Wednesday, December 30, 2009

Heading Toward Home

This does not go down as one of those trips you want to do all over again. Right now I just want to curl up with a blanket and sleep for a week.

We saw Dr. Crone. Talking with him is like talking to your grandfather. :) He is honest but kind. He requested a timeline of events of the last few years. It really made me realize how much Will has deteriorated just this year. When you live it, you realize how difficult it is and how far he has progressed, but you don't really realize how fast this occurred. Dr. Crone agrees with Dr. Cohen that this rapid progression may indeed be a result of a compression of the brain stem and that pressure needs to be relieved.

The process of relieving the pressure: We are due to return sometime in February for two full days of testing and visiting with numerous specialists involved in the surgeries, of which there will be two. At the end of that two days, the doctors will meet to discuss whether the surgeries move forward and, if so, exactly what that would look like.

Background info:
Parts of the first two vertebrae were removed during the first Chiari surgery to make room for his brain.

If the surgeries move forward, which is to be expected at this point, Will would then (in February while we are there), have the first surgery. Since some of the front of his skull now needs to be removed to alleviate the pressure, the vertebrae in the back need to be strengthened so that his head has sufficient support, so they would fuse those first few vertebrae.

After several months of healing, we would return to Cincinnati for surgery number two. This is the ugly one. They would need to go in through the back of the palate to remove part of the skull in front of the brain stem. As Dr. Crone put it, "This is a massive surgery" and would require coordination with multiple specialties. He would have an ng tube (a tube through his nose for liquid nutrition) for about a week, may end up with a temporary tracheostomy, his tongue may have to be split, and/or his mandible (jaw) may have to be divided. Not a little surgery.

Will knows the highlights, but not the details of this process. Dr. Crone sent him off for orthostatic blood pressures while he discussed the details of the procedure. We filled Will in on the highlights, but have chosen to wait on the details until they become a certainty. We should have more information in February when the decision to move forward is made.

Will this cure Will? Probably not. The thought at this point is that it will stop the progression of the autonomic dysfunction, but most often the symptoms created by the pressure do not generally improve. Stopping the rapid progression is certainly at the top of our list, so if the specialists agree that we should move forward, then that is the course of action that will most likely be taken.

We obviously appreciate all the prayers and support that everyone has provided. From taking care of our children on zero notice and willingly keeping them when uncertainty of our return date occurred, to praying for us, to helping to financially manage this trip. We are blessed beyond belief...The Lord IS our comfort and our strongtower. Without Him working through all of you, we would have been in a heap on the floor long ago.

Cincinnati Bound

Dr. Cohen talked to Dr. Crone who talked to his secretary who called me...all in about five minutes. :) The end result is that we have an appointment in Cincinnati at 1230 today.

Will's labwork was incomplete upon our departure, but Dr. Cohen said he would call us later. Will's case is a bit complex so sorting it all out is going to take some time.

So, the information as we know it is that Dr. Cohen is convinced that Will will require surgery to remove the pressure from his brain stem. Once that happens, it will be a waiting game to see how much of the autonomic dysfunction disappears and how much remains. There seems to be little doubt that the surgery is required.

We are currently enroute (4 hours) from Cleveland to Cincinnati. Gotta love technology. My blackberry (which is functional again!) is tethered to my laptop allowing me internet access. Having a techie for a hubby has unexpected benefits. :)

I should be able to send an update sometime this evening.

Tuesday, December 29, 2009

Cleveland Update

Sorry for the lack of communcation. We return to see Dr Cohen (for the third time) in a few minutes, so this will be quick.

We arrived safely. The last bit of the drive was in snow, which was supposed to drop a trace of accumulation. Clevelanders run on different measuring devices than us Southerners because we got 6 inches. :) It has been unbearably cold with strong winds, but what they lack for in outside temps here they more than make up for in the warmth of their treatment of us.

