My Beautiful Ballerina, Dr. C, and News

Someone has been blessing Caroline with ballet lessons since September.  And she has loved every minute of it (even if she does look utterly terrified in the picture.)  Her recital was last Saturday, and she was, of course, a beautiful ballerina in a sparkly, fluffy white tutu. She looked magical and totally enjoyed the process...except maybe the abject fear of being in front of a packed house.

So after her performance, the room mom came hurrying down the aisle to retrieve me, saying, "You need to come now."  Uh, okay.  I follow her into the room and Jen explains that Caroline had been white as a sheet and was laying on the floor.  Caroline says she is just tired.  I scoop her up and decide that maybe she just needs food.  So off I go to grab a chicken salad sandwich and feed her.  She seems to perk up and goes back to the room to await the grand finale.  Only a few minutes later...I once again am retrieved from the audience.  This time, I am not so sure it is a real problem, so I just keep her and let her sit on my lap. 

The next morning, getting ready for church, she comes down the stairs and says her legs feel funny.  Um, funny how...do they hurt?  ache?  feel heavy?  They feel heavy.  Why does this feel like Groundhog Day?

Tuesday I spoke with Dr. T and she informed me that they were ordering a new MRI for Caroline to see what happens to her brain stem when she bends her head back and forth.  She doesn't appear to have a Chiari, but the basilar invagination is distinctly possible. 

So I was determined to show her MRI to Dr. C here in Cinci.  Only I forgot it.  (I obviously lacked some organization heading into this trip!)  Billy managed to upload it, Will downloaded it and printed it on a CD, and I handed it to Mimi and Dr. C. 

Essentially...Will's MRI looks pretty much the same as in November after the procedure.  There is still a flattened area that they hoped would become a little more round.  At this point, they will perform an MRI in a year and see what it looks like then.  This finding would pretty much agree with Dr. Cohen in that the brain stem is permanently damaged.  Not news I want to hear, but neither is it surprising.

He looked at Caroline's MRI and agreed that we need the second MRI with flexion and extension.  His conclusion is that the Chiari issue is still in question because the bone structure used to measure the presence of a Chiari was not well defined.  The real issue was the brain stem.  There is evidence of cervical instability (the cervical spine is not stable) and the brain stem looks like it may indeed be compressed like Will's...just not as badly and for a shorter duration.  So, he believes she will need to have her first few vertebrae fused like William's, and the need for a chiari decompression or a transoral odontoidectomy is still unknown.

So, my beautiful ballerina does appear to be trekking down a similar path as William.  Am I in a panic over that?  No.  Am I sad?  Yes.  I have cried buckets this week.  But we do have a sovereign God, we do have amazing doctors, and we are catching this early.  Will that change her outcome?  No clue.  Since we don't know how much of Will's issues are mito and how much are brain stem issues, then we have no way to project how Caroline will progress.  But we can be thankful for today and for ballet recitals and fluffy, sparkly tutus that bring joy to her which in turn brings joy to us.

Sorrow, Take 2

Sometimes when you have been running a while, you want to stop and catch your breath.  But once you do, it can be difficult to start running again.  You can find plenty of reasons to walk or quit.  An object in motion tends to stay in motion.  An object at rest tends to remain, well, at rest.

So for me, a respite isn't always all that helpful.  Yes, the peace is lovely.  The slower pace is heavenly.  But the knowledge of what I have to return to is not so helpful or lovely.  The emotional rests is amazing, but in 36 hours, I will be back on the front lines where the fighting is most intense.

I love my family.  I miss them when I am not with them.  I miss the hugs and the kisses goodnight,  I even miss Chris's OCD need to know exactly what is going on tomorrow.  What I don't miss is the survivor mentality.

A while back I wrote about the whole survival mentality.  When a natural disaster occurs, typically what you see are people trampling their neighbors to obtain food and water.  The same thing happens within a family who lives in constant crisis mode.  The kids trample one another to obtain what they emotionally need, the couple are intensely focused on different things, and the whole concept of teamwork is literally thrown out the window.

I am not suggesting that my familly is trampling one another.  But the teamwork is definitely gone, and the level of angst is definitely at an all time high.  The question is, now what.  Where do we go from here and how do we help the kids develop healthy coping skills while trying to still deal with a spouse who is also struggling.

When I was told that 70-80% of marriages with a chronically ill child end in divorce, I was shocked and incredibly prideful.  There was no way that was happening to us.  But here we are three years later, and the entire family is struggling.  There is not a relationship that is not affected.  And when you are stressed, then generally your best foot is not put forward.  So the struggle escalates.

The question is...what now.  I have no answer...so we continue to get up in the morning and work all day and fall into bed exhausted at night, knowing that tomorrow brings another day.  Is that fatalistic?  Probably a little.  But it is more a case of not knowing what else to do.


So we cry...a lot and we realize that “There is a sacredness in tears. They are not the mark of weakness, but of power. They speak more eloquently than ten thousand tongues. They are messengers of overwhelming grief...and unspeakable love.” (Washington Irving)  and we continue to look for solutions, all the while praying that the Lord would work out His plan for our family and give us the strength we need to manage whatever comes next.

Where's the Pump???

Thursday, we met Gordon, the pilot for the first leg of our trip to Cincinnati. The flight was longer than normal due to a strong headwind. We finally arrived in Beckley to meet the infamous Charlie, who loaded our gear while harrassing us about how much stuff we bring. I had brought cookies for both pilots, so I threatened to take his back. 

