quick and brief

Like always it's been a bit since an update.
So much has happened.  December was hectic.  Eli was inpatient 3 times. The first starting to get sick.  The second he had full on RSV.  That was really rough for him to get past.  The 3rd was hickman issues.  At first it wasn't drawing blood, then it wouldn't flush.  They ended up having to surgically remove it and replace it with a different hickman in a different location.  It was a mess of a month.
Eli is also still struggling with absorption issues.  We have had to switch 1 med over to IV and will be switching another over next week.
Eli will be admitted on January 2nd and will get a loading dose of Keppra, followed by regular doses.  He will hopefully go home on Friday assuming there are no issues with his bodies response to the IV Keppra.  We really need to get his keppra levels up.  We are also working on his lovenox levels as we can't seem to get it spot on.
My surgery is on Jan 8th.  A lot of feelings and emotions surrounding all of that. The holidays kept us busy for awhile but the last thing now to focus on is Eli's upcoming admission and my surgery.
I'm not ready and keep trying to talk myself into rescheduling, even though I know that won't help at all.
I will get more updates up before my surgery!

Hell of a Week

Eli was hospitalized Nov 30-Dec 1st.  He was sent home assuming that he would get worse, but far better to be home then in patient around more germs.
By Sunday Eli was starting to struggle.  By Monday he was running a temp, vitals a mess, lungs sounding yucky, and requiring O2.  For sunday and monday eli only peed 3 times in 48 hrs.  By Tuesday he was considerably worse and was needing quite a bit of O2 and alot of extra breathing treatments and such.  Still minimal pee even with fluid increases.  By Tuesday evening he was struggling hard and at this point we had been holding him upright for over 24hrs.  They approved 24hr nursing and they stayed up with him all night. 
Wednesday morning Eli was admitted to the PICU.  His sats were low, his resps were 60-70 and his heartrate was super high.  Temp 102.8 and he was requiring more pain meds and breathing treatments than we can even run at home.
We learned on Thursday that Eli has RSV and pneumonia.  Both lungs were hazy.  Alot of gunk settling into his lungs and alot of trouble moving it all.  They started him on a pretty intense regimin of breathing treatments, CPT, and cough assist.
During his hospital stay alot happened.
His lovenox (blood thinner) levels aren't enough.  He had a major bleed inpatient, as well as clotted off 2 needles.  Strange combos.
He was switched to IV robinul as it was apparently clear that his body isn't aborbing the robinul thru his J tube. 
We also learned that his seizure meds aren't being absorbed either and have had to increase them.
We've had to increase his IV fluids as they just aren't enough to keep him well hydrated.
He had a modified upper GI (barium placed into his G tube) and his fundo is in tact and isn't causing reflux.  So we now know that for sure all of these choking spells are from secretions not refluxing. 
Eli came home late Sunday.  The hospital is full of RSV, Flu A and B, pertussis and rhino virus.  Eli has had pertussis twice so the goal was to get him out of there and get him home where he can be better protected.
He's still struggling.  Temps still, wacky vitals, alot of gunk in his lungs.  Requiring a whole lot of suctioning.  Still can't get him even weaned down on the O2, let alone off of it.  Last night he started having trouble maintaining his body temp, which is normal for him usually, but this time it's the opposite.  His extremities are constantly cold with a slightly delayed capillary refill.  He's NEVER been too cold.  Eli's body has always run super hot.  So this has been a new strange challenge as it's not something we are used to doing, keeping Eli warm!
Yesterday he also had all of his kidney scans.  There is some big improvements to his UPJ obstruction.  His surgeon is really happy with what he saw on the scans and so are we! 

In other household news....the world very clearly didn't stop.
Both bob's car AND my van broke down in the same day...Sunday.  Bob's car is assumed dead.  My van was towed to a shop.  Come to find out while it was parked in the hospital parking garage for a week that it was attacked by a squirrel or mouse.  They chewed thru my spark plugs, my O2 sensory and some other thing that I can't remember.  Ended up costing $450 to repair!!!  The mechanic saved all my parts and has suggested that we file a report with the hospitals parking garage.
Bob, Ben, Ari and Lauren are all sick.  It appears to be the same thing that Eli has.  Sadly for them it has to run it's course.
Last Wednesday Emily and Jace were both sent home with the stomach bug.  Wednesday 70 kids were sent home with it, Thursday 85.  The health dept ended up closing the school on Friday and they did a deep cleaning and de-sanitizing over the weekend.
Jace had his renal and testicular scans last week as well.  Lauren was able to take him.  His doctor (also Eli's primary doc) came to Eli's hospital room that evening.  He informed me that he was sorry to be delivery more bad news.  Both of Jace's kidneys are very small for his size but appear to be functioning well.  The biggest issue is that both of Jace's testicles are in his abdomen stuck with hernias.  He is putting in a referral and  Jace will need to have surgery.  It is unsure right now if they will be sterile or functional after being inside for 9 years.

We finally got the christmas tree decorated last night.  Christmas is right around the corner and I'm so not ready.


I'm pretty much done with it all.  I'm exhausted.  My surgery is in 27 days.  I feel as though the world is beating the fucking shit out of me.  Everything that could go wrong is constantly go wrong.  I don't know how much more I can physically, emotionally and mentally handle.  I'm struggling right now to keep going thru each day.

I will get more updates up soon.  I've got so much to get done today, and a sick Ben and Eli home!

Super Quick update

Eli is a bit more stable this morning, although definitely still struggling. We are getting pee, but his doc is still keeping his fluids running high. His temp came down some but is on it's way back up. Resps and HR still up there. He got a tiny spurt of awake this morning and sat up with his ipad but that wore him out. The docs suspect that he has RSV. They will be bringing him into urgent care for the day on Friday to start the IV robinul. Until then the plan is to hopefully stay home and keep working our way thru it.

