemed totally unaffected but then when we stood outside the OR door dropping him in the crib, he didn't make a peep. God is so good!!! Jonah was wiping our tears with his blankie. How strong! How brave is our boy!!

No word yet from the nurses on the inside. But we expect they are working on getting him to sleep, inserting his breathing tube and placing IV's. Cutting through the existing scar tissue takes time and they are careful to avoid excessive bleeding. We are in for a long day!!

Please pray for a swift start and NO SURPRISES!!!

All our love!!

Tuesday, September 25, 2012

When did we get here??

I keep asking myself this question. Along with How? and Why?

To answer these questions, I must face a truth that my head and my heart are in constant battle with. Yes-our precious little boy, our sweet and silly 2 year old, in-fact, has only half of a working heart. For at least the past year, we have rested in a peace that only our GREAT GOD can deliver. We have received blessing after blessing in our day-to-day lives since Jonah's recovery from his Glenn surgery. But this "comfort zone" that we have become so familiar with and...have enjoyed so immensely seems to be crumbling underneath our feet. I don't like it. I am really scared of it.

When I think of the How's and Why's of Jonah's heart, I actually feel very content. Very confident. I know without the shadow of a doubt that Jesus has given us the most amazing gift in our son's life. When I was about 22 weeks pregnant with Jonah, Rob and I had a very discerning conversation with God. Right there- in the waiting room of the doctors office. From that moment to present day, I have felt free of the burdening thought that anything different from Jonah's life being the most joyful and successful is even possible. But as I think of WHEN we got to this point, only days away from his 3rd planned open heart surgery, I must admit that I am very scared. This fear keeps creeping in. It seems heavy. And these are moments when it is my head that wins the battle. For, surely, it is my heart that holds that sacred place of peace.

I get so sad when I think of the tease that is the time we've spent between Jonah's Glenn and Fontan. How did it go so fast? Did we make every moment count? When I really wrestle with why I feel so scared and so sad, the one thing I keep coming up with is having to watch Jonah go through this difficult procedure. It has been our privilege to guard and to protect him, to love and to nurture him. We  are totally wrecked with the idea of letting him go...into the hands of his surgeons, under the abyss of anesthesia, to experience and endure the pain of recovery. We want take to take all of that away from him and take on all that weight upon our own shoulders. But if there ever was a 2 year old who can handle all of this, it is our mighty warrior. We have said this before, but when Jonah is faced with any kind of adversity, even the slightest bit of challenge... he will flourish...he will overcome. Any parent we have ever talked to, that of a child with a CHD or not, says that these types of life events are always harder on Mom and Dad. So much harder...I am not sure I even want Jonah to know. I want him to think of us as his brave and fearless parents.

With that, it seems like I have taken all of you on a bit of the emotional roller coaster that we have been on since waking up this past Sunday morning. I can't tell you how exciting it will be to get off this ride, to get on the other side of the Fontan. The anticipation of it seems like cruel and unusual punishment but we will get to the other side. And we will be witnesses to yet another miracle in our lives!!  Of this I am absolutely sure!!! I know 5, 10, 20 years down the road we will be asking ourselves the same question..."When the heck did we get here??" And then Jonah will recount all of the memories of his triumphant life from kindergarten, to college, to marriage...and we will wonder no more. Praise God!

We love you to the moon--

and back, little buddy!!!

Wednesday, September 19, 2012

Summer Fun and, oh yeah...the FONTAN!

Well, well, well. Here we are- 3 months since our last post! After Jonah's heart cath in June we received a "courtesy call" from Hope wondering if we would like to schedule Jonah's Fontan. At this point we had discussed our thoughts for holding out until October with our cardiologist. There did not seem to be any major red flags from the cath results so we we received the green light to enjoy the summer and schedule a later date.

And that date is......Drum roll, please......October 1st. That's right- 12 days!!! Those words sound like jibberish to me right now, but there is no denying it, summer is over, fall is beginning and we are kicking it off with a bang. We couldn't run from it forever. We can't ignore it and pretend like we are the parents of a healthy child with a whole working heart. The fact is is that we have to face this. We knew this day would come. But before I tap in to the emotions that come along with this day, we want to share with you some of the fun that we had this summer. After all, we were so blessed to have enjoyed the summer as we did and make the memories we have without the threat of another surgery hanging over our heads.

