Day +48... Domino Effect

Today was our first clinic day in six days. Our longest "stretch" not being at the hospital since February 1st. In a way this is great. In another way it creates anxiety in us about what we might be missing etc. Sam's numbers as far as her "blood counts" continue to look good. Hemaglobin is holding steady, platelets are holding, and WBC are stable in a safe range. This is all good except that we are still on steroids. It will be a more true test of her ability to produce and hold all three lines once she is completely off of the steroid for some time.

Unfortunately that may not be for awhile. Last week after clinic her mystery rash returned and we "restarted" the previous antibiotic we had stopped for about a week. This time she was definitely more bothered by the rash and would cry, and shake her head back and forth saying "itchy!". After re-starting the antibiotic on Thursday, by Monday it was clearing up again. Late Tuesday night though she started having bad intestinal issues and this has continued over that last two days. Diarrhea around the clock is no fun for anyone. So today they tested her for C-Diff which she has had twice before as the strong antibiotic that she was put on for the rash puts her at risk for infections in her gut since it is killing all "good bacteria" as well. So far that rapid result came back negative but a full "stool culture" result can take up to one week. I am hopeful it will be something we can identify not only so we can treat it properly, but also so that it is not indicative of GVHD (graft vs. host) disease moving to the gut/intestines. We have decreased the antibiotic to 1x per day instead of two and will return to clinic Monday.

At times it definitely feels like we are "chasing our tails" with a domino effect of one drug causing a side effect leading to another infection or the need for another drug etc. This can be certainly frustrating but I do feel like the docs are only giving her what "makes sense at the time" and not trying to overload her despite her intense continued med regimen. One of the side effects of cyclosporine which is a strong med she will be on for at least 6 months is increased hair growth and making hair darker. In this past week, Sam's eyebrows have significantly darkened for sure! It is very noticeable to us and in comparison of pre-transplant status. Her cheeks are also starting to swell from the steroids despite the fact that she hasn't gained an ounce:) None of these cosmetic things matter, but it is a visible reminder as a parent of the intense change your child has gone through and makes you wonder if these visible changes are happening, what other sort of side effects are going on/or are to come that we can't "see" from the strength and constant meds given at such a young age. These thoughts come and are quickly pushed aside though as you know you don't currently have a choice so the intense worry is useless energy. You ask as many questions as you can. Make sure you find the best docs that provide consistent care week to week and get to know you and your child. And from there, you have to start to trust... Not easy, but it's the only way.

Overall I know things really are going fairly well for Sam as a day + 48 bone marrow transplant patient and for that we are incredibly grateful. She really does laugh a ton and has an incredible sense of humor. Every day she is home she has fun most of the time. Regardless of numbers, meds, or symptoms, that is something to celebrate
for sure!!!

Oh Steroids... Necessary Evil!

So Mondays clinic visit was our shortest yet! Under three hours! SAMs numbers have all skyrocketed which is so crazy to see! When you live for months with 0 or almost 0 numbers constantly worrying how far to push until the next transfusion, numbers near a normal range almost seem unreal. The docs warned though that this is a positive side effect for Sam of the steroids and if we can safely taper down, her numbers will drop a bit but they really should stay stable enough at this point to be transfusion free.

So I asked bedsides graft vs. host , what other things could the steroids be holding at bay that were eating up her platelets before. When the answer came, I immediately wished I hadn't asked. A "long list of other autoimmune processes/ diseases" was the answer. One of those "whole other can of worms" responses we have gotten along this path that opens up new possibilities you never want.  Since we don't know what caused Sam's aplastic anemia in the first place, we have always been nervous that whatever this underlying process is could return. Post transplant, aplastic anemia patients are ar higher risk for the disease to clonally evolve into things like MDS, PNH, and other cancers. But many AA patients go on to live healthy, disease free lives. This is obviously our hope for Sam but the fear of "something else" coming along is truly debilitating at times.

