Take a look at our sometimes crazy but always blessed life as we homeschool our eight children, run a homeschool business, and serve God as He leads us. You can also follow our baby Noah as he struggles with some serious health issues - we covet your prayers for him.
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Our Quiverfull
Jan. 15, 2008
Balancing the budget
No, this isn't about money. :-) Quite a few of you have asked what Noah's diagnosis means for him. In case you missed it, Noah has officially been diagnosed with mitochondrial disease. Specifically he has a mutation in his mitochondrial DNA and his disease (at least for now) is referred to as mitochondrial encephalomyopathy. It's possible that that will get more specific later. If you are interested in reading more about mito, this site has lots of info (links on left) http://www.umdf.org/mito_info/whatismito.aspx .
The non-doctor version :-) is that mitochondria are found in every cell of the body except for red blood cells. They are responsible for creating energy. Because Noah's mitochondria are defective, his cell's can't produce the energy they need. Body systems and organs are no more capable of functioning without energy than your car is able to function without gas. Different body systems are affected in different ways, and to different extents. Generally as the disease progresses more and more body systems are affected. Noah's heart, autonomic nervous system, and GI system have been hit the hardest at this point.
Mitochondrial diseases are progressive (they get worse) and can't be cured. When you have a genetic mutation, the cells replicate with the wrong "blueprint" each time new cells are made. Certain nutritional supplements (the "mito cocktail") may or may not slow the progression. Certain mitochondrial diseases fit into named syndromes like MELAS or Leigh's. Children with named syndromes have a general prognosis in terms of progression, systems affected, and lifespan. Noah's disease does not fall into a named syndrome, so we have absolutely no prognosis. No one can tell us what to expect. We only know general things - for example, children who show symptoms in infancy like Noah did often don't live until age 3. If they do make it to age 3, sometimes they start to do better (progression may slow). Once body systems are affected, they do not generally get any better at all, but they may stop getting worse.
Noah has two primary challenges right now. The first challenge is presented by his nutritional needs. Because he is dependent on TPN for all of his nutritional needs, he requires a central line (his broviac). The broviac presents a constant risk of infection which can (and has) led to sepsis. The TPN itself will eventually destroy Noah's liver. Everyone is amazed that he is doing so well so far and we pray that this continues for a long time. The TPN/central line combo presents a literal "can't live with it, can't live without it" dilemma. TPN dependency is not compatible with long-term survival. The TPN and broviac present the most pressing threat to Noah's life by far. None of his other issues are life-threatening at this point.
The second challenge is knowing how to balance Noah's energy budget. Noah has much less cellular energy than other people do. I'm not referring to "I have NO energy - I need a nap" or "I had SO MUCH energy yesterday - I mopped all of my floors and washed my windows." This is how most of us think of energy, but when we talk about true energy we are talking about the energy needed for everything in our bodies. We all "spend" our energy budget on breathing, keeping our heart beating, digesting food, filtering waste, maintaining our temperature, fighting infection, moving, learning, growing, keeping our cells alive - absolutely everything our body does.
Most of us have a big enough budget that we can spend what we need in every category. You have probably noticed times in your life when your budget wasn't balanced - when too much energy was allocated to dealing with the physical effects of stress or illness and you were left exhausted, or when you expended so much energy working yourself into the ground that you shortchanged the immune system category of your budget and got sick.
Noah's life is like that every day but to an extreme level. He will be able to balance his budget. He will always come up short. So far in his life he has come up very short. Any expenditure in one category results in obvious, extreme shortfalls in other categories. Any stress - sickness, extremes of temperature, big spurts of motor activity, too much excitement, lack of sleep. etc. throws him for a loop. For example, he has always looked his very best right before getting very sick. He used so much energy being busy and active that he couldn't pay off his immune system account. When he gets sick, he regresses, often dramatically, in other areas. He has lost motor skills and once completely stopped almost all babbling for months. So far, he has always regained what he has lost, but it takes varying amounts of time to do so and there are no guarantees that he will always regain what he loses. If he goes out in hot weather he completely wilts and can turn totally limp pretty quickly. If he is out in chilly weather he becomes hypothermic and we have a hard time warming him up. Today he was showing off and working hard for his therapists and one of them pointed out subtle neurological signs that he was overdoing it. His balance suffered, his countenance changed (got "spacey" looking), his coordination got worse, his play skills weren't as good, etc. We see these trade-offs all of the time with him.
