Three Years of Kendall.

 

 

Ok first a few editorial caveats about the above video.

1.) It was created in powerpoint, which does not translate well to web uploading. That should just about cover most of the issues you will see with it (which means some slides are super fast and others are super slow and this gets the music timing all off whack.)

2.) Read that first slide all the way before you hit play – reference #1 above.

3.)Also I see that it took the liberty to change a few of my fonts. I hate fugly fonts. But that is what you will now see. At least its not comic sans….*sigh*.

4.) I’ll stop complaining now and let you just watch it.

 

Oh also – some people have said it needs the Kleenex warning attached to it. So consider yourselves warned!

 

I’ll post more about the party in another update – cause I need to get all the pictures off the camera and gather my thoughts about what to say. For now, I’ll just let the video do the talking.

 

thanks for watching.

 

Terra

The Strong Silent Type.

I need a serious flashing blinking warning of some kind on this post.

Not only will you need a kleenex or ten, but you will need some smelling salts or some other way of reviving yourself after the shock. Today I have a guest poster. He’s the strong silent type, and I can honestly say that except for our wedding vows I don’t think I have ever heard THIS MUCH out of him at once in my entire life.

This was supposed to be part of mito awareness week – but, for this much goodness, I’m willing to overlook a few days. I am going to backdate this so that it shows up in the right place in the archives – but if you want to share it (and i hope you do), you can click on any of the little social media icons on the bottom of this post, or copy and paste the address in your address bar for this specific post.

Anyways – Ben does a good job of intro’ing his own post, so I’ll stop talking now and let you hear it straight from him. But seriously on the kleenex…go get them now.

 

Hello all in Terra’s blog/web world.

I am Ben Atkinson and yes, I do actually exist. I am not an apparition. Occasionally Terra will mention me in her blog but rarely do you all ever see or hear from me. That is just who I am. I am a behind the scenes, hide in the corner, sit in the back row kind of person who rarely speaks but likes to think when I do, people listen. Also, I have not been blessed with the gift of grammar as Terra has. My thoughts are usually short and to the point, very efficient and conclusive. I have very strong opinions on most things but rarely voice them to others because it would not be efficient to argue and I just don’t like to talk. I will attempt to alter these aspects of my personality here today and expand on what I think.

Terra has been asking me for a couple of weeks to write a paragraph for her blog about what Mito means to me. Well I am finally getting around to doing this but am sure it will be longer than one paragraph. I can only hope that it will be in some form of coherent thought.

What does Mito mean to me? It sucks, I hate it! I would use more foul language to voice my hate but I know my in-laws would not approve and are probably reading this. How much do I hate Mito? I hate Mito with all the passion that burns inside of me.

Now I need to make this next point very clear and keep this in mind as I write the remainder of this. I do not hate Kendall, I do not blame Kendall. These issues that I talk about are not Kendall issues. These are Mito issues. I hate Mito issues. Kendall Quinn is a shining light of strength and joy that I have never witnessed in another human being. I do not have very many people in this world that I would consider to be a hero, Kendall is on the top of a very short list. I simply hate Mito and the affect that it has had on my family’s life. So my anger may be better directed to the results of Mito rather than the actual condition itself. However, I have some control over those Mito results and how it affects me and my family so maybe I will blame the disease and take no ownership of the following frustrations with Mito. But I will own up to them.

What does Mito mean to me? That question keeps running around in my mind and reality is I have been doing some soul searching when it comes to what this question means. What I say over the next few paragraphs may be a surprise to probably all of you including very close family members. But it is the reality of what I have been thinking over the past few years. It is thoughts that I have hidden away in my own inner core of my being because along with my lack of wanting to talk to people I also tend to hide away my own feelings from just about everyone who I interact with. You can image the challenge this posses for Terra who has to deal with me on a continual basis.

So the affects of Mito have caused me to question and wrestle with many differing emotions. Many of these feelings stem towards my ability to be selfish through all of this. Why has this had to affect me, why has my life needed to be flipped end-over-end? I have become bitter, some times more than others. I have been angry, again, sometimes more than others. However I am thankful that both are subsiding.

My bitterness and anger has been directed at so many different focus points. My bitterness runs deep because as I stated earlier I hide my emotions and hold on tight. I have been bitter and downright angry at God. “Why has he done this to me?” I have questioned many times. Yet at the same time I am questioning him I am not reaching out to him. I am not letting him carry me through these difficult times. I have always been the person that could keep it together. I stood under my own power and never needed anyone else. Unfortunately by doing this I have alienated many in my life. I still struggle with this anger and bitterness but it is slowly subsiding. Again because of my own self inflicted need to keep my emotions to myself I was only able to tell Terra of this about a month ago. What is funny (not in a laugh haha kind of way but more of a twisted what am I thinking kind of way) to me now is that so many times God has pointed out to me that He is there. He is taking care of our needs even down to the basic functions of life like toilet paper when we received about an 8 month supply. Terra was asked what we need on her Facebook or blog page and her response was toilet paper. So many people brought us toilet paper. It was awesome but I still refused to give Him the credit. I have made conscious decisions to not give him the credit for sustaining my family. I know in my heart that He is providing and yet I would get frustrated for all the Mito issues he abandoned us on. Twisted, I know. I struggle today to give him thanks for all he has given us but I think I am making a turn. Maybe this letter/blog post is a big step towards the right direction.

I have been bitter towards Terra. (This will not be a rip on Terra section but it is only used for me to explain my feelings through this whole Mito crap and why I hate it so much). I would ask myself “Why has she been so focused on living in the world of the internet instead of me?” I know that Terra has an absolutely amazing support group on the internet and this group is probably reading this right now. Terra has been able to get support and bounce ideas off of other individuals with kids dealing with similar Mito issues as Kendall. I can now say that I am so very grateful for all of you that support Terra. I know that she would not be sane today without you. I know that Terra has essentially received her Doctorate through this experience and internet searches if only for the sole purpose of keeping Kendall alive and wanting more for her. I am thankful that I have been able to steadily work through this and don’t feel bitterness towards Terra anymore. So, Terra I am sorry for this. I know that it is a selfish struggle that I need to continue to eliminate. Besides, how can I be upset with her for seeking the support of others when I myself am not supporting her because I keep everything held tight inside of me?

I always feel that we are separated as a family. Of course, a major part of that separation is my job and all the travel that I do for work. However, I am so thankful for a job. I am thankful that I have very understanding bosses within my department and work in a company that values the needs of my family. It sucks getting that 3:00 am phone call or text from Terra saying there is an issue but I am so grateful that I can pack my bags and hit the road for home with no fear or questions about my job. I am a very scheduled person. If my schedule is broken by some outside force (Mito) I cannot stand it. It frustrates me. However, again I am thankful that I have a position that I make my own schedule for about 45 of the 52 weeks per year. I am able to adjust as needed. Again, I am thankful for that but hate that Mito has caused the need for it.

I am angry at this situation that Mito has caused for my four beautiful amazing daughters. They don’t get to experience life as what many would consider “normal life”. We do not get to take what most would consider family fun “Clark Griswold vacations”. First of all we don’t have the financial capabilities to take said vacations due to all of the costs associated with Mito care but in reality we just have so much junk that we would have to prepare for us to take all the medical crap associated with Mito care. We have been able to take some one night get-a-ways to downtown Chicago but even with that we basically have a mini traveling hospital that we must tote around with us. I hate that most of my family’s get away mini vacations don’t always include the six of us. Too often it is me coming home from travel for the week and Terra going away for some mommy-big-sisters get away time. I am grateful that they can go away together as the big girls need that alone time with mommy. I just hate that this is how they have to get it due to all the Mito issues.

The girls also have to deal with a plethora of people coming in and out of our house. From some horror stories that I have heard we are very blessed to have the team of nurses and therapists that we do have. We get along with them, they have been in place consistently and I get the feeling that they genuinely care about Kendall and my family. In my opinion our two nurses go above and beyond almost daily when they come to our house. Not only do they take care of Kendall but have helped out in so many other ways with the older girls. Although I am grateful that we have care for Kendall it saddens me that the other girls have to deal with all these other people in the house. It saddens me because although they may not think anything of all the faces in our house this Mito crap as created a “non-normal” environment for them to live in and deal with.

