5:16 AM 2/20/2008

Sydney had a great day yesterday. As usual she was difficult to pull out of bed in the morning but she had the first full and complete day that she has had in a while. The fever was gone. She was also extremely excited to be back at school with her friends again. I personally did not want her to go to school. I wanted to hold her, spend time with her, and never let her go. It is such a selfish feeling. I know that. In the end, I simply put a smile on my face and ushered her out the door. I then spent the next 7 hours wishing that I was with her. I often contemplated going to pick her up early from her 45 minute stint in aftercare but I also knew she liked that time as well. In the end, I would just focus my head back into work to keep myself from going nuts. At this point I am focusing on tasks that keep my mind running so I don't have time to sit and wish and pine away. The trick for Sydney is to keep things as normal as possible. Currently, she seems happy and I don't want her to feel the way that I do.

Today we will be picking her up from school and taking her to the clinic. At 2:00 PM there will be a meeting with Dr. Gillespie and Dr. Eames to discuss the biopsy of Sydney's arm. As I had mentioned earlier, I think all of our minds have been made up. None the less, this will be a good opportunity to discuss the risks of the procedure and to hopefully get a better handle on what may or may not be ahead. It is a very, very scary time.

I am still just reeling from all of these tests and scans. I can so easily see that this could be cancer. Yet, on the other hand, I can also see logical ways to hold out hope that this is not my worst nightmare. I guess the difficulty is that we now have mounting evidence before us. The bone scan in and of itself is not that bad. As I said on day one, it is a nonspecific test. It could be anything. Yes, it could be cancer, but it could also be infection, trauma or fluke. When the MRI came along it did not surprise me that there was something in the same place. Although it ruled out some kind of technical malfunction I really did expect it to confirm the result somehow. Of course, I was hoping it would confirm it with more clarity and point to infection or trauma. Not so much. Instead, it confirmed the spot, but made the diagnosis even greyer. There are things about this spot that are cancer like and there are things that are not. Again, in and of itself and in combination with the bone scan, there is nothing screaming neuroblastoma. With these findings I would have been worried but I also would have felt somewhat confident that it could be something else. The clean marrows by morphology gave me even more ammunition. Either this was a very local relapse or nothing at all. When the immunocytochemistry on the marrows came back positive this further constricted the noose around our necks. This was further evidence, unrelated to the spot on the arm that this very well could be disease. It is heartbreaking really. This was the tough stumbling block for me. The harder part about it is that this result has been called into question so many times. The fact is that we know mistakes are possible and this test and lab has a history of them. In this case we are hoping that the test is simply wrong. We are hoping that the antibodies used to identify neuroblastoma cells are actually picking up something else. While it sounds wild it actually has happened many times before. With all of the things that we have done to Sydney's marrow this is certainly a possibility. Furthermore, we have not had any tests like this since we gave her the vaccine. It is possible - not likely - but possible.

It is all of these inconsistencies that dictates that we must go in. There is a lot of mounting evidence that this could be neuroblastoma. Unfortunately there is nothing definite. It is as Dr. Eames said, we need to prove it, one way or another. In the end, these are the reasons, that we will likely be opening up her arm and surgically removing a small wedge of her bone at the point of the protrusion. We will also be going for another round of marrows on her hips. We will be hitting 4 spots with the hope of increasing the sample size and our chances of finding a single cell, a single neuroblastoma cell. It is all so surreal.

Although I have had a purpose everyday, I had forgotten what it truly feels like.

4:26 AM 2/19/2008

Well, this morning isn't a particularly good one. Late yesterday afternoon Dr. Eames called with the further testing on the marrows from Genzyme and, sure enough, they were positive. While Dr. Granger had told us that everyone was positive I still did not want to hear the news. I quizzed Dr. Eames about this whole "everyone is positive" thing. I found out a little more. This company has had issues in the past. 18 months ago everyone was positive. They had some issue with staining and have acknowledged it. Essentially their were problems with both the antibodies and the staining procedure which illuminated cells which were not actually neuroblastoma cells. This test is an antibody/staining procedure so they do not actually see neuroblastoma cells. What they see is staining. The more staining - the more neuroblastoma, theoretically. They have had sporadic issues since. The company, of course, believes that they have improved their process and feel confident in their product. They stand behind their result. I would expect that. Unfortunately, they feel that Sydney is positive for neuroblastoma.

