Thanksgiving without Steroids... Woo Hoo!

So Sam had her first clinic visit in a month this past Monday. It was significant in a few ways. This is the longest stretch we haven't been at the hospital since her diagnosis January 30th of last year! It also was the day after months of tapering, that we finally got the OK to completely stop her steroid dose. This was great news as well. As we have significantly gone down on her dose, she has actually grown a cm this last month! The docs say that is a positive sign at this point as growth really isn't expected much in the first year at least post-transplant due to the amount of chemo and radiation her body received. The last significant piece was this was our first visit without our PICC line. Let's just say, the nurses were joking with me that I would be "dreaming of visions of PICC lines dancing in my head" that evening:) It's really hard to find a vein large enough that isn't "superficial" in her arm without a few trial and errors. It's hard to hold her down through this process for sure and as before she had her PICC line, we always end up with a vein that is not in an ideal spot. This time it was right on the inside of her little wrist so after they draw all her labs, she has to keep in a temporary IV until her numbers come back. A lot of tape and a plastic dome cover is placed over her hand so she doesn't rip it out and this makes it hard to distract her from it so she can play. It's one of those things, that we will just have to deal with from here on out and in the end is a good thing meaning we are moving in the right direction requiring less transfusions/IV medications.

Her counts overall really looked good. Her WBC is still slightly low at 4.1 (normal range is 6.0-12.0) but the doc says this is a fine number given she is still on an immune-suppresent (cyclosporine). For perspective a few months ago, we were at 2.1 and even that puts her ANC (overall measure of immunity above 1000). For MONTHS before transplant she had an ANC of 0 without one single WBC or neutrophil detectable in her body so a little below average is pretty darn good for us! Her chemistries are also at a good stable place and her weight gain while slowed down since steroid taper is still holding fine. The only slightly concerning number is her IGG (measure of passive immunity) was still pretty low even after getting an IVIG transfusion boost one month ago. The docs were really hoping to get to a place were she would only need IVIG every two to three months soon but that number is lagging a bit indicating her T-Cell line is still recovering slowly. This means on 12/22 we will have one of our dreaded LONG hospital days. We will start on the surgical floor where she will be put under for her g-tube replacement (has to be done every four months). We won't be able to give her any anti-anxiety type of medication through her PICC line before this either so I am going to have to try to advocate for a good anesthesiologist that will initially let me in the surgery room again. Then after she is done in post-op, we will have to go to the transplant transfusion floor for another three to four hours of an IVIG transfusion. Unfortunately these things can't be done simultaneously. We really hope this is one of the last times we have to have one of these long and eventful hospital days. In my head, I know we have gotten through worse and that we can do it, but it does bring up quite a bit of anxiety just thinking about it all and I know the days directly leading up to the procedure will be tense ones for sure.

Overall though we really can ONLY be incredibly thankful right now. As many people do, we love this time of year. Spending time with family and really slowing life down a bit to count your blessings is always a great thing. This year, we can honestly say those feelings of being humbly grateful take on a whole new meaning for us. It is my mission and hope that as a family we are able to carry that feeling on a permanent basis after this experience. I know it won't be possible every minute of everyday, but at least a few minutes of everyday seem like a great goal! We can't thank the close family and friend in our lives enough for this past year! I could list and describe 100's of acts of kindness we have witnessed and received. I do however want to briefly thank my mom, our BaBa for everything she's done and continues to do for us. Sam's health, Kaeden's emotional well-being, Brandon's work commitments, and my mental-health would be in a completely different place without her. I remember distinctly staring at my shaking hand as I trembled in the ER on January 30th saying to my mom "they think something is really wrong with her" as we were rushed to the oncology/blood disorders floor for our first night of treatment. The next day my mom was on a plane, she never left before transplant, left her job, and has lived with us ever since. I only hope I can follow this example of selflessness and giving for my children as they become adults and parents themselves. I know I have said it before but "it truly does take a village" and I hope you get to hug, love, and laugh with your village during this holiday of THANKS!

Risks vs. Rewards!

      Sam had a long clinic visit this past Monday with mostly great news. She received her IVIG transfusion which gives her passive immunity. This line of defense is made by your "t cell" line. While Sam's overall WBC has been holding steady on the lower end of "ok" over the last month, her t-cell and b-cell line are taking longer to recover which is not uncommon. The docs think she should be able to go 6 weeks before her next IVIG transfusion and because her counts have been steady, we decided to try to switch her anti-pneumonia medication from pentamadine (once per month transfusion) to a twice weekly g-tube medication. This medication can lower platelets and WBC though so we will have to monitor after a month of being on the medication and make sure her counts stay in a safe range. Her doc has put her on an effective but "conservative dose" and is hopeful she will not have any harsh side effects or drops in counts with this switch.

       This translates in "real life" for us being able to go one month until our next clinic visit which is a HUGE breakthrough for us! Because of this and after a lot of discussion and going "back and forth" we decided to "pull" Sam's PICC line this past Monday. So besides her G-Tube she is now "unhooked!". We were very apprehensive about this decision for a variety of reasons. On a practical one, now Sam will have to be "poked" at least once at every clinic visit and keep a line in her arm when receiving IVIG which will be taped down and not as "secure" as her PICC line, so transfusion and lab days will be a lot more difficult. She has gotten to the point where she honestly has enjoyed seeing her favorite docs and nurses at clinic and there has been little "pain and fear" involved besides her dressing change. The dressing change has just recently gotten slightly better as we switched to a different brand so it was a hard call as to whether the "poke" once every 2 weeks or so OR weekly dressing change would be worse given her improvement in the area. The other somewhat "irrational" fear is what if she regresses/gets a virus/ we end up in inpatient and she needs to be treated for a "bump in the road?" We will then have to place a new PICC line and removing this one almost makes us feel like we may be "jinxing" the steadiness of the last month or so. Again, this is not based on any logical medical thought process, just "mommy worry" so the rational side won out obviously! Sam's skin is very raw and sore where the dressing/line has been for over six months. She has another pressure dressing on it for 24 hours and then after 48 hours, if it has scabbed over, we can leave it uncovered. It took her about a week or so when her PICC line on her other arm was removed to feel more comfortable with her arm exposed and for the skin to not cause any discomfort so I think this time will be similar. This allows Sam to have a little more freedom in the bath (although her G-tube can not be completely submerged but can get wet), and allow us to get rid of weekly home care nurse visits for dressing changes, daily cleaning/heparain flushes before bed, bi-weekly cap changes, and the responsibility for Brandon and I to coordinate and ensure we order and maintain the exact supplies needed/ organize them and keep up with all the care. It also decreases a slight risk of infection and clotting for Sam (although again these risks were very small.) I know this sounds like a "no brainer" in many ways, but given all that Sam's been through, limiting any PAIN or FEAR from her routine care is something we really take as seriously as the medical side.

