This post is going to be a little bit different, and our long-time blog readers may even want to skip it.  We often get emails from readers who are new to our blog, and who find that reading a new post is like starting a book in the middle.  Some enjoy going back and reading lots of old posts to catch up, but many others just don’t have time.  In this post, I am going to try to summarize Noah’s story and what has been going on in our lives.  I’ll be adding it to the links section on the right so that new visitors can get to this post easily whenever they find our post, and I will edit/update this post to reflect major changes as they occur.  We hope this will simplify things for our many new readers.

 

We are Kate and Jeff Estes, and we take turns writing this blog.  I (Kate) do most of the writing, but Jeff does the funniest writing!  We have seven beautiful children ranging in age from 14 down to Baby Noah who will be one year old on August 10.  We won’t tell you how old we are - LOL.  We have five boys and two girls and they are the joy of our lives.  Jeff has a full time job working as an Organization Development Manager of an iron foundry.  The foundry melts iron to make parts for cars and other machines, and Jeff is in charge of all of the training, teaching, and personal development of all of the employees.  He is an incredible teacher and very good at his job.  (Proud wife bragging about wonderfully gifted husband!)

 

Jeff and I also own a homeschool supply company called Hands and Hearts.  You can see our home on the web at www.handsandhearts.com .  We develop and create hands-on living history project kits, and we also sell a few hundred other very carefully chosen homeschool resources.  Our business also sponsors a Yahoo group and a newsletter.  Jeff and I both work hard with our business along with some great employees who do most of the day-to-day work.  We also have several homeschool graduates, students, and moms who work for us – they make a wonderful team and they are like family to us.

 

We started this blog as a general family/homeschooling blog, but within days of starting the blog little Noah became gravely ill and he has become the focus of the blog.  I hope that one day there will be more homeschool articles, recipes, and funny family stories than there are prayer requests, but right now we are overwhelmingly thankful that this blog has given us a way to update friends and family about Noah’s complex and ever-changing needs.

 

Noah came into this world with a bang on August 10, 2006.  When Noah was a few months old, he began a serious and rapid decline in his health.  Since March, 2007 he has been hospitalized 5 times and has had two surgeries.  He cannot and will not take anything by mouth except to nurse, but because he is very weak physically he is not capable of nursing enough even to maintain his hydration.  In the middle of May, 2007 he had surgery to insert a feeding tube (g-tube) so that he can receive nourishment directly into his stomach.  He tolerated the surgery well but does not tolerate feeding well.  Most children with g-tubes are fed in a way that mimics normal baby feedings.  They receive six 10 oz. “meals” of formula several times a day over a period of 10 minutes or so like a bottle-fed baby would.  This is called bolus feeding.  Noah is completely unable to tolerate bolus feeding.  He is attached to a feeding pump 24 hours a day and is fed a very special ($25 dollars a day worth of special!) formula continuously at a rate equal to 5 oz every 4 hours.  Even though our insurance isn’t paying for his formula and feeding tube expenses, we are blessed in that we are currently receiving some financial assistance for these items.  We don’t know how long the funding will last, so funding for the formula is an ongoing prayer request.  It is a challenge to manage a baby who is always attached to a pump!  The pump looks a lot like the sort of pump used for an I.V. and his milk is stored in a bag that looks like an I.V. bag.  The bag, pump and tubing fit into a special bag that we have to pick up every time we pick him up.  It is run by battery power when he is awake and plugged in when he sleeps.  We are blessed that he really doesn’t ever try to pull out his pump, but we have snagged in things several times and snapped the tubing.  That is always a messy disaster!! 

 

During the first 4-5 weeks on the pump, Noah was gaining weight beautifully with his feeding pump.  Before he got the pump he was truly emaciated and you could see most of the bones in his body.  It was heartbreaking.  He started to fill out immediately after his surgery and was gaining more than a pound a week!  In the past couple of weeks his weight gain has nearly stopped, so that is one of our primary concerns right now.  If Noah has a mitochondrial defect (more about that below) it could explain the weight gain issues.