I hurt for Kealey Grace and the daily struggle she has dealing with all too often being the mommy to the other girls because I am on the road for work and mommy has no choice but to address Mito issues. Kealey Grace is such a strong kid, she amazes me how well she can keep herself together. Unfortunately I am seeing more and more that she is like her father when it comes to holding in her emotions and then lashing out at her sisters in frustration.

Karissa Lynn is such a lover. She clings to any kind of love that someone shows her. I only hope that I can provide the love and support that she needs so that some day she doesn’t cling to others that are not good for her. I hate that I too often am so wiped out from travel during the week and trying to take over Mito care on the weekends that beautiful Karissa is forgotten. For this I have been making a conscious effort to make sure she is loved at home but I know she still craves it from me.

Kaylen Hope, Hurricane Kaylen as we call her. I love this little bunch of spitfire more than I think I could ever express. Her stubbornness and individualism reminds me of Kealey Grace x 10. The big girls have always had the outlet of school to get away from the constant reminder of Mito issues. However Kaylen has never had that luxury. She all too often plays second fiddle to the daily dealing with Mito issues. This is just her reality because she is home. I am so happy for her that she started preschool this year. She can make her own friends and forge her own way in this world. However, I have told her many times that she can’t grow up. She’s not allowed to get any older than 4 yrs old.

Even though I have listed out so many things that I hate from the affects of Mito there have been some very positive and downright amazing things that have come out of our situation. As a family we have seen the unbelievable outpouring from so many. So many people that I don’t think Terra and myself could even list out half of the people who have so unselfishly helped us in tangible ways. We have had so many provide us with meals, financial support, babysitting etc. We had a baseball team take us under their wings and do a fundraiser for someone who they had never met or seen. Others have helped coordinate care for our older girls. We have also had so many pray on a continual basis for Kendall, for Terra, for me individually and as a family. I cannot express my appreciation enough for all who have kept us in their prayers. You all are the reasons that my family is still together and I cannot thank you enough. Being the provider for the family I need continued prayer to realize that I need to lean on the True Provider and stop relying on myself for everything. Nothing is mine any ways, everything I have has been given to me to be looked after, if only for a short while. It is not mine to keep.

I have also been able to witness what I will call an absolute miracle. Kendall Quinn is such an amazing person. Not just an amazing kid but an amazing person. Her strength, spirit, and personality are unmatched. Her smile is contagious. She may be in tremendous pain but will still flash that smile. It absolutely melts my heart and brings me to tears at the same time.

Terra and I have learned to take the simple things in life as precious moments. Although sometimes the craziness of four daughters running around the house and all the craziness that ensues drives me to a breaking point I have to take a step back and rejoice that I still have four daughters creating the very chaos driving me into insanity. Reality is that we don’t know how long that will last.

I have learned that next to Kendall Quinn who is the toughest person I know, Terra is right behind her. Terra’s strength and Wonder Woman type qualities (minus the cool outfit) are mystifying to me. I will make this statement that I’m sure so many of you will agree with. Kendall Quinn is alive today for two reasons. First and foremost our Lord is not finished with her yet here on earth. Although I do not know, nor may I ever know what his plan is, I am immensely grateful He has given us this beautiful gift that is Kendall Quinn. Second, Kendall is still here today because of her mothers’ strength to never take no for an answer, to never let a Doctor except mediocrity for Kendall Quinn. Terra I thank you for your strength. You amaze me.

So this has been slightly longer than the paragraph that Terra had asked for but realistically how can I explain all of this in one paragraph. Actually someone please check on Terra, she may have fainted knowing I actual had this much thought going on in my head. I guess my usual self of hiding my emotions and feelings has finally been tipped of kilter, well at least for a short while. I ask for your continued prayers my Kendall, Terra, the other girls and myself. As I work through my struggles in dealings with Mito Issues your prayers may just help me make the conscious decision to accept the help that He has been so wanting for me to accept for so long.

Thank you for letting me tell you my story.

To all of my Ladies; Terra, Kealey Grace, Karissa Lynn, Kaylen Hope and Kendall Quinn… I love you all so very much. Thank you for still loving me through all of this.

Benj.

A Great Day.

Today I was the guest poster over at Dana’s blog – you remember Dana, she blogged here on Tuesday about her son “Looking Great”!

October 20 2009 017 Anyways – today I wrote a post for her blog about what it takes for Kendall (and similarly so many other mito kids) to actually HAVE one of those “great days”. thank you to the many of you who stopped by to read her post here, now go on over to her blog and check out my post and some of her other awesome posts – both about mito awareness week as well as her other myriad topics! She’s got a fun little place over there!

 

Check it out HERE.

 

Anyways – today I had hoped to be able to have a little vlog of the girls telling you about their thoughts on mito and mito awareness week, but our videographer was…not having a very organized day!

I do have some more thoughts I want to get out here, but I wanted to direct those of you who don’t get the notifications on FB to know where I am today!

Go on over to Dana’s, drop me a line over there, or drop me a line here. Or do both! cause you know how much i LOVE COMMENTS! AND EMAILS!

 

thanks for checking in on us – check back later for you nite owls and west coasters on THIS post as I will probably update/edit it later.

and then your regularly scheduled random rambling from me, here on my humble abode, will return tomorrow!

Here is a list of all the blog posts from this week in case you missed any!

Sunday – What is Mito?

Monday – Journey to a Diagnosis

Tuesday – He Looks Great – guest post by Dana

Wednesday – Give and Take

 

Thanks for checking on us!

 

terra.

Give and Take.

Our days are filled with the “give and take” nature of  a sometimes invisible disease process. Most days when blog1 things are going well, there is nothing to give, and it takes nothing from us. Well, almost nothing. Or maybe its just that we’ve already given so much, we don’t notice what is being taken. But other days, the days i want to throw things at the TV when smarmy, smug pregnant women tell me that THEY are going to give THEIR BABIES the best things in life by breastfeeding/using similac/no enfamil/cloth diapering/huggies/drinking V8/taking 5hour Energy, or the commercials about chubby babies playing and cooing happily make me want to break down in tears for the fact that I was too busy pleading with God for my child’s life to enjoy that roly-poly time most babies get – on those days, it feels like so much has been taken from us. From her. From my friends who have had to bury their children.

There is a song that is sung in our church as part of our worship service. It is called “Blessed Be The Name of the Lord”. It is based on a Psalm, and is basically the Psalmists way of saying – no matter what happens, i’m going to CHOOSE to praise you, God.

(lyrics by Matt Redman)

Blessed Be Your Name
In the land that is plentiful
Where Your streams of abundance flow
Blessed be Your name

Blessed Be Your name
When I'm found in the desert place
Though I walk through the wilderness
Blessed Be Your name

Every blessing You pour out
I'll turn back to praise
When the darkness closes in, Lord
Still I will say

Blessed be the name of the Lord
Blessed be Your name
Blessed be the name of the Lord
Blessed be Your glorious name

Blessed be Your name
When the sun's shining down on me
When the world's 'all as it should be'
Blessed be Your name

Blessed be Your name
On the road marked with suffering
Though there's pain in the offering
Blessed be Your name

Every blessing You pour out
I'll turn back to praise
When the darkness closes in, Lord
Still I will say

 

You give and take away You give and take away 
My heart will choose to say Lord, blessed be Your name
That last part is so hard to sing most Sundays. it always has been. Because if i can’t sing it, then i am 
just doing a bunch of lipservice.
it’s not REAL. it’s just a religion, not a relationship.
 But that’s probably a whole other post. What I am trying to say here and now is this – our lives have been
 affected by mito in ways that are hard to
even put in to words.