    All in all, I do think it was the right decision, but it was a weird feeling putting her to bed without having to care for her "magic arm" for the first time in almost a year. One would think it would just be joy, but it is a strange mix of emotions because it was also a great tool that helped her receive needed medications to get us to this point without pain. In a way it's been a "security blanket" knowing it was there if we needed it. She will need to be put under anesthesia in mid-december again for her g-tube replacement and won't have her PICC line to give her meds to help calm her before separation or to provide fluids after the procedure as we are accustomed. If her counts drop on Bactrim and we have to go back to Pentamadine, that will equate to bi-weekly transfusions that are two and four hours in length without her "pain free" magic arm. I am hopeful that this scenario won't transpire but I know at the first few clinic visits without her "magic arm" I will question this no matter what. As I hold her and she screams/cries as they draw labs and IV insertion for transfusions I will momentarily curse myself and think that the home care/flushes/ small risk of infection is fine if I can avoid any more instances of pain for our sweet girl. But like all things she has encountered, I am confident and hopeful that this moment of doubt will pass and we will start to feel more  confident that we are truly on a slow, but finally steady path to full recovery!

       After a busy clinic day, we also will have a busy weekend ahead. There is of course Halloween on Friday and then Kaeden's b-day is the next day. All of these are great/fun things but require us to do some risk analysis! Should we let Sam trick or treat up and down one city block? Should she wear a mask? Should we only go early before it is crowded? Should I hold her on the sidewalk while Kaeden gets the candy? Will she protest and want to go to the door just like him (most likely)? Do the benefits of taking part in this joyous "normal child" occasion at all outweigh the small risks (if we take precautions) of encountering some "bad germs"? And what about Kaeden's b-day party? Do we let her attend his party knowing she will have a blast? But this is indoors and with a large group of kids and so this increases the risks even more than an early trick or treating outdoors? If she doesn't come to the party how do we coordinate set-up and Kaeden not feeling "second" because Mom, Dad, or Baba will have to be with Sam and miss the party?  In the end, we have agreed to "split the difference." We will go trick or treating but I will hold her and touch the candy and place it in her bag. I will wipe down all the candy she and Kaeden have in their bags with alcohol wipes before they touch them and Sam will not hand out candy at the door. She will also not attend Kaeden's b-day party this year and we will rotate Baba and Dad on Sam vs. party duties. It'll be OK!

     Every day, "normal activities" and annual events definitely do require an extra layer of planning and coordination given Sam's restrictions. At times this can make us feel resentful watching a family eat out at a restaurant together, attending b-day parties with siblings, Halloween parades, school activities together, neighborhood events without a second thought. Luckily these thoughts and feelings quickly pass though and are replaced with feelings of relief and gratitude as we know there have been so many times over the last year we were hoping and praying to even be in this position to have to make these decisions. We do know how lucky we are to even be thinking about future b-days and hopefully there will be many parties ahead for sweet Sam!

Another Milestone...

      On October 10th, Sam reached the 6 month post transplant marker! Although these dates are somewhat arbitrary based on overall statistics and averages, it still helps to have these concrete goals and milestones to focus on during a long road of recovery. This one had a special significance as well based on a distinct memory. After Sam's diagnosis and learning Kaeden was a perfect match, we had over a month of "waiting and worrying" while Sam quickly deteriorated. During this time, we started exploring second opinions at Milwaukee WI and Cincinnati. We were "stuck" waiting for a panel of genetic tests that Sam's blood "failed" several times simply because her body did not have enough cells due to the severity of her disease. These are very expensive and rare tests, where the blood samples are sent to a lab in Canada each time and it took weeks to receive each "failed" result. Her blood was also sent to a specialty lab in Boston MA for a clinical trial, but failed to yield results there as well. We moved onto the skin biopsy for results but knew these would take 2-4 weeks also.
       During this two week time frame, Brandon drove to Milwaukee and met with the most well-respected transplant doc for Aplastic Anemia in the country. We had already been in contact with him for several weeks via phone/e-mail and he was familiar with Sam's case, but we needed to go in person for a "formal second opinion" and to make a final decision as to where we would receive her transplant. This doc is a "character" but in a good way. He and I developed a good rapport from the start. He gave me his cell phone number without hesitation and often answered and texted on the weekends etc. He was invested, driven, obsessed even with treating aplastic anemia. He was confident, honest, and straightforward.
          As Brandon drove in the snow and traffic to Milwaukee, I stayed back with Sam because she was not well enough to make the trip. At the time, she was receiving three platelet transfusions per week, red blood once per week, and had stopped walking, talking, and eating. She would have fevers and was hospitalized at least one to two times per week to receive IV antibiotics. I spoke with the doc over the phone as Brandon made his way inching down 94. There was A LOT said during this conversation but I remember one statement above all else. When asked about outcome probabilities for Sam, he stated without hesitation, "IF we can get her to transplant without acquiring an infection and through the six months post, her outlook is pretty darn good!" He was honest. He was confident. He was a straight shooter. Upbeat even, but no one had laid it out that clearly to us before. I was appreciative but all I heard was the "IF." I remember it feeling like such an out of body experience. It was as if time stopped and the floor dropped out from under me. As I looked down at Sam on my lap and stared out the window watching snowflakes fall to the ground, the fear grabbed hold. "IF..... IF...... IF....." I kept saying out loud as I got off the phone. He was an expert, he has performed more AA transplants than anyone else, and he said IF! The reality of the situation hit me and it hit me hard. In the end, we obviously decided to stay in Chicago, but this docs recommendations and collaboration with our Chicago team helped push things along in a more urgent manner and we were very appreciative of this.
         So despite a huge change in her overall status, the six month marker was not lost on us at all. No restrictions have been lifted, her WBC count remains lower than it should, she is still receiving IVIG transfusions to boost immunity, is still taking a slew of intense medications, but overall she is doing "pretty darn good!" There may be some obstacles and hurdles ahead we know, but we REALLY hope that this expert was right and the MOST difficult days are behind us! We will return to clinic Friday and depending on her numbers, possibly again Monday for an IVIG transfusion, but we may be able to switch her from an IV pentamadine (anti-pneumonia) to a twice weekly medication through her G-Tube. This medication can sometimes cause a dip in white blood cell count though, so this will only be possible if her numbers "hold" from the last month and don't take another "dip." Either way, Sam made it to six months! Halfway to one year. Another milestone. Another marker off the list. Another memory of heartache and fear to start to "let go." Everyday, I hope we get further and further away from that "IF" until it's such a blurry memory, that it feels like another lifetime!
        We wanted to also say a heartfelt thank you to family and friends who donated or joined the Be the Match registry in September. During that month, the Villanova football coach matched donations up to 200,000 and that mark was met! We have received several letters in the mail informing us of people who donated in Sam's honor and we are truly thankful.  I can't imagine how scary receiving a bone marrow transplant was 10 years ago and I hope that 10 years from now research and medical advances will allow for even more lives to be saved. Thanks again from the bottom of our hearts for EVERYTHING and ALL of the support over the last year. Go Sammystrong Go!

