Joe with Aortic Valve Astresia, ASD, VSD and Hyperplastic Aortic Arch

England

Joe was born on a Wednesday near the end of March 2009. My pregnancy went fine and nothing was picked up as being wrong on my scans. After having Joe, I was trying to breast feed, but Joe was not feeding. I spoke to a breast feeding helpline who advised that this could be normal and to keep trying. Joe had his 24 our check with the GP on Friday 27th March 2009, and we were told that Joe was fine. We highlighted to the GP that his nappies were dry and he was not feeding, but not for a second did we think anything was wrong. The GP did not pick anything up either.

That Friday evening, Joe would not settle, and was “whinging” (not even crying) most of the night. I managed to get him to feed a little so I was a bit happier.

On Saturday 28th March 2009, the midwife came out to do her checks on me, and we pointed out to the midwife that Joe was “blotchy” and felt cold. She took one look at him and called 999. She told us not to worry, and that it was probably just an infection, but better to be safe than sorry.

Joe was rushed straight into re-sus, and eventually transferred to PICU. After a long wait, we were told that Joe was very poorly, and that they thought that there was an issue with his heart, so they wanted to do some more tests. We were then told that that they thought that Joe has Hyperplastic Left Heart Syndrome. We were also told that Joe had kidney and liver failure and that they were not certain whether he would make it through the night.

We were also given the option of being transferred to another hospital that specialised in this condition, and that this could be Birmingham Children’s Hospital. We live in Leicester, so this was fine with us.

Joe did make it through the night, and on the Sunday we were transferred over to Birmingham. Once there, more tests were done and it was confirmed that Joe DID NOT have Hyperplastic Left Heart, but had: Aortic Valve Astresia, Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD), and hyperplastic aortic arch. We were told that he could have surgery (Norwood stage 1), and this would need to be soon if we decided to go ahead. We were also told that Joe had two cardiac arrests while in hospital in Leicester (we had a long wait outside the PICU and this explained why).

We were told that the odds of Joe coming through the Op were about 40% , and that he could come through the op OK, but then the next 30 days would be vital. We decided to go ahead. Joe had the Norwood Stage 1 on the 1st April 2009. He had another cardiac arrest as they put started the op, and after 8 hours, Joe was back in Intensive Care. After an hour back in intensive care, Joe had another cardiac arrest.

From this point, Joe improved daily. He finally made it out of intensive care on the 13th April 2009, and we were able to bring him home again on the 23rd April 2009.

From then to October 2009, things went pretty smoothly. We have had a couple of times back in hospital with tummy bugs, but other than this, Joe thrived.

In September 2009, Joe had a cardiac catheter as his saturations were dropping. The team at Birmingham had to decide whether to do a “fix” – close his holes and put in a bigger tube, or to do the Norwood stage 2. They were concerned whether Joe’s heart could cope with closing the ASD and VSD due to the damage to the right side of his heart because of the cardiac arrests. A week after the catheter, we got the call to say that they wanted to do surgery that week, and that they were happy to attempt the “fix”. So, off we went to Birmingham again. Joe went in for his surgery, but just before his surgery, they decided that they were not comfortable to try and do the fix, so they done the Norwood Stage 2. We did not know this until Joe was back in intensive care. This time, Joe was only in intensive care for less than 24 hours and was home within a week. He done so well in regards to his recovery. Again, Joe has thrived even more. He is putting on weight, and has started to crawl and is doing everything that we would expect him to be doing.

We still go to Birmingham every three months for check ups. The next stage with Joe happens once his saturations fall below 75%. At this point, he will have another cardiac catheter to hopefully help the team decide whether they can now attempt the “fix”, or whether they have to do the Fontan. Again, much will depend on whether there has been any improvement to the function of the right side of his heart. We are keeping everything crossed that they can do the fix, as this will mean that Joe will just need tube replacements every 10-15 years.

There seems to be no development issues (this was another concern for Birmingham due to the 4 cardiac arrests) which is another thing we are really happy about.

Wrote by Emma Carter
February 2010

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Emma Carter has kindly given CHD-UK permission to use the photographs on this page.