Jax’s Heart

 

When Most people think of “heart disease” they do not think of babies and Children

In the United States Approximately 40,000 babies are born each year with a CHD. That means 1 out of 125 babies are born with a heart defect.

Making CHD’s the most common of all birth defects

There are approximately 35 different types of congenital heart defects

More than 50% of all children born with a CHD have defects serious enough to require at least one invasive surgery

20% of these children will not live to celebrate thier 1st birthday

*warning video contains some graphic material*

 

 

more about “Our Miracle Jax“, posted with vodpod

 

Our son Jackson was born with a congenital heart defect. His condition was discovered when he was 4 days old. He was born with Total anomalous pulmonary venous return (TAPVR).  Normally the blood in your heart is suppose to leave the right side of your heart and return to the left side of the heart.  In Jax’scase, the blood takes a bad route (via extra vein), mixes with the blood on the left side of his heart and returns to the right side through a hole in his heart, that without which he would have died. Therefore the blood that is pumping though his body is only partially oxygenated. Which explains why his oxygen saturation was always low.
They do not know if TAVPR is a genetic condition or if it is random defect that occurs during the 8thweek of pregnancy.  Most likely it is a combination of both. Due to the fact that the defect is in the back of the heart it is not picked up during routine ultrasounds. Jackson was lucky in that he had the “best” form of this condition, Supracardiac Total Anomalous Pulmonary Venous Return. 

Jackson’s Story

Here is the very long story of Jax and what we went though in the days leading up to and following his surgery. Updates on his condition are at the bottom, in case you can’t make it though the entire thing. This first part was written prior to me putting this page together.

Jackson came into the world quickly on only the second push. He was perfect, 7lbs 14oz withbigchubby cheeks. When he was born he was grunting and the Dr’s and nurses kept saying it was because he came out so quickly and that he still had fluid in his lungs that wasn’t squeezed out during his birth. He continued to grunt and a few hours after delivery a nurse on the Mother Baby floor decided to do a pulsox and discovered his oxygen level was very low. They rushed Jax to the nurse’s station for closer evaluation. When his stats were not improving they moved him to the NICU. Incidentally the initial reading was wrong, but his numbers were indeed low, just not as low as initially indicated.

They expected him to be in the NICUfor just a few hours, but his oxygen saturation levels were not coming up. After a chest x-ray he was diagnosed with TTN, which is basically wet lung, and was officially admitted to the NICU. They told us his lungs would clear up in 48hrs and that he would most likely be discharged at that time. As the x-ray showed the fluid going down Jax’s stats were still not where they needed to be. He was on and off oxygen support for a few days but even with the extra oxygen his numbers were never right, always just a little off. He had also developed jaundice and was now under photo light therapy.

Finally after being strung along for four days with no answers and no definite guide as to why they were keeping him in the NICU a nurse heard a heart murmur. An echocardiogram was ordered and he was quickly diagnosed with TAPVR. Within hours he was transferred to Johns Hopkins where his open heart surgery was to take place.

That day was the Sunday after he was born, March 9th what would be the worst day of my life thus far.
The day had started out with so much hope. I had stayed the night in the NICU with him because we were told he was probably going home the next day, and they wanted to see him nurse well though the night. I didn’t hardly get any sleep because you technically cannot sleep if you stay overnight there.  Prior to this day we were not really able to hold him expect for feedings and we had to cut out visitors. We were ready to go home hold our baby and start being our new family of four, as well as finally show him off.
Mike arrived early that morning withthediaper bag containing the coming home outfit and the car with the second carseat now safely installed. The excitement and relief was short lived though. A nurse came in shortly after Mike arrived to tell us that Jax would not be going home that day. His oxygen levels were still off and they had heart a heart murmur for the first time. They were concerned that something may be wrong with his heart and that more tests were needed.
Mike and I both lost it. Mike showed his pain and concern in anger. “Why are they keeping him, there’s nothing wrong with him” Neither one of us was prepared for this because at no time had anyone indicated that we were waiting for better oxygen saturation levels or that they were worried something else was going on. We thought we were going home.

