Sam's Story

Sam is a smiley and social 18 month old battling severe aplastic anemia. Until a few months ago, she loved going to the park, her little gym classes, and following her big brother around everywhere she could. Although not the easiest of babies, in general, there had never been major concerns about her overall health. She had reflux and some feeding issues, but she was growing well, and these were improving with time just as her older brother's had.

    This all changed on February 1st when Sam's mom , dad, and brother  took her to the emergency room for what they thought was a simple virus and dehydration. Sam's mom briefly mentioned she had been bruising easily on her legs but what toddler doesn't with hard wood floors? Within a half hour of being admitted, the er attending was talking to Sam's mom about leukemia or a blood disease causing bone marrow failure. They were both whisked to the oncology/ hematology floor where Sam recieved transfusion after transfusion of blood that first night. The next day she had a bone marrow biopsy and was surgically given a picc central line in her arm. Over the next five days in the hospital the family learned that Sam did not have leukemia which at first seemed like a relief until they started learning more about leukemia's "wicked stepsister," severe aplastic anemia.

      Severe aplastic anemia is a life threatening rare blood disorder typically seen in young adults. The disease causes your bone marrow to fail and all three of your major blood cell lines are impacted. Red for oxygen, white for immunity, and platelets for clotting. To be "severe" you have to have less than 25% cellularity from a bone marrow biopsy and an ANC below 1000. Sam has less than 1% cellularity and an ANC of 0. Because of this , she is technically considered " super severe" but the terminology and classification becomes less important.

 SAA ( severe aplastic anemia) is less researched than most cancers and thus treatments are not yet as effective . The two treatment options are chemo like immunosuppressive therapy which only has a 70% success rate in response (and 1/3 of these successes then relapse within a year), or bone marrow transplant. Most people don't  have a perfect matched sibling donor but this is where Sammi hit the jackpot! Her older brother is a 10/10 HLA match! This gives Sammi the only real shot for a cure and a normal life someday.

Getting to transplant though has been no easy road. Sams ANC (measure of immunity) has been so low (it is now 0), that she has been hospitalized almost weekly for fevers or any sign of infection. She has recieved platelet transfusions (needed for clotting) bi weekly and twice needed er care for internal bleeding due to such low counts. She receives bi weekly red blood transfusions to make sure her body has enough oxygen and her heart is not working too hard. She is not allowed in public at any time except to go to the hospital clinic, can not eat fresh fruit or vegetables , food prepared anywhere but at home , and can only  have reverse osmosis bottled water because of possible bacterial infections. She has stopped eating altogether and weighs less at 18  months than she did at 15 months. She was surgically given a peg feeding tube that she now receives nutrition and a slew of medications through up to five times per day. She has fissures and "open sores" on her bottom because her body does not have any working immune system to handle the basic bacteria that is in all of our digestive tracts on a daily basis. For this, she requires morphine almost every six hours, can not sit, and screams in pain shaking whenever she goes to the bathroom. It has been heartbreaking to see her deteriorate over the last six weeks while waiting for essential test results but those are finally here and we are moving ahead!

        As of now, Sam and her mom will check into the hospital April 3rd to start the transplant process. This involves 7 days of chemotherapy. Then it is harvest or transplant day where Kaeden will have a bone marrow abstraction procedure under general anesthesia. This is no walk in the park either but Sam's brother is one of the bravest little guys out there! From there, Sam  and her mom must remain in the hospital on isolation ( yep the same room) anywhere from 30 to 60 days. It all depends on how quickly and successfully engraftment occurs. When Sams body starts making its own cells and these reach safe levels, then she can return home (again under strict guidelines, no public places , tons of meds etc ). Typically these restrictions are lifted and risks significantly decline at one year post transplant. There will be more pain and challenge along the way battling thru transplant but its Sam's best shot so we aren't looking back!

        Please visit this site as often as you 'd like to see pictures or hear updates on Sam's progress. This has been a difficult time for our family and we've struggled with how much to share but in the end we want all those dear to us to be able to follow our battle with this and spread awareness on a small scale about this disease. So please feel free to leave us messages and PLEASE consider joining the national donor registry!  You could be a life saving hero too!

Three and Tubey Free!

I have been meaning to write a long overdue update on Sam's progress but the summer has just flown by. That is such a great thing in that we were busy just "living life!" Camps for Kaeden, lots of trips to the park, a few driving distance vacations as a family etc. It really was all around a great summer!

