This is a chronicle our everyday life as we live, laugh and love raising our teenager, our toddler and our special needs baby. Normal isn't always what it seems...

Wednesday, April 17, 2013

Aerodigestive Update

Oh where to start?  Let me start by saying this....If any of you have a private conversation with Lydon, feel free to tell him that Mommy is ok with him not maintaining his status of "medically complex".  I usually encourage my kids to do their very best in whatever it is that they are involved in and I tell them to strive for excellence but my dear Lydon Jefferson this is one area where Mom will gladly accept dull and boring! ;) That sounds funny to even say write because nothing about our life or Lydon has ever been dull and boring! ;0)
  The last time I updated, we had just completed the scope and biopsy procedure done by our Aeordigestive team.  We got the results back from it and it was indeed as we thought.  Everything looked pretty normal aside from his lungs. There were areas of unexplained redness and inflammation there. The pulmonologist felt like this was significant enough to get in touch with the mitochondrial disease doctor that we are going to see this summer. After some planning and coordinating, they had a teleconference last Tuesday afternoon. Together, they agreed that his poor endurance and excercise intolerance seems consistent with the effects of mitochondrial disease. However, the cyanosis or blueness that we see associated with these occurances is not commonly an effect from mito but since your heart is a muscle and any or all of the muscles in the body are dependent upon the mitochondria they felt it necessary to once again look at Lydons heart and rule out any myopathy. We are having an echo done on Monday to assess these issues.   
     The next thing was to address his excercise intolerance. Last Monday, I took him in to the hospital and he completed a little mini stress test.  This is more simply called a walk test.  Basically, Lydon had to walk for six minutes in an air conditioned hallway.  They measured the distance he was able to walk and monitored his heart rate and oxygen levels the entire time. Yesterday, we followed up with the Pulmonologist and he told us that it was very clear that Lydon needs oxygen for any type of activity, even walking. :(  We already knew this was the case. We've seen him turn blue and become short of breath with the simplest of activities but we didnt know just how low his oxygen levels were dropping. He told us that this was all just part of the disease process and providing Lydon with the supplemental oxygen was really the only thing that we can do.  Unfortunately, this isnt something like asthma where breathing treatments or steroids are something that would help or improve things.  Our doctors believe that because of his diseased mitochondria, he is lacking the ability to manufacture the amount of oxygen that his body needs and therefore he suffers respiratory wise and doesnt have the energy that is required to engage in even minimal physical activity.  He assured us that the mitochondrial disease doctor would talk to us and explain some things to us more at our appointment with him in May. We are saddened that this just seems to be where we are with Lydon and that there really isnt anything else that we can do BUT he is an amazing little boy.  He loves living life. He has taught us to live in every moment. He frequently stops to smell the flowers, literally. He enjoys watching ants, looking in the sky for airplanes and finding "wady" bugs.  And with a beautiful spirit like that you can bet your bottom dollar we are going to do everything in our power to smile and laugh with him despite our heartache. Our hope is that having him on oxygen he will have more endurance and be able to play more without the cyanosis and shortness of breath;hence, protecting his organs from oxygen deprivation during these times that he is struggling to compensate for what he is lacking.
     We are also working with the GI doctor to try and help him with his tummy pain and bloating.  He has some really good days and enjoys eating his "safe" foods but he also has lots of "Ritz days" where you guessed it, he eats nothing but Ritz crackers.  God bless Nabisco!   Our only drawback to some of these GI meds is that they either A.) dont work at all or B.) come with significant side effects.  So here we are again....just disease process.  Really starting to hate that word/phrase! Anyway, he has a few ideas that we are going to try after we get back a clear echocardiogram. For now, we enjoy the days that he eats like a champ and are thankful for his formula and J tube when he doesnt.
     I will try to update again after we get the echo results.  We are looking forward to some very special upcoming days.  Lydon is turning 4 on Sunday!!  I cant believe its been four years since we recieved our special blessing.  Then about two weeks after that, Jono and I are leaving on a cruise to celebrate our 10th Anniversary!! Thats another one that's hard to believe. We will come home for a week and then leave again for the appointment with the mito doc. We will get back from that trip and be home for about two weeks and this one might be the hardest of all to believe,....  Sissy turns 16!! Busy summer days ahead...Thank you for all the prayers and sweet comments and encouragement we've received already from many of you through Facebook, Twitter, or email. Until next time...Much love, Jana

1 comment:

  1. Wow that must be very hard to swallow. It is great that you are able to help him. Sounds like you have a great team. Congrats on your Anniversary too.