April 2007 Archives

Well the weather was beautiful today. We took advantage of it by going to a local nursery to look at alot of beautiful flowers. The girls and Simon just loved walking from flower to flower telling me "mom look at this one". It was so great to see Simon just running around like he was a normal little kid. We are so nervous to have him anywhere and outside is no exception. We are cautious but also feel he cannot just sit in the house and do nothing. He loves being outside, he is a boy who is discovering the world and he wants to explore it. We stopped for lunch and parked by a park, grabbed our blanket and sat outside to eat. Simon just ran all over the open field as the girls chased him. So noone ate much. Simons appetitie has decreased alot since we upped the immune suppressant and he has been vomitting more than he had been. I just wish we could get him off of all of it but we know it is a long way off. He is stooling much more too. I just don't know how to get him to grow or thrive. He seems to hold his own which I know the night feeds help with that. We have tried to get him off the bottle but really its the only way he will take in enough fluids and calories during the day so that is on hold too. All of this with the fact that he is 20 months old and has a mind of his own. After dinner we went over to the school so the girls could practice riding their bikes which did not get much use last summer and Simon tooled around in a pink and blue car (shhhh Dave is dying to spray paint it blue). My smile didn't stop the whole time we were there as we ran around pushing him and watching the girls all with big smiles on their faces having the best time just being kids. God willing, we will be enjoying that alot more. We go in for pentamadine IV infusion on Monday. I have to say I am very nervous as Simons skin has finally calmed down and I actually am getting rid of the thick scaring left from the last serious flair up of infusions or whatever is actually causing his skin to react the way it has been. So please, pray for Simon to make it through with no affects from the infusion and for his vntrs and igg to be way up there along with all counts that need to be holding strong and gaining strength with this time of healing. I think of what we still have ahead of us and I pray it just gets better. God Bless, enjoy your Sunday. Love Tracy and David

This Photo (posted on our carepage still to come on the web page) of Simon says it all for today. We got our counts back today earlier than expected and I have to say when Daves mom told me Simmys doctor had called when I went out to run an errand my stomach dropped so I immediately call stem cell and spoke to Kim. I was pleasantly surprised to hear that simons counts had come up overall to 94% this week and that his b cells and myloids were up to 97% donor while his lagging behind, pain in the butt t cells were strong at 91%. This is good news and we are happy to know that thus far the raise in cyclosporine seems to be helping to regain the donor cells. His igg level was also steady in the low 700s so we are not going to be giving him ivig yet. As you know this is a drug he needs to help keep his immune system fighting infections that it cannot fight but we also think either that or another iv drug he gets monthly, pentamadine, is contributing to the awful skin rash he has last month. Right now the plan is to hold all meds strong even though the cyclosporine is not at a therapeutic level but is aiding in getting our Marriana cells back, and we will most likely be switching to a daily liquid drug (which is not the best solution for us as we add another med to the mix) to replace the iv infusion of pentamadine.

Overall things are going well. Simon has been vomitting once daily and I"m not sure what that is due to, if its the meds being increased or what but hopefully it will pass again. We want to thank you all so much for sticking by us and praying for our son, our family. Please continue to ask for Gods hand in helping to completely heal our sons new donor immune system and to lead us to a more normal life. I did take the kids to the park yesterday and Simon was loving being in the midst of "life" and enjoying all the things to climb up on. I was running behind him with wipes ever step of the way but thats what moms do. Have a good night. God Bless, Tracy and David