So for now, we are down 25% on our steroid dose. If numbers look ok Thursday, hopefully go down a bit more. Some kids have trouble tapering off steroids with muscle/ joint pain, headaches, vomitting. That's the reason for the taper in addition to monitoring to ensure her platelets don't start dropping again. I will be happy to be off these hopefully in a few weeks though as the mood swings, night sweats, trouble sleeping, and hyperactivity is certainly a challenge. In all honesty though these are tolerable but the long term effects of the steroids are what scare us more. Growth problems, bone brittleness, joint damage, anxiety issues etc. No one really knows how long it takes for these sort of side effects to take hold, but with a little growing body that has been poisoned enough, if her numbers can stabilize without, we can't wait to get these off our mile long meds list:) PS despite eating better, the docs say she is their first patient that has so far not gained any weight on these... Of course! She really is eating more but I think it's just that she's going a mile a minut always and can't keep it on!

We have continued to receive so much love and support in all forms! Old friends and high school swim/dive teammates of mine organized an adorable gift box for the kids! Kaeden hasn't stopped coloring in his new super hero workbook for a week! Gramps visited and we put him to work around the house per usual and he actually got to eat some decent food for a change with the donated meals from the school group members! Now our extended family can also send their appreciation for this kindness because they are saved from my cooking:) We have tried to start our thank you notes and I hope those have been received as we roll them out. PLEASE know if we somehow miss something along the way or something seems to go unnoticed, it's not! Everything, no matter how small you think... Texts, emails, blog messages, voicemails, magazines, rides, cards, books, food, play dates... it all has been so incredibly thoughtful and we couldn't be where we are without this support.

Again hoping for steady CBC (blood) numbers while also tolerating a steady taper of steroid! Until next time...

Biopsy Results...

SAMs bone marrow aspiration showed both "mature and immature" cells. The doc said she was very pleased with the cellularity and it showed that Kaedens marrow is making new cells in her body. This is good news and nice confirmation for sure.

The skin biopsy showed no indications of possible infection as the cause of the rash but was generally inconclusive as far as if it is definitely graft vs. host or not. This was a little unsatisfying as we were hoping for a more definitive answer but at least it wasn't a harmful infectious cause.

The plan is to keep her on the steroid until Monday and evaluate her lab numbers and rash. If all looks ok still, we will slowly start to taper down the steroid dose and watch to see if rash returns or platelets begin to drop again. If they do, then we will need to increase again and they will have to try to again decide what is "eating up her platelets" that the steroid is keeping at bay.

The only other conversation going on is about SAMs ebv numbers. Ebv like cmv etc. is a common virus that many of is have in our systems that our bodies are immune to. EBV can cause lymphoma in transplant patients though which is obviously something we want to avoid. Right now SAMs ebv numbers have risen from the 10 thousands to the 30 thousands. If she reaches 100 thousand, she will need to have ct scans of her body examining her lymph nodes and then receive at least four weeks of an iv medication that can damage her kidneys. Again, we are hoping it won't get to this point and her numbers will hold steady, but these sort of things are always being monitored and watched. I told

So far it's been a quiet weekend besides a few stitches on her back that annoy her from the biopsy and a puncture mark and bruise from her now third bone marrow biopsy, she really has been doing quite well. Going to the bathroom is still painful for her which is difficult but all in all we are chugging along and hope to decrease steroids Monday.

