This morning, Will and I drove to Akron to finally see Dr. C, mito guru extraordinaire. This is the doctor who discovered the basilar invagination (brain stem compression) and sent us to Cincinnati in December of 2009. When we walked in, he totally remembered us. I was surprised given that he sees thousands of patients, but his statement was that it would be impossible for him to not remember us given that the brain stem compression was so severe and the initial Chiari was so huge.
That led him to the comment that said, "You do realize that when someone was hanged, the odontoid (the bony structure removed this past November to finally release the pressure from the brain stem) is pushed into the brain stem and that is what kills the person." Hmmm....nope. I didn't know that. He went on to say that he had no idea how Will had been walking and talking prior to this last surgery given the significance of the compression and that a fender bender could have killed him. He was relieved that the surgery was so successful and totally sang the other Dr. C's praises.
Well, when we Colemans do something, we don't do it halfway.
Which brought us to the whole mito question...Essentially, the mito testing done in Atlanta covers only a couple of possible genetic flaws that are responsible for Mitochondrial Myopathy. There is a new test coming out in the next couple of months that will test for up to 400 possible locations where the genetic error may occur. So while Dr. S in Atlanta was unable to locate the exact location of the defect, Dr. C in Akron is convinced that we may find it with this new test.
However, his belief is that the majority of the autonomic instability is a direct result of the brain stem injury. Which means that Will's brain stem was too compressed for too long and as a result, it is not going to recover. The actual management of Will doesn't change with this possible shift in primary diagnosis. The symptoms are handled identically. The significance lies in prognosis. With POTS or Mito, there is a significant chance of improvement in Will's 20's. If indeed the majority of the symptoms are a direct result of brain stem compression, then essentially the prognosis remains more murky. This is not going to go away.
The rapid progression is the remaining question mark as to which is primary...brain stem or mito. This is the reason for continuing to pursue the genetic component of the mito. That finding would also have significance for future family members (grandchildren).
So what are we doing? When I return home, the first order of business is to apply for a Medicaid Waiver so Will can enter rehab. By slowly improving his muscle tone and endurance, the body can utilize the larger muscles to assist the neuro-muscular system that is not functioning properly right now. But that is a painfully slow process and our insurance only covers 20 physical therapy visits a year. The Medicaid Waiver would allow him unlimited therapy services. Dr. C was adament that the rehab needed to occur in a medical facility so that rapid assistance would be available. I really have no idea how we are gonna manage this one, but this is essentially the only hope we currently have for improvement. Even so, the best we can expect is maybe a 20% improvement, but right now, I will happily do whatever is required for that 20%.
He did remind me that the heat of summer is going to make hydration significantly more challenging for William and that his need for infusions will likely increase. Yep, I am on that already...especially with the Make-A-Wish trip in the Keys looming.
I realize that this is a quick information dump, but I am literally falling asleep at the computer. The weather for our trip home is still iffy, but as of tonight, Charlie and Matt think it will clear enough that we can fly later on Friday. Today's drive and activity was obviously a bit much for William, so tomorrow is another round of IV fluids and a quiet, restful day. The storms here tonight have been severe, so quiet sounds pretty lovely right now.
Wednesday, May 25, 2011
Tuesday, May 24, 2011
Sorrow
When we travel to the doctors we see only twice a year, it causes me to focus on where William was last time we saw this particular physician. Last year, when we saw Dr. G in Toledo, William was receiving normal saline once a week and I had just started accessing his port. Here we are a year later, and he is receiving dextrose in his IV along with supplements to help the mito function more efficiently and he is receiving 3-7 IV's per week.
Dealing with the progression on a daily basis, you don't always see it. It is sort of like getting to the end of the summer and realizing that your teenage son grew six inches, but because you live with him, you don't recognize the full extent of his growth until he tries to put on a pair of jeans that fit him at the end of the school year.
Last night, one of our pilots who lives nearby was playing chess with William and ended up going out to dinner with us. But while they were occupied, I pulled out a box full of IV supplies and started sorting. Charlie took a look at the piles of stuff and asked if that was a month's worth of supplies. I laughed, and thought, "Why would I bring a month's worth of supplies?" when I realized that he really had no idea. I explained that it was nine days of supplies, and his eyes got huge. "No wonder you have so much stuff when you fly." He really hadn't realized that 90% of what he sees when we fly IS medical supplies and we really do ship a 40lb box of clothes and school books every time we take an Angel Flight. I had told him, but he hadn't really understood.
But that whole conversation and the reflections of what was a year ago, once again made me realize how far William has progressed and caused me to ponder the summer. Where will he be in three months? I just submitted possible dates for our Make-A-Wish trip, but in the back of my mind I wonder how he is going to handle it. Will he be able to snorkel without ending up in the hospital? Will the energy required for that trip cause him to have yet another lower baseline? How do we give him that week and not lose ground?
