Can't we just be kind??

To the judgmental lady in Walmart today:
Do you know that my little girl, (the one you referred to as an ungodly brat) is a medical kiddo with severe sensory issues?  Do you know that we had just been to therapy and that being in Walmart (or any store/public place for that matter) is completely overwhelming to her?  Do you know that she doesn't have enough vocabulary to let me know that she's completely overwhelmed and can't handle the situation around her?  No, you don't.  Do you know that I pushed her too far by simply taking her in the store?  Do you care if she's okay or for that matter if I'm okay after you watched her slap me so hard it instantly brought me to tears in the checkout line?  Do you know that I am a mother to 7 children?  You know, me, the same woman you said should never have kids because my child slapped me.... I know you didn't say it directly to me but you definitely said it loud enough for me to hear that my child's behavior and my reaction to it were not acceptable to you.  I wish I had the patience to stand and explain why that even happened but today we are tired so I simply turned and through tears and told you to "Shut the fuck up and mind your own business"  I also told you that you shouldn't judge what you don't know and that I would give my life for this child and that if you had nothing nice to say than maybe you should simply keep your mouth shut.  I also should have told you that we have 17 doctors, 8 therapists, an EA and more nurses than I can count all trying to help with the situation otherwise known as our life and they are much more qualified than you to help so just shush.

It's not often that I encounter someone that loud or outright mean about Madilyn.  Typically I would just smile and move on but the past few weeks have been exhausting and I just couldn't today....

Please please please think twice before you openly be so harsh to someone... especially when you have no idea what they are dealing with... Kindness is completely free....

Changes changes changes

Boy life has been crazy here!  So many changes.. Since her adrenal insufficiency diagnosis Madilyn is doing pretty good. She's making HUGE progress at therapy in all areas and it's awesome awesome awesome to hear all of her little words coming back!  (even if some of them are sassy..)

We had our 3rd shunt malfunction in early April... they scare the sh*t out of me!  She recovered beautifully though without any complications.  The downside is that her risk of another malfunction is 20% higher for the next year because they've been in her head messing around with it... ugh.. our only other 2 malfunctions were 6 months apart in 2014.  I can't explain the overwhelming feeling of fear that takes over knowing that a doctor is messing around in your child's brain, it's not something I will ever be okay with.

Seasonal allergies are wreaking havoc on the Peanugga right now and causing feeding issues, (at least we hope that's all it is) that may land us inpatient briefly if we can't get her gut to cooperate... ugh

We've recently had a formula change to attempt to get more calories in her.  Last summer Madilyn was up to 36 pounds and right now she's only 31.  With being so sick prior to the adrenal diagnosis last fall she lost a bunch of weight and we are unable to get it back on her.  At least the problem now is because she's so active!  Her GI doc put her on a higher calorie formula so we actually get to decrease the volume but she gets more calories and hopefully some weight gain.

Now the fun stuff!  Madilyn has a new sidekick...:  Meet Goose!

Goose is a purebred standard poodle and is Madilyn's best friend and service dog in training.  We are currently almost done with our first obedience class and are working with a local training agency and the C.A.R.E Dog Coalition to do all of our training.  Goose has already started coming to her therapies with us and when we go to Children's for appointments on June 5th he will be coming with us.  Madilyn gets so stressed out with the medical stuff that Goose is already being trained in deep pressure therapy (he literally sits on/hugs her) and it's very calming for her.  She can go from crying an puking to giggling within a minute or two thanks to Goose!  The bond between these two is absolutely amazing to see.

I'm procrastinating as usual on the school thing... we need to tour a local special needs program and see how willing to work with us they are.  I wish transition wasn't so hard for her (and us)... I think we may both need some new little pink shoes for that one....

Adrenal Insufficiency sucks

AI would suck enough if you could talk and explain exactly what you feel to someone... AI in a child who can talk would suck more... AI in a non-verbal, cognitively impaired child is horrible.  It's one giant guessing game about how she feels.  Up dose a little to much and she's wound tight, under dose a little or try to wean and she's aggressive and the vomit reappears.   UGH!!

