Bring on summer fun, family & friends!
Today: Monday July 4th, 2016, Day +466 Post BMT (Bone Marrow Transplant)
We are finally enjoying HOME after Bella's 3rd long term hospital stay.
Away from Home Recap:
1st long term hospital admission: Bella's birth on Dec. 6th 2013 to Sept. 8th 2014.
2nd long term hospital stay: Oct. 15th 2014 to December 13th, 2015.
3rd long term hospital stay: April 5th 2016 to June 8th 2016.
Totalling: 580 Days in Hospital
(Well... That's a nice round number. Cue massive eye-roll)
Bella is now 2 and a half years old, 63cm tall, 15lbs and 100% mighty.
Bella's BMT remains 100% successful and has cured her from SCID! Her Tcells remain at 100% donor and can fight viruses. Her Bcells are still in recovery after her recent need of steroids from a Hemalytic Anemia relapse back on April 7th, 2016. Bella's Immune System is still learning and growing, but working! We wait for her Bcells to recover before we can look at starting her vaccines and immunizations like any normal, healthy baby would receive from birth. Hopefully this can happen for Bella soon. We will know this information in hopefully less than 1 months time.
The steroids Bella was on (has been off of for 2 weeks now) remain in her system for roughly 6-8 weeks. They skew her immune panel results, so Bella's immunologist is still unable to see what her true immune function is. So we wait. Her Bcells are recovering and are currently sub-optimal. They need to get to normal range before they can test function and determine when Bella can begin to receive her immunizations. Therefore we wait another month and return to Calgary for a large immune panel blood test.
Isolation Protocol: Because Bella is still slightly immune compromised from being on steroids, we continue to protect her from anyone who may be sick, or not vaccinated/immunized. She could fight a cold but we still refrain from purposely putting her in a position where she may catch one. We also keep her protected because Bella is unable to receive her own immunizations & vaccines at this point. This means she is not allowed to be around babies under 1 years old who have not competed their immunizations. She is not allowed to be around any child over 1y old who is unvaccinated and she can not be around any child/person who has received a recent live vaccine due to the fact you shed those vaccines for roughly 2 weeks after receiving them.
Hemalytic Anemia Update:
We are in constant prayer that this remains behind us now, so that Bella never deals with this ever again. Why did this happen to Bella, and why did it happen twice? First off, her BMT doctors have told me this can be a rare complication from having a BMT. Just having to do with the way her Bcells learn how her new immune system should work. The Bcells were stupid and instead of only attacking bacteria, they attacked everything, including Bella's red blood cells causing her HGb to drop so drastically. Bella's doctors did NOT expect this to happen a second time. But it did, and worse than the first. So April 7th Bella received steroids once again, and blood transfusions. The big steroids knocked out ALL of her Bcells (because they were being stupid) and essentially we hit the restart button on her Bcells. We pray they have now had enough time and teaching from her Tcells to come back in smarter, and not attack her own red blood cells again.
Hopefully you're all keeping up with this science lesson ok. Remember, I am okay with questions now. I feel much more equipped to answer them. Please use Bella's Facebook page to ask questions.
Her Facebook page link: https://m.facebook.com/Isabellas-Mustard-Seed-848573408556136/
In a nut shell, the relapse of Bella's Hemalytic Anemia has been dealt with. She is done steroids, her HGb has remained stable above 110 and she is off more medications. But, for me, fear and high anxiety remain. I'm not saying I'm doubtful, but I'm on alert. And when you need to be on high alert for months on end, it is truly tiring...mentally and physically.
Given the time line of her first relapse, we could say I need to be on alert for at least 4 more months. Watching her HGb levels weekly, cleaning her cvc line diligently, watching Bella's color and energy levels, praying a 3rd relapse will not occur. Which her doctors have reassured me they do not expect, based on how well Bella's system reacted to steroids this time.