Somedays I feel “angry” at all that has been “taken” from my baby, or from our family:
The ability to eat and enjoy so many foods without pain (and usually other medical issues that result 
if she tries!)
The ability to play outside like a regular kid.
the ability to go places as a family (she usually stays home with her nurse to not over-stress her body)
the ability to swim, take a bath, splash with her sisters on a warm day in the sprinklers.
the ability to go climb into her own big girl toddler bed.
the ability to have a body free from tubes and wires and sensory overloads and 
not-quite-up-to-par functions of body systems
the ability to live a pain free, happy go lucky life free of hospitals and specialists offices

I could really get going on the things that i feel are “taken”. It would be SO EASY some days to give 
in to the bitterness that takes root so easily in the soul when life doesn’t go “just like we hoped it would”.
But the truth of the matter is, that it has not taken HER from us, so what really do we have to complain about.
And the truth of it is, that all of that, up there, is not the focus of our lives, 
and i never want it to be what takes front and center stage here on my blog.
With the bad comes the good.
So what have we been given, what blessings have been bestowed upon us “because” of what Kendall’s 
diagnosis brings us…
We have been given an insight into what “priorities” really means.
We have watched our daughter exhibit a strength and bravery at her young age that would make ANY 
parent proud.
we have been the recipients of people giving of their time and money to help us stay together 
in times and places it seemed impossible.
we have witnessed the GOODNESS of human nature – and let me tell you – that is a priceless gift indeed.
we have stood on the edge of a dark cliff, looked the abyss in the eye, and said 
“not today – you’re not takin her today”.
 the incredible amount of strength and confidence you get from being able to be in that place is 
an indescribable feeling. Most people will never know what i’m talking about. And for them i am both
incredibly happy, and incredibly sad.
We have seen people exhibit “friendship” and “walking beside us” in ways that are so much deeper 
than surfacey lip-service.
We have learned what it means to be “rich in happiness”, and how extremely healing 
a good deep belly laugh can be.
We have learned the meaning of “simple joys” – of rejoicing in the mundane, the miniscule, the miraculous
 – a first smile after an illness, a new word whose timing
is surprising, a mess made that represents a milestone met.
We have learned that CHOOSING TO BE GRATEFUL is to choose life.
We are learning that making that choice is rarely easy, but it is always worth it.


I read a blog post recently (go HERE to read it for yourself), and it struck such a chord of resonance
in my soul. It speaks of a word the Israelites would use after they had been given a blessing
 (seriously just go read her blog,
she explains it WAY better than i ever could!) How often can we say “Dayenu” – it would have been enough. 
when I think about this struggle
between “give and take away”, I realize that it is not quite right. We were never promised that we would
be “given” anything in life. No one ever sat me down and promised me that i would have four beautiful
baby girls and they would all be happy roly poly Gerber babies free of problems and brattiness.
No one promised that our lives would be without storms and cold nites and hard days and really crappy stuff.
No one has ever promised me that Kendall would be healed completely and live a totally normal life.
No one ever promised us that WE would love from one sunrise to the next.
And so in our good times and our bad times, we must learn to say “Dayenu” – It is enough.
It is enough that we are given this breath, this moment, this day.
It is enough that we have what we have been blessed with by our God.
It is enough that we have memories, treasured times, blessings, life.
God is good when our prayers are answered, and HE IS GOOD when they are not.
It is not mine to say “you give me this, you can’t take that away”.

And so I will continue to sing, with tears in my eyes, and the words faltering out, 
and with a slight tinge of jaw clenching because
these words are not easy to get out:
you give and take away.
you give and take away.
my heart will choose to say,
Lord, blessed be your name.



Terra.


Many many thanks to Lore, author of the blog referenced above, for her post that inspired this post today.

“He Looks Great!”

Today I wanted to share the story of a different face of mito, a little boy nicknamed “Klaw”, who deals with a fatty acid oxidation disorder, or part of that metabolic cycle I talked about on Sunday, where the cycle doesn’t complete due to an inefficiency of the body to be able to break down fats into usable energy sources. His mom is here to share a little about what mito looks like for them, and how for so many mito kids, hearing the words “He looks Great!” can be just as hurtful as it is supposed to be helpful. Mito has been called an “invisible disease”, and it makes the hard work of these kids, and the caregivers and parents who pour heart and soul into their care, be so diminished. This is part of the educational aspect of awareness – learning that there are really so many moving parts to even the simplest of tasks. I hope you learn something new from Dana’s post, and then head over to her blog to find out even more! Leave her lots of love in the comments please! And, as always, thanks for reading!

 

He looks great!

A little phrase that most parents love to hear.

He looks great!

A little phrase that can be detrimental to the care of a child who lives with one of the many mitochondrial disorders.

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My son, Klaw*, popped positive for Very Long Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) thanks to the expanded newborn screening in the state of Virginia. VLCADD is a fatty-acid oxidation disorder that prevents his body from metabolizing very-long chain fats (the tasty ones like dairy, breast milk, olive oil, etc) into energy.

He looks great!

He had four hospitalizations before he was a year old (two were for the same illness). Four emergency room visits. Four sets of medical professionals, only marginally familiar with his disorder, who all thought he looked great.

Children (and adults), like Klaw, can look great moments before they are thrown into a metabolic crisis and at risk of death.

He looks great!

Part of Klaw’s ER protocol for VLCADD states that he has to receive D10 (10% Dextrose) iv fluids as quickly as possible, regardless of his initial vital signs. Most people get D5 iv fluids but the caloric needs of Klaw and others like him necessitates a higher amount of sugar to prevent his body from attempting to metabolize stored fat.

One ER doctor who was so impressed with how great Klaw looked caused a delay of almost 4 hours getting these fluids to my son. This could have, literally, killed him. As a mere parent, I don’t have the authority to call the pharmacy and put a rush on things and my standing in the hallway in the middle of the night asking nearly everyone I saw when they were going to get my son’s iv hooked up did nothing. Feeling powerless over the care of your child is absolutely terrifying. Knowing that you understand the severity of your child’s condition more than the people who are trained to provide medical care to your child is absolutely terrifying.

He looks great!

Most days, I am so incredibly happy that his disorder is being managed well. He leads a fairly normal life. I’m glad that he looks great and is, for the most part, a healthy and active toddler.

Then I remember that he also looks great when he’s sick enough to require hospitalization, 24 hr iv hydration, and a PICC line.

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My hope is that by blogging about Klaw’s disorder and sharing information during Mitochondrial Disease Awareness Week, more people will understand these rare disorders and recognize the often silent severity of them. Please visit my blog Really, What Were We Thinking and feel free to follow me on Twitter at TheDanaK

Mito:Journey to a Diagnosis

This post might be a hard one to get out. Not because of the emotion of it, although that plays a part. But more because it brings up so many questions than answers. That’s always been kind of the nature of it with Kendall, though. More questions than answers.

We are often asked when we first knew something was “wrong” with Kendall (and people usually ask it with a sense of trepidation – like they hesitate to say that something is “wrong” with someone. but it’s ok, there’s something wrong with all of us – i am not personally offended if people word the question like this – because i’d rather have you ASK the question than make assumptions!). But the answer is that we knew from the second she was born. And if we are honest with ourselves, we knew it from a few weeks out from her birth, when i started having lots of problems with the pregnancy, things we just chalked up to my age, to back-to-back pregnancies, to my own chronic fatigue/fibromyalgia problems. Essentially what it seems like was happening was that as her energy needs were becoming more and more overwhelming, my body was failing to keep up with the production.

She was born “blue”, essentially not breathing very well at all. The midwife whisked her away image to begin administering oxygen and sternal rubs, and the NICU was called. You can read more about what happened at birth here. So from the start,  she was having issues in many areas. Her list of “diagnoses” started growing in the NICU before we even brought her home. But we did eventually bring her home. The doctors kept telling us that “any day now she will wake up and she will know how to breathe and eat on her own then”. So we sat and slowly slowly slowly bumped that oxygen regulator down and we shoveled 15cc’s of formula into her with a bottle every four hours (and maybe ignored a little too much the fact that at least 10 of those cc’s were coming back up as puke right away) – but whatever – we just wanted her HOME with us! We wanted to believe what the doctors were saying, that she was just going to wake up and outgrow it and life would be back to NORMAL.

But it wasn’t normal.

