Day +186 • Back To "Our Normal"

Stunning...

I just can't get over this photo I took and created. I just snapped it after a usual bath time. This is one of my most favourite moments in the day with my sweet girl...when I snuggle her all up in towels after a bath.

She's happy and doing so well this week. 

•Bella has now spent 529 days/nights in hospital, total, from birth.•

Summary of Bella's Recent Hospital Admission: 

25 days

Aug. 24th - Sept. 18th 2015

Unit 1 at ACH

1st week: fight off a Micrococcus bug infection of her blood. 

2nd week: Caught Norovirus and fought that off.

3rd week: Find out how to deal with the new diagnosis of an extremely rare bug called NTM-MAC. 

4th week: Work on balancing her fluids, lytes, potassium, sodium, urea, creatin, etc. 

In those 25 days Bella had underwent: 2 CT scans, 2 blood transfusions, 2 ECGs, 1 ECHO and 28 blood draws. (Thank God she has a central line for all those blood draws eh.). And all of that because of being admitted for a fever, infection and virus. Side note: I don't think Swift Current could not handle any of that. A tiny eye-opener as to why we are still in Calgary. But we'll get to the 'when we are home' discussion later. 

Even though we are out of hospital and back to the Ronald McDonald House Bella's doctors continue to check Bella's electrolyte levels and sodium levels. Her fluids have been really hard to manage/balance. This is because of a few reasons: her kidneys have been damaged and don't have reserve, aka- they need much more time to get over episodes of viruses and antibiotics, Bella's has no colon, she's on a harsh post BMT immune supressent med called Cyclosporine that causes issues with almost everything, and she is still recovering from the Norovirus. 

Even though we can strictly manage Bella's fluids via her Gtube in her tummy, her lytes and fluids have been tricky to balance because of all those reasons I just mentioned. But we are getting there. She's feeling great and the docs are checking her levels twice a week. It was a rocky road in hospital after she had puked the one day, then wasn't allowed to eat, was on IV fluids, etc. So she needs way more time to get back to her "normal" and balance her fluids. 

I was quite upset because her eating and drinking skills were so amazing right before the admission. Those definetly took a hit and she has to work hard at getting back to where she was again.  She'll get there. 

When Bella is admitted in hospital it's bittersweet. It means she's not at her best, but it also means Lyle and I can enjoy breaks together. While Bella was naping we had lunch on a downtown patio during a beautiful afternoon. 

That New Rare Bug

So it is a big name and that's why I use the shortened version: NTM-MAC. In a nutshell this extremely rare bug that the doctors never see was found to have grown from Bella's recent lymphnode biopsy surgery. This bug would do nothing to you or me because our immune systems would not let it harm us. This is why we never hear of it. So Bella's doctors are fairly certain she had caught it before we even knew Bella had SCID, way before her bone marrow transplant when she had no immune system to fight against any bug. This MAC bug could only harm a person who's immune system could not gaurd against it. 

So what do we do about it? Well we support Bella's growing immune system with 3 antibiotics for a year. And a year once her immune system is actually fully working. So Bella will actually have to be on the 3 antibiotics for over 1 year. We are hoping we see her system rid the bug before then but her doctors tell me this is the course for this bug and these drugs. I'm still not sure if they will still do surgery to cut out more of Bella's larger Granulomes (large nodes). Her doctors are currently just watching to make sure the Granulomes shrink and not increase in size. The antibiotics should be enough to rid this bug from Bella's system.  Right now the nodes are like bumps under her skin and I can feel them on her skull behind her ears. So far these 3 antibiotics are proving to be not too harsh on Bella. She does not have diahrea from them but I'm wondering if they are making her urine harsher on her skin. Poor girl has quite a sore bum but not from diahrea. I will have to ask her docs about that tomorrow. 

The Night of No Sleep

You may remember my post on Bella's Facebook page from Friday, Sept.18th when we were discharged from hospital... Well sort of. We weren't actually out of hospital until 7:30pm and then back in a 1am - 4am because of a fever.. So we thought. Yes, I have a newer, better thermometer now. Ugh. I was concerned with Bella's temp that night and what woke me to check it was her grunting with breathing. She also felt hot all over. However after the docs checked her out and took blood cultures nothing ever came back and her fever was gone. Not sure what happened that night but I must say this grump bear may need to send the unit 1 nurses an apology note for the few that had to deal with quite a battle-axe that night. It's was a perfect storm. A month of little to no sleep in hospital followed by a night of worry and no sleep the day we get discharged! I was so upset and stressed. I'm glad it ended up being nothing. 