 

Noah is by far the easiest and most pleasant baby we have ever had.  He brings tremendous joy to our family.  He loves everyone he meets and smiles all of the time.  He is very generous with his slobbery kisses.  Even though he has had dozens of  I.V. placements and over 100 blood draws, he is never resentful or fearful toward his doctors and nurses.  In fact, he has a reputation at three hospitals and several doctors’ offices as a world class flirt – and he is so good at it that everyone with whom he flirts is convinced that he or she is his personal favorite.  His affectionate personality is made more powerful by his huge blue eyes and fluffy blond hair with a big cowlick in front!

 

Noah has significant motor delays.  At almost 11 months he cannot roll over or creep.  He is just starting to briefly maintain a sitting position when placed in one.  He is a very sleepy baby and needs to nap for a few hours after being awake for about an hour, even though he may not do more than cuddle and nurse and chew a toy during his awake time.  He gets sick easily and when he gets sick he gets extremely sick.  He is allergic to five different families of antibiotics, so we have to work hard to keep him healthy.  When he nurses or sleeps he sweats so heavily that his sweat will drip off my arm or soak a folded towel.  If he sleeps on a dark sheet we can see where he leaves a raised ring of evaporated salt and other minerals from all of his perspiration.  He has some other G.I. issues that need further testing and he may need more surgery to correct some of them.

 

Extensive testing has ruled out a lot of potential diagnoses.   He doesn’t have cystic fibrosis.  A recent trip to MUSC Children’s Hospital in Charleston has ruled out any serious cardiac issues (he does have a small heart defect but it isn’t causing these symptoms).  He doesn’t have a chromosomal abnormality.  He is strongly suspected of having a mitochondrial disease and his geneticist is arranging for us to take him to Emory in Atlanta to be tested for mitochondrial defects.  The test requires yet another surgery under general anesthesia.  A large muscle biopsy will be taken from his thigh.  This isn’t a tiny sample.  The incision will be more than half the length of his thigh.  Some of his doctors have suspected a mitochondrial defect from the beginning, but because the test is so invasive, part of the diagnostic process involves ruling out every other possibility before doing the biopsy.  There are many different mitochondrial diseases and they vary in severity, but they are all terminal and degenerative.  Some just move faster than others.  If Noah does have a mitochondrial defect, the doctors might be able to narrow it down to a specific syndrome with a known prognosis, or they might not.  If you are very interested in mitochondrial diseases, a Google search will yield lots of information.  I won’t take up room here unless Noah is eventually diagnosed with one.

 

Of course, with six brothers and sisters, Noah certainly doesn’t exist in a vacuum!  Recently things have been even busier than usual at our house, and there are several ongoing dramas that are being regularly updated on our blog.  When they are resolved, I will probably just remove these stories from this overview post, but for our new readers who may wonder about the stories behind the updates, here goes! 

 

Our four year old daughter, Sarah, was recently attacked by a squirrel that is believed to have been rabid.  It charged her in our yard (she says it tackled her!), climbed up her leg, dug its claws into her little hand, and bit her fingers to the bone repeatedly.  Officials were unable to catch the squirrel for testing but are fairly certain it was rabid because of its behavior (attacking Sarah, attacking but failing to bite our ten year old, running into things, biting itself, etc.)  As a result, she is currently undergoing the rabies series which has been VERY hard for her.  Not only are the shots excruciating painful, but she has had some adverse reactions to the vaccine.  One of the reactions earned her a night in the E.R. because she was delusional, hallucinating, biting herself, etc.  We are enormously thankful that the other episodes have been far milder. 

 

A couple of weeks ago our ten year old son Matthew slipped and badly sprained his ankle and probably suffered a hairline fracture of his fibula right above his ankle.  So far he is healing miraculously well.

