You are currently browsing the monthly archive for September, 2008.
My brother Craig (you can see pictures of my nephews and his process of trying to build a wall in Mongolia on his flickr site) sent me an email that he had written a song for Michael. To be honest, I thought it was going to be a humorous song as Craig is a pretty funny guy. So I printed out the lyrics and started to read them out loud and we all started to cry and I handed it over to my husband who started singing it and then couldn’t either. We thought it sounded like a country song, Craig said it was a bad cross between Springsteen and Willie Nelson, which I think is accurate except for the “bad”! From a non-singer/songwriter, we are very impressed, especially given he mixed the music in a ger with 2 small boys!
Lyric’s to Michael’s Song:
Heroes are born in bad times
And bad times God knows you’ve seen
With a heart of Gold and a Mother’s love
You’ve walked a road where few have been
Well everyone of us, yeah, we’ll see a fight
Take our best shot sometimes for the wrong but mostly for the right
But on the high and pure road in the struggle for a life
The hero is born like a lone star breaking through the night
And you are my hero
The little guy with the heart of gold
Your laughter sparkles down like a sunny sky rain
Sure am glad to know you, and you’ll be in my heart until we meet again.
I guess a hero’s job is not to break though he may bend
But of tubes and doctors it seems there’ll never be an end
And like a life line’s that made up of a thousand thorns
It’ll save you but you’re skin’s still bleeding and, it seems, forever torn
With a story bigger than it seems your shoulders could ever bear
You struggle on ’cause another choice, well, it just ain’t there
We feel a strength in you that tells us more than we could ever see
With a faith to step out on the water and find the rock beneath
And you are my hero
Like a knight from tales long ago
A reason to smile; to walk another mile
Sure am proud to know you, and we’ll meet up again in just a little whileAnd you are my hero
The little guy with the heart of gold
Your laughter sparkles down like a sunny sky rain
Sure am glad to know you, and you’ll be in my heart until we meet again.
Yeah, I sure am glad to know you, and you’ll be in my heart until we meet again.
—————————
Thank you, Craig, Michael loves it and is very excited that you wrote him a song, he has it on his iPod!
Coming up:
Friday, Oct. 30th Michael is having a central line (semi-permanent IV port) placed into his chest and also doing a Bone Marrow Biopsy at the same time to maximize the sedation.
**My brother Craig lives in Mongolia and has a long standing joke with Michael about being arch nemesises (a word?)–or rather Craig jokes and Michael rolls his eyes and laughs. I just wanted to share this because I think he is right and that there are so many kids out there who are unrecognized heroes**
Hellooo Michael,
I have been reading your mom’s reports on all the testing and what is going on. I have decided that I don’t want to be your arch nemesis anymore. Quite frankly, I think you are too tough for me. I’m going to have to find an arch enemy that is a bit less tough than you are as I really don’t like getting beat in my epic battles. I say that because you are definitely one of the toughest people I know. You have been through a lot and you are still fighting on. I’m really glad to be your uncle and although I’m just a bit (and I emphasize the “just the bit” part) older than you I am learning a lot from your tough spirit. Keep it up. A hero is born through difficult times and I think you are definitely a hero. I told your mom this but I think you should hear it as well: they just finished giving out a bunch of medals to some people who can swim quickly from one end of a pool to another, to people who can run around a big circle really fast, to people who can throw a heavy ball a couple of yards, to a person who can jump high, etc.. Everyone is making heroes of those people but this world,as usual, has missed the real heroes and given the medals to the wrong people. The Olympics seems very stupid in comparison to what you all are doing. In my book, you get a gold medal. Keep up the good work.
Craig
When we left Cincinnati last Thursday they told us that the doctors would be getting together this week on Wednesday to gather all the results and discuss their findings, hopefully come to some conclusions and come up with a “next step” plan. I had expected to hear from them tomorrow but got a call from them today.
There are still some tests out there but with the information they had before, the bone density scans, and the survey of x-rays they made they have concluded that he does indeed have Shwachman-Diamond Syndrome. This is not surprising to me as going down a list of SDS symptoms I can pretty well check each one off as “yes, got that covered.” Still, it is disconcerting to us all, Michael included, to set aside a label (Jeune’s Syndrome) that has been so much a part of our mindsets over the last 18 years. I feel out of depth going from relative Jeune’s Syndrome expert to knowing nothing about something that has such an impact on Michael’s health–I’ll have to quickly remedy that.