Bella's team takes the slightest fever or issue with Bella VERY seriously because she is a SCID case, which just has had me on edge. For Pete sakes they kick other patients out of the unit to make room for Bella! She's literally VIP on unit 1, no joke. Even now, I must call even if Bella's amount of stool increases in a day. Any little change, they demand to be on top of or have her admitted. It's wearing me out a bit, but I'm remaining strong. As much as I don't want to have her go back in to hospital, it's good to know her BMT team of doctors takes everything very seriously to keep Bella safe. 

Forever Daddy's Princess 

6th Month Immune Panel Bloodwork

Even though Sept. 25th 2015 marked exactly 6 months from Bella's BMT, they drew her panel on the 15th. I hope to hear about the full results by the end of this week or next week. They take 2-3 weeks to come back. So far all Bella's counts remain stable and increasing. The question is have they increased enough for us to set a home date for Swift Current?  Ie: Is her immune system proving its working well enough for Bella's doctors to be comfortable sending her home, 6 hours away. Well the doctors are hopeful because they have already made a couple huge decisions that have us really happy and excited. 

First they started weaning Bella's main BMT med: Cyclosporin, the one I just said causes issues with a bunch of stuff. This is a HUGE deal! Weaning this med is calculated out at 10 weeks until completion. Now that the weaning has started we don't have to draw levels anymore either! If you remember, the 9 days before Bella was admitted they were drawing (blood) levels every day because this med has been such a problem. No more of that! Accessing her line that much causes risk for infection. No more of that! This is the med that is still suppressing Bella's immune system so her new cells don't come in too fast but instead just the right speed. Once this med is done, we really see Bella's immune system working entirely on its own!!!  So when his med is done that would bring us to the end of November. And it takes a couple months to completely leave Bella's system. So we would see Bella's immune system fully working on its own by Feburary 2016. She would still be very fragile as at that point she will be 2 years and a few months old without any immunizations yet. 

 Once this med is done that means a bunch of the other 8 medications Bella is currently on are also done! And the most exciting news about weaning this med is that her doctors have said that they are comfortable with us being home for the remainder of the wean, which would be in 4 weeks. Therefore, there is a good chance we could be home by the end of October. BUT, there are a few other major things that need to fall into place before then, one of which being a 9th surgery for Bella. 

Before We Go Home 

A few things need to be looked at.

1. Weaning Bella off this major Cyclo. med needs to go well. She must not develop any rashes, sickness, etc from coming off this medication that she has been on since November 2014. 

2. Bella must have a 9th surgery. Bella's doctors are not comfortable sending Bella to Swift Current with out a central line aka: her Broviak sewn into her chest. However, this line poses risk for infection. But it is also life saving incase of an emergency and provides easy access. So they are converting it to what's called an Ivad Port. Instead of lines dangling from her chest, the port sits like a little button under her skin. Much less risk of infection and it'll will mean Bella still has a cvc line for blood draws, monthly ivig infusions and any line access that may come up. The Ivad port is also great because then I don't have to cover Bella's entire chest with a bandage dressing or Saranwrap for baths! 

Ivad port

This photo shows 3 types of cvc lines for blood draws. The picc line in the arm, the Broviak with lines dangling out of the chest (what Bella has now) and the Ivad port button above the heart (what Bella will get). We still need to find out Swift Currents experience with ports. The nurse would still need to poke through Bella's skin to access the port pad but numbing cream on the area can help and it is quick access. It would never involve fishing around for a vein while Bella's crying in pain. 

3. The 9th surgery will also involve Bella changing her long Peg Gtube to a Mic-key button. This would mean she would no longer have the long tube dangling from her stomach either. Instead it would be a button on her belly with a little cap. Switching from the tube to a button is actually usually bedside procedure, meaning I could do it. But not for Bella. Her surgeon who knows her bowels well from the previous surgeries explained why this must be a surgical procedure. Right now Bella has a little disk inside her stomach holding her peg Gtube. Usually you would cut the tube, pull it out and let the patient pass (poop out) the soft disk. Bella's surgeon said no to that. They don't want to risk any complications of allowing her to try and pass the disk. So they will surgically remove it.