 

The large 15 passenger “big red van” that our family needs for transportation is currently gravely ill.  It may be terminally ill.  Because there was no earthly way for us to afford the repairs needed to make it drivable again, my hard-working husband accepted the offer of a friend to help him learn to fix it himself.  They have been working SO hard on this, but to no avail so far.  Our friend is a perfect genius with cars and knows “how” to fix our van, but because it is so old (1993), they are running into parts that have literally rusted into place.  We are without family transportation at this time and are earnestly praying that Jeff and his friend will be able to fix our poor old van!  When the biggest issues are fixed so we can drive again, we will need to pray about our future transportation needs.  We bought this very old van for cash about a year ago because we needed something that would hold our entire family.  Since we live in a small town and I seldom drive more than 5 miles to go anywhere, the van has been great for us.  We obviously never anticipated the astounding number of out-of town trips we have had to make for Noah’s doctors’ visits and hospitalizations.  We have just asked too much from our faithful red van!  We are thankful that God is allowing Jeff to work on repairing it at a price we can manage, and know that God will give us wisdom about what to do about transportation in the future.

 

I think those are all of the main, recurring things that currently appear on our blog.  The other thing you will see in nearly every post is a blessing list.  Jeff and I truly embrace James 1:2-4, which says “My brethren, count it all joy when ye fall into divers temptations; knowing this, that the trying of your faith worketh patience.  But let patience have her perfect work, that ye may be perfect and entire, wanting nothing.”  Those verses and other precious verses in James became very meaningful to us during my difficult pregnancy.  The fact that Jeff was teaching through the book of James at our church (did I mention that he is the best teacher in the universe??!!!) was such a blessing and encouragement to both of us – so much so that we named our baby Noah James Joseph.  The phrase “count it all joy” has become like a family catch phrase, and shortly after we started this blog we decided to end each post by literally counting our joys and blessings.  This is a very real thing for us and it doesn’t always “look” ultra spiritual or pious – in fact, Snickers bars and the Red Sox have appeared in more than one blessing list!  We are just ordinary people who serve an extraordinary God and want everyone to know all of the deep and meaningful AND fun and silly ways He is blessing us every single day.  If life ever calms down enough for me to start posting mundane things like recipes or homeschooling tips, I can’t promise that I’ll list ten blessings along with my chocolate cake recipe, but I do know that right now in these trying days that I need to very intentionally keep my focus on all that is so good in our lives.

 

I hope this loooong post is a help to those of you who have wondered about some of the things you have read here.  We want to keep this updated and hope that it will continue to be a blessing to our readers.  If anyone still has any questions after reading this, please leave your question in the comments section (or email us) and we will promptly update this post to reflect those questions. 

 

Love, Kate and Jeff and the children

 

AUGUST, 2008 UPDATE

 
SOOO much has happened and changed in Noah’s life since I wrote the above post.  I haven’t been as faithful as I intended in terms of keeping this part of the blog current, but I’ll try to bring you up to speed as briefly as possible.

 
As of today, Noah is just 5 days away from his second birthday!  He was hospitalized not long after I wrote the above post.  The hospitalization was meant to have been for minor surgery to convert his g-tube to a gj-tube, but it was the start of very grave complications for Noah.  That surgery was a failure and Noah ended up staying in the hospital for over six weeks, during which time he had MAJOR abdominal surgery to correct severe birth defects in his gut (which had gone undiagnosed until then).  He became septic twice, had two broviacs (central lines) placed, was medflighted to CHOP in Philadelphia, and was started on TPN.  TPN is a form of nutrition that goes into the bloodstream through a central line.  It contains fats (lipids), carbs, proteins, vitamins, etc.  It saved Noah’s life but it is also slowly destroying is liver.  He can’t live without it, but he can’t live for long with it either.  The TPN was started because his ability to be fed  by g-tube was actually decreased rather than improved after surgery.  The discharge plan was to gradually increase tube feedings and decrease the TPN, but not long after discharge Noah became unable to tolerate any g-tube feedings at all.  He is now completely dependant on TPN for his nutrition and hydration.  He is hooked up to an IV for 14 hours each day to receive his TPN.

 
After that memorable hospitalization he continued to endure repeated hospitalizations, surgeries, and four more episodes of sepsis.  He was rarely home and spent most of the rest of the year in the hospital including most of our birthdays and even Christmas.  We nearly lost him more than once, but this amazing child just kept fighting.