But this is all why I had to give up my job, my full time permanent teaching position...to remain on high alert for my daughter's safety and health. It's so hard, but we are pushing on in faith and so many good things are happening right now.
Recent Victories!
I usually use my blog as an overall update on everything with Bella and I use Facebook for quick updates, pictures and videos. So hopefully many of you follow Bella's page on Facebook to capture our day to day victories, but if not, here are a few she has accomplished:
1. Starting in May 2016 at 2 1/2 years old Bella has learned to drink! In a bittersweet way I believe the 6 days she was forced to be NPO (nothing to eat or drink) during our recent hospital stay, forced her system to crave this skill. On that seventh day she was only allowed liquids and she definitely took advantage of that, and thus, her consistent drinking skill was born.
She can drink all fluids she requires in a day. (800 mLs) We are mainly having to only use her Gtube button for medicine or when she has sick days for fluid maintainence. Bella continues to eat all of her calories and she has kept up with this victory since she learned how to eat at 1 1/2 years old.
2. Bella is climbing like a super monkey! She is going up and down stairs like a boss and with much confidence. She can climb up and go down small slides all on her own.
3. She is off way more medications. Hopefully off ALL medications come September.
4. She enjoyed her first kiddy pool swim in a bathing suit. (First picture of this blog post)
5. She has been on her first boat ride in a lake! This is special because we have always called the baby scale in the hospital a boat...but now she knows what a real boat ride is like.
6. Lastly, she is beginning potty training!
I thought she was ready about a month ago when she increasingly became upset with her dirty diaper. But after being through so many bowel tests and issues in May I waited to try training. Within the last few weeks Bella has started to run up to me after she poops and say, "Mommy I poop'd!" Or point at her bum and say, "Mess!" Which is utterly adorable and hilarious. She has also, for a while, greatly enjoyed passing gas really loudly and shouting, "I toot! I toot!" ... I really need to get that under control before she goes to school. Haha. But hey, when you have a child with Hirschprungs, those toot victories are everything.
Bella's Bowels Update:
Looking back at our most recent hospital stay from the Hemalytic Anemia relapse, Bella's stay in hospital was extended because of the horrible tummy troubles she had during the month of May. Her doctors speculated high dose steroids being the culprit but there was much confusion and termoil around what was actually happening with Bella's sensitive bowels causing her drastic distention and pain. Bella's surgery team was first up to bat. They had to rule out serious issues from a surgery stand point before anything else could be done. If you followed on Bella's Facebook page, you'll remember the pain she had went through for all that testing. The numerous X-rays, straps, enimas, rectal exams, medication, tests and trials. It was horrible, and it broke me down, just like all of her bowel issues and surgeries from ALL of 2014.
But this put me in to warrior momma mode and I sought information and help while surgery had to do their thing. And while we had to wait on a GI consult.
Without pumping my own tires too much, I must say, Lyle and I figured out Bella's tummy troubles and how to manage them, on our own, with help from a few other medical parents. And once Bella's GI doctor got involved, he completely agreed with us. Problem is...we didn't get paid like he does. (Cue, second large eye-roll)
Anyway, this is what we figured out. SIBO (Small Intestional Bacteria Overgrowth)
Bella has no colon. It was taken out due to her having Hirschprungs (the dead cells that affected her whole colon caused a blockage). Bella had an ostomy for 8 months and miraculously received a surgery that reconnected the end of her small bowel to her rectum, enabling her to poop normally! We were celebrating the fact that Bella would go as far as to only poop once or twice a day and pass thick normal, formed stool. (BTW sorry if this is TMI for you. But you'll understand soon.)
But...Celebrating this stopped, once we realized how SIBO affected Bella. It is better for Bella to poop more than twice a day. And it is better when her poops are gassier, and much softer. In Bella's case, more pooping and softer pooping does not mean she is sick...its means her bowels are comfortable and at no risk of SIBO. In Bella's case when any trigger causes her to be "backed up" or even slightly constipated...SIBO can begin very quickly in her case, because she has no colon. Causing massive distension, bacteria overgrowth, gas build up, and pain. Which is very hard to get rid of.
