Friday, September 25, 2009

And the answer is...

I don't know.

We took Will to see Dr Johnson (ENT). He looked in Will's ears. He performed a hearing test. Yep. Normal. Um, so how do you explain the transient hearing loss, ear pressure, tinnitis, and headaches? Well, Dr Johnson thinks that Will's sodium levels spike after an infusion (it is saline after all and he is on medication that causes him to retain sodium), and the fluid builds up in the mechanisms of the ear and muffles the hearing.

Except that Will's sodium levels remain on the low end of normal. We don't have a post perfusion sodium level, but we do have them only a few days apart and the number is rock solid. Three tests. Almost identical results.

Dr Johnson (ENT) recommended slowing the rate of infusion down (give the fluids over a longer period of time). The prescription calls for two liters over four hours. Last Saturday's infusion took 5 1/2 because it was gravity fed. Well, the nurse can't change the order, so today's infusion was four hours. So I guess we'll see tomorrow whether we have issues or not.

Dr Teasley returned my call this afternoon. We're adding a new med to Will's regime to try to sustain the blood pressure. His heart rate is finally stabilizing, but now his bp is unstable. She thinks his body is just compensating...not a great sign. She called Dr Cohen's office (Cleveland Clinic...where we are scheduled to go in Dec), and apparently his wife is gravely ill and they won't see us any sooner. She recommended finding another specialist and she would happily refer us to wherever we decide. A message did come across the mito forum that a family called every day to see if there was a cancellation and they got in this week. So guess what I'm gonna do every day??? Dr Teasley is referring us to a neuro-ENT specialist at MCV to re-evaluate the hearing issue. Not sure when that will be...

So, as a recap...we just don't know. We get to add a doctor (neuro-ENT), we get to locate another doctor (mito specialist if we can't get to Cleveland), and we pray that things do not continue to deteriorate...esp the hearing.

I asked Will today how many days a week he didn't feel well. His answer? "Are we talking my version of feel well or yours?"

Hmmm..."What IS your definition of don't feel well?"

His answer, "Not having pain more than a 2, not being more tired than a 5, not feeling awful in general."

And how many days a week is that?

His answer, "About half."

"So, by normal standards how many?"

His answer, "None."

Ugh. I just hate that answer. I just want him to be a little boy...the goofy boy that coudn't sit still during school so I finally gave him an exercise ball for a chair. The crazy kid who was always bouncing off the wall, climbing a tree, or redefining mischief. The rambunctious, silly, boy who always smiled. You don't realize what a gift that child is until he disappears into a tired, exhausted, grumpy, I-don't_feel-well, can I just sleep? kind of child who knows the pain scale, what his "normal" blood pressure is, when his heart rate is above 85 without bothering to take his pulse, and knows the medical jargon of a fourth year medical student.

As much as I want a cave and a blanket and a piece of chocolate, I think he needs it more than I do. If only it were that easy.

Tuesday, September 22, 2009

Mito forums...

While most of you don't hear from me in forever, I'm busy reading my email and studying the mito forum on yahoo. Being a little fly on the wall of this forum has yielded more information that I gained all summer doing google searches.

Friday, Will again complained of random hearing loss and pressure. I really thought he just was being non compliant with his medications and his allergies were an issue. Well, he actually cried over his ears hurting when his little sister shrieked into them, so I decided to take him in. I fully expected he had at least fluid in his ears, but instead, his ears were perfectly normal. And he passed a quick hearing test. Really. No explanation as to why the hearing issue. Saturday, he came to me after his infusion and said that he was having a hard time hearing again. It just didn't make sense, but I had this growing fear that we were looking at a mito issue.


Today, on the forum, they were discussing Meneire's Disease...headache (check), pressure (check), transient hearing loss that worsens (check), unstable balance (check). Dizziness. (check) Oh, great. Degenerative. (What with mito ISN'T degenerative????) Eventually leads to deafness.