We still had to take her for weekly weight checks, where we got a different doctor every time, telling us something different every time. At her 2 month checkup she got her first set of shots and this is when literally all hell broke loose. she started puking and pooping (at the same time) literally almost nonstop for the next ten days. She was pretty much not ever awake during this time, but again, we were told this was normal and fine and we were just still experiencing ‘NICU anxiety”. When this episode started was when i first began to have my suspicions that something more was really going on – as they had never been able to pinpoint her issues in the NICU beyond “transient tachypnea of newborn” – essentially she breathes too fast cause she doesn’t know any better. This did not explain her temperature dysregulation, her inability to gain weight (she actually lost a lot of weight in the NICU), her apparent infection with some unidentified bug (immune issues?), or the fact that she failed the newborn screening three times. We were not told any of this information at that time, only found out after we had left the hospital. They told us that her newborn screen fails were nothing to worry about, and i still to this day do not know WHAT or HOW she failed them. Three years later, its kind of a moot point. She’s been tested for basically everything under the sun that can be tested for, and we get the same results: Abnormal but not diagnostic. Meaning, she has a lot of characteristics of a lot of different metabolic diseases, but does not contain that particular gene defect in her chromosomes.

By 13 weeks old, 3 weeks after the psychotic vaccine issue, she had a wicked cold. She was rushed from the ped’s office girls summer 09 049to the nearest hospital by ambulance, and that was when i knew this stuff was about to get REAL. She somehow survived that nite. I still to this day do not know how. My mom showed me a picture a few weeks ago of a blue skinned, glassy eyed, scrawny little baby, and my heart stopped as i realized that was MY baby, that nite, breathing what i can only imagine is something a step or two above “the death rattle”. If I ever doubted that God has something in store for this little girl – I look back at those pictures, and I think of that nite, and I know HE saved her, for some other purpose or reason. After narrowly surviving that nite, she was transferred to a regional childrens center an hour and a half from our home where she was fully worked up by a team of about 7 specialists in every specialty you can think of.

This is where we first heard the words “possible mitochondrial disease”. A neurologist at this hospital was quite concerned with the fact that Kendall was so extremely hypotonic (floppy), and that she had issues systemically. There was much talk of doing a muscle biopsy at that time (considered to be the gold standard for diagnosing many muscular and metabolic myopathies), but no one felt she was strong enough to survive such a procedure. She had gone in to the hospital with RSV, but ended up with bilateral pneumonia, which quickly turned into sepsis (systemic blood infection). She needed to have a PICC line placed for extra fluids and heavy duty medications, and even sending her to be sedated for that procedure was one fraught with anxiety and worry that she could not withstand the demands placed on her body.

But she did! And in spite of a few more hospitalizations throughout that horridly long spring, she finally began to do well with a specialized formula pumped into her stomach via a small tube we passed through her  kqNG nose and into her tummy (hopefully. sometimes we’d get it into her lungs and have to start the whole horrid process over again.) To say she was “doing well” is somewhat misleading in itself though too, because there were still many many nights when one or both of us would just lay awake with our hands on her chest, making sure she was still breathing. “Doing well” meant simply that she was not in the hospital being septic as we had seen her get in February, so we considered this an improvement.

She ended up getting a g-tube placed and a Nissen wrap performed to try to help force her stomach to work and absorb foods. When even these measures failed to “fix” many of her scary symptoms, we were referred to Childrens Hospital of WI in Milwaukee where they had a motility center to assess more of what was going on with her. From the first ten minutes we spent with our GI (gastrointestinal) doctor up there, we knew we were in the right place. The first few vials of bloodwork that he ordered began immediately to show issues with her metabolic system, and at our second appointment when we had JUST gotten some results back, he paged genetics from the room and told them to clear their schedule NOW and see this kid. Again we were schlepping back and forth from specialist to specialist, getting some pieces of the puzzle here, missing this or that symptom there, trying this that and the other thing in an effort to make her better. But after a few weeks, a lot more tests, and TONS more vials of blood sent to heck and gone, we finally got more confirmation that she had a presumed mitochondrial disease, etiology unknown, shortly after her first birthday.

august 2009 044She was continuing to struggle with meeting milestones developmentally, and at 9 months after yet another immunization trial, she lost head control and many of the skills she had worked so hard to gain in the previous four months of intensive therapy.

She  also at this time had extremely high levels of a metabolic byproduct called “methylmalonic acid”, leading her geneticists  to believe that she had a particular form of mito known as “SUCLA2”. We began to combat the acid levels that were building up in her system with daily injections of very potent vitamin B12 (read: the amount most adults would need in one month, we were giving to her every day, in her little teeny 9 lb body). Genetic testing came back negative for the known genes that cause SUCLA2, but she did have an “as yet unidentified” variant on one of the genes. Still though, this was not enough for our geneticist to say he had found her form of mito disease, and by then we were well into having more pressing issues to deal with than what we would call kendall’s disease!

She had her G-tube switched to a G-J (gastrojejunal) tube to feed her formula directly into her intestines when she was 14 months old, an idea which her team had suggested for about 5 months before I could finally wrap my mind around it. It just seemed like such a step backwards. But it was a huge help to Kendall that august 2009 049 was undeniable. She struggled with lots of illness that winter, at one point sleeping upwards of 21 hours a day, with a body temp that never rose above 94 degrees. Clearly things weren’t all rosy still! But we were still getting little to no direction as to WHAT was going on – just a lot of shoulder shrugs and "try to keep her warm!” I am sure if we were closer to her team, things may have been different on that end. But we’re a stubborn bunch around here, and we were bound and determined to just fix her with our love, so we didn’t run off to the doc every time she did something that scared us to death, and we just learned to try to help her deal as best as she could, as best as WE could.

So even though she had had the prerequisite “3 or more body systems involved in ways that do not otherwise seem to relate to each other” since birth, and even though we had LOTS and LOTS of bloodwork and even a muscle biopsy that all pointed towards a metabolic mitochondrial disease, and even though pretty much everyone on her medical team (roughly 12 docs, 5 therapists, a handful of nurses) assumed it was mito – we still felt like we needed to make sure no other stone was unturned, make sure we weren’t missing something that might be fixable.  So in August of 2010, Kendall and I rode a series of little prop planes across the midwest to the Childrens Hospital of Pittsburgh, to meet with one of the few known “mito experts” in the country. Kendall was admitted for a week of intense testing, and at the end of the week, while we had a few other red herrings we COULD try to chase down, the consensus was that Kendall’s mitochondria were indeed dysfunctional, and were responsibleDSC_0148 for causing her multiple issues. And just in case I had any doubt about this diagnosis, or needed time “for it to sink in”, Kendall started having a massive metabolic breakdown literally within minutes of walking out of that hospital that week, and ended up sustaining damage to her GI tract that she still has not recovered from. Sure she got better, and eventually did come home from the hospital after that episode, and even managed to have a GREAT fall season last year. But shortly after her 2nd birthday, she got RSV AGAIN, and began a series of hospitalizations that ended finally this past June of 2011. {On Wednesday I will blogging more about what life looks like for Kendall these days, how she is affected currently, in case you are wondering about that!}

to say that our road to a diagnosis has been anything but easy is quite an understatement.

in fact, i know of very few people for whom the diagnostic journey was “easy” or “straightforward” in any way. Getting news of a diagnosis of mitochondrial disease is always bittersweet – sweet in that there is finally, blessedly an answer or a REASON for all the issues that so many parents watch their children suffer through; bitter because this diagnosis brings with it no comfort.

Mitochondrial disease is currently without treatment, save for a wish and a prayer attempt at slowing down the progression of mitochondrial failure with a “cocktail” of vitamins and supplements aimed at helping the body complete the metabolic cycle of energy production. Since this cocktail is experimental at best, and contains “vitamins”, insurances do not cover it. The amount of these vitamins and supplements required to see any effect is so ginourmous that it is cost ineffective to purchase them for many families, especially considering that the only feasible way to consume the cocktail is to have it “compounded” – the hundreds of pills required on a daily basis are crushed into liquid form. $$$$!!!  Not only is there no known treatment or cure for this disease, it is often fatal at a young age. This is a hard stat to type because it is the part of Kendall’s diagnosis that I do my hardest to ignore. 80% of the kids who are diagnosed in the first two years of life with mito do not live to see their tenth birthdays. It is my sincerest hope that this statistic somehow magically changes over the next 7 years, for the much improved. This year,though, an exciting announcement was made that some scientists have created a drug that is being proven to drastically reduce symptoms for some of the most affected mito patients, most notably those with severe neurologic implications. This is SO exciting! It is still in the testing phase, and is not available to “the general mitochondrial population” yet, but it is a glimmer of HOPE. It makes me so sad that this news comes too late for so many of the angels we have lost this year, and last, and the year before that…But as the mito logo says – kendallBHOPE. ENERGY. LIFE.  These are the things we hold on to.