 The button she would have would look similar to this pic: 

It's exciting to start organizing for home but at the same time it's terrifying. So much needs to happen and it's hard to sleep at night when you have a world renowned  immunologist telling you she needs to be 'comfortable' sending you home to Swift Current. 

I have a lot more phone calls to make. We need to make sure the SC pharmacy understands and can make Bella's Meds. We need to secure a suitable Pediatrician and make sure there is always someone available and capable for Bella's unique care. Swift Current, please don't let me down. You are my home, I want to be there, not here. 

Bella and I just spent a wonderful weekend with my dad. Grandpas are great at projects! Bella's playtable is just too short because she's a growing girl, so my dad was able to make new legs and make it taller so Bella can stand at it. 

From yet another tidal wave of hurt and hardship that has come upon my daughter on this medical journey she endures, comes the anchor of love from our family, friends and even some strangers holding us steady through the storms. Honestly, I don't know if I can say "Thank You" in one way that fulfills how my heart truly feels from all the support we have received recently. 

My cousin Sandra made the Walk of Hope for Bella through her hard work in the Queen City Marathon. She raised money to support Bella. The blessings Bella receives from this support makes my heart full of joy. It brings me relief. 

My heart is full from everyone's generous support and I can't thank you all enough. I've recently had friends I've barely seen give in ways that melts my heart and helps me through the daily struggles. Thank you. Thank you for the flowers, the gifts, the cards, GoFundMe money, messages, letters, texts, clothes, visits... I will not forget any of it, it all means so much to me. Thank you so much to my sister in law's, sister in law who ran in the Rock the House Run for Bella! In Bella's name she supported the Ronald McDonald House. Thank you Trista. And Thank you to a beautiful girl I know, Natasha for the heartfelt support of the Fall Photos for Bella. Even just writing this now brings me to tears. I'm overwhelmed with how much love so many of you have shown for my family. 

I want to say, "Thank You" in a special way. For all of you that have given to us in anyway you can I want express my thanks when I can't be there to hug you, I  can't get out to pick up a card or pay you back. But until then, I wanted to write this for everyone that has supported us in so many ways lately, this is for you: 

"Once Again,

We have reached calm waters.

Many waves of hardship have come 

But your love and support continues to be the vessel that sails us home."

On to day 520 in hospital • Day +169 post BMT

Like many of my blog posts I often can't start typing. I stare at the screen, paralyzed, trying to fathom the pain that's encompassing me, trying to fight that off and put it into words. It's extremely difficult... And then I just stare some more. My mind continues to race and dart like a ball in a Pinball machine. The blur mangles my unfinished thoughts and sentences. I just continue to stare at the screen. And then this comes out: 

It's been an exhausting 2 weeks and 2 days admitted in hospital with Bella and we don't get to leave yet. We have both taken hit after hit, rebounded... just to take more hits. I'm about done, but I'm not allowed to be done. Can enough punches just eventually turn you into rock hard steel? 

This has been our recent weeks in a nut shell: 

Aug 24th- admitted for fever

Fight the bug/ 10 days of antibiotics

Sept. 3rd- (2 days before we are supposed to be discharged) Bella gets real sick and is vomiting. 

Help her recover 

Not discharged on the 5th. / Now she's fighting something else.

Bella continues to have horrible fluid imbalance, continue admission.

Sept. 8th- Find out it was the Norovirus

Talk about discharge once her fluids are balanced on her own.

Sept 9th- Receive horrible news about her lymphnode biopsy results.

Continue hospital admission...

Ya, I'll get to the biopsy news later in this post. I've basically been a mess about it since 3:30pm today (Wednesday). How many rare on rare on rare issues and hits can one little human take. I want to scream so loud right now that security would have to remove me from this unit. 

But then I guess I'd leave you all hanging... So I'll contain my anger for now and continue this post. 