This brings us to the challenge of trying to help Noah balance his budget better. It is better that each budget category get something, even if it isn't enough, rather than have one or more categories run in a serious deficit. We try to protect him from physical stress. We have to be hyper-vigilant about germs because stresses to his immune system are a big problem for him. We have to make sure he gets enough rest. He used to sleep so much that this wasn't a problem, but as he gets busier and more engaged with life, the less he wants to sleep. Slowing him down is the hardest thing right now. He is taking a lot of steps forward with his motor skills and is very inclined to overdo things. Obviously he has zero judgment skills and isn't capable of knowing that it is time for a break, :-) so we have to do that for him. One of his home health nurses said that he should have a maximum of two hours of activity of any kind before taking 30 minutes of rest. After seeing him today his therapists suggested a maximum of 1 1/2 hours of activity OR resting as soon as Noah starts showing those physical, neurological signs of crashing - whichever comes first. He can't really chill out on the couch with a good book at this point, so we are finding ourselves in the unique parenting position of taking a happily playing child and restraining him in his high chair with a video (mostly Baby Einstein). It's an odd thing for folks like us who tend to avoid much video or computer time for children, ESPECIALLY for little critters like Noah, but other than naps there aren't many ways to force a break. When our lovely balmy SC spring hits I want to see if a stroll around the neighborhood will be restful for him. I don't know if he would relax or if it would be exciting. Sometimes he is willing to hang out in his high chair in the kitchen and just watch me cooking or cleaning.
We are looking into getting Noah a high-quality stroller. We looked at some on Amazon with his therapists today and they suggested an all-terrain jogging type of stroller. It looks like Noah WILL eventually walk, which is GREAT, but walking is to energy what our 15 passenger van is to gas LOL. Most children with mito who can walk end up in strollers/push chairs/wheelchairs for at least part of the day so they don't overspend. Lots of them have arrangements where they are free to walk at home but are in a chair when they are at school or out doing errands or whatever. Since he can't walk yet, this is a good time to get him accustomed to a new stroller and make it part of his routine. Again, restraining a toddler that WANTS to walk seems to go against the grain - but we have to do everything differently with Enigma Boy. :-) From a practical sense, he is getting heavy (23.5 pounds!!) and very difficult to carry for any length of time - especially for me with my belly! Add a full, heavy bag of TPN and a TPN pump and he becomes a real heavyweight. It makes me a little sad, but we are approaching the point where he needs to be in a stroller even at places like church where we would usually carry or hold him. An all-terrain stroller looks relatively normal, would have storage for his pump and any bags of supplies and meds, and would be easy to use anywhere we go. You can pray for us as we research and try to find the best stroller for him. If you have a jogging-type stroller that you love that that has a swivel front wheel, plenty of storage, and plenty of padding (think napping), I would love a link to see it online!
This is a lot of information, and if you are still reading, I applaud and appreciate you! I know it seems like a lot of negative news without much good news. The good news is this: that while Jeff and I have both feet planted firmly on the ground and know that our little boy has been diagnosed with (I'll say it officially for the first time here) a terminal disease, we also KNOW that he and all of us are firmly in the grasp of a loving God. We have Noah right now. We don't know if we will have him for weeks or months or many years, but we do know that we have him right this very minute. God has used Noah to teach us that every minute of every day is a gift of incredible beauty and sacredness. God has used Noah to bring me to the place where things like going to the grocery store, sitting down to dinner with my family, reading to or playing with my children, and snuggling with my husband make me feel like the richest woman in the world. God has given us a wonderful, terrible gift in this journey and we are learning that there can be beauty and light in the darkest places. We wouldn't choose this journey, but we have learned to be thankful for it all the same. We love and appreciate all of you, and we pray that you would all know the same peace that comes from embracing the journey you are on as coming from the hand of God.