We saw Dr Cohen yesterday morning at 8. He spent about 2 hours with us. He set aside the whole mito diagnosis and took a detailed medical history. Fortunately, I had requested discs of Will's MRI's related to his Chiari; they were of huge interest. Dr Cohen did loads of labwork (the tech gave Will juice afterward because she drew so much...she was a riot!). We did have to convince Mary Washington Hospital to overnight to us the disc with Will's two additional head MRI's, and they arrived safely at 8 this morning.

We returned to Dr Cohen this morning. The mito lab work won't be back for a month. The other lab work created the need for yet more lab work. Essentially, he's looking to see if there is diabetes or pre-diabetes due to ketones being spilled in the urine. He also talked with one of his favorite neurosurgeons and here is where it gets a bit...painful.

The short version...we see Dr Cohen again in about half an hour. He is chatting with a doctor in Cincinnati who specializes in Chiari's and in brain stem surgery. The basic presumption here is that a good portion of Will's autonomic dysfunction is likely caused by pressure on his brain stem. Prior to his decompression, there was no kink. Now there is a significant "kink". The brain stem should be nice and straight and Will's is not.

So, we are now adding vitamin C to his regimen since it helps the Complex III mito issues. We are waiting results on the mito bloodwork. We return to Cohen in half an hour to hear the results of his conversation with the doctor in Cincinnati and determine when we have to go there. (We really like touring Ohio!) We should also have the new blood work back and hopefully have a few more answers.

So this hasn't turned out the way I thought it would. It is extremely overwhelming to consider that we are facing more brain surgery and nowhere near home. And we pray that we aren't looking at diabetes to add to the fray.

Will is managing. He is obviously not thrilled at the moment. We are so grateful for the Lord's provision of this trip...and hopefully answers...or at least more of the right questions???

Will update as I can.

Saturday, December 26, 2009

Where do I begin????

This is gonna take a grab a cup of coffee!

I was graciously informed by Billy's mom that I had not updated this blog in a timely enough fashion, but I've been insanely busy with negative progress. Really.

So, five months ago we made this appointment with Dr Cohen in Cleveland. Immediately thereafter, I applied for an Angel Flight. (Pilots with their own planes donate their time and resources to transporting chronically ill kids to distant medical facilities.) We were approved and scheduled. Someone offered to take the younger kids. Then out of the blue, a friend's brother donated an airline ticket so Billy could join us in Cleveland. Even the money needed to make this trip happen (hotel, rental car, food...) was miraculously provided.

And then came this past week...and I watched my lovely, detailed plans unravel before my very eyes. I am so not in control and the Lord apparently needed to remind me of that little detail.

So let's back up to Wednesday...two days before Christmas and the day before Billy's birthday. The shopping has to be complete because Will gets his infusion that day and that takes 8 hours, so there's no way I can leave home. So, the plan is to bake birthday cakes, prep Christmas dinner, and prep Billy's birthday dinner. I did say that was the plan, right? My plan? Uh, huh.

So Brooke arrived to start the IV and the pump keeps complaining of an occlusion. Generally that means there are air bubbles in the tubing that I need to remove (bubbles, not tubing). But today...the stupid pump just refuses to work so Brooke changes the tubing thinking maybe it's just a bad set. She heads out...and the pump plays with us again. I end up calling the Home IV company who state that apparently the motor is dying and I need a new pump. Did I mention that this company is in 2 1/2 hours away? This is an 8 hour infusion and it's already 10 AM. Oh, and as Brooke walked out the door she says, "Um, Will has expiratory wheezing." Oh, ok. So I have to unhook Will from his IV, attach sterile ends to the tubing and port, and take Will to the doctor. And he has bronchitis. How did I miss this??? Off to the pharmacy. Then to Billy's office to fax Will's records from Atlanta since I kinda forgot to do that earlier in the week. Eventually we arrive back home to receive the new pump and hook Will back up to the IV which will now finish at 10 PM. It's time to make dinner and put kids to bed...and nothing has been done for Billy's Christmas Eve birthday nor Christmas, but Will has lovely drugs (including the lovely prescription for prednisone) so maybe we can still make Cleveland.

