Day + 344.... Sam is near her one year post BMT marker. This is such fabulous news in so many ways. She continues to show strong engraftment. Her chimerism (measure of Kaeden's cells vs. her cells) at her most recent draw were +98%. Some labs will say 100% but for some reason at Lurie's lab 98% is the strongest number they report so we'll take that for now! We will monitor this number as we continue to decrease her cyclosporine dose. Cyclosporine is the drug that actively suppresses Sam's immune system to limit GVHD (graft vs. host disease) symptoms and to help decrease changes of engraftment rejection. After one year, engraftment rejection chances decrease again and are very rare (although with Sam's disease being literally one in a million these numbers don't alwas provide the comfort they should.) Cyclosporine actively suppresses T and B cells though so the longer one has to remain on cyclosporine the longer one is at increased risks for infections and developing certain cancers. This is one of those "balancing acts." We hope to have Sam off of cyclosporine in April or early May. As we decrease her dose, she is showing some mild skin GVHD symptoms with rashes etc. We are working to find a skin care routine to help alleviate these and hope they stay in the "mild" zone so we can successfully wean completely. On Monday at Sam's most recent clinic visit, her WBC count, hemoglobin, and platelets all looked pretty good. The remaining number as we have discussed in previous posts that is not showing adequate recovery is her IGG level. Again I will oversimplify based on my understanding, but there are almost countless different types of WBC that each have different jobs. For a "strong" immune system one does not just need high numbers in one area, one also needs the right "balance" and types of cells to work together. Sam's WBC that fight fungal and bacterial infections are looking pretty good. IGG is needed as the first line defense for viral infections. Since Sam's IGG remains low, this means she has a less than optimal response against viral infections. Over the last year, when her number has dropped below a 400 threshold, we have been transfusing her with IVIG to boost her IGG number and help keep her protected. Now that we are through the cold/flu infectious season, the doctor wants to let Sam's number "go" a little bit and see if this will force her body to step in and produce more actively on its own. We will not let her bottom out to 0 and she said if at her next appointment in April, she is below 100 we will need to give her IVIG no matter what. We also can not start any re-vaccination process until this IGG line shows recovery because her body will not be strong enough to produce an adequate antibody response to make the vaccine effective. Typically post transplant patients start slowly revaccinating with inactive vaccines at one year post transplant and live vaccines at two years post transplant. SO.... the IGG mystery is like the last big "missing piece of the puzzle" right now. We REALLY hope that in the next month or so her T cell and B cell line will "wake up" and start producing IGG. Like so many other parts of this process, we'll wait, we'll hope, and we'll see!
Overall though for a transplant patient, Sam has done remarkably well and we are so incredibly thankful for where we stand right now. It is almost unfathomable at times to look at her and realize how far she has come in one year. Despite these overwhelming feelings of joy and relief we feel daily though, we also long for more for her. We really hope she will have more opportunities with other children her age soon. We hope she can attend some sort of preschool option in a year or so. We hope she can go swimming or that we can take a trip on a plane together somewhere as a family. We really can't feel comfortable with any of these scenarios though until this missing puzzle piece falls into place. It's such an internal struggle though because as soon as I start to feel frustrated by these limitations, I feel guilty. I know so many others have had less fortunate outcomes post transplant and would give anything to be sitting where we are right now. So we are going to try to focus on what we can control over the next month which is to acknowledge Kaeden and Sam's strength and love for each other. That bond has gotten us where we are today and that is something truly remarkable to celebrate. GO Sammy strong! Go IGG!!! Go! Go! Go!:)
Friday, March 20, 2015
Friday, January 30, 2015
Looking Back... Looking Forward
I have written at least ten updates in my head over the last few months but none of them have managed to make it to a keyboard. I promised myself though that I would write a post today as it is now "one of those dates" that will forever stand out on a calendar for us and it seemed like we needed to honor that. Exactly one year ago today we were admitted to the ER from our pediatrician's office after Sam seemed unable to "shake" what we thought was a routine virus. In many ways, that first night that we learned something was terribly wrong is such a blur. In other ways though, so many details remain gut wrenchingly vivid. I'll never forget the look on the ER attending doc's face as he walked into our room and tried to explain what Sam's blood work was like and what her "numbers meant". So much of the medical explanation I didn't understand at the time, but his sad eyes and the way the fellow could only stare at the ground while a nurse put her hand on my shoulder, told me so much. My hand shook uncontrollably as I called Brandon as he was at home with Kaeden. "They think something is REALLY wrong with her and they are taking us to the oncology floor right away" was all I could get out. He had so many questions and I had so few answers. I remember staring in utter disbelief as bag after bag of blood was hung on a pole next to our bed that night. Sam would receive three transfusions over the next 10 hours. Little did I know at the time that transfusions would soon become as frequent and routine as grocery runs in our life. Two days later, she had her first bone marrow biopsy and received her first PICC line. The next three days were a waiting game as there were several steps following the biopsy process before a definitive diagnosis. My initial shock and denial began to give way over those days to the reality we were facing and the fear and sadness begin to creep in like a tidal wave. The irony and twisted beauty of having a critically ill child though is you soon learn there is simply no time for those feelings.
