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!!!