I know this is progressive. I know that the future is uncertain. But the grief of today can be overwhelming, knowing that there is more to come. I came across a quote this morning by Kalil Gibran that said, "When you are sorrowful look again in your heart, and you shall see that in truth you are weeping for that which has been your delight." So true.
Dealing with the progression on a daily basis, you don't always see it. It is sort of like getting to the end of the summer and realizing that your teenage son grew six inches, but because you live with him, you don't recognize the full extent of his growth until he tries to put on a pair of jeans that fit him at the end of the school year.
Last night, one of our pilots who lives nearby was playing chess with William and ended up going out to dinner with us. But while they were occupied, I pulled out a box full of IV supplies and started sorting. Charlie took a look at the piles of stuff and asked if that was a month's worth of supplies. I laughed, and thought, "Why would I bring a month's worth of supplies?" when I realized that he really had no idea. I explained that it was nine days of supplies, and his eyes got huge. "No wonder you have so much stuff when you fly." He really hadn't realized that 90% of what he sees when we fly IS medical supplies and we really do ship a 40lb box of clothes and school books every time we take an Angel Flight. I had told him, but he hadn't really understood.
But that whole conversation and the reflections of what was a year ago, once again made me realize how far William has progressed and caused me to ponder the summer. Where will he be in three months? I just submitted possible dates for our Make-A-Wish trip, but in the back of my mind I wonder how he is going to handle it. Will he be able to snorkel without ending up in the hospital? Will the energy required for that trip cause him to have yet another lower baseline? How do we give him that week and not lose ground?
I know this is progressive. I know that the future is uncertain. But the grief of today can be overwhelming, knowing that there is more to come. I came across a quote this morning by Kalil Gibran that said, "When you are sorrowful look again in your heart, and you shall see that in truth you are weeping for that which has been your delight." So true.
Monday, May 23, 2011
Absence Makes the Heart Grow Fonder?
I know there have been large gaps in the blogging lately. Part of that has been limited internet where we are staying. At home, I hardly sit down, so blogging takes a back seat to the rest of life. And part of it is my own mental state. You have to be able to communicate effectively and I have been exhausted both physically and emotionally as of late, so I have kind of circled the wagons and kept to myself.
That being said...I am currently in the Toledo area with Will. Our Angel Flight was canceled due to weather, so we left very quickly Tuesday evening so we could break the drive into two days. The weather was ugly, but we arrived safely.
We have a couple of doctor appointments that are days apart, so I find myself with all kinds of down time and I am truly at whit's end. I dream of down time but now that I have it I have no clue how to handle it. :) We are in a little township that is very laid back, so it has made for fabulous running opportunities which really does help. William sleeps til noon, so I awaken around 7, listen to the cars outside, then finally get up and run...then I either walk to the coffee shop a couple of blocks away or the cafe across the street for breakfast. It has been lovely to be able to walk everywhere and to not have to drive a bazillion miles! I could get used to this. :)
As for the rest...here's a quick update.
Will is not handling the trip all that well, but it could be worse. He is getting IV's not quite every day. He should get them every day, but it is a fine line you walk with a 14 yo who does not wish to deal with the daily encumbrance.
I did run the half marathon a week ago. It was truly a monumental experience. When they say running is a mental sport, it is absolutely true. I owe a huge debt of gratitude to the coaches at my local running store, to Billy for watching the kids twice a week so I could join the group runs, and to KT who started and ended it with me...and who fed me afterward so I wouldn't pass out. Will I run another? I hope so. Stay tuned for more info on that...KT is working on a fund raising effort to raise awareness for mitochondrial disease. As soon as all of those details are worked out I will pass them along.
Caroline finally had her MRI last week. We are still waiting on the results. I had hoped to get a call last week, so tomorrow will be another call to the doctor's office.
As the school year draws to an end, we are scrounging for answers on how to handle next year. The little guys will most likely not return to their current school, so we have to make some significant decisions...the options are not all that great, so it is a huge challenge.
Thank you for your patience with me as I deal with the insanity of life and occasionally take a break from the outside world. I deeply appreciate your understanding and your prayers as things get a bit crazy and I find insufficient time to update the blog.
That being said...I am currently in the Toledo area with Will. Our Angel Flight was canceled due to weather, so we left very quickly Tuesday evening so we could break the drive into two days. The weather was ugly, but we arrived safely.