I can't possibly say it any better than this song. I'm going to cut and paste the words before the video clip because the words are so incredibly perfect that I don't want you to miss any of them. The video clip follows the lyrics. The video sound quality isn't great, so I encourage you to play the video while reading along.
Love,
Kate
PS - One quick clarification on this song. There is ONE thing that we do (or SHOULD) know about the future, and that is our eternal destination. This song isn't about living in the moment because our earthly life is all we have. It's about being secure in the here and now because we know that God holds our earthly tomorrows and offers us the gift of an eternity with Him. It is both the earthly and eternal security that bring us so much joy and so much peace.
It's time for letting go
All of our "if onlies"
Cause we don't have a time machine
And even if we did
Would we really want to use it
Would we really want to go change everything
Cause we are who and where and what we are for now
And this is the only moment we can do anything about
So breathe it in and breathe it out
And listen to your heartbeat
There's a wonder in the here and now
It's right there in front of you
And I don't want you to miss the miracle of the moment
There's only One who knows
What's really out there waiting
And all the moments yet to be
And all we need to know
Is He's out there waiting
To Him the future's history
And He has given us a treasure called right now
And this is the only moment we can do anything about
So breathe it in and breathe it out
And listen to your heartbeat
There's a wonder in the here and now
It's right there in front of you
And I don't want you to miss the miracle of the moment
And if it brings you tears
Then taste them as they fall
Let them soften your heart
And if it brings you laughter
Then throw your head back
And let it go
Let it go, yeah
You gotta let it go
And listen to your heartbeat
And breathe it in and breathe it out
And listen to your heartbeat
There's a wonder in the here and now
It's right there in front of you
And I don't want you to miss the miracle of the moment
And listen to your heartbeat
There's a wonder in the here and now
It's right there in front of you
And I don't want you to miss the miracle of the moment
that song fits perfectly!
The Lord blesses us with gifts - our babies. They are not perfect - but we are not either! They are "given"to us on loan. The Noah's and Maddie's in this world are put here for a reason - to bring others to Christ! Among other reasons!! Cuddles, hugs, kisses, loves, smiles, etc!!!
Thanks for the update.
Hugs and Blessings from the Kiessling family from Fresno, California
Thanks so much for the thorough update. It helps me know how to pray for you all and for Noah. Though I don't always post (especially when many others are posting too), please know I read every update and pray regularly for you.
What God has and is doing in your heart is incredibly beautiful! I read all of your postings and I am challenged by how grounded you are in the sovereignty of the Lord. In my prayers, a homeschool mom
Dear Kate,
We have been praying for Noah and your family each night. I know how you feel about stating the term ( terminal illness) we like you are walking through the same with our daughter, she was diagnosed at age 22 with HIV, she has been really sick and she was pregnant with our first grandchild...Isaac. Isaac is testing negative but we dont know how long our beautiful daughter will be with us. Treasure each day as a gift...I know God is able to heal Noah and all we can do is pray and trust!!! God is faithful, even when we dont understand, I can tell by your posts you have a loving family who sticks together like ours does, we have too. We have 7 children and the Lord has brought us through some awesome things. Give Noah a hug for us, my 3 little guys get together and pray for him every evening, they are 9,11 & 12. God hears our prayers!!!
thank you for the wonderful song and the update Having a child with any illness is heart-tugging but when you have one that is severely compromised on all fronts it makes you appreciate the little things so much more. I have a severely medically and neurologicaly impaired son who has major set backs he to cant 'spend ' to much energy without paying the price elsewhere, we use a Safari transport chair, it tilts so we can lay it flat,you can get all kinds of attachments (iv poles,backpack for storage) they are pricey but insurance will cover if doc writes a script. praying for you and your family. Hug Noah and the other kids tightly. www.caringbridge.org/visit/curtisshubert
((hugs))
thanks for helping us understand. I know it's hard when folks don't "get it". Thank you for facting "facts" and trusting the Father.