Struggling

Eli is really struggling.
He has hardly slept the last 2 nights.  He's been up choking and requiring alot of suctioning.  last night he started not being able to keep his O2 sats up without oxygen.  He was needing suctioning every 15 minutes.  His temp went up, which left him with a high hr and resps.  By this morning he could barely sit up or open his eyes.  He has spent the entire day sleeping and is on O2, needing suctioning and still running a fever.  Yesterday he only peed twice and today isn't going any better. 
This morning his primary doc and I had a long talk.  He's starting Eli on antibiotics.  We also discussed the options we are left with to deal with eli's secretions.  The goal is to get Eli started on IV robinul, hoping that this will allow his system to absorb it better.  If this doesn't work the next option (and last option) is a Scopolamine patch. 
We also discussed the seizures and the neurological changes.  Eli's keppra levels came back incredibly low.  Again either he's outgrown his dose, it isn't working for him anymore, or his body isn't absorbing it properly.  For now he's having us up the dose.
He's thinking that there are multiple things going on here.  The seizures, the secretions, and Eli being sick.  He doesn't believe that fixing 1 will fix all.  He's also not sure right now how much of this is disease progression and how much of it is fixable issues.  He told me he doesn't want to make any promises as he isn't sure. 
He and I discussed the suggestions from the new palliative care doc.  He really likes her and trusts her thoughts and instincts.  He believes that she will be a good fit on our team. 
We also talked about how we hate progressive disorders and how I'm not ready for another progression.  How I'm not ready to lose something.  I'm not ready to take a step back, knowing that there is a chance that we will never regain ground.  Rarely have we regained ground in regards to Eli's health and interventions. 
I'm not ready.  I wasn't ready to learn the true meaning behind the word progression.  I wasn't ready to watch my child suffer thru something this torturous.  I'm not ready to hold my child while he's choking knowing there is nothing I can do.  I'm not ready to feel his body limp against me because he doesn't have the energy to hold himself up. We've been here before and I'm not ready to be here again. 
Please keep our family in your thoughts.  Keep my tiny little warrior, eli, in your thoughts as it's so hard for his little body to fight thru all of this.  Please keep our oldest 4 in your thoughts, as it's just as hard for them to watch Eli struggle.  This round has come on fast, and just yesterday afternoon they were able to sit up and play with Eli as he followed them thru the house.  They will come home from school today to find a very sick little man unable to sit up at all.  Please keep Bob and I in your thoughts as we have hardly slept for 3 days.  We are exhausted and having to make big tough decisions.  Never a fun mix.
We will update as we can.

Admission and a THIRD birthday!

What a weekend.
Friday Eli was really struggling.  He was choking quite a bit and started to run a fever, as well as incredibly high resps and a high hr.  We ended up calling the doc and they suspected pneumonia.  Off we went.  Eli ended up being admitted and they ran cultures on labs and urine.  The choking episodes are really causing his little body to struggle.
Eli was discharged on Saturday, his 3rd birthday.  We had a quiet evening at home and he was able to open his presents and spend a bit of time with the family.
He had an incredibly rough night Saturday night and was up choking a large bulk of the morning hrs.  He slept in Sunday and has had countless choking episodes today. 
The docs have tried to change around his meds but so far no change.  While we were inpatient we met with a Palliative Care doctor and she is going to be joining Eli's team.  It's time to have some serious quality of life talks and begin to discuss some "out of norm" options for Eli.  She has some ideas and we are definitely on board with discussing them and working out a better plan.  It was really refreshing to chat with her and we are going to be scheduling with her for a full sit down conversation. 

I woke up this morning sick.  I've got a sore throat and a bit of a cough.  I'm definitely hoping that it doesn't progress as I can't afford to be sick. 

I will get more updates up soon.

It's Wednesday Already!!!

We ended up talking with Eli's primary doc again on the choking.  The newest meds have made no difference.  The last guess is the robinul.  They are increasing his dose over the rest of the week and then if that's not enough then another increase on friday.  If that still doesn't make a difference then they will do some exploratory things under anesthesia to try and figure out if we are missing something.  We were here so long ago and are not ready to be here again.
Eli also appears to be having a potential reaction to the neurotin.  We have seen what are possibly breakthrough absent seizures as well as he is either very sluggish or very intense.  He is also tripping and falling and running into things way more often than normal.  Neurologically something has definitely shifted.  He's also struggling with body balancing of fluids.  He's bloated, but dry. 

I  met with my orthopedic surgeon again today.  My hand incision has been struggling and continues to open up.  He asked me today if I have been diagnosed with a thyroid issue or a metabolic issue as that's when he sees these issues.  I'm also struggling to use my hand and the muscles are tight, my thumb is now pulling into my palm some and I have limited wrists movement.  Still having trouble gripping things and major loss of strength.   It hit me like a ton of bricks when he asked if I had a metabolic issue.  He is one of the docs that hasn't been involved in the Mito workup and knew nothing about the suspicion.  It terrifies me that so many doctors are independantly coming to the same damn conclusion.  The surgeon has taught me a new way to steri strip and is sending me to some intensive OT.  I start the OT on Monday. 

Some of my testing has come back and my primary doc is calling around to get me into a Geneticist.  We know how much time this takes, but are hoping we can get this done fairly quickly. 

We will also be meeting with a lawyer this week.  We will be drawing up a will.  It's something I feel we shouldn't avoid.  I think I will feel more comfortable if this is all done and prepared as a just in case.  I never imagined that at 33yrs old that this is something I would ever consider.  I don't want to think about these things, let alone say them out loud, and write them down.  But alas here I am.  No we don't think I will die.  But yes we are being realistic and knowing that some major things are coming up and with any major thing comes great risk for complications and unexpected turns.  We want to be prepared.  We have that responsibility as parents to be prepared.  We are now even more thankful that we have been seeing a grief counselor for months, before my journey really began for myself.  She has been an amazing support and a place for me to openly state my thoughts and fears, and be able to plan and talk out loud. 

Right now we are chugging along best we can.  We have begun Christmas decorating and are trying to throw ourselves into the holidays as much as we can.  I am getting a better control over my emotions (atleast at this point) and have done a better job of staying in the numb realm during the main parts of the day and only fully letting the emotions roll thru when the kids are either at school or late at night.  This may not be the options that therapists all push for, but nothing changes the fact that I'm the mom to 5 young children...4 of which already have to deal with the reality of their little brothers illnesses. 

