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I lost my son Harrison on 04/02/2010 to EA. He was born sleeping, I was 27 weeks pregnant when he died. He was diagnosed at 21 weeks at Liverpool Womens Hospital (the staff there are fantastic).  Harrison had severe EA and pulmonary atresia and when he died his heart was so enlarged that it filled his chest. Losing him has been the hardest thing me and my husband have ever had to face but, we know that it was a blessing for Harrison to be born sleeping because it meant he wasn’t suffering. There was no genetic reason for his EA and as the doctors put it, it is a case of sheer bad luck but I am very concerned that if we ever tried for another child that the same thing could happen again. I have been reading all the comments people have made but haven’t found any which say you have gone on the have perfectly healthy babies after having a baby with EA.

Tia Elise, Our second child was born 12 days overdue on 29th December 2004 after a textbook pregnancy. I remember so well the midwifes words after she was born “She’s perfect”. We thought so too & her brother certainly did when he met her later that day.

In the early hours of the morning, after having fed her for the 4th time in just over 12 hours (she was a hungry baby), she seemed a little unsettled to me, I just felt something wasnt right.

I decided to take her to a midwife for reassurance. There was nothing apparent & she soon settled. After having talked for a while about delivery etc the midwife thought Tia maybe now looked a little cold & was flaring her nostrils slightly.

It was suggested her temperature be taken, this was a little low. They felt maybe she had a cold/viral infection & a paediatrician was called for a second opinion. He found she had a slight heart murmur, but I was told not to worry asthis was common in babies/young children and they are usually innocent (I knew this to be true as our older child was diagnosed with one at 6wks old).

I was offered a choice of keeping her with me on a heat pad & a touch of oxygen or to send her to S.C.B.U where they could investigate just what was going on & get her warm quicker. I opted for the latter & was told they would settle her in an incubator & come back for me.

I was asked if I wanted to call my husband, i decided to wait until we knew more. It was around 4.45am & i knew he would worry.

Around an hour later I was beginning to get restless, it didnt take this long to settle a baby in an incubator surely? Then i heard footsteps coming along the corridor, lots of them & hushed voices. Instinctively I knew something was wrong.

Two doctors & a midwife came to me, the male doctor began to speak. They had some bad news about my daughter. He took my hand as he said “its the worst possible news, Im so sorry”.  That was the moment my world fell apart.

They said they’d had no idea she was so seriously ill, she showed no signs, her heart had failed & they’d tried to resucitate. She was just under 15 hours old.

We had no choice, a post mortem had to be performed. It showed that Tia was taken from us by Ebsteins. Its something I will struggle with for the rest of my life. We were told the coroner despite his years of experience had never ever seen it & had to search through books in order to give what he found a name. How then can I fully believe that she would of been inoperable? How can someone who had never seen Ebsteins  say there would of been no hope? Maybe, if Ebsteins had been detected in utero she would of had a chance at life.. We will never know.

The ignorance here in the U.K is so very wrong, we were told Ebsteins is so rare, it affects so few people that so little was known & no research was likely. I know of several other families devastated by Ebsteins & it doesnt help any to be told were the minority.

Our son Marc was diagnosed  with EA at the age of 2, when our GP noticed an unusual heart beat. He was put on medication and monitored twice a year until he was 16 and then once a year as he had no changes or symptons. Because he was a large child and grew to be a 6’5″ adult and had an enlarged aorta, he was also monitored in case he had marfaans, for which he had no other classic symptoms. At 16 he developed narcolepsy/cataplexy and was given another drug to keep him awake. Long term these may have conflicted. He started at the local University and partly moved out into a shared student house, dropping home as and when he felt the need! I know he wasn’t taking his heart medication every day, but did take his narcolepsy drug to keep him awake in lectures, and he took full part in University life – partying and rushing about at high speed and holding down a weekend job. He didn’t take recreational drugs as he was well aware of the dangers with his two medical conditions. He was living life to the full. The week he collapsed and died, he had been VERY stressed about a number of issues. He had a quiet evening out, came in with his girlfriend sat in the chair and passed out and his breathing became very irregular. We called an ambulance and I did CPR until they arrived and took over. At the hospital they worked on him for some time, to no avail. We didn’t have a post mortem because all the medical staff were convinced that his aorta had split (a marfaans outcome) His heart consultant on reading all the reports from the night thinks that it was more likely connected to the Ebstiens and was a massive heart arrythmia . We have since discovered that he often had mid chest pains and irregular heart beats and had fainted on more than one occasion, but had ignored them as they passed quite quickly.

He was 21 years old, and our only child. He was very bright.

When your child leaves home and has to take responsibility for their own health and medication, it is a huge risk. They know it all and don’t want you nagging them. They only tell you what they want you to know. Maybe it would have helped if he had still been monitored twice a year still, something may have been picked up.