- Some good news in that the lesions on his stomach they biopsied came back normal and the Impedence probe testing came back normal as well–no reflux.
- As I noted, the DXA scan came back with low bone density and a definite need of supplementing vitamin D at the least, a doctor will be calling directly with other results and any other supplement needed for that. What way his having low bone density will affect the major surgery we’ll be facing in the future isn’t clear right now, but I can imagine that will add a complication to an already complicated problem.
- A Silverman Survey (sp?)–which was a series of x-rays on the long bones, knees, and various other bones–was conducted and was shown to be consistent with and very clearly Shwachman-Diamond Syndrome (apparently there is “tubulation of the long bones” but I’m not clear what that means as yet).
Where do we go from here? Well, the next step is a trip back to Cincinnati for a sleep study for purpose of seeing how shallow his breathing is at night, whether he is actually getting into full sleep, and whether he is releasing enough carbon dioxide. This will help decide whether to implement something like CPAP to force him to take deeper breaths both to improve his health currently and because he will almost certainly have to be on this after surgery and getting him used to it before hand may be a good idea. While down there for the sleep study they want us to have consulations with an orthopedic doctor to start the process of “what do we do” with the spinal and rib cage issues. Along with that, a consult with a hemotologist as Shwachman-Diamond Syndrome is a bone marrow failure syndrome and his Severe Neutropenia (along with his low lung function) will be something that greatly concerns the orthopedic doctor. Finally, a consult with an Endocrinologist for nutritional and hormonal issues. I’m suppose to call on Friday to start the ball rolling on setting those up.
As before, we are at this point just overwhelmed with the new information and and some of the results that last week gave us–we understand the importance of information, but digesting so much that is not the greatest news, all at once, is difficult. Regardless, we have learned from 18 years of experience that the best and only way to live with chronic health problems is to arm yourself with knowledge to the best of your ability, and then simply take each day and each decision as it comes. We’re doing what we can nutritionally, supplementally (adding in vitamins A, D, E, & K), and figuratively drowning him in water as he buckles down and has been drinking 8 glasses of water a day. Sometimes you just have to run with the little things you can change.
I know that it has been a long week for everyone, last night Michael was talking about how he couldn’t understand why so many people were calling and emailing–he just wasn’t that important, he thought. I told him that he would just have to accept that fact that there are so many people out there who love him and care about him being as healthy as possible.
I guess I didn’t want to leave things on the last email of images of a curved spine and crazy right rib cage–we are all trying to make sense of what do do with all the information we gained this week and will still be trickling in. We’re thankful that we have it as information is paramount to taking the proper next steps–but in many ways some of it is information we’d like to erase from our minds because, at least for me, it makes it difficult to sleep at night–it is all very overwhelming. But there is more than that and I tried to sort that out in an essay, and as I know not all of you being updated all week read that, I thought I’d send it along:
IMPERFECT BEAUTY
Even though I have more reason than some and less than others to understand how tenuous and fragile life is, how precious it is for its very fragility–I forget sometimes. I also forget how beautiful and strong the human body is–even more so in its flawed forms. Like trees struck by lightening, damaged, deformed, that twist around and grow towards the sun into stunning sculptures of resiliency. There is a Japanese term “Wabi-sabi” that identifies the beauty in things that are “imperfect, impermanent, incomplete”. It’s more than something that is imperfect but still beautiful, but an understanding that some things are even more beautiful because of their imperfections.
We live in a day and age where a mother can sit in a doctor’s office and look in stunning, 3D detail at the bones and organs of her son–can watch it spin and turn at the click of a mouse, travel down the trachea and into the lungs and see all the details once upon a time people never dreamed of seeing–and maybe were never meant to see. Something shifts, painful and raw, in seeing with such clarity the tangle of abnormally formed ribs and a spine once straight curve, twist, and bend. In the wake of the pain, though, in the long pause of a breath stifled then regained–there is an awareness of awe. That a body struggles against such odds, shifts and adjusts itself, accommodating and maximizing its strengths. One airway lost and the other takes up the slack, breathing in and out with a steadiness of purpose. “Put one foot in front of the other,” Kris Kringle sings to the snow monster, and the body does, just moves along with what is and works to the best of its ability. There is a simple, graceful beauty in the curve of a spine–an imperfect, albeit dangerous, beauty.