Another finding that was bothersome to me was the fact that she was significantly more positive on one side of her hip than on the other. While I could probably dig very deep to come up with a rationale of why this could be the case, it certainly isn't comforting. This is clearly not what we were looking for. It is bad enough that we feel that we need to get more aggressive with identifying what is going on. It looks like we will be meeting with an orthopedic surgeon on Wednesday who we already know is going to want to biopsy the spot. We will know more later but it looks as if the procedure will happen next Tuesday.

This is so reminiscent of 3 years ago. Although we have migrated to another bone and we are talking about an arm instead of a leg it is eerily similar. Talk about putting our money where are mouth is. Do you want to know if we relapsed three years ago? We are about to find out.
This is becoming more difficult by the minute. It is one thing to exist in the world where you know your child may have relapsed and have the hope that she really did not. It is quite another to be faced with the reality. I know we have to know the answer. I just don't know if I can handle the truth.

Yesterday we also took the girls to see Dr. Debbie. Ainsley has a confirmed ear infection and has been placed back on antibiotics. Surprisingly, Sydney was not. Seemingly, however, she seems to be having some pain related to her Eustachian tubes. When Dr. Debbie applied pressure she squirmed a bit. We have put her on Rescon G/G and are hoping that we can get it cleared up relatively quickly. Last night, however, she produced a fever of 100.7. I don't think I need to tell anyone how nervous that makes me. Regardless, I can feign blaming it on a sinus infection and that still gives me a bit of hope that this is unrelated to neuroblastoma.

Other than this Sydney is in excellent condition. She has had a blast running and playing and could not be happier. She is having fun with her brother and sister and even took out time to play a couple games of cards with me. By the way, I am not one of those nice Dad's that let their kids win. They have to earn it. Sydney is slowly learning to beat me. She is getting better and better everyday. That is progress.

That is purpose.

5:31 AM 2/18/2008

I probably should have written a bit before today. Quite frankly, I just did not know what to say. In the meantime we have completed Sydney's MIBG, taken a mini-vacation to Great Wolf Lodge, and received most of our results. It has been a roller coaster of emotions.

I guess we should start back on Friday afternoon. I should tell you that as a result of last week's ridiculous scheduling of Sydney's scans which subjected her to multiple pokes unnecessarily, I have lost all modesty when it comes to advocating for Sydney. I have always been the polite one and even when I know things are not going correctly (as long as it does not hurt Sydney) I am always willing to follow directions and be relatively accepting of the bureaucracy. I would go along with things even when I knew them to be completely wrong. I always thought that the process was the way it was for a reason or because things had to be done a certain way. After watching this go on for almost 5 years both with by child and others the switch finally flipped. I am still polite by I am firm and resolute. I will no longer just follow. I have found that it does no one any good. Sometime when I have more time I will tell you some stories.

The new me came out of the box on Friday while we were waiting for Sydney's MRI. As I was checking her in they said "anesthesia" as though she was scheduled to get some. I said 'no.' She looked at me quizzically and then closed the window and talked to someone back in the room. You see, the funny thing is that they had called two days prior to talk to us about anesthesia. At this time I told them Sydney did not need it. After a thorough discussion I reluctantly agreed to NPO her (just in case), however, we were fundamentally in agreement that she would not need anesthesia but that we would prepare her just in case. We had devised a plan.

A few minutes later, a nurse came out to meet with us in the waiting room. She asked me if Sydney would be needing anesthesia. I said, "No." She then proceeded to tell me that unless Sydney had anesthesia that we would need to reschedule. Knowing how difficult it was to get a slot at the MRI and knowing that it could be weeks, I said, "No." The issue was that the radiologist was not there and without him there was no one to oversee the contrast that she would receive. Had the anesthesiologist been caring for Sydney he would have done the job. I said that is fine. Have the anesthesiologist do it. He was still there. He was planning on anesthetizing Sydney anyway. She became difficult. I did not. She tried to explain the process again. I explained that I had NPOed my daughter all day. I followed every direction and we weren't going to reschedule due to the back office miscommunication. The next thing I knew she was being called over to the window. They mumbled for a bit. She walked over and apologized and explained that they had not received the information. Sydney would be scanned as planned - without anesthesia.