So when you do not have a colon, you do not have a storage tank for your poop. The colon can manage the bad bacteria. So where does Bella's bad bacteria go...directly to her small bowel. The steroids caused Bella to be constipated and triggered the SIBO. So with no colon the bad bacteria takes over Bella's small intestine causing an overproduction of gas, bloat and pain. Once Bella's GI specialist heard that this is what we believed was happening, he promptly agreed and helped us with the best forms of management for Bella. Because the pain and bloat was so bad for her we needed to start with a antibiotic called Flagyl. We were also told to start Bella on the FODMAP diet. This includes Gluten Free, Lactose free and no foods that cause fermenting (gas build up) in the bowels. This diet alone was a huge adjustment for me more so than Bella. She has blessed me beyond words with being such an adaptable child. She'll try everything at least once, and even surprise me with a new favourite food she likes almost every other week. We also have Bella on a constant dose of probiotics (good bacteria). So far this is all helping, a lot, but Bella still has had "bad tummy days." Thankfully we have not needed to go back on antibiotics and have many other ways that help manage her tummy while her body continues to rid the steroid from her system.
Looking at the Summer Ahead
This is Bella's first summer at home! Can you believe it?! We were hoping for less trips to Calgary for her check ups and IVIG, but I revcieved the call today from her specialists, telling me that because of her Bcells still needing time to recover, they can not test her complete immune function yet and we must come to Calgary in one months time, again. The news we are hoping to hear soon is that Bella's Bcells have fully recovered and do not come back with any "memory Bcells" in term causing any more Hemalytic Anemia problems. We also want to hear that Bella's system is making its own Immune Boosters (IGA's & IGM's) Once this happens they can trial Bella off IVIG, watch her levels and hopefully she will no longer need IVIG infusions. This would also mean less trips to Calgary.
For now Bella's first summer at home will still involve: a cvc line in her chest, blood tests weekly, Calgary monthly and a continuation of 3-5 medications. But we will work around all that, we will adjust and make this her best summer yet! She's finally home!
Bella is due for a MRI and CT come September. We are hoping for these tests to show great news to end our summer with. The MRI and CT will be used to look at quite a few telling facts about Bella's lymphnodes. These tests will also be used to tell us if Bella can come off of the MAC bug medications she has been on for a whole year. They will also use the MRI to look at the nerves in Bella's right hand/wrist to see if they can figure out any more knowledge about why she has limited use of that hand and weather surgery could help restore her wrist/hand function.
My main focus looking ahead at this summer is Family Catch-up. I have constantly been struggling with the stress and anxiety that has come along with all the TIME I have lost with family while living in hospital with Bella. It eats away at my mind. I want that time back! ...so badly. It's so difficult for me, having Bella's medical requirements, to just pick up and go. We can't do that. Along with trying to catch up on time, I feel held back by all the continuous needs of packing for Bella, remembering all her meds, her 'just in case' meds and supplies, emergency kits, no one else knowing how to use her cvc line & Gtube lemergency kits, and on and on and on. I'm struggling with this anxiety a lot lately. I mentioned this a bit in a previous blog post and I'm still having issues with it.
Try and imagine a sailor (they swear a lot), with cement boots on, in a thick mud puddle with a backpack twice her size strapped on her back, holding her child while she struggles to reach her sinking ship in the distance. That's me, everytime I'm packing to leave the house with Bella.
Time is precious.
Recently I've added to my support system! I have finally found a doctor for me, that's right, me...just me...finally. I have not been able to see a doctor for myself since before Bella was born. My c-section was my last interaction with a doctor, for me.
Fast forward to this week and I am beyond happy with my new doctor. He is wonderful and everything I need for all that is 'Kyla's medical needs.' It's a huge relief.
