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Monday, February 25, 2013

Baby Joey's rare congenital heart defect(cor triatriatum dextrum)

want to share your view privately? email me at MYOWNOPINION@WOH.RR.COM

                              It was on September 19 when our nightmare began. Baby Joey was born in January of the previous year and at 20 months old we were about to discover that he was born with a congenital heart defect that had gone undetected and if it weren't for the intuitiveness of the nurse on duty we may have never found the cause. Let's start at the beginning.:
                    Baby Joey came into this world a happy and very healthy baby. The pregnancy was a good one with  no problems medically. He came into this world at 9lbs. 11oz. and 21 1/2 inch in length. This was on Jan. 12th and for those of you familiar with the APGAR rating system, he received a 9 and then a 10. Both mother and father so happy and proud, looking into the baby book at the first thoughts of the parents , mom wrote:"Thank God for a normal and healthy baby boy". He grew like a weed as babies do and all through that first year and beyond he was so healthy, one would never suspect this child was about to be at deaths door.
              Shortly before all this took place(few weeks), Joey slowly started to not want to eat everything on his plate. No reason for worry as many start to develop this and as days and weeks passed he was getting more and more dependent upon people to carry him instead of walking on his own and then not wanting to eat at all. He then started to be kinda lazy as to playing with his toys as far as lying on the floor and sometimes only looking at them instead of playing and we then started to be concerned.We decided to make a doctors appointment the next day and my wife went on to work for the evening and I took Joey to his grandmas for a birthday party for grandma. He wouldn't get off my lap all evening and wouldn't even eat any ice cream or cake(his favorite) and was just listless. Mom(Joeys mom) came home from work and we took him to the emergency room where after some tests it was decided to keep him overnight in a croup tent because x-ray showed a little fluid in his lungs.
            Over the course of that night and the next day, a series of tests were done and a couple doctors looked him over and it was said that there was a murmur in the heart and the EKG came back slightly abnormal but nothing to worry over.My wife was concerned with the swelling of his feet and ankles and the his legs which were growing so fast you could almost watch them.The nurse on duty would come in and check his vitals and my wife would express her concerns over his cough worsening and the swelling and at one point when I summoned her to the room she told me she also was concerned and had made arrangements with our doctor to have him life flighted to Toledo, Ohio at the Medical College of Ohio. This was because they had a ICU for pediatrics
                 This was mind blowing, you bring in a healthy child who you think is only ill from a childhood illness and less than 24 hrs. later are making arrangements to get to a Medical college known for their work in pediatrics.Okay, my wife made plans to go now so she would be there when he got there as per the nurse and I stayed to wait for the helicopter.I kept carrying him to the nurses station to alert them to his breathing or something and so they placed me in a rocking chair with him in the nurse station. After an hour passed, I inquired as to their ETA and was told a large accident had both copters held up but a new service was available and they had called them. Well, one had not opened up for business yet and the other was fogged in but, one of the 2 busy ones was now freed up and on the way.About another 45 min. later I was told they were forced to turn back because of a thunderstorm and winds so now we would go by ambulance. I then made plans to ride with relatives behind the ambulance and a nurse came in to make the ride with him as I did not want to be in the way. The driver invited me to ride with them and I told him we would just follow them, he told me not to even try to keep up.They turned onto the entrance ramp to the highway and we waited for 1 other vehicle to turn and when we did, we never saw the ambulance the whole trip.Going the speed limit, this would take 1 1/2 hrs. to make it there.We made it in about an hour and my wife said the ambulance had been there and gone about a half hour. This was a 60 mile trip and this guy drove through wind,rain,thunder and lightning and it was at night. I never knew who drove that ambulance but, my hats off to make a 60 mile trip in about a half hour, no wonder he told us not to try to keep up. I thought I had conveyed to my wife that the nurse in the hospital told me that Joey was in heart failure but she says I didn't and was surprised when they told her that  her baby was the one coming in with congenital heat failure and she said no, my baby has a mild cough and some swelling(remember we are in our mid twenties)
                    We were introduced to a cardiologist who was the one the original nurse had spoke to. I knew we had a special doctor because when I asked the nurse at the first hospital in Lima if they were aware we had no insurance because I had been on lay off she said yes and the doctors reply was "let's save the child and worry about who's gonna pay later". Anyway, he told us that he had them give him lasix which is a diuretic and he had went all over on the trip up so, his kidneys were still functional and he had also given him something to make him sleep so they could do a echo cardiogram to see why but, Joey was still wide awake. Which he said we could take as a sign of what a fighter he was. So he had my wife hold him to rock him and he refused to sleep so the cardiologist said lets see if we can do it while he was awake.
                       If you have ever seen a tv series either comedy or drama taken in a hospital where there are always students around with the doctor, this was exactly like that. We were in the middle of the pediatric ICU with about six or eight students all writing and asking questions and answering questions the doctor would ask of them. So they do the echo cardiogram and the doctor(Dr. Erlich) says okay, do you see it.? I wasn't sure what I was seeing but he wasn't talking to me. Yes came the answer and then a whole lot of tech talk and Dr. Erlich said he would explain in a minute and gave some orders to the nurse and we walked out of the room.
                       Dr. Erlich sat us down and drew a primitive sketch of the heart and told us that as the heart forms, it starts as many tubes, some being veins and some being arteries. They twist and turn and separate and fuse together and all sorts of things  and as they fuse together to form the chambers, the walls that divide them as separate tubes dissolve to form the chamber and in Joey's case the wall above the valve that sends the oxygenated blood to the body had started to dissolve but only slightly so there was basically a thin membrane over the valve in the reservoir section acting like a dike with a hole in it so he was getting some of the blood needed and as he grew he needed more blood flow and wasn't getting it.This dike was causing the blood to back up in the lungs and not letting the blood that needed the oxygen into the lungs. He then handed us a paper to sign allowing him to run a tube from his leg to his heart and release a dye to watch his blood flow to confirm this.He explained the possibilities of puncturing the heart but a team would be ready if anything went wrong and the most dangerous thing was not to do it because till they know, they can't prepare and without surgery , Joey would surely die. Basically he would drown from fluid back up in his lungs which is what would have happened had we waited any longer before bringing him into the hospital.
Needless to say, I grabbed the pen and signed. What choice did I have?