I won't bore you with the details, but the next day pretty much followed suit, except that I did manage to bake and decorate Billy's birthday cake.

So fast forward to today. We've celebrated, we've enjoyed having everyone home, but Chelsea is now in the hospital with regular contractions. She isn't due for another month and we are supposed to have Chloe when Emily Grace debuts. Um, I leave for Cleveland tomorrow. So we set up a plan in case Chelsea does go into labor and I'm not here.

Then Angel Flight calls and tells us that one of the pilots has had to cancel so our flight to Cleveland is canceled. I had just finished making the arrangements with Teresa's brother who gave us the ticket for Billy. So now I have to call him back and tell him we can't make that flight because now we have to drive to Cleveland because airline tickets for Will and me would be $600.

Then...Erlene calls. Her mom has fallen and broken the femoral head (long bone in the upper leg that attaches to the hip) and needs surgery and a pin. Erlene is supposed to have my youngest three. At this point, it's almost comical. My lovely, orderly plan is now shot to pieces. So now what do we do? I call Anita who lovingly and graciously agrees on pretty much zero notice to host my three youngest for four days.

So, Chelsea is probably going to welcome Emily Grace into this world earlier than anticipated. Andrew, David, Caty, and Chris will be on red alert to get Chloe. Anita will have my youngest. And Billy, Will, and I leave in the morning for Cleveland.

Ya know, I really like to be in control. I like to have all the details in place. I can handle change and I generally roll with the punches. But sometimes those punches are a little more painful than others...but despite their pain, and despite wondering what in the world the Lord is doing, He still faithfully loves us and shows us that it isn't our plan that's the best. Really.

Saturday, December 12, 2009

It keeps creeping closer...

It is hard to believe that Christmas is two weeks away, which means that Cleveland is two weeks and two days away. Guess that means my shopping needs to be completed. Yikes! Where does that fit?

Just for the record...I write a lot about how difficult all this has been, but I hope that you also remember the blessings that we have received. God is very real and is at work in time and space and in our family. As difficult and painful as all of this is and continues to be, so does His grace abound. Maybe we are fortunate in that we have the opportunity to really see where He provides and where we have no choice but to trust that he WILL provide. Most of you don't have the opportunity to so clearly see His provision.

Right now, we have the Christmas and Cleveland expenses staring at us, but we also have this huge deductible looming. At times it seems utterly insurmountable. But we have God's promise that He is right there looking at those needs as well. So, for those who need to see God's ya go...

First. We were accepted for an Angel Flight for our Cleveland trip. It means that Will and one parent and no more than 50 lb of luggage will be transported in a private plane by a pilot that donates his time and resources to our family so we can get to Cleveland.

Well, then Billy's work scheduled lightened and now he can go. So now what do we do? Well, just yesterday, a friend's brother donated an airline ticket (on our favorite airline!) to Billy so he could also be at this dr's appointment. This brother doesn't even know us. Does he realize what a huge gift this is?!

Does that cover all of the expenses in Cleveland? Nope. But it helps. We won't be driving in uncertain weather. Will will hopefully handle a short flight better than he handled the last Ohio trip. And Billy gets to join us.

On the days when we just can't see daylight, we have to trust that even in this, He loves us and cares for us...We may not like how that looks sometimes, but I would rather be close under His wing and struggling with the ills of this world than having no hope. Because without Christ, there is no hope. Unfathomable.

Friday, December 11, 2009 more ways that one

Written on Friday...but kinda not posted on Friday. :)

Will is finally stabilizing. Still moderately tacchycardic, but stable. The autonomic dysfunction seems to be wreaking less havoc on daily life.