Today was one of those days, filled with lots of things to do but not accomplishing much. The girls, Simon and I went to clinic, got blood draws, met Dave for mickey d’s in the car downtown and came home to put Simmy to bed and spend a much needed hour on our van, which I’m embarrassed to say was like driving a fast food restaurant/toy/book store with lots of dust on the dash and empty diet coke cans in the holders (trying to make up for lost time during abstaining for Lent). Felt good to get that done and the girls were an amazing help, I think the popsicle reward helped motivate them along. As I look back at the last 2 + weeks I feel the weight of the world lifting somewhat but also somewhat heavy. Its hard to explain the daily emotions or even minute emotions we have felt lately. We have had STRs drawn twice now that have told us simon is at 100% donor cells and fish labs (not totally sure) but these tell us we have 5% male cells looming which coinside with our stem cells vntrs of 94% donor and await antibody draws to tell us if we will have a problem with rejection from Simon of Marriana’s cells. We are so in the realm of thinking that this is our summer for Simon to be born, to live as a 20 month old boy should be able to and our setbacks always remind us that transplant or NEMO are not just an operation to be healed by time and be done with. Its always scary to think about the meds he is on and long term effects or the time it may take to get him off of them and have the result we long for. Today while at clinic I had the rare opportunity to speak with the “head” of stem cell. Dr. Kletzel who is a very empathetic man and one who you know wants the best for your child, why else would he do what he does, but is also Mr. Rose colored glasses and sometimes makes you crazy for his optimistic outlook when you feel so shitty, but today I found much comfort in talking with him and seeing him sit in a chair in front of me speaking to me like a friend, after all 20 months gives me that right, to think of all the stem cell doctors, nurses, nurse practitioners as friends or family right. Simon has some bad scaring on his legs and arm from the last skin flare up and I was offered a “home remedy” from Mexico from Dr. Kletzel and really it made it feel genuine. I cannot explain all the feelings that cross my mind daily. A lot of it is guilt, self doubt, fear, and hope. I wonder how life got this way, how my son was meant to suffer and how much we will rejoice at the time of complete healing. I also look at things like the moon tonight and think back to how much I used to love to take my telescope onto our deck and look up at the night skies and wonder…….and how much of that I have forgotton or lost or grown not to have the energy to care about. it’s a hard balance. Most days I sit and think in time our life will be as it was, daily work, school, activities and sibling fights that drive you crazy. Right now I wonder how all the families going through serious illness make it. How do they continue on. We have 3 immediate family members in desperate need of help right now and the sad thing is none of us have well enough situations to be there for anyone else. Its just not right. After thinking about how this all came to be it is my destiny to get the word out to healthcare providers through pamphlets or something about NEMO and signs of carriers that noone even suspects is a problem so that other families, perhaps can make the choice if they can bear the pain and all that goes into trying to cure this. I could not imagine life without Simon. The girls and him love each other so much. They play and seem to know words with one another that I cannot decipher. Its totally amazing and I thought it was just a twin thing. Simon loves everything about life. He loves to come into the kitchen when I am cooking and say “uppy” so he can sit on the counter, pour salt or pepper into whatever I am making, help stir pots of bubbly foods and taste things as we add spice or sauce to them and say mmmmm more……Those things to me make my days and worries all disappear. He deals with this everyday, I get a break to go to the store or spend time with just the girls or a much needed dinner alone with Dave, but Simon, he gets the pokes and pains every week and still smiles at those of us who make him go and put him through it all in hope of finding the answer, providing a solution to a healthy life. I will learn so much from him and there is no doubt I will find things in life that matter more than any mother ever could have imagined. Please pray for him, for vntrs of 100% donor cells. God Bless Tracy

The waiting is always the hardest part. I sat for the last 24 hours with what felt like a 2 ton brick on my chest waiting to hear simons numbers today. For all the prayers and thoughts we have been honored with increased percentages of Marrianas (donor) cells. Today the vntrs came back at 91% overall. Some numbers of broken down cells still in the 80's but the doctors are working on trying to find the right balance. The relief today is that we don't have to start iv steroids. So around 5pm I felt a relief from the worry for now. They did up the cyclosporine again yesterday and Simon gets it 3 times a day now along with all his other meds. He has been doing well. His skin is blotching up again today so that is always worrisome. I was able to contact our expert NEMO doctor in philly and he did inform me that Simon's NEMO mutation is very severe so we have no choice but to get him a new working immune system and keep it. So with that bit of news (which we were somewhat aware of) we march forward to try and save and keep this engraftment for the long haul. I've had some very weak moments the last few weeks and all your strength, kind words, treats and meals have helped pick us up. Its awful hard to continue to be on the receiving end of things when you so want to just be helping someone else out now. We ask that you continue to pray for Simons donor cells to completely return and to stay long and stay strong forever. Interestingly NEMO carriers, which is what I am, are living on a 50 - 60% working immune system. So I have to think since I've managed to get through many things we must know in our heart, Simon too, will prevail. Dr. Duerst likes to use the word hope alot and I really couldn't hear, or feel that this week but in search of pray and inspiration I came across this and it really struck me...."Hoping is not Dreaming. It is not spinning an illusion or fantasy to protect us from our boredom or our pain. IT MEANS A CONFIDENT, ALERT EXPECTATION THAT GOD WILL DO WHAT HE SAID HE WILL DO. It is a willingness to let God do it his way and in his time. So we Hope for Simons full recovery, in Gods time. God Bless you all. Tracy and David

wanted to post this for two reasons, one to say thanks for the many calls and e mails we have received. I'm sorry that I just am not feeling up to talking about all of this right now but it feels good to know people care so thank you. Its been a busy weekend and Simon has had alot of fun. He is full of life and to look at him you would never guess what is going on inside. It breaks my heart and I honestly have not been able to go anywhere without breaking down. I stood in the Jewel parking lot in tears on the phone to Dave trying to pull myself together and act like everything was normal just running to the store like everybody else. In my heart I feel okay but in my chest I feel heavy and scared. I don't know what to believe. We have had a lot of smiles this weekend with the kids and its a shame to have them overshadowed again with all this doubt, fear and anxiety. Its with us every minute of every day now again. I go to clinic tomorrow for more labs and to make sure the cyclosporine is not too much as it can be toxic. We ask again for prayers, I don't know what else to do. I'll keep you posted as we hope to get vntrs back on tuesday. I have never wanted anything more in my life than to have his donor numbers up and steady. Thanks and God Bless. Tracy

