Sunday, March 28, 2010

Here We Go Round the Mulberry Bush...

Woke up Will this morning for church, and he said, "I am so tired." Hmmm...well so am I (that is what I get for going to Richmond at o dark 30 to see Caty run the Monument 10K, paint the bathroom, and do all the grocery shopping...all in one day!), but I have this sinking feeling we aren't talking about the same kind of tired. Sure enough, he finally got up and was waiting for me, lying outside my door with the bp cuff and stethoscope which is how every child greets their mother in the morning!

So, the family headed south to church while Will and I headed south to MCV and our friends at the pediatric ER. Angela, the same nurse as two weeks ago, was our assigned nurse, and was able to convince the resident of what we needed since the on- call neurologist was our least favorite and by far the least helpful. The blood work was drawn, the IV fluids were hung, and the long wait began.

Last time, Will's diastolic bp (the bottom number) dropped extremely low, so when we saw a similar trend, Angela got suction and a non-rebreather mask ready. Really, this is just not comforting. It is comforting that they are prepared, it is not comforting that they need to prepare...

To give you the short version...Will did drop, but the respiratory assistance was not needed. The nurses and I did brainstorm about what might be causing such dramatic issues. So, for now, we are checking his glucose during infusions to see if he is becoming hypoglycemic, and we are ordering a pulse oximeter to see how low his oxygen saturations fall in conjunction with falling diastolic blood pressures. In other words, how is he perfusing? He gets mentally fuzzy, so is his brain lacking oxygen?

What we do know is that Will's baseline has dropped. We struggle to get his bp over 100/50. That is a drop from the 120/76 that we consistently had just two months ago. We know he is dropping even further during infusions. This is either worsening autonomic dysfunction, which is really not good news, or this is ... something else not working right which is again, really not good news.

Thursday, March 25, 2010

No, Really???

I took Ben to the orthopedic for a follow-up for his knee. It is slightly improved, so we are to continue the same treatment and return in a month...which means six weeks because in a month we'll be in Cincinnati.

While he was talking to the orthopedic, he mentioned that his hip hurt and pointed to his belly button. The doctor was so gracious. This big hulk of a guy just sat down next to Ben and chatted with him for a while and then checked his abdomen. He poked and prodded, then had Ben lift his head while lying on his back. He then blithely said, "He has an umbilical hernia that needs to be repaired."

Um. I'm sorry. This really doesn't fit. How do I manage to have two children undergo surgery in two different states and then recover at the same time? May I please have a do over?

After having to look up the surgeon's number again (ok, I really had Billy look it up because I had had no food and had only my blackberry with me and wasn't firing on all cylinders.), I finally put it in my Blackberry. This will be the third Coleman child on which this surgeon will practice his skill. Most people don't have the phone number for surgeons listed in their phone...I could move to LA, write for a soap opera, and never run out of material. Really. This is getting a little unbelievable.

Wednesday, March 24, 2010

A Great Doctor Is Hard To Find...

Yes, I do realize that I have now posted twice in the same day twice now. :)

For those who wanna know...We saw Dr. T today and, for the record, may I state that I love this doctor? While she may not have all the answers, she is one of those rare birds who is willing to state that she doesn't know and ask questions of others until an answer can be found. Many mito patients go for years without a diagnosis or a physician willing to think outside of the box. From the outset, we had an amazing neurologist who has been spot on with every diagnosis that has been added to Will's pedigree...and trust me, he has quite a pedigree at this point.

So, after last week's fiascoes and this week's traumatic infusions, we were very happy to have an appt with Dr. T. Did she have all the answers? Nope. We have no clue why his blood pressure is dropping so dramatically during his infusions. She agrees that he isn't perfusing adequately, which, in lay terms, means that his organs, including his brain, are not receiving enough oxygen which means that they suffer damage every time this happens...which right now, is twice a week. Not a happy thought. She agreed that we needed to see our cardiologist.

