Saturday, January 18, 2014

VW ... Where the Nightmare Began

This blog is going to be about something so very close to my heart.  If you know me well you know what VW means when I bring it up.  If you do not know me you need to know that to be very close to my heart essentially means that it involves my children.  This particular post is about my daughter to be more specific.  It has been a long arduous journey with my Lexi girl for the past few years ... but the light is actually close to the end of the tunnel and we are on our way there (hopefully).

When my daughter was five years old, and after many rounds of antibiotics and treatments the decision was made to remove her tonsils and adenoids.  Simple procedure essentially, right?  Wrong.  The surgery itself went well.  The recovery went as expected and the release from the hospital was routine.  But ... seven days later was a nightmare in itself.  My beautiful little girl began to hemorrhage out.  Literally losing so much blood her life was in danger.  Long story short, after two in clinic attempts to stop her bleeding she hemorrhaged for the third time in less than 24 hours and one call to the emergency number to reach her surgeon and I was told to get her to the hospital immediately for another surgery.  A few hours and lots of prayer later, she was back in the room recovering.  However, she had lost six units of blood and we had no guarantee she would not repeat what caused her to be back in this bed in front of me.  So, the decision was made to give her a blood transfusion and plasma units to cause her blood to clot long enough so she could heal.  This began nightmare number two.  There needed to be enough blood to draw in order to do a cross reference so that the types of blood could be matched and the transfusion could begin.  This began a four straight hour ordeal for her weak and frail little body.  Her veins are low, tiny, roll and blow ... she simply was not blessed with my veins.  Her body was weak.  She was dehydrated and she was low on blood.  So, this literally turned into six different medical staff, seventeen holes and 32 attempts to draw blood out of her little body.  The final attempt was by the anesthesist and was into her groin for a straight hour.  At the moment he was giving up to give her body a break he whispered "Don't move, baby, I just got a flash."  This turned into a moment of tears and relief for all in the room.  You see, in that moment I had my arms on her legs and my head on my arms praying.  Facing down.  My dad was at her side holding her hand and my mom and her surgeon were at her feet rubbing on her feet.  There was the anesthesist and there were two lab techs that were in the room.  But what I didn't know until I lifted my head to see her smiling face was that there were also three nurses and two admin clerks in the room.  All had a hand on the person in front of them and were praying for my baby girl.  Very emotional moment that helped her heal and save her life and keep her here with me longer.


This is her face when the transfusion started.  All smiles, little tiny body, weak and dimples shining.  Those dimples literally light up my soul.  Always have.  Always will.

Fast forward the next four and a half years of massive nose bleeds, large deep bruising that is unexplained, two concussions within a year, what was believed to be an allergic reaction to mosquitos, more massive nose bleeds (literally an hour at a time saturating a small towel) and major headaches and lethargic weekends.  I say weekends because you have to know my little warrior to know that she is a total fighter.  She never shows weakness and puts 150% into whatever it is she does.  You see, five months after her fight for her life it was discovered she has Celiac Disease.  This means that she cannot have glutens, wheat, whey proteins, barley, casein, lacto-albunin, cows milk or chicken eggs.  She cannot have any foods, seasonings, drinks, sauces, etc that have any of these things in them.  Want to do your research go here:  http://www.celiaccentral.org/Celiac-Disease/21/

Now, my angel baby is ten and a half years old and still dealing with all of these things for the past four years.  She was hospitalized in December 2012 for what was believed to be her appendix, but what turned out to be severe ovarian cysts (on a then nine year old girl).  This compounded all of the other health problems she had been having.  Fast forward again through the following November 2013 and on into this month, January 2014 ... she has seen a total of 19 doctors in that twelve month period.  NINETEEN!  But, thankfully we have an aggressive family physician who got her into the right channels and we ended up at Cook's Children's Hospital.  The hematologist tested her and we got our first answer (but mind you it was the third time she had been tested for this) ... VW aka Von Willebrand's Disease.  Want to do your research go here:  https://www.nhlbi.nih.gov/health/health-topics/topics/vwd/