We have our first speciality appts in VA tomorrow. Hope they are good! GI in Roanoke and then early intervention here.
Seems like we are kinda on the same wave-link lately.... I went to youtube for the first time today, and typed in my favorite artist...and listened to most of this video!
Monique
I don't know if this has crossed your minds or you already know the answer. Is baby Mary Faith's stem cells from her umbilical line an option for mito defects? Obviously if it's a match. Would it help even a little bit? Just a curious thought. I'm not up too much on all this stuff. If you are like me, you are learning way more medical then you ever wanted to know. I know I did each time my kids went through NICU.
Here is the single version of the double stroller we have enjoyed tremendously for two years. None of the joggers are particularly padded, though this one does have a padded insert - for more padding I would just throw in a favorite blanket before placing Noah in. There is plenty of storage and a free-spinning front wheel - it is the easiest thing in the world to maneuver and all my kids (6,4, twin 2's) fight over getting to cruise in it. It is also easy for younger ones to help push, my 6 and 4 year old steer our double without any trouble through Costco and grocery stores. Here is a link to the stroller, you can check on google to try to find the best price, this just has some reviews and good photos. God bless, we pray for you often. http://www.amazon.com/Schwinn-Safari-TT-Jogging-Stroller/dp/B0007MVBXQ
Oh, Kate! I listened to this song Sunday afternoon & thought of YOUR FAMILY & how well you're learning to do this! You haven't been hanging out with Steven Curtis Chapman, have you?
So many situations, big & small, make us not want to go down the paths that we're clearly on ... be it watching the kids grow up, or <quivvering lips & tear-ing eyes> not getting to watch. Once again we thank God that we do walk those paths alone -- hallellujah.
Just wanted to let you know that i understand more than most about People with Mito diseases. My Husband has Mitochrondrial myopothy. Since it is in his muscles and was not as much as it could have been. It didn't show up until he was around 23 or so. I would say that we are learning more and more all the time about how amazing the GRACE of God is and how GOOD HE is. My husband needs his relationship with Jesus. His disease helps him to remember that he is in desperate need of a Savior. In many respects this disease has made him and all of us better. The amazing thing is the understanding that each day is a gift from God to be lived whole heartedly. I also want to let you know that I pray for you all each day. It is hard to watch someone you love have struggles, yet it sure makes me spend lots of time in the presence of God and praising Jesus for the gift of eternity. and right now as well. Thank you for your transparancy, and pointing us to our Lord and Savior. Linda
So much to digest...
Feeling relief (if that's even the right word) that there is finally a diagnois, that the unknown has been brought to light.
Hurting with you in the official, terrible word, "terminal".
Rejoicing with you in the moments you cherish.
Praying with you for daily wisdom. While not of anything close to the same magnitude, I've lived with the "energy bank" concept for the past 17 years of chronic illness and feel for you in trying to manage this in a toddler!
My heart is so full for you all tonight. I could go on rambling, but instead turn once again to prayer for all of you.
{{{hugs}}}
Jenni in Reno
Hi Kate,
What a beautiful post and absolutely wonderful song. It made me cry. The way you explained how you deal with your "just now" moments was just great. The overall message about needing to appreciate our moments right now was truly a God-send to me. I needed to hear it "right now". I appreciate the full detail on Noah's illness. I know it probably brings a lump to your throat to say that word "terminal". You are very special to be able to appreciate your days and not be consumed with the anger that comes with the "why?". I know some days are more challenging than others but it sounds like you have more appreciative days than the suffering kind. That is a testament to your faith and your ability to bring joy into your life, even at the toughest times. I really appreciate you sharing your life with us here on the blog. I look forward to your next post each and every time. I pray for you and your family. You are a wonderful example of how we should all live our lives. . .live for the moment and appreciate what we have RIGHT NOW. Thanks for the message. :-)
I don't even know what to say. Part of me thinks I should be glad you finally know at least what to call Noah's disease, but the greater part just wants you to know that my heart is breaking for you. I have a 2yo son and I look at Noah and just imagine what it must be like for you--
I'm so thankful you and Jeff have the Lord as your Rock and salvation. I guess those of us not in your shoes can't grasp what He gives in the way of comfort in the midst of such a struggle. I've known His peace during lesser trials though, and it is a comfort to know that we can all call on Him to be with you all.