I will continue to keep some updates coming over the next few days.
And don't forget to check out the post just below this one for a slideshow of our family photos.  They turned out absolutely amazing!!!

Family Pics

I don't have time to get up a full blog post right now but just wanted to share the link for our family pics!  Enjoy!

Family Pictures Slideshow

Family Pic and Updates

Saturday we had family pictures done.  It was alot of fun.  So far we have a sneak!

We spent the rest of the day at the park and relaxing as a family. 

Sunday the big 4 went to the farm with Lauren for the day.  Bob, Eli and I went to church, ran errands and had some quiet time together.

Monday I had an appt with my primary doc.  We regrouped from everything that has been going on. She began testing me for mito.  11 tubes of blood and 1 cup of pee.  She also is testing me for Factor V Leiden.  She really wants to verify if I have the same blood clotting disorder as Eli before I have brain surgery in January. She re-ran my anemia labs and if my B12 is still really low, then it means my body isn't absorbing it and she will switch me to B12 injections.
My hand is still struggling to heal. The incision doesn't want to close up, and I'm still struggling to move my fingers and wrist. The surgeon will be working to get me into OT next week.

Eli is also having a rough time. He's choking and gagging so much. His sats are dropping and he isn't moving air well. Then afterwards his lungs are really wet and he's worn out. The docs originally thought it may be his stomach so put him on more meds to try and help his stomach move, but no change. So now they have added a new med to try and help calm all of the nerves in his stomach. They think the nerves are in overdrive, hypersensitivity. The ultimate suspected reason for all of this is that Eli's body has built up an immunity to his robinul. This is a worst case scenario. We are worried. We avoided a trach about a year ago, and are fearful that it will be brought up again. Eli's restless leg syndrome has also been in full swing lately. He just got a weighted blanket yesterday from his grandparents for his birthday. The docs are hoping that if he sleeps with it that it might be just enough pressure on the nerves to keep things calmer during sleep.

We ended up heading to Wichita late in the evening Wednesday. We spent thanksgiving day with my family and then Bob, Eli, and I headed back here yesterday afternoon. The 4 oldest are hanging out with my parents until Sunday afternoon. I'm looking forward to some quiet time around the house these next few days. Bob and I are planning on going out for a much needed date night tonight. Today I may go do a touch of black friday shopping but nothing to major.

This morning I have another nurse interview. The agency is working hard to find us 2 more RN's. We are going to need round the clock nursing during my surgery and immediate days in the hospital, and then nursing 7 extended days a week during my at home recovery. Currently we have nursing 6 days a week 12 hrs a day. So they are having to find 2 more RN's to help cover such extended hours. I'm hoping to get both new hired and trained during the month of december. The goal for the 1st week of January is to simply spend as much quiet family time together as possible.

The oldest 4 are ok. They are so busy with school and choir and violin and math club and student council and book club and church and friends and so many other activities that they participate in.
The kids now all have their own bedrooms after some basement re-arrainging and they are loving it.  The youngest 3 (Eli, Ari, and Ben) all have bedrooms on the 2nd floor down the hall from Bob and I.  Emily and Jace now have bedrooms in the basement.  The kids also have their own family room, complete with Tv and couches.  They absolutely love it.  We are glad we were able to come up with a way to allow them to have their own space.  They are busy decorating and organizing and they are all hoping to get a few more decorations for their rooms come christmas time!

As always we have alot going on. Emotions are running high. I'm doing the best I can to appear as strong as possible for the kids and for friends and family. I'm exhausted and feel like crap each and every day. I feel rushed as my surgery is only 46 days away. There are so many things I want to get done and ready and organized. Now that Thanskgiving is past that throws us straight into Christmas and this is a huge holiday for our family. I'm trying to constantly remind myself to be in the here and now with everyone but my mind can't stop. I'm scared about Eli's instability right now. I'm scared about something happening to him while I'm in surgery or the hospital. I'm scared about the fact that I'm the only one in the world who knows EVERY drop of Eli's medical information and care. I'm scared about having 5 children and going in for brain surgery. I'm scared about what could go wrong. I'm scared for the recovery. I'm worried for my family and caretakers. I know the mental, emotional, and physical strain and exhaustion that comes from caring for a sick loved on. They will not only have Eli, but me now too. In short..I'm scared. And nothing makes it better.

I will update again soon.

checking in

It's been alot. 
I had surgery on november 2nd on my right hand.  They ended up having to cut 2 ligaments and shave down my tendon.  I got my stitches out and large bandage removed 1 wk after.  I have a smaller brace now and am able to start moving my fingers, but not my wrist.  I'm not able to hold anything yet and am still having to only use my left hand.  that makes everything very slow and frustrating, but I'm slowly figuring it out.  Next week I get to start practicing with my wrist and if still struggling then he will start me with some occupational therapy.
I also met with the neurosurgeon last week.  My surgery is scheduled for January 8th.  He said I will be in the neuro icu for a few and then on the floor for a few.  Then a minimum of 8 wks recovery at home.  We are rushing to get life set up.  We have so many things to organize between eli's nursing care and ben's respite worker. 
I meet back with my primary care doc next week.  The docs have all decided that we need to run some more labs, do some hearing tests, discuss my vision loss more, and send me to a geneticist for.. yes as most of you have guess...mito testing.  It's been talked around for a bit by us and the docs, but is now being strongly suggested.  We haven't said anything up till now, but we are being asked frequently so it's easier to simply mention it at this point.
I'm exhausted and sore.  My mind is constantly spinning.  I can't completely focus on anything, although my mind is always going.  I've moved past the crying phase into numb.  I've been in the numb phase many times as we have made our way thru Eli's journey, but it feels strangely unfamiliar when it's in relation to my journey.

Jace's GI issues have been flaring and his GI doc has been working hard to get it back under control.  He's had tests run and meds changing.  Hoping to see some relief for him quickly.

