Catching Up from Jan.- April 2018

"Writing is medicine. It is an appropriate antidote to injury. It is an appropriate companion for any difficult change." 

~ Julia Cameron

#BellaBrave
Current age: Fierce, Firey and Four!
Days in Hospital: 655 total from birth
Days at Home: More than 655 now!
Most Recent Hospital Stay: Feb. 2nd - Feb 6th 2018 / Emergency due to Bowel Blockage.
Immune Status: No Bcells yet... yet! But weekly SCIG treatments are protecting her well!
Bowel Status: Living her best life with her ileostomy pouch.

I have been putting off my writing since my last post from the beginning of January. It has just been too difficult for me to sit down at this keyboard with all the tragedies that have occurred recently, not just in our world but the many from my own hometown province of Saskatchewan.  Like the quote from Julia says, writing is medicine, and from my own experience, writing brings healing... But I'm just finding it hard to write with this painful pit in my stomach knowing so many out there are feeling an unimaginable pain from losing their loved ones or recently coming to grips with their child's harsh new diagnosis.  Recently there has been the astronomical tragedy of the Humboldt Broncos Bus crash, the passing of Eva Peters (a little girl diagnosed with ITP an autoimmune disorder, and the passing of Jonathan Pitre, a young boy who had EB then coming across the story of a Saskatoon baby, Leo, who was just diagnosed with the same disease.

Writing can bring healing and I'm happy to have that outlet available to me. But whats more important right now is the understanding that we need Jesus, we need to give blood and we need to do what we can for others from where we are at ourselves. So before I begin my blog post today, or before you continue to read on, please stop and take a moment to either; pray for those that I have mentioned above, book a time to give blood or seek out a way to support those financially. Thank you.

Catching Up From January

In my last post I noted a month by month summary of Bella's 2017 year, her success at daycare and her SCIG treatments. I also mentioned an important day that was coming up! January 22nd 2018 was the first day that Bella had finally spent more days/nights at home than in a hospital bed. It was a day that helped heal my heart just a little bit more from that scar. I knew my daughter finally could physically, visually and emotionally feel more like a kid, than a patient.

Bella went on to need 5 more nights in hospital later in February, but that still did not allow the hospital nights to out number the nights at home. And I'm confident it will stay this way for a while to come.  So this brings me to our next catch up, the Emergency situation that brought Bella to the ER for a Bowel Blockage.

Bowel Blockage... To hear those two words said out-loud to me, brings back a fear that is choking. Physically those words are wrapped around my neck for the rest of my life and I am constantly prying them off. They were said to Lyle and I before so many of Bella's bowel surgeries in 2014, and they have been mentioned to us so many times prior to her ileostomy, they have been said when trying to diagnose the actual issue while Bella wriggled in pain in the background, they have been (for a lack of a better word) threatened as a possibility when it ended up being something else.  To hear them again on February 2nd was again, another nightmare coming true.

The reason these words choke me is because their grip continues to tighten, and just when I think I've released their hold, another situation occurs that brings possible "Bowel Blockage" back on the table. They will always have to be on the table because Bella has suffered from Hirschprungs, because she has had multiple bowel surgeries, and because she is still dealing with a bowel motility issue they are still trying to understand.  When Bella vomits, with no fever... the choke hold begins. My brain has to dive right back in to ALL the possibilities, so that either her life is saved... (just chewing on THAT for a minute) ... or we can find calmness in knowing it's something minor. The problem is that once Bella has vomited (which she has done quite a bit lately, at least once or twice a month) there are so many serious possibilities that we have to rule out, they are the choke hold, and until they are ALL ruled out...I can't breathe.

Bella noticing the deep purple bruises from multiple pokes and IV sites on her arms. 

Vomiting, in my mind, equals these questions: How long since she's had output (pooped), is her bowel blocked by scar tissue that grew, a kink, a twist? What is every possible piece of food she ate in the past 24hrs to a week that may have caused this? What did she eat that I didn't see? Is it food poisoning or is it a stomach bug? How long until she is so dehydrated that she needs IV? Is it just her bowel's poor motility? Did she just eat too much, or did something just not "sit well.?"  ... every. time.  she pukes.

On a side note, we are looking into a connection to Bella's recent vomiting episodes possibly being connected to her SCIG Hizentra treatments, the IG (Human Blood product) that she receives weekly. She has been vomiting at least once or sometimes twice in a month, but ironically the episodes have been 24-48 hours after a SCIG treatment for at least 5 of the times the vomiting occurred. However, The major amount of vomiting that came Feb 1st was from a Bowel Blockage.