The song you chose is perfect and I've shared this post with others, hoping it will be a light of salvation and a blessing to witness your undying devotion to the Lord of Lords in the midst of what has to be one of the hardest things a parent can live through. Kate, we don't even know each other and so it's hard to communicate how grieved I am for you all. I love your family in that way that Christ enables us when we're dealing with virtual strangers across miles and miles. I've known others who have had to give children back to the Lord before they would have chosen to, and I'm just certain that our faithful God is using this to bring glory to His name. I'm sure you all want that and He will bless you for it. You are in my prayers and this post is just incredible. Thank you for sharing so perfectly. I can't stop crying--we serve an amazing God and I keep seeing Him in your lives through this. I wish it weren't this way though--I don't want it to be true.
Very well explained Kate. It's a good review even for us regular blog readers. My heart breaks for Noah and for you, but I am grateful for the opportunity to pray with more understanding, and for the lesson you've shared about capturing each moment. That is such a beautiful song at the end of your post! Praying for wisdom as you balance Noah's energy budget.
Kate:
Your words are perfect for a person from the outside looking in!!!
I do understand how it is with a compromised child & how worn they get from the ennergy the spend with out juding first.
As you know with Blake, he has been sick most of his life(well, ALL of it). But God has given us the strength to "find the answer" He helped us to endure through the unknown. He led us to what "can treat but not heal".
When we received the DX of Severe Combined Immunodeficiency & had to endure TPN feeding tubes & central lines, IVIG on a bi weekly basis(now weekly through sub-q), then to hear about his RARE Blood Clotting Disorders,and to have to deal with Autism it was a long, long ride & still is. God has been my strength & given me the pateince to "deal".
As you have said, "We do not know what tomorrow holds, but We DO know who holds tomorrow"!!
Thank you for your strength, sharing & honesty. Thank you for bringing each of your children to the Throne of God before all of your friends. God has brought you to this, He WILL bring you through it!!!
Just keep up the good work & know that you have friends far & near that are constantly praying for you, your precious family, baby Noah & the soon appearance of Baby Mary Faith!!!!
Julie
http://www3.caringbridge.org/sc/blakester
The Greatest Adventure of MY Lifetime!!!!
Here's a link to the double stroller I've use for the past couple years. It's holding up really well. We take it everywhere..walking on roads, shopping in Walmart, etc. Front swivel wheel too....ours is a double, which actually might be pretty handy if you needed the storage space to set things next to Noah. It's easy to load in the back of the van..and I can unload it easily with one hand, and a toddler on my other hip. Just an idea. :)
http://www.instep.net/index.php?cat=SW&fuseaction=
products.details&id=38&encodeKeyURL=%2Fswivelwheel
_stroller.html|
(there's a couple spaces in that link, so it wouldn't post funny halfway across the page)
As always..we're praying for Noah. May the Lord give him strength and ease his pains so he can enjoy life. (((hugs)))
~andrea, mom to five boys, 1 lil girl in MN
I know how you feel about -- forced television and down time. Our situation was never as dire as Noah's; but, when my youngest was an infant (2002) he had some issue that they didn't know if it was seizures or a brain tumor -- at 6 months they started testing, just after 1 they diagnosed him with severe migraines -- it seemed anything could trigger them: weather, food, household cleaners, noise, of course learning to walk and crawl led to bumps, distruption in sleep or routine -- you name it! He got all the symptoms: partial paralysis on his left side, nausea, vertigo, aurora -- maybe others that we couldn't identify. Belive it or not what helped was watching Barney (the old ones -- not the new flashy ones but the ones that came out about 1980 -- 1998) it forced him into a type of what they called "biofeedback" at the time. We did exactly what you did -- we could see the signs but how do you tell a 1 yr old to stop exploring and lay still on the couch or his bed? The tv! It mesmerizes them! He is now 5 -- tv is far more limited becuase now he can tell us what is happening and he understands when we say you can't eat this, or you need to lay down, or stay in your room while we vacuum, clean, etc.
