VACTERL




 I don't remember really understanding anything that was going on on the day Evelyn was born, I was completely absorbed in her and trying to get my head round our situation. I knew Aly had agreed for her to have had a longline put in and that she constantly needed suction, but the details of why were lost on me at the time.

We spoke again to the consultant on Saturday and it started to sink in what was going on. Evelyn needed surgery urgently and she was booked in with a surgeon who specialises in her condition first thing Monday morning. She had a tracheo-oesophageal fistula - a complicated term, I know. When a baby develops the oesophagus is attached to the trachea. During normal development the oesophagus detaches from the trachea and attaches to the throat. This had not occurred in Evelyn. This was why she needed a longline to provide the fluid and nutrition her body needed in the short term. A pouch is created where the oesophagus should have attached which fills with saliva, having nowhere else to go. Suction is used to clear this pouch. In Evelyn's case suction was required every 12 minutes. It was very disturbing as her oxygen saturation would drop, sounding alarms, every time this 12 minutes was nearly up. Surgery is urgent under these conditions as acid from the stomach could leak into the lungs and gas from the lungs could get into the stomach. One positive was that she didn't require excessive breathing help, something the consultant was pleased with. 

This is when VACTERL syndrome was highlighted again to us and for the first time I really understood what the consultants of the fetal medicine unit had been preparing us for. VACTERL stands for the following:

Vertebral defects

Anorectal Abnormalities

Cardiac Defects

Tracheo-oesophageal fistula 

Oesophageal atresia (I am guessing the E is the US spelling)

Renal Abnormalities

Limb Abnormalities

When just one of these is obviously present it is likely that more may become apparent once baby is born. We already knew that Evelyn ticked the C and R as her heart was on the wrong side and she only had one kidney. We just never really understood what could further present itself once she arrived. Her rib cage on the right hand side was fused in places, also not developing fully. 

VACTERL syndrome has no clear cause, neither genetic nor environmental. VACTERL syndrome occurs in between 1 in 10,000 to 40,000 births. 

Our next step was to face watching our tiny baby girl go into surgery at only 3 days old.


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