Eli is keeping us forever busy as well.  He's back to choking and gagging.  It's not yet as often as it used to be but alot more violent and large o2 drops (70's and 80's).  we have been draining his stomach frequently to try and help.  His Gi doc thinks that the nerves in his stomach are becoming so sensitive that any sensation is causing them to fire.  He's also concerned that Eli's body may be building up an immunity to his robanul which is VERY bad news.  They are changing around some of his meds and hoping to see some changes.  If this doesn't help over the next 2 weeks there will be more changes that bring more risks. 
We've also been working hard on Eli's vision.  He's having a full functional vision evaluation next week.  It's very clear that his depth perception and peripheral vision are shot.  we are hoping to find ways to strengthen them and help him be able to safely move around unfamiliar environments independantly.  In our home we use red tape on danger areas and we never move furniture. 

The other kids are staying busy.  Alot is on their plates and on their minds.  Our kids are so strong and amazing!

As my hand strengths returns i will try and update more.  this update has taken almost 1 1/2 hrs to type.

Numb

It was a roller coaster of a weekend.  On Friday we obviously got the news on me.  We told the kids Friday evening and had an evening home.
Saturday was Emily's birthday so we tried to keep the focusing on celebrating her 12th bday!  We went to Legoland and out to dinner.
Saturday evening bob and I stopped by a friends halloween party and hung out for a little bit.
Sunday Bob and I took Eli to church and the older 4 went with Lauren and my parents to lauren's church.
Sunday evening the olders went with lauren to a bonfire at church and Bob and I had some quiet time at home with Eli.
This morning has brought around 20 phone calls, some emails, appts, and alot of planning.  It's all in planning mode at this point.  They have scheduled me a rush appt with the neurosurgeon on November 8th.  I have my surgery on my right hand/arm on November 2nd.  So it's alot of things happening really quickly.
Today was luckily Monday so I had my standing appt with my grief counselor.  I absolutely love her and have no idea how I would make it thru without her.  I was able to openly really talk to her today about some of my thoughts and feelings and things I wanted to get done.
This evening is my first class titled "Support for the Supporters".  Right now it seems so far out and strange. 
The weekend was an unpredictable emotional rollercoaster.  Some songs simply caused me to cry.  Someone talking to me.  Just sitting there thinking in those few un-busy moments.  Today I feel numb.  It's been a whirlwind of planning and that's kept me mostly busy.  I've only cried once today and that's once to many for me.  I'm so tired of crying.  I'm so tired of feeling any emotion right now.  I'll take numb.  After these last 3 yrs of continual bad news and heartache, numb is very much welcome.
I will get more updates up this week, but just wanted to hit a brief check-in.

Crushingly Devastatingly Terrified

I've been behind on updating as I've been pretty sick.  I've been having migraines daily.  They are there when I wake and when I fall asleep.  My eye sight has been struggling as well as my hearing.  I've had facial and eye twitches.  I can no longer lay on or touch the back of my head.  My ears hurt to the point where I can hardly talk on the cell phone without them throbbing.  They put me on meds that made me even sicker.  I've been to the neurologist and the family doc.  They did an EMG and discovered that I have severe carpal tunnel with sensory nerve, motor nerve, and muscle damage.  They scheduled me for surgery November 2nd. 
Then they did neck xrays.  They saw some issues with arthritis at the base of my neck.  Next they did an MRI of my brain and neck.  This was on Tuesday.
My doctor called me today with results.  She apologized 10 plus times while giving me the results.  I listened and wrote notes as she talked.  Then cried and cried and cried.
I have an Arnold Chiari Malformation.   She is sending me to a neurosurgeon for surgery on my brain and spinal cord.  I'm terrified. 
I feel as though I'm being crushed.  I have felt my strength wavering over these last few months as we've gone farther down the road of Mito with Eli but this is more than I can bare. 
We told our kids tonight and our immediate family.  I simply don't have the strength to call everyone so please don't be offended if you weren't on the list of people we personally spoke with.  I just can't say it outloud that many times right now.
I don't know how we are going to make it thru, but I know that my friends and family are amazing and I know that they will drag me kicking and screaming thru this.
Feel free to text, call, email, drop by, or whatnot.  We know that we need the support of everyone around us.
I just don't know if I can do this.

I will get more updates up on Sunday.

Quick Check In

I apologize for missing Mito week.  Eli ended up with 14 appts, 6 therapies, I made 36 phone calls, signed for 5 deliveries, gave 336 medications, 14 shots, 2 lab draws, 2 dressing changes, 34 breathing treatments, and more medical care than I can even begin to list. 

Last week Eli was admitted to the hospital for 24 hrs.  His lab work has been a mess.  We ended up needing to change his IV fluids.  He is now on D5 NS so that he is always getting glucose and salt.  It seems to be helping give some more consistency to his sugars.  We are still waiting for them to get his Mickey GJ tube ordered so we can get that changed out as the valve is again broken and his body is again eating thru the silicone.  Also Eli's geneticist / Mito doctor is leaving the country.  This means more big changes on the horizon for Eli. 

Final news.  My doctor was finally able to get me into the neurologist this last week.  He ran an EMG on both arms.  He is unsure how I'm even able to use my right arm at all.  I told him I hardly am.  I have severe motor nerve, sensory nerve, and muscle damage in my right hand, arm, elbow, shoulder, and neck.  My left hand and arm and elbow only have some mild sensory nerve damage.  He said I definitely need surgery.  We also discussed my migraines.  He made some adjustments to my meds.  He is sending me for an MRI and is concerned about the severerity and frequency.  My vision is struggling and so is my hearing.  I can't remember the last time I didn't have a headache.  He is meeting with me next week to come up with a firm plan and get everything scheduled.  More info but right now I'm not ready to go into full detail. 

Please keep our family in your thoughts as we navigate continually uncharted waters. 

Missing

Missing.  This is a word that no parent ever wants to hear.  But we did.  We were told that our middle son, Benjamin was missing.  Benjamin is our 7yr old who is Autistic, has OCD, Anxiety and Sensory Integration disorder. 

