Cold and Flu Season
remember last year I waited and waited for the beginning of spring and the end of the cold and flu season. We wanted so badly to be able to take Max out of the house and be a "normal" family. I had no idea what was in store for us.
Much has happened this past month and the ups and downs of grief continue; if only the ups could be much higher and the downs not so deep. Last week I finally had the courage to call our social worker at Fairview to see if Max's autopsy report had been completed. A few days later I received a call from Dr. Grewal, Max's primary BMT doctor. We went over the report on the phone, which confirmed just how sick Max really was. I hesitated to give details in this update, partly because I didn't know if I could bare rehashing my beautiful boy's suffering, partly because I didn't know if anyone cared to read such a thing. But then I realized that we had the autopsy done to help other Hurler and BMT children, and this information could quite possibly help one of our readers. I will do my best to explain everything, and I hope I understood all of the information correctly.
As we already knew, Max was suffering from uncontrollable GVH of the skin and gut. The report revealed that his gut was SEVERELY damaged and scarred. In addition, his pancreas was enlarged (reason unknown), and his liver had excess bile from either GVH or TPN (IV nutrition). Hurler deposits (GAGS) were found in his coronary artery, but the deposits were not significant enough to explain his need for blood pressure pressers (the more likely reason was infection). As we knew, he had hydrocephalus (fluid on the brain). At the time of diagnosis, it was unclear whether this was caused by the brain bleed he had as a preemie, or by Hurler Syndrome. The autopsy showed Hurler Syndrome to be the culprit.
Two things found in the autopsy took the doctors by surprise. The first was the presence of cytolytic thrombi in Max's lungs. These are kind of like a stroke in the lungs. The doctors were surprised at this finding because they are not very common, and because Max had so many of them. They are only found in patients with GVHD, and often are misdiagnosed at first as fungus via a CT scan. They are definitively diagnosed by a lung biopsy or autopsy. They went undetected on Max's CT scans because of the bleeding in his lungs.
What frustrated us so much during Max's treatment was the disagreement between different doctors on whether or not GVH of the lung exists. Some argue that GVH of the lung does not exist because of the lack of apoptotic cells (cells which have self destructed), present in GVH of the gut and skin. Cytolitic thrombi are not referred to as GVH of the lung per se. However, they are treated the same as GVH. Some medical personnel think they are manifestations of GVH in the lung, others believe they are a separate entity. They also found bleeding in Max's lungs, something we also already knew, which also was the cause of death listed on Max's death certificate. While Hurler children are more likely to have lung bleeds during transplant compared to patients transplanted for other diseases, thrombi are not known to cause a lung hemorrhage, though in Max's case they may have been a contributing factor.
The other surprising bit of information learned from the autopsy involved Max's brain. More Hurler deposits were found in Max's brain than in any other Hurler child autopsied at Fairview, including those who were significantly older than Max. Theoretically, children older than Max at the time of death should have had more deposits since the disease had more time to progress. Dr. Grewal and Dr. Charnas, Max's neurologist, are very puzzled by this, especially since Max was doing so well neurologically at the time of transplant. They have pulled the autopsy reports of the other Hurler patients and will be reviewing them in more detail in hopes of learning something from this.
Where does all of this information leave us? I wish I could say we now have total closure, but that is not exactly true. We do know that we made the right decision to withdraw life support. Dr. Grewal said that knowing how sick Max was he feels that they did not have the technology to save him. We could see that he was suffering greatly, the autopsy showed that he was suffering more than we imagined. Our hearts are forever broken at the thought of this, and we will never understand why such a beautiful child such as Max had to endure so much. The world simply is not fair.
One thing we do know for certain is that Max was stronger and more determined than we realized. Because he was born so prematurely, we held our breath for every developmental milestone. We were filled with sadness, as we knew he was further behind than he should have been. Then he was diagnosed with Hurler and we realized how much he was overcoming. In some ways his diagnosis set us free, and we were able to enjoy what he could accomplish each moment, instead of worrying about what he wasn't able to do. Now that we know how severely his brain was damaged by Hurler, we are even more proud of him. Max was born 9 weeks early. He had a brain hemorrhage at birth. He had asthma. He had all of the problems associated with Hurler Syndrome. His brain was filled with Hurler deposits. Despite all of this, before transplant he was pulling himself to stand and walking along the furniture. He was sitting and crawling. He knew how to feed himself, and give hugs and kisses when asked. During chemo he mastered pushing to sit from his belly. He was a star and we knew it, we just didn't know exactly what he was battling in order to perform all of these tricks. He had amazing will and spirit and we are so very proud. We are also thankful for his three therapists who worked with him each week to help him gain these skills and have a better quality of life.