Her endocrine doctor wanted us to wean back a little on her daily dosing because it's technically at an adult dose but it's apparently what her little body needs right now.  One minuscule little decrease and the big circles around her eyes are back, the vomiting is back and she's sleeping horribly.  We'll up dose again for a while and try again.  I feel like the little engine that could sometimes, constantly chanting to myself "I think I can, I think I can".  It really sucks sometimes knowing that how she feels and is able to function is all dependent on me being able to read her needs since she is unable to tell me.  I know I have big shoulders but it's really heavy sometimes.... especially at 6 am when she's kicking my ass and being an aggressive little monster and I don't know how to help her.  This morning it consisted of her getting baby gated into the play room.  She clocked me in the face and I just needed to separate myself.  She was able to sit in there and throw her toys everywhere and yell (I'm pretty sure our neighbors think we are lunatics) for over an hour until her steroids peaked and she finally calmed down. *Note the completely trashed room below

We tried going back to school because Madilyn loves to be outside the house but it didn't work.  She gets so overwhelmed just pulling up there.  The school was willing to work with us this fall but with this attempt to go back they were "willing to work with us" but not really.  Not in any way that is appropriate for Madilyn or her disease. She scares them and they could no longer hide that.  I get it, she used to scare me too (hahaha)... she's not obligated to be in school until fall so we'll see then if it's in school or home based, either way I refuse to ever force another person (teacher or otherwise) to be responsible for my child when they are uncomfortable with her.  Her life depends on their ability to care for her.  In the meantime we are impatiently waiting for the call from the rehab facility to start outpatient therapies.  Physical, occupational, vision and speech... I think the regular weekly outings will be good for us.  They're working on aqua therapy too but we may have to travel to Milwaukee weekly for that one.  I think it would be worth it though so we'll see what they decide.

We are definitely enjoying that since our move we are able to get out in the stroller at this time of year for walks.  It's so peaceful to go down by the lake and being outside anywhere is Madilyn's favorite thing to do. Here's some pictures from yesterday's stroll:

Time for me to stop hiding since the lovable little miss has decided to finally surface.  It's time to build some puzzles and maybe shop for some new little pink shoes....

True Fears

I really have no words to explain what happened tonight.  We dropped Shyanne of at driver's education and barely made it around the corner and the vomit started...she was mad cause Shyanne got out of the car??  maybe??  she was upset cause it was dark outside and her DVD wasn't turned on??  carsick??  I don't have a clue but oh the vomit... the thick, choking vomit... she can't breathe, she can't get it out and I'm caught in traffic trying desperately to find a place to stop....

I stopped right in the middle of North Ave....half in traffic half out...it's snowing and slippery and there's so much traffic by the school it's chaos... I pulled over 3 times in less than 2 minutes...we came home and scared the daylights out of my hubby who's upstairs sick.. we were supposed to be gone to Walmart to pick up some crackers, 7 up and a few other things and instead we're back in the door 10 minutes after we left with Madilyn yelling her head off at me and me sobbing, yelling, begging her to stop puking.

Madilyn dying from choking to death while I'm driving and stuck in traffic is one of my biggest fears... I've watched her turn blue right in front of me when we were in the house...when the puking starts and I'm in the car alone with her panic ensues.... I try to keep my calm the best I can but tonight I lost it... I drove home the last few blocks sobbing, yelling and begging her to stop..... it makes me never want to leave the house alone with her again.... I will, because I have to but it leaves me with this pit of fear in my gut that makes me nauseous and I somewhat hold my breath just waiting....

I've always been a deal with it and move on kind of person...a don't dwell on it kind of person... I still don't dwell but the PTSD doesn't always let me move on either.....

The truth behind the new diagnosis

We've been quiet again... for a really long time...and here's why:  I don't know how to put into words that my heart and head have been telling me since last December that we were slowly watching Madilyn being taken from us.  Something was brewing and slowly taking over her little body and she was slowly fading away.  It's devastating and beyond any words that I had/have to explain what it's like to watch your child dying.  I gave only the little glimpses on Facebook of the good moments.  I didn't let new people in and shut out the others.  We moved to another state to be closer to her doctors in late July and progressively watched her deteriorate.  My child that LOVED people and shopping and busy places couldn't leave the house without major major anxiety attacks.  Walks in the stroller became our only out.  No loud noises, limited people and only outside or in our own home.  Even in our own home at times it was too much for her.  She became super aggressive and I started getting questioned by doctors and others about bruises and other marks on me, I would burst into tears any given moment and even went on antidepressants because it was so overwhelming.  She stopped sleeping, staying up for days at a time and when she did give in it was only for an hour or two... it was like living in hell.... a hell you don't want anyone else to know about simply because it's too hard to deal with the constant pity and questions.  You start to feel a little ridiculous constantly repeating "I don't know" when asked the reason for her decline.

