Last week, after Ethan went with the angels, Erik, his dad, was telling me about him. Erik said that conversations with Ethan were great. He told me how Ethan was kind, smart, and funny. How much he liked music. How he’d start each day dancing away with the nurses in CICU.
It’s no surprise that Erik said Ethan was his best friend.
Mothers, they’re rarely shy of telling us, bond with their children in the womb. Perhaps I’m alone in this one, but when Ari came out, I looked at him and thought, “Whose kid is that?”
I had to remind myself, “Hey Mike, he’s your son.”
Your son.
Your son.
Maybe us guys are different, but at first I was more thinking about the cosmic connection than feeling it, trying to sort it out at 2:30 a.m. when I was alone with him in CICU just under 2 hours after he was born. It was a quiet time, just him and me, before all the people and all the procedures.
It seemed to last forever, but looking back, it was barely a moment in time. I’m not sure I thought about it much until Erik named Ethan his best friend.
Immediately after Erik said that to me, I thought, “Ari is my best friend. I love him.”
Wait! When did that happen?
Just a few months ago I was holding him for the first time, correcting myself when I thought, “I better give him back to his parents.” Or at least to someone who knows how to hold a baby.
Here I am now, hanging out with him every day at the hospital, posting pictures on Facebook, looking at pictures of him for the few hours here and there that I’m home, and writing him love notes here on his blog.
I didn’t expect this.
Don’t tell anyone. It’ll kill my rep. And don’t tell Toby! He’ll never stop moping.
What do I know about Ari now? He’s a happy boy. An easy baby (well, minus all the heart stuff and medications). And he’s a good kid. Do we know much else yet? Will he love music? Basketball? Fishing?
Art? Poetry? Ballet?
Dirt? Bugs? Buzz Lightyear?
I don’t know yet. All I know is this: he’s my son, my best friend, and I love him.
And the rest? Can’t wait to find out.
**** Medical Update ****
Sorry for no smooth transition from musings to medical, but I’ve been thinking about my best friend and I wanted to get this out, but I know everyone is more interested in what’s going on since the surgery. Here’s what’s up:
Thursday – Surgery Day
After the cath revealed how dire Ari’s condition was, there was a big shuffle all week trying to get him into surgery as soon as possible. Dr. Tworetzky worked on shuffling logistics all week to make it possible. As he put it, he did everything but call the White House to intercede.
Ari went down at 7:30 a.m. on Thursday. Dr. Emani, our surgeon, said he attempted to repair his mitral valve, but ended up replacing it because we needed it to be as perfect as it can be. The reason for coming as close to perfection as possible is that we believe Ari’s mitral stenosis was contributing to his pulmonary hypertension.
Think of it like this: put a redhead in the sun without sunblock, and 5 minutes later you can hear the sizzling. A redhead can be that sensitive to burning.
We believe obstructions to Ari’s blood flow in his heart are like the sun to a redhead. If we left stenosis in his mitral valve, even a little, it might not be enough to prevent pulmonary hypertension. The only way to get him “in the shade” would be replacement.
Unfortunately, they actually don’t make mitral valves for kids Ari’s size. The Melody valve is built for pulmonary valve replacement. The pulmonary valve is an outflow valve, and the mitral valve is an inflow valve. Pretty different.
What Dr. Emani said to us is, “Well, I turn it upside down, make some other adjustments, and put it in the mitral position.”
Mike and Erica: “How many of these have you done?”
Dr. Emani: “4”
Mike and Erica: “How many have ever been done anywhere?”
Dr. Emani: “4”
Let’s make that 5 now.
In fact, the Melody valve is new even for the pulmonary position. On the Melody website, it’s says right at the top, “Humanitarian Device. Authorized by Federal law (USA) for use in pediatric and adult patients with a regurgitant or stenotic Right Ventricular Outflow Tract (RVOT) conduit (≥ 16 mm in diameter when originally implanted). The effectiveness of this device for this use has not been demonstrated.”
That last line is confidence building, isn’t it? And that’s for what’s it’s built for.
Good thing we have Dr. MacGyver on our team.
Anyhow, it went in fine, and he was also able to remove more scar tissue as well.
Friday to Saturday
First, here’s what we need:
- Pulmonary pressure 50% or below systemic pressure. This is the measure of the pulmonary hypertension. A “normal” heart is around 20% to 30%. Below 50% is probably manageable. Above that, bad news.
- Low left arterial (LA) pressure. This is the measure of how well his ventricle can relax. Relaxation contributes to pulmonary hypertension and cardiac output. Normal is 5 to 9. 15 or below is probably manageable. Above that, bad news.
- Cardiac output of 3 plus. This is how vigorously the heart is pumping blood to the body. 3.5 to 4.5 is normal. Above 2.5 is probably sustainable for growth. Below 2 or 2.5, bad news.
When he came out of surgery, here’s what he had:
- Pulmonary pressure as a % of systemic pressure of 55%.
- LA pressure of 15.
- Cardiac output – estimated 2 to 2.5.