Mitochondrial disease is in itself an enigma. there is little rhyme or reason as to when or how it will strike. Some children have relatively mild symptoms right up to puberty, and then cannot survive the stress of the demands puberty puts on a body. Others are hit hard and heavy in the early years, but then seemingly find their stride and even have hope of making some slight improvements. Still others tend to stay stagnant in their symptoms, wherever on the spectrum they happen to fall. But one thing is certain – mito is unpredictable. It keeps you on your toes. The next fever your child gets could lead to major regressions and a lengthy hospitalization, or it could be just a blip on the screen. It is a state of constantly waiting for the other shoe to drop. That was a very hard adjustment for Ben and I as Kendall’s parents – not knowing when or how to react to some of her “issues”. And we still don’t “know” – but I think we’ve come to a kind of understanding with Kendall’s body, with her disease, however or whatever it will eventually be named. We will do our best to support her body in the areas it is weak, and her body will do its best to try to stay even keeled. We have no guarantees…

but does anybody really?

Our path to a diagnosis has not been an easy one. But we are not alone. On a long hard journey, having companions is always best. And above all – we are blessed. oh so very very blessed. Our daughters diagnosis makes us appreciate every sunset, every sunrise, every laugh, every crazy moment. We see life in a way differently than so many people get to experience – seeing the joy in the mundane, the smiles in the rain, the blessings in the midst of the storms. It’s kind of like getting a room on the 87th floor of a building with no elevator when you really would have preferred the 2nd or 3rd floor. It’s redonkulously hard some days, but if you just sit back and take a look at the view…oh its breathtaking. Gorgeous beyond description. Sit back and take it in.terratalkingdays1

I could ramble on this topic for another 37 pages. If you’ve made it this far, thanks.

To all of you on a similar journey – my heart is with you. It’s a journey no one wants to be on, but the rewards that come with the challenges – incomparable.

To our friends who have gone ahead of us and laid the path that we can pick out – thank you is not enough.

To those who walk beside us and with us on a day in and day out basis – keep on keepin’ on.

 

terra.

What is Mitochondrial Disease?

In honor of Mitochondrial Disease Awareness Week, I thought I’d start off with a post about what exactly IS mitochondrial disease (“mito”)? And truthfully – it’s a question that some of the best and brightest minds in the medical world have a hard time answering.

At the base of the science behind mito is this explanation from the United Mitochondrial Disease Foundation website:

Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole systems begin to fail, and the life of the person in whom this is happening is severely compromised. The disease primarily affects children, but adult onset is becoming more and more common.
Diseases of the mitochondria appear to cause the most damage to cells of the brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.

And they have this cute drawing to explain it:

image

Got it now?

 

Neither do I!

As we have been on this journey to figure out a diagnosis for Kendall for almost 3 years now, I have made it somewhat my mission in life to UNDERSTAND more of what “mito disease” is and means. Because quite often not even our doctors can understand or explain it to us. So here is my somewhat more simplified (and perhaps not quite so scientific) version of what exactly mito is and does.

Essentially, every cell in your body has a little engine called a “mitochondria”. The cells know what they are supposed to do because the mitochondria help map/direct the cells – so the liver cells know to be a liver, your eyes know how to be eyes, your gut knows how to digest food, etc. (again – very simple, not scientific – stay with me!) Those mitochondria help make the ENERGY for those cells to do their job, by taking the byproducts of the food you eat, and the oxygen you breathe, go through a super complex metabolic process, something about the Krebs cycle, and bam – they create a little packet of energy, the cell does its job, and you live your life.

BUT – if you have a mitochondrial disease, something is wrong with the directions that that mitochondria is giving out. Maybe it has the wrong info encoded into it because of a genetic defect, or maybe it is just super inefficient at its job of creating energy packets. But basically, that fun little cycle of metabolism is messed up, and not enough energy packets are made. No bueno.

You know how when you are getting sick, or ARE sick, or are getting over being sick – and you just feel kind of “blah”? It’s probably a big combo of things (maybe you puked nonstop, or just couldn’t breathe real well, or some other unpleasant combo) – but the point is – your body is diverting a TON of energy to your immune system to help you GET BETTER from that sickness, so you may not have the energy you typically do to go running or exercising, or even get off the couch and make dinner. Your mitochondria in all those areas are probably still all working, but their energy packets are being “borrowed” by your immune system so it can activate your T-cells and WBC’s etc etc etc.

In a person with mito, that “blah” feeling is kind of “as good as it gets”. Their bodies are in a constant state of needing to “borrow” energy packets because they just can’t make enough energy to go around. This is part of why there can be such a wide variation of symptoms and severity among mito patients – because some people are able to create more energy to go around, and others are just never going to be able to get on top of their energy stores.

I tell most people who we are first meeting and describing kendall’s issues to to think of it like this:

Imagine that you have to pay $5000 in bills every month (i know – i can only think in round numbers, go with it). But – in spite of working 3 jobs and pinching every penny you can, you can only come up with about $3500. You have a choice – you can either shuffle a lot of bills, and pay everyone a little bit, enough to keep the collectors at bay but never enough to actually PAY your bills, or you can choose to only pay certain important ones. Now say you get sick – you can only work maybe ONE job, and your income is only enough to cover the mortgage that month. Everyone else suffers, but the mortgage is paid. Eventually all those late and overdue bills are going to catch up with you though, unless you can figure out some huge way to pay them all off.

this is how Kendall’s body is affected by mito.

k4hosp2i Every day, she is only able to produce a certain amount of energy. Prior to being able to get on top of some of her biggest issues (respiratory, metabolic, and GI – tummy), she was in a constant state of robbing Peter to pay Paul, and even Peter was going bankrupt real fast. Her body is not able to produce enough energy to sustain ALL of the demands her body has. She does not appear to be able to utilize oxygen and food in an appropriate manner to complete the metabolic cycle necessary to produce an efficient “energy packet”. This seems to affect her in just about every system – and we will get more into HOW it affects Kendall this week, but for now, that’s the basic summary of WHAT mito means.

It is a disease of energy metabolism.

So little is known about HOW this disease occurs. Scientists and doctors only began to identify  mito as a unified disease process (vs just a bunch of “really bad luck” hitting some kids multiple times over) in the late 80’s-early ‘90’s. Progress on the research and understanding of mito has been painstakingly slow as due to the breakdown of mitochondria, other diseases can be more “fitting” than this little known and little understood disease process. There is now much talk of connecting diseases like Parkinsons, Alzheimers, Autism and many many many cases of SIDS to an underlying mitochondrial disorder. There are only a handful of doctors around our country who are able to properly diagnose and “assess” mitochondrial diseases in many kids, and their labs are sadly underfunded, and these doctors are terribly overworked. Of the nearly 3000 genes responsible for mitochondrial function, scientists have thus far been able to identify (breakdown and understand and “map out”) roughly 300 of them. Those 300 genes have been linked to about 20 of what we call the “named” mitochondrial disorders – they are initials aimed at naming the prominent symptoms of that disease – MERRF, MELAS, MNGIE, NARP, KSS, and a handful of others. Sadly in med school, most doctors have one class that quickly goes over ALL of those diseases, and again, its mostly only the named ones. THOSE diseases are quite rare in the general population – but unfortunately mitochondrial disease as a whole is NOT rare. It is believed that just as many children are born every year with mito as are born with cystic fibrosis. Mito disease affects just as many kids in varying degrees as pediatric cancers.