But, on the other hand, she is willing to pursue the ketone issues and plans to chat with our previous neurosurgeon regarding the diagnosis by Dr. Crone in Cincinnati. (Dr. Crone just happens to be our previous surgeon's mentor...) She is also going to talk with her favorite sleep doctor for a second opinion on the sleep study/sleep apnea diagnosis. Her belief is that we have central sleep apnea, not obstructive...which would make total sense.

So, do we have all the answers? Nope. But we have a great doctor who truly cares about William and is willing to doggedly pursue answers in the hopes of sustaining William for as long as the Lord allows... And really. Isn't that what anyone who is sick wants? A doctor who truly cares and is willing to go to the mat for her patient even when he has a rare disease that no one understands? While none of this is easy, Dr. T helps make it all a little more bearable. And that is not to be underestimated.

When you start adding it up...

We have an appointment in Toledo on April 12th with the cardiologist. We have to be in Cincinnati no later than April 18th. So what do we do? We call Angel Flight!

The current thought on the subject is that Will and I may fly from Fredericksburg to Toledo via Angel Flight. We will then get a rental car for our time in the lovely city on the lake. Then, Angel Flight may fly us from Toledo to Cincinnati. Billy would drive to Cincinnati and meet us there...saving his leave for the second surgery. is the drawback. In order for Angel Flight to locate pilots willing to fly us, they need the weight and dimensions of all the medical equipment we will take with us. So, off I went this week to measure and weigh everything.

Which means I had to list every piece of medical equipment we would have to tote with us. Which really means that I had to realize how much had changed since...December. Now when we leave home for more than three days, we have to tote a nebulizer, an IV pump (We can't take the pole because you can't take anything with wheels on an Angel Flight.), a box of IV supplies and dressings, a bp cuff and stethoscope, and a cooler with enough IV fluids for a week. Let's not forget the 13 drugs he takes a day. Add up the equipment...and you have 45 lb. Did I mention that you are limited to 50 lb of luggage on an Angel Flight?

So now the question do I pack this stuff so I can manage it on the other end with no wheels? If Angel Flights are possible, will we have to go with only the clothes on our back?

Most people weigh their suitcases when they go on vacation. We get to weigh durable medical equipment...Do I take my hair dryer or a nebulizer? These are the moments that make me realize that we do have a bit of a unique life...which I realize is a bit of an understatement, but occasionally I forget that...

Saturday, March 20, 2010

I Have No Idea

Other than this blog, we really don't make a public display of the situation. But, Will specifically, and our family generally, are prayed for regularly on Sunday morning. So, those that really don't know us that well are left not really having a clue as to why we are regularly mentioned.

This past Sunday after church, someone asked me what was wrong with Will and was this serious. I gave them the brief overview of the situation. They presume Will just gets headaches. Well, no, because a brain stem compression actually affects your autonomic nervous system which is what controls pretty much your basic functions of breathing, heart rhythm, and organ function. OH! You see reality begin to dawn. Then they voiced the question that no one has ever actually asked me. Is he going to live to be an adult? And you stand there looking at them going, "I have no idea how to answer you." To say, I have no idea is to voice the fears that I have. To say yes would be a lie because I truly have no idea. Will we lose him tomorrow? I don't think so. Is this shortening his life? Absolutely.

So I answered them, "I have no idea." They really did not have a clue how to handle that answer, and I had no idea how to further respond. We stood there for a moment just staring at one another. Am I afraid? Um, yeah. Do I trust in God's providence. Yes. Do I want to cry in grief because this shadow hangs over us? Definitely. So, they graciously and with shock all over their faces hugged me and headed home while I stood there, knowing my family was waiting in the van...watching this conversation...and not wanting to have to explain to Will and everyone else what had transpired.

So, once again, the non-emotional face goes on while inside, I'm stomping my foot and screaming because I don't want to have to answer "I don't know." I want to be able to say, "Of course he will be fine!" But I can't. And there is still so much to be endured...yet we will have to continue to say, "I have no idea."

Friday, March 19, 2010

Save the Date!

Yes, we have a surgery date. April 20. Which means being in Cincinnati no later than the 19th for a pre-op appointment. And no, this can't be easy.

