Update on Adam

Well it has been an emotional roller coaster regarding our wonderful son.  Adam has generally been doing really well which gives my heart such a full and happy feeling.  Adam has obviously recovered from his tonsilectomy/Adnoidectomy and has since had 2 echo's that unfortunately show no changes regarding his heart.  We were really hoping that it would make a difference limiting his Obstructive Sleep Apnea and that maybe that was the cause of his disease, or contributing but alas the numbers right now show that isn't the case.  Luckily though things have not worsened just stayed the same.  We have re applied for the inhaled drug that Adam was denied, PHA Canada has spoken with Bayer and they are now trying to get it on a Special list for those who really need this treatment as they have no plans to try and get it approved by Health Canada.  I know a girl in BC got this drug so fingers crossed for Adam he gets it as his other option is to start on IV Medications.  We have just upped his current medications in the hope that it will help as for the first time Adam had some really bad days that had me worried.  I am thinking it is time I get an oxomiter machine to test his levels on days like that to determine if I take him to the hospital for 02 or just lay low.  I am determined to give Adam a typical life that will have hospitals and meds but in all other ways is just another day in the life of a kid.  Health Canada makes me so angry as there are so many other treatments elsewhere in the world that we can't get here that would be beneficial and give children a chance of swimming, normalcy, lack of equipment, tubes, infections, tape and less worry.  Our children are supposed to be getting some of the best treatment in the world and there is so much lacking!  The uncertainty in life makes me cry often as even the idea of a lack of understanding at school makes it hard.  Will Adam be teased for all sorts of medications or as his disease progresses for not being able to do things?  Will teachers understand or will they push him to unsafe levels because it is such a hidden disease.  Will there finally be a treament of the disease in his lifetime or will transplantation be our only true final option?  It is terrifying to think that ultimately in the end replacing his lungs and heart may be the only thing that will allow him into Adulthood.  In every other way Adam is playing, laughing, running, being crazy and finally potty training!  That child has humbeled me in so many ways with always having a smile on his face, wanting to help and pushing through even on those tough days.  I am starting to understand his disease more and more as I connect with more Adults and am now even more understanding to when he whines for no reason or what seems so, his pulmonary cough, his ups and downs in just a day, his sleeplessness, and the crazy "medicine energy" which usually means the next day is going to be a crash.  I was right about how up to 3 weeks after starting a new medication or upping meds makes him a nut bar, apparently that is common.  My now 3 year old is such an amazing child, an inspiring boy and truly the face of how hidden this disease is.  I know as a parent there is always so much uncertainty with their decisions, my hope is that his decisions are what makes life uncertain as opposed to health holding him back.  If my boy has the kindness to now put a bandaid on my forehead to "make me better" from a headache I hope that it continues through life and that he doesn't become juilted with the cards he has been dealt.  If he carries that through his life he will change the way people look at this disease, sick children and give hope to so many that those who have been dealt awful cards can surpass the negativity and embrace the positive.