This weekend we were able to summon the strength to finally go and purchase the marker for Max's grave. We didn't do it after he died because it was winter in Chicago and they cannot be placed in the frozen ground. How we wished we had made the arrangements back in November, when we were somehow possessed by people able to make life and death decisions. We put it off as long as we could, but now that the snow finally melted we knew it was time. Grace picked out the color (we had to steer her away from pink), and the stone will read "Our Brave Boy, Max Ciacciarelli." We also ordered a ceramic tile with Max's picture on it to be placed on the stone.
Now on to Grace news. This month was busy for her as well. It started off with parent-teacher conferences at preschool. Mike and I both went, and were most proud when the teacher told us what a lovely transition Grace made into the class mid-year. Other parents have commented on her social skills as well. We are so lucky to have her, and so lucky that she is so adaptable. It certainly helped throughout the past year. Grace's teacher also told us that she talks about her brother all of the time, and seems at peace with him being in heaven.
Grace recently gave us quite a scare. She had the stomach flu last week like half of her preschool class and then she caught a cold. She developed a pretty bad cough and the next day she started breathing fast. I called the doctor and she told to me try a few things which I did and they seemed to work. Then right before bed it happened again. It was if she had just run a race when all she was doing was laying in bed. At first I thought I was overreacting or imagining things but then Mike got home and told me I wasn¹t crazy.
We took her to the ER which was stressful for all of us. Grace told me recently that if you go to a hospital you don¹t ever get to come home again. She must have been so scared, but she was really brave. She got a breathing treatment, which is a really simple thing, but was hard for Mike and I to watch. They gave her the same oxygen mask Max had several times in the hospital. She also got a chest x-ray (something Max had one or more times a day). She handled it all really well until they swabbed her nose for RSV then she cried her eyes out (I don¹t blame her).
The x-ray showed a little pneumonia, though her pediatrician said it was borderline and could have just been bronchiolitis. Her ear was also infected. We stayed up all night in shifts watching her breathe. We were so freaked out, and I think I was nearing a nervous breakdown at one point. You would think with all we have been through that this type of thing would be no big deal to us, but it was extra scary I think.
The other day I had to break the news to Grace that her life-long best friend and next door neighbor was moving. Some of you may remember our neighbors who ran the awesome Mighty Max raffle, and who have been so helpful to us this past year. When hearing the news Grace immediately began sobbing. Her brother died, her cousins moved to Maryland and her best friend is moving soon as well. I think sometimes it is just too much loss for one 4 year old to bear in such a short period of time. She calmed down when I told her they would only be a short drive away, and she seems to be okay with things now. She loves her friend so very much, and we are so grateful to have had such wonderful neighbors for the past four years. Though we are sad to see them go, we are glad they are not going too far. They are really great people who deserve every happiness in life.
We are so sad that we lost our son, but we are most sad about Grace losing her brother. Being Max's big sister brought out so many wonderful things in her. He was so much entertainment for her. He was her buddy, her Bubba. When discussing lucky things on St. Patrick's Day Grace listed not having Hurler Syndrome and getting to be on earth as her lucky things. While we realize Grace probably understands the important things in life better than most adults, we often wish she could be a "normal" four year old for a day and list some things like her classmates did - toys, money, and treats.
Before I end this rather long update I must also extend our deepest thanks to those who made contributions to the RMH in MN in Max's name. Because of your donations, we recently learned that Max's name was placed on a tile on a wall at the house. On the tile is a glass heart, and a replica of the heart was sent to us as well. It is such a nice way to honor Max's memory. The RMH truly is the house that love built.
Thank you to everyone who continues to check in on us. It means so very much. Happy Easter and Happy Spring!