The point here is that if I did not stand up and say something Sydney would still not be scanned today. We would have left and she would have been the one that lost - all because of some back office miscommunication that had nothing to do with her. I have put my foot down, politely, but firmly.

Okay, so we proceeded with the scan. Amazingly Sydney stayed still through the entire MRI with her arm straight over her head. I could not have done it, but she sure did. We were enormously proud and a little gratified after all of the discussion about anesthesia. To be honest, there was little doubt in my mind that she would do it perfectly but, after watching all of the radiology personnel giving us that look that said there was no way she could do it, I was just that much more proud.

Ordinarily, I would go onto tell you about our incredible time at Great Wolf Lodge but I can see that there is still too much to cover so that will have to wait for another day. I have to get to the results. Up front, I will tell you that they are mixed and we, once again, find ourselves in a very strange place. It turns out that Sydney's bone marrow aspirates and biopsies were absolutely clean. There was no evidence of disease. This is great news. Today we should be receiving the results on some more sensitive tests of her marrows which everybody is preparing us to be positive for neuroblastoma. I have not done the research on the lab that they are getting these results from but, from what we understand, nearly everybody receives a positive result from whether they have neuroblastoma or not. So, yep, we have that to look forward to. But still, we have the existing report to be happy about.

The MRI was a different story and although it did not come out and scream neuroblastoma it did not do the opposite either. It is a mixed result and although there is clear evidence of abnormality its meaning is questionable. You see, part of the report is very "cancer-like" and part of the report is not. Here, I will show you the report so we can discuss it. Enjoy.

CLINICAL HISTORY: NEUROBLASTOMA, QUESTION OF ABNORMAL BONE SCAN INVOLVING THE MID RADIUS TECHNIQUE: Extended MRI of the right forearm was performed in the coronal, sagittal, and axial planes using spin echo technique. Sequences included TI weighted STIR, T2 weighted, T I weighted fat sat with 3 ml of IV Magnevist. A total of 223 images were obtained in the evaluation.FINDINGS: The area in question demonstrates abnormal increased marrow signal on the STIR sequences involving the entire proximal third of the radius. Furthermore, there is a small focal area off the medial aspect of the cortex at the junction of the middle and proximal third of the radius that demonstrates a small area of protrusion, with central high signal and seems to be related with the inner osseous membrane. No edema is noted in the adjacent soft tissues and no abnormal enhancement with contrast administration is identified. The overall appearance is not normal but is not particularly good for involvement with metastatic neuroblastoma in that the cortex in general of the proximal third of the radius is intact. Furthermore, no adjacent edema is noted. This latter finding would also go against trauma and infection. The significance of the findings are uncertain, but does not appear to be aggressive. A follow-up exam in 6-8 weeks to determine if there has been a progression would be worthwhile.OPINION: ABNORMAL AREA INVOLVING THE MEDIAL ASPECT OF THE JUNCTION OF THE MIDDLE AND PROXIMAL THIRD OF THE RADIUS WlTH ABNORMAL MARROW SIGNAL BUT WlTH NO ADJACENT SOFT TISSUE EDEMA AND NO DESTRUCTION. THE FINDINGS ARE OF QUESTIONABLE SIGNIFICANCE BUT A FOLLOW-UP STUDY WOULD BE WORTHWHILE.

As you might expect, I have spent most of the weekend studying this report and doing research. The crux of the report comes to some contradictory findings. First, the abnormal marrow signal is cancer-ish. However, it is also infection-ish and trauma-ish. There are all kinds of studies on this but the fact that it is a large area of abnormal signal actually points to a higher probability that it is injury or infection related. However, you will also note that there is no indication of injury. The really confusing part of all of this is the boney protrusion. This is strange in that cortex is intact. Furthermore it is a protrusion and neuroblastoma is a predominantly osteolytic (bone destructing) process and I am confused by that as well. Finally, there is no edema in the soft tissues next to this protrusion which is normally seen with cancer, infection, or trauma. It is a very strange result and not one that I am particularly comfortable with.

I happen to know a little bit about how cancer (neuroblastoma in particular) metastasizes to bones. In fact, I would even go as far as to say that I know about all that has been printed. I know exactly what is known about how neuroblastoma seeks out bones and how it infiltrates them. What I know less about is how neuroblastoma leaves the bones. For me this is the big unknown. Without this picture clear in my head it is hard to truly make an informed decision. I need to figure out what is going on in that bone. Regardless, we are in a holding pattern until we find out more. I have emails out to just about everyone I can think of to come up with a plan. Unfortunately, I just don't know that they will have an answer either. We are all in a quandary.