The next five months, Sam and I spent more time at the hospital than we did at home. When you are told your child might die, every hour, every minute is spent researching and learning more about your child's illness, coordinating care, monitoring symptoms and pain control etc. Many kind friends would ask me "how I was holding it together?" It did not (and still does not) feel that way a lot of the time, but my only honest response was "because I don't have a choice." There really is just no room for tears or fear at that time, so you detach from the emotion of it and just focus on doing what you have to do to make your child better that second, that minute, that day. I remember asking a fellow AA mom I met online "when does it get better?" She told me her daughter was medically better a year after transplant but that it took her and the rest of the family at least another year to begin to "emotionally heal." I remember at the time, that statement kind of just washed over me because I really wasn't in a place where the emotional aspects of critical illness were even registering. I couldn't and wasn't "going there." Now 9 months out from transplant and a year out from diagnosis, those words are ringing so true. The anger, fear, and sadness that were pushed deep down start to surface and catch you off guard because things should be getting better right? It's often seemingly little things that trigger memories, ignite fear and doubt, and can send you into a tailspin, and unlike before they aren't so easily chased away by the pace and chaos of hospital life. In many ways this is a wonderful thing, but I get why so many other parents have told me that "it's never really over, but it does get easier."
In SO many countless ways it is infinitely better and we are utterly grateful. Sam's counts have remained relatively stable and safe. She was put under and got a new G tube right before x-mas and did really well with that surgery. The GI surgeon felt this was the first time that it was easily replaced and that it could be considered to be done without anesthesia the next time (every 3 months). After a relatively brief frustration with insurance that denied several claims for Sam's IVIG transfusions, it appears that this has been sorted out with the help of an insurance liaison from Brandon's company. All good things! The remaining "waiting game" as we look ahead is for Sam's t-cells and b- cells to recover. We have learned so much about the immune system over the last year, but it is such an amazingly layered and complex system that we have probably only scratched the surface of true understanding. My best explanation is that there are two types of t-cells that are vital to a good immune system. There are "helper t cells" and "fighter t-cells" that are responsible for triggering an accurate and targeted response to infection. So at this point even though we are incredibly grateful that Sam's WBC and immune system overall is "stronger," we are still waiting for it to become "smarter." She now has the number of cells needed to fight an infection but she does not have the appropriate "management crew" to direct these cells in an optimal way if that makes sense? We are back at clinic this next Monday and I plan to ask more pointed questions than I have in the past about recommendations and options if t-cells and b cells continue to show limited to no recovery as we approach the year mark. My current understanding is t-cell therapy does exist (although not at our hospital) where t-cells would be taken from Kaeden's blood and transplanted again into Sam. The benefits of this therapy would need to be carefully weighed against the risks though as it opens up the possibility of a GVHD (graft vs. host disease) response. GVHD is highly unpredictable in it's severity and is not a risk we are eager to take on again unless absolutely necessary.
As I stared at my phone this morning at the January 30th date, I was caught off guard and overcome with emotion. Some of it was anger, some of it was sadness, and some of it was fear. But as I watched Sam and Kaeden "rock out" tonight to some "Just Dance" videos before bedtime, I was so thankful that this January 30th was full of new memories. Twirls and spins replaced transfusions and giggles replaced cries of pain. I honestly can't believe how far Sam has come or how hard she has fought since one year ago. For awhile we really hoped that all or most of the painful memories of the past would just fade away with time, but now I'm not so sure that's even what we want anymore. They make those family moments of dancing, laughing, or just "being" all that more sweet! My heart truly feels like it will explode with joy sometimes when Sam and Kaeden are laughing hysterically together, but my heart also aches with such pain knowing that almost everyday a parent will have to experience their own "January 30th" with their sweet child. As always, thank you so much to all of our family and friends who have supported us in so many ways over the last year and thanks so much to those of you who still follow Sam's story. As she would say with a sweet smile, "Kanks!"