Before we even got to Sept. 3rd, and Bella some how catching the Norovirus, I was exhausted from this hospital admission. Bella was used to her nights at RMH. Though she spent her first 17 months of life falling asleep to hospital beeps, lights, sounds and sleeping sound through it all and right through many diaper changes... That went all out the window once she got a taste of real peace and quiet at RMH for 3 months. So this admission back in hospital has proven to be a feat. Between her waking up countless times and crying for me and me already not sleeping anyway, and getting up to settle her or shut the monitor off or rather rip the thing off the wall and beat it with a bat... I've been a zombie since August 26th anyway.

It's better this week. We have worked out  her being off beeping monitors at night, my mom and Lyle have been around to give me breaks and reboots. But mentally, physically and emotionally I'm still drained and trying to catch up. 

How bout we just throw this into your already shitty mix eh! (Can you sense my Hulk emerging? I feel my purple pants starting to rip)

Bella's Blue Lumin of her CVC (ie: her blue noodle) got damaged, weakened and needed repair. This coming after we were just stressing, praying & hoping to not have to remove her life line due to a bug that contaminated her blood from it... Then it gets damaged. Thankfully this did not wreck it enough either and Bella gets to keep both of her "life line noodles." Thank God. Her yellow lumin is larger and should be used for the majority of blood draws. Moral of the story...please don't reef on her tiny blue lumin for 15min to try and draw blood and damage it. Ya, that happened. I'm really really mad at myself for not waking up in time to prevent this. Our nurse had to do blood work at 6am and I was sort of half asleep half awake so I knew the nurse was drawing blood. After 15min they were still there... I should have clued in and woke up. Then the next thing I know, they are in telling me her lumin is damaged and needs repair. After realizing what all happened I immediately requested it to be made very clear on Bella's chart to only do blood draws from her yellow lumin. 

Sick on Sept. 3rd

From that Thursday until yesterday we did not know what caused Bella to be sick. She had one day of vomits, a slight stool change and then horrible fluid/level fluctuations that her docs had lots of trouble balancing. We now know it was the Norovirus, a common stomach flu bug. She most likely got it from being in hospital. It was reported on the unit prior to our admission and well I never got to take her anywhere else! We only got 2 days of passes and those were spent in our RMH room. Bella's system took a hard hit from this because she is so sensitive. Her doctor put it in simple mathematical terms for me. You or I with normal working immune systems can take a hit of 10 particles before it affects us and we get sick... Bella's system can't handle 1 particle and she's sick. Her kidneys have both their middle fingers up in the air at us right now as we speak. 

Why is Bella sensitive and her kidneys so angry? This is why: Her first 15 months of life were on constant antibiotics, septic shock recovery, countless medications, then the Bone Marrow Transplant, then the large amount of strong Meds after that, then the Micrococcus blood infection (more antibiotic treatment for that) and now the Norovirus. 

Middle fingers to the world kidneys, I don't blame you. 

So because of all that Bella's kidneys don't have reserve when they receive blows like this, like the Norovirus. So that is also why she could not balance her fluids on her own, she needed a lot of support, IV support.  Between Sept 3rd and now we have been trying to balance and manage Bella's: fluids, water, hydration, potassium, bicarb, magnesium, etc. 

What does this mean for her future? Well, like any normal kid with a strong immune system with undamaged kidneys it might mean a day in bed and drinking more water. For Bella, it would mean her and I back in hospital with the IV fluid support to get her back to normal. How ever long that takes. How might it feel to think that every time your child becomes ill THAT means a stay in hospital not knowing how long the stay will be. sigh**

The good news from this was when her doctors told me this is all fixable, her kidneys will recover they just need time. 

And then the hit of new information came today about her lymphnode biopsy results... And it does not look good for her kidneys. But keeping in faith, I hope I'm wrong and they will be ok. 

In the middle of this stressful week I had a wonderful, thoughtful friend send me a Box of Sunshine. Thank you Chantelle. The delivery time was perfect and it was a much needed pick me up. It brought me to tears cause at that moment, with that amount of stress on me I knew I have friends and family thinking of me and doing what they can. I needed that so bad. Thank you again.

Bella requests medical supplies to play with. She could and has had ten different colourful toys around her and she requests a medical item to play with. She says the S sound for syringes, the K sound for clamps. She points at the chords, blow-by tube, bp cuffs, etc so I retrieve them for her, and she's happy.