 
On January 29, 2008 we were blessed with the birth of our 8^th child, Mary Faith Adele Estes.  Noah had been very gravely ill and in the hospital until less than 72 hours before I went into labor - we were starting to think I would give birth in the hospital with Noah!

 
Since Mary Faith was born, Noah has stabilized more than we ever dreamed he would.  In June we went to Atlanta for a consultation with a specialist and Noah ended up getting sick and being admitted to Scottish Rite for almost two weeks, but this is the only time he has been inpatient since Mary Faith’s birth!  This time that he has spent at home has been a healing and refreshing time for all of us.  You can’t imagine how wonderful it has been to all be together as a family for all this time.  (When Noah is admitted, it is to a hospital an hour and a half from home.)  Noah has truly blossomed and is walking (!!), saying a number of words, becoming proficient in sign language, playing with toys, and loving life.  He is the most joyful person I have ever met and is truly a delight to all of us.

 
Unfortunately, the fact that he is stable doesn’t mean that he is getting better.  Geneticists in Philly found that Noah has two defects in his mitochondrial DNA and he is now considered to have mitochondrial disease.  There is no treatment and there is no cure.  This is a progressive disease that is probably terminal.  In June Noah had a muscle biopsy (his second) in the hopes that we would learn more about how his mitochondria are being affected by this disease.  He has developed dilated cardiomyopathy which is being managed well with medication.  He is in partial bone marrow failure which means he needs regular transfusions to stay alive.  His immune system isn’t functioning well so he receives monthly infusions of IVIG, a human blood product that contains immune antibodies.  If he weren’t getting the IVIG he probably wouldn’t be with us today.  While Noah is now able and willing to eat, he only eats a teeny amount of food and he doesn’t seem to absorb nutrition from it at all.  He is also not absorbing the TPN well and seems to struggle to stay hydrated and nourished.  He has frequent episodes of pain that we can’t identify.  He can’t tell us what is wrong and all we can do is rock him and love him until he feels better.

 

In spite of all this, Noah has the most amazing spirit.  He is considered very medically fragile, but he doesn’t look it!  He is a sturdy, beautiful toddler who has a way of stealing hearts everywhere he goes!  We are beyond blessed to be his parents.  To see some current pictures of Noah, click here!

 
Other changes in our family since the above update include . . .

 
1.  We said goodbye to the Big Red Van and are now the proud owners of the Big White Van.    Unfortunately the Big White Van took a page from the red van’s playbook and is giving us a run for our money right now.  The big sliding door was damaged when it was opened on a steep hill and now it won’t open . .  . ugh.  At least it runs well!!

2.  Sarah has completely recovered from the rabid squirrel incident but continue to be Nature Challenged.  Since I posted last she has had a number of other nature-related tragedies including getting into a nest of ground bees, being head-butted by a cow and sent flying, and others.  We’re thinking of not letting her go outside anymore until she’s thirty. 

3.  Jeff lost his job the first week of May.  It was a career level upper management job, and he had always had fantastic performance reviews and consistent promotions, but the position was eliminated in a round of cutbacks.  He has not since found employment, but we are waiting on God’s timing and being blessed by God’s hand of provision.  Jeff was only given four weeks of severance pay, but we have continued to see our every need met.  We’ve even been blessed with luxuries like the occasional Starbucks card or meal out!  He is a wonderful man and a hard worker and is doing all he can to both find a job and to build our homeschool business (www.handsandhearts.com ) to the point where it could perhaps fully support us.

4.  Of course, having our newest little girl is probably the biggest change.  Mary Faith is a true delight and joy.  She is a very petite little thing but seems to be totally healthy.  She is unbelievably happy and content.  We are so blessed to have her in our family, and Noah really adores her.

5.  We have learned and grown in ways that we never dreamed possible.  God has shown up over and over as He has drawn us into His arms, loved us, and provided for us.  None of this has been easy, but it has been beautiful.

 
Blessings,

Kate