The next five months, Sam and I spent more time at the hospital than we did at home. When you are told your child might die, every hour, every minute is spent researching and learning more about your child's illness, coordinating care, monitoring symptoms and pain control etc. Many kind friends would ask me "how I was holding it together?" It did not (and still does not) feel that way a lot of the time, but my only honest response was "because I don't have a choice." There really is just no room for tears or fear at that time, so you detach from the emotion of it and just focus on doing what you have to do to make your child better that second, that minute, that day. I remember asking a fellow AA mom I met online "when does it get better?" She told me her daughter was medically better a year after transplant but that it took her and the rest of the family at least another year to begin to "emotionally heal." I remember at the time, that statement kind of just washed over me because I really wasn't in a place where the emotional aspects of critical illness were even registering. I couldn't and wasn't "going there." Now 9 months out from transplant and a year out from diagnosis, those words are ringing so true. The anger, fear, and sadness that were pushed deep down start to surface and catch you off guard because things should be getting better right? It's often seemingly little things that trigger memories, ignite fear and doubt, and can send you into a tailspin, and unlike before they aren't so easily chased away by the pace and chaos of hospital life. In many ways this is a wonderful thing, but I get why so many other parents have told me that "it's never really over, but it does get easier."
In SO many countless ways it is infinitely better and we are utterly grateful. Sam's counts have remained relatively stable and safe. She was put under and got a new G tube right before x-mas and did really well with that surgery. The GI surgeon felt this was the first time that it was easily replaced and that it could be considered to be done without anesthesia the next time (every 3 months). After a relatively brief frustration with insurance that denied several claims for Sam's IVIG transfusions, it appears that this has been sorted out with the help of an insurance liaison from Brandon's company. All good things! The remaining "waiting game" as we look ahead is for Sam's t-cells and b- cells to recover. We have learned so much about the immune system over the last year, but it is such an amazingly layered and complex system that we have probably only scratched the surface of true understanding. My best explanation is that there are two types of t-cells that are vital to a good immune system. There are "helper t cells" and "fighter t-cells" that are responsible for triggering an accurate and targeted response to infection. So at this point even though we are incredibly grateful that Sam's WBC and immune system overall is "stronger," we are still waiting for it to become "smarter." She now has the number of cells needed to fight an infection but she does not have the appropriate "management crew" to direct these cells in an optimal way if that makes sense? We are back at clinic this next Monday and I plan to ask more pointed questions than I have in the past about recommendations and options if t-cells and b cells continue to show limited to no recovery as we approach the year mark. My current understanding is t-cell therapy does exist (although not at our hospital) where t-cells would be taken from Kaeden's blood and transplanted again into Sam. The benefits of this therapy would need to be carefully weighed against the risks though as it opens up the possibility of a GVHD (graft vs. host disease) response. GVHD is highly unpredictable in it's severity and is not a risk we are eager to take on again unless absolutely necessary.
As I stared at my phone this morning at the January 30th date, I was caught off guard and overcome with emotion. Some of it was anger, some of it was sadness, and some of it was fear. But as I watched Sam and Kaeden "rock out" tonight to some "Just Dance" videos before bedtime, I was so thankful that this January 30th was full of new memories. Twirls and spins replaced transfusions and giggles replaced cries of pain. I honestly can't believe how far Sam has come or how hard she has fought since one year ago. For awhile we really hoped that all or most of the painful memories of the past would just fade away with time, but now I'm not so sure that's even what we want anymore. They make those family moments of dancing, laughing, or just "being" all that more sweet! My heart truly feels like it will explode with joy sometimes when Sam and Kaeden are laughing hysterically together, but my heart also aches with such pain knowing that almost everyday a parent will have to experience their own "January 30th" with their sweet child. As always, thank you so much to all of our family and friends who have supported us in so many ways over the last year and thanks so much to those of you who still follow Sam's story. As she would say with a sweet smile, "Kanks!"
Wednesday, November 26, 2014
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!
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!
Wednesday, October 29, 2014
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!
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!
Wednesday, October 22, 2014
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!
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!
Friday, September 26, 2014
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!
Tuesday, September 9, 2014
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.
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.
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