It is no wonder that Buddhists love the image of a lotus blossom, rising out of the mud, fed not by perfectly balanced soil and fertilizers but by the muck at the bottom of shifting, murky waters. It reminds me of the medieval concept of the wheel of fate and the idea of people tied to that said wheel, sometimes swung up to good fortune at the top, sometimes dropping down to bad fortune at the bottom, sometimes on the upswing, sometimes on the downswing. I think we often live for those moments of perceived perfection at the top, floating like the lotus and soaking up the sun–but life isn’t just the top of the flower, it’s down deep in the roots and the muck too, and in between on the long stem shifting nutrients back and forth. Life is the high moments and the low moments and the beautifully boring moments in between. It’s in a daughter’s turned up nose and cleft chin and ginormous big toe; in a son’s bright blue eyes and graceful hands and imperfect body. Life is all about kid’s dressed up and lip syncing to “Secret Agent Man” and a brother and sister leaning their heads together on a hospital bed playing “Final Fantasy” while waiting for one to go back to an operating room–life is dirty and painful and sunlit and joyous and sometimes it’s all of them tangled up into one.
Paul Simmons, who had, and eventually died from, ALS, wrote in his book Learning to Fall:
Don’t talk to me about flowers and sunshine and waterfalls; this is the ground in which life sows the seeds of our fulfillment. The imperfect is our paradise.
Let us pray then that we do not shun the struggle. May we attend with mindfulness, generosity, and compassion to all that is broken in our lives. May we live fully in each flawed and too human moment, and thereby gain the victory.
* Michael had a bit of a rough morning with a jagged coughing spell that made his oxygen drop quite a bit but once he had a breathing treatment it pulled it up. They came in and pulled out the Impedence probe that was placed during the scoping procedure that had stayed in all night–he wasn’t too thrilled with the tube out of his nose, but it wasn’t too bad. The point of this test is to see if he is having reflux of stomach acid up into his esophagus and if so, how far up. We’ll find out results of that next week.
* Next stop was down for a Video Swallow Study where they mixed dye paste into pudding, coating fruit loops, and liquid dye into Coke–it was pretty disgusting looking. Michael sat in a chair and ate/drank in different ways while they recorded the foods going down the pipe–it was kind of gross to watch according to Michael and definitely gross to eat, but he really cooperated well and we got a good laugh out of it. The point of this test is to see if food he is eating/drinking is getting aspirated into his lungs and causing extra problems. Again, results next week.
* He went for a series of x-rays called a Silverman Survey that x-rayed his chest, hips, knees, spine, and etc. to look for other bone abnormalities, they will be using these to help diagnose his syndrome correctly and try to pick up on any other problems that may have been missed in the past. Results next week.
* Finally he went for an DXA scan of his hip to show bone density. The bones in his right rib cage are not very hard and they want to see if this is an overlying problem or just in that area and will also help with diagnosis. Results…..next week! On realizing this was the final test, Michael about ran out of the hospital and had no patience for us moving slowly–he was done!
The plan is that all this information will be compiled over the coming days, all the doctors will meet together on Wednesday to discuss results, options, plan of attack, next step, etc. Amy, a very wonderful and helpful nurse, will be in on it and will call me on Thursday to go over it all. As I said, they are all on the same page that some serious intervention is going to be necessary in a relatively short time span, but they do not want to rush into something either and will be consulting with spine and orthopedic doctors.
We got to the hospital this morning and went through all the pre-op things and then the main anesthesiologist had some serious concerns about putting Michael under due to his very compromised lung function (low twenties in percentage). We were told this might be the case and had her call the lung specialist who convinced her the necessity of the testing and that no matter when we did this we would face the same concerns and they went ahead with the procedure.
