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Mike and Michael dressed up for Halloween, our family all dressed up as Hippies, you can see more pictures in the Halloween set on our Flickr page.
Apparently this will be the fall of monthly visits to Cincinnati! NOT that we are complaining, we are thrilled with the hospital and the people there and appreciate the work they are doing to coordinate all of this. We’ll be heading back down on Nov. 17th, Tuesday (the week before Thanksgiving), for the big consultation with the hematologist Dr. Harris to go over all the the testing that was done last week as well as the genetic test results. We’ll be staying the night and then Wednesday will be for any followup testing that Dr. Harris needs/wants. On Dec. 16th, Tue (the week before Christmas) we’ll be going down to Cincinnati for the much anticipated orthopedic consultation to discuss surgery options 8am on Wed; Thur. night will be the sleep study and then we’ll be coming home Friday. Sometime in there (possibly Tuesday) we hope to get the endochronologist in as well–I’m waiting to hear from them. It’s all a little overwhelming, but we’re hoping this means by the end of that week we might have a good idea of where we are going and what we might be able to do to improve Michael’s health.
NOTE: They were able to squeeze him into the Endochronologist so we’ll be going there on Tuesday at 1:30.
**As before, I apologize for how long this update is, but it helps me to line everything out for memory’s sake and some people want all the information**
Today started with a lesson in Cincinnati traffic! We’ve done pretty good so far, in timing things right to get down 71 into the city, but today we ended up in stop and go traffic that made us late–which was okay as there wasn’t anything first thing in the morning that was time specific. There was more blood drawn, it seemed like a lot, but nothing compared to yesterday, we joked about never seeing so many vials of blood drawn for testing and the nurse said she hadn’t even heard of many of the tests they were doing. Since they needed an IV later they just placed the IV for the blood draw and left it so he didn’t have to be stuck twice, something Michael greatly appreciated. Then Dr. Harris came in and we had a lengthy “interview” with questions about health background, family health, history of diseases, etc. He was very thorough and very easy to talk with. He went over some of the results that came back from yesterday’s blood draw. His white blood cells were up quite a bit (5.3) due to being on the GCS-F consistently for the last month+, so that was expected. His platelets were low (60,000), but again, expected as he hovers around 58,000. His lymphocytes were low again, 12% (34-42% is average) with an absolute count of .69–it has been explained that while neutrophils are the “brutes” of the white blood cell family, lymphacytes are the “brains”. Dr. Harris said that we will likely consider IVIG treatment to try and help boost his immune system–IVIG is a procedure that either requires once a month IV infusion or weekly infusion called SCIG which Dr. Harris seemed to suggest would be the better route as you don’t have the increase after the IV and then a month if it declining down and not working as well by the time the next monthly infusion is due. It is my understanding, also, that SCIG has less adverse reactions that the IVIG. Looking at the pictures of two boys getting SCIG treatment from a woman who has been very helpful in sorting out all the new information on Shwachman Diamond Syndrome–I can say that Michael will be horrified by this, given his aversion to needles. But it appears to be quite effective in boosting the immune system in people who have a suppressed immune system so we’ll cross that bridge when we come to it–likely after we visit Dr. Harris in a month to go over all the test results. His Vitamin K (which is connected with blood clotting) levels were also indicated as being low due to something in the coag studies (they tested all the fat soluble vitamin levels which we’ll get back later). Last November when he was hospitalized he had had to have an IV bag of Vit K due to deficiency at the time. Dr. Harris explained that with people who have SDS not breaking down fat properly they tend to be deficient in the fat soluble vitamins, he prescribed a vitamin for all four.
Michael has two out of the three cell failures (Neutropenia and Thrombocytopenia), Dr. Harris explained that the neutrophils were usually the first to go, which we know Michael has had since birth or thereabout, the platelets generally fail next, Michael’s failed at around age 8–or rather, we became aware of it then due to his platelets not responding to multiple transfusions when he was severely sick. The last to go are the red blood cells (Anemia) which Michael has not had an issue with at this point staying in the past around 7 for RBC levels. The problem with judging that, however, is complicated by his severe respiratory problems. Dr. Harris explained that people with lung issues (smokers, people with lung cancer, people like Michael with severely compromised lung function, etc.) are having trouble getting and moving oxygen around the body (the job of red blood cells) so that the body forces a higher amount of red blood cells to be made to try and help the deficient lung function. So that while we might see a HGB level of 13.8 (average 13.3-17.7), that is a false reading that may actually be a 10 or under if he didn’t have the compromised lung function. His RBC levels were at 4.26 which is lower than usual even for him. Dr. Harris is concerned that these are indications that the third cell line is actually compromised, or at the least, moving to that direction. We will, I understand, have a more complete picture of that after all the tests results come in from the other blood work as well as the bone marrow aspiration and biopsy.