 

Some Roller Coasters Aren't Fun

After a slight rebound three weeks ago, Sam's WBC and platelets dropped again the week before last. These "up and down" weeks create a "love-hate" relationship with Mondays (our clinic days.) If the numbers are good, we feel a sense of relief for a week. If they aren't great, we worry endlessly until the next week, or the week after that, or the one after that for more promising news. This past Monday, was a "good one" in a sense that her platelets had doubled and her WBC were up slightly. Her "white count" is still lower than it was before starting the steroid taper and this could as usual be a "variety of things." We have successfully slightly raised her cyclosporine level after experimenting with a  "new" method of administering this medication through her g-tube. We worked for hours with her transplant doc last week at different options and are now cutting a PICC line tube at one end and using specific syringes directly into her g- tube "button" without using the extension tubing. I then attach the extension tubing and administer the rest of the meds after this one. So far we have had promising results with this "workaround" and I'm very thankful for the doctor taking the time with us in experimenting with several different non-conventional ways to try to make this work for Sam. She had developed a slight rash around her neck and upper back similar to what she had a Day + 30 and it seemed no coincidence this was happening right after her anti-GVHD drug was dropping to a non-therapeutic level.

On a different note and away from the medical side for a change, this is Childhood Cancer Awareness month and I have come across so many great articles, posts, and first-hand accounts written by parents, doctors, and siblings on the subject. Below is a link to one of them that particularly hits close to home, While technically a different diagnosis, the rippling effect throughout the family felt before, during, and after treatment rings so true. Here is what she shares...

http://communified.org.au/the-emotional-costs-of-childhood-cancer/



We personally didn't blog about our time of diagnosis or the heart wrenching 3 1/2 months leading up to transplant for a variety of reasons. This was a time of crisis and fear and there were truly no words to share. For a long time I didn't even speak to my closest friends because I felt like if I started talking about it out loud, the fear and sadness would overwhelm me to a point where I would not be able to "come back" and pull it together enough to put one foot in front of the other and get done what had to get done. But, this brave woman above eloquently touches upon the emotional toll that is truly devastating.


 While I feel most days we manage our "new normal" fairly well, there are days when the trauma of it all is more evident than others. You can't erase the vivid memories in your mind: The numerous Sunday trips speeding to the ER to get your toddler a platelet transfusion as she bleeds from her ears, nose, and mouth because she can't make it a weekend without. The feeling of powerlessness as you sign documents agreeing to "poison" your child in a way that will bring them tremendous pain and suffering, but the alternative is even worse. The days that turn to weeks and then months as you watch your once chubby and vivacious toddler stop eating, drinking, walking, and talking until they are laying lifeless and pale in a bed hooked up to perpetually beeping machines which you have grown to love because it means she's alive. The countless times you hold her down screaming while trying to convince her you REALLY are trying to help. The gut wrenching conversations with your "well child" as he asks you "when you are coming home?" and if his sister "is going to die?" and you can't truthfully answer "no." The resentment you see in him when he visits for 5 mins. in the hallway as you tell him that he is "just as important" but your actions can't help but somehow tell a different story.  It's not an easy road. I could have never imagined what it would be like and don't expect anyone else to. It DOES change you in a way that any truly traumatic life event does, but we're learning it doesn't have to define you. The "regular" conversations with other people become easier. You start to feel less like you are walking in a constantly dazed state in an alternate universe that no one understands. You start to laugh more, smile more, and live more. There is something uniquely heartbreaking about childhood illness, but something equally amazing about how a child's will to live is so strong. I know as a family we will always have "scars" that run deep from this experience, but we also have two INCREDIBLE daily reminders of human resilience, compassion, and giving. I hope most days our minds are able to focus on this and are clouded less and less with the vivid memories of the past. To all those who continue to follow Sam's story... Thank you... It's a marathon, not a sprint and we feel you cheering her on all the way!

A Transplant Journey... Always learning, growing, and fighting!

     This week's clinic visit was a longer one because Sam was due for her two hour Pentamadine injection to help protect her against specific pneumonia strands. Tack on an hour on the front and back end and we were all tired getting home per usual. The good news is that Sam's platelets rebounded from last week. Her WBC though,  not as much. They increased but ever SO slightly regardless of going down on her colitis medication. Her doc wants to try to start to taper her steroid again this week regardless and hope that her WBC hold or increase next week despite this. This will be another "wait and see" test of trial and error. If Sam can tolerate the resumed taper, we could be off steroids by the first or second week in October! It has been a long taper over several months and I'm trying not to get excited too soon because if her counts drop at all or she starts to show GVHD symptoms, we will need to increase again, but we've come this far, hopefully we can make it to the steroid taper finish line!

      The other good news is they checked Sam's chimerism analysis as well this week. This is a test seeing how many of her cells are Kaeden's vs. her own. She is over 98% Kaeden:) The nurse said the lab at our hospital does not ever state above 98% to allow for a margin of error so this is as good as she can get! Sometimes over time this chimerism number can start to drop and below 90% can start to be worrisome, so we will most likely check this monthly from here on out and especially when we start to try to taper her anti-rejection drugs. We also checked her EBV which is the common virus that can turn to lymphoma in post-transplant patients that we were worried about a month or so ago. This is down a GREAT deal which is also good news (from 40,000 before to 4,300 today). Her CMV and adenovirus (two other scary post-transplant possibilities) were negative also. Her IGG (passive immunity) was low but not at a transfusion level yet. Hoping to make two more weeks before that four hour one! All great stuff!

       The NOT so great news from this week (because there is always some), is that her cyclosporine level was a LOT lower than usual. This is the VERY important but horribly tasting and scary drug for anti-GVHD and rejection that almost all post transplant patients have to take. It has a LOT of side of effects and is hard to find a good therapeutic level. Besides Sam's awesome unibrow and tremendous dark hair growth, we have thus far avoided the majority of the most bothersome side effects and maintained a therapeutic level of around 150 - 190 (supposed to be 150 - 450). Today Sam was down to 54 from 99 the previous week. Unfortunately this has always been a concern b/c cyclosporine is extremely "sticky" and adheres to plastic so her dosage going through a G-tube through her stomach was something the docs weren't sure would work. It seems the "old" PEG tube was made of a different material and I told the nurse today I think the new tube extension tubing is causing this drop because the medication is sticking more. The RN didn't have a lot of suggestions as of yet, so I will do my "Mommy Research" with other moms, call pharmaceutical companies, our homecare nursing company, and anything else I can think of to see if we can get an extension tubing of different material, a shorter length, or a syringe small enough to fit in her tube portal directly without extension. If Sam starts to show GVHD symptoms or signs of graft rejection, we will advocate quickly to just put her under and have the old type of PEG tube placed again. Although it does extend and hang from her body 24/7, she had it before for months and we can do it again if it helps us over the next 6 months hump that she needs to be on the med.

         We have spent the last eight months tirelessly attempting to become experts about this disease, treatment options, and transplant complications. I can honestly say that despite this effort, there are always questions that come up each week that cause us to search for new answers. Among the transplant community, it is common to hear one say "each person's journey is unique and different" and we have found this to be true of Sam as well. Despite the tremendous medical world's knowledge of "general" outcomes etc. so much of this is still educated "guesswork." There is no "typical course" followed by most. So this next week we'll continue to try to learn more, find possible solutions, and FIGHT this next mini-med battle.

Birthdays, School Days, and the "New Normal"....