We need to see Will's cardiologist who also specializes in autonomic dysfunction...Will's deterioration is a bit alarming. Not only are we now infusing him twice per week, but now at night his hands and feet turn red and are hot. Lovely. He asked me what it was...our lovely nemesis... autonomic dysfunction. So we need to see the cardiologist, but the only available appointment is April 12...8 days before surgery. So do we kill a few days in Ohio or do we come home for two days and then leave again?

So, Will and I will leave no later than the eleventh toting with us an IV pole, an IV pump, a CPAP machine, a nebulizer, twelve drugs, a box of IV supplies, a cooler with IV fluids, and a few clothes. The question is, if we Angel Flight up and back, how do we manage all of the equipment needed to keep my son functional? If we drive up and end up staying, then how do we afford this?

Wednesday, March 10, 2010

Do You Still Trust?

Will was in the ER this afternoon/evening. (See prior post.) On the way home, we chatted, stopped for a slushy from Sonic (not on his diet, but sometimes the occasion warrants something special...), got gas, listened to music...We'd been together for hours on end, but we do this a lot, so it was a companionable ride.

Then the Aaron Shust song came on my IPod..."One Day." I started to cry and thought, "One day, Will is gonna be before the Lord and he's gonna be perfectly well!" It got me to thinking...

Before Will's first ever surgery, his first Chiari Decompression, we talked about what it entailed, and I asked him if he was afraid. He said, "No. I know in Whose care I am." He totally trusted that the Lord was in control of the situation. Fast forward almost three years and lots of medical issues...and 3 additional surgeries with 2 more looming. He is followed by almost a dozen doctors in three different states. He takes 12 drugs a day, most of them multiple times. One is an oily, gritty, foul liquid that he tries to avoid. :) (Can't say I blame him!) He receives a weekly IV which ties him to a pole, a bag, and the inside of the house for at least 8 hours. He has seen more ER visits in the last year than most of you will seen in a lifetime. (We don't know these nurses so well cause we just met 'em!)

Yet he doesn't complain. Yes, sometimes he's angry. Sometimes he just wants to be normal. But he doesn't complain and say, "Why me?" So in the car tonight, I was listening to this song, and tears came to my eyes, and I asked him if he was scared. And he said, "Wouldn't you be?" Well, yeah! Then I asked him if he still trusted in the Lord's care of him...and he whispered, "Yes. I couldn't do this if I didn't." And he was crying. I pulled over and just wanted to hold him. I really don't know how he does it.

You Know You Are in Trouble When...

They start to recognize you. You walk into the ER and the guy that greets you says, "I remember you. When is his birthday?" I suppose that if he had remembered Will's birthday, then we would have really been in trouble. :)

So, yes, we were back in the ER today. There was the headache, the random pain (neuropathy), the "not feeling well", combined with the mean-as-a-snake attitude. Um, why don't we check that blood pressure? Um, why don't you go get your shoes and your ER bag?

So we arrived at our favorite ER at a good time. They weren't prepared for us despite calling our doctor, but they very graciously listened (yes, we have seen this doctor before) and did exactly what we needed. Part way through the eval, our favorite nurse walked in. I had looked for her when we arrived but didn't see her. Unfortunately, our nurse wasn't willing to switch, despite Diana asking her to do so, so we didn't get to see her much, but it was wonderful to see a familiar face.

Will's bp did drop to 80/33 which was a bit guaranteed us a couple of nurses pretty promptly. :) Fortunately, we got here in time before it reached crisis point, and the infusion is doing its job...while his bp is anything but great, he is at least more stable.

We should finish here soon...our nurse at home is contacting Dr. T to request a second infusion each week to try to stabilize this bp. Not sure what the next few weeks/months hold as we head toward these surgeries, but fortuntely, I don't have to worry about that. That is totally in the Lord's hands. All I have to manage is today. And as for today, we were very blessed with good care, and amazing son who filled in the gap, and Beth who helped calm Caroline when I had to leave...again.

Friday, March 5, 2010

Is it April Fool's Day?

So, we're not heading to Cincinnati this Sunday. I received a call this afternoon from Beth, the nurse, who said that the orthopedic who was scrubbing in with Dr. Crone was going out of town on an emergency, so the surgery had to be rescheduled. Not that we have a date at this point, but she hopes to call early in the week with one.