--Margaret, Mike & Grace
Much has happened this past month and the ups and downs of grief continue; if only the ups could be much higher and the downs not so deep. Last week I finally had the courage to call our social worker at Fairview to see if Max's autopsy report had been completed. A few days later I received a call from Dr. Grewal, Max's primary BMT doctor. We went over the report on the phone, which confirmed just how sick Max really was. I hesitated to give details in this update, partly because I didn't know if I could bare rehashing my beautiful boy's suffering, partly because I didn't know if anyone cared to read such a thing. But then I realized that we had the autopsy done to help other Hurler and BMT children, and this information could quite possibly help one of our readers. I will do my best to explain everything, and I hope I understood all of the information correctly.
As we already knew, Max was suffering from uncontrollable GVH of the skin and gut. The report revealed that his gut was SEVERELY damaged and scarred. In addition, his pancreas was enlarged (reason unknown), and his liver had excess bile from either GVH or TPN (IV nutrition). Hurler deposits (GAGS) were found in his coronary artery, but the deposits were not significant enough to explain his need for blood pressure pressers (the more likely reason was infection). As we knew, he had hydrocephalus (fluid on the brain). At the time of diagnosis, it was unclear whether this was caused by the brain bleed he had as a preemie, or by Hurler Syndrome. The autopsy showed Hurler Syndrome to be the culprit.
Two things found in the autopsy took the doctors by surprise. The first was the presence of cytolytic thrombi in Max's lungs. These are kind of like a stroke in the lungs. The doctors were surprised at this finding because they are not very common, and because Max had so many of them. They are only found in patients with GVHD, and often are misdiagnosed at first as fungus via a CT scan. They are definitively diagnosed by a lung biopsy or autopsy. They went undetected on Max's CT scans because of the bleeding in his lungs.
What frustrated us so much during Max's treatment was the disagreement between different doctors on whether or not GVH of the lung exists. Some argue that GVH of the lung does not exist because of the lack of apoptotic cells (cells which have self destructed), present in GVH of the gut and skin. Cytolitic thrombi are not referred to as GVH of the lung per se. However, they are treated the same as GVH. Some medical personnel think they are manifestations of GVH in the lung, others believe they are a separate entity. They also found bleeding in Max's lungs, something we also already knew, which also was the cause of death listed on Max's death certificate. While Hurler children are more likely to have lung bleeds during transplant compared to patients transplanted for other diseases, thrombi are not known to cause a lung hemorrhage, though in Max's case they may have been a contributing factor.
The other surprising bit of information learned from the autopsy involved Max's brain. More Hurler deposits were found in Max's brain than in any other Hurler child autopsied at Fairview, including those who were significantly older than Max. Theoretically, children older than Max at the time of death should have had more deposits since the disease had more time to progress. Dr. Grewal and Dr. Charnas, Max's neurologist, are very puzzled by this, especially since Max was doing so well neurologically at the time of transplant. They have pulled the autopsy reports of the other Hurler patients and will be reviewing them in more detail in hopes of learning something from this.
Where does all of this information leave us? I wish I could say we now have total closure, but that is not exactly true. We do know that we made the right decision to withdraw life support. Dr. Grewal said that knowing how sick Max was he feels that they did not have the technology to save him. We could see that he was suffering greatly, the autopsy showed that he was suffering more than we imagined. Our hearts are forever broken at the thought of this, and we will never understand why such a beautiful child such as Max had to endure so much. The world simply is not fair.
One thing we do know for certain is that Max was stronger and more determined than we realized. Because he was born so prematurely, we held our breath for every developmental milestone. We were filled with sadness, as we knew he was further behind than he should have been. Then he was diagnosed with Hurler and we realized how much he was overcoming. In some ways his diagnosis set us free, and we were able to enjoy what he could accomplish each moment, instead of worrying about what he wasn't able to do. Now that we know how severely his brain was damaged by Hurler, we are even more proud of him. Max was born 9 weeks early. He had a brain hemorrhage at birth. He had asthma. He had all of the problems associated with Hurler Syndrome. His brain was filled with Hurler deposits. Despite all of this, before transplant he was pulling himself to stand and walking along the furniture. He was sitting and crawling. He knew how to feed himself, and give hugs and kisses when asked. During chemo he mastered pushing to sit from his belly. He was a star and we knew it, we just didn't know exactly what he was battling in order to perform all of these tricks. He had amazing will and spirit and we are so very proud. We are also thankful for his three therapists who worked with him each week to help him gain these skills and have a better quality of life.
