Long overdue update.

So after being very confused about how Logan can be doing so great heart wise and yet still be blue and breathing heavy, I pushed Dr. Patel to do an echo sooner rather than later.  For my own piece of mind I just couldn’t wait another 2 weeks.  My biggest fear was that he had pulmonary vein stenosis once again.  This is what caused us to have the last pop-up open heart surgery.  unfortunately, if this does happen to Logan again we will most likely have to put him on the transplant list.   I was A WRECK waiting for this echo.  I mean really a complete nervous wreck. 

 Luckily Dr. Patel was able to squeeze us in fairly quickly.  Once we got there the nurses immediately commented that he looked bluer (thank you this is what I have been saying – Mom’s know these things) and once his pulse ox was taken it became very apparent, his oxygen is now around 72-73 instead of 80. 

(watching Elmo while getting his echo done)

Long story short Logan heart still looks good.  There is NO pulmonary vein stenosis that is visible.  His last sutureless repair on his pulmonary vein stenosis still looks excellent, his Glenn looks great, his valve is still holding up.  The only thing is that he still has relatively small pulmonary arteries.  So most likely because his pulmonary arteries are on the smaller side blood is finding alternative paths to travel down i.e. collaterals.  Heterotaxy kids are known to have abnormal vasculature so this crazy collateral growth is more common in kids with heterotaxy.   But keeping in mind the perspective that he could have had pulmonary vein stenosis, I was hugely relieved.  I mean collaterals….come on,  that’s just a cath fix. 

So the plan right now is to do nothing until Logan’s PO (pulse ox or oxygen saturations) drops further he is going to need a cath to coil more collaterals off.  This poor baby’s chest is just full of platinum (that is the metal used in coiling) his chest x-rays look really bizarre with over 25 metal coils in there right now.  I can’t imagine what some doctor one day is going to think when they do a chest x-ray and they don’t know about him. 

And then there was the G-Tube drama.  I just can’t get over how something that is typically no biggie in 99% of population quickly becomes so complicated and life threatening to a child with heterotaxy.  It was a one situation that clearly illustrates what Logan is going to have to struggle with for the rest of his life.  So to begin with, the pediatrician (who by the way- should we win the award for best pediatrician EVER!!!) had set up a conference call between Dr. Patel (cardiologist), our GI doc, myself, and him to discuss the big picture plan for Logan and to go over different contingencies (this phone call was one of the most helpful things we have had during Logan’s care and with a whole post in-an-of-itself) but one of the unanimous decisions that was made was that Logan needs a G-tube put it to help him gain weight. 

You would think that this would be a pretty straightforward and simple task to get accomplished.  Well, not for a heterotaxy child.  As it turns out his mixed up abdominal organs make placing a G-Tube much more risky.  Because Logan has a small misplaced stomach and a hiatal hernia he is at a very high risk for aspirated milk into his lungs. Because Logan is asplenic and any major infection could kill him the last thing we want is milk sitting in his lungs and causing an infection.  So to avoid this risk a fundiplication surgery would also have to be done. 

(fundiplication illustrated)

Basically wrapping the top half of the stomach around the the bottom half of the esophagus. 

*Excerpt taken from WebMD.com

How Well It Works

Studies show that laparoscopic fundoplication improves GERD symptoms in about 6 to 9 out of 10 people who have the surgery (depending on how experienced the surgeon is). But no studies have proven that laparoscopic fundoplication surgery is effective in maintaining healing of the esophagus over  the long term.1

  • A successful surgery does not guarantee that you will never have symptoms again. Some studies show that only about 1 out of 10 people who have fundoplication surgery done by an experienced surgeon have symptoms come back in the 2 years after surgery.2 But there isn’t much research on how many people have symptoms come back after more than 2 years.
  • About 2 or 3 out of 10 people who have surgery to relieve GERD symptoms have new problems (such as difficulty swallowing, intestinal gas, or bloating) after the surgery.2 These new symptoms may or may not respond to treatment with medicines.

Risks

Risks or complications following fundoplication surgery include:

  • Difficulty swallowing because the stomach is wrapped too high on the esophagus or is wrapped too tightly. This complication may be more likely to occur in people who receive fundoplication surgery using a laparoscopic surgical technique.4
  • The esophagus sliding out of the wrapped portion of the stomach so that the valve (lower esophageal sphincter) is no longer supported.
  • Heartburn that comes back.
  • Bloating and discomfort from gas buildup because the person is not able to burp.
  • Excess gas.
  • Risks of anesthesia.
  • Risks of major surgery (infection or bleeding).