First, the not fun stuff... Last week's clinic visit started out GREAT. Sam was in good spirits and the doctors were talking about how overall she is doing so well. We were discussing removing her PICC line in about 8 weeks and hopefully getting her off of steroids in 4-8 weeks as well. Then when I got home the phone rang. Sam's counts came back and her white blood cells and platelets were down 50% of where they have been over the last few months. Not in a dangerously low level as they used to be but still not the trend we want to see. The docs are hopeful it is a reaction to the medication she is on for her colitis as this can interfere with the cyclosporine and be somewhat of a marrow suppressant. We will be lowering that medication significantly this week and next hoping her counts rise again. The other option is it is due to the steroid taper which is not great news as it is indicative of the steroid treating some unknown "autoimmune component" and would require us to stop the taper. My last personal hope although not the docs theory is that Sam's surgery for the replacement G tube "ate up" some of her platelets and white cells for recovery/healing. This happened right after transplant and we briefly saw a dip in her numbers so I'm not "just" basing my opinion on my self-appointed interned M.D. license:)

       This past weekend was Sam's bday! She is two years old now and she knows it:) We had a quiet day at home with Brandon, Kaeden, and both of my parents. Sam opened gifts in the morning and we did cake in the evening. She had been specifically "asking for things" for her "birfday" for weeks and she was pleasantly surprised to find all the wrapped gifts in the basement after waking like x-mas morning. She felt special and we celebrated her all day! We took a long family walk along the lake and took some pictures in our "Sammy Strong" t-shirts! Even though it wasn't a "party" with lots of folks around, she felt loved and so it was a successful birthday for sure!

        Kaeden also started Kindergarten this past week and so another transition in our house took place. He was excited and ready to go back to school! Sam loves riding in the car to take her big brother to school as it is one of her few "outings" but she must remain in the car with BaBa while I take him inside. She has gotten used to this routine as this is what we do to grocery shop, target runs., Kaeden's soccer lessons, playdates etc. She stays in the car or drives around while I go inside to quickly get what we need. Not ideal, but again it's our normal and not something we think too much about anymore.

Each night, I lay out the kids bedtime clothes and outfits for the next day. I paused the other night before Kaeden's first day as I looked at Sam's "night time gear." Some of it is normal toddler stuff: a diaper, rash cream, pjs etc. A lot of it though is not. Heparin syringes, sterile gloves, alcohol swabs, green caps, and an ACE wrap for her PICC central line. Myleplex transfer bandage for her G tube and still a long line-up of meds with her attachment stomach tube now as well. Every night this routine takes place and it is pretty much second nature at this point. Sam has gotten SO much better with all of this care and so have we. I remember the first weeks with her PICC line and G tube and how terrifying and overwhelming it all felt for all of us. Sam screamed when we had to do anything and we literally would shake and sweat for fear of doing something incorrectly that could lead to infection or worse.  I feel deeply for the many families that I see join the support group "Band of Mothers Against Aplastic Anemia" on FB each week/month as they are filled with this same overwhelming sense of fear and anxiety as they begin their battles with this disease. While I still feel that fear and anxiety creep in almost daily, the routine of it all has truly become a "new normal."

         As I looked at all the smiling babes and toddlers being carried in their mothers' arms into Kaeden's school on Tuesday, my heart sank a bit as I peered back at Sam through the car window. Her excited eyes looked back at me and then to all the kids walking into the building. It can be sad seeing everyday things that she misses out on, but I know how far we've come and if we keep heading in the right direction, there will be SO many opportunities for her to have these experiences in the future. This gift is not lost a bit on us especially with continued reminders of those lost within the Aplastic Anemia community over the last few weeks and months around the country. Very hopeful her counts rebound this next week and we continue in a relatively "calm" way with our "new normal" which gets easier and easier each day!

A Blessing and A Curse....

       In many ways, Sam's G tube has been so beneficial to her. During transplant, this is the only way she received much needed nutrition as I pumped breastmilk for her. If she wouldn't have had this, she would have only been able to receive TPN (nutrition intraveneously through her PICC line). She did receive some (about a week) of TPN prior to transplant as she had stopped eating for months, but long-term use of TPN can cause damage to your kidneys and screw up your digestive system. This is also how she takes all of her daily medications. Sam has always had a strong gag reflex that leads to vomiting and there is just no way she could successfully take the slew of meds she needs 3-4 x per day without this.

        On the other hand, from the day we received the g-tube, there have been issues. It initially became very infected and was a source of TREMENDOUS pain and concern for the doctors as at the time she did not have any immune system to fight it. Then during transplant, the balloon that holds the tube in her stomach "burst" and the response time and medical response/communication within the hospital caused another surgical procedure at a fragile time in her post BMT recovery (not to mention frustration with the response we received from one surgeon in particular.)

       So this past Monday Sam's new GI doctor who has been following her colitis treatment was scheduled to come to clinic and "change out" her G tube. These need to be changed every 3 months and Sam was past due. We were told this should be a relative quick and easy change since Sam's tube is older than four months, the site should be well established and healed now that she has white blood cells. We would need to hold her down still, but there should be little to no pain and it should be done in 5 mins. Well, that was the plan anyways. After 20 plus mins of holding Sam down, she was vomiting, sweating, and screaming in pain. The GI doc could not replace the G tube and something was obstructing the pathway to her stomach. She was very calm, considerate, and attentive to how this process was going and placed a catheter in Sam's stomach to hold the site "somewhat" open. This was an experienced attending physician and her RPN who apologetically said this had never happened to her in her 10 plus years of experience and that we would need to have the interventional radiologist place Sam's tube again under anesthesia. This is NOT what we wanted to hear as this is the dept. we have NEVER had good experiences with and we really wanted to avoid putting her under for the 11th time since February.

          Sam received her IVIG transfusion in the transplant clinic which takes three plus hours. We then went to the IR dept. and Sam was "taken back" at about 3:30 pm. All while she, myself, and my mom had not eaten since the night before. It was the same surgeon I had formally complained about, but he was a professional throughout the situation and was bending over backwards this time to acknowledge ALL that Sam has been through. Unfortunately, it was also an anesthesiologist we have had before and NOT the one we have had positive experiences with. He is gruff, dismissing, and condescending. He of course is a "separate entity" from the surgical staff. When procedures are "planned ahead of time" I have worked hard to request specific staff that we have had positive experiences with in the past and we can plan to be on their schedule. When it is an urgent situation like this, you have to "go with whoever is on for that day." After calmly but sternly sharing my concerns with just having him rip her from my arms and take her back screaming and hold her down until she is asleep b/c she "won't remember it", I insisted on carrying her myself into the operating room if he would not give her something to "relax her before." Again, I am NOT for drugging my kid more than needed but this is the same drug that they are going to give her in the O.R. anyways before giving her the mask as well, so in my mind it is just a matter of timing. I carried Sam into the room with one of those "full body" zip up white suits and hats on. She was still scared but much more calm than she would have been. I held her as he gave her the propofol into her picc line and laid her down on the table when she was asleep.