And yet it is so unheard of, so unknown how to treat it…

There are so many amazing families out there living with mitochondrial disease, families like us who are having to forge our way ahead in this crazy world, educate doctors out of our own home-grown knowledge, trying to find that balance between doing anything and everything to try to MAKE THIS BETTER for our kids and letting them just be kids in their own ways. I hope that over this week you will enjoy hearing some other stories, learning more about what mito IS and what it looks like, and mostly that you will walk away being able to share with someone else that mito is real, and it needs some REAL attention by people who can help us DO something to cure this horrible disease!

As always – if you have any questions about mito or about anything I may write this week – PLEASE feel free to email me!  terra@2sisphotos.com

 

Thanks so much for stopping by, for reading, for being interested in learning more about mitochondrial disease. Your interest is part of the cure. Your desire to understand how mito affects Kendall’s life, and our lives, gives it a validity and a meaning that some days is hard to figure out. I cannot thank you all enough~ from the bottom of my heart!

 

terra.

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Wednesday.

I am sorry this post is so late. It’s going to be a looonnnggg nite around here. I am sure I have something of more value to blog about later this week – but for now, i just wanted to put out a little update on what is going on. And i hope hope hope that this turns out to be nothing, but since you guys are so good at praying for her, and for us, i thought i’d update first, pray hard, and hopefully eat my words of worry later.

HOPEFULLY what we are seeing tonite is just the end result of the last three days of greatness that Kendall has taken advantage of. As much as that would suck to realize that being halfway normal, and being able to be outside for a total of 10 hours over three days, could have THESE drastic of consequences – the alternative right now would suck even worse.

So HOPEFULLY we are dealing with just some “autonomic craziness”. and not a line infection. We aren’t even going to breathe life into that one right now. But right now, she is having lots of bad apneas. Basically she is not breathing (probably holding her breath due to pain) until the alarm goes off on her oxygen monitor because her oxygen is dipping into not great numbers. I’ve already adjusted this once tonite, and will probably have to adjust it again before i go “recline”, since I probably won’t be sleeping a whole lot! Because she isn’t taking good breaths, her heartrate is pretty high for her normal, and this means essentially that she isn’t getting great sleep. It is probably also elevated due to pain, presumably from her stomach. It’s been giving her a lot more problems over the past few days, and tonite is showing signs of definitely not wanting to cooperate with life. We’ll just say that her gut is working very hard to rid itself of any and everything that has been put into it over the past couple days. It’s not pretty.

blogterratalking1 Adding to the respiratory issues is the fact that Kendall is refluxing MUCH worse over the past few weeks due to a current shortage of her IV reflux medication. We have had problems trying to keep her comfortable without that medication (they are trying to have us substitute a different kind of medication), but the alternative right now is to go on a whole separate infusion to try to control the acid production in her stomach, and we do NOT want or need to add any more craziness into the situation! It is probably becoming an inevitability though if we continue to have issues like she is having tonite. So the reflux is irritating her airway, making her cough very harshly, which further irritates the airway, which makes it hard for her to breathe super effectively either. How many issues is that now, 4? Pain, reflux, coughing like a TB patient, oxygen/HR issues. And diarrhea equal to roughly two weeks worth of poop in one day. Annnndddddd the piece de resistance –

now she’s puking. I cannot even describe to you the horror of watching a child try to puke when their stomach is wrapped around their esophagus in order to PREVENT {all of the above crap i just listed}. yes its as bad as you are imagining. It wipes her out. Luckily we have a pretty good stash of zofran on hand – but the hard thing will be to get it INTO her, see paragraph 3 above – stomach does not want anything in it. Poor kiddo is definitely worn out. our only silver lining right now is that her temp is actually LOW. Typically with “that word which i am not breathing life into” she is super high very quickly. Leading me to believe that her autonomic dysfunction that is annoying on most days, has just kicked it up a few notches into “life-affecting”. her body just doesn’t know quite where to find a happy medium and stay stable. Hoping it figures itself out more on the “not so many issues” side soon. Adding to the craziness is the fact that her poor skin near her dressing is very broken down from an apparent new allergy to pretty much every adhesive under the sun. Not being able to stick her dressing down and protect her central line site is just NOT an option, so we are working with our amazing home health company to find something that will work. We think we have a brand new to the market dressing that is used for mostly burn victims to try – and are praying that this will allow her skin to heal up a little bit. i am sure that fighting that large scale “burn” for a couple weeks is also adding to the stress her system is currently under.

So while you may have seen her in person this past week, and commented on how “awesome! wonderful! great!” she was looking, trust me when i know that it seems unbelievable that she could be doing this badly now. Welcome to Kendall’s world. We like to call this “Residents’ Rude Awakening”. the 50 to 0 crash that Kendall has perfected. *sigh*…

like i said.

it’s gonna be a long nite.

 

Thank you so much for praying. Hopefully I can update with super great news in the morning!

 

terra

When things work out.

Alternatively titled “Just in case you thought you were going to complain about being in the hospital again, your air conditioner will also concurrently bite the dust, and you will be grateful for the air conditioning your baby can receive in the hospital.”

But that wouldn’t fit in the title box.

So anyways – Kendall’s back in the Big House. She’s been doing a few of her little “off” things since last week, but nothing that her nurses or I could put our finger on. So we made it through Showcase (the girls’ dance exhibition for four straight nites) with no major medical issues. Then Monday morning we go to change her dressing like usual, and she FLIPS out. Like the three of us all needed a drink after we finally got things calmed down kind of flip out. As we took off her dressing, her arm instantly puffed up about two inches, and the screaming started. Her TPN nurse said we should not run anything else into the line until we could figure out what was going on with it, we started making calls to milwaukee and the nursing agency supervisors trying to figure out what to do, and eventually came up with the plan to bump her feeds up as much as could into her J-tube, and get a line study done Tuesday morning since we would already be up here for a regular appointment.

So Tuesday morning we wake up and I start the crazy run around getting stuff packed for the day, getting Kaylen ready to go to her friends birthday party, making sure we have snacks and all that kind of stuff – and I realize its kinda hot. Sure enough, the air con had stopped working again and it was already 80 degrees in the house. Which doesn’t seem that warm until you add in the like 200% humidity we have been dealing with, and then its kinda like sitting in a sauna at the gym. *sigh*  So I called Ben who was in Pittsburgh for the week, he calls Mike, the most awesome friend/HVAC person anyone could ever ask for, and we hit the road. Where, about ten minutes into the trip, the CAR air con goes out. And by “goes out” I don’t just mean “lack of cold air”. I mean the sensor goes wacky and it thinks it has to HEAT THE CAR UP to 74 degrees or whatever is on the dial. EVEN IF I TURN THE WHOLE THING OFF. It seriously needs an old priest and a young priest and a silver bottle of holy water. Imagine driving in the hot sticky weather and holding a blow dryer against you the whole time. That’s kind of what the three hour trip up was like. Luckily the hot air only blows in the front, so Kendall was warm and flushed and starting to show signs of wear and tear (breathing hard) by the time we got up here, but she was mostly ok. (and for the record, the air con thing comes and goes in the car. It could be fine for two weeks, and then not work for one, but then randomly come back on – seriously possessed.)

My original plan had been to have the girls just come with me since it was going to be a quick line study and then just one appointment, but we stopped at my aunt’s house so Karissa could pee and we could get cooled off a little bit. She offered to call my cousins and have the kids come over to play, which just lit up the girls’ faces, so thankfully that worked out. I am sure they had FAR more fun there than they would have if they had been with me.

The line study actually went well, they saw no obvious holes or reasons why her arm would go stay-puft marshmallow man on us. (which, of course, I rolled my eyes at. Pushing a few cc’s of contrast into her arm slowly is FAR different than running her TPN, which chugs along at 3 ounces an hour. Plus its very irritating to the tissue vs the contrasty saline. Basically, it was like comparing apples to steaks. I was glad they saw nothing obvious, but none of the rest of her team nor myself felt like it was compelling enough to say for sure what was going on.) The fact of the matter is that this line is 15 weeks old, which, in itself is a small miracle. We have successfully treated through two infections in it, TPA’d (clot busted) it once, had it re-sewn into place, had it checked for placement twice, and have really never been able to use one of the lumens (it has two). It just seems like at some point, enough has got to be enough. It’s a lot to ask of that vein to hold up through the near constant coursing through of her TPN, antibiotics, extra fluids…And most picc’s aren’t designed to withstand that kind of long term use. (yes some do, and i know some last super long with zero problems – but that is obviously not what we were dealing with here.)  If someone had been able to tell me unequivocally that turning on her TPN would be 100% ok and we wouldn’t just dump 40 ounces of tissue-burning fluid into her arm muscle, I’d have been fine with the whole thing. But no one could say that.