Procedures, Procedures... Day +34

Sam returned to clinic this morning and while the rash had improved, it was not gone so doc wanted to go ahead with plan for skin biopsy and bone marrow aspirate. Her lab numbers were much proved today though. So much so, that I asked if we should re-run them to make sure they weren't mixed up with another patient (this did happen to us once during transplant). The doc said she thought it was because of the steroid though which was "fending off" whatever she thought was eating up her platelets. We should have results from both biopsies by tomorrow morning at the latest. We are supposed to continue the steroid until Monday clinic visit at least which has so far not produced any hopeful benefit of increased appetite but does make her more dizzy and irritable. Seriously, not something a toddler needs additional dosage of:)

Sam's transition into and out of surgery was not as smooth today as in the past as she is building a tolerance up to the iv anti-anxiety they usually give thru her picc line. Really hoping this is the last procedure we will need for awhile! The only other changes are decreasing one gvhd prevention med from twice per day to one and we get to stop one antibiotic tomorrow. I only mention this because the amount of meds Sam is on is insane. Below is a picture of her "morning meds". We call this her "pre breakfast" of champions:) So anytime one is even decreased in frequency, it's a son for us. I hate Sam having the g tube, but have no idea how we would get all that is needed in her otherwise. Today her chemistries showed slight dehydration and low magnesium but hoping the dehydration is just from not drinking much before the surgery. If she is eating enough and we can decrease her meds enough, the doc said we can talk about getting rid of her g tube at day + 100. I am all for it as long as we aren't staring at this "line up " two to three times per day!

Hoping for a quiet weekend, great bone marrow results showing tons of healthy baby cells, and a as harmless as possible rash explanation! 

Mystery Rash! Day +32

Turns out Sam's rash is not shingles like they thought last Wednesday and so far negative for all viral panels they've run and not responding to antibiotic. Today at clinic, we had another platelet transfusion and will now start a trial of an oral steroid. If the rash is graft vs. host disease but just atypical in presentation, this should show improvement. We were hoping to avoid steroids if possible because of their side effects but we are starting with a relatively low dose. They cause increased appetite which is probably good for Sam, but also intense mood swings and swelling of her cheeks/ head etc. We'll see how it goes but if her reaction to steroids during chemo is any indication, could be a rough few days:(

If rash is not improved by Thursday, she is scheduled to have a skin biopsy and a bone marrow aspirate procedure. She will yet again be put under for this (sixth time I think in a few months?). They can then run more tests on the actual skin sample for infections and look at the marrow to see if their are "baby platelets". If there are, this means her body is producing them but something is eating them up. Docs just feel she should be showing a more positive trend by now and holding onto platelets longer than she is. White blood cell line and red blood cell line appear more stable which is good.

I hope the steroids work with minimal side effects and we can avoid Thursday's procedures, but I do appreciate the doctors being diligent in looking for answers proactively and not just taking a "wait and see" approach.

I hope everyone had an awesome Mother's Day weekend! Although not at all how I would ever imagined our life to be right now, I was truly thankful to spend it at home with Brandon, my mom, and both kiddos. It was another day I couldn't be more appreciative of my own mom as I had a touch of some stomach bug. It's a really delicate balance throughout life to be a good friend AND a good mother, but our baba is both to me and our while family is just so lucky to have her around. She is our rock and life boat in times of need and somehow, always helps get me back to shore!

Also thanks to all my mommy friends and such an awesome group of mom's at Kaedens school! The continued organized help with meals, rides, cards, conversations, and gifts is amazing and gives our stamina a much needed kindness boost:)

Fingers crossed the steroid works and our little angel isn't a TOTAL crazy drama queen for the time being:)

Day +28 "I Hate Coming Here"

Yesterday was another clinic day. Docs think SAMs rash is shingles and so now we doubled the dose of her antiviral med and give it to her four times per day instead of two. They said this is common for immunocompromised kids and things like this will crop up throughout the year ahead. The red bumps are mostly on her cheeks, neck, and the back of her head. It seems to only itch when she is trying to sleep and her bald little head gets sweaty. She also received her monthly iv antibiotic of pentamidine which helps protect her from one dangerous type of pneumonia. This causes nausea and vomiting but this was the first clinic visit Sam didn't get sick, so that's a small win!

