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!