Our appointment with Genetics went about like I expected. Kendall’s latest round of testing came back “abnormal but not diagnostic”, which isn’t surprising since the stuff they were testing for is all completely random and doesn’t cover everything Kendall deals with. Her second muscle biopsy did come back confirming “mitochondrial myopathy, not genetically diagnostic” meaning that while something is effed up in her mitochondria, we still don’t know where or HOW they are messed up. Considering they have less than 15% (I think – I get the stats confused sometimes) of the mitochondrial genes mapped/known/discovered, this isn’t a huge surprise to anyone. They actually do have a couple more directions to go based on how this testing came back, and both of them actually KIND OF make sense – except that both of them combined still don’t really explain ALL of Kendall, and both of them combined also fall under “crap that happens to you when your mitochondria don’t work” (dysautonomia of non-Jewish origin, and ataxic migraine syndrome something or other).  We aren’t moving forward with testing for either of those right at this moment mostly because the tests are still VERY new for them, and because neither of them would guarantee that any other treatment would help. So for now we just stick with “mito of uncategorized origin” as her “diagnosis”. Could have told you all of this WITHOUT having to sit down with the geneticist for two hours.

About the only thing worth mentioning that came out of this appointment was that the doctor was NOT happy with the 101.8 fever Kendall had while we were in there, nor the fact that she had been off TPN/fluids for over 24 hours at that point and wasn’t tolerating the J-feeds of sugary pedialyte we were replacing with. So he and our complex care nurse (who is at all of our appointments) went out to “conference” about the plan and came back in to tell me we had to head over to the ER for labs and cultures “just to be safe”. And that if the labs came back ok “we might be able to head home later that nite”. Which, as I came to later find out, was complete BS. They actually had called the ER and instructed them to admit us. But i think they knew if they said that I would have fought them tooth and nail, and possibly ended up not taking her. Which is probably only half true. I wasn’t confident that we were dealing with just a benign fever from being hot and cranky, and with all the line problems, I figured it was probably worth it to at least draw labs/cultures. So off to the ER we trucked, me still blithely thinking we’d be out of there in time to get a late dinner and be home in our own beds that nite.

After a couple hours in the ER when I asked the doc if they had labs back yet so we could start discharge, she actually laughed at me and said, they didn’t tell you they admitted you guys??? we’re working on a bed right now! Oh i was so ticked. But I did realize that it was probably the best decision, as by then Kendall was looking even punier, and within about fifteen minutes of that conversation, we got to see her metabolic crappiness at its finest. She had been getting a normal saline bolus because its the easiest to shovel in fast AND add antibiotics to. As her labs came back showing she was pretty hypoglycemic, they came in to hang D5, did another dexy (blood sugar check) and she was trending lower, so they ordered D10, and then she started kind of zoning out on us so they had to hand push dextrose boluses and call for the D10 STAT. Fun times. She remained mostly stable that whole time, and i think my own blood sugar was probably bottoming out by then (I’d only had a few bites of pasta salad and a coffee all day and this was about 9 pm) but either way – I never got freaked out. They got us up to our room shortly after all of this happened, and we once again had our awesome “room-coming” to our home away from home, complete with pretty pink blankets for Kendall, extra toothpaste for me, a bag full of little goodies for Kendall- just the kind of warm touches that our nurses always give us! Since I was not prepared in the LEAST to stay the nite (I had, as a superstitious precaution, put a tank top and a pair of underwear in a bag that i had left in the car, which was locked up into valet for the nite!) – I FINALLYYYYYY got a pair of hospital scrubs!!! And a cute little bathrobe that adds that “did she just walk out of the mental hospital” flair to any outfit. i love it. I want to wear it all the time. It goes well with my shoes that had broken completely the nite before in the ER and that I had used a whole roll of medical tape to stick back together, and was wearing my taped up shoes with the grippy socks you get from the hospital…so hot. And dangerous. I definitely looked and felt like P.Diddy.

ANYWHOO. We settled in for the nite, and I knew that if I got shaken awake in a few hours, we were in trouble, but that if we made it to the morning, I was probably right in saying she was just super dehydrated. We made it to morning. She actually behaved all nite, no crazy stunts, and my awesome dad was able to leave work after a meeting to come up here to relieve me so I could go gather up all my babies and get us all back in the same state (well, except for the sick one…)  I got the big girls from my cousin, grabbed some food for myself finally, drove home as fast I could, stopping at the mall to get the director’s present for the big dance banquet that nite (I was in charge of the gift for Karissa’s team and of course what I needed to get was only at ONE STORE, but luckily it was on our way home). I just LOVE feeling like my life is an episode of “amazing race” and I’m constantly running (driving) from one location to the next trying to beat the clock. So we made it to my friends house to pick up Kaylen who clung to me like a baby monkey and just started sobbing. My kids break my heart sometimes. I can’t imagine how she must have felt, and and I know my friend did a GREAT job being a mommy to her and shielding her from the fact that Kendall was in the hospital again and instead turning the nite into a “fun sleepover” for her friends birthday, but oh my babies…all of them. It is so heartwrenching feeling torn between caring for the one who cannot be her own voice, laying pale and puny in the hospital crib, and feeling like my others babies must feel like I have abandoned them, favoring the one over them. And Ben in a whole other state…stab me. I just hate it. It never gets easier.

But – its been a good long run this summer of being together. Very few appointments for Kendall, and therapy mostly crammed into one day. And even now, as we still have clear cultures, and she isn’t “sick” – we’re still doing good. Waiting on a decision to be made yesterday with regard to her line status was definitely an exercise in FUBAR – I think at one point I counted five different plans that were on the table, changed, put back on the table, changed….In the end, we decided that doing an aggressive (understatement of the year) wean off of TPN right now (as in no more TPN, no more lines for hydration), while that would be everyone’s wish and hope, was not feasible. Since she is currently inpatient due to “dehydration with metabolic decompensation”, and that was just 24 hours off of TPN, logic would dictate that we probably wouldn’t get very far in a “TPN weaning trial”. Probably like the IL border. As in an hour away! So then the thought was back and forth between another PICC which would have to be threaded in through her right side (harder to do but not impossible) in order to give the vessels on this side a break, and because we aren’t positive that this vein is holding up real well anyways. In the end, I think one of her docs flipped a coin and it landed on the “broviac” side. Surgery then got involved and the discussions involved placing a line in a kid who STILL had a fever and was on three meds to treat a possible line infection (even though no one really thought she was infected). So we compromised, stopped the antibiotics, and put her on the OR schedule for Saturday morning.

which will mean a LONG day today of just sitting here, but we are doing good things still. I just had a good long talk with her dietician about goals and starting to wean her off of the TPN support, and am excited that we are FINALLY starting to make forward progress towards pushing her gut farther and harder. We are getting an actual fluid protocol put into place for the days when she just needs that little extra boost (to hopefully AVOID situations like this!!!) We are restarting some meds that have been stopped for various reasons, and addressing with an AWESOME attending (main) doctor some of the frustrations we have had regarding the continuum of care from the upper levels here. The big girls are with their wonderful Memaw and Poppa and are, I believe, on their way to a jellybean factory right now (my dad is trying to time the height of the sugar rush for the precise moment Ben should be getting them). Ben is on a flight home right now, and in a few hours we should all be together for a little bit. I think our air conditioner parts have been ordered so our house will soon be cooled off from its current high of 90 back to a more reasonable/breathable temp of ….well – whatever it is – it will be better than what it is right now!

