Vascular Ring : Double Aortic Arch

posted in: Apollo, Special Needs | 46

I won’t leave you in suspense. Before I go into the details of our trip, Apollo got his diagnosis. He has a vascular ring and specifically a double aortic arch. It is exactly what we suspected, but had to push so very hard to get a doctor to test for. Sharp blog reader, Lorrie, first brought this to our attention probably six months ago. Her son has the very same rare birth defect and was TEN YEARS OLD before he was diagnosed. Lorrie, feel free to leave a link to your post in the comments if you like.

Now on to our trip.

As I said yesterday, we headed down to Seattle Tuesday night because we were determined not to miss this appointment that we had pushed so hard to get. I am so thankful for my mom who braved the snow, came out to the house and spent the night with ten children so we could go a day early. Thank you mom!

In some strange, cosmic coincidence, my brother and sister-in-law from Alaska are in Seattle right now as Laura gets treated for cancer. How strange that our medical needs would coincide like that. It gave us the opportunity to meet up with them. Laura got to meet Apollo for the first time. Neale and Laura will be down here several months as she receives treatment. Please pray for them. They had to leave their three children (ages 12, 13 and 20) in Alaska while they attend school and work.

Apollo had a very rough morning. We woke him up at 5:30 am, the last time he could eat solid food. Being Apollo, he barely ate anything. I was soon regretting the decision to wake him. But I also knew I would have regretted not trying to feed him if I just let him sleep. He did a lot of crying in our hotel room as he asked for food and to nurse and we had to say no. He did take an hour and a half long nap and then pretty much cried until we left for the hospital.

He did enjoy the novelty of a walk in the snow as Chuck checked us out of the hotel.

We had to check in at 11:30 for his 12:30 MRI. They checked him in and took him back promptly.

They put the identification bracelets on his foot and then we didn’t see anyone again for an hour…I mean no one. Not a nurse, not the anesthesiologist. No one explained how we were going to proceed.

At 12:35 I walked out into the hall and let them know we had been in the room for more than an hour, no one had talked to us yet and we wanted to know what was going to happen. I wasn’t rude, just assertive. No more sitting around and being the “model patient” for me.

At 12:40 a nurse practitioner was in the room, telling me they were behind because of the weather. Okay, but they need to let us know. And  there was no reason to have Apollo in that tiny room with no distractions instead of in the equally empty waiting room. If it was me or Chuck, no big deal. But we had an 18 month old who hadn’t eaten since 5:30 am! She explained what was going to happen and took his  history.

At 12:52 the anesthesiologist came in (yes, I was taking notes). At 12:55 he was finally taken back.

At 1:36 the anesthesiologist poked her head into the room and said, “They found something! I don’t want to tell you what and steal their thunder, but they found something and it can be fixed!”

At that point I wanted to cry with relief. I was so afraid they were going to do the MRI and say everything looked fine…and I knew everything was not fine. And then I felt guilty for being happy that they found something. Who’s happy that the doctor’s find something wrong with your child? Sometimes being a mom is really hard.

At 2:20 they wheeled him back into the room. I immediately noticed the airway in his mouth (it was a small one, just about he length of my pointer finger, it just held his tongue down and opened the top of his airway). The nurse said they had to put it in because as they were doing the MRI he would start thrashing around “like he couldn’t breathe“.  Oh. My. This is my son who thrashes around at night like he’s in a boxing match. I’ve suspect for months his sleep issues were related to his inability to breathe easily.

I asked when we would find out the results from the MRI. We were told use the results should be in by that afternoon, blah, blah, blah. At one point I asked Chuck if he was going to tackle the anesthesiologist or was I? She knew the problem, after all.

As it turned out, neither of us needed to accost her, because the cardiologist who helped read the MRI walked in. He introduced himself and said they found something but it was “complicated”. Apollo would need more testing, he needed to see a cardiologist. “Right now I’m wearing my radiologist’s hat, but I should put on my cardiologist’s hat” he said. Finally,  finally he said, “he has something called a vascular ring”. He talked about that for a bit and I asked what type and he said a double aortic arch. He was surprised we had heard of either. It is a very rare heart defect. He said he would require surgery, but because the heart developed this way, he may not have a normal sized airway. It might have developed small because of the vascular ring.

