Hi Ladies - it's been a long time since I updated - everyone has probably forgotten us by now !! - but for those who remember me / James, we got some big news this week and I'm finally trying to come back to the land of the living. Last time I updated, we had the diagnosis of HLH and were trying to wean James from the ventilator and were in the middle of chemo. Fast forward several weeks: James' breathing is still a struggle because his lungs were so badly damaged in the initial illness, but after 2 false starts (he's now been intubated a total of four times - don't get me started) we're finally on CPAP with regular cannula trials that are going well.
And even in the face of this craziness, this week has been insane. When we transferred to our current hospital, their first step was to sequence the DNA of me, my husband and James to figure out whether the HLH was genetic, because genetic HLH requires a bone marrow transplant for a cure. Everyone was sure that James' HLH must be genetic because it presented before 2 months of age, and we thought we were in a terrible situation because the transplant doctors did not think James could survive a transplant - so either he would die from HLH (which is 100% fatal) or die from an illness associated with his transplant. Either way, on Monday I started looking into hospice care for infants, which is literally the most unspeakable thing I've ever done.
And then yesterday we received the DNA results ... which show that James has something very rare called Kabuki Syndrome. His cleft palate, pelvic kidney and anal atresia are all explained by Kabuki, and his HLH is secondary (not genetic) and related to the Kabuki (which can have immune system involvement, though the association is not well understood). He had his last chemo treatment today. The doctors now say that James does not need a bone marrow transplant to finish treatment, although they will still watch him carefully because he is literally the first kid in recorded medical history to have Kabuki Syndrome with secondary HLH. In other words, he's now very likely to survive. And while he is going to need a lot of therapy and rehabilitation after all of this, has the chance of being a happy kid
At some point I will hopefully be able to share with you guys more of the craziness of the past months. But for now, I have to stop thinking of myself as giving James palliative care, and start thinking of myself as a mom of Jan '14 baby, because he's going to be here and with us for a long time. So hopefully you guys don't mind having me back... our experiences are still going to be pretty different from yours, but since I've been through it all before with Max, I can still relate! xoxo
@vstevens I am so happy to hear from you and more importantly hear this amazing update! BIG hugs mama! Sending James prayers for continued good news. Know that we think of you often and hope that we'll be seeing more of you.
"Do not be anxious about anything, but in everything by prayer and supplication with thanksgiving let your requests be made known to God. And the peace of God, which surpasses all understanding, will guard your hearts and your minds in Christ Jesus." Philippians 4:6-7
I'm SO happy to read this update! I think about you and James all the time! Continuing to send many thoughts and prayers your way!
Missing Our July Sparkler BFP#1-11/12/12, MMC 1/16/13-baby stopped growing @ 9wks, found out at 13wks, D&E 1/25/13 BFP#2-4/23/13 EDD-01/02/14 baby BOY born 12/31/13 Michael Cameron
You're not forgotten. This is great news! Big hugs to you for all you've been going through. Our current experience may not be the same as others here, but we of course want to hear updates and be of support to you when we can. Hoping James continues to make great progress.
I'm so happy for you that you have received such wonderful news! I've been thinking about you and James recently. I will continue to keep you both in my thoughts and prayers, and look forward to hearing more updates on James's progress!
This s amazing news, v! Brought a smoke to my face!!!
Of course we want you back here. we have missed you and James! Can't wait to hear more about him!
Well now you've done it @ChuggingWater, taken a beautiful moment and made it about smoking the drugs. Sheesh.
This s amazing news, v! Brought a smoke to my face!!!
Of course we want you back here. we have missed you and James! Can't wait to hear more about him!
Well now you've done it @ChuggingWater, taken a beautiful moment and made it about smoking the drugs. Sheesh.
If I didn't have a 2 year old at home, there would have been a LOT of smoke to my face in the past two months.
Instead there's been wine. Lots and lots of wine ... that's more acceptable...right?
That's amazing news! I'm so glad you're back and that things are looking up. I can't even begin to comprehend how difficult the past couple of months have been. But you, baby James, and your family will continue to be in my thoughts in hopes that things continue to get better!
So glad you updated! We were all wondering how you and James were doing. That sounds like some amazing news. Big hugs for all that you've been through.
BFP #3 9/24/12, Missed m/c at 9w1d (baby measured 8w5d)
BFP #2 9/23/10, healthy baby girl born med-free June 2011
BFP #1 5/21/10, Missed m/c at 10w4d (baby measured 8wks), D&C 6/29/10 "Life is like a camera, just focus on what's important and capture the good times, develop from the negatives and if things don't work out, just take another shot."
This post made me smile!! I'm so so glad he's on the mend and your not having to looking at palliation and hospice but a full recovery. So happy for all of you!!
So happy for your positive update! I can't tell you how sorry I am for all you and James have gone through. I hope things only get better and better each day forward.
Wow @vstevens! What a great update! I am so sorry that you have been going through this. My thoughts and prayers are continually with you and your family. Keep us posted J14 momma!
What a great update! I'm so glad that you got some good news. I can't even imagine looking into hospice for infants. Continuing to send love and positive healing vibes ((hugs))
So sorry you are going through all this. I'm truly happy to hear the good news. Glad you will be able to take him home soon. Thinking of you and your baby boy!
Re: James Update (AW)
BFP #2 (5/18/13) due 1/26/14. Grow baby grow!
Its a surprise! Team green!
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Missing Our July Sparkler
BFP#1-11/12/12, MMC 1/16/13-baby stopped growing @ 9wks, found out at 13wks, D&E 1/25/13
BFP#2-4/23/13 EDD-01/02/14 baby BOY born 12/31/13 Michael Cameron
BFP 11.8.12 * EDD 7.17.13 * MC 12.20.12
Of course we want you back here. we have missed you and James! Can't wait to hear more about him!
Baby Chugging born 12.28.13
induction due to HELLP
If I didn't have a 2 year old at home, there would have been a LOT of smoke to my face in the past two months. Instead there's been wine. Lots and lots of wine ... that's more acceptable...right?
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DS1 born 08.02.11
DS2 born 12.05.13
DS1 born 11/3/06 * DS2 born 3/29/08 * DD born 3/15/11
Scarlett Mae born 1/14/14 Our family is now complete!
Emma Kate - born 10.16.03 @ 29 weeks, weighed 1lb 13oz and 13.5" long.
TTC #3
BFP #5 11/15/14, Team Green EDD 7/22/15
BFP #4 4/30/13, baby girl born med-free Jan. 2014
BFP #3 9/24/12, Missed m/c at 9w1d (baby measured 8w5d)
BFP #2 9/23/10, healthy baby girl born med-free June 2011
BFP #1 5/21/10, Missed m/c at 10w4d (baby measured 8wks), D&C 6/29/10
"Life is like a camera, just focus on what's important and capture the good times, develop from the negatives and if things don't work out, just take another shot."
@chuggingwater Pass the smoke!!!
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BFP 10/8/12 | Missed Miscarriage - D&C 11/12/12
Married: 4/2/13
DS born 12/19/13
Due Date 11/10/16
J14's January Signature Challenge: Favorite Post-Delivery Indulgence
My cat can read EKGs, can your human do that?
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