The bright yellow form in our hands was our ticket home - to finally experience life as a new family. DJ was exactly one month old. We had all been through hell. The first month of his life was very traumatic and we were so very happy to be heading home.
The bright yellow form was confusing. It listed stuff like Left Isomerism with Polysplenia, Situs Ambiguous, Heterotaxy Disorder, and Ivermark Syndrome.
The bright yellow form made my stomach churn. I hated the words malrotation of the intestines, congenital heart defects, bypass of the duodenum, and Ladds procedure. That is what my 9 day old baby boy endured. Those terms meant pain, heartache, worry and more pain to us all. My stomach churned just thinking of the long nights and days and hours and minutes we had spent in the NICU not knowing if DJ would make it or not.
The bright yellow form also made me smile. I was finally taking my little boy home and he was no longer sick, in pain or dying.
The bright yellow form made me cheer. There on line 4 was the medical term "congenitally corrected". YES! To this day that expression makes me smile. It is music to my ears. It simply meant that while DJ's internal make up was strange, abnormal, the impacted organs (for the most part) had corrected themselves to function, differently from the normal Joe, but function!
Even though that bright yellow form contains lots of information and medical terms, it does not tell the entire story. DJ's remarkable story. And one day, I will recount to all of you his amazing story.
Today's post is not about the struggle, the surgery or the time in the hospital. It is not about the agony a mother feels when her child is in pain. It is about DJ. I know that his medical condition does not define him, or make him who he is, but his condition is a part of him. And sometimes, for all good reasons, we forget that he is different. When you look at him, it is hard to imagine how different and unique he really is!
At first the doctors thought DJ had a simple malrotation of the intestines. This occurs in 1/200 births. They did an upper GI test and confirmed that there was a blockage of his intestines. But when they opened him up, boy, was everyone surprised! Most of his internal organs are misplaced or not formed properly.
Over the last 4 years we have become very knowledgeable and even intimate with the terms Left Isomerism with Polysplenia, Situs Ambiguous, Heterotaxy Disorder, and Ivermark Syndrome. But here is how one of the many doctors working on DJ's case explained it to us:
His body tried to create two left sides. This syndrome primarily affects the asymmetric organs, including the heart, lungs and bronchi, liver, intestines, and spleen of the body. In DJ's case, his heart, lungs, intestines, appendix, gallbladder, portal vein, stomach, liver and spleen are all impacted.
We were told this syndrome occurs in an estimated 4 per 1 million live births. Approximately 80% of patients with polysplenia have congenital heart disease. Mortality can result from congenital heart disease, biliary atresia, intestinal malrotation, or sepsis.
In one large retrospective review from Canada, the 1-year mortality rate was 32% and the 15-year mortality rate was 49%. The incidence of polysplenia has not been studied extensively, but it appears to be rare, comprising less than 1% of congenital heart defects. In a 26-year retrospective study at the Hospital for Sick Children in Toronto Canada, only 91 cases were identified.
Looking at DJ now, you would never even guess something was wrong or different. We like to say that he is PERFECTLY IMPERFECT!
When DJ was 9 days old we rushed him to the hospital. We knew something was wrong. He was very ill. Surgery at nine days old found that his main tummy vein, the portal vein grew through his duodenum, therefore the doctors placed a shunt just above the opening of his stomach to create a bypass of the obstruction. Normally that part of the intestines is 25 inches long, DJ's is only about 4 inches long. During the surgery they also repaired a twist in his intestines. Since his gallbladder and appendix had switched sides, they removed his appendix. The main reason for this is that if he ever had an appendicitis, he would have complained of pain on the opposite side and it would probably not have been diagnosed as such, and he could die from complications - better to have it out right away!
Most people have a tri-lobed lung and a bi-lobed lung - both of DJ's lungs are bi-lobed. We are not sure if this will cause issues in the future. Since both DJ's lungs are "left-sided lungs" their intake capacity is not as much as having one tri-lobed lung. This may mean he is not an Olympic sprinter or marathon runner!
DJ's stomach and liver are what they call mid-line, this means that they are out of place and more in the center of the abdomen.
In terms of DJ's heart - well - WOW - is all you can say. DJ has 4 main heart defects:
1) Dextrocardia - Basically, his heart is on the opposite side and flipped backwards. This in of itself is not serious and is more common than one may think.
