Elusennau Efan
First months
Efan Wyn Evans was born Sunday morning, March 26th 2011, he weighed 8 pounds and Elan, his big sister came to take him home with Bleddyn, their Dad later on that afternoon.
Efan was very slow gaining weight , Bleddyn and Bethan (Efan’s Mum) were back and forth to the GP often. Efan always had a dry cough and went through phases of breathing rapidly, usually between 60-80 b.p.m. It was a very worrying time for his Mum and Dad.
When Efan was about 6 months old they decided to take him into A&E in Ysbyty Gwynedd, Bangor. Whilst in A&E it was discovered that he had very low oxygen saturations, he was immediately put in an incubator filled with oxygen for a few days. There was no improvement in his condition so it was decided to take him with NEWTS , the children’s emergency transportation team to the nearest Paediatric Intensive Care Unit. Efan was put on a ventilator in Ysbyty Gwynedd and taken to Mancheter RMCH by NEWTS.
He was admitted to Paediatric Intensive Care- PICU. At first Efan tested positive for the RSV virus but it soon became apparent that he wasn't recovering from it. During his time in RMCH Efan underwent numerous tests for all sorts of conditions. Several Consultants were liasing about his condition but it was obvious that there was something terribly wrong with his lungs. X-Rays showed un-explainable shadows on his lungs and the only way to truly find the cause was to perform an open lung biopsy. Efan was extremely poorly after the biopsy but Drs discovered that the interstitium of his lungs was hard and yellow rather than soft and pink. His respiratory Consultants were sure he had a condition called Surfatctant Deficiency-Protein C.
Eventually Efan’s condition stabilised and after 2 months in Manchester he came home dependant on home oxygen. Days after our return home we received a phone call to confirm that Efan did have SDP-C—a rare interstitial lung disease with only a handful of children in the UK having the same condition.
Interstitial Lung Disease
Interstitial Lung Disease ILD is a group of lung diseases that affect the interstitum (a specific tissue in the lung). Efan’s condition was called Surfactant deficiency Protein-C. Efan’s lungs didn’t produce enough surfactant which stopped his lungs being ‘lubricated’ and expanding properly.
His lungs were then ‘fighting’ this by trying to repair themselves therefore causing scarring. There is no known treatment for ILD and some patients are given steroids. The steroids suppress the immune system to try stopping the lungs from healing/ scarring themselves. The only other ‘cure’ for ILD is lung transplant. Transplant is by no means a quick fix, survival rates are very low and there is a huge risk of rejection and other serious complications.
Great Ormond Street Hospital were unable to offer this option to Efan as the procedure hasn’t been developed for under 3’s in the Uk, it is regularly done in the US.