 

All I can say to the above information is NO THANK YOU.  Logan has enough problems as it is.  And to do a surgical procedure for aspiration which may or may not even happen and potentially end up with a child who can’t swallow?  Not to mention the fact that some studies are showing only a 60% success rate?  And these risk are actually very minor in comparison to the major risk would be how Logan’s heart would handle such a large operation.  So needless to say,  Dr. Patel and I had a long talk about this and decided very quickly this is not the path we are ready to embark on.  So after countless hours on the phone talking and discussing and go back and forth between the pediatric general surgeon, the GI doc, Dr. Patel, the pediatrician, and myself.  We all settled on the fact that the once unanimous decision to have Logan get a G-Tube is now a resounding NO.  

  We are very lucky to have such an experienced and insightful pediatric general surgeon on our team.  He was a surgeon at CS Mott’s in Ann Arbor MI (one of the nation’s top Pediatric Heart Centers) before coming to the Cleveland Clinic and has tremendous experience with him.  He has experience with heteortaxy kids and has seen how quickly things can ‘get messy’ with them. 

A little side note here on second opinons……GET THEM.  This whole situation clearly illustrated the importance of finding a surgeon with actual experience with the type of illness you have.  I have spoken with 2 general surgeons and 3 GI docs to get a team I felt comfortable with. 

So after I had finally excepted that Logan was going to get a G-Tube I began picture him growing, and starting to sit up, and develop at a much increased pace and I started to get excited about the idea….until it was pulled out from under me. 

As Logan is quickly approaching the one year mark, I have been particularly emotional.  It is so much to take in, where we have been in this last year, and the road ahead as well.  The other issue with his 1 year mark  coming up, is that I have been avoiding calendars, timelines, charts, ect… in hopes of not having to worry about the comparison.  The comparison to where he is and where the average child is.  It is heartbreaking to see a baby almost a year old just start to have enough strength to hold his head up and for my own piece of mine I block out all the comparison charts and just accept him for him.  With his birthday looming it pulls me out of my world and into The 1 Year Old World.  The world of 1 year olds is crawling, walking, talking, waving, peek-a-boo, babbling, clapping, eating, sitting in a high chair, ect…. Logan can’t do any of those things.  In terms of physical strength Logan is a 3 month old.  This has been so tough on me that I have even considered canceling his B-Day party. 

 

I have come around though and accepted that Logan is very special and been thru a lot and it would be a shame not celebrate his life, because even though he is very small and weak, he has fought battles larger than most of us fight in our life. 

Logan’s birthday party is open to anyone in that would like to celebrate his life with us. If you are interested in coming on Logan B-Day Party which will be on Sunday June 5 please leave a comment with your email address and I will give you the details.  I look forward to meeting some of my fellow heart Mom friends that I only know thru words.

12 thoughts on “Long overdue update.”

  1. Oh WOW.There are no words to explain what you have been through this past few weeks.My heart goes out to you.You have been through so much.Unfortunately we will not be able to attend his party.
    But wanted to say we think of your family all the time.We are so happy that you have a great team.That is so important for our LO’s.
    Take care.

  2. Kristin, I can only commend you on how strong you and your husband have been through this past year. I worked with your mother and continue to follow Logan’s progress through your blog and my heart goes out to you for the strength and commitment you’ve shown.

    Logan is a very special child and fortunate to have such dedicated parents continually working in his best interest.

    I will continue to follow Logan as he turns one, which seems to be a milestone in his life. He has put up a good fight and my thoughts and prayers are with you always!

    Thinking of You! Therese Schustrich

  3. Kristin,

    I love that you are going to celebrate his birthday – you’re right, he’s fought harder battles already that most of us ever do in 50 years or more. It’s such a wonderful thing that you can mark on that calendar that you’ve had this amazing little boy in your life for a year, and are getting ready to start another one!

    Don’t worry about comparisons – every child is different in one way or another. Didn’t your mother ever tell you not to judge yourself by what everyone else is or does? Logan is your sweetie boy and that’s all that matters. One year is just a celebration, of all that he’s made it through, and all that you have made it through. Hopefully that one-year reminder will stay with you of the strength you both have shown.