We headed off to Cincinnati amidst headwinds of 65 knots and lots of storm build up. We can't go over the storms due to their height, so we have to vector around them. At one point, looking out the window, it looked like we were surrounded by mountains of clouds. It was beautiful, but incredibly stressful for Charlie.

As a result of the head winds, the entire flight took significantly longer than normal. Charlie needed to let the storms die down before heading home, so he went to Dewey's Pizza with us, then off to the Riggs's to unpack and let Will rest. It had been a long day.

After returning Charlie to his plane, I headed to the grocery store, then back to Will to start an IV...only to discover that I had forgotten our IV pump. With a port, you have to use a pump; you can't run the IV by gravity, so we were totally stuck. Of all the things to forget, this was probably the worst.

So Friday morning, Will isn't ready for an ER, but he certainly is not looking great. Our IV company at home graciously agrees to overnight us a new pump, so now all I have to do is figure out how to get him safely through his MRI, the day, and the night without needing to go to the ER.

We had a long talk about how much Powerade Zero he would have to drink in order to compensate for the lack of fluids. And he would have to eat. There were no guarantees that he would avoid the hospital, but he would have to be the one to put forth the effort if he wanted to avoid it.

We were very fortunate that the weather here was cool and fairly dry which is significantly easier on him than the heat and humidity back home. He was able to hang out with his buddy, Chris, after his MRI, but that visit centered around the computer, so it was not physically stressful. By last night, he had done a phenomenal job of drinking and eating as he should, and while it went against my better judgement, we stayed home and did not head to the ER. The pump arrived safely this morning, and he has had fluids all day to try to catch up. He still looks pretty rough, but better than he did this morning. I am so grateful to our IV company for being so gracious and overnighting the pump to us.

This morning I was blessed with a long walk and chat with Becky G and yet more time with Becky R. It is such a gift to know that we have a second home and family who love us here in Cinci. Will slept through almost all of that, but tomorrow he gets to arise with us mere mortals and attend church.

We see Dr. C on Monday to review the results of the MRI and determine what state the brain stem is now in.

I'm Leaving on a Small Plane...then a BIG Plane

Will and I head back out in the morning (today by the time most of you read this) for Cincinnati. He has an MRI on Friday morning and then Monday morning, we see Dr. C, the neurosurgeon. Hopefully, he will state that the brain stem looks lovely and no more surgeries will be required. If all goes well, we will fly home on Tuesday.

Just as a quick aside...in the past year, we've been blessed with a large assortment of gift cards to restaurants, book stores, and Visa gift cards. The generosity of those who love and pray for us astounded us. As I am packing the last of those cards tonight, I am once again reminded of just how much we are loved. So thank you for the encouragement and the physical support that so many of you provided.

The most heard question at this point is, "How is Will?" That is such a tough question to answer. If you look at him, he looks well. His blood pressure is moderately stable. But all he's really doing is sitting on the sofa with his computer. He is receiving IV fluids on average every 36 hours.

Which brings us to the big plane...Make-A-Wish has called, and Will's wish is ready to go. We depart on July 6 for the Keys and return July 11. Jervetta, our Make-A-Wish representative, is coming Wednesday night to reveal the details of the wish. Will is psyched. The kids are psyched. And I'm psyched and worried.

Will does not tolerate a lot of physical activity, travel, or heat. Anything that requires energy has the potential to create a crash. That being said, the travel to the Keys, kayaking, and snorkeling (where not only is he swimming...exercising with resistance...but his body has to warm itself in the water) are all huge energy drains. So my job in all of this is to contemplate how much we need in terms of medical supplies. When we travel, we typically carry one bag of IV fluid for each day we are gone. For this trip, we'll carry two for each day, knowing that the potential for a massive crash exists but praying that it doesn't happen.

I need to pack for tomorrow, hopefully pull Will's IV soon, and head to bed.

I Do Not Do Sick Well

I do sick fine, actually. As long as it's someone else who's sick. Me sick? Not so patient.

I arrived home from Ohio with a roaring bacterial infection and a virus. Not just any virus, but the virus that spawned from the pits of hell. So, I have been in my bed, under the weather, and unable to eat for days. Coxsackie is in the family with chickenpox, but instead of itchy sores all over your body, you get lotsa blisters in your mouth and in the back of your throat.

So rather than just taking to my bed the first few days, I decided instead to just deny that I was sick. Really, I am fine. Which proceeded to, "Ok. I am sick, but I can still move, so I will be fine." which proceeded to, "Would somebody please shoot me and put us all out of my misery?"

So last night, when passing out from lack of food was deemed imminent, I returned to the doctor and basically begged for lidocaine in a mouthwash. The doctor took one look in my mouth and went, "Wow. Nice job." Yes, I am a perfectionist and try to do everything to the utmost perfectness, including getting really sick with this virus. I grew lotsa big, beautiful blisters. He provided warnings galore including, "If you swallow this, you will have seizures and it can be fatal." No problem. Without it I may have seizures from the pain and would prefer to be dead.

Now that this heinous virus has a name, google became Billy's friend. He decided that I really should be quarantined, so here I am in my bed, alone, with my computer, my knitting, a book, and a DVD player and after yet another nap, have decided that I may feel human enough to use the computer. I am considered contagious until the last blister pops and scabs over. (Doesn't that sound lovely?!) So if I exposed anyone to this virus unintentionally yesterday, I do apologize. When the fever broke, I presumed that I was no longer a menace to society...and without regular caloric intake, you would feel pretty crummy too...so I figured that just was part of the recovery.

So now that I have unlimited time on my hands, I may actually take time to update the blog and regale you with more information than you wanted. But right now, I think it is time for another nap...

Dr. C Today

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.

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.