Today, I am confused by my purpose.

4:56 AM 2/15/2008

As you could tell from my last entry, yesterday went pretty well. The rest of our day pretty much rode on the euphoria of our earlier discovery of nothingness. Her bone marrow aspiration went well and was relatively uneventful. For some reason, outpatient surgery was packed. In fact, Sydney would wake up from anesthesia a full hour before they would have a bed available for her in step down. In fact, it would be another 30 minutes after that until we saw her. That of course did not make Lynley or I particularly happy. We had visions of Sydney calling for her Mommy and Daddy. It was bad enough for her but I can only imagine much worse for the younger children that were being held up or for the families that had not been through this 24 some odd times before. Regardless, we would eventually get to see her smiling face.

It was actually quite funny. She is following in her father's footsteps of being relatively obnoxious (in a cute way) after anesthesia. As we were waiting to be officially discharged, the little bugger kept trying to make an escape. Her mother and I were standing at both ends of the gurney and she would try to sneak around us to get down and make a beeline for the door. She was obviously having a blast. This round of anesthesia was pretty clearly a great trip. Eventually they would wheel her down to radiology where she would finally get an x-ray of the correct arm. We still don't know the results. So, I am assuming it was not life altering and a stress fracture was not clearly evident.

The other piece of good news that would come from the day is that they would leave Sydney's IV in her arm. Potentially, if it survives the night and day, it will save her a fourth poke this week which we are all in favor of. The less exciting news of the day is that the want us to beep her NPO (no food or drink) all day today just in case they have to sedate her. I have tried to explain to them that I had a high level of confidence that she could make it through the entire scan without anesthesia based on her past performance but they would have nothing to do with it. What do I know? They want her to be ready for anesthesia. This has Lynley in a bit of a funk and she is feeling pretty defiant. It would not doubt me that she feeds Sydney a five course meal this morning.

So, what does all of this mean? Why a spot on the bone scan and not on the MIBG. Well, the big question of the day has to be, is Sydney still MIBG avid. This debate has gone on for well over 3 years. The fact is that we don't know. If you believe that she relapsed in the tibia 3 and a half years ago you would have to assume that she is no longer MIBG avid because the spot never showed up on the scan. However, if you are inclined to believe that she never relapsed in the first place then you have to assume that she is probably still MIBG avid. If that is the case, then this finding on MIBG (or lack there of) would be a pretty good indication that this is not neuroblastoma. It is still a 50/50 deal and an unknown but, oh so much better than a confirmation of our worst fears. In fact it is possible that we might get through all of this scanning with no clear answer. I am hopeful that the MRI will show a stress fracture or something clearly not neuroblastoma but that is a pretty long shot. Hopefully this morning we will get a report on the marrows. I imagine it will be next week before we here back on the bone biopsy.

Regardless, I want to thank everyone for their thoughts and prayers. It has been a huge lift throughout this week. Sydney seems to be handling this all very well - probably better than the rest of us. She has tolerated the hectic schedule well and even commented on yesterday as being a fantastic day. You know, she is right. Perspective is everything.

So are my purpii.

10:54 AM 2/14/2008

We met with the radiologist this morning after the MIBG scan. The report is not yet official but I can tell you with a pretty high level of confidence that the MIBG scan was negative. They did not see a spot on her right arm. As I mentioned before, this is incredible news. This is no guarantee that she has not relapsed. For many reasons that I do not have time to go into right now, it could still very well be neuroblastoma. Bottom-line we no longer know how MIBG avid she is. Regardless, this is still wonderful news and a step in the right direction. Currently, we are in the preoperative waiting room and Sydney is watching TV. She is scheduled to go under anesthesia for her bone marrow aspiration and biopsy at 12:00 PM. We are continuing to hope and pray for the best. Hopefully, these bone marrows will continue to show no evidence of disease and keep us moving in the right direction.

Still with hope, purpose and perspective,
Mark

4:19 AM 2/14/2008

Valentines Day. Hmmm, I noticed. Interesting.