In early September Madilyn ended up sick with croup... after that Decadron injection at the hospital it was a week or so of heaven.  She was sick but I could see Madilyn... my happy, non-aggressive, loving life child... which was weird because she was so sick.... fast forward 3 weeks and we end up inpatient.  Madilyn would have an anxiety attack at school or an outing and not tolerate her feeds, it would start vomiting and I couldn't transition her back.  It happened again the weekend before Halloween.   We had been struggling desperately to try to get back on track since she got sick in September and just couldn't do it.  I broke down to our Special Needs medical team that something was horribly wrong and that her doctor's were missing something.  It was the first time I spoke the words that no parent ever wants to speak.  I told them that my gut (and my mommy heart) told me that she was dying, slowly, painfully and all because they were missing something. (Not them directly but her entire medical team).  At this inpatient stay she was diagnosed with Cyclical Vomiting Syndrome.... I loudly disagreed with this diagnosis from the moment it was given... more meds were added and I argued that all they did was mask whatever the real problem was and they were wrong.... my shouts fell on deaf ears.... we were sent home.

After a day or two of being home Madilyn was the worst she's ever been and I posted pictures of her on Facebook... I gave the world a glimpse of just how sick she was and that glimpse saved her life.  A friend (and medical mom) messaged me and started asking questions... then I heard those words.. Adrenal Insufficiency.  I knew Madilyn was in a really really bad place and we had steroids here since we regularly use them for her respiratory issues.  Steroids are what is needed with adrenal insufficiency, I gave them to her.  Within hours it was like having a completely different child.  She went from completely lethargic and hazy to up and wanting attention, she gave us a glimpse of Madilyn.....the next morning I messaged our Care team demanding testing for Adrenal insufficiency... the day after that I messaged and calmly explained why I wanted it.... they contacted our Endocrine Dr. and the first test was scheduled.....when the Dr called with those results she said they were okay for a normal situation... then I explained that when the labs were drawn Madilyn was hysterical... to the point of vomiting everywhere hysterical... that's when she scheduled test #2, which Madilyn failed miserably.

The stim test that gave us our Adrenal Insufficiency diagnosis also pushed Madilyn's little body too far and we ended up inpatient.  It's amazing how quickly we could watch Madilyn reappear right before our eyes super quickly with the introduction of hydrocortisone on a routine basis.  Adrenal Insufficiency is the lack of the body's ability to produce cortisol, a steroid, that you cannot live without.  Every time you are sick, stressed, injured etc your cortisol production increases to combat whatever is going on.  Madilyn's body doesn't do that.  Her Endocrine very blatantly stated that Madilyn was only still alive because "for some reason or another some doctor has always thrown steroids at her when she's been sick and it's the only thing that has kept her alive".

Adrenal Insufficiency can be fatal and that's the first thing they repeat to you over and over... Madilyn now wears a medical ID bracelet and we carry an emergency injection with us everywhere we go.  She also receives oral steroids 3 times per day, every day and more when she's sick to compensate for what her body does not do naturally.

Adrenal Insufficiency actually gave us answers to every single unanswerable medical issue Madilyn has had since our very first med flight in February 2013.  It explains the vomit, feed intolerance, sleep issues, anxiety, aggression, her regression (it causes memory issues and brain fog), her hypoglycemia, the circles around her eyes...ABSOLUTELY EVERYTHING!  This diagnosis and proper treatment have given us our life back!  For the first time in years I feel like we can breathe... We have been out shopping... out to dinner at a super busy restaurant on a Friday evening and Madilyn has done awesome!  It shocks me how quickly steroid replacement therapy has given us our child back.



We have a lot to learn and when she gets sick we will need to be inpatient for IV help but that's ok, that's totally livable... for the first time in a long time we are starting to plan ahead a little... we are excited to return to school and to educated as we learn about Adrenal Insufficiency and how to live with it.  I'm thankful to my friend for reaching out to me and giving my child her life back.. I'm thankful that I can come back to the blog and keep you all updated on the sassy shenanigans of the Peanugga...  and I'm thankful that her medical ID bracelet is pink... just like her new pink shoes..:)