Here's What Happened With the Bowel Blockage

I hope that if anything, this helps any other parent/child suffering from similar bowel issues.

Monday Jan 29th: Lyle, Bella and I sat down to family supper. I made home-made chilli. Knowing Bella can not have beans or any food with shells and/or peels, I picked out the kidney beans (at least I thought I had) and gave her the yummy hamburger sauce.

Tuesday Jan 30th: 24hrs later, three whole kidney beans came out into Bella's pouch. What!? How!?

Wednesday: Jan. 31st: We noticed what we thought was and confirmed later, blood in Bella's stool.

Thursday, Feb.1st: At 8 am (we later realised) was when Bella's output stopped. She went on to not poop for 54 hours (2 1/2 days) For a person with an ileostomy, if you don't have output(stool) for even 8-12 hours, it is bad, it's serious and you start to worry.
Vomiting started at 7pm and continued until it was green bile coming up, dry heaves, and we went to the ER in Swift Current. Xrays were taken and a Bowel Blockage was confirmed. Plans were then put in place to figure out what caused it and  to get her to Calgary incase she needed surgery.

Bella was put on gut rest and NPO. So she was allowed NOTHING, not even water. Her gut rest came with a straight drain tube placed in her tummy button. So any build up in her stomach would flow/drain out her Gtube and allow her gut full rest and stop the vomiting.

We got to her Surgeon in Calgary, at ACH and he saw her in the ER. At that point he started to rule out an actual kink/twist in the bowel but not surgery yet. If the bowel actually did twist on itself, she would have been way way worse. But the gut rest seemed to be improving her state. However the NPO was torture for her once again. I pray you never have to hear your child beg for water. It's horrible.

At this point our surgeon set protocol in place that allowed us to see if surgery was avoidable. He guessed at scar tissue being the culprit due to the amount of bowel surgeries Bella has had. Any time you go in and touch the bowel, you cause scar tissue. The problem with that theory was that scar tissue is usually at it's worst in the weeks/months right after a bowel surgery. On this day, it had been at least one year since her last bowel surgery.  Oh right...Jan.26th was the one year anniversary of Bella having her ileostomy pouch.  So it was very very odd that any scar tissue would grow to the point of choking her bowel this much, a year from surgery. He gave a time limit as to when output needed to start happening, or else he would have to open her up surgically and find the source, or just cut away at existing scar tissue hoping it was the source.

Saturday Feb 3rd: At 2pm, Bella had output in her pouch. Her bowels showed signs of emptying and nothing was building up in her stomach anymore. She could start to drink fluids again. We had avoided surgery and beat the time limit. And then... 2 more, partially whole kidney beans came out into her pouch. Cue extreme mom guilt and breakdown. My mistake caused this. My mistake could have caused it to be worse. The multiple IV pokes, NPO for days, pain, possible surgery... on me because I didn't find all the kidney beans to take out.  Honestly, there may be a little humor in this, but I will never be able to eat chilly without crying, or feeling this guilt...ever again. As much as I would like to joke light heartedly about this supper I made... I can't, it still hurts too much.

 Recouping with Daddy on Unit 2 at ACH.

After this, Bella continued to improve and her doctors were very confident that the kidney beans were the cause.

Visiting her Unit 1 Nurses & Medical Staff, the ones who brought her through BMT.  

The night before discharge Bella was moved from the Bowel Unit 2 down to Unit 1 (Oncology) because Unit 2 had much more critical cases coming in. We were asked, and of course more than willing to move units to make room for more critical. Because Bella was not ill with any sickness or virus she was able to stay her last night on Unit One and boy were her BMT nurses happy to have her! The staff on this unit have always brought an immense amount of comfort to us. They saved her life when she was so close to death. They built Bella's strength to an overflowing quantity.

I can barely get to typing this next story from our one night stay on Unit One... the tears are already flowing and dripping, but I need to share.

It's quite beautiful to tell really, and the tears are more happy than sad.  So as we spent this quick evening on Unit 1 before discharge the next day, I found it quite hard to walk the 4 hallways of patient rooms. Too many of them we had occupied for all of 2015. But that wasn't the hard part, the hard part was relating to the little ones on the inside of those rooms, going through what they were going through. Knowing a part of their pain but not knowing their story. And just wanting so bad to reach out and offer hope instead of saunter by their door.