Well, here we are. This is it, the early morning hours before the rest of our lives. This is the day. It is truly an odd feeling to sit here in front of my computer this morning. I can't really explain the sensation. First off, I am calm and strangely some how at peace. In one sense, prayers have been answered. I have strength. Yesterday was an incredibly beautiful day with my children. Oddly, it was really no different than any other. Now, I did pick up Sydney from school at noon to take her down to the hospital for her MIBG injection and her second of four IVs this week. That went particularly well and I can tell you that we have nurses Linda and Carla to thank for that. Regardless, it was very routine stuff. We read books. We sat in the waiting room. Lynley talked to Sydney while I shortened her jump rope. There was nothing else. Just us.

Later that afternoon Sydney and I would pick up the others from school. We would giggle and chat on the way home in the car. Once at the house Graham and Sydney made a beeline to climb the fence and disappear into the back yard. Ainsley and I would go in the traditional way (through the door) and make everyone juice and snacks. After that there would be cleaning the leaves out of the pool, climbing trees, jumping on the trampoline, pretend play in the playhouse, chasing the dogs, and various other normal activities. Later on there would be such routine tasks as making dinner and eating. The day would be capped off with stuffing the kiddos in their jammies, a little tickling, and some kisses goodnight.

See, I told you - routine. It was really no different than any other day. So what made this day so incredibly beautiful and special? Why would I rank this up there as one of the best days of my life? The one great thing that neuroblastoma brings into your life is perspective. I liken it to one of those machines that optometrists use to get the proper prescription. You know the one. The machine that you look through. There are lots of lenses and clicking. You look through the lens, they change the lens and then ask you which is better. Many of them are blurry but eventually, when you finally get the right lens, everything becomes crystal clear. It is the same eye chart that you were looking at before but all of a sudden you can see it with such clarity. You can see every detail of the letters and even the words "Made in the USA" can be made out along the bottom of the page. It is revolutionary. It is clarity. It is a miracle. You can see!
Neuroblastoma has the same effect. Although it will not improve your eyesight, it will certainly give you the focus to see what is important. It is clarity in life. The immediate sense of what is important. It is appreciation. It is an entirely new perspective. It so clearly defines purpose that there is no doubt.

I am a typical parent. I am no more or less special than any other. Just like others, I get caught up in life. I am lucky. I have a neat job. I get to fight for kids like Sydney everyday. I get to "do" something about it every day. However, just like any other person, I also have the habit of getting too wrapped up in my work. I come home worried about another child, concerned about whether a drug is going to be released so that we can open another trial, or thinking of another family who needs some help in making a tough decision. The list goes on. I get so consumed with my work that I sometimes forget to sit back and appreciate the wonderful family I have. I let stress, work, and everything else get in the way. This is not to say that I am a bad dad. I pay attention to them. I play with them everyday. I am physically there - just not mentally.

That was what yesterday was for me. It was my time to get in touch with my family - to prioritize them, not only in body but in my mind. I had the opportunity to once again see life through my own children's eyes. I had forgotten what a beautiful world it could be. I am such a lucky, lucky man. I have the greatest family in the world and I know that - again.

So, here I sit. In a little over 4 hours from now I will have a very good idea of whether this will be the best day of my life or the worst. It is just so odd to be sitting here and know that the answers sit so closely on the horizon. I am still strangely calm. I am confident in knowing that no matter what happens that we will get through it. I never want to forget this feeling. I never want to forget what I have again. Today we will get either great or horrible news but the one thing I promise is that I will never forget what yesterday gave me. You may find us shouting with joy or huddled in tears but you will find us together - completely.

At about 8:30 this morning Sydney will begin her MIBG scan. There is no doubt in my mind that by 9:00 AM I will have a clear picture of Sydney's arm on the screen. I will know if that spot lights up or not and whether our worst terror has been realized. The spot will either be there or it won't. If it is there then we will know for sure it is a relapse. If not, we will have hope that it could still be something else. God, please don't let it be there. Don't let this be a relapse.
This scan will be followed by a trip across the street to the clinic where Sydney will have a quick check up before her bone marrow aspiration at noon. Amongst all of this, at some point, it is likely that we will have an x-ray of her right arm. Boy do I hope it is necessary.

