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...

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)

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!!

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. Well...you 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! Broke.my.heart!! 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!!!!!

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!!