Our daughter was diagnosed with severe EA and ASD when she was born in Bath (UK) 2007. She spent a week or so in PICU in Bristol before we finally got her home.  She had a glen shunt procedure in 2009, but since then her oxygen saturations have been in the mid-70s, she has had little exercise tolerance and is quite blue.   About 2 years ago, our consultant in Bath  Andy Tomeztki told us what we had long suspected, that Emmeline would definitely need a second operation. Although it wasn’t clear what the best way forward was to be, as the valve was in a pretty bad way and really too severe to attempt to repair. The most likely outcome seemed to be the “fontan”, which is a bit of a last resort and doesn’t have a great prognosis. So, we had two years of scans and waiting, then had an appointment with Andy Parry last September when he told us they thought it was worth trying a repair afterall  (the “cone” procedure), and if this didn’t work they would go for an artificial valve, or else a fontan. We then had word on Valentines day2014 that the operation was scheduled for the 28th Feb, but this was postponed on the day due to lack of beds in ICU and re-scheduled for the 12 March. The night before (11 March) Massimo Caputo came to see us and explained that both he and Andy Parry would carry out the operation. Massimo explained had done 12 cone procedures previously, 11 of which were successful – Emmeline was number 13. The youngest patient he had carried out this operation on previously was 14, Emmeline is 6. He estimated that the chances of a successful repair were 70%. Anyway, after 5 agonising hours, Masimo came to see us and told us the operation had gone well, and the valve had good function, and they had largely closed the ASD -the only nuisance was that she would need a pacemaker (this, he explained, was a small price to pay). She had the pacemaker fitted last Thursday (20 March), and we got home from hospital yesterday (24 March). Emmeline is doing incredibly well so far; it really feels like a miracle. Her O2 sats are now 98-99%, and she is a completely different colour – her fingers and toes look pink for the first time in her life! She should be back at school after Easter. Massimo has now gone, or is about to go, to Chicago for a years sabbatical  – Andy Parry will now be carrying out the operation in Bristol his absence (I think this is also why he was present at Emmelines operation). We owe a huge debt of gratitude for the pivotal part played by the Ebsteins Society in disseminating the expertise on the cone technique from Brazil to Bristol and beyond.  We had long been under the impression that there was little chance this procedure could ever help Emmeline, but all being well she should now be able to live a basically normal life. I also want to big-up the Bristol Childrens Hospital which has had such bad press recently – in our experience it is full of the most amazing doctors, nurses and other support staff.

Posted by Ed Feil.
 

3 Comments »

  1. https://www.youtube.com/watch?v=BHks9fH79Gc

    Surgeon da Silva, the one who created the Cone procedure in Bogotá Colombia.

    Comment by Camilo Cruz — March 26, 2014 @ 11:48 am

  2. Great to hear positive stories like this one. Good luck to you and Emmeline.

    Comment by Mao — March 27, 2014 @ 4:07 am

  3. They did 4 surgeries in Bogotá – Colombia last year (2013). “Cone repair” should be done all over the world. It saves many lives if they do in every places.

    Comment by Camilo Cruz — March 27, 2014 @ 12:00 pm

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