And then... Bella had an idea. Yes, this all came from her and I've never been more of a puddle...along with many of the nurses in that moment. Bella stops me, looks up at me and says, "Mommy, play my Elsa song, I want to sing it for them. Come on mom, turn it on on your phone!"
So I asked the near by nurse if this is alright and she said, "Of course." I clicked my Spotify app open and pressed play for "Let it Go."  It began to play, Bella started to sing and dance as she sang. My goodness, looking back I wish I would of recorded this moment, but of course we were too caught up in the love that was pouring out of my daughter's heart. This wasn't her want to show off her Let It Go vocals and dance...she saw, as we passed by this little girl's door earlier, that she could make her smile... and Bella wanted to make her smile more. As the nurse brought the little girl out of her room to watch Bella sing and dance for her, singing a song that even had such meaning behind the current situation... I just melted. Bella brought a light to this little girl's life that day as she was enduring treatment, Bella brought her some joy and it was beautiful.

Pool Shark pose

On To More Updates > Ostomy & Motility Study

Compared to my last updates on the daily routines and issues involving Bella's ostomy pouch I am happy to report that we have found a steady rhythm with the current supplies and system we are using and experiencing less of those mid night or unexpected leaks and full changes. This has been a big thanks to advice from Bella's Calgary ostomy nurse and having her provide me with very helpful information that I wish I would have known a year ago when Bella first got her pouch. She reminded me of some tips and tricks when applying the wafer and skin protection tools. She also helped me understand how the Supplier World works for those living with an ostomy. The hospitals are only contracted for one particular brand of supplies, but there are many more brands out there to try! When the supplier knows you, the parent, are the buyer they will send you free supplies to sample knowing that if these supplies prove to work better, you will buy from them! So I was able to contact 5 different suppliers, obtain free samples and figure out what supplies and tools worked best for Bella...for free! Now we know what works and are not sad at the fact that when it wasn't working, the loss of money was high. The cost of the supplies it takes to get Bella's pouch on is quite high. So when it leaked and was off within hours of putting it on.. she not only had to go through the whole application process again but we had lost $30.00 right there. Remember when it was so bad that some days involved 5-6 full changes.  Think of it as a very expensive sticker that we want to last at least one week on Bella's abdomen. We need that $30 sticker (her ileostomy pouch) to last at least one week. But without the proper tools and application it was coming off within hours. Given our new tools and supplies to help combat the space around Bella's stoma filled by scar crevices...we are able to get the pouch to last 4-5 days now! YaY Success!

Motility Study

With ironic timing as I just finished typing that sub-heading... Bella's GI doctor called me and sadly this motility study has been postponed yet again. It was supposed to happen in March, then May and now maybe, hopefully by September.

The reason why we are so eager for this procedure is because it will #1 tell us if reconnecting Bella's bowels is possible and take away her pouch, and #2 tell us SO much about how and why her bowels operate the way they do.

The reason why this study has been postponed was explained to me very well by Bella's doctor and he is also disappointed to say that this decision is out of his hands. Basically the company that is to supply him with the proper tools/catheters has not only been bought out by another company, but also that the new company has a programming issue do to Bella's custom protocol for the specific catheters. Hers need to be custom because of her size and anatomy. So we must wait a little longer and her doctor will do what he can to have this procedure hopefully done by September. He'll call me back in a month or two to tell me a new date.

It's not hard waiting for this type of day long study that Bella will have to go through, it'll be a tough one. But it is hard waiting because of all the good news that could possibly come from it, and all the information that will help her.

What the Motility Study Involves
When this actually happens, Bella will be put under anaesthetic in the OR. Her GI doctors and surgeon will place the catheters throughout her bowel. So tubes will be put through her Gtube button (stomach) into her upper bowel, through her stoma (mid bowel), through her Mucus Fistula (lower bowel) and bum.  These will provide censors from inside her bowels that a machine will read for 8-10 hours after the catheters are placed. So she'll have to come out of anaesthetic (which is never fun), and then she must sit in one spot with all these tubes coming out of her, connected to other machines, for 8-10 hours after. Doesn't sound pleasant at all, but this is the best study right now to do our best at understanding Bella's bowels and if reconnection is possible.

For Bella's 3rd Life Day (Anniversary of her BMT for SCID) we celebrated by going to the Medicine Hat Lodge waterslides and had a mini family vacay at the Medicine Hat Lodge. It was Bella's first time on waterslides and she loved it!