At the end of June we took a vacation with my family to Holland, Michigan for a week. We rented a house right off of Lake Michigan and packed in 8 adults, 2 kids and 2 dogs just a short 3 hours away from home. Jonah experienced alot of firsts during this time...cruising in...& driving Poppa's boat, fishing, roasting marshmallows around the campfire, and building sandcastles at the beach. But the most exciting could have been going to bed and waking up in the room adjoined with his cousin, Nola, which included running the length of the house in hysterical giggles.

We were honored to celebrate this blessed little boy again on July 30th, Jonah's 2nd birthday! This year was a smaller gathering short a few close family members due to their being sick that same week. The day brought a pleasant reprieve from the scorching heat we had all summer which was perfect for a party. To say the least, Jonah really loves anything in the family of cars, trucks, buses, trains or tractors. On a road trip to MN in May we played the movie Cars for him and he has since become best friends with Mater and McQueen...and all of their car buddies too;-) Around the same time Woody and Buzz joined the group and the movie Toy Story 3 had a permant spot in Jonah's DVR list. I mention this because we had the most adorable birthday cake made for him and it was, of course, Cars-themed. It must have happened sometime this spring when Jonah's reaction to or recognition of something he loves made his eyes absolutely dance with excitement that Rob and I seemed to indulge in the moments that provoked such joy. It is absolutely impossible to resist and utterly priceless. This birthday was full of those moments.

With the blistering heat we had this summer, we became regulars at a few local pools. To be honest, Jonah's very first pool experience was not all that pleasant. It was indoors, in the spring, but it was chilllly. On a 90+ degree day at the waters edge of a comfortable zero depth pool- Jonah found a new love for swimming.

We visited the Lincoln Park Zoo for the first time too. Next to cars, animals of all varieties are favorite of his.

Most recently, we took the kids for a ride on our Metra train. We don't live far from our train station which is very nice. Seeing how much fun Jonah had taking the choo-choo a few stops heading East makes the daily morning commute for Rob a little more cheerful:)

Finally, this summer included spending lots of time with family and friends. I can't think of anything more nostalgic than letting the kids stay up late on a hot summer night, running around in the yard with all the dirt and grime from the days play on their hands. So fun!!

P.S. I finished this on my phone so the format of this post is kinda weird;)

Friday, June 8, 2012

Light bulb!

This past week of being home after Jonah's heart cath were some confusing and stressful days. Jonah came home last Saturday and went easily down for bed at 6:45p...he slept for 12 hours-fantastic!
Some time around Sunday evening when Jonah was starting to run out of gas, his body and head felt warm to the touch. Of course we were thinking fever, even though his cath sites looked good and infection-free. When we took his temperature- no temperature. But he was also acting like he wasn't really feeling good, not just tired. That night we woke up to hear him moaning and groaning on the monitor. Nothing like we have heard him do when he's gone through a typical teething phase, which is about the only reason he would be waking up in the middle of the night over the recent months. I couldn't shake the feeling that I needed to get up and check his temp again. No fever, but clearly uncomfortable and distressed. O2 Sats, normal. We brought him in to bed with us and he fell asleep pretty quick after getting some Ibuprofen. Come Monday, Jonah woke up feeling so-so and nothing seemed all that out of the ordinary. By nap time he was a disaster. He absolutely would not go down- after falling to sleep in my arms, the second he hit the crib he was up like a bolt of lightning. After several attempts, he finally got 2 hours in. When he woke up I gave him some Tylenol because he just seemed uncomfortable again; he wouldn't play, had a hard time finding a content position on my lap, and was over the top sensitive. About 30 minutes after Tylenol, he was like a totally different kid!! Bouncing off the walls, acting goofy, playing hard. We felt like we could maybe rule out a brewing infection by this point and by the way he acted before and after a little pain reliever, it really seemed like something else was bugging him. All the while, Jonah was getting back to his normal eating and potty patterns, so nothing there. We were making ourselves crazy trying to think of the possibilities, questioning whether we should bring him back in to have him examined again=Torture!

I called our Cardiologist that night trying my best to explain how Jonah had been acting and having a really hard time putting my finger on something definitive. He asked questions about facial swelling, oxygen saturations, signs/symptoms of infection. All I had for that was No, Nope, Nada. We hung up with the agreement to have him looked at by the Pediatrician in that maybe we were dealing with bad timing on teeth, an ear infection, or something totally random.

A top contender in the realm of possibilities Rob and I were stressing over was the adjustment that Jonah's body was experiencing to the change of blood flow after having 7 coils placed. After the Glenn, we knew that headaches were expected. Big change to blood flow with the Glenn. So we thought since they definitely tweaked things again going to the upper half of the body, a headache was certainly possible.

