Gone too soon-Efan
“Efan was born on the 26/3/2011, a long awaited baby brother to Elan! The normal ‘joy’ supposed to be felt after bringing a newborn baby back home was short lived. It soon became apparent that Efan’s behaviour wasn’t ‘normal’. We as parents were back and forth to the GP worrying about his breathing, weight gain, a ‘fruity’ cough and general health. He would cry for prolonged periods of time and was always a challenge to settle and feed. It was a stressful time, we couldn’t quite figure out what the problem was and I even convinced myself I had post-natal depression as coping with the crying and worry of Efan’s slow weight gain was terrible.
Eventually at around 5 months old Efan was admitted to Ysbyty Gwynedd, our local District hospital where it was discovered his oxygen saturation levels were extremely low. Tests showed he had RSV, he was on oxygen for several days and fed with NG tube but to no avail. Efan was intubated and transported via the NEWTS team to the Paediatric Intensive Care Unit at the Royal Manchester Children’s Hospital.
It soon became clear that there was an underlying condition causing Efan to require oxygen. After weeks of blood tests and x-rays, most common respiratory viruses had been ruled out and there was no option but to perform an open lung biopsy to take samples of his lung to test.
Efan was extremely poorly after the biopsy and remained intubated for several weeks. He suffered from a pneumothorax. He was on IV steroids to try reducing the inflammation in his lungs but the steroids were suppressing his immune system and preventing his lung from healing after the biopsy. Efan eventually recovered and was discharged home on oxygen after 8 weeks at Manchester. After our return home we received the diagnosis that we had been dreading- Efan had a rare interstitial lung disease called Surfactant Deficiency Protein C. The disease is very rare with only a handful of children in the UK known to suffer from it, there is no known treatment and prognosis varies from child to child.
The next months were challenging for us as a family, Efan was in and out of Ysbyty Gwynedd often with coughs and colds requiring more oxygen than we could give at home. He had monthly 3 day stays at Manchester where he received x-rays and IV steroids and several appointments a month at feeding/ speech and language/ eye/ gastro clinics. Efan continued to make steady but slow progress with eating, talking and normal baby milestones. Even though hospital appointments were the norm these few months were fun-filled and precious to us, we spent as much time as we could together as a family and even managed to take Efan and Elan to EuroDisney to see Mickey Mouse!
Happiness was short lived- in August 2012 Efan’s breathing got continuously laboured. He was taken into Manchester and within a few days was intubated as it became apparent from a CT Scan that the condition of his lungs has greatly deteriorated. He was now described as having respiratory failure which meant that his lungs couldn’t function without the pressure support of the ventilator. Several procedures and test were done to try and help, GOSH were contacted about the possibility of lung transplant but this wasn’t possible due to Efan’s age and also monetary and logistical implications.
It was at this stage when my close friend -out of sheer desperation and helplessness decided to google SP-C , ILD...... she came across the chILD lung foundation. I had never previously found the site and no Drs had ever given me any information about it before. It was a Godsend, I got more answers from other parents in a matter of days than we had in months. I realise Mums and Dads generally have no medical experience but as the saying goes:- in unity there is strength!!!! All the information gathered by all these fab parents gave us a fantastic insight to Efan’s illness- at last we felt NORMAL!!!
Even though his lungs were so poorly Efan’s Doctors managed to wean him off the sedation and bring his ventilation settings down to enable Efan to re-learn to sit/ play etc. Efan was eventually stable enough to operate and give him a tracheostomy instead of the nasal ET tube. He also had a gastrostomy for his milk feeds but managed bits of food and beaker drinks. The long term plan was to get Efan home with his portable ventilator. He came along in leaps and bounds. He was able to sit and roll and occasionally stand, he could manage to say a few words like Mam, Dad and Nenan (Elan). Efan had an air leak around the trachey site which enabled him to vocalise- much to our joy! He would enjoy his bath every night and loved wearing his Bob the Builder Hat and doing thumbs up at the nurses.
Even though Efan was given steroids and anti-inflammatory drugs to try to suppress the disease process in his lungs his oxygen requirement gradually increased to 15 litres. It was decided on the 14th December 2012 that there was nothing more to be done for little Efan, his oxygen saturations weren’t being maintained in 15 litres of oxygen and watching him struggle was heart-wrenching. It was terrible to hear those words out loud even though we knew there was no miracle cure for him. The cruel thing about SP-C is that mentally and physically Efan was fine apart from his lungs. Efan was heavily sedated at bedtime and soon fell unconscious. Dr Samuel gradually turned his oxygen and ventilator off. Efan fell asleep quickly and peacefully holding Bleddyn and my hand tightly, Elan stayed with us to say her goodbyes too. The nurses came in and out of Efan’s ICU room throughout the night to kiss him goodbye.
We were very fortunate to be allowed to stay with Efan throughout the night and the fantastic staff and Funeral Director were so efficient that we were finally able to bring Efan home the very next day . It had been a gruelling 4 months in hospital- away from home, family, friends and normal every-day life. The relief we felt getting back home was immense, the saying “there’s nowhere like home” is SO true!
In Efan’s last minutes Dr Samuel said “Efan, you graced our lives!”. This is how we choose to remember and treasure his short life. We terribly miss his big blue eyes following his sister around the room full of adoration for her but we will always cherish his ‘thumbs up’ attitude to everything life threw at him.
Life will never be the same without our beautiful baby boy and no words will ever ease our pain and loss. However we take great comfort in the fact that Efan’s life story may one day help another family in a similar situation.
Whilst in hospital friends and family started raising money and opened “Efan’s fund”. We still organise social fundraising events every now and again to remember Efan, and have raised nearly £50,000 over the past few years. Many charities touched Efan and our lives, so money from his Fund has gone to several charities such as Ronald McDonaldHouse Manchester, PICU, the ChILD lung foundation and JUMP childrens charity. Our hope is to continue to keep the Fund open to help other families through Efan’s legacy!”
For more information or support, visit http://child-foundation.org/.