Medically, Sam began the process of re-vaccination in the late Spring. She has since then received three rounds of vaccines. Like a newborn, this often means receiving several shots at one time. These clinic days are pretty tough on her as they begin with a large number of blood draws and "pokey pokes" followed by 3-4 more "pokes/shots" to both of her legs. For at least a day or two after she is very sore walking on her tender legs. These days are often every 4-6 weeks though now, which makes it bearable. The big question still remains whether these vaccines will be effective or not given her continued low IGG levels. On our next visit in early September, we will test her "titers" to see whether her immune system is strong enough to post an adequate response to the vaccines she has received making them effective or not. Basically her body has to respond strongly enough to the "invaders" to remember or recognize them if she ever encountered the live virus. She will NOT begin "live" vaccine administration such as measles (MMR etc.) until at least two years post transplant. So even though her immune system is MUCH stronger than it has been, she remains at higher risk for viral infections and diseases she has not yet been vaccinated against. We are VERY hopeful her titers will show strong numbers ensuring our peace of mind that things are on track and she is progressing as she should. We are still cautious about starting her in a school/ larger group kids environment until we have a better understanding about her vaccination response and ability to fight more general viral infections. She could "technically" start preschool this year but we will not be sending her for at least one more year at this time (which Mommy doesn't mind at all!)

This past week, we also decided to remove Sam's G-tube. In many ways this is another great step towards her leading a more "normal" life again. We have successfully taken her off all prescription medication and were only using the tube for vitamin/mineral supplementation and to keep her digestive track moving (which continues to cause her problems at times following her colitis etc.) Now I am using trial and error with some rewards/"bribery" to find some quality vitamin and mineral chewable supplements that will work for us. She did really well overall having her tube removed. The exterior site has thankfully closed very well and quickly. The only thing we have to watch out for is whether it is healing properly from the "inside out" as well given that the surgical site is not just superficial but goes into her stomach. That we can obviously not tell as parents just from observing and caring for the site from home so she will be examined and possibly have an ultrasound at the next clinic visit to make sure the internal site is healing as well as it looks on the outside. There is a chance she would need to have an additional surgery if the internal area is  not healing but we do not anticipate that at this time.  Kaeden observed (per request) when the tube was removed and was a little shaken up by it but in her typical fashion, Sam was back to smiling soon afterwards and in turn, so was he! After having PICC lines in both arms, then down to one, and a G-tube for the past year and a half, Sam is now completely "tubey free!" Of course, there is a part of me that is anxious about setbacks and how we will manage if there is a need for a return of medications etc. but as always, we will just cross that bridge when we come to it! 

In more exciting news, this past weekend, Sam turned 3! We were so thrilled to celebrate with family and close friends at the house and Sam truly had a BLAST! We are always so eternally thankful for our little girl's physical health. Seeing her starting to grow again and work her way back up the growth chart, run, jump, and play outside is truly a remarkable feeling. Equally elating to us though, is that her smiley, funny, outgoing, and bubbly personality is growing again too! She fought so hard to earn her physical health back, but she fought equally hard to become Sammi again! Clearly we are biased, but anyone who is around her is always drawn in by her infectious smile, positive attitude, and fun-loving energy (ok also her adorable curly hair that chemo provided). We are so excited about the idea of more and more of the world getting to know her now too!

Thank you SO much to our families, friends, and ALL the people who still ask, care, and follow Sam's incredible journey. There is so much kindness in the world and I'm so glad we have been witnesses to so much of it! Please remember to sign up at www.bethematch.org if you haven't already! We have gotten a lot of letters and notes from people who have joined and it is such a great feeling to think Sam's story could hopefully help someone else someday! So here's to a happy, healthy, and "tubey free" Fall for all!

365!!!! 1st Re-Birthday Time!

April 10th was exactly one year from Sam's transplant. This was when Kaeden underwent the bone marrow surgical extraction and a few short hours later, they were transfused into Sam. In the transplant world this is called Day 0 and is literally thought of as a "new birth." So one year post-transplant is referred to as the patient's 1st Re-Birthday. April 10th coincidentally also happens to be "national sibling day" and the "Be the Match" organization hosted a 5k walk/run on April 11th in Chicago this year. So all in all this past weekend was just really special for our family. We had family and close friends fly and drive in from near and far. Brandon and I pushed Sam in the buggy for the 5K run and several family members and close friend joined "Team Sammystrong" as well. Kaeden participated in the kids 1K race which was unfortunately at the same time as the 5K so Brandon and I missed it. Luckily Baba, Uncle Kyle, Bapa, and Uncle Kurt had videos because he won! He was really excited and followed his plan to stay at a "steady Eddy" pace until he saw the finish line. I guess I forgot to mention a few minor race details though because he "Forest Gumped" the finish line and just kept going and going until he finally heard Baba and Uncle Kurt running after him that he could stop:)