         The procedure lasted longer than expected because the surgeon said Sam is developing "clumps" of skin for some reason and her site is closing much more quickly than typical. He had to "rod" a medal line through her site to remove the skin clump and enlarge it enough to place the new tube. "Nothing that could have been accomplished without anesthesia on another floor." He said he recommended her next few changes be planned to be under anesthesia until they are confident that this can be done smoothly and easily without it. This is again the doc who last time thought Sam was "just being dramatic" basically. While we certainly want to make the process safe and minimally traumatic for Sam, we are not thrilled with having to plan to "put her under" again in a few months and the additional drugs that this entails. The anxiety of waiting for that surgeon to walk through the door and tell you "everything is ok and we are waking her up now" truly does not get easier, no matter how many times we've done it. In some ways, it gets harder because you know you are taking a small risk every time she goes under and you keep taking that risk OVER and OVER....

       Sam now has a new type of tube called a MIC-KEY tube. It is smaller and the "tube part" is not always attached to her. We have to open a valve and lock/twist the new tube into place to administer her meds. It is also made of a different material. So far to be honest, we are not sure this is the better option for her as she is very sore from the procedure and the manipulation of locking and twisting the attachment is very bothersome to her. I also think her meds, most importantly the cyclosporine (anti-rejection/GVHD) drug is sticking the plastic more and thus we may have to go up on the amount we are giving her to achieve the same therapeutic effect. We'll see.... For now, we are home and we are watching carefully to make sure the site does not start to look infected again. All in all Sam continues to march on as her smiley and strong self. She bounces back from these long and stressful days much more quickly than Mommy does:)

       Thanks to all for the continued comments on the blog, e-mails, texts, and calls checking in. We really do appreciate all the words of encouragement and read/listen to them all! Sam's b-day is coming up and we look forward to celebrating this courageous and hilarious little girl turning 2!

Finding a Balance...

I have started a blog several times the past few weeks with detailed medical updates but haven't gotten through any of them to finish. Most of the time, I have been falling asleep at night when Sam does leaving little time to get anything done before the hustle and bustle of the morning begins. The previous "unfinished" blogs shared that Sam's EBV numbers dropped briefly, but have now risen again. We have started a SLOOOW steroid taper which is great in the long run, but also has some "fun" behavioral side effects currently. Hoping we can get through this taper with blood counts holding steady and not another colitis "flare up" so that we are off them for good in a month or two! Sam's G-tube needs to be "changed out" and we are trying to find the best way to get this done without putting her under anesthesia, while also avoiding a painful and traumatic experience. She is due for IVIG this next week which means a looong clinic visit which I'm not looking forward to, especially after this past week's visit which was full of hospital trials and tribulations/miscommunications and a nice dose of BS:)

 As Sam and I left to drive home at 5:45pm this past Monday both covered and smelling of vomit (yes she still does this frequently and almost every week during her dressing change:), I was feeling a little "beat down." I had started to mentally "organize" and "pack" for Kaeden and Brandon to go to Myrtle Beach SC this next week after  Brandon has been in NYC for work this past week as well. Although I am SO happy Kaeden and Brandon will spend some awesome quality time together on the beach, I started to feel the bitterness creep in that Sam and I can't go. After Sam and I got home this past Monday night (after sitting in Cubs game traffic:), I went onto the FB page that I joined shortly after Sam's diagnosis to ask the other Mom's in the group (whom all have kids with Severe Aplastic Anemia) how long post-transplant did their children need/receive IVIG. I hadn't been on the group's page in awhile so I started checking some of the most recent postings. My heart immediately sank. There were three stories that brought tears to my eyes and made me feel like all the life had been sucked out of me at the same time. The first story was about a girl who had a transplant a few weeks after Sam. She was just re-admitted to the hospital to battle pneumonia and a fungal infection in her lungs.... NOT what you want! The second was a posting from a parent sitting at her daughter's gravestone with her other two children honoring six months since she passed away 150 days post-transplant. The last story was another child being remembered by her parents and family after passing away one year post transplant due to severe GVHD that infected her organs and lungs.

The vomit on the clothes, hospital challenges, and missed vacations/outings suddenly felt so trivial. Our little girl is HERE! She is now walking, talking, and eating again. She has FUN every single day and is full of energy. This is what I decided I needed to share this week instead. One is always told it is never a great idea to "compare" your children to others while growing up, but sometimes I have to disagree with this. Sometimes you have to be reminded to be thankful and unfortunately sometimes that happens most clearly when you know others are hurting more than you. Although I don't know any of those before-mentioned families personally, my heart breaks for each and every one of them. After fighting SO hard, to lose this battle now or anytime in the future usually seems impossible most days, but I know it's not. Whether we are post 100 days after transplant or two years post transplant, I truly hope that we can find a balance of knowing there will always be health risks and fear, but not allowing this fear to stop us from having fun! Whether that fun is always in our home or an almost empty park... OR someday on a crowded beach ALL together, we WILL find a way to laugh, smile and LOVE! We owe to all the other families who no longer have that chance. We owe it to Sam.

And.... It's BA-ACK...

    So I guess I spoke too soon in sharing or celebrating Sam's apparent improved colitis. This week she unfortunately returned to the diarrhea, stomach pains, nausea, and vomiting. We have started her on a new medication and will see on Monday what they say. Unfortunately, it seems this is most likely something we will be living with "off and on" for awhile. The other factor we are considering is her PEG tube is "due" to be replaced. This typically takes place every three months or so. It is complicated because Sam doesn't have the more typical type of tube used today that is more easily replaced. It can technically be down without anesthesia but it is not pleasant for anyone and I think the docs would prefer to "piggy-back" it into another procedure if possible. Her transplant doc mentioned another lower endoscopy in two weeks to one month if the colitis continues, so we will have to factor in the pros and cons to our options with this one like everything else.

     This Monday Sam will be "due" for Pentamadine which is her once per month injection that helps protect her from specific strands of potentially fatal pneumonia. The next week, she will most likely need IVIG which helps boost her passive immunity again. These will both mean longer clinic visits. We will keep her PICC line (central line in her arm) until she no longer needs these sort of injections/transfusions and clinic visits/blood counts are less frequent (1-2 per month instead of weekly). Like everything, this has pros and cons. Her central line allows for no "pricks" and digging around in veins every time she requires IV medication, or labs, but on the other hand it requires daily cleaning and heparin flushing at home and can not ever get wet which obviously poses challenges in bathing and eliminates swimming/water table play etc. It also requires the weekly cap and dressing changes which are very painful for Sam and potentially poses a risk of infection having an open line running to her systemic blood supply. In a way we can't wait for these things to be "out of Sam," but on the other hand, we are terrified of doing it "too soon" only to have a road bump creep up and have to surgically insert new lines if needed.

     Some people have asked me to better explain why Sam still can't do many things until one year post transplant. My best analogy is that she has a "brand new" immune system like a newborn baby that is still learning how to work properly. Only after a minimum year to 18months post transplant, will she will begin the process of complete re-vaccination. This is a VERY complicated process based on what her body can tolerate at the time and which vaccines can safely be given, but overall like a newborn, we will be starting from scratch. The second element in this equation is that Sam will remain on immuno-suppressant medication (cyclosporine) to avoid GVHD and graft rejection that leaves her vulnerable to many common illnesses/infections until at least 12 months post transplant as well.