Her labs looked pretty good although we expected that given she just received platelets Monday and red blood a week ago. Her ANC was up a bit from Monday and was 1.2. The other good thing is her "chemistries" were mostly all within range as well. These measure her electrolytes (sodium, magnesium etc.), nutrition (protein, calcium), and markers for liver and kidney function. Her liver numbers were elevated Monday but looked better today. This is great because Sam has been eating better and I haven't had to pump as much for supplemental feeding thru her g tube which is helpful because now that we are home, it's virtually impossible to fit it in :) I'm hoping we will stay on this track of starting to enjoy food again! Her tastes have changed a bit which they said is a common side effect of the chemo so we are just trying to learn what the "new Sam" likes now:) So far hummus is a huge hit:)!

We got home after only five hours yesterday which was a bit better than the typical six. Wednesdays are the worst because Sam is due for her picc line dressing change and she screams and cries through the whole process every time. It is painful and scary and requires multiple nurses to hold her down every time. This is after a throat swab, typical blood draws, about five sets of vitals, and a swab of her bottom. I feel more anxiety on these days than almost any other because it's completely unavoidable and does not get any better no matter how many times we've gone through it. It's hard for us to fight back tears and the nurses hate doing it, but it gets done... One way or another.

As we were finally leaving in the elevator and Sam's little eyes were peeking out of the top of her  mask and she was sniffling still from crying so hard after her dressing change, my eyes met the eyes of another mom who was holding a little boy with his head resting on her shoulder as well . He looked about 3 and was wearing a mask like Sam. I have no idea his diagnosis, but his bald head, slight frame, and the fact that he was also leaving the cancer/ blood disorders floor let me know he hasn't had an easy road either. He looked up and said "Mommy, I'm sorry but I hate coming here." His poor mom looked at me and we just gave each other knowing smiles. At that moment, I felt the exact same way and realized that despite the hardworking and kind nurses, docs, and volunteers on the 18th floor at Lurie's hospital, after four months of spending at least three days per week there (and about 45 days total in inpatient) I felt like saying "I hate coming here too!" Here's to hoping next week or the week after we can be decreased to two days per week, SAM stays virus free, and we need fewer or no transfusions!

Home Sweet Home!

So Friday night Sam and I returned home. She was literally smiling and squealing in the car with Kaeden as we drove home. Everyday for the past month, at least once per day, she would point to the door and ask "car?" It was great to finally be able to say "yes!" Walking out of that room was a wave of emotion but my anxiety started to ease seeing Sam smile ear to ear.

 Overall she had a pretty good first weekend back at home. She is still vomiting frequently and having some pain with diaper changes but overall, she really seemed happy!  It is even more obvious though the physical changes she has undergone now that she is back in a familiar environment for visual comparison. She just looks so different. She has lost five pounds since her diagnosis which for a little nugget is almost 20% of her total weight. A lot of this loss was muscle and even though she is walking now, she is very weak still and is a lot more unstable. Her balance and coordination are greatly decreased and Kaeden says she looks like a "little old lady" now hobbling around and having to steady herself to bend over etc. I hope with time this strength comes back on its own with everyday physical activity and we can avoid spending more time at the hospital for physical therapy.

Today, we are at "clinic" where she is receiving a platelet transfusion. Her doc doesn't like that her platelets and ANC are down from Friday and so drew more blood work checking for viral infections. She has a rash on her cheek and back of her head but they don't think it looks like graft vs. host disease and are leaning towards a viral cause.

Some people have asked including Kaeden now that we're home is it "all done?" I wish I could say yes but the answer really is not yet. We will come to clinic three times per week for continued monitoring and transfusions two to three times per week. These are long days (4 to 6 hours) and in some ways tougher than inpatient days. Graft vs host disease can really crop up at any time but an average time is day + 30. Also graft rejection can unfortunately still take place but this chance drops greatly at day +100 so that is our next goal.

