Evan M. – updated November 2020
Evan is now 13 years old and has come on so much since his last update at 8 years old. Whilst his eating is not good and he refuses to eat orally, he is predominately tube fed via a Mic-key button feeding tube. He has a mixture of Tentrini Energy Multi Fibre, SOS 25, MMA/PA Express 15 and Calogen for calories. We give him boluses during the day and pump over 3 hours at night.
Thankfully, this is the only thing he has gone backwords with. He is toilet trained (day & night) for a few years now and is doing great. His speech/communication has come on leaps & bounds and whilst we still can’t have a conversation with him he can express himself quite clearly and understands commands and requests. He loves helping me at home doing little jobs around the house.
Evan loves all technology, he enjoys watching tv (his favourites at the moment are Friends, Two and a half men, cooking shows and cartoons) films, music and dancing. He loves animals and has a fascination with Penguins. He loves books and reading and loves playing with our dog Elmo. He is a smiley boy who lights up any room and has such an infectious laugh. He has a zest for life like no other and loves people and people love him. He is an entertainer for sure.
Whilst he has come along way we do have a long way yet to go. Mainly with his eating and he also currently has some mobility issues needing knee high AFO supports when walking. He is considered ‘severe’ with PA. The severity has lessened somewhat after transplant but no matter how well he may be now, the future is always uncertain with PA, especially now with Covid. He is currently being homeschooled and is doing great despite my apprehension. His health will always remain our priority, his development can be worked on.
Evan M. – updated March 2015
Evan is now 8 years old. He had a partial liver transplant in March 2012. Whilst the 1st years was very tough, unfortunately he was diagnosed 6 months after transplant with Lymphproliferative Disease. A type of Lymphoma brought on by the immunosuppressants needed to stop his body from rejecting his new liver. He needed a few months of chemotherapy but thankfully got the all clear from this a few months later. Since this however, Evan has come along so so much.
The transplant wasnt a ‘cure’ but is a huge help in managing Evan’s condition and giving the best chance at living a normal life. He can tolerate alot more protein which in turn helps his development and generally makes him look healthier. He is in his 2nd year in the local special needs school and he absolutely loves it. He runs out to the bus in the morning and always has a smile on his face wen he gets off the bus when he gets home! He loves the attention and being with others his own age. His speech and communication is coming along although we still can’t converse with him and his eating is steadily improving. Although foods still have to be pureed, he is open to trying new things and experimenting and playing with food whereas before he showed absolutely no interest in food and mealtimes.
He is still tube fed most of the time via a mic-key button in his stomach. His feed consists of Energivits, Nutrini and MMA/PA Gel and all medications we give to him via this tube. So even though he still picks up every bug and infection going and does have long bouts out of school and the odd admission to our local hospital the future is looking bright for Evan. There is no doubt life is tough living with PA, we do our best by our children.
Not everyone agrees with our decision to go for a Liver Transplant but even though the 1st year was tough we don’t regret our decision. Our thinking was that if something happened during or after the transplant at least we are doing everything possible and in our power to give our child the best chance possible to have a normal life. Life with PA is so unpredictable.
Sarah (Evans mam)
Evan is 4 and he was diagnosed with Propionic Acidemia when he was four days old. normal pregnancy, normal delivery and evan arrived a healthy 7lbs 11ounces three days early. On day 3 he was getting very lethargic, he wasnt feeding and was breathing funny from his nose, like he had a cold. He was brought to the Special Care Unit by a nurse and we were told he would be back up soon so we presumed it was just to be checked out. An hour later, he was on a ventilator as doctors were afraid he would stop breathing. A number of tests were done and doctors in Cork narrowed it down to a Metabolic Disorder but they were not the experts in this field, The Metabolic Center of Ireland is in Dublin so Evan was transferred up when he was 3 days old where they diagnosed him the day after. Evan was in the hospital for 3 months until well enough to return home. Since then he has been admitted to the local hospital a handful of times, for flus, bugs and infections but thankfully since birth he has had no major decompensation. He has a good enough appetite and drinks his XMTVI Maxamaid orally during the day and eats about 6 of his restricted 8 exchanges of protein orally, however, anything he does eat has to be liquidised with no lumps whatsoever. He also has a continued feed during the night through PEG Tube. We travel to his doctor in Dublin every 3 months for check ups, where they take blood and check his levels are normal, and we speak to dieticians etc. Evan is a wonderful, happy, sociable, and boisterous 4 year old , he struggles with speech and communication which can get him frustrated and he has regular speech therapy, physio and occupational therapy. Evan has been on a waiting list for an Auxiliary Liver Transplant for 7 months now.