We told Ben and his teacher that we would be picking him up at the end of the day on thursday and that we would all meet in the office.  They said great he will meet you there.  His other siblings came but Ben never did.  They searched the buses 3 times.  No Ben.  They called the bus company to search the buses 2 times.  No Ben.  All pages thru the school.  No Ben.  All of us looking all over the school and playground.  No Ben.  Looking in parked car windows.  No Ben.  Finally after 45 minutes of no Ben the principal and I called the police.  8 or 9 police showed up. 

Did I mention it was 50 degrees, pouring rain, and Ben was last seen in shorts and a tshirt?  He's only 45ish lbs, skin and bones.

Finally after an hr of searching my nurse called and said that Ben made it home.  He had gotten on the wrong bus and then since it was raining he said the windows were so foggy that he couldn't see anything so he didn't know where he was and where he was going.  He said that he heard his name over the loud speaker and that he tried to talk back but that we couldn't hear him. 

The school openly admits to dropping the ball.  Police reports were filed as well as reports with the district.  Currently they are having someone walk Ben straight to and from his bus and we will be meeting on Wednesday to discuss a permanent plan.

I hope to never hear those words again.

Pictures from the Mito Walk

Walk or not we still get to do medical stuff!  Someone tiny was having issues with not peeing, he had no bowel sounds and diminished lung sounds in the lower lobes.

eli got to walk the beginning lap of the 3k walk!

Fundraiser Totals

I'm exhausted so this will be a very short update.  The day started out well, although emotional, got VERY rough in the middle, and ended decently.  I will get full info up tomorrow but for now here are the numbers.

The school fundraiser is finished. The money is totalled. We are beyond humbled. They raised .....DRUMROLL.....

$828.55!!!!!!

My kids have raised a total of $1,105.55 for the UMDF Energy for Life walk this year!!!!
We are amazed by our kids and amazed by their school community!

Thursday Announcements

Thursday

Mitochondrial disease is the body’s failure to turn food into energy.

Mitochondrial disease is underlying in many different illnesses.  Some of these are cancer, alzheimer’s, parkinson’s and many more.

Eli and so many other mito children and adults are warriors.  Eli is our families superhero.  Battling each day to stay strong. 

How can you help those with mitochondrial disease?

You can raise money for research and patient support just like you have done this week.

You can tell your family and friends what mitochondrial disease is.

And you can join our family for a fundraiser walk on Saturday, September 15^th at the TBones Stadium.

Our family would like to thank you for the outpouring of support and generosity this week.  We are forever blessed.

You can continue to follow Eli’s journey on the blog or by simply asking whenever you see us in school.

You can join our team or make an online donation by clicking HERE for Team Eli's Journey

Wednesday Morning Announcements

Damaged (sick) mitochondria can cause children and adults to have problems in making their bodies work.

Mitochondrial disease is NOT contagious, which means you cannot catch it by touching or being near someone with the disease.

It is a genetic disease, which means it is part of your family’s health history.

Currently there is no cure for mitochondrial disease.  Our family wishes every day for a cure, as do so many other families out there.

This fundraiser goes to helping learn more about Mito and to help try to find a cure. 

Remember to continue bringing in change and bills to and placing them in your donation bins in your classroom.  Remember that the winning classroom wins a pizza party!

Tomorrow is the last day to bring in your donations and it is also the day to wear green!  Green is the mitochondrial disease awareness color! 

Remember if you ever see Eli in the halls of our school, remember that he loves to talk and play and that you can’t get sick from talking and playing with him!  He enjoys books and laughing and having fun!  Feel free to say hello!

Eli updates & thoughts & emotions

I'm sorry I didn't get a post up yesterday evening.  I'm exhausted.  Like always...physically, emotionally and mentally.

There are so many days lately where I'm simply unsure how we are going to get thru.  Then we get thru the hectic roughness of the morning.  I make it thru all of the medical pieces that remind me how sick my little guy is.  We make it to that sweet spot time, his Pinocchio time.  I live for this time, our whole family does.  Everything about the other 18 hrs is simply making it to our next chunk of Pinocchio time.

For those new to Eli's journey I will stop quickly to explain.  Pinocchio time is Eli's "I've got no strings" time.  This is the little precious time of our life each day where he is unhooked from everything.  All of his medical paraphernalia is in his room or tucked under his shirt.  He's able to run and play independently without someone right behind him holding his tubes and machines.  He climbs on the couch, chases the puppy, tumbles around on the floor with the kids, gets into trouble dumping my purse and his diaper bag all over the floor, and digs thru the kitchen cabinets and drawers.  This is his time to be a toddler.  Don't get me wrong we still have to do medical things.  He gets meds and flushes and blood sugars every hr during this time.  But we do it as quickly as possible chasing him thru the house to get it done.  It's not just HIS Pinocchio time, it's OUR Pinocchio time. 

Why am i down you may ask?  Last weeks lab work is showing more signs of trouble.  Out of a page of labs only 3 or 4 things were within normal range.  His protein is at an all time low.  His red blood cells are dilated.  His hemoglobin is making big drops.  It's just one lab result after another.  His doctor and I spoke at length.  He personally called me to talk.  He says that we are going to have to make quality of life decisions and we can no longer continue to chase numbers.  He says that we are going to have to pick.  The hydration is causing Eli's body to flush out things it should be holding on to.  We are going to have to decide between good numbers on lab work or hydration.  Clearly neither is a good choice.  No parent wants to pick from 2 important needed things.  We are having to make a choice that helps part of Eli but hurts another part of him.  It's not fair.  It's not fair that we keep hearing that we need to make quality of life choices for Eli.  I don't want to do this.  I can't do this.  But I have been given no choice.  His doctor has adjusted Eli's IV fluids and that has helped with some of his numbers, but not enough, and presumably not for long enough. 

I watch Eli and sometimes I laugh and smile.  Sometimes my heart swells to the point of feeling like it is going to burst.  Sometimes it hurts.  It hurts so damn bad.  I used to feel numb.  When I was numb I never understood it and wished to feel.  But feeling isn't always good either.  The roller coaster of emotions that come with this.  It's the hardest most painful thing in the world. 