Part of me is grateful for a few extra days (or weeks?) to get things in order here. The other part of me is screaming inside. Don't they realize that Will is not stable? His systolic blood pressure (the top number) has not been over 100 in two weeks. And the diastolic is constantly in the 40's. He's taking his meds. He's receiving his infusions, but it's not holding him.

So do we go to two infusions a week? How do we manage this knowing we still have months to go to get to the big surgery?

I do realize that they are doing their best in Cincinnati and that Will isn't their only patient...but could we please get this show on the road? Literally?

Thursday, March 4, 2010

Surgery Bound

Since the last post was so whiny and it was all about me, it would be better if I filled you in on what is medically happening.

We had the pre-op appt today, and Will's blood pressure remains low. (92/47) His next infusion is set for Saturday. Chris is officially joining our church on Sunday, so we will be departing Sunday right after worship. We probably will have to stay overnight enroute, but we have to get to Cincinnati for the appt with anesthesia.

Will's surgery is scheduled for 7:15 on Tuesday morning. I really don't have a clue as to how long it will take. I do know that he will go to ICU for 24 hours after surgery, then will be in the hospital for 5-7 days.

If you would like to send an ecard, you can go to, part of Cincinnati Children's website, and they will print it and deliver it to Will. And it's free! While I recognize it would take valuable time, and I understand if you can't get to it, but I do know that he would be encouraged to know that he is loved and that people are praying for him.

He is handling all of this fabulously, but it's so hard. If you think of it, please pray for peace for him and for us and for the rests of our family as we continue through the murky waters...with full confidence of the Lord's care and protection.


Btw, I will post updates as I have them. If you would like to post a comment to Will, feel free to do so on the blog's comment section, and I'll make sure he hears it...and I'll print them as I have opportunity so he can have a physical reminder.

THE Call

They apparently are not big on advance notice. I received THE call yesterday afternoon that our surgery was at 715 Tuesday morning, and we have a pre-op appointment with anesthesia Monday. I am so glad we finally have a date, but oh my, what has to happen in a very short time.

It isn't like we can just throw clothes in a suitcase and hop in the car. THAT would be easy. Instead, there were a bazillion phone calls to make...requesting refills on he two meds that are compounded which the hospital there does not have, making a pre-op appt with our pediatrician, requesting IV supplies for the extra infusion that has to be Saturday, requesting a nurse to start said infusion, finding a place to stay for almost two weeks that won't absolutely bankrupt us while also not putting us in the worst area of town (We were told to NOT walk around outside the hospital..not a great neighborhood...), calling dietary at the hospital to find out what is available since Will has such a healthy (ahem) list of food allergies, getting the van serviced so we don't void our warranty, ordering Caroline's birthday presents since there is now NO time to shop and her birthday is Saturday, and rearranging doctor appointments for the next three weeks. I know I've forgotten something, but at this point, the kids think the phone is a permanent appendage and that their mother is present in body but totally absent in every other way. Needless to say, that creates its own little nightmarish there is NO parent here and mutiny is about to occur.

I don't mean to complain about the difficulty. I feel like that is all I do these days. It's just so overwhelming. It's like a massive earthquake is hitting and we're holding on to the walls to hold them up but you feel like they are gonna fall any minute. I know they won't, but that doesn't mean that permanent damage won't occur. When your daughter cries because she just needs her mom and her mom just isn't available because of the tyranny of the urgent, well, it makes me want to sit in a heap on my floor and cry with her. I miss my kids. I miss playing with my kids. I miss joy.

I'm grateful for my kids. I'm grateful for the doctors who are striving to help. I'm grateful for insurance despite a massively huge deductible. (If the deductible doubles every year and we still manage to meet it in a couple of months, what exactly does that say???) I'm grateful for the women who come and help educate my children. I'm amazed at the Brooks' willingness to move into our home and care for our children while we're in Cincinnati with William for an unknown period of time. I'm not ungrateful. I'm just weary...and very tired of being so.