He was very surprised we were there for an MRI at all. He said he’s never seen a patient get an MRI for a vascular ring without it being ordered by a cardiologist. “It is highly unusual that he hasn’t seen one yet.” In other words, our persistence, and our doctor’s willingness to order the MRI himself, just shaved weeks, maybe months off of Apollo’s suffering. Apollo is now listed as a heart patient who needs treatment. The cardiologist said there were a lot of things that needed to happen “behind the scenes” and that he would get them moving. The surgeons would meet with the pulmonologist who would meet with the cardiologist (whenever Apollo sees one). “These things need to happen anyway, and I’ll get working on them” he said. He tried to arrange for us to see one right then, but because of the weather they had only  a “skeleton crew” working. He asked to examine Apollo (who was still sleeping) and we were more than happy to let him. Once Apollo woke up and wanted to nurse, the cardiologist left and called our doctor right then. Unfortunately, his office was also closed due to the weather. But here is a doctor, a cardiologist, who feels just as urgent as we do!

While we know Apollo has a double aortic arch, we don’t know about his specifically (hence the need for more tests). I won’t go into all the possibilities, but there are definitely better and worse ways to have a double aortic arch. We will be spending a lot of time in Seattle seeing doctors and having tests. And it will culminate in (closed) heart surgery to repair the defect.

I asked the cardiologist how many vascular rings they operate on per year at Seattle Children’s and after hemming and hawing for a bit and saying “I don’t want to quote you the wrong number” he finally said, “It’s not in the double digits”.

Thank you so much for your thoughts and prayers. I have more to say, but I’ll save it for another post (this one is long enough). If you have questions, feel free to ask and I will try to answer in my next post.

For those of you interested in learning more here are some resources:

Double Aortic Arch- while this articles says “double aortic arch is a common form of vascular ring” that  is not to be confused with a common heart defect. Vascular rings represent less than 1% of all congenital heart defects- double aortic arch just happens to be the common type of vascular ring.

Here is Seattle Children’s hospital’s page: Vascular Rings

And for those of who aren’t faint of heart, here’s a video of the repair of a vascular ring.

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46 Responses

  1. I’m so sorry it’s been such a long struggle, but I’m so relieved you have a diagnosis at last. I am keeping you all in my thoughts!

  2. So relieved you finally have a diagnosis! Thanks for the update. Praying for a quick cardiologist appointment.

  3. Echoing Emily & Kate, I am so glad you have a diagnosis finally! And way to advocate for your son. There really isn’t an excuse for leaving you hanging for an hour when you could have been in a more comfortable place for him. I’m hoping you got home safely.

  4. It’s wonderful that you finally got a diagnosis. I don’t think you should feel guilty about being glad the doctors said there was something wrong. You weren’t happy because there was something wrong. You already knew something was wrong. Your happiness was because the doctors finally noticed and agreed to do something about it. Those are two very different things! Getting someone to notice the problem and fix it is definitely something to rejoice over! Hope the rest of his tests and surgery go as well.

  5. So glad you found an answer and Apollo’s problem is Treatable 🙂 keeping you all in my thoughts.

  6. Thank you, Jesus, for answers!

  7. I’m so glad you’re finally getting some answers! And treatable ones, at that.

    When we were waiting and waiting for our (then) newborns diagnosis, I also reached the point where I WANTED something to be found so we had a chance of helping him. (He had a rare-ish type of epilepsy which had been misdiagnosed earlier. At the height, while we were weaning from one med to try a new one, he had 140 seizures in one day. Now he is an eight year old who has been seizure free for seven years, and med free for nearly six years. Because of answers.)

  8. So delighted you have an answer, and a “good” one, as far as answers go (treatable!). I was worried this would be another type of congenital heart malformation leading to pulmonary hypertension, a much LESS treatable disease. And now that he’s on the cardiologists’ and surgeons’ radar, things are going to move along in setting up his treatment. Prayers of thanks and continued concern for you and your sweet boy.

  9. I am so happy that you have answers… I cried knowing that as a parent it has hard to be happy for knowledge when it is not the best news, but that there is relief in KNOWING. Praying as you await the steps ahead!

  10. Brenda Colvin MD

    I am so very happy that you have found your answer. I know that you don’t know me – I met your older kids at Mountain School- but I feel that I know you through your blog. I have been thinking about Apollo and his problem daily and praying for the answers to come your way. I am a retired physician in Spokane, and sometimes it just kills me when I can see a problem when other docs just give folks the run around or ignore them completely. It is as I expected it to be (see July 24th blog replies) and have almost been ready to come over to the “other side” of the mountains to fight the fight with you. I am so sorry that it took you so long to find out, and I am so glad that you were your child’s advocate to see this diagnosis to the end. This is such a treatable problem and you should see immediate improvement in Apollo’s life after his repair. You are an amazing mother which is why I follow your blog. Apollo is blessed to be in your family. I look forward to the day that I read about his repair and how it will change his life. Keep up the vigilance and assertive attitude. My prayers are with you. P.S. Sawyer Colvin says hi to Tilly, Judah, Enoch, and Adalia.