Some of his liver counts came back high, and while in the past he has had mildly elevated liver counts they were never symptomatic, I don’t know what those counts were. They sent him down for an ultrasound of his liver and his pancreas–I’ll call on those results Monday.
The bone marrow aspiration and biopsy went smoothly and quickly, he was completely sedated in the OR and other than having a very hard time waking up–once we were released and sitting down in the cafeteria waiting for a better traffic window to head up, he woke up from dozing looking very confused and said, “I thought I’d wake up in bed…not in a cafe!” He remembers being in the OR and having the mask placed, and then he remember waking up in the cafeteria, nothing about recovery at all! We had a good laugh about that all the way home.
Some of the results we will have back ranging from the next day or so to a week and a half, others will take longer. The plan is to go back down the week of November 17th and meet with Dr. Harris again to go over all the blood work, bone marrow results, and genetic testing results. We’re hoping to piggyback this consultation with the sleep study the Cincinnati pulmonologist wants done. Otherwise, the other earlier results I will call about in the next week or so. The worst is over for Michael, he is thrilled to be done with this and, in general, fed up with the entire testing arena. The worst is starting for me, I do not wait for test results well–a day at a time.
We were back in Cincinnati today for a consultation with Dr. Harris, a hematologist who specializes in Shwachman-Diamond Syndrome, for multiple types of blood tests and tomorrow a bone marrow aspiration and biopsy if all goes well. Today was fairly quick as far as consulting as they had just gotten faxes from Michael’s local hematologist so they skimmed through those to decide what tests to do and opted for the full spectrum of testing. Michael had a very difficult time as vial after vial was taken, he really has issues with blood tests and the longer it went and the more vials taken the more panicked he became. He did well, though, and stayed still until it was done. Tomorrow they will do a bit more blood work and then the bone marrow testing–we’ll also go over some of the labs done today that should be ready tomorrow–much of the testing, though, won’t be in fully until about a month.
He did agree that looking at all the testing done last time at Cincinnati that he seemed to fit very well with Shwachman-Diamond, that the bone abnormalities were consistent with SDS, and, so far, the blood issues such as Neutropenia and low platelets as well. He checked with the genetic testing lab to make sure they had received and were processing the genetic testing drawn that time around and they do have it, so by next month that should be in as well. He was also very clear that while they were using it to treat certain cases of SDS, even if indicated, he would not be able to have a bone marrow transplant due to his other health issues. He did tell us that Michael’s lymphocytes were low, something I’ll have to get clearer on before I explain what that means, and he also talked about a form of immune system boosting called IVIG. Tomorrow when we talk to him for the extended consultation I will try to get a clearer understanding of these. I know that it is a type of white blood cell separate from the Neutropenia issue that are quite important. We’ll see what today’s CBC shows and I’ll go into that more tomorrow night.
All in all it was a longer day than expected and Michael had a difficult time, but by evening he was perked back up again and we are getting ready to get some sleep to be prepared for tomorrow. Because of his ongoing respiratory issues they were reluctant to do the testing in their clinic OR as planned and moved him over to the regular OR–so as of this afternoon, the procedure is rescheduled for 1:30pm.
I used to have the whole fabulously integrated uniform of a super hero. I had the armor plated jumpsuit, the all important mask settled carefully into place so as not to reveal my true identity, and a cape that fluttered behind me in the wind. I knew just what to say, just how to act, I was the mom that doctors thought was a nurse because I had it all together, I was the mom that sat in on procedures because I would not fall apart, and I very rarely cried in front of anyone. I’ve cried in front of three friends in the last week and I want to know–who took my cape?
Of course, I was also the one with the terrible “irritable bowel syndrome” thats true medical description was “woman who won’t cry or talk about her emotions or fears and so her body is forced to release all the pent up emotions SOME way so please pass her the Immodium and make sure it is the Advanced formula.”
I know that the way I handled the stress of Michael’s illnesses the first few years of his life was not healthy, I am so much older, and so much wiser, and I have learned so much–so why do I find myself digging through the back of my closet for my mask? I’ll do without the jumpsuit, and really, you can keep the cape, but I need the mask! I find myself annoyed with the world, irritated at the grown women at the mall stealing $6 earrings–really? you really MUST have those earrings even though don’t you realize there are children in the world with serious medical issues and PLEASE just let me buy you those earrings if they are so vital. I am irritated that our whole world has stopped and we are holding our breath yet again and waiting for it to, please, start turning–and the rest of the world keeps moving.