am posting this with the heaviest of hearts and really don't have alot in me so the numbers today were even lower. We now only have 85% donor cells overall. We are angry, upset and saddened. I don't know what else we can do. My hope is in limbo right now because I just don't know how to keep going on like this. They are upping the immune suppressant quite a bit, not sure how its going to work but the hope is to have donor cells rebuild stronger than simons. Not that the immune suppresant can pick and choose. Please pray harder than ever. I am trying to find the strength to reach out to God after all of this. Tracy

cannot believe I am even posting this, almost a year to the date of us going home last year after transplant number one, I am asking that you pray long and hard for our son this weekend at one of the holiest times of the year. I am devasted to tell you all that we just found out today that Simons donor cells are declining. I am sick. I don't understand how this can be happening. Overall we are down to 94%, down 5% since the last check a little over 3 weeks ago. What has changed, cyclosporine an immune suppresant has stared to be tapered and he has had this huge skin problem come up. I don't even know what to think. I had a sick feeling in my stomach from the moment I woke up this morning and sure enough it proved to be true. I am asking that all of you and everyone you know, pray for Simon that this somehow was error on the lab or something that it is not happening to us again. Its more than my whole family can take. Please, please pray and say a special prayer that his vntrs the next few weeks will be back up to 99%. Please. God Bless Tracy

Okay what a week and a half it has been. Just when you think things are progressing....The girls, Simon and I spent a long morning at the hospital today. At least the girls and Simon were rewarded with an Easter basket of goodies. The childrens hospitals are great about that. It was a worthwhile trip. We met with Allergy/Immunology doctors and I was very impressed with them. Its been a long time since Children's has had anyone in their immunology dept who said anything that was helpful. They hired a new head of immunology who we will meet next tuesday but today he sent his colleagues. After speaking to them, having simons skin examined and Dr. Mancini (our savior and lead dermatologist) consult they all think the same thing, Simon has erythema multiforme major. Basically one step away from Steven-Johnson syndrome, which is a very serious disease caused by a drug reaction. Since the only timing we could link his outbreaks to is the IVIG infusions we are concerned this is the culprit. Since his reaction has gotton worse over the last two months right after IVIG they are contemplating stopping those infusions. This brings up great concern for us all. IVIG is given because immune suppressed people cannot make their own and it helps to keep his immune system fighting against infection. Their thinking is that since simon has sores in his mouth and other areas it could affect his organs next time and that could be very serious maybe even fatal. So we are at a hard decision. Everyone is meeting, including our faithful Dr. Uzell in Washington, and they will discuss and come up with a plan. We have about 3 weeks before he is due any infusions. They are also tapering cyclosporine again in hope of allowing Marrianas immune system to start producing some IGG. Believe me I knew nothing about the immune system before simon and to look at counts and either get happy or worried is something I dread. We all walk around getting colds and things and don't even think of how our systems work to fight them off but when confronted with a situation like this every number sets the mood. Today our platelets are down. My stem cell drs say "don't worry you have bigger things to worry about right now" but my heart gets scared as these are the core of watching the immune system rebuild and Simons were great last week and down today. So we go back on Tuesday. Right now our biggest worries are that he will not drink or eat due to sores in his mouth and possibly esophogus and he is hooked up to his feeding tube which we will increase and lengthen tonight to keep him hydrated, otherwise his skin will get worse. He needs to have lotions applied 4-5 times a day and we pray all the blisters evaporate back into his skin and skip the popping otherwise we are open to more infections. So please, pray for these things along with the vntrs we drew today to be 99%+. I'll keep you all posted. Thanks for hanging on with us. Please pray for full recovery and no recurrent outbreaks along with if stopping IVIG there will be no side affects of infection. Thanks and God Bless. Tracy

Well I am completely exhausted. Simon and us have had a rough weekend. His skin is worse than I have ever seen it and it is so painful for him. I put all the meds to use last night and he still kept getting up and crying. His redness is calming down but he has blisters the size of quarters all over his arms and legs and his lips are pretty well scabbed. His mouth has sores inside too so hes not too happy to eat or drink. It is heartbreaking and scary all at the same time. EM can be a very serious skin condition especially if its associated with other diseases. Since not alot is known about NEMO noone knows if this is from that or what? We are still wondering about the IVIG even though they say they have never seen it to cause this and its acutally suppose to be something they prescribe to help with it. So they are thinking the pentamadine which he gets to prevent pneunomia but he gets that at the beginning of the month and doesn't break out. The other day with piggybacked the IVIG and pentamadine and a day later had this. So please continue to pray that Simons skin clears, quicker and we don't have another episode of this in a long time. We get the infusions once a month so I am very worried. He has been throwing up and not eating as well so its sucked all around. On a good note he did spark up some happiness when we all decided to get into the girls sleeping bags and have potato sak races today. At least he knows fun when he sees it. Please pray for Simon, he needs your prayers. We will check vntrs on the 10th and then not again for 3 months so pray they are strong and steady. God Bless Tracy and David