    • Brenda, thank you for your input. I don’t think I will ever understand why it took so long to get this diagnosis. And we wouldn’t have it now if we hadn’t of pushed and pushed and pushed. At least he’ll be getting the help he needs now.

      Sent from my iPad

      • Brenda Colvin MD

        You have done all the right things and made all the right decisions concerning Apollo. I am very impressed with how you have taken care of him. When I was in practice, I quickly learned to listen to a mom who just KNEW that there was a problem with her child. One time in particular there was a new baby who passed her newborn physicals with flying colors, but the Mom was just uneasy about her baby. She was a first time mom, and they are particularly easy to “blow-off” as being highly anxious. But in this case she really felt a sense of urgency about her baby’s health despite NO symptoms of distress or problems. So, I slowed down, listened very carefully to her concerns, and did an unusually methodical examination – and you know what? I found a subtle abnormality in the physical exam and sent the baby directly to a cardiologist who did the studies that diagnosed a major cardiac defect. The baby was medevac’d to the states from Germany where she had her heart surgery and she did just fine. The moral of the story for me was — listen to moms when they have a concern. Moms are given a special gift of discernment for their children. Now that I am out of practice I tell all my friends to make noise just like you have for Apollo. be just fine throughout all of the upcoming trials. closer so that we (our families) could be “real” friends.

        • Brenda- thank you so much for your input! It helps to have some encouragment. My passive-patient days are over. That ENT probably did me a favor by ticking me off. I am now Tiger-Mama when it comes to all things medical.

  11. Simon Schmidt

    Hello,

    when I read your text just like I have shot the tear in the eye! Because my older son who is now already 4 years is the same in his childhood had. But the doctors have seen him in quite early.

    Many heartfelt thanks to us so that you can turn to participate

  12. Although the diagnosis sucks he is in good hands, we just got back from the big city up here (ack while on the mountain pass they stopped allowing cars onto it the snow was that bad I made it off safe and sound I went early to avoid missing the appointments and the results were also not great (but hey we know now!) K’s is fixable but she needs 2 feeding tubes to proceed with treatment. Apollo is a fighter like K and there is now light at the end of the tunnel

  13. So glad you have confirmation! Praying for a smooth journey through repair and recovery!

  14. So glad you guys finally have a diagnosis! And it is fixable 🙂 Praying that the double arch is the ‘better’ kind, so to speak. Would you mind if I asked for prays for Apollo from my church cell group?

    Best wishes,
    Emily

  15. I am actually in tears reading this. So many emotions! While I am so sorry you have all gone through this, and know how scary anything wrong (especially with the heart) can be, I am SOOOO thrilled you finally have not only a diagnosis, but a doctor who is pressing so hard for getting him treated ASAP. I’m praying for all of you and will continue.

  16. http://semiorganizedmom.blogspot.com/2009/08/story-of-our-heart-baby.html

    Here is the link to Brenden’s story. He was 11 and a half when he was diagnosed. Brenden had a Double Aortic Arch & Vascular Ring. The way his aorta split was also complicated and he will always have a narrow esophagus due to the way it grew and also the length of time it was constricted. Both sides are very narrow, so they just chose the best side to reattach it to. He’s 18 now and still seems to get upper airway infections (sinus, bronchitis) with most normal colds. He still gets a build up of mucous and has to cough it out, like someone who has Cystic Fibrosis. Colds that last a few days for us, last a couple weeks for him and sometimes he needs steroids to help open up his airway. He also complains of sharp stabbing pains where they did the surgery…especially under his arm. I’ve been sharing Apollo’s journey with Brenden and he is praying for him and says, “He’ll get lots of ice cream in the hospital!” Apparently that’s what he remembers. 😉

    The extra testing that Brenden needed the month prior to his surgery was an EKG, blood work, ultrasound and something else. They wanted to see exactly how the blood was flowing and that’s where they found the PFO, too. I’m sure they needed lots more info than that, but those tests seemed to cover what they needed.