I remember going to the small bathroom (the only place for privacy) calling my Mother and bawling hysterically that we were not bringing him home (incidentally this would be one of the few times I cried during the entire ordeal and the only complete break down). I had no sleep, been discharged two days prior, hadn’t really seen Ethan, needed a shower and was dealing with Mike being so very angry at the situation. Oh and I had given birth 4 days before, emotions and hormones ran very high that morning.
My Mom said they would be out to the hospital with Ethan shortly and even though I didn’t really want them to come it was what I needed. We were now back in the holding pattern. They had ordered an echocardiogram and told us it would either be that day or the next depending on when they could get someone to read it.

Doing what my parents do best, they showed up with reinforcements. Ethan, Jeremy, Nicole, one of my best friend’s Michelle (bringing a few small toiletries for me), and gifts for baby Jax.  We all decided to go for lunch and Mike’s Mom Judy was on her way as well. We went over to the mall and I remember just being numb as we sat there eating lunch. I went back to the hospital by myself anxious to get back to Jax, Mike stayed with thegroup to spend some much needed time with Ethan.
When I arrived back at the NICU Iknew something was up as there were several people buzzing around his room and for the most part we had been ignored, because no one really thought there was anything seriously wrong with Jax. One of the very nice nurses was on that day and met me in the hallway, she said they had done the echo on Jax and that a Dr. would be calling for me to talk to about the results. I could tell it was bad, just by the look onher face. I said you have to tell me if they found something, I understand if you can’t give details, but you have to give me a heads up. She was nice enough to tell me that yes, they had found a problem with his heart, and that the good news was they could fix it.  That was all she could tell me and that a Dr. would be calling me soon.

Soon came in about 5 minutes and Mike still was not back, so I fielded the call by myself in Jax’s tiny NICU room. Pen and paper in hand I was trying very hard to listen, get the correct information, and process what was going on. From this point on I was pretty much on auto pilot. I remember him telling me that Jax had TAPVR describing to me what it was and that it would require open heart surgery within the next few days. We needed to make a decision on where to send him, either to Virgina (where this Dr. was) or to another hospital. Three quarters of the way though the call Mike walked in…thank God! Support! I circled open heart surgery on the page for Mike to see and finished up the call as quickly as possible.

Things moved pretty quickly at this point. We had about an hour to decide where to send him. After some quick phone calls to some great friends, because we had no idea where to send him,  we made the decision to send him to Johns Hopkins. We called family though I don’t remember who or when. I do remember standing in the hall outside of Jax’s room calling my other best friend Alli, who was in Oregon, and telling her that Jax needed open heart surgery and to please call my Mom for more info becuase  I just couldn’t talk about it. Within three hours of lunch the team was there to transport him to Hopkins. They had to re-hook him up to iv lines, oxygen lines, and other monitors for the transfer. This part was very hard on me as he had been off all support for 24hs, and I hated seeing him poked and prodded. Finally they had him all hooked up and ready to go and said we should go home for a few hours and meet up with him at Hopkins later that evening after he was settled. He was getting his first, and lets hope last ambulance ride, lights, sirens and all. I remember telling them to drive very carefully.
I went home for the second time since giving birthto a quiet house filled with flowers, gifts, and no baby. We went to Hopkins for the first time that night and Jax was in the NICU there. What a different atmosphere from Anne Arundel. We met with a Cardiologist who had done another echo when Jax arrived and discussed the game plan. Mike and I were very impressed as it was late on a Sunday night and yet all of this was happening. He was only in the NICU for a few hours before they transfered up to the PICU, which was where he would be post-op.  We went home tired, weary, and numb from everything that happened in that one miserable day.

