Category: Krabbe

Q&A Part One: Krabbe Disease, Stem Cells, and Treatment

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We asked our Team Tori followers if they had any questions we could answer for them about Krabbe, Tori, etc. and such great questions were presented! I tried to include as many links to respected resources as I could for the medical questions. I also condensed similar questions to avoid repetition.

As I receive more related questions I will add them here.

Because there were so many questions, I have split them up based on category and made several blog posts out of them. 🙂

Krabbe Disease

This is the information our neurologist gave us when she told us the official diagnosis. It is a great resource about Krabbe. The Hunter’s Hope Foundation also has a great page with information about Krabbe that is very well-written and easy to understand.

  1. What was the process for diagnosing her? What were her initial symptoms? How old was she when she was diagnosed?
    Tori was diagnosed when she was 6.5 months old. She started showing symptoms at 5 months. I detailed the progression of symptoms in this post. Our local paper also wrote about her here.
  2. If my child was born in a state that did not do newborn testing, can I still have her tested?
    Yes! Anyone can be tested through a genetics doctor. You can also purchase a kit and take it to the hospital to be used at birth.
  3. Can you tell if Tori can still hear and see you?
    We know that she can still hear because her startle reflex is still excellent and she will jump at little noises still. We believe that she can still see us but we’re not sure how well. The ophthalmologist believes that her vision is low because of the testing results and the MRI images. She is believed to have Cortical Visual Impairment. However, she will often make eye contact with us, and she definitely can see light (based on how she stares at her giraffe).
  4. How do they determine when she has entered stage 4?
    According to other Krabbe families, loss of body temperature regulation, loss of hearing, increased sleepiness.
  5. Where is she getting treatment?
    Tori is under the care of Dr. Escolar at the NDRD of the Children’s Hospital of Pittsburgh as well as many physicians here in Hershey. Dr. Escolar is one of the leading experts in the disease and has seen over 130 Krabbe babies/children to date. You can read more about the NDRD here.
  6. What does the breast milk help? I know easier digestion, but anything else?
    We chose to keep her on breast milk because of the immunity benefits as well as the nutrition. It doesn’t help the disease in any way, as far as I know, but it has been far easier on Tori’s stomach than the formula they had her on in the hospital. We have been so blessed with donor milk for her!
  7. Can Krabbe cause Failure to Thrive?
    Yes. Krabbe babies have a difficult absorbing fat – Tori included. At diagnosis her body was in the 2nd percentile while her head was in the 97th. We have to fortify her breast milk with MCT oil, which is an easily absorbed fat, to help her gain weight.
  8. I understand the brain will eventually lose control of life sustainable abilities; do they need oxygen later as it progresses?
    Yes, from what we understand.
  9. Is there a national network for Krabbe with a support group?
    There are several that are excellent. Hunter’s Hope and Judson’s Legacy are two major non-profits that help Krabbe families, and there is also a Facebook group for Krabbe Families that has been a tremendous source of support for us.
  10. What is her muscle usage like? Can she move her arms and legs anymore? Will she eventually lose all muscle function?
    Tori doesn’t move much anymore but can still move her neck, arms, and legs.
  11. Do you know if New Zealand screens their Newborns for it?
    I haven’t been able to find a definitive answer…
  12. At what age does Krabbe not affect a child? Or can it come about at anytime throughout life?
    It can start as early as birth and can appear at any point in life, though it is the most rare after age 11. Judson’s Legacy has a great graphic about this here.

Stem Cell Transplant and Treatment

  1. Will Tori have further testing? Do they do more testing once a child is diagnosed?
    When we saw Dr. Escolar in Pittsburgh, she did an MRI, spinal tap, vision tests, and blood tests using her specific Krabbe protocol to properly assess the status of Tori’s case. We aren’t sure if Tori will undergo more testing when we return next month to Pittsburgh.
  2. I know the science with cord blood transfusions is very new but how do they determine the cut off point for when babies are no longer eligible for the treatment? Are they certain that remyelination will not take place? Why are babies no longer eligible for stem cell transplant treatment once they have the onset of symptoms?
    Krabbe damages the myelin in the brain and once it gets to a certain point it is irreversible. The damage has been done. Therefore, doing the stem cell transplant can only stop the progression but cannot reverse anything. In Tori’s case, because of how aggressive her case is, the process of the transplant (including chemo) would actually cause the disease to accelerate. She was therefore not a candidate.