    The vaccination issue scares me as she will technically be three and ready to start Pre-School before she would be able to be vaccinated. Knowing that today many parents are choosing to not fully vaccinate their children or on a delayed schedule, but not having any right or knowledge of WHO or IF Sam will come into contact with any of these kids will definitely factor into our decision-making. I always pretty much "stayed out" of this debate as we personally decided to fully vaccinate both kids, I felt that each family was entitled to their own decision. While I still feel this way on an intellectual level, on a more emotional one, I have to be honest that I wish that immuno-compromised kids like Sam who have no choice but to rely on "herd immunity" factored into the discussion/debate more overall. I know this Fall, our family's view of the "flu shot" will certainly be different as we will all be vaccinated less for "our own sake" and more for Sam's and all those we see weekly on the 18th floor  of Lurie's Children's hospital fighting a bigger battle just like her.

Mustering Patience....

       Sam's Monday clinic appointment was her Day +102. This is a great marker in post BMT but for Sam, a bit anticlimactic. If you live over an hour away from where you have your BMT done, you usually have to stay in a Ronald McDonald house or an apartment until this date. This is due to the high risk of serious infection with a suppressed immune system that can statistically take place during this time period. This is another way that we have been "lucky" in a sense during this process. We live less than 5 miles from one of the "better" transplant hospitals in the country. This weighed heavily in our decision when we received second opinions from Milwaukee WI and Cincinnati pre-transplant. If we had chosen either of these options, Sam and I would JUST be coming home NOW. I can't even imagine that at this point! Being in a comfortable and familiar environment with Kaeden and Brandon on a daily basis is definitely some of the best medicine for both of us! 

      At Monday's clinic appointment, Sam's platelets, WBC, and several of her chemistries were "down a bit." I was surprised to see this because "knock on wood", her colitis symptoms have FINALLY been improving over the last week. She has not had diarrhea for the first time in months! Her EBV numbers were also slightly down from the week before which is great. I am VERY hopeful that her platelets especially will rebound next week and that this was just kind of a "fluke". We work really hard on her nutrition but low phosphorus, magnesium, and potassium are unfortunately very common post-transplant. We have been utilizing her G-tube to supplement as well as transdermal approaches with bath salts, magnesium lotions, etc. 

     The hardest challenge at this point is maintaining patience with the process. As summer is in "full swing" and we try as much as possible to "go about daily life," it's hard to not to start to feel "antsy" and frustrated by all of our limitations for Sam. I want to take her to a museum with her brother, let her play outside in the grass/dirt/sand, go on a vacation somewhere, enroll her in a toddler class, even just taking her inside a grocery store would be a start:)! In some ways, especially with her G tube tucked under her shirt, and when her PICC line is covered in clothing, she just seems like such a "regular kid"(minus the bald head and dark unibrow growing from the cyclosprine:) I only wish she could experience more "regular kid" stuff. It almost broke my heart the other day as we drove past The Little Gym in Chicago. This is a place I have taken both kids to during what seems like a lifetime ago. Sam saw some kids swinging from the rings through the window while we were in the car at a stoplight. She made an adorable high-pitched "OOOOH!" sound and said "Sisi PEASE!" meaning, "Sam Please?!". I know she would have a blast there. She is a seriously fun-loving and hilarious little person full of life.... But, for now... car rides, walks in the stroller, trips to the hospital, and a trip to a vacant park is all she knows. 

         In the grand scheme of things, we know that we should be SO thankful for where we are and how well overall she is doing. Spending every minute with her every day is truly a joy, but as she starts to seem stronger and better, it's hard not to ache for more. I'm sure a good Chicago winter will cure us of this "antsiness" though:))) Thank you to all of you who still follow our journey. I can't wait to share a picture of our first family trip post transplant next year!!!

        

Day + 96...

      We haven't written in awhile because life has been even more hectic than usual over here. We had a sewer line break outside of our house due to "the city" doing work throughout our neighborhood. This caused extensive damage to our house in several rooms and two levels. After a tremendous amount of research, pleading, and coordination to get the clean-up/construction work under way ASAP, it then took a lot of brainstorming and planning as well to find a place where Sam could stay during the work where she didn't have to fly to, wasn't a public hotel, and didn't have any pets/other kids germs etc...:) Luckily we are almost done with most of the work and we were able to drive to Brandon's family's house. They were kind enough to find a pet friendly hotel to take their sweet dog too as well!!! Very much a life saver for us!

     Although it was a nice change of pace to get away for a few days, we returned just in time for 8+ hours of clinic on Monday. Sam needed an IVIG transfusion to help boost her "passive" immunity. Although she has an adequate total number of WBC (white blood cells), her b cell and t cell line is still depleted and will be for at least a year. This injection takes 3 - 4 hours and she has to have "vitals" taken every 30 minutes. Her EBV numbers have gone from 6,000 to 12,000 to 14,000 and this week to 11,600. We are still watching these VERY closely and every week I anxiously wait for the phone call on Tuesday to see where we are at. I'm not sure this feeling will ever fully go away. The fear, the waiting, the lump in the back of your throat...

    From there, we had a follow up with the GI doc regarding her ongoing colitis. We are starting her on a new medication and if possible, slowly taper her steroid down more. When I asked the doc, if she can give any indication as to where this is headed, she responded, "that she hoped Sam would grow out of this as her body continues to adapt to its new immune system and that we won't be having this conversation in 3 or 4 YEARS!" Unfortunately, how they typically treat colitis is the exact opposite of what we want to do to avoid "activating" the EBV in her system. Typically immuno-suppressants in conjunction with antibiotics are used to treat colitis and thus the GI doc would want to "up" her steroid and cyclosporine to see if there is any positive impact, but given the EBV is the more dangerous factor and the colitis seems to be getting slightly better or has at least stabilized, we would like to taper both of these down as low as possible to allow her limited immune system to keep the EBV stable or hopefully decrease.

      As day +100 approaches, this is typically a significant marker in bone marrow transplant recovery and we are very thankful that this day has come for Sam! In many ways, it is almost hard to believe this happy, energetic girl is the same one that didn't eat, walk, or talk for months and tolerated an unthinkable amount of pain. At times I feel like I will never forget one minute of the journey and other times, it feels like a blur. Overall though, we truthfully did hope to feel less anxiety at this point about the EBV and the ongoing colitis. For us, this day will feel pretty sweet for sure, but it only makes us more anxious to get to the one year post BMT marker and be able to live a life with more freedom, fewer life restrictions, and less fear! Someday.... Someday...

Up and Down...

Sam's Monday clinic visit showed that her CBC counts are still holding steady. Her "tummy troubles" have continued but seem to be improving slightly with less cramping and pain it seems. We have a follow up appointment with the GI team on July 14th. If there is not significant improvement on her current antibiotic regimen for the colitis, we will have to see what they recommend for the next step.

The "not great news" from the week were that Sam's EBV titer numbers increased from the week before. Not to a "treatment level" yet but still another anxiety provoking call for us. Due to this, we went down slightly on her cyclosporine (one of her immune-suppressing) drugs to help allow her body to attempt to deal with the EBV. We found a study in Baylor TX that is using T-Cell therapy at day +10 as preventative treatment for EBV, CMV, and adenovirus and having some great results. I contacted the researcher from this study and unfortunately Sam would not be eligible for their work, but she gave me the name of another doc in NYC at Sloan- Kettering in NYC who is also doing T-Cell therapy work. T-cells are one of the many complicated, interconnecting lines of immunity defense. In transplant patients, even with a "normal WBC", t-cells do not start repairing until at least one year post transplant.