Right on the line. Just like Ari, isn’t it?
While they got done what they wanted to in surgery, it doesn’t mean he’ll get better. With these numbers right after the surgery, the doctors were universally noncommittal about his prospects for getting better. (Getting better means chance at life.)
A lot of wait and see. No optimism, but no pessimism either. Just guarded. Walking the line.
After the surgery, he was having “episodes” of spiking pressures every hour. His blood pressure was through the roof, and his LA pressure was as high as 30. This was very bad.
The attending in the CICU was standing over Ari like a guard for hours and hours, arms folded, staring at him.
It was comforting to have him, but scary that we needed him. He, as well, had no optimism.
Given all the episodes, they ordered an EEG to measure brain activity to make sure he wasn’t seizing.
It wasn’t a good night for him or us.
Saturday to Now
His episodes stopped and the EEG came back normal. They believe that the spikes were pain related from the open heart surgery. Ari likes his meds and is hard to gauge because he’s been on a lot of narcotics and paralytics already. He’s been stubborn since even before he was born when they had to stick him twice in utero to knock him out.
His pressures have slowly improved to what you see here:
If you take the yellow 31 and divide by the red 79 you get 39%. This is his pulmonary pressure as a % of systemic pressure. Improvement since the previous day.
His LA is 7 here. It’s had been floating between 7 and 10 while he was paralyzed. Again, improvement.
They lifted the paralytic to see what would happen, and his numbers went up but didn’t spike. This was a good sign.
By the time we left yesterday, the attending (guard from previous day) said, “Yesterday I was very worried and unsure of his prospects. Today I’d say he’s giving us some indications that he may be headed in the right direction.”
Doesn’t sound like much, but he might as well have been Ed McMahon at the door with our ten million dollar check.
We barely have an indication that he’s headed in the right direction. He’s very heavily medicated right now, and still on a lot of support that he can only have in CICU to keep his pulmonary hypertension in check.
His breathing tube is still in. We’re just starting feeds of 1 ml per hour. (Go measure 1 ml and see how far you’d get on one of those per hour.) We don’t know what will happen as we remove his supports one by one.
And after all that, he needs to have enough cardiac output to eat and grow. It’s going to be a few months before we can really say one way or another how the chips are going to fall.
A lot of chips have to fall between now and then.
The first ones fell Friday after the surgery…and landed on their side.
Yesterday one tipped ever so slightly in the right direction.
Echo of Hope 
[…] forward: This is how I imagine Ari in his battle after his mitral valve surgery from Thursday June 21 to Saturday June […]
[…] for a uneventful life at home for 4 to 6 months. Then Ari will come back so we can balloon the Melody valve to keep it it right-sized as he grows and stay ahead of the […]
[…] when it’s warranted. Nice job on the fetal interventions, dream team. Nice job on the valves, Dr. MacGuyver. Nice job on the bath last week, hot […]
Dear Ari,
I do not know how I ended up reading your blog… It is a middle of the night here in Europe and I should be sleeping because tomorrow is a working day….
(Perhaps I am here now, because I once knew a person called Ari – He was born and lived his life in Finland. Short while before he died, he asked me a favour.. and I did not do it for him. And now it is too late…)
But, what I do know that you have a beautiful smile Ari; and you have such knowledgeable wise eyes, too!
Little Ari: I wish you lots of luck in in the days, the months and years to come: a long, healthy and fulfilling life!
You are in my preyers now.
And, thank you for reminding me what is truly important in life!
Hi there..I came across your post on Facebook through the CHB page…my son was born with critical aortic stenosis and was a patient at CHB. I did not know of my sons condition until after he was born. I am so thankful that we lived so close to Boston where he could get the best help and quickly.. I loved reading your blog because it truly sheds a little humor into the scariest moments of our life. I always thought to myself how no one could quite understand what like is like in CICU or 8 East until you have lived it. It seems like you have a great support team…when I go to sleep every night know that for all times I will be adding your sons name to my prayers as I do for my own son, as he just celebrated his 5th birthday!!! Much luck to you and your family…Stephanie
Hi Ari, you don’t know me and you probably never will. I’m a pediatrician too but from all the way in the Philippines. I came upon this blog through the Boston Children’s Hospital Facebook account. I just want you to know that patients like you are the reason why I do what I do. You give so much inspiration to make me be a better physician. Hang in there little guy! I’ll be keeping you and your family in my prayers.
Ari, as young as you are, you are an excellent role model for us all. Never give up. Keep showing those cardiologists just how tough you are. My thoughts and prayers are with you and your family.
Jen from Maine
[…] Comments « When Did That Happen? […]
I work with your Dad and I pray for your son everyday. Just wanted to say I am enjoying your updates even though I am teary reading them. I would like to thank you for sharing your family and hope you find some comfort knowing that there is one more person pulling for Baby Schultz 🙂
Praying, hoping, praying that you see steady improvement soon, soon.