Bella and Grandma on her last day of pass from hospital (Sept. 2nd) Bella has been on isolation since Thursday, Sept.3rd. Isolation for those of you that forget is when Bella is not allowed to leave her hospital room and who ever comes in must wear mask, gown and gloves. Looking back and realizing now she got Norovirus from just being in hospital, she should have been on protective isolation from the moment we walked in the doors, to protect her. I'm discussing this with her team of doctors today. The Norovirus infection could have been prevented.

Lymphnode Biopsy Results: Round 2

I had to receive this news alone, without my husband, and as soon as all 6 doctors left the room... I broke down into a puddle of tears on the floor. 

And what resonates in my mind is what Lyle said to me right before he left this past weekend. He said, "Hunny, just turn on the Jays game. It'll be like I'm still here."

What I would have given for a Jays game to be on at that moment, in the room after the doctors news. But it wasn't. However  Bella has been enjoying the sports channel being on a lot more often this week.

This scenario has happened too many times and my heart is ripped out of my chest each and everytime. And everytime it's a group of doctors, telling me horrible news, I don't have Lyle with me, no one is around and I just sob. 

A bug has grown from Bella's lymphnode biopsy. It's an EXTREMELY RARE bug. I'm sick and tired of hearing them say, "This is rare, we've never seen this, we don't know how or why." I'm sick of it! Bella has had enough of this and I just can't take another RARE diagnosis. 

The bug is called: Non- TB (Tuberculosis) Mycrobacteria. Or NTM.

As I mentioned in the previous blog post Bella had surgery to remove 2 of these larger nodes from her neck. They checked them for PTLD (cancer) and that was negative. Then before finalizing the report they found a bug has grown, the NTM bug. They just told me this yesterday. I want to go back to when they said these larger lumps are just her New Tcells reacting normally to normal viruses we already have in our systems. No, these lumps have not gone away and what the docs think to be happening is the Tcells are still doing their job but trapping this rare bug inside the nodes and causing them to become these larger Granulomes. Bella's Tcells are protecting her so this bug does not go into her blood system. They are checking for that right now and just took the bloodwork last night. 

Why is this so bad? 

On Tuesday they just finished telling me the smallest hit sets Bella's kidneys back, because they have no reserve. And then Wedneaday they hit me with not only this news of a rare bug she has, but that the treatment would be a YEAR on antibiotics..minimum. They just finish telling me her kidneys can't handle small hits like the Norovirus and need in hospital IV support to recover and THEN they hit me with the news of her possibly needing antibiotics for a year, NOT what her kidneys need. In fact it's the opposite. They needed time to recover not a diagnosis involving a year of antibiotics.

The doctors are hoping to know the sensitivity of this NTM bug by tomorrow. They need this to know exactly what antibiotics to use to ensure it leaves Bella's system and that her nodes shrink and go away. There is a possible 20 different antibiotics to use depending on this bugs sensitivity. I'm praying that it only requires a mild antibiotic. If it's a bad strain of bacteria there is one antibiotic that would basically ruin Bella's kidneys to the point of needing a kidney transplant... We DONT want that. 

They think right now that it may require a minimum of 3 antibiotics for minimum a year. That is also a lot of money. The day I planned on celebrating Bella being off all BMT medications has now transformed into a disappointing day of more long term meds. 

How did this happen? 

They don't know and they may not even be able to tell me. What they do know about NTM is: it's in the environment, soil, water, non-treated water. Etc. Its in the environment but not explainable as to how Bella got it.  Its very confusing to me at the moment but her Infectious Disease docs can tell me more when they receive more specific sensitivities that the bug has. As for now I can't beat myself up over a possible prevention, there wasn't any. 

NTM is even more rare in non-immune compromised patients because you or I could walk outside and get it, but our working immune systems could handle it. Bella's team of doctors have also not seen it in any SCID patients. It is mind boggling how Bella got this bug because she has been robbed of being in the environment! She's been on over 300 days of isolation! Of all people, Bella has been strictly protected and lived in a hospital her whole life. We just don't know how this has happened, but it has. Rare. 

Since discovering this bug that was found in two of Bella's lymphnodes the doctors have been trying to figure out how to best tackle this and get it out of Bella. At one point they actually considered cutting out every node. 