Although the thoughts have been through my head a thousand times it is to early to speculate what all of this will mean. There are so many possibilities that could come from today. There is still a long process ahead to truly figure out what is going on. We need success with each and every test.

So, here we go.

I will do my best to update as I can throughout the day. Obviously there will be issues with internet access and time to sit and write but, I will do my best to let you know where we stand. Your prayers are being felt.

With hope, purpose and perspective,
Mark

4:41 AM 2/13/2008

Yesterday, on the way back from Dallas, Dr. Eames called. She spent a little over 20 minutes on the phone with me. You know, after being away from her direct care for so many years, I had forgotten how comforting she was. I knew there was a pretty good reason that we had named Ainsley after her its true impact had just temporarily left my mind. It is difficult to describe. Even with all that is up in the air, she seems so incredibly in charge. She is so reassuring. I would follow her anywhere and do what ever she asked. She was very clear in our conversation. She left no doubts. I did not know where this journey would take us but I was reassured that she had a plan and that she knew exactly what to do. Somehow I had clarity without a single clear answer.

In actuality this is all very simple. We have a spot on a bone scan. For those other parents that have some experience in looking at these the spot appears to be about a centimeter (in life size) and has about 1/3 the intensity of a joint when compared on screen. The spot is not black. It is dark grey. The spot ccould be cancer, trauma, infection, or fluke. The other thing that we know definitively at this point is that it truly is on her right arm. This still hits me as strange but it is something I have come to terms with. We do not believe that this is one of the spots that lit up originally at diagnosis but we do not know that for sure.

Dr. Eames said it was worrisome but not to get myself into a tizzy. We still do not have enough information yet. This still can be nothing. Don't give up hope. We just need more information. On Thursday we had previously scheduled a MIBG scan and bone marrow aspiration and biopsy. We will continue on with those. She has added a x-ray of the right arm on Thursday and an MRI at Cooks' Northeast campus on Friday afternoon. We hope to hear something on the bone marrows and MIBG as early as Thursday evening or Friday morning. I honestly don't know when we will hear on the MRI. Based on all of this we should be able to form some type of opinion as to what is going on. We want the MIBG to be negative and I should have a pretty good idea from looking at the scan myself as to whether or not this spot lights up. We obviously want the bone marrow aspiration and biopsy to come back negative. I think at this point we would like for the x-ray and MRI to show a fracture or nothing at all. A hairline fracture or stress fracture would be a definitive answer to the bone scan where as anything unremarkable would leave us with out any real explanation as to the bone scan. In short, disease anywhere would be bad. A discovery on MIBG, in the bone marrow aspiration, or in the biopsy would be catastrophic.

In the end, Dr. Eames did not provide me with the answer that I was looking for but she did comfort me. She gave us a plan and a direction. She was clear. She was exact and she left no doubt. Somehow, she gave me strength. Once again, I owe her.

Yesterday afternoon Sydney had a little talk with Lynley. "Mommy, is my cancer back?" She really is wiser than words. As always we were completely honest with her.

Lynley: "Honey, we don't know. The bone scan showed a spot on your arm but we don't know what it is. We are going to hope and pray really hard that it has not come back but, if it has, you have the best doctors in the world to take care of you."

Sydney: " At school I did not want to eat lunch. My stomach was very nervous and it did not feel good. Am I going to lose my hair again? Will I still be able to play with my friends? Will I lose my hearing?"

Tough questions and there were more! Her mother would spend quite a bit of time talking to her and I would do the same. I hate that she is burdened with this. It hurts me that she is worried. It is obvious that her mother and I are not nearly as good at hiding our worry as we thought. I wish we could have kept this from her mind until we were sure. It is one thing for me to carry this on my shoulders. I can do that. But, not her. She should not be burdened by this fear. She is just a little girl. In the end, I was honest but tried to provide her as much comfort as possible. I told her that it could be cancer but that it could be something else or even nothing at all. I told her that we loved her and that, no matter what, we would get through it. We are all hoping and praying very hard.

For some unknown reason I spent the afternoon researching bone scans instead of working. It was a useless activity but I could not help myself. It was nervous energy and something to do that felt worthwhile. In the end I know a lot more about bone scans but nothing much that gives me much assurance one way or another. It took about 3 hours to figure out that although I knew a lot more I really knew nothing of value. I was simply filling time until I could go pick up the kids.

I just want to sit and hold my purpii.