I don’t know any of you – I’m a friend of a friend, but know that I think of you every morning when I wake and every evening when I go to sleep. I pray for health and grace for all of you. Your family and friends are so loving. Blessings all around. Love you Ari! Your smile is amazing!! ❤
I am praying for Ari and hope he will continue to improve steadily. You and your wife are made of pretty strong stuff! Hang in there and know that others are thinking of the three of you. Fondly, Marlene Goodman
All my prayers are for you.
Mike, Erika and Ari – You are all so fortunate to have one another, you all sound like an amazing team working together toward a common goal. I’m hopeful more chips will tilt in the positive direction, I’d love an opportunity to meet Ari one day. In the meantime keep up the fight and we will keep you all in our thoughts and prayers! –
Sue
Such a beautiful post, Mike. We are praying for you guys. I have faith that some day Thatcher, Elliot and Ari are going to be running circles around us old folks. Lots of love!!
Our prayers are with you. I am a friend of Kelli’s in San Antonio. Our daughter born on Feb 23, 2012 had heart surgery for a coarctation of the aorta on 6/4. We were put in contact with a wonderful support group here in SA, but they have a national reach. Mended little hearts. Here is their website http://mendedlittlehearts.org/
We were put incontact with families that had similar conditions as ours in our area. It was a blessing to talk with others in similar situations, I hope this helps you as much as it has helped us. Many blessings to you and your little hero. Keep the faith. —-Dale
Can’t stop thinking of you and your new best friend …
Praying for your sweet boy!
(Elisabeth Hunter’s sister)
I think you are long overdue for some good news. One day at a time….
Ari and mommy and daddy. What marvelous strength you are showing to all of us. Ari, as Benjamin’s grandma, my sister, said, GET BETTER! I’m Benjamin’s great aunt. I pray for all of you every day. Bless you all. Sigrid
Hi,
You guys don’t have a clue who I am / my name is Joanne and I live in Houston. I’ve been best friends with Lesley Cohen for many many years and the rest of the Schultz family as well. I have been praying for your son and the rest of y’alls family since before Ari was born…
Please don’t ever give up – my step-son was born almost 17 yrs ago with HLHS (heart disorder as well) – and has had multiple open heart surgeries. He is doing wonderfully and has a pretty normal life (aside from not participating in full contact sports)…..so no matter what kind of a day little Ari may have — never, ever give up hope!
I will continue to pray and to send hugs from Houston for as long as they are needed….
Joanne Jumper
Thanks, Joanne. Great to hear about your step son. Ari’s original diagnosis in utero was aortic stenosis with evolving HLHS. The fetal interventions we had at 20 weeks and 26 weeks gestation helped slow the progression of HLHS and save his left ventricle. It’s amazing how far we’ve come. I wish step son and your family the best.
Erica
Ari, you are such an inspiration to so many people. Please, please, please get better. You and Benjamin will be best friends now. Your parents are awesome, and some day you will learn this. Be strong, little lion.
Bless you guys…..think of you all the time….that Ari is one amazing boy with some amazing parents…xoxoxoxo
Mike, thanks for the updates on Ari. It’s because of you and Erica that I check Facebook daily now, just to see how you all are doing. Our prayers and high hopes are with you guys and I’ve got my fingers and anything else I can cross, crossed!!!! Love you guys.
Thanks, Aunt Lesley. Love you guys!
Thanks for sharing, Mike (and Erica). Ari, keep heading in the right direction.
John
Been thinking about you every day since we first saw you at Children’s earlier this month. All three of you are remarkably strong and Ari is a fighter who is giving everything he has. It was inspiring to be around you, meet members of the family, and see the support you have as you navigate through these challenges. The Celtics have their Big Three and we know you are fans. But you are the Big Three in our book. Here to help in any way possible.
Thanks for sharing your life with us, such a moving post once. Ari is on my mind throughout the day everyday. I understand this to be a very critical time and we will keep you in our hearts and prayers. You are doing all that is possible. Sweet Ari your fan base is growing and we are routing you on every step of the way.
Thanks, Brita!
I wonder if you realize how many people that you don’t even know, are praying for Ari and for you and Erica every day. Just wanted you to know…..
With love and prayers, Carol Cohen (Jill’s mom)
Carol,
It’s amazing how many people, people we don’t even know, have reached out to us and have Ari in their prayers. We are so thankful for all of the support.
Erica
Thank you my wonderful son in law, Ari is truely a lucky lucky boy to have you as his Dad and Advocate. Appreciate the “heartfelt” story of your love and positioning to “fatherhood”. AND appreciate the indepth discription of our “little boy’s” struggle for LIFE. For so precious it is, it is everything now. xxx
Thank you, Mike. I couldn’t help but have tears roll down my face as I read this. The world is praying for your brave boy. I so wish I could give you hugs of strength and encouragement. You have got to be beyond exhausted. Much love to all.
Missy
Don’t tell Toby or he’ll be mopy! 🙂 Love that! So true! Pull through Ari, Benjamin needs you!
Toby’s found a new lover in Grammie Linda! Miss you guys and hope Benjamin is doing well and loving being home.