No cure. But the treatment is a low sodium, low fluid diet. You're kidding, right? He's on medication to stabilize his blood pressure that requires him to retain sodium, he's supposed to take sodium tablets, and he is supposed to drink a gallon of water a day and receive a 2 liter infusion each week.

Am I jumping to conclusions? Maybe. Is it a little too real? Yep. He gets an infusion and he has hearing loss.

Nothing with mito is easy. Nothing. How much more can a 13 yo take? How do we even deal with one more thing?

Oh, Lord. I believe, but my unbelief because right now, I just don't know how on my own to manage mito, much less this. I've never hoped for me to be wrong as much as I do at this moment.

Saturday, September 19, 2009

So how did you spend your Friday?

Last night, Billy and I went to Pancho Villa Mexican Restaurant with our friends the Brooks. The weather was lovely. The food was wonderful. And the margarita was very much enjoyed...after what was a very long day in which NOTHING went according to plan. And I do mean nothing. I have no doubt in my mind that it was indeed the Lord proving once again who was really in control. And it most definitely is NOT me.

The plan was school in the morning while we await the dishwasher repairman and Will gets his infusion, and finishing school and household chores in the afternoon.

However, the day started with my oversleeping (the incessant Blackberry did sing its song, but I apparently dismissed it and continued snoozing) and awoke to an acrid odor emanating from my kitchen. I hopped out of bed and hobbled down the stairs (ugly knee injury in a fall down the stairs...story for another day!) just in time to hug my very tall sons as they head to Williamsburg for the day. They did a fabulous job of packing their lunches and fixing their breakfasts (acrid odor and all) and leaving on time without the presence of Mom. Part of me is every so proud of them and the other part is very sad that I am indeed working myself out of a job. They are becoming independent adults!

Fast shower, put on my referee stripes because the children are uncooperative, and head to the schoolroom...and the beagle escapes. Again. We leave the gate open for when he returns, take the labs, and head to the schoolroom. (Mary had a lamb, but we have labs!) Call the pediatrician because Will's ears hurt. 4 PM appt. That should just work...the home health nurse who was supposed to come Wednesday is coming today at 11, so we should finish the infusion and have just enough time to make it. Or not. At 11:15 I receive a call from her office telling me that she has had an emergency and won't be here til 1. A four hour infusion means that I now have to see if Dr Aaronson will see Will in 30 minutes. They say yes, so we race for shoes and into the van and into Fredericksburg. His ears are clear. He passes a hearing test. He is having random, unexplainable pressure and hearing loss and Dr A has no clue why. But, we do come away with a prescription for a cream to kill the chiggers that he and Caroline picked up in the woods on Wednesday. We immediately head back home because the dishwasher repairman is supposed to come. Only, upon arrival, we realize that we left the other gate open for Jackson, and now all three dogs are gone. At 1230, I call Sears and they inform me that he is coming, but he is running late. Great. Lunch, then back to school til the nurse comes.

But no. We have no bread and that simply cannot be, so I throw bread in the Bosch and send the kids to ride their bikes for 30 minutes. But now the dogs are home. And they are labs, so they very obviously found the local muddy pond. Instead of one chocolate and one black lab, we have two chocolate labs. They can't be bathed outside because the hose was run over by the mower. (First lesson to teach your kids when they mow the lawn is make sure the hose is put away!) If I leave them muddy, then they will probably "accidentally" be let in by one of the kids and then we'll really have a mess. Fine. So, I pick up this 80 lb lab and dump her in the tub and start the bathing process. I hurt my knee so I can't kneel on it, so I finally sit on the edge of the tub with my feet in the tub to bathe the dog. I'm looking like I took a swim in the local muddy pond, but eventually all 3 dogs are once again silky and beautiful. It's also now 330 and the nurse still has not arrived. I hurriedly cleaned the bathroom and then race for the shower. And she arrives. I throw on some clothes and the phone rings. It's Sears. The dishwasher repairman isn't arriving because he is headed to the hospital for a knee injury. Tony, the nurse, meets all of the children and my granddaughter and I finally fly down the stairs...only she realizes that she is supposed to actually infuse William. And it's now 415. And it takes 4 hours. And she has 3 other patients to see after William. So she reschedules for Saturday.