2) Narrowing of the pulmonary Valve - this causes a mummer since this main valve is blocked a bit.
3) Complete Interruption of the Inferior Vena Cava. The inferior vena cava (or IVC) is the large vein that carries de-oxygenated blood from the lower half of the body into the heart. In DJ this is completely blocked - not good! But in his body, (AMAZINGLY!) a small vein the size of a thread, in a normal body, has re-routed itself and has taken over this function!
4) Congenitally Corrected Transposition of the Great Arteries - this means that his left and right ventricles have switched position and are doing the opposite job than they normally should. DJ has not needed any heart surgeries because his has "corrected itself" and is functioning at good levels. The heart specialist explained it in terms of leg muscles: pretend that the quad muscle now has to do the work of his calf muscle and visa versa. Over time his quad muscle, a nice big muscle would get lazy since not much work is being demanded of it, and the poor, weaker calf muscle may get tired quicker since it has a more demanding job to do. I thought this was a brilliant explanation!
For those who are visual learners, click HERE to see a drawing of a normal (or as we call it) boring heart. Click HERE to see a diagram of DJ's amazing heart!
Every day we hope and pray he has no heart complications. Though we know that stats seem to indicate that DJ may need a pace maker at a young age. DJ is monitored at the hospital and has yearly heart tests.
The final component to DJ's syndrome is polysplenia. This means multiple spleens. Normally, there is one present: DJ has "six sizable spleen, and countless others'.
I had a very hard time understanding this, especially since the doctors indicated that based on a blood test, they believed he has some splenic function. One doctor said that they are not sure if one of the spleen is working or if they are all working together. So, in my head I envisioned all these little "kidney bean" shaped organs, all lined up together, jumping rope. (I know, strange!) So, I asked the doctor if his spleens are all together, trying to look and act like a regular spleen. Nope. They are all over his abdomen! CRAZY! Since we do not know how well DJ's spleens work, he must take antibiotics every day as an infection could be very serious for him.
Well, that is it in a nut shell! Very hard to imagine and to believe. Especially when you look at him now. He is a healthy, vibrant 4 year old.
I cannot believe that four years ago today, we walked out of the hospital with our little baby. Back then, we were not sure what we would face. We did not know if DJ would lead a normal, active life. Well, we have been blessed. And we thank the Lord and all the wonderful doctors every day! CHEO saved my baby's life.
I am proud of the following previous written posts - Mommy Moment (September 5, 2007) and That Means You are Special ( August 17, 2007).
Click HERE to read the newspaper article featuring DJ and our family.
DJ just days after surgery
DJ improving and showing his true personality - or jsut gas!
January 10, 2004 - going home!
DJ today - well, actually July 13 at his last heart check up!
I promise one day to write a bit about our hospital experieinces, as I have so many stories in my head that deserve to be shared and preserved.
8 comments:
What an amazing story! You definintely have a little fighter on your hands. So precious. My son was born with a transposition of the great arteries as well as a hole in his heart. We too were feeling the way you were when those discharge papers were in hand!
Wow - what an amazing story. Thanks for sharing that Laura.
You guys have gone through a lot to keep your little guy healthy. What a blessing.
Holy cow, he is a miracle! It's amazing what you are able to learn when it is about your child isn't it? I'm sure you never thought you would be an expert on something like this. I'm so glad that he continues to do so well!!!! The human body is just so amazing....
Oh, my goodness. I have this image of little spleens scattered about, tucked neatly behind other organs. How terrifying that must have been for you!
And, how much it says about you that it has just become a part of your everyday life.
Wow! What a miracle he is! Your family has to be so strong to go through something like that and see the positive -- that he has an amazing heart.
Thank you for sharing that amazing story. My daughter was diagnosed with congenital heart disease at the age of 1 month. She had 3 holes in the heart. Fortunately those had been surgically corrected. And she's a healthy 3 year-old now.
My little grandson has left isomerism with polysplenia. We knew he had a unique heart and arteries in the 'wrong' place, but recently his mother, aged 35, had volvulus and malrotation. She survived but it caused the cardiologist seeing Ben to carry out extensive tests. He does not seem to have malrotation but he has polysplenia and more tests to come. He is so far very well and healthy normal baby. Your story fills me with hope and joy.
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