March 16, 2021
The new information and detail is below. Some you received this in an email as well. The video is from our meeting with Dr. Haw a couple days ago. He is describing the multiple things that need to occur in the procedure. Additionally the Nuss procedure will be added on to the end of the entire heart surgery. Basically they are going to repair the Aortic Root, the Aortic Valve and the section of the Aorta that connects to the Aortic branches at the top of the heart. (kind of 3 things in one), plus the Nuss chest reconstruction. This video explains some of the surgery and Dr. Haw has some great interaction with Ayden. It is so great that Ayden's entire team of Doctors is willing to interact with him and allow him to speak, ask questions etc...
This is a lot to take in. Hopefully this video and the links below will give you some more detail and context.
From March 11, 2021
Hello family and friends.
This is not the news I was hoping to be sharing with you all. Please sit down for a long and difficult read.
THE CHANGE IN PROCEDURES
As most of you know, Ayden needs to have a procedure to correct the Pectus Excavatum in his chest. The sternum is pushing down on his already taxed heart and lungs and it must be surgically corrected in a surgery called a "Nuss"procedure. However….. among the many steps in preparing for this he had an MRA scan. We and the Cardiac team were blindsided that the results showed his aortic aneurysm has rapidly grown since his last MRA and is far beyond the safe numbers, and is at risk of dissection/rupture. Of all the tissue problems that can arise because of Loeys-Dietz Syndrome, Aortic dissection is the most serious. We hoped and prayed that this would never become an issue for Ayden but it has.
Ayden will need to have open heart surgery in the next couple weeks.
After a week of tests and meetings Ayden’s team of doctors have concluded the surgery will take place Monday, March 29. There will be other tests and preparatory items between now and then. The surgery is a complicated one. It is basically 4 in one. They will be replacing the Aortic Root, the Aortic Valve, the Section of the Aorta connecting to the Aortic Branches, and then also piggybacking the Nuss procedure at the end. This extensive procedure will remove his entire aortic root and other damaged aortic tissue, (which must also be disconnected from the branches of the aorta). This will be replaced with a new artificial “Dacron” tube that will be then be attached to all the top branches of his Aorta. His coronary arteries will be unattached and operating with machines during the procedure.
Additionally, as if that were not enough, we have learned that Ayden’s Aortic Valve also needs to be replaced. Dr. Haw and we have elected to do this at the same time. His valve is leaking and is just going to get worse. This will be replaced with an “Onyx” carbon fiber mechanical valve. A real tissue valve replacement was also an option, but theoretically, the Onyx valve can last a lifetime if proper blood thinning is followed, so we opted to go with it. Unfortunately this will require Ayden to be on a blood thinning regiment for the rest of his life along with regular blood draws etc… But lifetime blood draws were already in the cards for him. The blood thinners are new though.
Antegrade Cerebral Perfusion (ACP) is a cardiopulmonary bypass technique that uses special cannulation procedures to perfuse only the brain during aortic arch reconstruction. During part of the Aortic procedure Ayden’s circulatory and pulmonary functions will be rerouted to external machines to allow the surgeons to work on the heart. For a period of 20-30 minutes the tissue will also be cooled to 22º Celsius in order to elongate the safe time for the items to be worked on without regular blood flow.
The “NUSS” Procedure
Now in addition to all of that, Ayden STILL needs the “Nuss” surgery to correct the Pectus Excavatum. To complicate matters, he can not have the Nuss procedure before this heart surgery as his body would not have time to heal, and he can not have it after the surgery as the chest wall closure method after this surgery will not allow for additional corrective surgery. So, they are going to piggy-back the Nuss surgery at the end of the heart surgery. When Dr. Haw and his team are done, before they close the chest, Dr. Marc Schlatter (Pediatric Surgeon) will be coming in to perform the entire chest reconstruction and repair of the Pectus, then the entire team will close him up together…. Recovery time will hopefully be 24-48 hours in ICU, 7-10 days in hospital, 90 days of various recovery levels, at home.
The good news is that Ayden still has, literally, the best medical team in the world for his illness. His team of 22 doctors, which includes Dr. Hal Dietz of Johns Hopkins (discovered Loeys-Dietz Syndrome) is adding a new member, Dr. Marcus Haw, Congenital Cardiac Surgeon and Pediatric Cardiothoracic Surgery Specialist, from the University of London, UK, and is the Chief of Cardiothoracic Surgery at Helen DeVos Childrens Hosptial. Dr. Haw has performed over 9000 open heart procedures and hundreds of the aortic type surgery Ayden needs. It is important to note that he has also performed this surgery on patients with Loeys-Dietz Syndrome and its cousin disease Marfan's Syndrome. This is very important as patients with LDS have a much different set of issues throughout the body than a standard patient. Dr. Haw is aware of this and experienced in exactly Ayden’s needs.
Link for Dr. Haw: https://findadoctor.spectrumhealth.org/physician/profile/1211
Link for Dr. Schlatter: https://findadoctor.spectrumhealth.org/physician/profile/2613
Link for Dr. Hal Dietz: https://clinicalconnection.hopkinsmedicine.org/participant/dietz-iii
Glass Half Full
As you can see in the video Ayden is still typical “Glass-half-full” Ayden. He is calm, collected, and engaged. We could not be more impressed and amazed at how Ayden takes all of this in stride and at 13 acts as his own informed participant and advocate, and really understands his illness and the medicine. His response to us upon learning all this was “Well that was not on my bucket list, (corrected to Life List :) but we knew it might have to happen sometime. At least we can get it all done at once”. His younger brother Alec (11) said, “It’s Ayden..., he goes through stuff all the time, he’ll be fine.” God has blessed both of these boys with such amazing attitudes, positivity and strength. Ayden and Alec have been virtual schooling at home last year and this year and both maintain straight A's! Although you’d think it’s the parents that keep the kids calm, many times it’s the other way around. We are very blessed. Additionally Ayden’s Doctors like Dr. Haw are so wonderful with him. They listen and engage directly with him, not just with us. His team is literally second to none. Recently the boys have gotten into the older Star Trek movies and they just saw "First Contact". I joked with Ayden with his Dacron Aorta, his Carbon Fiber Onyx heart valve, and his Stainless Steel Nuss bar, he's going to be a Borg, lol. We try to keep the humor wherever we can. Not gonna fake it thought, we are anxious and tense. This is the most serious thing we've had to experience thus far on his journey, and we will be seeking for and believing for peace, calm, and success.
Alec and Ayden will be spending the next 14 days at home, safely relaxing and preparing for this next challenge. We will not be going out pre or post surgery to keep Ayden as healthy as possible, especially from things like Covid and other viruses. We will also not be accepting visitors for the same reasons. We know you all love to cook for us, but we’ll be cooking in-house during recovery until Ayden is not susceptible to outside illness.
We are so blessed to have an amazing support system of you family and friends and will be appreciating all the thoughts, prayers, positive vibes and love you throw our way. Ayden says he can feel them and we know that it’s true. We’ll be keeping up with emails, posts, texts as we can.
Thank you for being part of Ayden's Journey.
Your thoughts and prayers mean everything.
We love you all.
Here are some good links to the LDS related items above:
LDS Characteristics https://www.loeysdietz.org/en/medical-information/#characteristics
What is Pectus Excavatum? https://surgery.ucsf.edu/conditions--procedures/pectus-excavatum.aspx
What is Aortic Root Replacement? https://www.mainlinehealth.org/conditions-and-treatments/treatments/aortic-root-replacement