End of part one.


  The doctor said before anything could be done they would have to get as much fluid from him as possible so he was giving him diuretics and to maintain his heart rate he was given dijoxin  They readied him and the sight was terrible. He looked like some of the starving children you see in the videos wanting money for food charity and hard to bear. He was taken in and had the catherization done and then put in private room. We made arrangements for a room at Ronald McDonald house but we only kept our bags there. I could not believe how many parents were leaving their sick children there and come in to check from time to time.We made some acquaintance with other parents who felt the same as we did also.We became accustomed to sleeping in an upright position in a chair and would wake from time to time to go in and relieve my wife and sleep in a recliner. It was  hard to friend someone and then have their child not make it. Not just hard, it was horrible. 
          After the heart cath we had to keep an eye on him for a while and then he seemed to be fine although, that was not the case.The only thing keeping him going was the medication. We went to speak to the heart surgeon to find out his method and he would answer any questions we might have.He started by telling my wife that this was not anything she did or ate during pregnancy as this defect was around long before red dye #5 and so forth. When we then questioned about it's rarity he said it is very rare. He was brutally honest telling us there were only 5 known cases of this in the U.S and of those only 2 survived after surgery. He also said this was so rare that there was nothing in any text book to show us as he normally does to allow parents to better know what their child has.He informed us he had never performed this surgery but had been in touch with a place in N.J. where they have hearts from organ donators to study and they had a heart with this defect. He was planning to go there and do a dry run. The very biggest worry to him was getting it done in short time as possible as the biggest problem was they have to hook his body to a machine that would pump and oxygenate his blood while they work but if on that machine too long, the heart would not survive and so would not perform after being fixed. He did stress that afterward, if all goes right, that he did not forsee any need for medication as he was only cutting into his reservoir chamber and not the muscle that does the pumping. We walked out knowing this had to be done but when the thought of your baby boy having his ribs split open and his heart being removed and worked on, and you look at him now that all the fluid was gone and he was becoming normal acting, you just pray. You pray a lot.
          I have just recently been able to find informative description of this defect:
Cor triatriatum dextrum is extremely rare and results from the complete persistence of the right sinus valve of the embryonic heart. The membrane divides the right atrium into a proximal (upper) and a distal (lower) chamber. The upper chamber receives the venous blood from both vena cavae and the lower chamber is in contact with the tricuspid valve and the right atrial appendage. The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chambers. If the communicating orifice is small, the patient is critically ill and may succumb at a young age (usually during infancy) to congestive heart failure and pulmonary edema. If the connection is larger, patients may present in childhood or young adulthood with a clinical picture similar to that of mitral stenosis. Cor triatriatum may also be an incidental finding when it is nonobstructive. The disorder can be treated surgically by removing the membrane dividing the atrium.
      When we asked why there was no sign of this earlier we were told that actually him being a little older before it showed as a newborn would have some more trouble and would in all probability succumb to the defect. At the time ,it appeared that he was getting enough blood flow until he had a growth spurt and then the supply was not enough. The brain we were told is like the CPU in a computer and when it noticed the heart having trouble, it told the stomach not to be hungry as digestion process is hard on the heart. Also the kidneys had started to shut down until the use of diuretics.
          A couple days passed and we were given counsel on the fact he may not be coming home with us given the fact the mortality rate was not good.They had to take him off his heart medication in case that during surgery he would need a jolt of it. On the eve before the surgery, Baby Joey began to run a temperature which by late night got to 101 and Dr. Davis(heart surgeon) cancelled the surgery because if there was other problems it would not help for success. Surgery was re scheduled and again on the day before, he started running a temp. and Dr. Davis called in the CDC who did not make an impression with my wife. He began asking her questions like does he walk backwards which she snapped back no, he walks normal when the question was can he walk backwards and they were