    Renee- when you read my blog post, don’t miss the part where I prayed, Please find something! Please find something!” as they were testing my son. I felt the same way you did. I didn’t want them to find nothing because I KNEW there was something wrong with my child…that doesn’t make me sick, nor does it you. I remember the moment I was free to talk to my husband. My husband was always extremely supportive, but also didn’t quite have the same urge that I did to find something wrong with our son. He didn’t come to the appointment because it was only supposed to be an appointment…the tests were what I begged the doctor for and he gave in. He had watched thousands of dollars be spent on medications, ER, After hours care, pediatrician and specialist appointments. We had spent 11.5 years being told this and that and my husband knew that’s what this appointment would be, too. I should have called him to see if he wanted to come up, but I guess I thought that maybe those tests would fail my son as well. It was embarassing for me and I always felt bad that he missed work for such a crappy appointment. I wish he had been there that day….I really needed someone to lean on, to be there with me. :*(

    Anyway, I used a hall phone and called him at work. I was shaking from adrenalin, euphoria and pure fear. I had just been vindicated. I was no longer the crazy mom. I could finally check “Munchausen by Proxy” off my ‘Am I Really Crazy?” list. Not that anyone ever accused me of having that, but when your child is sick and doesn’t ever seem to get better and you think it’s something terrible, but the doctors say it’s “asthma” or “allergies”, you start to question your own sanity. Does that make sense? Who am I kidding? Of course you understand!!! 🙂

    God is so amazing and I can’t stop Praising Him for how he orchestrated all of this. I’m here for you Renee. Mama to Mama…we share a very rare bond that I will never forget. If you have any questions or just need to talk, I’m here. Email me and I’ll send you my phone number if you want it.

    I’m praying ceaselessly for you guys and I know that God has BIG things in store for Apollo.

    XOXOXOX

    Lorri

    • Lorri Dean

      I just read this again and realized I said he had a narrow esophagus, but failed to mention he also has a narrow trachea…and tracheamalacia.

  17. Hooray for an answer! Double hooray that it is treatable!

  18. Tara Bergeron

    I am so glad you lost your nice and got your boy the help he needed. I just can’t express my joy that he was fast tracked, with your persistence, and a diagnosis was made. It is awful to think how he has suffered for 18 months. Yet the emotion of knowing relief is on it’s way. It’s hard to be so far away from family and not being able to help. WE LOVE YOU ALL lots and lots. Praying daily for your needs.

  19. I feel horrible saying this but I am so happy for you that you finally have a diagnosis. I’ve been reading your blog occasionally for a while since I came across your family’s story about a year ago — I, too, was struggling with a little peanut who didn’t nurse well. I’m so glad you have an answer, and I’ll pray that it’s easily fixable. Poor little Apollo. I hope he gets some relief soon.

  20. Joan Moline

    I am so glad you are finally on the way to some relief for both Apollo and your two. I can’t even imagine how you have coped so well. Thank goodness you were so persistent, Renee!
    I have only been following your blog since last spring when I met Keziah.
    You are truly an amazing family. May God continue to bless you and watch over you. May the doctors you have be competent, wise and understanding of your whole struggles and in their plan to help Apollo.
    thanks for sharing you lives in this way.

  21. I have been following your blog for quite a while now, but I don’t usually comment. Lots of times I read while nursing at the keyboard, which doesn’t help with typing. 🙂 I just felt I needed to comment this time. I have a daughter a month younger than Apollo, and I would have felt similar to you in them finding something. At least it is treatable, and there is a way to go from her. I am so relieved that there is an answer. 🙂

  22. I’m so glad that you’ve finally gotten a diagnosis. I’ve been lifting Apollo and you and Chuck up in prayer and cried when I read your post. It’s entirely due to the efforts of you and Chuck that you’ve finally gotten an answer. I will continue to pray for all of you and appreciate the way you’ve filled us in so thoroughly (I even watched the film). Hoping that it won’t be too long until Apollo can have his surgery and get some relief.

  23. So happy that you’ve got some answers at last. Will be thinking of you, and hoping for the ‘best case scenario’ now, and for a relatively easy resolution.

  24. So glad that you finally have answers!

  25. Yay! What a blessing to have some answers and a cardiologist who is off and running to start the wheels moving. And a pediatrician who listened to you and just ordered the MRI instead of waiting and waiting for the cardiologist to be available. I read the links above and I have to say the symptoms seem to be very clear so how in the world does a child who exhibits every symptom keep getting kicked around from doctor to doctor with no answer. Praying for his tests to show the “better” way.

  26. I know exactly what it is like to know something is not right but no one believe you and think you are trying to create a sick child. That was many years ago for us.