People are always asking us how we do it.  They are always saying they could never do it.  But they would.  What else would you do?!  Walk away, leaving your child laying on a hospital bed alone??  You do it.  You give your entire self over to the journey.  You sacrifice every molecule of yourself, willing and fully.  I'm not saying this as a martyr type of mentality.  I'm saying this as a mother.  My sole job is fighting for my little man.  Carrying him when he can't.  Being his voice, his protector, his everything.

Every morning I look at Eli and marvel at how amazingly strong he is.  He is a warrior, thru and thru.  I have no idea how he endures what he does each day.  I am continually amazed at his strength and determination and his accomplishments.  He is my hero.

It's not just me that has these feelings.  Everyone in our household will say these same things if asked.  My 9yr old, Jace, has been working on keeping his own journal.  He allows us to read it and the other night he wrote some incredibly deep painful insightful perfect words, that I feel like I need to share.
"I have a baby brother that has a disease called Mitochondrial.  It is like a battery that does not charge.  So they say that he may not live very long but we all love him a lot.  It is sad that he might die when he is a teenager.  We are currently raising money for the walk-a-thon.  right now we raised over $200 which is a lot especially to me since I am bankrupt.  I love my baby brother and I play with him because he is so cute."
To Jace this sums it all up.  His baby brother is his world.  He is the great emotional protector.  He is always making sure that everyone he meets knows exactly how amazing his little brother is. 

They are all like this.  They are amazing. The things they know.  The things that they understand and pick up on.  It breaks my heart to know what they are having to endure.  How strong they are.  Not one single day have they complained about Eli and how he has changed our life.  They are so proud of him, and so in love with him.

Our household is strong.  We are close and tight.  We can function like a well oiled machine at the drop of a hat when needed.  My kids can be as hyper and out of control as can be, and the moment there is an Eli issue everyone can fall in and do what needs to be done with virtually no communication in seconds.  It's amazing. 
Our household consists of the 7 of us who are immediately in our family, 2 nurses, and 1 respite worker.  We are all tight.  I can't imagine life without our in home community.  We are so blessed to have this group.  We laugh together, cry together, and get into water gun fights with 60ml syringes together.  We are family, in this thru the good and bad.

I will spend these next two weeks sorting thru a truckload of emotions.  This week being the awareness week and fundraiser at my kids school, and next week being world wide Mitochondrial disease awareness week.  We are mourning those lives lost, we are wrapping our arms around the families at the end of their journey, the families struggling, the families going thru the diagnosis process.  We are joyfully thankful for the milestones and accomplishments not matter how small.  We are mourning our losses and thankful for our Pinocchio time.  Every day is a grieving process and a joyful time all swirled and blurred into one, but on weeks like these it is so much more defined as we focus even deeper.

We are forever thankful to our communities.  Our in home community, our school community, our teams, our families, our friends, and our Mito families. 

I will keep updating as often as possible.  Life is busy.  Sometimes the news is to painful for me to immediately process and then turn around and type up on the blog.  Sometimes I simply don't have the energy to get it done.  Like we have always said at any time you are wanting an update please feel free to call or email or text!

More soon!

Here is us.  This is our Family.
On the left is Lauren, Eli's primary nurse.  Ari is on her lap.  Then Me and Bob in front holding Eli.  Jace is laying on the bed.  Next to me is Lindsay, Eli's nurse.  In front of her is Michaella, Ben's respite worker.  In front of her is Emily and Ben is in Emily's lap.

Tuesday Morning Announcements

Mitochondria are like tiny batteries within our cells.

Have you ever tried to use something that wouldn't work properly because the batteries were dying?

That's what a person's body is like that his mitochondrial disease.

A new (healthy) battery generates energy for boom boxes, cameras, and flashlights - producing light, sound, and photographs.

An old (sick) battery has a lot of trouble making your things work.

Eli is not able to eat.  He’s not able to swallow food and his stomach doesn’t work to process the food either.  Because of this Eli has a special feeding tube that feeds his intestines. 

Eli’s eyes and ears don’t work well either.  He has hearing aids and glasses that help him see and hear.

Eli learned to walk recently and is now able to walk and play with us more.  He enjoys chasing us and playing with his new puppy.

Please remember all week to bring in your change and place it in the collection bins in each classroom!  The class to collect the most money wins a pizza party!

School Awareness Week and Mito Drive Day 1

We all have Mitochondria.
They exist in nearly every cell of the human body
Mitochondria generate 90% of the energy the body  needs to see, hear, walk, and breath.

Eli’s Mitochondria do not work the way yours and mine do.  That is why he has 14 parts of his body not working properly, is on 62 medications and treatments a day, uses 13 pieces of medical equipment and sees 37 doctors.  He also reads around 20 books a day, and pulls out 2 bookshelves full of toys each day scattering them all over the house for us to step on! 

Please remember all week to bring in your change and place it in the collection bins in each classroom!  The class to collect the most money wins a pizza party!

 

Above was the morning announcements.  We passed out all of the donations bins to the classrooms, hung posters, made the announcements, and chatted with the principal and office staff.  It was a great time and the kids are so excited.

I am running in circles this evening but will get a full update up on Eli this evening hopefully.  Alot is going on with Eli and I know that everyone is always anxiously awaiting Eli updates so I promise to get one up tonight!

Welcome Brookridge Families!!!

Today fliers went out at school!  I wanted to put together some basic information all in one easy place for you to read as you go thru the fliers and hear your kiddos stories of what they are learning next week during Brookridge's Mito Awareness week and fundraiser!
First our family would like to thank you!  The brookridge family is amazing!  Our kids are so blessed to be involved in such an amazing community that is willing to learn and raise awareness about Mitochondrial disease and so willing to fundraise and help the cause!
Secondly we would like to give the parents some insight into what your kids will be learning about.  Each morning there will be announcements with information about Mito.  Below is the Mito facts that will be given.  Each day there will be snipets of information about Eli as well.  I will post those details here on my blog each day.

Monday, September 10, 2012

·         We all have mitochondria.

·         They exist in nearly every cell of the human body.

·         Mitochondria generate 90 percent of the energy the body needs to see, hear, walk, and breathe.

Tuesday, September 11, 2012

·         Mitochondria are like tiny batteries within our cells.