Can you imagine?! She has these nodes on her head, neck, armpits, groin, abdomen... She has many. You can't just CUT out every node! How do you even know if every one of her nodes is infected!? You don't. Hence the start of antibiotics. They can't just cut into her little body to take out all the nodes, but they are still considering cutting out the larger ones. So possibly another surgery. Right now they are discussing another CT scan for Bella (her 3rd) to help with these decisions. 

Prayer: 

My prayer request list for Bella is a mile long but she needs it so so much. Right now I'm praying that this is all just wrong, the lab is wrong, the biopsy is wrong, the rare bug doesn't exist.  I'm just so angry and upset.  But we need to pray that Bella's doctors have the wisdom and knowledge to know exactly what is happening with her lymphnodes and that they can some how know exactly where the bug is and is not and how best to treat Bella. Pray for strength and full recovery for her kidneys. Pray that how ever the doctors need to get rid of this bug that the medications Bella requires will not harm her kidneys or cause any long term damage. 

We will know more on this new diagnosis tomorw after more blood work, tests, etc.

Bella's Current Counts 

Aside from everything else going on Bella's new immune system is still growing very very well. Her counts continue to go up and remain stable during this incline. Her virus fighting Tcells have shown growth! In terms of numbers from her last large panel of blood work, the CD3s have gone from 10-60! Bella's immunologist will be coming by soon to show me the numbers on paper and talk about her continued progress of her growing new immune system. 

Her next big panel should be drawn next week on Tuesday. This will be her 6th MONTH PANEL! This is the one that the doctors hope to see the most improvement on. This is the panel that we have hoped tells us we can go home. They should draw this panel of blood work from Bella next week and then a few weeks after that we will have the results. 

So please continue to refrain from asking me when we are going home. I'll be honest, I despise that question. And like I've said before, trust me, you won't have to ask,to know when we get to go home. 

I also REALLY hate it when people ask me how I am doing. Please stop asking me this. I don't want to answer it and really, I'm an open book. You just have look at my face. I hate to be a grump about it but it's really wearing me out. I know I really can't prevent it just by writing in this blog, but I just wish that if anyone sees what I am going through, just know. Don't ask, just know. Please. I'm not sure how else to deal with this right now. Maybe I'll come up with a jollier response at some other time. 

Thankful News

The Ronald McDonald House sent a Thank You card to Bella's grandma and Grandpa (Lyles parents). As you can see from the card, their friends made a generous donation to RMH in their name. So the RMH sent Lyles parents a beautiful card.

As hard as it is to call the RMH home right now, they are. I go back and foreth from the hospital to RMH right now and it's like a boost that I need each time. The support that RMH has received gives them the ability to feed me when I don't have the energy to cook or the time or money to go and buy groceries. They make this happen for families. By supporting the RMH you are essentially supporting Bella and her family, Lyle and I through our toughest times in life. It means so so much. Thank you for supporting RMH Southern and Central Alberta.

September is Newborn Screening Month

This is mainly a United States thing. I follow the Immune Deficiency Foundation on Facebook and they share tones of valuable information related to Bella's condition, SCID. They share the importance of newborn screening and the USA have almost every state conducting the screening now. I still need to find support in advocating for Canada. If Bella was screened at birth I can't even list all of the painful scenarios that could have been avoided. What's previlent right now is the first 11 months of Bella going undiagnosed harmed her kidneys and caused a year of trauma and antibiotics. Could have been prevented if Newborn Screening was available in Saskatchewan for when Bella was born.   

Here is a link that talks a bit about SCID from the IDF Facebook site: 

https://www.facebook.com/ImmuneDeficiencyFoundation/posts/10153009132131781:0 

Keep sending the well wishes!

Because we are still admitted Bella can still receive the electronic well wishes. These are so exciting for her to receive. And I'll admit, I love getting them for her as well. I am not sure how much longer this admission will last but for now (after the recent news) we'll most likely stay in hospital for another week. So no matter where you are, who you are, please send more Well Wishes Bella's way! 

Her full name: Isabella Thomson

The link: http://www.calgaryhealthregion.ca/ACH/pff/mail.html 

Apple is Funny Mom

A couple weeks ago I caught Bella's beautiful, contagious laugh on camera. It needs to be shared with this world, so I added it to YouTube on my channel. Please feel free to enjoy and share her adorable chuckle. 

https://youtu.be/jQYP2dU8tfM