Here's to Reality

Did you ever leave your 4 year old in a room alone for a minute or two and think... oh, they'll be fine?  I did it with my typical children all the time and guess what?   They were fine.  I had somehow convinced myself that we were doing just fine.  In spite of two emergency medical flights in 4 months and all of the escalating "issues" we've been dealing with.  I let myself slip into this nice little zone thinking "we're just fine" and then I walked into my living room last night after only being around the corner in the very open kitchen for less than 2 minutes.  I walked in to my living room to find my child turning purple and choking.  She couldn't (can't) get the "junk" out of her chest...In a moment of panic and hysteria I managed to get her up and flipped over and started hitting her back hoping it would help...I yelled (probably more like hysterically screamed) for a burp blanket (she vomits so much we use receiving blankets instead of burp cloths) and I think I yelled she's purple.  The moment seemed to last forever and my husband had to help but we succeeded and the Peanugga snuggled right into my lap completely exhausted while daddy did her breathing treatments and I sobbed my heart out while clinging to her.  Barely four hours later it happened again....and then again this morning.   This is our new reality....and once again the doctors have no idea what's causing it.  

When our journey started all I kept telling myself is it will get easier as she gets older.  I convinced myself it would get better.  In many ways it is great... she's thriving and growing and learning new skills every single day and on the flip side of that always seems to come another I don't know and a new diagnosis to add to our never ending list.  This time it was the fancy term of Neuromuscular Respiratory Weakness... it came along with an order to our DME for suction equipment so incidents like last night and this morning are a little easier to deal with.  Now we are in the middle of the waiting game of insurance approvals and DME shipments and then we have to actually learn how to use the equipment.  In the meantime I pray to a God that I'm not sure I even believe in to not take my child from me... again.

I never thought I would be willing or need to make a deal with the devil to keep my child here but I've done it.  I've done it multiple times now.  Every time another one of our "friends" has to say goodbye to their child my heart breaks and my anxiety level rises another inch.  I never thought when this journey started that PTSD would apply to me, boy was I wrong.  

I never thought that at 4.5 years old I would be contemplating moving her bed back into our room because I'm terrified to close my eyes at night.  I do have a video monitor but now I'm seriously questioning if her room would make a nice dressing room/walk in closet.  

We always try so hard to stay positive...we deal with the bad stuff in the moment or with the new diagnosis and move on.  We adjust our routines and live in the moment.  We try not to think about yesterday and we don't plan for the future because every moment we have matters.  Every single moment in our life with Madilyn is a gift and not one that came with a guarantee either.  If we are planning for a future or dwelling on yesterday we are wasting precious time that matters....

When I started this blog it was to raise awareness for what (multiple) rare disease looks like.  It was therapy for my soul.  It still is but I find that I will write a post and never hit the post button because I don't want to hear the "I'm so sorry".  There is nothing in our life no matter what is going on at the moment for anyone to be/feel sorry for.  I (we) get the gift of loving a beautiful little girl that has taught me more about life and love than I ever thought possible.  She will continue to do that for however long the powers that be decide we get to keep her.  Her little giggle is contagious...we waste more tinfoil and paper to hear it than I ever thought possible.  She gives me the best excuse ever not to do the dishes or sweep the floor when she crawls over, tugs on my pants and says "momma up" with her arms in the air.  I've learned that whatever it is can wait.

  I am not sorry and you shouldn't be either.  I am hopeful every single day that we have a very long and love filled road ahead of us and I can guarantee it will be very full of little pink shoes.

My Hydrocephalus sweetheart!

Hey everybody..sorry we weren't around this summer but it was summer and ours was crazy!  With summer typically comes camping, bonfires and lots of beach days, but not for us.  We do try to enjoy a bonfire or two but usually only one beach day.  Madilyn has Hydrocephalus caused by her Dandy-Walker malformation.  It's excessive build up of spinal fluid in her brain.  Her ventricles do not work properly so they placed a VP shunt to regulate the fluid when she was around 5 months old.  At that point her head was growing 5 times faster than her body and the excess fluid was starting to literally crush her brain.  The shunt is amazing and traumatizing all in the same moment.  We are thankful for the relief it brings and traumatized by what happens when it malfunctions.