Finally, I talked with some fellow heart mom's who helped me think of some more pointed and specific questions to ask our cardiologist. I called him Wednesday morning on our way to work...and this is when the light bulb went on...
He said that the collateral vessels that they coiled off in the cath fed areas in and around the chest wall and ribs. "This can cause some pain and discomfort". Hellloooo?!?!?! That had to have been it! It shed A LOT of light on our particular situation. We love, love, love our cardiologist and entire medical team and respect the fact that they can't go around scaring parents over every single potential side effect under the sun. Because these kids are so different, there is literally no "textbook" to follow. But this kind of seemed like a "nice to know" sort of thing:)

A huge "thank you" to our fellow heart families out there!! The network of sharing our most personal joys, triumphs, pains, griefs, and "has your child ever experienced x-y-z" is PRICELESS!!!

A few pictures of our brave boy...

Sunday, June 3, 2012

Cath update #2...and fast forward 48hours

Alrighty...a total of 7 coils placed in both arterial and venous collateral vessels. We just talked to Dr. Patel. Pressures in the heart and lungs look really good. There is no narrowing of either pulmonary arteries. Aorta is of good, normal size. No enlargement of the heart muscle. No arrhythmia's. All in all, not too bad. They said those collaterals were pretty typical of child at Jonah's stage pre-Fontan. 2 were really twisty though and 1 of those will need to get clamped during the Fontan.

Jonah came out of sedation like a bear. He was so out of sorts and irritable for a good long while. As soon as we saw him he took it up a notch. Thankfully we were able to pick him up right away as long as we kept his leg straight. We were in recovery for really only an hour and then headed up to the 2nd floor for our overnight stay. Jonah continued to allow staff to touch him without loosing his marbles but it is very likely that general anesthesia helped in that department. He stayed pretty content in our laps for a long time but he was not able to get up and walk until 4:30 in the evening. Problem was, the kid would.not.sleep. Took about a 20min snooze but that was it. He did perk up after a little Tylenol; sat up, played, ate, laughed. We tucked him his crib about 9:00 and by 9:30 he fell asleep comfortably. Unfortunately, I could not relax enough to drift off so I was kind of up & down for a while. At about 11:30 I saw a red light swirling around (oxygen monitor taped to Jonah's finger) and realized he was intent on pulling his IV out. He still needed it for antibiotics so I got him up to rock with me and he did sleep there for the most part from 1:45 until 4:45am. I, still did not sleep, at all. Rob was able to catch some z's on and off, I just couldn't wind down. The plan was to have his discharge tests start earlier with the hopes of leaving earlier so when the tech came in to do his EKG at 5:45am we woke him up to do the test. But...nothing else happened after that. Ugh. So, we decided we would just try to sleep again with very specific "do not disturb" instructions for the staff. At 8:30am we were up and at 'em again. Now it was a chest xray. We had to go to main radiology to get this done and since it was a Saturday we had to go to the adult side of the hospital. Yuck. They strapped him in to what looked like a mini electric chair to accomplish this. The very minute we walked upstairs the ECHO tech was there. By now, Jonah had completely come undone. He was so hysterical from the chest xray we needed to stop the ECHO halfway through to give him a break. He was out of control. His fear of all things medical had officially gone to another level. He absolutely lost it. This is when Rob and I basically did too. We were beyond exhausted and seeing him go through this was absolutely gut-wrenching. We tried not to, but it was hard not to think of what his 3rd surgery/recovery was going to be like when he was dealing with something minor in comparison and he was having SUCH a difficult time:( After all was said and done, Jonah passed out in Rob's arms for about 2 hours or so. And we had plenty of time for napping because cardiology didn't come by until about 1:30pm to discharge us.

All of these tests were done to make sure that Jonah's heart and body was adjusting to not functioning with those collateral vessels. Over time, the heart function adapts and changes with these vessels but it is not necessarily in an efficient way. So clotting them off could be enough of a change to tweak things a little. Thankfully, all of Jonah's results were good. No areas of concern. Nothing new. Soooooo, after Dr. Ilbawi and surgical team reviews Jonah's cath on Wednesday, we will get a call from Dr. Ilbawi's nurse to schedule the Fontan. To lock it in. To commit and never look back. I can't even begin to describe our mixed bag of emotions on that one...

We are home and Jonah is not feeling great but not feeling horrible either. He for sure needs some time to reset but he is clearly happy just to be here. He had a big smile on his face when we walked in the door, immediately wanted to look for Sasha, and had a good 12 hours of sleep last night. Home IS where the heart is.