I dry my hair, make tacos for the kids for dinner, and open a can of vegetables. (I almost never use canned vegies!) Only my can opener breaks. Really.

Dinner is finally on the table, everyone is ready for bed, and I head out for a wonderful dinner with Billy and the Brooks, and I enjoy my margarita. It was a lovely evening filled with laughter and friends and a respite in the middle of a swirling storm.

By the way...the nurse is wonderful and loves kids and dogs. But she hates cats. :) The Lord loves to show us who really is in control. :)

Tuesday, September 15, 2009

Myrtle has entered the building...

Myrtle arrived this evening. She is a lovely, slender thing standing rather silently in the corner of the room with her little green "battery charging" light on. Andrew has decided to call her Bob. Bob just doesn't seem to fit, but I'll allow him to call her what he will. Here, she shall remain Myrtle. :)

The nurse is due to arrive tomorrow. This could get interesting. The order on the bag states that the 2 liters should be administered over four hours. Four hours is a long time. Especially when you have a stranger hanging out in your house with your son. Better yet, she is hanging out in a house with loads of kids and 3 dogs and 2 cats. The yard hasn't been mowed in weeks since I blew the engine in it (No, I wasn't racing it!)and the guys are working on it in their spare time. The deer got the garden so the garden is one huge overgrown disaster. The young chickens keep escaping the coop, so we even have chickens running around the property. We won't even discuss the house. We look very Beverly Hillbillies right now, so I have this sense of trepidation that she is going to dislike children and animals and she will totally be stuck with us...and us with her. So while I pray that we have this wonderful, maternal nurse with twelve of her own kids that are all grown and so she dearly misses the bustle of an insanely busy home, I fear that we have...the complete opposite. There's faith for ya, huh?

Myrtle is charged and ready to go but my batteries are on empty. I shall bid adieu and head to the sweet comfort of rest while I remind myself that the Lord really does have this under control and I really don't need to try to manage it all by myself...really. :)

Monday, September 14, 2009

First day of school....not.

A week later than most "normal" families, we were scheduled to begin our classroom work today. But we are not normal, are we? Nope. So, instead of eating breakfast and meandering our way in a nice straight line to the classroom in our very detached garage, we overslept (my alarm did NOT go off!), raced through a shower, (fortunately) threw on some clothes that could be seen in public, made oatmeal (it's fast), and called everyone to breakfast. And then found Will on the sofa.

His bp was down, his heart rate was up, he was fatigued, and sick. And in a lovely mito crash. Erlene, the amazing friend, answered my call and came to substitute teach the first day of school while Will and I headed to the ER. Dr Teasley had called ahead to Mary Washington for us so we didn't have to explain everything when we got there. Or so we thought.

Let's just say that no one understands mitochondrial disease and leave it at that. They finally got an IV started, but then they put us in a room with at least nine recliners and four televisions...playing different stations...all competing for the loudest volume. Will, of course, prefers Mary Washington Hospital, because they have cable tv in their rooms and he gets to sit for hours watching Animal Planet and the History Channel. Unfortunately, they were competing with CNN and an entertainment news channel. Our personal DVD player recently died, so we were pretty much stuck. Not a great environment for my 13 yo son who has a compromised immune system and was in the same ER room with who knows what illnesses and watching television that really was not appropriate for him. I was a bit frustrated, to say the least.

On the other hand, they did recognize that they had no clue and allowed me to help them determine that he needed a second bag of fluids. His bp and heart rate did stablize, and he finally came back to life and got goofy. Yep, he's back.

Fortunately, home health is finally ready to roll. Myrtle will come live at our house tomorrow, and the nurse will arrive Wednesday morning to begin an infusion. I hope she likes kids and dogs, because she will get to spend lots of time here. These infusions take a while, so she is kinda stuck with us. And she gets to do it every week! :)

Hopefully Myrtle and her magic fluids will eliminate some of these lovely ER runs...and maybe we won't have to figure out how to replace our portable DVD player quite so quickly.