This weekend we were able to summon the strength to finally go and purchase the marker for Max's grave. We didn't do it after he died because it was winter in Chicago and they cannot be placed in the frozen ground. How we wished we had made the arrangements back in November, when we were somehow possessed by people able to make life and death decisions. We put it off as long as we could, but now that the snow finally melted we knew it was time. Grace picked out the color (we had to steer her away from pink), and the stone will read "Our Brave Boy, Max Ciacciarelli." We also ordered a ceramic tile with Max's picture on it to be placed on the stone.
Now on to Grace news. This month was busy for her as well. It started off with parent-teacher conferences at preschool. Mike and I both went, and were most proud when the teacher told us what a lovely transition Grace made into the class mid-year. Other parents have commented on her social skills as well. We are so lucky to have her, and so lucky that she is so adaptable. It certainly helped throughout the past year. Grace's teacher also told us that she talks about her brother all of the time, and seems at peace with him being in heaven.
Grace recently gave us quite a scare. She had the stomach flu last week like half of her preschool class and then she caught a cold. She developed a pretty bad cough and the next day she started breathing fast. I called the doctor and she told to me try a few things which I did and they seemed to work. Then right before bed it happened again. It was if she had just run a race when all she was doing was laying in bed. At first I thought I was overreacting or imagining things but then Mike got home and told me I wasn¹t crazy.
We took her to the ER which was stressful for all of us. Grace told me recently that if you go to a hospital you don¹t ever get to come home again. She must have been so scared, but she was really brave. She got a breathing treatment, which is a really simple thing, but was hard for Mike and I to watch. They gave her the same oxygen mask Max had several times in the hospital. She also got a chest x-ray (something Max had one or more times a day). She handled it all really well until they swabbed her nose for RSV then she cried her eyes out (I don¹t blame her).
The x-ray showed a little pneumonia, though her pediatrician said it was borderline and could have just been bronchiolitis. Her ear was also infected. We stayed up all night in shifts watching her breathe. We were so freaked out, and I think I was nearing a nervous breakdown at one point. You would think with all we have been through that this type of thing would be no big deal to us, but it was extra scary I think.
The other day I had to break the news to Grace that her life-long best friend and next door neighbor was moving. Some of you may remember our neighbors who ran the awesome Mighty Max raffle, and who have been so helpful to us this past year. When hearing the news Grace immediately began sobbing. Her brother died, her cousins moved to Maryland and her best friend is moving soon as well. I think sometimes it is just too much loss for one 4 year old to bear in such a short period of time. She calmed down when I told her they would only be a short drive away, and she seems to be okay with things now. She loves her friend so very much, and we are so grateful to have had such wonderful neighbors for the past four years. Though we are sad to see them go, we are glad they are not going too far. They are really great people who deserve every happiness in life.
We are so sad that we lost our son, but we are most sad about Grace losing her brother. Being Max's big sister brought out so many wonderful things in her. He was so much entertainment for her. He was her buddy, her Bubba. When discussing lucky things on St. Patrick's Day Grace listed not having Hurler Syndrome and getting to be on earth as her lucky things. While we realize Grace probably understands the important things in life better than most adults, we often wish she could be a "normal" four year old for a day and list some things like her classmates did - toys, money, and treats.
Before I end this rather long update I must also extend our deepest thanks to those who made contributions to the RMH in MN in Max's name. Because of your donations, we recently learned that Max's name was placed on a tile on a wall at the house. On the tile is a glass heart, and a replica of the heart was sent to us as well. It is such a nice way to honor Max's memory. The RMH truly is the house that love built.
Thank you to everyone who continues to check in on us. It means so very much. Happy Easter and Happy Spring!
--Margaret, Mike & Grace
posted by Mike Ciacciarelli
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