Our transplant doc here stressed that Sam is not a "t-cell therapy" candidate yet, but that she would want to find an institution that would use Kaeden's t-cells as opposed to a third parties if we wanted to attempt this route in the future. This would help limit late onset GVHD. So the search continues on this front...

Another issue is Sam's magnesium, phosphorus and a few other chemistries have continued to be low. This was not the case a few weeks back and so the docs definitely think is a two fold reaction to the colitis/gut issues involving decreased absorption capabilities and the fact that cyclosporine in particular causes low magnesium in patients. Typically they provide oral supplementation for this but this often cause diarrhea and obviously this is the LAST thing we want to promote in poor little Sam right now so we have been researching other ways to help improve these mineral balances.

The last "negative" from the week was we had some water damage to our house two days ago after a big storm. We are assessing how to get the damage repaired and avoid any possible mold issues because this can be very dangerous for Sam. Unfortunately having any construction/repair work done with her here is also tricky so if anyone has any great restoration construction company recommendations?:)))

On a more "fun note" Uncle Kurt was in town this past weekend for a big triathlon in Chicago and had a photo shoot with some of the top triathletes in the world. Kaeden got to sit in on the shoot and wore his favorite "super hero" outfit taking some professional shots with both the winners of the male and female elite races from the weekend. He was pretty pleased with himself and we can't wait to see/share these pics:)

Again, overall Sam is still a happy camper most of the time with a goofy sense of humor. We really do hope that these EBV numbers hold steady or decrease over the next few months and soon we will be counting down to her one year post transplant date... It feels like forever right now, but we'll get there!!!

Day +74 EBV = Murky Waters

        Last Friday our transplant doc called with the rest of the information she got from the lab regarding Sam's biopsies. The CMV and adenovirus stains came back negative but the stain for EBV was positive. She said though after consulting with the GI docs, they feel it is possibly a "false positive." The explanation is this: Sam has had a low level of EBV in her blood titers since engraftment. They monitor this weekly. There is blood in tissue and they feel the tests sensitivity possibly picked up on the EBV in her blood and that it is not actually "disease infected" tissue. They do feel that the tissue is infected but as of now are treating her with "infectious colitis - cause unknown."

       EBV is one of the "scariest" post transplant complications. While 90% of people have EBV in their "blood streams" their immune systems can keep it at bay. Sam's immune system is still suppressed and thus EBV in post transplant patients leads to lymphoma (cancer of WBC) and can rapidly spread throughout the body and be fatal. Unfortunately there are cases of people who develop PTLD (Post transplant Lymphoproliferative Disorder) with very low levels of EBV in their blood stream and those who have higher levels who never develop PTLD and they just really aren't sure why. ATG (a specific chemo that Sam had for pre-transplant conditioning) is one factor associated with PTLD. PTLD is very difficult to treat but the options include surgery to remove infected/organ tissue if isolated, Rituxumab (chemo), radiation, and donor t- cell therapy (can't be done at Lurie's currently so would need to travel to a hospital that has this capability). None of these are things we want to even think about, let alone face. Even hearing anything related to a possibility of this situation with a "hopefully false positive" sent us into a mental tailspin this weekend. Emotionally, it really knocked us out for a few days and just takes us back to a place of such raw vulnerability staring at a sweet 21 month old who just deserves a break.

      Today at clinic, I shared these thoughts with our doc. Overall Sam's numbers looked good including her liver numbers which were high last week. Her EBV titers are "hanging around" 7,000- 8,000. If these numbers rise significantly or Sam starts to show any additional clinical symptoms including fever, night sweats, swollen lymph nodes etc. she will have several CT scans done again looking for lymphoma/tumors. From there we will have to decide based on what we see what comes next. The doc pressed upon me that she knows it is difficult, "but our worry isn't going to make this better or worse." The treatments for EBV/PTLD are dangerous and comes with a host of risks/side effects so this treatment will only be done if absolutely needed. She said that "this may need to take place in the future, but the time is not now, so we need to just breathe and go one step at a time." We do really like this doc and she has gotten to know our family and all of our different personalities very well over the last few months. We know she cares about Sam a great deal. It's so tough to essentially "ignore the possible awful elephant in the room" as I look at her, but I know for all our sake, it's time to go back to one step at a time. Day to day. Live in the moment and just enjoy her because she is honestly such a hoot! I wish she had less restrictions and could share her ridiculous personality and sense of humor with more of the world.... Someday... Someday!!!

Scope Done... "Very Sick" but still waiting for Cause

After a loooong night of a "clean out" that included "Go Lightly" through Sam's G tube for 12 hours and IVIG with monitoring vitals every thirty minutes until 1am, we had the upper and lower endoscopy procedures done late Tuesday afternoon. The GI doc who performed the surgery talked to us for awhile after. His initial findings from the scopes were that her esophagus and stomach looked pretty good. Her intestines looked fairly "beat up" with some swelling etc. but he attributed this to that she "has been through a lot" through transplant. Unfortunately he said her colon most certainly looks "very sick." Ulcers, discoloration, inflammation etc. He took several biopsies throughout the procedure and has sent them to the lab. He can't diagnose from a scope on it's own but did think it looked like either infection or GVHD.

The procedure overall went fairly well. The night preceding and the typical hospital logistics were frustrating per usual with an initial surgery timeframe of 8am - 11am and then actually taking place at 2:15pm. All while Sam had not eaten since 6pm the night before. We did end up getting the anesthesiologist we requested and she was MUCH more empathetic and in tune with Sam's age and the toll these accumulative "procedures" can take on a child her age. She gave her versed and propofol in pre-op. She let me carry her down the hall and said if she protested when I handed her off, she would let me come into the operating room until she was safely "asleep." She really was kind. Sam was basically "mush" while I carried her and they came and got us before she opened her eyes so I could pick her up immediately and nurse her when she woke. This part went much more smoothly than the previous two experiences.

At this point we are still waiting for definitive answers. The only thing that has been ruled out is GVHD. This is good in a sense because having GVHD start after day +30 puts you at higher risk for it being a chronic condition which many have to deal with. Unfortunately some of the other "scary" options like CMV (seems to be the front runner as docs' guesses), EBV, or adenovirus have not been ruled out. Our transplant doc asked the head GI doc to take a look personally at Sam's smears and they have been sent out for molecular analysis. We will be basically holding our breathe until we hear the next steps. For now though, we are home again and Sam is doing pretty well when I can keep her cramping/pain from ulcers etc. under control.

Thanks as always for all the kind words, encouragement, and support! Hoping for answers with effective treatment soon!

Day + 67 Heading "Back in"

Today we are being admitted into inpatient in preparation for surgery tomorrow. Sam will have a lower and upper endoscopy done with biopsies taken from her esophagus, stomach, intestines, and colon. Her diarrhea has persisted for weeks and we are looking for answers and to help ease her discomfort. She will remain for at least one or two nights in the hospital to monitor internal bleeding risk etc,

If it is graft vs. host, we will have to increase her steroids and cyclosporine significantly. In addition to the awful side effects this brings, it also runs the risk of "awakening" the ebv virus that is in her system which can wreak havoc on transplant patients. We are hopeful for answers but would prefer something easily treatable another way.