The next day Mike and I met with a million people, Dr’s, nurses, surgeons, anesthesiologists, all preparing us for the “big day”, March 11th, surgery day. We had to sign consent forms to allow Jax to have his surgery and Mike stepped up and signed them for I could not bear to do so. Throughout all of this I was still planning on breast-feeding Jax so every 2-3hrs I had to pump. This was our excuse to slip away just the two of us and either talk uncensored or sit in silence, what ever was necessary. I think we both really looked forward to this time “away”. My Aunt Jodie flew in from NY that day to offer much needed support and love.
March 11th, what a hard day. Mike and I drove to the hospital in near silence for if we talked the tears would start. We arrived super early as we were hoping to be able to hold him before they took him. He was still receiving light therapy for his jaundice and since he was having surgery he was not allowed to eat either, so there were few times when we could hold him. The nurses were nice enough to let us hold him that morning.
Jaxwas really fussy as he was hungry and craving to be held more. When it was time to wheel him to the operating room they put him in his little bassinet and we walked down the hallway, Mike’s arm around my back for support, following our baby and his team, most of our support group was already there standing in the hall. Because he was so fussy at the last minute they decided to let me carry him into the actual OR. We changed into scrubs and they placed him in my arms. I remember looking at him and  wanting to run away with him. But I carried him down the hall, Mike right beside me and laid him on the operating table. I stood there looking at him and was frozen, I literally could not move and did not see anything other than his little face. I felt that if I moved the world would shatter around me. Finally a nurse nudged me along and we walked back into the arms of our family and friends to begin the long wait.

I have never in my life felt as loved as I did during this time. I knew I was loved and had good friends, but I never realized how really loved we were. We had so many people praying for us,  helping us with our house, animals and Ethan. I was able to focus on Jax and did not have to worry about anything else. Our amazing support team stepped in and took care of everything!
That day our support team was what my brother Patrick called “Team Haslup”. We actually got kicked out of the OR waiting room because there were too many of us. There waiting and praying with us as our baby underwent the fight of his life was My Mom, Dad, Mike’s Mom, Nana, Aunt Susie, Bob, Aunt Jodie, Jeremy, Patrick, Tina, Michelle, and Jimmy. We could not have been more loved or supported then we were that day. And more friends had offered to come and be with us.
The surgery went quickly. We got a call that they had made the first incision, then the one when he was on the bypass machine and then the longest wait was for the call that he had came off the bypass machine. When we got that call we decided to go grab a quick lunch. What a scene. I ate quickly and was ready to get back up to the waiting room. I had a feeling news would be coming soon. So Mike came with me (we learned our lesson) and sure enough Mommy intuition was right. About 5min after getting back up there the surgeon came in and told us he was done. Everything had gone great and they would be wheeling him by to recovery soon. By the time they wheeled him by, Team Haslup was standing against the wall and we all saw him for the first time post-surgery. He looked pretty good for what he had just been though.
The next few days went quickly. We had a few minor bumps in the road, some very emotional times, but Jax did remarkably well. Alli flew in from Oregon to be with mefor a few days and that meant the world to me.  And slowly with all of our friends and family at our side Jax got better and better. They said the average stay was 7-10 days post surgery, but Jax, beingthe super star that he is, came home on day six, St. Patrick’s Day! Mike had gone to work that morning because while we thought he might come home we did not want to be let down the way we were before. He met me and my Mom at the hospital (I was very rarely alone, people were always with me), and we finally were able to pack up our new baby and take him home. When we walked out I was afraid they were going to say no, sorry you really can’t go, but they didn’t. I drove Jax home alone that day and as I did I thought about how blessed I was to be taking home this amazing child who will forever be our miracle. 

 

*UPDATES*

His first follow up with the cardiologist went well he was growing and gaining weight and his heart looked good.

 The next cardiology appointment, about a month post-op, I took him by myself.  Bad idea. When they did the echo they found mild stenosis along the stitch line. Its basically a narrowing of the vein at the site where they reconnected the vein to his heart. So now we were in another waiting pattern. As Jax growsthey need his heart and that vein to grow with him or there will be blood flow issues.  Right now there is a bit of turbulence, but his heart is squeezing well and his lungs are not affected.

We had to go back about a month later to check on the stenosis, Mike came with me this time, and the stenosis has stayed the same. Meaning it hasn’t gotten worse, but it hasn’t gotten better either. We are now waiting for his next appointment.
We were suppose to go back this week, but the appointment was cancelled and we now go back August 12th. The wait is very hard on me. After the last appointment we did call his Dr. and got a few more answers on what this stenosis might mean.