Immune System / SCIG Treatment Updates

We have some good news to share! Bella's IGG is finally above 5! Normal range is between 5-14 and Bella's recent bloodwork showed her IGG at 6.6! This  shows that her weekly SCIG (subq IG) treatment is working and protecting her. Your IGG is Immunoglobulin G, a major type of antibody found in the blood that can enter tissue and fight infection. Bella can not make this antibody on her own because she has no Bcells, so the weekly SCIG treatment gives her these antibodies to protect her. It is good to know her current dose of Hizentra (IG) is working. Back when she was on IVIG via IV lines, she still never could hold her number above 5 before the end of the month. The weekly SCIG treatment has proven to allow Bella to hold her IGG number in a safe and protective range.

It was hard to hear that Bella still does not have Bcell numbers but it actually doesn't mean she does not have Bcells.  She does have them, they are there, but they are babies and have not matured yet. So its like she doesn't have any because they are not maturing to a state where they do their job. We are still praying that Bella's Bcells will not only grow in number but mature and form the antibodies she needs on their own.

Bella recently received her Long-Term Survivor Clinic Letter!
This 2 page letter was quite endearing to receive and made me so proud of Bella. It tells us how Bella's BMT doctor has officially transferred her to the Survivor Clinic for all her follow up care. This clinic is a new team of professionals that follow up with survivors of childhood cancer and/or Bone Marrow Transplants. We look forward to meeting this team and working with them for the rest of Bella's life.

Bella the Kid

Enough about the medical side of things, lets talk fun, growth, excitement and just being a kid!

My little girl's character is fun, fierce and sensitive all in one. One moment she tells me to stop and check out her new ninja move and the next she's holding my face, telling me to look into her eyes as she tells me she loves me. She blurts out with sheer joy that mommy and daddy are her best friends and once in a while we wake up to her pleading by the side of our bed for a cuddle. She's not shy, and if you think she is, she's faking to get a giggle out of you. And I couldn't be more relieved to see that she still wows people, perfect strangers, with her contagious happiness after all she has been through and continues to go through.

Her independence is inspiring. Given the hard work she puts in at Physio to strengthen and just simply learn to use her right hand, is admirable. This girl has been given road blocks for things as simple as getting dressed, turning on the light or going to the bathroom... but she's overcoming each and every one.

 We have lowered switches to help her independently turn lights on. Or she drags around and positions her step-stool to reach the higher ones.

My handy dad built these potty stairs and now Bella can go to the bathroom independently without a floor potty!

Bella has passed her first level of Swimming Classes and is looking forward to conquering the next level in July. She has also completed two Gymnastics classes! The strength she has acquired from these extra curricular activities have proven to broaden her movement,flexibility and strength so much.

Bella has continued to love Daycare and looks forward to starting Pre-K in the fall. She also shows her excitement for my ability to now substitute teach at schools. She is so excited at the thought of me teaching at a school because she absolutely loves school buses, she gets pretty excited when she sees one. Every time I work she asks if I got to ride the school bus to school or if I even just saw one while I was there. ha ha.

Bella loved her first hair cut at the salon! A lot of children with CHH (Cartilage Hair Hypoplasia) deal with having little to none or very very sparse fine hair. Part of Bella's wait on her CHH diagnosis was not just due to her being so proportional for having this form of dwarfism, but for the first year of her life, the reason why we thought she had no hair was because of all the times it was shaved off for head IVs...and then what little hair she did have by the time she was 1y and 3 months, was taken away with chemo. Also, her official CHH diagnosis did not come until 5 months after that and then we began to understand why her hair didn't grow as fast or as thick.
Many children with CHH can qualify to be given wigs and hair pieces. For now, Bella's hair is doing quite well, it was growing enough that it needed a trim! But if one day a hair piece gives her the confidence she craves, she'll be able to have that option as well.

 Bella adores all her grandmas and grandpas so much. Here she's hugging my mom's leg after my mom came over for a visit and Bella didn't want her to go.

 Easter fun with cousins!

Easter Games at Daycare.

A Special Thank You to All Saints Catholic School in Swift Current

Recently the staff and students of All Saints held a fundraiser along with a Dessert and Drama night for Bella. This school community was there for Bella during her last major bowel surgery back in January 2017 and continued to cheer her on through recovery and the steps after that. Their understanding of what Bella has gone through and continues to face is an amazing love and we can't put in to words how thankful we are for their support for Bella. We love you and we thank you All Saints.