* The first doctor in was the lung specialist who used a very thin, flexible probe to investigate the lungs. We were hoping to find that the airway collapse was short in length and floppy in “consistency” so that maybe placing a stint (a tube) would prop it open and give access to the right lung again. Unfortunately the airway was compressed VERY tight and for the full length of the airway as far as they could get–he had difficulty even shoving the probe through and we are talking a very small tube. The issues with this are multiple because not only does it mean that little air is getting in–but the lung secretions that a lung constantly makes can’t get breathed/coughed up so that his lung is full of mucus that can’t get out until it gets full and then sort of oozes out the top where he can cough that top bit out. They did find that the lower right lobe that is collapsed is at least still getting blood supply so that it isn’t so damaged that recovery isn’t completely impossible.
* The second doctor went through with a larger, rigid scope to get a good look at the trachea and airways and confirmed what the smaller probe showed (and also showed he has very large tonsils that could pose a minor problem)…the trachea starts out round and as it comes up to where the airway splits off it gets more oval as if even the trachea is being pressed on. The airway to the left lung shows some signs of being compressed as well, but it is open enough to function fully, thankfully. The right airway is visibly squeezed shut, not just collapsed. As his spine twists and rotates away, it is twisting the right airway around it, stretching it and flattening it, kinking the whole length of it flat. Obviously none of this is what “best case scenario” looked like and is discouraging to say the least–we are, though, glad for the tremendous information this gives to doctors to try and formulate a plan for treating Michael to the best of their ability.
* The third doctor was looking at the esophagus, pancreas secretions, and stomach–we’ll have the results of the pancreas testing back in around 10 days. The primary reason for putting a probe into the stomach was to try and clamp his old feeding tube hole shut and stop the leaking. The area surrounding the hole was very tightly stuck to the above skin layer which made it very difficult to clamp–he used a bunch of clamps and we’re hoping for the best, he can’t guarantee that it will work and surgical closure would be next. While in there he found lesions on the stomach that he couldn’t identify as to what they were–he has biopsied them and we’ll have to see what turns up although he seemed to anticipate some type of treatment being needed for whatever that was. He also took blood for tests to try and confirm an alternate diagnosis of Schwachman-Diamond syndrome, that will take 3-4 weeks to complete.
Obviously, the doctors stressed what a very complicated case Michael is given the severe abnormalities we found in his rib cage, the severe twisting of his spine, his very poor lung function, and his immune system compromise. Something needs to be done quickly but not so quickly that they take the wrong road–they are going to compile all the information gathered from this week and have a “round table” discussion with all the doctors next Weds and try and see where to go next. The immediate concern is to improve his health to the fullest potential so that whether a spinal surgery, a rib cage reconstruction or some combination of the two are done he has every possible advantage to going through them well. There are also other concerns that need to be addressed, the first of which is issues with how he is breathing when he is sleeping and whether he is breathing so shallowly that he isn’t getting enough oxygen, but even more that he is getting rid of enough carbon dioxide which they don’t think he is. This affects a lot of areas of health in him and a sleep study will likely be the first additional testing that is done with the possibility of putting him on one of two machines that essentially forces his breathing to be deeper. This will help his current health condition and prepare him for being on it after whatever major surgery is coming. Anyway, Amy, our liaison of sorts, will be calling me next Thursday to discuss all the various results and what was discussed and etc.
Tomorrow he has a video swallow study to see if he is aspirating food/liquid into his lungs due to poor functioning, a bone density scan to get a good picture of his bone strength as the bones on his right rib cage show signs of not being very solid, and x-rays of his limbs and other places than his rib cage to get a full picture of his skeletal issues.
This morning Michael had a CT scan with contrast (dye injected) and then the data was processed into a 3D resolution of his body and organs. This afternoon we met with the three groups of Dr’s who will be doing different kinds of scoping tomorrow afternoon (1pm).
* The first doctor we saw is a gastrointestinal doctor of some sort (Dr. Putnam)–he will be scoping to see how his esophagus looks, going down into his stomach and putting metal clamps onto the hole in his stomach left from his feeding tube. We are hoping this will be sufficient to close it up and stop the leaking–the clamps will fall out naturally in 5 weeks. He will also be stimulating Michael’s pancreas to do enzyme testing–there is some question as to whether his pancreas works correctly and they believe he may not be digesting his food properly, flushing the food out before he can absorb nutrients–which has caused his long battle to gain and keep weight on. He has said that he will place large sums of money that Michael does not, in fact, have Jeune’s but another syndrome (Shwachman-Diamond Syndrome) that would account for his white cell problems, weight issues, and rib cage deformities. The enzyme analysis will help determine that and we will have results in 10 days. He will also place a tube down his nose with a probe on the end that will stay in his nose over night at the hotel and take readings that will help determine if he has reflux.