Our first shunt malfunction was in March of 2014.  Madilyn woke up from a nap (she never naps...first clue something was wrong) and her eyes just didn't look right to me. They seemed really puffy and they did a ton of bouncing which wasn't normal.  After talking with my husband I took her to our local emergency room and a few hours later we were on a med flight to our Children's hospital. By the time we arrived at Children's Madilyn had begun to twitch on her left side and had lost all strength.  I could not keep her awake or rouse her.  Her shunt series x-rays appeared normal to the doctor that looked at them and he was ready to send us home.  I ended up screaming in the emergency room at the doctor to do something because my child was dying and they were doing nothing.  I've never been so scared and angry in my entire life.  The doctor agreed to order a quick MRI to keep me quiet and proceeded to inform me that the results could take quite a while and then we would be discharged.  Thirty minutes later as I'm sitting in a chair with Madilyn begging her to hang on and not leave me the same doctor came running into the room apologizing and telling me that the surgery team was coming to get us.  It showed up on the MRI that the catheter part of her shunt had dislodged from ventricle and the fluid was building so fast it was crushing her brain and killing her.  I was shocked after surgery at how quickly she bounced back.  The very next morning she was up and playful and ready to go, even with approximately 20 stitches and a 2 inch gash in her skull where they had just been digging around in her brain 16 hours prior.

Our second shunt malfunction happened way to quickly after the first.  September 25, 2014, barely 6 months and Madilyn took an excessively long nap, was extremely hard to wake up, the bouncing eyes were back and the vomit started.  Off to the ER again for a med flight we went.  Thankfully this one didn't get quite as extreme as the first one but the recovery was much harder for her and we were inpatient for an entire week.

I am absolutely terrified of another shunt malfunction, I have no problem admitting that.  I can't really put into words how traumatic it is to go through.  I think I prayed to everything I could think of and probably would've made a deal with the devil in those moments to not take my sweet child.  It terrifies me to know it can and will happen again.  It's life with Hydrocephalus.  It's life when you rely on a piece of equipment to do what the body can't.  I'm so thankful for that equipment and our neurosurgeons and at the same time out of all of her diagnosis Hydrocephalus is probably the one I hate the most.

Back to summer with Hydrocephalus....heat and humidity = boatloads of vomit and screaming.  Screaming while holding her head and rocking back and forth on the floor, all while vomiting. This is what happens when heat and humidity raise the pressure levels in her head.  It's so difficult to watch...I can't touch her, she's sound/light sensitive during these episodes.  I can only sit nearby and whisper soothingly to her to let her know that I am there.  She lets me know she needs me to stay right there.  Quietly, patiently just be there.  It's horrific to watch.  I tend to sit and cry with her as there is nothing else I can do.

We hide during the summer, in our house with curtains drawn and air conditioners running in hopes of avoiding the trauma.  Sometimes it works and sometimes it doesn't.  An adult with Hydrocephalus described to me what it felt like to have this happen to him,  imagine your worst possible migraine (I suffer from migraines so this was not hard to imagine) and add some extra vice grips to it....UGH!  I have other words for it but I won't pepper the blog with my foul mouth.

I'm focusing on Madilyn's Hydrocephalus because September is Hydrocephalus Awareness Month!  We are the blue that gets lost in the gold in awareness raising in September.  Don't get me wrong, I'm all for the gold awareness, I just wish there was a little more blue!  Neither one has enough funds for research and neither one has made any advancements in treatment.  The whole thing is sad and scary especially for me because Madilyn has chromosome abnormalities that put her at 3 times higher risk for pediatric cancer ( I won't even go into that fear).........

On top of the Hydrocephalus daily dealings we were inpatient for 4 days in June for her sugar testing which did lead to answers but not easily.  Madilyn has severe hypoglycemia and is not on continuous feeds into her intestines because her stomach does not tolerate them.  We do constant sugar monitoring and all new protocol has to be put into place for any procedures she has done as she cannot be without her continuous feed or D10 IV fluid without causing life threatening issues. We are adjusting to it, it's our new normal.  Thankfully my parents bought her the cutest little Minnie Mouse backpack for her feeding pump so it's functional and adorable and of course it's pink.  We also started wearing foot braces to help with support for hopeful walking, her wheelchair (also pink) will be here in the next few weeks, her theratogs (leg wraps) are in and we pick up her hearing aid (also pink...haha) in a few weeks at our appointments in Milwaukee. We also have to see Cardiology for the first time since the NICU for a complete exam and some tests.  Madilyn's heart rate likes to be full speed ahead and her heart is enlarged so we just need to make sure everything is okay.  Life has been crazy like I said but if she can do it and smile every day so can I.  The new pink shoes help too...after all, life is all about the little pink shoes..:)

The picture is from Madilyn's first shunt revision in March 2014.  The second picture is because she is just too cute for words in spite of everything she goes through.