Tuesday, November 24, 2015

Low lymphocyte level and IgG levels

William had his 9-month post-transplant blood panel last week. The studies show that William's lymphocyte subset panel was lower (a good bit lower actually) than it was when he left in August. Meaning that, based on these results, William's immune system isn't quite up to par yet. His BMT (Bone Marrow Transplant) Team in Pittsburgh believe those results may be incorrect as low as they are. They would actually like us to have that blood panel repeated in addition to trying to get another blood test, which takes a bit more blood and with William and his chunky (and oh so cute) arms, its hard for nurses to find a vein. Usually, William has to have an ultrasound in order to find a good vein for large blood draws (more than 5 mL) or to have an IV. For small blood draws where they check electrolytes, white blood cell counts, etc, what we usually get when we go to the doctor, they can do a finger prick. But not with these tests; the blood needs to be pulled from a vein. Which leads us to our next endeavor:
William's IgG level was borderline low. IgG protects the body from infection. To “boost” this level, a dose of IVIG needs to be given to help keep William healthy throughout the Winter season. IVIG (Intravenous immunoglobulin) is a blood product administered intravenously. He's had a number of these infusions while in Pittsburgh. It contains the pooled, polyvalent, IgG antibodies extracted from the plasma of over 1,000 blood donors. IVIG's effects last between 2 weeks and 3 months and will get him through until our Pittsburgh visit in January. IVIG is EXTREMELY expensive and our insurance has covered this in the past. We are now waiting for our insurance carrier to preauthorize the IVIG treatment and once they do, we will have the infusion done about 30 minutes from where we live. We won’t have to drive all the way to Salt Lake City for that procedure thankfully.
We are also waiting on another preauthorization to get William’s EEG done. Because of William’s seizure scare he had a few weeks ago (which may or may not had been a seizure), his Krabbe doctor wants him to have a 48-hour EEG monitor to check for any seizure activity so she can have that information for our visit in Pittsburgh. An EEG is a test that measures and records the electrical activity of your brain by using sensors (electrodes) attached to your head.
Finally, our flights for our return visit to Pittsburgh are set for January 11. This will be his 1-year post transplant testing and will involve a lot of testing, everything from a lumbar puncture, MRI, vision and hearing test, etc. Testing will last about 3 days. So much for just a little boy.
I hope that all made sense. I had to talk to his doctor at length this morning to explain on the blood work. I just hope it doesn’t take long to get the preauthorization for those procedures. Our insurance has been very good, but because his levels are “kind of low”, and it “may had been a seizure”, sometimes it is difficult for insurance companies to pay for such procedures.
Other than that, William is doing quite well. He is comfortable, smiling, not showing any signs of discomfort, and very alert. As for his Mama, I worry all the time!
Much love, Abbey

Monday, November 16, 2015

how to DEMAND Krabbe be added to your states newborn screen

Want Krabbe added to your state's newborn screen?  Let your state representatives know by following this link.  It will take you 5 minutes tops.

Supplemental newborn screen

Are you or is someone you love expecting?  Ensure your baby has the best chance at a healthy start to life through Supplemental Newborn Screening.  Order your kit today no matter what state you live in!  For $50-$100, depending on what you would like tested for, its worth it.  If only we knew about this simple test.

For all parents, especially parents to be

All of our babies were screened at birth for diseases by a simple heel prick. Each state has their own unique list of diseases they screen for. But did you know that there are NO STATES that include all possible potentially fatal, but treatable, diseases? Only a few states screen for Krabbe, is your state one of them?
Find out what your state screens for by clicking the following link:

First seizure :(

I have some sad news. Although William is doing okay now, he had his very first seizure this morning. It is considered to be a partial or focal seizure. This is brand new territory for us as this was his very first so I have a lot of learning to do. What I do know, after speaking with his nurse, is that there isn't any interventions for a partial seizure, as they remain alert (part of Krabbe Disease progression we cannot control). What it looked like to us was his eyes were rolling up and down, not quickly, kind of like when you're watching someone watch the credits of a movie. It lasted about 30 seconds and both Robert Branch and I were there to talk to him. We believe the trigger was me opening his bedroom door (the sound startled him), as he cried out and then the seizure began. William didn't look scared or anxious, but did look tired afterwards (although he gave Mama a smile when I asked him if he was okay). He's the sweetest little boy I have ever met and I will never understand why this is happening to him. Yes, he was transplanted but also at a point that his body will continue to fail, rate unknown although much slower than had he not been transplanted.
As this is an isolated incident, no action will be taken (as we were directed). He is already on seizure prevention medication, and that obviously will be titrated as needed, secondary affect will be lethargy. If another happens before our trip to Pittsburgh in January or so (we've almost hit our goal to get there, thank you for those that have donated!) We will see his neurologist in Salt Lake City for an EEG.
I am terrified. Please think of our little one and hope this was something that is not going to become the "normal" for him. He's been doing so well.

Wednesday, November 4, 2015

Please join Williams facebook page


1st head start visit

William and I met with his Early Head Start teacher today.  I had met her previously, but that was the first time William and Vanita OBrien met.  I think he is going to love her.  Carolyn Branch was holding him, and when Vanita was talking to him he kept looking back at Carolyn because he wasn't too sure yet (as he does with new faces).  But after a few minutes, he was making eye contact with her and sucking and moving his lips, all things he does when he feels safe and comfortable (or getting what he wants.)  Miss Vanita also brought him new toys to play with and they are just perfect for him.  She has a huge heart and I look forward to collaborating ideas with her.

I was apprehensive about him being ready to start "school", but I couldn't put together a more perfect combination: home visits, a great, wonderful, caring teacher, lots of new resources, and a stimulating environment for our little William.

Besides starting swim lessons, we will now be taking advantage of our local library and their toddler story time.  William loves books and being read to.  Anything with bright colors, music and anything tactile he can feel with his hands he loves.

We also have speech tonight.  We are working on prerequisite communication skills to eventually try out some communication devices.  He's almost 18 months old and is communicating quite well, I think!  He sure tries!

We are also getting some visitors tonight! Uncle William Branch, Aunt Angela Rankin Branch and family will be here soon.  William loves his little girl cousins and I love seeing how well they play together.

As for me, I feel like a got hit in the nose with a softball (because I know how that feels), just being a few days post septoplasty.  No bruising, but lots of puffiness.  I look AMAZING! 😤 So glad I can lay down a little bit with Grandma Carolyn taking care of William.

Much love from Utah.  Thank you for pulling for our little guy.  He needs a lot of support, and prayers, and love!

William got accepted into Early Head Start!

William got accepted into the Early Head Start Program! Head Start is an amazing program that will help support his mental, social, and emotional development until he turns 3, and then he may transfer to Head Start or another pre-K program.  It also is a way for Robert and I to meet some other families in our community.  We are very fortunate as most of the time this program is for lower-income families, but sometimes they make exceptions.

We are very excited for this opportunity!

With the Early Head Start program, an early childhood educator will come into the home with supplies and ideas.  We are not sure of frequency, but I believe it will be at least weekly.  I've already met the teacher and she is great!  I believe it starts next week!  There will also be group meetings with parents and their children monthly if not twice monthly, and again, I'm excited to meet other families.

As always, we are setting the bar high for William.  Although he has severe physical limitations, he is proving every day that he is eager to learn and communicate!

Head Start is separate than the early intervention program he is currently enrolled in.  Within the early intervention program, he gets at home physical, occupational, speech therapy and also baby massage. 💙