She can not go in public (grocery store/ target/ park/restaurants)  but we can take her on walks in non crowded areas if she stays in the buggy. If she gets a fever, we still have to head to the er and she still has dietary restrictions to limit possible bacteria exposure as well. She can walk in the grass with shoes but can't touch it. She also can't play or touch sand or dirt. So most of our time will still be spent solo at home with limited visitors but again, home is much better than 30 days in the same hospital room!!! Most of these restrictions are lifted based on her progress and ability to come off specific meds which we've been given a 6 to 12 month window. We're obviously hoping for the six month mark!  The house was steam cleaned, evaluated for mold, and a uv filtration system was installed while air ducts cleaned. Kaeden will wash hands, shower, and change clothes every day as much as possible  before seeing Sam but sometimes this gets tricky.

Now that we're home, blogging has actually gotten tougher but I will try to keep up as much as I can. I can't say enough about how helpful the extra meals have been now that I'm able to experience it first hand at home.  Not having to plan meals, get groceries, and make meals, on these long days at the hospital makes all the extra things she needs for her care, possible. Despite her physical weakness, SAMs spirit is strong again and as she spends more time at home with Kaeden, daddy, and baba, we know her laughter and giggles will give us enough strength to keep on chuggin! We have started our thank you notes and I'm humbled to say the stack is quite tall, but we will get to them one by one as so many people near and far continue to reach out in the kindest ways.

Here's a pic of Sam at home and I'm hoping to see if I can also post a video of a great "giggle fest" with her brother! :) Music to our ears!

Day +21 Excitement!

SAMs ANC has continued to slowly rise and is now above 1.0 which is fantastic! Over the last few days, we have transferred almost all of her meds from iv to oral which for her means "g tube" and we are now facing the possibilitilty of going home tomorrow! Her platelets unfortunately were a false alarm of recovery and have now been dropping steadily again but this is not atypical of transplant as they are usually the last line to recover. Unfortunately this just means more transfusions and more worrying as they drop to avoid any bump or bruise! Tomorrow she will most likely receive a platelet transfusion in the morning and then they want to observe her for any adverse reactions through the day. She has been getting a red rash all over her body with red blood and platelet transfusions since day 0 but so far no other adverse reactions-which is good. They pre medicate her with Benadryl to help with this.

SO as the prospect of going home looms I feel an utter mix of emotions as I look around this hospital room that we have spent 29 straight days in. On the one hand I want to sprint out of here and never look back and play four straight hours of leggos with Kaeden hugging him until he can't take it anymore. On the other hand, I'm petrified to carry little Sam out of this isolated door where she has been monitored and hooked to machines 24 hours per day, with pre measured meds brought around the clock, feeds administered they her g tube throughout the night, blood and nutrition labs done daily to track every change... At times you resent this constant interjection but now I also realize how much anxiety I will feel initially without it...

Now it will be up to us. Measure and track all nine meds are given two to three times per day. Make sure if she vomits within thirty minutes to re-administer meds. Change feeding bags, tubing, and pump throughout the day and night to give nutrition. "Hep lock", flush , and clean her picc line everyday and ensure it never gets wet. We will initially be seen in the outpatient clinic three days per week so I know we won't feel totally on our own and they will closely follow her progress.

But just as we were scared but anxious to start the process of getting to transplant initially, we are now scared but excited to get home and get into a groove with our new daily routine and responsibilities. I'm sure we won't be perfect, but I hope we can do what we need to keep Sam as healthy as she can be to now reach our next goal of day +100.  This is a big milestone in the transplant world equating to many decreased risks.

Whether it ends up tomorrow, the next day, or the one after that, I know we leave here stronger than when we came. Thanks to Super Kaeden ("kee kee" as Sam calls him) Sam has decreased pain, a growing immune system, increased strength and stamina (she's walking more), and most importantly... her smile back!!!!

So we bid our "prison of hope" (Jon Ackley :)) farewell and continue to the next chapter of this journey. Thanks to EVERYONE who has helped us come this far! Really feeling Sammy strong tonight!