Friday, June 1, 2012

Cath update #1

We made it! So...we got here this morning a little bit before 630am. I pretty much started sliding down the slippery slope of my emotions on the walk in from the parking lot. I am surprised the sweet lady that checked us in didn't call the psych ward to have me checked in:) After she told me a personal story about her own daughter, I was able to get a hold of myself and rest with the fact that, as always, God puts people in our path for a purpose.

Jonah's usual reaction to all things resembling the outside physical structure of a hospital or doctor's office began at pretty much the same time. He settled nicely with Rob of course. He continued to be upset pretty much through taking his weight and vital signs. He soaked his jammies (and Rob's shirt) pretty much right away so we quickly got him into his gown, got his "cool cars" and iPad out and he actually did really, really, REALLY well!!! Rob and I were prepared for a solid hour of uncontrollable crying but in true Jonah fashion he pulled up his bootstraps and handled the whole process like a champ!! His versed surprised me the most- I thought they would need to give him enough to take down a small elephant in order to pry him from our arms but he swallowed it easily and in what seemed like only a few minutes he was drunk as a skunk. We carried him to the cath room doors, he gave us both kisses with the silliest grin on his face and went in to the grips of the RN and MD to get this ball rolling. He cried out for "daddy" a couple of times but that was it. Barney, Blanket and his pacifier accompanied him to the table and the nurses assured us those buddies of his did not let him down:)

As I started this, I got a call from the nurse and Dr. Patel was able to get the appropriate access he needed on the first attempt"s"...plural-because they actually have to insert the catheter in two places in order to obtain all the data they need. In the groin and in the left arm. He goes in through the arm in order to get the most accurate info from above. Given the nature of Jonah's new anatomy, they are not able to fish all the way up from the bottom. We also were told they need to coil some collateral vessels around Jonah's super vena cava. These collateral vessels are completely expected and pretty much unavoidable. The body is always trying to compensate for the compromised blood flow to and from Jonah's heart. These collateral vessels are kind of like a little spider web or finger-like vessels that are very small but they can allow for increased blood flow/pressures to areas of the heart/lungs that the Dr's do not want. This intervention means we will need to spend the night here while Jonah is observed and gets some antibiotics. We knew this was a possibility but in our heart of hearts wanted nothing more than to leave this place in our dust tonight:( Good thing we brought reinforcements!!!

Stay tuned!

Thursday, May 31, 2012

Pre-Fontan Cath Tomorrow

Hello world,
You might be asking yourselves, just as we are, Uh...Fontan? What? Seriously, where has the time gone?? We say on almost a daily basis, "We need to post an update on the blog".  We are very bad bloggers and we apologize.  When we start what we think will be a quick post, it turns in to the process of what one could imagine writing an article for the New York Times or something. The amount of time we try to make everything perfect takes much longer for us than just putting our thoughts out there.  So tonight we are going to make this quick and skip the editing process.

We have so much to share to bring this blog up to speed.  The last post highlighted Jonah's walking progress.  I guess the next major milestone has been his speech.  He is quite the jibber-jabberer (crazy about singing) and we have loved watching/listening to him pick up new words each day. By the way, he has had his ABC's locked in for a few months now:) Health-wise he couldn't be better and we have been so blessed with our time between the Glenn and the Fontan.  Time. Has. Flown. By!

And now the Fontan is upon us.  Jonah has his cath tomorrow morning.  Just got the call today that he has a been moved up to be the first case tomorrow.  So instead of planning at being at the hospital at 9:30am, we have to be there at 6:30am.  Good and bad I suppose.  Worlds of emotions going through us as we try to pack up Jonah's essentials, an overnight bag (just in case), and make arrangements for our dog Sasha to stay with Stacie's sister.

It will be much harder this time around to let the doctors/nurses take him from us.  He is just so much bigger, more aware, than the last time we did could possibly be his worst nightmare...and I don't blame him because what could be worse for 23 month old little boy than having "white coats", who have poked and prodded him his whole life, drag him into a strange room where he will be without the two people who are always right there by his side.  Stacie has argued numerous times to let her go back with them until they sedate/gas him, but it doesn't sound like they will make an exception for even a fellow nurse.

Not only will we be anxious to get the cath over with, but we will also be anxious to get the results of the cath.  We know the results could mean anything.  They could find something that requires immediate intervention or maybe the surgery needs to be moved up.  Or they could find nothing and let us have more freedom scheduling the Fontan.  We just pray that whatever the results are, we will have Jonah back home with us tomorrow night.

For now, we must "Let Go, and Let God". If there is someone out there reading this tomorrow around 7:30am...please say a pray that we are reminded of this!!