We have a couple of doctor appointments that are days apart, so I find myself with all kinds of down time and I am truly at whit's end. I dream of down time but now that I have it I have no clue how to handle it. :) We are in a little township that is very laid back, so it has made for fabulous running opportunities which really does help. William sleeps til noon, so I awaken around 7, listen to the cars outside, then finally get up and run...then I either walk to the coffee shop a couple of blocks away or the cafe across the street for breakfast. It has been lovely to be able to walk everywhere and to not have to drive a bazillion miles! I could get used to this. :)
As for the rest...here's a quick update.
Will is not handling the trip all that well, but it could be worse. He is getting IV's not quite every day. He should get them every day, but it is a fine line you walk with a 14 yo who does not wish to deal with the daily encumbrance.
I did run the half marathon a week ago. It was truly a monumental experience. When they say running is a mental sport, it is absolutely true. I owe a huge debt of gratitude to the coaches at my local running store, to Billy for watching the kids twice a week so I could join the group runs, and to KT who started and ended it with me...and who fed me afterward so I wouldn't pass out. Will I run another? I hope so. Stay tuned for more info on that...KT is working on a fund raising effort to raise awareness for mitochondrial disease. As soon as all of those details are worked out I will pass them along.
Caroline finally had her MRI last week. We are still waiting on the results. I had hoped to get a call last week, so tomorrow will be another call to the doctor's office.
As the school year draws to an end, we are scrounging for answers on how to handle next year. The little guys will most likely not return to their current school, so we have to make some significant decisions...the options are not all that great, so it is a huge challenge.
Thank you for your patience with me as I deal with the insanity of life and occasionally take a break from the outside world. I deeply appreciate your understanding and your prayers as things get a bit crazy and I find insufficient time to update the blog.
Monday, May 9, 2011
Wishful Thinking
Will is officially off of medical hold for Make-A-Wish Foundation and they are working on planning his wish. It was originally supposed to be in Hawaii, but the concern over how to safely get him there has prompted a change to the Florida Keys. He wants to snorkel and kayak.
Thursday, week before last, Will swam for about half an hour while the littles had swim club. I was swimming laps in the pool while he kinda messed around. He really wasn't swimming as much as he was walking and playing. But by the time we got home, his resting heart rate was 130 and his diastolic pressure was in the 40's. We started an IV in what ended up being a long line of IV's.
Essentially, he had 8 IV's in a ten day period. He ended up catching a cold as well, so the combination of swimming and illness was too much. I probably should have taken him to MCV last Tuesday. He slowly improved, but it has prompted a whole host of questions.
If being in a pool for less than half an hour crashed him, how is he going to snorkel? We have started him in physical therapy, but I have no idea how effective it will be and the risk of it creating more crashes is a very real possibility. How do we handle his wish as well as keep him healthy?
As a side note...Next Wednesday Will and I head to Toledo for 9 days. We only have two doctor appointments, but the fact that they are in cities two hours from one another and the fact that traveling is so hard on William, we have opted to stay in Ohio. We are actually going to stay in a bed and breakfast because it was the same cost as the hotel, but it gives us two bedrooms instead of one. (Traveling with a snoring 14 year old gets really old when you spend hours in the same hotel room!) The owners are providing an additional dorm sized fridge since we will be traveling with 11 1.5 liter bags of fluids, but they wanted us to be able to store food as well. That is a huge blessing. I have no clue how we will manage all of this both financially and with Billy's long work hours, but the idea of traveling twice is not appealing at all due to the toll on William who is obviously struggling.
Thursday, week before last, Will swam for about half an hour while the littles had swim club. I was swimming laps in the pool while he kinda messed around. He really wasn't swimming as much as he was walking and playing. But by the time we got home, his resting heart rate was 130 and his diastolic pressure was in the 40's. We started an IV in what ended up being a long line of IV's.
Essentially, he had 8 IV's in a ten day period. He ended up catching a cold as well, so the combination of swimming and illness was too much. I probably should have taken him to MCV last Tuesday. He slowly improved, but it has prompted a whole host of questions.
If being in a pool for less than half an hour crashed him, how is he going to snorkel? We have started him in physical therapy, but I have no idea how effective it will be and the risk of it creating more crashes is a very real possibility. How do we handle his wish as well as keep him healthy?
As a side note...Next Wednesday Will and I head to Toledo for 9 days. We only have two doctor appointments, but the fact that they are in cities two hours from one another and the fact that traveling is so hard on William, we have opted to stay in Ohio. We are actually going to stay in a bed and breakfast because it was the same cost as the hotel, but it gives us two bedrooms instead of one. (Traveling with a snoring 14 year old gets really old when you spend hours in the same hotel room!) The owners are providing an additional dorm sized fridge since we will be traveling with 11 1.5 liter bags of fluids, but they wanted us to be able to store food as well. That is a huge blessing. I have no clue how we will manage all of this both financially and with Billy's long work hours, but the idea of traveling twice is not appealing at all due to the toll on William who is obviously struggling.
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