·         Have you ever tried to use something that wouldn’t work because the batteries were dying?

·         That’s what a person’s body is like that has mitochondrial disease.

·         A new (healthy) Battery generates energy for boom boxes, cameras, and flashlights – producing light, sound, and photographs.

·         An old (sick) Battery has a lot of trouble making your things work.

 Wednesday, September 12, 2012

·         Damaged (sick) mitochondria can cause children and adults to have problems in making their bodies work.

·         Mitochondrial disease is NOT contagious, which means you cannot catch it by touching or being near someone with the disease.

·         It is a genetic disease, which means it is part of your family’s health history.

 Thursday, September 13, 2012

·         Mitochondrial disease is the body’s failure to turn food into energy. 

·         How can you help those with mitochondrial disease?

·         Tell your family and friends what mitochondrial disease is.

·         And, join the Tomkins family for a fundraising walk on Saturday, September 15 at the
T-Bones Stadium. 

 

The kids will have an opportunity to bring change (and bills or checks made out to the UMDF) to their classrooms each day and place it in their class buckets.  The class who collects the most will win a pizza party!

On thursday the kids are encouraged to wear green, which is the awareness color for Mitochondrial Disease.

There will be an Energy for Life walk here in KC on the 15th of September.  You can join our team as an in person walker, virtual walker, or make a direct donation to our team By clicking HERE

You can learn more about Mitochondrial Disease by visiting UMDF or MitoAction.  Also please feel free to contact us with any questions that you may have!

Lastly here are some pictures that the kids will be seeing to help understand a bit more about Eli's journey!

 

Our family is always wishing for a cure!

 

Eli's special bed that helps him sleep upright and safely.  Also his oxygen tank,  IV pole, and medical equipment required for sleeping safely.

Eli's medical equipment storage, and 2 fridges to store some medication and all of his IV fluids.  Shelving to store out of fridge meds.  And no little boys room is complete without a toy shelf!

Eli has to wear a special mask when he is in public.  This is not because he is sick, it's because so many others are carrying germs that they might not even realize that they are carrying.  Eli's immune system isn't strong enough to fight off things like a healthy person.

Eli in his wheelchair!  He's able to walk now but tires easily and isn't able to keep up in a public place.  This allows him to be able to move around quickly and easily, but is low to the ground so he can be close to other kids his age!

Eli with his O2 canulas.  He is on oxygen when he sleeps and during the day when he's having trouble moving enough air on his own.  They just sit right inside of his nose and tuck behind his ears.

Eli with his portable O2 tank.  It holds liquid oxygen.  We can refill this tank off of the large metal tank(seen in the bedroom picture by his crib).  This allows him to breath easier by giving him extra support.

Eli using a nebulizer with a mask.  This gives him medication that helps open up his lungs so that he can breath better.  His mask is in the shape of a dinosaur and some are fish.

Elis eyes aren't as good as most people.  One eye doesn't work very well at all so he wears a patch over his good eye each day to help his bad eye start to work more.  He always wears glasses to help him see.

Eli's ears don't work as well, and it is also harder for his brain to process what his ears are hearing.  He's able to wear hearing aids to help him hear better!

Eli's throat and stomach don't work.  Because of this he has a feeding tube called a GJ tube that goes into his stomach.  His tube allows us to drain his stomach and then it has a long tube that goes down into his jejunum (his intestines) and that is where Eli gets his food.  He is on a special formula that is elemental (no allergins) and predigested.  This helps his body not have to use so much energy to process his food.

Because Eli can't eat he has a special cake that lights up and plays music that he uses for his birthdays! 

On the bottom is Eli's GJ feeding tube hooked up to his formula.  The top is called a Hickman, which is a central line.  This is a special long term IV that goes straight into his heart.  This allows us to give Eli all of his water and some medications and it also allows us to be able to draw labs without having to stick him each time. 

This is Eli with his feeding pump and food bag on the left and his IV pump and fluids on the right.  He keeps all of these items in one large bag that we are able to carry around so that he can move about.  He is on both of these pumps for 18 hrs each day.

These are Eli's feet.  Walking can be hard for him and these special leg braces called AFO's (ankle foot orthotic) that are able to help give him more support while walking.


In closing!  :)  Feel free to visit by blog, especially these next 2 weeks.  Next week I will be posting updates from the school events and daily information about Mito as it pertains to Eli and our family.  The following week I will be posting daily logs of life inside of a Mito family! 

Feel free to look around our blog and enjoy!
Again thank you for being such an amazing community for our family!

 

 

 

Primary Care Doc Appt Update

Today we met with Eli's primary doctor.  We spent 1 1/2 hrs talking thru the most recent changes and going over some of the things that need to be immediately addressed.
1.  We discussed Eli's lovenox.  His levels are a drop over and the dosing is a real struggle.  Right now it's coming in prefilled syringes made for adults.  Eli clearly needs a much smaller dose.  It's rough dosing accurately.  He is going to work with the pharmacist to get it switched over to glass vials to draw ourselves.  He is also going to continue weekly lovenox levels until we are at a safer dosing point.
2.  Eli has been having a few mild nose bleeds but ANY bleeds with a kiddo on blood thinners is scary.  We've had to restart one of Eli's meds for his sleep apnea, but it's a nose spray so this is obviously increasing nose bleed risk.  His doc wants us to try some new drops to help keep his nose over moist. 
3.  We discussed the liver.  The last rounds of lab work didn't look bad.  Still elevated but not near as much.  He is unsure if moving ahead with a liver biopsy at this point is a good idea.  Being so new and still trying to get stabilized on blood thinners is a bit of a concern with a liver biopsy.  We are going to start doing liver blood draws weekly instead of twice a month. 
4.  He has put a cap on how much blood can be drawn from the line weekly.  Everyone has been gung ho to get all of their labs drawn, but Eli is paying the cost.  His hemoglobin is dropping quickly and it's causing alot of energy issues for him. 
5.  We discussed Eli's central mixed sleep apnea.  He is in agreement with the pulmonologist at this point.  Eli will move forward with meeting with the ENT and most likely a plastic surgeon.  Their thoughts are remove his tonsils and adnoids, dialate his nasal passages, place tubes in his ears and do his bronch all under one anesthesia.  Everyone is in agreement that the sleep issue really needs dealt with next on the list.
6.  He is leaving 1 dose of iron a day and will redraw labs in 3 weeks.  Trying to see if we can find the balance of not overloading him with iron, but also keep his ferritin levels up.
7.  We talked kidneys.  He wants to get a firm plan in place before the end of the year as to what surgery and when and all that good stuff.  This surgery has been a long time coming and I'm still no more ready now than I was when we found out about his kidney problems during my 18wk ultrasound.
8.  Lastly we are going to be  making some changes to Eli's hypoglycemia protocol.  This will be our first change since Eli was diagnosed with his adrenal insufficiency and started his daily hydrocortisone.  We are going to begin weaning off the cornstarch.  If Eli struggles maintaining his sugars then we most likely switch his IV fluids from 1/2 NS to D5.  We are going to wearn the cornstarch slowly so we are hoping that will help with no dramatic dumps.