* Dr. Rutter is an ENT and he will be doing a rigid scope that will be focused on seeing the extent of the issue with the collapsed airway. He is apparently “the” doctor to see for these airway issues and we are lucky to have him seeing what there is to see. He’ll also be taking biopsies and samples to check for infections and bacteria that have been missed.
* Dr. Boesch is a pulmonologist and will be doing a flexible scope that will be going deeper into the lungs to see the condition of the lungs and will be trying to get into the bottom right lobe that is completely non-functioning at now, collapsed–he wants to see if there is any possibility of salvaging it. He showed us the results of the scans and it was, to be honest, disturbing to say the least. I have seen many x-rays of Michael’s chest, but the 3D technology takes it to a new level and I have never seen anything like his right rib cage–nor had the doctor, I believe. I will scan the picture and send it once I’m home (for Craig, at the least, who will likely be interested to see it). The ribs are a mess of abnormalities and deformities slitting off into y’s at the end, a gaping area that just doesn’t really have anything, very strange. The spine is not just bent to the right but twisted in and twisting and pulling his shoulder blade out of place and putting pressure on the already collapsed right airways. As I said, the bottom right lobe is collapsed and useless, putting strain on the upper lobe which looks “off” and overinflated–tomorrow will give us a better picture of it’s actual condition. The left rib cage looks remarkably normal–he said there are small abnormalities and range of motion issues but really in good shape. The lung itself has obvious damage along the airways, probably from many pneumonias–we’ll know more about that tomorrow as well. It also has the “over inflated” look that the doctor finds curious and says is likely from having to compensate for the right lung for so long. There was more, about issues with the way he may be sleeping because of his shallow breathing being even more shallow and ramifications of that but I started to loose retention of information and he said that was issue for long term after we see what we see tomorrow.
Unfortunately all the lung function testing we did showed his functioning at a total of 22-24% without medication and 35% with medication–this was after a lot of workout so he said that the 25% could be higher than his actual functioning ability. It is possible that the anesthesiologist will not want to sedate him, but all the doctor’s agree that the scopings must be done and waiting a week or a month isn’t going to change anything so it is pretty certain that the procedure will take place tomorrow at 1pm.
We saw a geneticist in the morning to confirm the diagnosis of Jeune’s with the new group of doctor’s…it was confirmed (no big surprise). They are perplexed by the Neutropenia (where his bone marrow doesn’t work correctly and doesn’t produce white cells) and are contacting Cedar Sinai hospital where there is some research going on about Jeune’s to see if there is any new information about the syndrome. From there we went to see the anesthesiologist who will be putting Michael under for the procedure (they call it a “scope fest” as there are three doctors who will be scoping him in different ways and for different reasons). She wasn’t able to hear any breath sounds at all from the right lung (the side that the airways are collapsed on) and said he was working on one lung alone. This isn’t surprising but we weren’t sure if he was getting some use out of it or not, apparently not–although we’ll have that confirmed with results from tests done this afternoon and tomorrow (Tuesday) morning. That makes some concerns for putting him under, but she said that if his other lung is working pretty fully that she thinks it could go fine–there is some possiblity that he wouldn’t come right off the vent and have to keep it in over night but from what she could hear of the left, she felt that there was a good chance that wouldn’t happen.
Then he went to get a lung function test that was an hour and a half of exhaustion for him, I wanted to lay on the floor and I have two good lungs and was just holding my breath and blowing along with him! He’s had pulmonary function tests before, but what he had was the very beginning of this version–poor guy was wiped out. We’ll find out the results tomorrow afternoon when we have consultations with the main three doctors.
Tomorrow morning EARLY he has some chest scans and a 3d reconstruction of of his lungs which will (combined with the pulmonary function tests) give us a very clear picture of where things stand for his lungs and whether or not for sure they will go ahead with putting him under on Wed.