He said that while he hasn’t seen this situation too often (TAPVR is a somewhat rare CHD), he would say that in just a bit more than 50% of the cases the vein does not grow and it does require another surgery.

He was encouraged that even though Jax gained weight between the two appointments the stenosis did not get worse. He also said that as long as the stenosis doesnot affect either the lungs or function of the heart he can have the turbulence with out having corrective surgery. So I was ok with all of that.

Now, the bad part, if the vein does not grow and it does require surgery it would be another open heart surgery. They tend to not do the heart catherizations with this type of stenosis because of the location of the vein in the back of the heart. If something were to happen and they were in the “cath” lab it can be hard to control the bleeding, where as if the surgeon can see what he is doing he can better control the situation. So that part pretty much sucks.

Some warning signs that something is wrong would be Jax not feeding well and not gaining weight (clearly not a problem at this time), low energy, and labored breathing indicated by sharp intakes of breath, seeing his ribs, and him shrugging his shoulders.

At this time we have seen none of this in Jax. We are praying for a clean bill of health next month. Though I have a feeling we will never quite get that clear. I don’t believe the stenosis will ever require another surgical repair, but I don’t feel it will completely grow out either. I guess time will tell. If we do get the clean Jax can lead a normal life with no limitations on activities or sports. He will need to see his cardiologist until he is grown, but at that time if there are no other complications he will be completely cleared.

 *UPDATE* 8-14-08
We had Jax’s cardiologist appointment on Tuesday and it went as expected. He continues to have the mild stenosis across the stitch line.  He continues to have some turbulance in his heart due to the stenosis, but it is not causing any other problems at this time. They will continue to keep a close eye on it and we will go back in 3-4 mths. At that time we will go back to Hopkins (he has been seen at a Hopkins outpatient center in Odenton) and they will do a sedated Echocardiogram. He has echo’s done at every appt. but a sedated echo will allow them to get a closer look at everything with him laying completly still. I am hoping at that time they will clear us for 6mths.   So now we just continue doing what we are doing, love him, watch him grow, and pray for the best.

Update October 15th
I posted on the main site as well. But Jax had his sedated Echo bumped up due to a few concern we were having about his behavior. He had been fussy with no ear infection and no teeth coming in, started spitting up/vomiting after eating and was sleeping more. After our regular Dr. found nothing wrong and the behavior continued for about three weeks we got worried that something else was going on and emailed the cardiologist. After talking with him he decided to move up the sedated echo appt. which had actually been pushed back to Decemeber 8th.
Mike and I were very nervous heading back to Hopkins for the first time yesterday, but in the end we got great news! While he still has the stenosis, or narrowing, across the repaired vein, it has gotten better and is no longer causing turbulence though the heart! His heart is pumping nice and strong with no backup and the blood flow through the heart is much smoother. Yeah!! Mike and I knew during the echo that it looked good b/c you could see the difference on the screen.
Jax took the sedation very well though he was hard to wake up and ended up sleeping most the day away.
We go back at the end of January for our next appt. but right now we are thrilled that we are headed in the right direction and can most likely avoid another open heart procedure.
Thank you for all of your prayers!

*Update January 27*
Jax had his cardiology appointment today and it went great!!! They did a quick echo, which is hard with Jax moving so much, and said that his heart looks normal for a heart with his type of repair. He has no limitations and and we do not expect any further complications! I cannot even begin to tell you how thrilled we were. We received similar news at the last appt. but this time everything has stayed the same and he looks great! The Dr. kept commenting on how he didn’t look like a heart baby and how advanced he was.  I was beaming! During the Echo he kept waving and saying Hi it was soo cute! He was cleared for ear surgery and we meet with the ENT next month. Hopefully we can get a set of tubes in him and be done with all of this ear infection nonsense.
We go back in 6mths! This is the longest we have been cleared for and will probably be seen every 6mths until he is around 2 years old and then we should be able to go annually.
Praise God for a normal heathy heart!!

Below are great sites with information on CHD’s

Little Hearts

The Congenital Heart Defects Family Association

The American Heart Association