Friday, September 11, 2009

Do you treat pediatric patients?

That became my favorite question today. I needed to find a home health agency to administer Will's weekly infusions. Ha! After I called the first two...and the next 20 agencies, I discovered that most do not treat pediatric patients! I finally located one that did...only to discover that they don't participate with our insurance company. Well, since we've already paid out a gazillion dollars, we would really like to not lay out another $6,000, so I finally broke down and called the insurance company as well as our case manager.

There is now a company waiting to provide this lovely ER-avoiding treatment, but now I have to call the dr back and ensure that they have ordered an IV pump and the fluids that the nurse will administer. I thought this was gonna be easy, but, alas, tis not.

However, Will is approved for weekly infusions, we don't have to leave home to get them which means I don't have to take him to an ER to be exposed to who knows what, and the rest of our life can continue while he's hooked up to his new friend, Myrtle. At least I think we should name his pump Myrtle, don't you agree? Should you have a different name that seems more appropriate, then please let me know! In the meantime, I am going to get some sleep so I can call tomorrow and request that Myrtle arrive unscathed and in a timely fashion so that maybe, just maybe, we'll not need the ER again for a long while!

On a serious note, please pray that his veins hold up well to the infusions such that we don't need to put in a port. Will's immune system is compromised thanks to this lovely disease, and a port is like a constantly open avenue for infection...

Off to slumberland I drift....

Tuesday, September 8, 2009

I'm so out of creativity

Sorry. I can't come up with anything catchy and cute tonight with which to title this one...But I can promise it will be short! :)

Talked to Will's dr tonight. Short answer: She is calling Cleveland for us to see if she can get our appointment moved up. Will's autonomic dysfunction seems to be worsening, so we obviously need to do something more to get him back to something that resembles stability, esp before winter and cold and flu season hit.

She also is going to contact our new and very appreciated case manager at Anthem to request home health for six weeks for weekly infusions to see if that will help the stability issue and prevent the many ER visits that always seem to be looming.

She asked me to call Dr Grubb in Toledo and discuss the cardiac arrhythmia we saw Friday night and continued to see today. Sitting, his heart rate is in the mid 80's which is ok but not awesome. If he moved around today it hung between 130 and 164...I don't mean running around, I mean just moving around. That is way too high for so little motion.

We're gonna talk by phone again as soon as we have more information...and I'll keep you posted.

I appreciate your prayers. I am blessed beyond belief with the outpouring of love and support I've been shown in the last few weeks, and I deeply appreciate it all. I am overwhelmed with Will's issues, with the needs of my other kids, with impending school needs, and with my need for sleep and running. :) Thank you for your prayers and for loving my family.

Saturday, September 5, 2009

How do you spend YOUR Friday nights?

Our family is often seen swimming upstream. We have 8 kids. We home educate. We drive a very non politically correct vehicle. (15 passenger vans are NOT green! Ours is actually silver!) And we spend our Friday nights at the ER while most of America is sleeping.

Will thoroughly enjoyed playing with a friend on Friday. It was a rare opportunity that was enjoyed to its full capacity. Unfortunately, what had been simmering all week became a full blown mito crash that night. He went to bed on time even though he argued with me that he had not slept well all week. At midnight, he was wide awake, came downstairs where I was working (yes, I know. I should have been in bed as well, but that basement is almost finished!!!) and said, "Feel this." He was referring to his heart rate which was a whopping 132 beats per minute. Considering that 100 is tachycardic and is our "protocol number" for an ER visit for fluids, he was well beyond the point of wondering whether we should go. No waffling here. I sent him off to get dressed while I gave instructions to my oldest two boys who also should have been in bed but weren't, emailed Billy who then came home from work in order to go with us, and packed "The Bag." (My gym bag is always ready to go. All I have to do at any given time is throw in either gym clothes or regular clothes and I'm set for an indefinite period of time.)