    BUT my grandson has just been given a “probable DAA” diagnosis as well. He is two months old and did not thrive. He is improving now but had to regain to do further testing. I am sorry you are facing this but knowing is better. After all, as he gets older it can cause terrible problems.
    I will look forward to following your blog. It is strange as after Owen came home from the hospital he went to his country doctor and they have two other children with the same thing.

  27. I have been reading your blog lately and just wanted to say how happy I am for you and your son that you finally have answers. Although it’s a rare birth defect, I still can’t believe it takes this much effort to get a diagnosis in the year 2012. I have a similar diagnosis of a Right Aortic Arch and a Vascular Ring and my mother had to fight to get doctors to listen as well, but that was in 1975, I was three years old. Luckily, they listened and I had a repair then as well as another follow up repair later. I can tell you from personal experience that your son with be forever grateful for your fight to help him have a healthy and happy life. The time of surgery will be hard, but it’s worth it.

    Best wishes to you and your family.

  28. I’m so glad you now have an answer. Apollo has been in my thoughts for many months. I do hope that he can get treatment soon and will then be a much happier little boy!

  29. So happy to hear you now have answers!

  30. Hi There…felt compelled to comment after stumbling across this blog. I had surgery to correct my double aortic arch 25 years ago, when I was just 3 months old. Though I did have ongoing bouts with pneumonia and 2 incidents with croup, I have continued to lead a full and normal life. Though I’ve noticed that many websites suggest future limitations on exercise and dietary restrictions, I have not experienced any such need. I was 4-year college athlete (women’s lacrosse), and continue as an avid runner. I have been diagnosed with cough-variant asthma, possibly a lingering post-surgical outcome. However, it’s been well controlled with an inhaler and self-monitoring.

    Anyways, I just wanted to leave a note and wish your family well. I’m sure it’s a scary time dealing with a sick child, however the worst should surely be over! Clearly I can’t speak on behalf of everyone who has ever been diagnosed with DAA, but I can say for me, this medical anomaly has not hindered my development or my life. All the best to your family and little one!!

  31. Hi, I am constructing my website, so please check back periodically. Your child is lucky to have gotten a diagnosis, early in life. My son, 19, was diagnosed just last year, after injuring his wrist. It was serendipity! They found a right aortic arch with aberrant left subclavian artery (vascular ring) and a kommerell’s diverticulum (aneurysm). He had a very successful surgery, six weeks ago at Hopkins. Because his esophagus was constricted by the ring, for his entire life, the next step will be to normalize (as much as possible) his swallowing and digestive process. I am setting up my website to bring awareness to this congenital malformation. It is rare and often overlooked. I want to connect patients and the medical communities to bring this to the forefront of medicine. Don’t stop asking questions.

    • bakersdozenandapolloxiv

      Adriana- thanks for your comment. Apollo’s case is very complicated. His left subclavian artery is no longer working and he has a compressed esophagus which required a g-tube to be placed in May. The doctor’s still don’t know what’s compressing it since he’s had his double aortic arch divided. Let me know when your website is up, I’d love to check it out!

  32. […] to thrive. He had his adenoids out and a bronchoscopy and we still had no answers. He was finally diagnosed with a double aortic arch through an MRI on January 18, […]

  33. I know I’m very late posting this, but I wanted to share. I had this exact congenital defect, double aortic arch with vascular ring when I was born, almost 40 years ago! Most doctors look at me funny when I tell them my past medical history, and I have to explain what it is and what it means. I have a much bigger scar than your little guy, around my shoulder blade to the front near my sternum and a scar where my chest tube was. It was diagnosed when I was a month old, because, as I’m told, I would ‘turn blue’ when I was fed. A constricture was around my trachea and esophagus. I had the surgery. I spent the first six months of my life in a hospital incubator, battling pneumonia and complications, but I have been good since. I have served in the army, regularly exercise, and am very active. I have never had any problems with it. I hope Apollo is still doing well too and hope he lives a normal and active life.

    • bakersdozenandapolloxiv

      Thank you for sharing your story! It is always so encouraging to here stories of people doing well.

  34. […] reality is, Apollo would not have gotten a diagnosis and treatment without my firm advocacy for him. One of the most important things I have done is educate myself on […]

  35. […] I can tell you the exact day it all went down and his anxiety began: January 18th, 2012. He awoke from his MRI (his second time under anesthesia in a month) with anxiety. After his first heart surgery March 7th […]

  36. […] of most difficult typical issues (not heart defects, FASD or hearing loss) we have dealt with in our parenting has been disrespect in the early teen and […]

  37. […] January 18: MRI and diagnosis of double aortic arch. […]

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