here - Little Hearts Matter

Medical22The article below looks at Protein Losing Enteroopathy whichis a complication that can occur following a Fontan procedureCan you make a difference toProtein Losing Enteropathy(PLE)?I am David Simpson, a medicalphysicist by training, husband to Helenand father to Alasdair (10), Eleanor (5)and Catherine (2). Alasdair was bornwith Aortic Atresia and had threesurgeries before the age of three tocreate a Fontan circulation. Herecovered well and developed into abright smiley boy who attendedmainstream school.Written byDavid SimpsonSuzie HutchinsonFast forward to 2007 and Alasdair,who had been in great health, wasdiagnosed with PLE. Helen and I foundourselves in another paediatriccardiologist's office having the'breaking bad news' talk again.Alasdair was put on a new regime ofPrednisolone and Sildenafil and,though it was five long weeks beforewe left hospital, he went back to fulltimeschool with the same energy hehad before.As a health professional withresearch experience, I started lookingat the scientific literature around thiscondition. I discovered that the use ofSildenafil had been reported in theliterature for the first time only sixmonths before Alasdair needed it. Ialso came across a small charity inBuffalo, USA, the Children's HeartFund (CHF) which is committed tocuring protein losing enteropathy. Thecharity was set up by the family ofColin Colson, a child who sadly died ofPLE.I had so many outstanding questionsI decided I wanted to talk to the expertsface to face and was lucky enough tobe given a small travel grant by myprofessional association and supportfrom my local hospital. I was verygrateful to be able to talk to SuzieHutchinson from Little Hearts Matter toconfirm that I was talking to the rightpeople.Children's Hospital ofPhiladelphiaI met with Dr Jack Rychik who gaveme two hours of his valuable time totalk about clinical care of children withPLE. He is setting up a single ventriclesurvivorship programme, a specialistclinic where Fontan children will beexamined by Gastro Intestinalspecialists as well as having their bonehealth assessed. This way, any signsof PLE can be spotted and treatedearly. He has had a good deal ofsuccess using Sildenafil in conjunctionwith Budesonide, a steroid used totreat Crohn's disease.We talked about how best practicecould be shared throughout the worldof Paediatric Cardiology and whatdirections future research may take.Dr Rychik talked about how thesupport of the CHF had been importantto him and that an InternationalSymposium organised by CHF hadhelped co-ordinate research efforts. Iwas reminded how small the worldofpaediatric cardiology was when Imet Dr Gil Wernovsky in the corridor,one of the experts Suzie had told meto look out for!Sanford-Burnham Institute, LaJolla, San DiegoOne coast to coast flight later I met upwith Dr Hudson Freeze and his group.I saw the plaque he has on the lab wallacknowledging the support of the CHFand also the picture of my family hehas on his group's notice-board! Hisgroup had cleared two hours to listen