November 11, 2011

Answering Questions & a Liver Update!

Thank y'all so much for your encouragement, support, and congratulations on our news. 

I'm so thankful that we have so many people praying for our sweet boy (and the rest of the family).  The comments, emails, tweets, facebook messages, calls, and texts have been so overwhelming.  I'm still trying to wade through them all - if Kate would start sleeping/ napping I would have more time to respond!

I've received some questions and thought I would dedicate a post to answering them!  If you have a question that isn't answered here, leave it in a comment and I will do a part 2 and cover those questions!

Since you know about Will's condition now, when will his transplant be?
That's a great question and I wish I had an answer for you!  Ideally, we would like to get Will to 12-14 months before his transplant.  We also have to take Kate's health into consideration as the BMT doctors would prefer we didn't have two immune compromised children living in the same house.  If we can get Will to 12-14 months old, then that would put Kate at about 1.5 years post transplant. 

I'm not sure if I've mentioned this before but, Kate is currently 100% unvaccinated due to the intense chemotherapy she went through.  She is also on immune suppressant medication to ensure her grafts holds and that her body doesn't attack the donor cells.  Typically, BMT patients immune systems begin to rebuilt around the one year post transplant mark and vaccinations are re-started between 12-18 months post transplant.  It would be wonderful if we could get Kate to the point of starting a vaccination schedule before Will starts chemo and jumps on the no immune system bandwagon.

However, CAMT is an extremely variable disease.  There is no concrete answer as to how aggressive the disease will be.  Some kids make to to 4 or 5 years of age before ever being diagnosed where as others are born with essentially no platelets and low blood counts.  Kate starting showing signs of the disease around 6 weeks but was not in immediate need of a transplant until about 10 months when she was finally diagnosed.

There is a chance Will will be impacted my sooner than Kate.  If this is the case, he will go to transplant as soon as necessary.  On the flip side, he may not be showing signs of CAMT at 12 months.  However, the only cure is a BMT and studies show that children that have a transplant in the 12-14 month window often have a much better long term prognosis. 

The short answer is Will's transplant could be any time during the first year of his life but will be no later than 14 months of age.

Will your pregnancy be treated differently due to the diagnosis?
I will be under the care of a perinatologist (the same one I saw while pregnant with Kate) for my entire pregnancy.  I have a handful of health issues (heart condition, had heart surgery as a child, blood issues that worsen during pregnancy, etc) that require a bit more care.  Add in the CAMT diagnosis and there was no avoiding the high risk label.

I will deliver no later than 38 weeks but there is talk about the possibility of delivering as early as 36 or 37 weeks.  My doctor and Will's BMT doctor are trying to figure out the most ideal time to deliver.  We know that I will not go farther than 38 weeks as we need to evaluate his blood levels and how his body is reacting to the CAMT and this cannot be done until birth.  The thought is that if he is deteriorating quickly, the extra fews weeks on the outside to prepare for a BMT will be extremely beneficial.

I will also be delivering at the new Texas Children's Pavilion for Women - I'll actually be one of the first people to deliver there as deliveries won't start until April!  There is a chance Will will go to a Special Care nursery after birth and all of our doctors believe it is best for him to be at Texas Children's from the get go.  All of this will depend on his health when he arrives.

Is there a chance the diagnosis is wrong?
I wish I could say yes but, the CVS and genetic testing are 99.8% accurate making a misdiagnosis extremely rare.

After the BMT is done, are people cured from CAMT?
Theoretically, if a CAMT patient is at 100% donor cells, they are cured.  CAMT holds other risks/ health issues that can arise if any original cells are present.  Our prayer is that Kate and Will can both hold onto a 100% donor graft and will go on to lead normal, healthy lives!

What were the chances of this genetic disorder being passed on again?
CAMT is a recessive genetic disorder.  This means both the mother and the father have to be carriers of the same genetic mutations to pass it along to their children.  Obviously, Alex and I found out the hard way that we are in fact carriers.

With all recessive genetic disorders, the following things can happen: a 25% chance your child is not affected at all, a 50% chance your child is a carrier, and a 25% chance that your child is positive for the condition.  Obviously Kate and Will have both fallen in the "unlucky" 25% of being positive for CAMT.

Will you do prenatal HLA typing?
No, we will not.  As I mentioned in my post about our CVS testing, we were unable to get a good size tissue sample.  We barely had enough for the genetic testing we needed to do, much less extra for HLA typing.  We have decided that at this time the risk of an amnio (it's too late for another CVS) is not worth the benefit of typing Will while in utero.  We will do HLA typing as soon as he is born.

Can Kate's donor be Will's donor as well?
Yes, he can.  There is a 25% chance that Kate's HLA typing and Will's HLA typing are identical.  If this is the case, Kate's donor would be a perfect 10 out of 10 match for Will as well, making him a perfect match.  Obviously, he would have to agree to donate again but, I am confident that he would. 

Feel free to leave any other questions you may have and I will do my best to answer them!

And, a quick Kate update.  We finally received her liver biopsy results but they were "inconclusive".  We were able to rule out GVHD, a medication reaction, and VOD of the liver but, the pathology report claims there is chronic irritation and inflammation of an unknown origin".  Um, what?  Her doctors don't seem concerned since her liver enzymes are coming down (but still elevated) but I'm kind of worried over here.  I'm hoping her liver specialist can shed some light on what could be causing this.

P.S. - don't forget to scroll down and enter the Canvas4Life giveaway I posted about this morning!


Abra said...

Wow. So much great information. I know it's taxing on you to share so much, but I hope you understand how much I (and hundreds of others) appreciate you doing so. I'm thinking about all of you every day and am so happy that Will is going to come and double your joy!

Allison said...

Thanks Lindsey--appreciate your willingness to share. We continue to pray for you guys. I "sounds" like you are in good spirits which is awesome. One day at a time :)

Ali said...

Thank you for being so open about everything you and your family are going through. Your children are very blessed to have such committed and proactive parents. Will and Kate are both in our prayers.

Wendy said...

This is COMPLETELY random but I have a friend that had high liver enzymes and the doctors couldn't figure out why for the longest time. It turns out she has a sulfur allergy (she had recently started drink wine and got very sick for a long time b/c of it but she also had a rash). Just a random thought. :) I doubt that someone as young as Kate has much exposure to it but I thought I'd throw it out there. Glad her levels are on their way down!!!

Adellemaria said...

Hi Dear, I had been reading you blog right before kate went in for her surgery, but my computer crashed and I lost your blog which I had bookmark on my computer, I am so glad that I found your blog again am so happy that everything went well with kate, and I want you to know that God will give you the strength to take care of you babies, you will always be in my prayers.

Missi said...

Thanks for sharing. I've been sending up prayers here in WV for your family! Hope your sweet girl is starting to feel better!

Stephanie said...

I'm just getting the first chance to catch up on blogs. What an amazing story Will already has! I've already been praying for sweet Kate and now will be fervently praying for Will and your pregnancy. I hate that anyone has questioned you and your husband for bringing this baby into the world. Kate and Will are abundantly blessed to have parents who are giving them such wonderful opportunities to live happy, healthy lives!

Jen J. said...

I do have one additional question. While it was divine intervention (based on your IF history & the measures you took to prevent pregnancy this time) that brought you sweet Will, I assume that your desire to have no other children will remain after he's born. What are you going to do to ensure that you do not get pregnant again?

As always - praying for all of you. ((hugs))

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