We check on you and Noah everyday -- and we are praying for strength and grace for all of you!
This was very clear and thorough information. It will help me know how to pray...although I've been trusting the Holy Spirit to intervene in my frailty! Praying as always...
Blessings,
Laurie
Dearest Kate, thank you so much for sharing your life with the world. You are truly an inspiration because of your faith in the Lord and heart for your husband and children. I cannot imagine all that your going through. I do feel so honored to "share in your struggles" before the Lord. My husband also named Jeff and I are praying for your whole family. Cherish these moments as I know you are. In Christ, Lauri Lewis Toledo, OH.
Kate,
Thank you for sharing all of the details on Noah's condition. Thank you for being real and for sharing your heart here. I believe Noah's life (as well as you & your husband's) are just a great testimony of God's grace and mercy.
May the Lord bless you today with His peace and His strength and His grace to meet every moment with His love. You have written a beautiful post, and have done an incredible job of explaining what seems to be a very confusing, and difficult to describe condition with Noah. The Lord has gifted you with communication. You have glorified Him, and for that I am so very grateful. What an incredible ministry you have as you witness to God's Hand in Noah's life, and the life of your family.
I live in SC as well, and grew up in Greenville. I originaly found your blog because another blog linked to it for prayer. Although our family has been in big transition with a move since last June, I have come back here sporadically (as I have had the time) to read and pray for you. Thank you for sharing so that others can pray with and for you. What a blessing to be able to lift you up.
Finally, as a teen, I started to work with handicapped children in the summers. I remember that every time my parents came to the camp, they would sit there in tears (especialy my dad). They (with marginal faith) wondered at the Hand of God creating imperfect people, with a myriad of difficulties and challenges. I tried in vain for years to express the joy that my campers had, their zest for life, and their ability to concentrate on what is important- living life to its fullest. In this blog post you express so well all of that. Each day, each moment is to be treasured.
Thank you for reminding me of that this morning.
I am especially praying for you and your labor and delivery coming up. I love the name change for your baby girl (although I love"Abigail" too, I love the reasons you changed it).
We recently got a stroller for Cadence and Haven to share as it is difficult with Cadence's autism to let her roam free in public places and umbrella strollers are getting to be too small. It's not an all terrain, but works well given her size and level of activity. This particular one has the baby seat that goes with it. Given the situation we use the baby seat and stroller for Haven and if we need too we take the seat out and use the regular part of the stroller for Cadence and put Haven propped up in an umbrella stroller. I've included a link. This particular one is "girly", but I'm sure there is a more neutral color you could find and you could use it as a double for Mary Faith and Noah. She can use the car seat and he can use the stroller. http://www.gracobaby.com/catalog/product.aspx?modelNumber=7B13EMM3&subCatId=51
I got an email from www.joggingstroller.com this morning and they have a wizard that will help you find the stroller that fits your priorities. I thought it might be helpful.
Click on "Help Me Choose" under one of the products on the home page.
I read your blog from time to time, as I remember. I can't put HomeschoolBlogger in my feed reader for some reason, so I only catch up every once in a while.
I can't remember now if I posted this before:
http://www.especiallyheather.com/emma/
This lady has a child with Complex 1 mitochondrial myopathy who has been through a lot of what your Noah has been through, it seems. I thought you might be blessed to read about her little girl and how far she has made it.
I discovered your blog, quite by accident, as I was checking Leslie Brasher's blog. I saw the adorable picture of a little boy, your little boy, Noah, asking for prayers. I decided to check why prayers were needed for this lovely child, and after reading about Noah, I sat here with a heavy heart, crying. I know there is a reason for "bad" things in our life, our oldest son died 8 years ago of cancer. But the good is, that we are so lucky that God gave us this child in our life, if only for a short time. I will say prayers for Noah, and for your family, and the new baby on the way. Hang in there, God is watching over you, I know . Bonnie in WI
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