Our attitude going in this time is "let's just get this over with!" As I mentioned in previous posts, I think this will now be the 8th time we are "putting her under" since February 1st and each time she has a more traumatic reaction to the process. Unfortunately in our experience, efficiency is not always a hospital's strength so "get in and get out" as quick as possible attitudes often lead to headaches and frustration. Will be doing a lot of deep breathing over the next 48 hours and will advocate as much as possible for things to help sweet Sam stay as comforted as possible. Some docs/ nurses have a gift of knowing just because you are their 10th patient of the day, doesn't mean you need to feel that way.... Others, not so much:)

So, here we go again... Hoping for answers... And not scary ones please...

Day + 60 Tushy Troubles Continued

       Today's clinic visit wasn't as "painless" as the last few weeks. Sam was due for her pentamadine injection. This is an IV medication that she gets once per month that helps protect her from a specific strain of pneumonia. It takes over two and a half hours for her to receive a full dose of this medication and the zofran that she receives prior to avoid nausea/vomiting.  She was also "due" for her dressing change, throat swab, bottom swap, cyclosporine blood level, CBC blood levels, chemistries, and urine sample (Monday Fun- Day!). Kaeden is also out of school this week and doesn't start summer camp until next week, so he tagged along for this four hours of fun as well:) When Sam is receiving an IV medication there is a line hooked up to a "pump" that is on a tall vertical bar with wheels (similar to what I posted as her "caddy" during transplant but smaller.) Now that Sam has more energy, strength, and is on steroids, she loves to run around like a maniac (most 21 month year old toddlers do right?) So as she is attached to this medication for over two hours, I am trying to push the pump around a small hospital room or hallway at the perfect pace to maintain slack going to her PICC line, so it doesn't "pull" at her line at all. After our visit today the nurse practitioner called to tell me next week she will need an IVIG injection now too. This is a drug to give her "antibodies" a boost which are slightly low. This is a four hour injection. Hopefully we just get back home before Kaeden returns on the bus from his first day of summer camp!

      Sam's WBC count was down slightly as well as her platelets this week, but still in a good range. Her hemoglobin was stable from last week and in a normal range. Unfortunately her tummy issues/diarrhea have continued and we are at a cross roads as to what to do. Stool samples have all been negative for C-Diff and other infections that they were trying to rule out. The only way to confirm a GVHD (graft vs. host) diagnosis is through the lower GI scope and biopsy which requires general anesthesia. If it is GVHD, the treatment would be to initially increase her steroid dose significantly as well as her cyclosporine. If this doesn't work, then the transplant doc said there are other "choices" for medication to try. Typically, GVHD presents at an average of day +30 so they are skeptical to jump to this conclusion, but stated several times that Sam has obviously not been the "typical" case from the "get go" so they just have to follow what seems most logical for her at the time.  If these medications didn't have serious long-term side effects that are more likely at higher doses, doing a month long trial at a higher dose would be a "no brainer" but kidney/liver damage, bone brittleness, growth issues are all things we obviously want to avoid. I am getting the sense that the docs would like to "know what they are dealing with" more definitively which means the biopsy. Obviously though this is easier for them to view as a relatively "safe" procedure from a medical standpoint, but as I mentioned in previous posts, our stamina for the surgical floor and recovery is really waning as a family. The decision at the end of clinic today was to slightly increase Sam's cyclosporine level until next week but to keep her steroid the same. If her symptoms worsen over the week, the choice will be out of our hands, but that is where we are for now. Hoping for a resolve soon of these tummy issues so Sam's poor bottom gets a break!

Day +53 - Stamina...

Last night, we didn't sleep much at all. After speaking with Sam's primary transplant doc on Friday, she wanted to schedule a lower GI scope and biopsy of Sam's gut and intestinal tract today if her diarrhea did not significantly improve. This would entail "putting her under" again and at least a two day/one night stint in inpatient. On Friday, we were able to get a urine sample to rule out a urinary tract infection causing her intense pain when she urinated. It was not a simple task to get a "clean catch" from a toddler but luckily we did so successfully, and avoided having to catheter her. This didn't give us much information leading to what had been causing the intense pain for the last two days, so they think she may have passed a kidney stone... Ouch:( She also had a violent vomiting episode on Saturday that resulted in petechia all around her mouth and chin. This is a rash that typically happens when platelets are low. I was surprised because her platelets have been more stable, but this added to the nerves for today's appointment.

      As of this morning, I wasn't sure what the doc was going to want to do about the GI scope and biopsy. Luckily after her numbers came back from the lab, all three blood lines (hemoglobin, platelets, and ANC) looked stable and I explained that her "tummy issues" seemed to be slightly improved although not her "norm." I asked if it was reasonable to avoid the scope/biopsy at this time given things are improving slightly and assess throughout the week if things take a turn for the worse. She agreed to continue a 10 day cycle of her new antibiotic and take a close "wait and see" approach as far as her digestion issues go. Like all things have been, it's a delicate balance but the docs really try to be proactive because if her graft vs. host disease (which is what they now think the rash is most likely) moves to her gut/intestines, they want to be aggressive and try to treat it as early as possible to avoid it spreading to other organs. We totally agree and want to avoid this, but we are also all too aware of the toll each of these "procedures" takes on Sam.
   
      Since February 1st, Sam has had two bone marrow biopsies, one bone marrow aspirate, two skin biopsies, two PICC line insertions, one G tube placement and two replacement procedures due to infection/balloon burst, one heart ultrasound, an EKG, one stomach ultrasound, CT's of her chest/abdomen/pelvis, a chest x-ray, 26 platelet transfusions, and 13 red blood transfusions. This isn't even considering the countless swabs, dressing changes, meds/chemos, "samples", etc. etc. Even though the scope and biopsy procedure with a few days in the hospital pales in comparison to what Sam has been through, it is just hard to maintain the stamina sometimes. Especially as summer approaches and it's Kaeden's last week of school, we just long for a more "normal" existence. It's also harder to "distract" her and she has more of a "fight vs. flight" response now that she is stronger, older, and wiser as a 21 month old. My heart aches for the parents and kids who have to go through a second transplant or face a different disease after "beating" the first one. My mind races even thinking of this possibility. The future is exciting and scary all at the same time, but I guess that's the same for any parent:) Luckily today was a relatively "calm" day and I hope this week only gets better! Pushing lots of toast and bananas around here!

      On a side note, Kaeden went to the Dells this past weekend with Brandon and my Dad. He had a BLAST! It was his "summer vacation/overdue spring break trip" since Sam can't be in public/airplanes, travel too far from the hospital, play in sand/dirt... our other options are a bit more limited as a whole family this year. Luckily the "boys" had a ton of fun. Sam was asking where her "Kee Kee" was all weekend and was so excited watching at the window when they pulled up. We took a quick picture as they were unloading the car. The first one with all four of us in it for awhile! This is Kaeden's last week of school in Junior Kindergarten. Our hero really is growing up fast!

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!