    I won’t be able to make his birthday but hopefully can see you in mid-June, I am pre-planning a few days in the area and would love to see you both. Have a wonderful, wonderful celebration with him and your friends and family – every birthday is a celebration 🙂

    Thinking of you often!
    Nichole

  4. Kristen, you are a pillar of strength. Even through all the difficulties I have faced in my life, I could never come close to what you have had to endure. We must celebrate this special little guy because he deserves to be celebrated. He has brought so much to you and all that know his story. Every time I look at a picture of him or see him in person I get an overwhelming feeling of hope and joy. He brings out a special feeling that no other child can. Perhaps it is because I know he has so many struggles behind him and ahead of him, yet he still has the most wonderful little face that shows this intense smile. I am very much looking forward to joining you and seeing him for his celebration. I love you so much and am so grateful to have you and little Logan (and Ethan and Scott) in my life. Much love, Ann. 🙂

  5. Lottie is also Hetero and will celebrate her 1st birthday in July. She doesn’t sit, crawl, talk, eat food or the other things associated with 1 year old. But Lottie laughs and smiles and breaths (with oxygen) and is a joy everyday. We spent her first Thanksgiving and Easter at the hospital, while she recovered from surgery. My daughter drives 2 hours one way to the specialist and hospital. I have seen the fear and tears in my daughters eyes when the diagnoses was made at 24 weeks gestation and again last week when Lottie is throwing up and she can not help. I have seen the joy and smiles last week when Lottie was medically cleared to have a baby bottle. My daughter focuses everyday on the blessing that Lottie is alive and that Dr’s are there to help.

  6. Oh, Kristen, reading this made me cry. I remember those feelings with both my youngest and oldest who were not doing anywhere near what most kids their age were doing. I know my children’s heterotaxy is not as severe as sweet Logan’s but they eventually caught up and I will pray that your beautiful Logan does, too. He has just been through so much, all of his energy has been spent on recovering instead of learning new things. You are a wonderful mother to him & such a fantastic advocate for his care. He is a very lucky boy in that respect. May God bless and comfort you. -Teresa

  7. Hey Kristen. I’ve been following your posts quite a bit lately. Our son, Jack, was born on June 4, 2010 with an VERY similar set of conditions to Logan (Right-Sided Isomerism, DORV, Common AV Valve, post-Glenn, Fontan to come, etc.). He and Logan must be almost the exact same age, and I’m amazed to have stumbled upon another case so similar. Jack has been treated at Duke thus far. Like you, we’re finding that the extra-cardiac issues caused by Jack’s heterotaxy are much more problematic than we initially thought. Jack was fed via NG for the first 6 months. Due to chronic vomiting/reflux–and the entire feeds lost to it–he received a Jejunostomy (J-tube) at 6 mos. Like Logan, the location of Jack’s stomach made a G-tube and a Nissen problematic. Jack’s reflux and wretching, though, continues, as his stomach continues to produce acid despite the absence of food. Jack is hemodynamically stable (from a cardiac standpoint), but it’s the GI issues that are keeping him from moving forward with feeding therapy and physical therapy. Like Logan, Jack is staging out at about 3 months of age. From the outside, he looks GREAT, but he can’t tolerate tummy time, rolling over, etc. He can’t take oral food without wretching. We’re about to get a second opinion at Cincinnati Childrens’ on his GI issues. I’m at the same point–even–with Jack’s birthday party. I’ve thought of just canceling the whole thing because of the hour-by-hour “limbo” that we seem to live these days. I don’t reach out too often, but your words just leapt off the computer screen. We’ve found that there’s no real standard of care yet on how to handle feeding with these little guys and their atypical organs (after the life-saving cardiac issues have been addressed). Please feel free to contact me. BTW: Will you be at the Heterotaxy Conference in Chapel Hill on 06/25?

  8. I started following your website when my newest grandson was diagnosed in womb with heterotaxy last Nov. He was born in April and thank you God, has many fewer problems than your beautiful little boy. He has a normal spleen, had his abdominal surgery shortly after birth, is eating normally, and his first heart surgery will be postponed until he is 3 – 6 months old as his oxygen level is in the 80’s. He was able to come home at 2 1/2 weeks and only needs oxygen at night to maintain levels in the 80’s. Know that Logan is in my prayers as are you and your husband. Having had 12 healthy children I can only imagine what your last year has been like. May God be with you and your beautiful family and I will continue to pray for you and Logan daily.

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