The port was accessed successfully Thursday and he received his full infusion with little discomfort or irritation. He is extremely sensitive to the adhesives, so I contacted the infusion company and they are very graciously sending several different options for us to try.

We requested a very specific nurse for Will since ours apparently resigned. For the record, can I state that I love this new nurse? She is a strong believer, gentle, very experienced, knowledgable, kind...and loves pediatrics. She is truly a gift from God at this point. I will have to deaccess the port from this point forward (I have pulled his IV's almost since the beginning.) The procedures for accessing and deaccessing the port are different than your normal IV, especially in a person with a compromised immune system, so precautions (like how to put on sterile gloves without desterilizing them) need to be learned as well as the need to pull out the IV without dislodging the port.

So the question has been posed as to how we are doing. Overall, we are still putting one foot in front of the other. The main project that Billy has been working on for forever was successful and is drawing to a close, so he may be home more. Camden is struggling with his OCD a lot, and Caroline is sruggling with abdominal pain of unknown origin and is not wanting to eat. Emotionally how is everyone doing? Well, that is the trickier answer. To say we are fine is a lie. To say that we can't keep doing this is also a lie. We are somewhere in between...

The adjustment of being the only decision maker in the home to now needing to actually check with Billy is a big adjustment. Not because I don't want to check with him, but because it is no longer a habit to check with him. That obviously can create some stress.

Andrew never complains, but at the same time, that means you never really know how he is. Chris is working at the Christmas tree farm, so he is as happy as a clam. Will varies every day from fine to not feeling well from happy to is like living with a constantly changing ocean where the storms blow in without warning. Camden is highly reactive, Ben flies under the radar, and Caroline is Caroline...sometimes she feels great and sometimes she doesn't.

To say we are without hope is wrong. To say that it is just hard...well, that it is.

Friday, December 4, 2009

Looks Like We Made It!

We arrived at the hospital this morning, and whom should we meet, but the very same anesthesiologist from Will's T&A in January! She read through the chart, thought, "This must be the same kid," and sure enough, it was. She was very willing to read through all of the Atlanta information, compare it to what she did in January, and feel very comfortable that we would not have a repeat performance. In other words, she was fairly confident that Will would not remember this procedure...and she was right. It is amazing how the Lord works!

The procedure went well. The surgeon is not only very competent but a truly gracious gentleman who was very patient with all of our questions. The anesthhesiologist offered to infuse him fully today so he wouldn't have to do it again in two days. While I realize that it didn't really affect her as much as it did the nurses, it was still beyond thoughtful that she was willing to do it.

David Charles came by and brought a couple of slices of chocolate cheesecake that he had made, and Caty came bearing the lovely gift of food. Will was fading and needed calories along with that lovely bag of fluid (the popsicles and soda I had brought for him were just not enough!); Caty brought him some soup and me a salad and a lovely Diet Coke.

We arrived home around 7 Friday evening. He has since struggled with some energy issues, some tacchycardia, and some autonomic problems, he is doing remarkably well. Thank you for your prayers...

I apologize for not posting this earlier... I just opened my netbook and here it was and I realized that it had patiently waited for me to return to it. I apparently suffered from short term memory loss...Now...what was I doing??? :)

Thursday, December 3, 2009


Briefly....William will be undergoing outpatient surgery Friday morning to implant a port to make his infusions less painful.

That being said...his last surgery was a bit traumatic in that he wasn't really asleep...he came back from recovery telling me what happened during the procedure. He was paralyzed but not really "asleep" so he couldn't tell anyone that he was actually "awake".

So, tomorrow brings with it its own issues...mito is a funny disease in that his body processes drugs differently than the average kid's. He may end up in a full blown mito crash. Or he may be fine.

So, we would be grateful for your prayers for peace, wisdom, and strength for what tomorrow brings. Our God is a mighty, sovereign God. He has Will firmly in His hands. Of that we are confident. Please pray that Will will know that confidently. Please pray for the other kids who will remain at home...