And then HOPEFULLY if little missy behaves, we’ll be home together as a family tomorrow nite, in our cooled off house!

(but i’ll still probably be wearing my hospital bathrobe).

 

thanks for checking on us.

 

terra

(or you can address me by my new hip-hop name, T-cup).

Summertime.

Dear Grandma Marge –

this one’s for you. You are at the top of a very short list of people who can drop hints that its been a long time since I’ve blogged that will actually spur me to write instead of just clamming up further. I love you – thank you for everything~ hope we get to see you soon!

I’ve titled this one summertime because it just might be one of the few updates you get this season, since it’s already been almost a month since I updated! overall – things are going GREAT for miss KQ, and i cannot tell you how happy I am to report that. I am just a little superstitious about saying actual lengths of time since our last infection/illness/admission – but it has definitely been a good long run! We will take every minute we are given in our OWN beds! Thanking God for the mini hospital room that we have in Kendall’s bedroom, it has saved us a few ER trips at the very least, if not stupid admissions for “tune ups”. Our standards for what “worries” us have definitely dropped, as I think they naturally do when you have witnessed your child literally clinging to a very fine edge between “super sick” and “hanging in there”.

All of that to say that while things are not “normal” or completely healthy around here, they are definitely good. And we will take good.IMG_1832

Kendall has definitely struggled with the heat and humidity a lot more this year than in years past. I don’t know if that is because it is truly stressing her system out more, or if its because she is actually able to independently WANT to go outside and play this year (vs last year when she slept most of the day still, and didn’t really understand what her sisters were doing when they would go outside). I am sure it is a combo of both. Either way, her nurse and I play a good game of crapshooting every day with whether we let her be a kid and go play outside for a few minutes, or do we play “ER nurses” when she comes in and we are hooking her up to more fluids, taking temps and BP’s, packing her in ice packs, putting her on oxygen, etc to try to get her brain reset so she stops literally burning up from the inside out. We know kendall suffers from “dysautonomia”, or a failure of the body’s autonomic system to regulate itself. Things that you take for granted – like sweating when its hot, shivering when its cold, taking the perfect amount of breaths per minute, having your digestive system work in forward progress, feeling pain when something hurts and comfort when things are just right – these are all thanks to your autonomic system. For whatever reason, Kendall’s system likes to either not send the message or it sends the wrong message to the wrong system. One of the biggest issues we are having from that right now is with temperature regulation. Outside in the muggy heat, her body doesn’t break into its stores to help hydrate her, and it doesn’t tell her body to sweat. Imagine parking your car in the middle of a blacktop parking lot on a 105 degree day, and not rolling down the windows. Slowly the temperature inside that car will just rise and rise and rise, because it has no way of cooling itself off. That is Kendall’s body. We are talking within literal minutes (maybe 10), Kendall is dealing with some pretty chaotic internal vital signs. RARELY will she self-regulate during these times. Meaning, she will not come sit down or ask to go inside. Because at the heart of who she is – that fighting spirit that has saved her time and time again – she FIGHTS to be a regular kid. She does not want to stop playing.

So we drag her in kicking and screaming and begin the process of cooling her down. I am pretty sure that if she knew how she would be swearing at us too. and yes it does take her nurse AND myself MayJune 2011 084 working for a good hour to get her back to an acceptable baseline. Once she is inside and in her bed hooked up to everything, she usually will pass out, and depending on how hot she got, she will sleep for anywhere from 3-5 hours, and when she wakes up she’ll want to do it all over again! By that time of day though its usually a little bit cooler, and she might get as many as 20 minutes outside. And by playing outside, I don’t mean she’s running around and climbing all over things. Usually playing outside entails sitting in the little barbie jeep being “driven” around by Miss Daisy Kaylen who likes to do donuts and rooster tails and push the limits of what Power Wheels ever intended for “ages 4-7” to be able to accomplish in their plastic cars. Maybe Kendall’s issue isn’t the heat as much as its her adrenal glands from the adrenaline rush of driving with her sister….(I’m only kind of kidding!) Anyways the point being – she isn’t being that active. It is heartbreaking to watch – and yet inspiring at the same time. I want her to keep fighting. I want her to NEVER think there is anything different about having to lug around a backpack and click-click-click with every step cause of her AFO’s. I want her to be like any other kid! And she will be. She is. She just has a few extra attachments.

Anyways – so besides that fun battle on a daily basis, things are movin’ right along for her! And for the rest of us! We finally were able to get new library cards after waiting the prerequisite six years in order for the massive library fine we had from an unreturned CD case to be cleared off the records. This has just amazed the girls – we get to go to this huge fun building where they have computers and movies and even Wii games that you can checkout FOR FREE!!!! And then you can bring them back and get MORE!!! (I have obviously failed as a mother. They really had no idea how the library worked. Admittedly – its a bit hard to get into and out of ours prior to this new construction, especially with a stroller – which we have not been without for at least 4 years running!) Our days aren’t filled with tons of crazy running around, but I am slowly starting to build up stamina to at least get the oldest three out of the house for fun quick little things. It is so hard to leave Kendall crying at the front door that she wants to “go-mommy” (go with us), but I know that its so tough on her little body to be schlepped in and out of the car to run errands when its hot and muggy outside like this. It’s easier to hunker down in our cooled off little house and play games or read all our fun new library books.  We have our little metal frame pool (about ten feet big!) set up, and occasionally the girls will spend the afternoon splashing around in there, or go down to a friends house with a bigger pool, but that is really the gist of our summer vacation.

We do not take big trips – we can’t afford them nor can we plan on taking Kendall anywhere. The few road trips we have taken with her end up wearing us out from the packing process let alone trying to convert a hotel room into a April 2011 060 hospital room! And the thought of going places without her is very hard to think of. It is hard to hear about/talk to friends who are planning fun vacations for their families. It is hard to not get just the tiniest bit jealous when I hear those things. But i realize that that is not our life right now. We are blessed in ways far beyond family trips or uber exciting summer vacations. We have each other, all here, all mostly healthy. Our needs are met. We are surrounded by an amazingly supportive community – made up of an odd mixture of close family, good friends, and complete strangers. It is those complete strangers I think that sometimes get me the most, make me cry good happy tears, remind me why my family is so much more important than a trip. So if you are a complete stranger who somehow reads this blog – thank you. Thank you to everyone who came out to the Culvers fundraiser a few weeks ago – and to the baseball teams who decided to raise the bar even higher! words will never be able to express my gratitude for all of you who find time and love in your hearts for Kendall, or for our family.

Last week was not a good week for me emotionally. The week before that (and really the month before that) weren’t too great either. But this week is looking up. my birthday was yesterday. I was surrounded by wonderful family and friends that have become like family. I got a whole Portillo’s chocolate cake for my birthday cake. I have a fridge full of Coke. My brothers almost blew themselves up making homemade fireworks and my sister in laws had us all laughing while they tried to make adult beverages measuring alcohol out with 60cc medical syringes (all i had to measure ounces!). We laughed until we cried and then we laughed some more.  It was a good birthday. So yeah, looking up definitely. I am not quite ready to turn my blog into a morose pity party – which is why sometimes weeks go by with no post. Not that I want to only blog about sunshine and roses – but because I know myself. If i get started just whining about things I WISH were different, I will start living that way. And that’s no way to live – like a whiney spoiled brat when things don’t go MY way. I know far too many people who have chosen that as their preferred method of operating and it is definitely no way to live!

So instead I’ll just continue hunkering down here in the house, reading books and biding my time till the tide turns and my attitude improves and I have something good and nice to say. And now that it has taken about 8 hours to get this much out – I have lost my train of thought completely! But tomorrow’s another day – I’ll try to start earlier and end earlier so you get just one cohesive blog! Well, actually, I hope there’s nothing to update! I like nice boring weeks with no emergencies! Anyways – i’ll try to update more frequently. We have a lot of stuff coming up in July – I’m sure i’ll have lots of fun pics and whatnot. Like i SHOULD have pictures from the parade we were in yesterday in Wheaton for the UMDF float – but right now I don’t. Hopefully soon!

Ok now i am really going to bed.

Peace out. See you on the flip side.

 

Terra

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