They have put in all of his orders for the med changes, lab changes, and all of those things.   We will get it all transitioned over in the next few days. 
Eli is still struggling thru this cold and we are having to do a decent bit of rescue nebulizer treatments as well as a few extra cough assists.  Luckily hydration hasn't been an issue. 

The older 4 are doing well.  The biggest 2 have been busy as ever fundraising for Eli's Energy for Life team.  They are up to $264 on their own!  They also put together a poster and fundraising project and presented it to their principal today.  She is so excited about the fundraiser and awareness week.  Each class will have a competition collecting coins (and bills) and the winning class wins a pizza party.  They will also have awareness facts each day as well as a Wear Green for Mito awareness day.  The kids are working on a poster to present to the class so they can better understand all of Eli's issues and equipment when they see him.  We are so very proud of them and Eli is so blessed to have siblings who love him unconditionally!!

Today was my eye doc appt.  My eyes are a mess.  Which I was already knowing on my own.  My doc has ordered some special contacts and glasses for me, as well as some new prescription drops.  He warned that it's going to take my eyes awhile to try and adjust but he's hoping to be able to bring me some relief. 
All the adults in the house are still sick with this darned cold as well.  It just seems to hang on. 

I will get more updates up as the week progresses!

Sunday updates

We are home from the hospital, we got home last week. 

Me, Bob, Ben, Lauren (Eli's primary nurse) and Eli are all sick with a cough and cold that just seems to want to hang on.  So that pretty much atleast sums up the adults. 

Emily ~ She's busy as heck.  She's participating in choir 2 mornings a week, silver strings (the extra advanced violin for 6th graders) one morning a week, strings 2 afternoons a week, math club one afternoon a week, and private violin lessons 1 evening a week.  This doesn't even cover all her basic classes and homework.  She stays busy.  She finally saved up enough money to purchase her own phone and now has an Iphone.  She's loving it and we love not having her friends calling or texting us all the time. 

Jace ~ He's doing service club once a month and math club one afternoon a week.  He is looking at doing some gymnastics classes but hasn't fully decided.  He's also in speech and is enjoying his new speech pathologist. 
Benjamin ~ He's doing ok with school although he's having a few issues staying on task.  We had an IEP check in last week and discussed some of the changes we were all thinking needed to be made.  His IEP comes up the end of September so we will have a much more intense talk then.  He's enjoying playing with friends after school and is also going to be starting gymnastics.

Ariana ~ She's loving kindergarten.  She's getting in trouble almost every day though as she simply can't stop talking.  Her teacher is learning the same struggles that we have.  Nothing we have done  has been able to break thru this hurdle with Ari.  The good news is she is loving school.

Elias ~ Like I said he came home last week.  We still aren't where we need to be on his Lovenox levels.  Some of his labs are looking a bit better, some not.  His red blood cells are dialated and they aren't 100% sure why.  His protein is also still low even though we've greatly increased it.  He's doing pretty good hydration wise, so that has been a definite blessing.  We met with his geneticist last week and he went over some of his labs and thoughts and such.  Next month Eli will begin some more genetic testing, that will hopefully get us some more answers.  He also believes something is going on with Eli's liver and is working closely with Eli's GI doc on that.  We also met with Eli's pulmonologist and sleep medicine doc this last week.  He believes that Eli's adenoids are enlarged and thinks that's contributing to Eli's sleep apnea.  He is sending us back to our ENT to have that evaluated.  He wants Eli back on BiPAP but wants to get this issues fixed first.  He put Eli on a nasal spray before bed each night to see if that helps some as well.  Another sleep study is probably in our near future.  Never fun.  Lastly there has been alot of talks about Eli's ferritin, iron, and restless leg.  Eli has been taken off 1 dose of his iron supplement to see if that helps his iron levels any.  Eli's issue has always been that his iron levels are ok but his ferritin is very low.  That's proving harder than expected to supplement.  We are unsure if we will have to stop the other dose as well but for now they are taking it one at a time.
Lastly we learned that Eli has an Alpha 1-antitrypsin deficiency.  Eli has the milder form "M" and "S" and his levels are barely under normal range, but with his history it's something that his doctors want to keep an eye on.  Ultimately if his levels were to continue to drop it's something we could supplement thru his IV. 
Right now we are simply trying to stabilize with all of the new changes, as well as simply getting over this sickness that so many of us have. 

Mitochondrial Disease Awareness Week is right around the corner and we are gearing up for it as well!  Our local mito walk is on the 15th and we are so excited to see everyone again this year!  Our oldest 2 kids have been fundraising away and have raised almost $200 on their own!  We are so very proud of them!!!  Eli's Journey Energy for Life Team  This link will take you to Eli's team page where you can join our team as an in person walker, a virtual walker, and/or make a donation to Eli's team!!


For Mito week I'm going to answer the question we are asked the most.  How do we do it, how do we find enough time in the day.  We are going to walk you thru a week in the life of our Mito family.  We will record our days and nights, and of course will take fun pictures to go along with it all. 

More updates soon!