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Evan M. – mis à jour en novembre 2020 Evan is now 13 ans et a tellement évolué depuis sa dernière mise à jour à 8 years old. Alors qu'il ne mange pas bien et qu'il refuse de manger par voie orale, il est principalement alimenté par sonde via un tube d'alimentation à bouton Mic-key. Il a un mélange de Tentrini Energy Multi Fiber, SOS 25, MMA/PA Express 15 et Calogen pour les calories. On lui donne des bolus pendant la journée et on pompe 3 hours at night.
Thankfully, c'est la seule chose avec laquelle il est passé des backwords. Il est propre (journée & nuit) depuis quelques années maintenant et va très bien. Son discours / communication a fait des bonds & limites et bien que nous ne puissions toujours pas avoir de conversation avec lui, il peut s'exprimer assez clairement et comprend les commandes et les demandes. Il adore m'aider à la maison à faire des petits boulots à la maison.
Evan aime toutes les technologies, il aime regarder la télé (ses favoris en ce moment sont Friends, Deux hommes et demi, émissions de cuisine et dessins animés) films, musique et danse. Il aime les animaux et a une fascination pour les pingouins. Il aime les livres et la lecture et adore jouer avec notre chien Elmo. C'est un garçon souriant qui illumine n'importe quelle pièce et a un rire si contagieux. Il a une joie de vivre pas comme les autres et aime les gens et les gens l'aiment. C'est un artiste à coup sûr.
Bien qu'il ait avancé, nous avons encore un long chemin à parcourir. Principalement avec son alimentation et il a également actuellement des problèmes de mobilité nécessitant des supports AFO à hauteur du genou lors de la marche. Il est considéré comme «sévère’ avec PA. La gravité a quelque peu diminué après la greffe, mais peu importe à quel point il se porte maintenant, l'avenir est toujours incertain avec PA, surtout maintenant avec Covid. Il est actuellement scolarisé à la maison et va très bien malgré mon appréhension. Sa santé restera toujours notre priorité, son développement peut être travaillé.
Evan M. – updated March 2015
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.
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, cependant, 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. |
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.
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.