After deciding to head to MCV instead of Mary Washington since they have a pediatric ER, his records are all there, and his specialists are all there, we headed south. The entire trip down I kept hearing him sigh in the back seat. Not a good sign.

The short story is Will's body does not make enough energy and he has no reserve capacity. While this seems like no big deal, it's a very big deal. His organs all require ATP (energy) in order to function properly. In its absence, damage occurs and it's irreversible. That is why this disease is progressive. With each crash, we potentially damage those organs even more. Not crashing is the goal but we haven't been able to prevent these crashes from occurring regularly. The sighing on the way down was an issue because it represents his body burning excess oxygen trying to create more energy that it just is not able to produce. It is the first time that has happened, which has me concerned... Is this a progression of the disease?

In previous ER visits, one bag of fluids is all he has needed to stabilize. Last night, one really didn't quite cut it. They were looking at admitting him because they really have no clue what to do with mitochondrial disease, when I asked them to hang a second bag and run a second bolus (a lot of fluid in a short period of time). Finally, his heart rate dropped below 100 but it still bounced all over the place which means that his heart rate was inconsistent...another sign that his autonomic nervous system is not coping well.

So, we finally were discharged and arrived home at 645 this morning, just in time to say good morning to my youngest three. Billy and I managed a couple of hours of sleep (thank you older kids for helping!) but we are exhausted.

On Tuesday, I will have to call Dr Teasley and determine if we need to make any further changes in his protocol and/or medications. Until then, we just watch him for further signs of distress which will provide us with another opportunity to visit the MCV ER and hang out with Kati, our nurse last night who promised to keep a look out for William and snag him as her patient when he returns. She actually read through Dr Shoffner's notes (Atlanta dr) which totally earned my respect because they are anything but an easy read. (She just wanted to understand Will's condition so she could help. Amazing!)

Thursday, September 3, 2009

Mito. It really is a four letter word.

I finally found a mito forum, thanks to Traci. I read daily about other families and their struggles with this heinous disease. Everyone who has it presents differently, but no matter how they present, you realize that they are fighting the same battles you are.

This week, there was a family who rejoiced in the birth of a new daughter...and that night grieved over the loss of one of their other children who suffered with mito. How do you even process that? There are single moms out there who have a child in the hospital for six weeks and have to fight the school system to get their child enrolled in a home bound program.

So, we are fortunate. I home school, my son is not in the hospital, and I'm still married. Does that make today easier? Nope. When Will comes to me complaining of random pain, I have nothing to offer him. He can no longer take Tylenol, so do we take Advil? Do we just learn to deal with the random pain? For how long will we be able to do that? What about his body temp? He's suddenly burning up...but now he's cold. He's not sleeping so he is exhausted and has an insanely short fuse. There's always this sense of impending doom, like I am going to wake up tomorrow and it's gonna be an ER day instead of a day preparing for school. Oh, and we have to plan our school year to accomodate Will being non-functional and maybe my not being here to teach while still moving everyone forward.

One of the moms on the aforementioned forum wrote a blog for the National Organization of Rare Diseases. It can be read at It makes me very sad for her and her family because I understand what she is going through. It also makes me realize I'm not so alone in this world in grieving over my son's health issues and what it is doing to him and to my family.

Today we got a call from a case manager with our insurance company. Woohoo! We finally got a case manager. This is one of those, "I wish we didn't need it, but boy am I glad we have it" moments. Finally, one person who can help navigate the insurance nightmare. Someone who can help find ways around the appeal processes. (I have two appeals I am currently writing.) She asked me loads of questions about Will's medical history, does he take any meds (um, you mean the ten he takes every day???), and finally asked how this was affecting our family financially. Amazing.

SO, now that I am finished being depressing, I'm heading to bed. My basement is almost finished, but my office and school room look like a tropical storm took up residence. Tomorrow is another day! Maybe we'll even spend it cleaning and not in the ER!