Our amazing Geneticist (Dr. Billie) in Calgary has worked so hard to confirm that both Lyle and I are carriers of the same gene sequence that causes CHH linked SCID. Up until this past month they could only find the CHH causing gene in me and not in Lyle. This made things difficult because it just didn't make sense and without finding confirmation of the gene in Lyle, we would not be able to move ahead with PGD IVF in order to have a second child without the deadly disease.
So they found it!!!! In a nut shell, the lab who did the initial testing was not experienced in looking for this gene. Which our geneticist told us was a small gene and should actually be easy to find. So given Bella's clinical diagnosis of SCID, Hirschprungs and presenting in other physical ways as CHH, it was obvious that she had CHH but we needed Lyle's gene to be found in order for us to receive PGD IVF. CHH is an autosomal recessive condition where both copies of the RMRP gene need to be affected for there to be disease. So in order to have CHH (like Bella does) it's because she received one affected gene from Lyle and the other from me. What are the odds of this happening again? A 1 in 4 chance for our next child if we conceive naturally.
Because Bella's CHH is linked with the deadly disease SCID, this is not a risk we will take and instead we will use PGD IVF to ensure our second child does not need a BMT to survive.
It's difficult making this decision. Suprisingly there's no support from our government. I wish I could make the plea that given proof from our doctors that we need PGD IVF to ensure our child does not have SCID... You would think the government could financially support this for us. Versus: we risk it and cost our health care millions of more dollars for a child needing a year long hospital stay for a BMT.
I have been asked many times and in turn asked Bella's specialist..."What are the odds?" What are the chances I find the love of my life (Lyle) and our love means our unique gene make up causes a deadly disease?! How devastating is that? It's heart breaking and I think about it every day. Every second I see Bella playing alone and wish she had a sibling, right now, playing along with her. And that thought shatters into a million pieces because the love of my life happens to be a carrier of the exact extremely rare gene mutation that I have. What are the odds? Well, our doctors can't explain that, our geneticist can't explain that. It's so extremely rare that they don't know the odds. I mean, it took years to understand that Bella, having Hirschprungs can be apart of having CHH, and SCID can be apart of having CHH, but does she have CHH? It was a question for so long. But now that it's confirmed and we realize she was born with all 3 of those extremely rare conditions... WTF are the odds, right!?
I had an excellent convo with Dr. Billie at Bella's last appointment in Calgary. And although we knew for quite some time this would get confirmed eventually, she agreed this news from the lab is like closure for us. The searching is over and we can move ahead with plans for baby number two.
Links to Our Lives
I absolutely love SnapChat. If you understand or use this app on your cell phone, you'll know what I'm talking about. The main reason why I like it is because it uses photo and video you can share with others, without making you save a photo and clogging up your device with a large amount of photos. I also love SnapChat because it's about being in the moment. I want to worry less about saving this photo, or saving that video, or spending money on devices that save all the thousands of photos I take. Sometimes it's okay to have a fun moment captured, and let it go. Memory. SnapChat is like that.
It's also so much fun using all the funny faces the app has and I love to make jokes. Life is better when you're laughing! If you are a snapchat user, you can follow me @kylakcco.
In May I was asked to be apart of a promotional video commercial for the Calgary Ronald McDonald House. You may have already caught this on my Facebook page but I'll add the link to the video on this blog post as well. These commercials were made by a Calgary film crew, to be shown in Calgary Cinemas/ Movie Theatres to promote support for the house. Once the initial commercial was made, the crew kindly approached me and asked if I would mind doing an interview commercial as a second video for the house. It worked out so well. During one of Bella's naps I was able to talk with the crew and share how this house held me up when I was broken by my daughter's medical needs and hospital stay. As hard as it is to share the pain in our journey for Bella, sharing our story means more support for RMH.
Here's the link:
Family photo at my dads cabin.