However, I can tell you in a nutshell that it is a blood disease that is genetic and hereditary (just like her Celiac Disease) and it is not contagious.  It is simply a disorder in her body that means she cannot clot and stop bleeding properly.  There are three different stages to VW and she is luckily stage 1.  However, the statistics of this mean that there are 1% of the United States population that has this disease and I was told when we got her positive result that only 20% of patients respond to treatment.  WOW, right?!  Just wow.  So, we moved forward into another round of testing, to see if she responded to the Stimate medication, which is a nasal spray.  What this spray does is tricks her body.  Lexi being a stage 1 patient means that her body produces the VW protein in her body, but it does not dump it into her blood supply.  So, the Stimate will trigger her body into dumping these proteins into her blood supply to reduce bleeding.  It is important to realize that this medication DOES NOT cease bleeding and DOES NOT prevent bleeding.  It simply reduces the quantity of blood she loses until her body can play catch up or until she can receive medical attention if necessary.  So ... we begin the treatment which includes more blood drawn (remember how hard a stick she is?) and an IV of the Stimate for thirty minutes and then a wait for an hour and then more blood drawn.  (I want to mention this is not one or two tubes of blood, it is eight tubes of blood and her lucky spot is in her wrist if you want to attempt getting blood without digging up her arms all day long.  If you have had your blood drawn in your wrist you know how this feels, if you haven't consider yourself blessed and say a thankful prayer right now before you continue reading.)  Now this Stimate has some pretty severe side effects that are possible.  They can cause water intoxication.  This means that the medication causes your body to store fluids and if you drink too many fluids within the few hours of having one nasal spray you can have a seizure.  Imagine my concern when she had this pumped through her body for thirty straight minutes.  




So ... we head home after this round and then four days later end up at an additional specialist, and endocrinologist.  Another brilliantly relatable specialist that we are thankful for.  However, this requires my girl to be hospitalized for a twenty-four hour period of treatments and more tests.  Which means more needles, more bruises and more side effects.  This involved one medication every four hours for ten minutes and two other medications every six hours and eight hours.  This also involved more testing on her blood and ANOTHER round of Stimate through an IV which means a repeat of the water intoxication risk that causes seizures.



 My girl is a WARRIOR.  Have I mentioned this?  Well, in case you missed that earlier up this page ... she is.  Totally.  Without doubt.  My hero.  My fighter.  My angel on this Earth.  She came through this twenty four hour period with a decrease in dimples but still a positive attitude and the ability to kick my butt in a game of Yahtzee.  Did I mention the good news we got before we left this hospital stay?  The call that her body responds to Stimate.  Yup, worth repeating.  SHE RESPONDS TO STIMATE!  My daughter is within the 20% of people who can be treated.  Thank you, Jesus and for continually taking good care of Your Girls.

 This does not mean she can be cured.  This does not mean she will not bleed out.  This does not mean she is low risk.  This means that we can head off as best we possibly can any major issues that can take her life.  That is a window of opportunity that I will take any day of any week of any month of any year.  This medicine will help her at home if she is bruised badly, if she has dental work done, if she gets a nose bleed, etc.  You get the idea.  However, if she ever has major head trauma we have to go to the hospital IMMEDIATELY.  If she ever has surgery she has to have a blood transfusion and plasma before and after the procedure and an extended stay to ensure she heals before heading home.  This means that VW is something she will monitor and treat for the rest of her life.  Forever.

Through all of this, what is her outlook?  That everything going on in her little body is genetic and that means there is a Perfect Storm going on inside of her.  Her body may be weak in some areas but her mind is sharp and her soul is pure and her heart is big and her God is BIGGER.

Lexi girl wants to attend Harvard University and become a Medical Examiner.  Why?  She wants to go to the best school for the best education and she wants to give people answers.  My daughter has a servants heart.  Well, a servants heart and dimples that will stop traffic.

 
What is next?  We continue monitoring her on her new medications (yes there are more than one).  We have regular testing and we continue to watch her for side effects.

AND ... we get her little brother tested.  Yep, you guessed it.  It is genetic remember?  If one child has it the odds of another child having it increase.  Head trauma is an absolute NO NO with VW.  If he has it, no football.  So ... boys will be boys and they play rough.  So, fingers crossed and prayers up that little man is VW Disease free.







 

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