Still. I catch myself breathing little worthless staccato breaths and I stop and take a breath. I am kinder to myself this time around, I look in the mirror at tired, puffy eyes and tell myself it is okay, it is okay to be sad and afraid and it is okay to not be “just fine, I’m just fine, it’s all okay” to myself or to the world in general. It’s also okay to laugh, to stay up too late and watch TV with my kids, to dance around the house singing “I’m a Survivor” with Destiny’s Child while a ridiculous lump builds up in my throat and my daughter laughs and my son groans at what he deems to be the most irritating song ever–it’s okay.
I found a picture the other day when I got the sudden and uncontrollable urge to clean up a little shelf under my records that is full of a mess of everything I didn’t bother to find a place for–even though no one can see it and it isn’t bothering anything but who needs to work on that paper when I need to clean this space out NOW…now would be the time for that breath.
It’s a picture of my grandfather holding me when I was a baby and even though he died when I was eight and he never knew my children I know that he would have loved them so much, and I miss him painfully and inexplicably.
I’ve been thinking a lot about coping the last few weeks, and this is what it looks like. It looks like a woman who is going on too little sleep, cries more than usual, but also laughs and dances, who forgets to breathe but then remembers, who snaps too quickly at her husband but loves him and is glad he is here. Coping looks like me with no armor plated jumpsuit, no cape fluttering in the wind, and no mask. Just me.
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(Note: Originally posted in my personal blog but feel it is relevent for this one as well, still haven’t sorted out how I’ll deal with that in the future)
We got a call yesterday afternoon that Dr. Harris (a hematologist/oncologist who specializes in bone marrow failure issues, runs the Bone Marrow Failure clinic, and is very active in Shwachman-Diamond care and research) wanted to see Michael and that he could do so next week. So we’ll be heading down for a consultation on Weds. morning and testing on Thurs. morning. They’ll be doing lab work, a bone marrow aspiration (where they insert a needle and withdraw the liquid part of bone marrow) and a bone marrow biopsy (where they remove a more solid sample of the spongy material in the bone). Michael’s platelets and white count were quite low from the labs in Cincinnati last month, although he is always pretty low so that wasn’t a great surprise. It is my understanding that they will be looking, among other things, to see if the cells are shifting towards leukemia, which can happen with people whose bone marrow doesn’t work correctly like Michael’s. Doctors from the last testing also had said that orthopedic would want information gathered from this testing to help determine if/what they will do surgery-wise.
Michael is having a difficult time with this, he has an extra hard time with anything related to needles and is just frustrated at this point in his body not working and the testing being done and the hovering issue of surgery. To say that we are all a bundle of nerves and anxiety is an understatement and we appreciate your thoughts and prayers.
A lot of people have been wondering where things are after 3 weeks so I thought I’d write a bit of an update. We’re sort of in a holding pattern now waiting for them to set up consults and the sleep study. I’ve been talking with the scheduler and hope to have some dates soon. The tests they did on Michael’s pancreatic enzymes came back normal, which, if I understood correctly, wasn’t really surprising given his age. The only other test we are waiting for is the genetic testing, which can take up to 8 weeks, and we are at 3 weeks.
I talked to Michael’s hematologist and she is going to call down to talk to everyone and see what plans are and whether they are going to want a bone marrow biopsy before he would undergo any major surgery. Normally with Neutropenia they do one yearly, but due to his lung function issues, they have opted not to take the risk of putting him under for it. She did say that from a hematologic point of view, the change of diagnosis doesn’t change Michael’s treatment as he would be on GCS-F shots either way, so that was good to hear–that what we have been doing is what we would have done if he’d been diagnosed with SDS earlier.
The extra coughing from the cold Michael had is cleared up now, but he is still coughing quite a bit and gets out of breath easily. Our main concerns for the immediate future is keeping his weight from dropping and trying to add some weight on–his face looks like he has lost more in the last week and we need to halt that. They put him on medication last year that did make him gain weight, but he hated the side effects so much (it made him so tired he couldn’t hardly function) that I’m hoping we can find an alternative.
So that is where we are, thank you so much for keeping Michael and our family in your thoughts and prayers, as soon as we know what is happening next, we will let you all know!
