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.