“Do you ever tell God that you are angry at him?”

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We are asked fairly often if we are angry at God or frustrated with Him.

We can honestly say no. We are not.

Are we curious to know why this is happening? Of course.

Do we have our moments of sorrow and frustration at our circumstances? Definitely.

But anger at God has not been and is not currently something we are experiencing.

God is God and we are not.

“My thoughts are nothing like your thoughts,” says the LORD. “And my ways are far beyond anything you could imagine.” Isaiah 55:8

As I wrote before, we trust God completely and, even though we don’t like what is happening, we know that we will eventually (even if in Heaven) understand why this happened. We know beyond any doubt that He is trustworthy, faithful, and loving.

We know that He will be glorified through her life no matter how long we have her here on earth.

This doesn’t mean that we are “okay” with Tori’s situation, blindly accepting it without emotion. But, it does mean that it’s “okay” in the sense that we know that God is in control, we know that He loves us – and her – and we know that He has an amazing plan for all of our lives. We are unable to comprehend His plan at this moment, but we trust that He has one.

We are filled with His peace that exceeds all that we understand. There is no earthly reason for us to be so at peace with all of this – it is absolutely from God. Being at peace doesn’t mean that we are okay with any of this, but it means that we trust the One who knows all and created all. We trust Him fully with our precious Tori and know that He loves her even more than we do. We know that He eventually uses all things for the good of those who love Him (Romans 8:28).

It is a moment-by-moment process of surrendering all of this to Him and choosing to not worry, to not be angry, and to continue to hope that He will choose to heal her here on earth. He is more than able, and nothing is impossible for Him (Luke 1:37)!

The daily reading for today is from Psalm 66, and it is so fitting for our situation, and for this question:

Psalm 66

1 Shout joyful praises to God, all the earth!
2     Sing about the glory of his name!
    Tell the world how glorious he is.
3 Say to God, “How awesome are your deeds!
    Your enemies cringe before your mighty power.
4 Everything on earth will worship you;
    they will sing your praises,
    shouting your name in glorious songs.” 

5 Come and see what our God has done,
    what awesome miracles he performs for people!
6 He made a dry path through the Red Sea,[a]
    and his people went across on foot.
    There we rejoiced in him.
7 For by his great power he rules forever.
    He watches every movement of the nations;
    let no rebel rise in defiance. Interlude

8 Let the whole world bless our God
    and loudly sing his praises.
9 Our lives are in his hands,
    and he keeps our feet from stumbling.
10 You have tested us, O God;
    you have purified us like silver.
11 You captured us in your net
    and laid the burden of slavery on our backs.
12 Then you put a leader over us.[b]
    We went through fire and flood,
    but you brought us to a place of great abundance.

13 Now I come to your Temple with burnt offerings
    to fulfill the vows I made to you—
14 yes, the sacred vows that I made
    when I was in deep trouble.
15 That is why I am sacrificing burnt offerings to you—
    the best of my rams as a pleasing aroma,
    and a sacrifice of bulls and male goats. Interlude

16 Come and listen, all you who fear God,
    and I will tell you what he did for me.
17 For I cried out to him for help,
    praising him as I spoke.
18 If I had not confessed the sin in my heart,
    the Lord would not have listened.
19 But God did listen!
    He paid attention to my prayer.
20 Praise God, who did not ignore my prayer
    or withdraw his unfailing love from me.

Praise God who does not ignore our prayers or withdraw His unfailing love from us.

What NOT to Say…

· 38 Comments ·

When we made Tori’s story public we expected to hear from people genuinely trying to help save her life. We have greatly appreciated the time and energy people have put into trying to help her, and us!

However, we have also received messages and comments from people who clearly haven’t researched Krabbe and are merely trying to push their own beliefs and platforms on us. There is a huge difference between the two, and it is always obvious which camp the person commenting is in.