taking measurements of his head and chest and conferring among themselves and my wife was getting very impatient and looked at me and said "get Dr. Ehrlich here now".I went and found him and they all came out and told us a lot of mumbo jumbo to which Dr. Ehrlich looked at my wife and asked if she understood any of it.She shook her head no and the good Dr. then said me neither and I'm a doctor.He then turned to the spokesman of the CDC and said say it all again but use words we can understand. Well, it seems they thought he had spinal meningitis because there was some cases in the hospital and he could have that because of his head and chest size and the temperature and all.To which my wife then gave them his measurements at birth to which they then receded the previous thought and said then they could not find anything else. This seems minor but it actually upset us all day as this took place over an entire afternoon with all kinds of questions and doctors and nurses in and out. 
              The temp worried Dr Davis and wanted to cancel again and we said make sure they don't misplace the blood they withdrew for a type and cross because they did after the first was cancelled and had to stick him again. This then led us and the Dr to thinking his temp was due to being off his heart meds and the aggravation of having blood drawn again and surgery was a go. We then sat in that waiting room for many hours.Remember, the sooner he could get him off the heart and lung machine the better and they said they would call as soon as he was off that. Finally, the call came and they had him off the machine and so far so good.They said they were warming him up and getting ready to close.They had told us they would chill the blood to inhibit blood flow.The call then came that he was back in ICU and we could see him.They had prepared us that he would have a respirator mask on as well as pacemaker wires sticking out of his chest and 2 tubes coming out of his lower chest and another tube down his throat. We went in and as if he knew we were there, he woke and when tried to make a sound only gurgled because of the tubes so we went back out and when we tried to go in again he awoke when we walked up to him. They said he would be on the respirator a day or so because they had to ween him off slowly and we could hold him then. Dr. Davis had told us that the small opening in the membrane was so small he was surprised he had went so long before the signs showed.He had cut him an opening large enough for 2 adult fingers to go through.By late evening he was completely off the respirator which was a good sign. In the next day he ran a temp again and was getting higher and they didn't give anything till it hit 103 and we found out later this is how they rule out or in different causes.They did blood cultures to see if maybe a bacterial infection.When nothing grew, they assumed it was viral. By the end of the next day they removed the tubes but, the wires stayed in case of cardiac arrest, they could hook up a pacemaker.Amazingly the temp went away. Again, we can only assume that the chest tubes were aggravating him to the point of causing a temperature.
                         Joey was moved to a semi private room and a week later we took him home. There was a little girl in the room with him we became kinda attached to that had a minor heart problem and a few weeks later we returned to have a check up and went up to see her and found out she did not survive the surgery.This almost riddles you with guilt and relief at the same time.I can only imagine what this could do to a surgeon who has a success with a rare defect that only few have performed only to lose a patient with a minor fix. We were there for three weeks that seem like an eternity and is so vivid in my mind. As far as Joeys life after that:
At his second birthday which was about 3 mos. after surgery, he was still mighty thin but very active and would eat whenever there was a meal to be had. He started growing like a weed again and was always learning more and more.
           I might mention they were concerned he might have some organ damage because the kidneys had stopped funtioning on their own before the surgery.
Below:Joey at Eater with his sisters and cousins. Aged 2 yrs 3 mos. Slowly but surely starting to fill out more in his face and his body


At age 9 and 10 he played midget football and by age 11 couldn't make the weight limit. At 10 he played first string offense and defense

At 15 he played high school ball and lettered in the following years.
One incident he had at practice had doctors puzzled when they said he should not be on the field and really had my wife thinking he was having more heart trouble. Thank God one of the cardiologistds at MCO was around to look at it and put her mind at ease because they said his echo cardiogram will not ever look normal to most cardiologists who aren't familiar with his case.
At age 31(present day) Joey attends father - daughter dance with his own 2 daughters who while in the womb were checked  and cleared of any heart defects. ------->


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