After the race we had a small celebration for Sam and Kaeden in our neighborhood. It was truly awesome to see so many familiar faces that we haven't been able to see for some time now. A few friend's children came to the party too and this was the highlight for me for sure! Since we have been keeping Sam basically isolated socially, she really has had little to no contact with other kids besides Kaeden. At the race she was dancing up a storm with one of my best friends' daughters and at the party wanted to stay near the other four boys ranging in ages from 1-6 the whole time too. She was having a ball! I was worried she may feel overwhelmed by the days events but she was the same social butterfly she was before her illness and this was extremely comforting and delightful to witness. The whole weekend honestly felt a little bit like we were living "someone else's life" for a few days. "Out and about" at the run, a party where Sam was there.... eating chicken nuggets and cake! Truly an amazing view after the journey she's had.

Following this amazing weekend, Sam had a long day at the hospital for her post one year BMT check-up. The day started with an echocardiogram followed by an EKG. She was at first terrified of this process even though it is a pain free procedure. Luckily we had a really patient nurse who allowed me the time to calm her, demonstrate etc. etc. This makes a huge difference! Both exams are looking at her heart health due to chemotherapy risks. Next, she had a full audiology exam because she will remain at risk for hearing loss for some time due to chemotherapy and high-dose broad spectrum IV antibiotics that she received pre-and post-transplant due to her having no working immune system and the numerous infections we were trying to fight off. She did really well with this and they have no concerns about her hearing at this time. Lastly, we made our way to clinic for a full blood work-up which she normally has with some additional tests tacked on as well. Since she no longer has a PICC line, drawing blood from her is not easy on her or us. Luckily it only took two attempts this week and we did not need to keep the "line in" because the doc is still holding out on giving her IVIG. All her counts looked good except her IGG is still in the lower range. HOWEVER, it really held at almost the same number over the last month instead of dropping quickly as it has, so this is promising that her B cell line in particular could be starting to show some recovery! We also were told to stop her cyclosporine medication and two others at this visit. This is also a great step because it actively suppresses her immune system to ward off GVHD (graft vs. host disease). We have to keep a close eye on her skin rashes as well as her colitis symptoms which can be indications of GVHD but are really hopeful that she will do well since we did a really long and slow weaning process over the last several months. The last thing on the "to do" list is next week, she will need to get a new G-Tube as it has already been four months since the last one was placed. We are going to attempt to do it without general anesthesia first. I REALLY hope we are able to keep her relatively calm and that there are no blockages along her tract this time. If not, I have spoken with the IR team and ensured she is already scheduled for anesthesia that morning and she will arrive "NPO." This will make sure we don't sit around with her open wound for 8+ hours awaiting a break in the typical surgery schedule as we have in the past. After this, if there is no need to go back on any more transplant medications over the next few months, we will probably remove the G-Tube by the end of summer. The healing process varies but we will cross that bridge when we get to it:)!

We really want to say THANK YOU to every one of our family members, friends, and all those who have followed Sam and Kaeden's transplant journey. We would not be where we are today without an awesome support system. This experience has forever changed our family in numerous and incomprehensible ways. One positive outcome though (besides Sam's adorable curly hair:), is being able to witness first-hand the giving and good nature of those around you. Whether its' people you barely know, your family, or a best friend you've had for years it's a blessing to witness love at work. I hope this is a lesson Kaeden and Sam will be able to hold onto for life.

Below is a link to a slideshow my younger brother made to show our family after our little celebration. We hope as Sam's immunity grows, team Sammystrong and the Be the Match team, as well as the after-party will grow with it! Some of the images feel a bit personal, but we decided if it inspires one person to either join the registry or donate cord blood, it's worth it! Thanks again Uncle Kyle for the beautiful memento! Technology is not my strong suit, so hopefully this link works:)

http://youtu.be/MxG41Y9wnc8

youtu.be

IGG - Missing Puzzle Piece

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!:)

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!"

Thanksgiving without Steroids... Woo Hoo!

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

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

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

Risks vs. Rewards!

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

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

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

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

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