Below is an email we received yesterday from a woman who claims to be a health and wellness coach on her Facebook page. She has stated in the past that she believes that things like ranch dressing cause cancer, to give you an idea of her beliefs. She is part of a Facebook community that I am also involved in, and that is how she heard about Tori. She doesn’t like to be confronted and always deletes comments from people questioning her “facts” with actual research. She strongly believes what she preaches but is apparently very insecure when questioned.

This is an example of what NOT to say to a family that has a dying child. I included my responses as well to paint the entire picture.

NOTE: this post is not to stir up a debate about vaccines. Please do not comment with your opinion about them as it is completely irrelevant to Krabbe and it isn’t the point of this post at all. 🙂

Hi Lesa,

I’ve been thinking of you and your family. How is Tori doing? And yourself?

I wanted to get in touch with you as I find out the resource I had for you never got in touch with you. I am sorry.

When I first read of Tori’s story I immediately sensed why she had the reaction she did. To confirm, she had these problems right after vaccinations, correct?

The toxins that are in the vaccines include aborted fetuses, heavy metals, and a host of other things that God never intended our bodies to have. Vaccine injury is very real and to date the US gov has paid out over $3 Billion to families.

Here are some resources to get you started in the healing process of Tori and your family:

I highly recommend that you start a vaccine detox with Tori to help her body get rid of the toxins. 
(Links omitted)

Please let me know what questions you have and how I can support you in Tori’s healing and in discovering the truth. 

It is apparent that this woman has not actually been following Tori’s story or diagnosis at all. So, the fact that she wrote to us with those suggestions was not helpful or welcomed. But, I decided to still answer with grace.

I kindly responded to her email:

Thank you for sharing your thoughts and resources! 

Unfortunately, Tori has Krabbe Leukodystrophy – a genetic disease. Brennan and I both carry a recessive gene for the disease (we now know) and unfortunately Tori inherited both recessive genes. 

Krabbe destroys the central and peripheral nervous systems because the body doesn’t produce the GALC enzyme. 

I wish it were as simple as a vaccine. We are pro-vaccinations and believe they do more good than harm. Her shots had nothing to do with her genetic makeup, so without a miraculous healing from God Himself, she isn’t going to live past the age of 2 years. 

For more information on this terrible disease you can see http://www.huntershope.orgor http://judsonslegacy.org/krabbe-disease 

Thank you for your concern! If you want to keep up with Tori, I have been blogging extensively about it at https://thebrackbills.wordpress.com and her Facebook page is http://www.facebook.com/prayingforTori

Her only response?

Even though they contain aborted babies?

 I thought for a long while about how to reply because I wanted to be kind:

With all due respect, I have never seen a legitimate source write about vaccines containing aborted fetuses. And, if it is true, it’s not like they aborted the babies just to make the vaccines. If anything, that aborted fetus is saving lives by being in that vaccine, which takes a horrible act and brings good from it in a way…if it is even true.

But, that isn’t what this is all about. Our baby is dying (and suffering in the process) and right now I honestly couldn’t care less about the vaccine debate. What I care about is getting legislation passed in each state to ensure that every newborn is tested at birth for Krabbe so that no more babies lose their lives to this horrific disease.
If the issue had been left there, I wouldn’t have minded. However, she posted the following status on her PUBLIC page not long after this conversation happened:
IMG_9894I was stunned when I saw this. The timing of it made it impossible to believe that it could be about anything – or anyone – else. I commented on her status asking if she was writing in reference to me, and she promptly deleted my comment (and the ones from other friends of mine who saw the post) and then deleted the post and blocked all of us.
The whole situation made me laugh, honestly. Yes, I was annoyed when I saw her status about us. How anyone could say something like that, claiming that we aren’t doing everything we can to save our child’s life, is unbelievable. But, more than anything, I feel sorry for this woman. She is so misguided and her Facebook page is filled with ridiculous health claims. She doesn’t appear to have any credentials or health education, yet she is offering health claims and advice to the public as a business. Sigh.
Krabbe is genetic. Nothing we did caused it. Vaccines sure didn’t. In fact, vaccines are highly recommended for Krabbe babies.
Moral of the story: Don’t say that parents of a dying child are not trying to save their child’s life. Don’t offer advice without researching the disease. It isn’t helpful, it hurts feelings, and it wastes precious time.