Michael has several appointments leading up to his surgery on July 23rd: Michael is currently being prepared for surgery in every way possible, medicated, vitamined, IV nutrition, and etc. There is a concerted effort on all doctors parts to do everything they can to prepare him and we appreciate it greatly! We are down to appointments on July 15th and 16th for some last minute checks, his hematologist wants a last check on his status, his GI wants a last check on his nutritional status, and Dr. B (lung) wants to do a 6 minute walk test to qualify him for portable oxygen. The next week is surgery.
We went back to Cincinnati on Tuesday for a pulmonology visit to evaluate his lung function. Michael really struggled to get through the pulmonary function test until he was frustrated and crying and feeling like he was failing–it was obviously worse that the last one in September. The respiratory guy was very kind and gave him a minute and went and talked to Dr. B who came in and talked to Michael–love Dr. B. He talked to him for awhile and said it was up to him if he kept going or not but that it wasn’t meant to be so difficult for him and that he shouldn’t stress about it. Michael said he’d give it another go and he did and they did some milder versions, in the end they weren’t able to get any concrete numbers other than to show he is significantly worse than in September.
He was very concerned about the coming surgery given what he saw of Michael’s lung function. He was trying to phrase his word’s carefully so I had my mom take Michael out into the waiting room and he sat and talked with me for over an hour. He asked me if Dr. C had told us the risks to the surgery and I said we were made to understand that Michael was complicated and that it was going to be a difficult surgery and that there were high stakes, but nothing bluntly stated. I cannot tell you how calm, patient, and compassionate he was talking to me, giving me the blunt honesty but with utter graciousness. It was the hardest conversation I’ve had to date. He said that his concerns were not so much with the surgery itself, but with the days and weeks after it and that he wouldn’t really recommend the surgery being done without Michael being trached first. His reasons were multiple, but boiled downed to serious risks of pneumonia and lung complication without out–with it he will still have serious risks but without it he feels those risks double. Pain management–he feels he will likely be repeatedly intubated (tube down the throat, sedated) and then attempted to pull out, then pain meds make him breathe shallower and they will have to repeat, and etc. Michael will also be under less stress and more comfortable with a trach than with repeated intubation. We all agree that while a trach was the last thing we expected, that the reasoning is sound and worth doing.
The initial thinking was to go in the next week or so and have the trach placed, stay in the hospital 5 days for healing and training. Orthopedic surgeons are routinely concerned about things like trachs/feeding tubes, etc. where bacteria can colonize around the opening and then when they open up the back, there is a risk of the bacteria migrating to the back and settling around the rods. Dr. B (lung) talked to Dr. C (ortho) yesterday as well as Dr. R (ENT) and they all decided that a compromise was to complete the surgery with regular intubation and venting (something they’d do even if he had a trach) and then once his back was closed up they would put the trach in and take the breathing tube out and use the trach.
We had literally just sat down with Michael yesterday afternoon to explain what the doctor’s wanted to do when Dr. B called. Can I just say how impressed I am with him that he called me himself and not his coordinator? Anyway, he explained the above and I was very relieved that they would be doing it during surgery as he’ll be pretty drugged up for awhile anyway and it can heal up while he’s loopy and less stressed about it. He’ll most likely come home with the trach capped off until Dr. B feels he is completely out of the woods of needing it. Michael was, understandably, stressed and upset about this, it is very scary and in many ways he (and all of us) are at the end of our ropes in terms of stress and dealing with yet another thing. He does understand why they need to do it, though, and agreed that it was something we had to deal with and that he was glad he would be pretty loopy for awhile after the surgery.
We are all stretched thin emotionally, we keep thinking we can’t deal with one more thing and then, as I told my friend, “one more thing” walks up and slaps us in the face. I know that we are all strong, but we feel like glass that has crackled and only needs a tap to shatter. We won’t, but it is exhausting trying to hold it all together. We keep moving forward though, as that is the only thing we can do. I am so proud of my children, they are both strong and beautiful human beings and as I have told them since they were born–had I the chance to pick them out of all the millions of children in the world–I would have immediately picked each one of them.
What’s Next? We are heading down to Cincinnati on the 15th and 16th for some last minute checks, his hematologist wants a last check on his status, his GI wants a last check on his nutritional status, and Dr. B (lung) wants to do a 6 minute walk test to qualify him for portable oxygen. They’ll be doing a sleep oxygen test here at home to determine if he needs it during the night–he suspects that that status has changed from the fall and that he will need it.
The week after that is surgery week.
We are back home from Cincinnati (although we had to get up early and head to Toledo Hospital today for IVIG treatment from 8:30am through usually 3:30pm) and will be home until next Tuesday when we are heading back for one night. Anyway, GOOD NEWS first, and yes, we do have some good news, Michael gained SEVEN POUNDS in two weeks…let me repeat that just because we don’t get a great deal of good news, SEVEN POUNDS in two weeks. He looks really good and I cannot tell you how it makes me feel to rub his back and feel padding instead of bone. His GI is very happy with his weight and his nutritional numbers that show he is using the nutrition well although we have to add iron pills to his accumulating pile of pill (which he hates). Okay, just once more, SEVEN POUNDS. So, at 19, Michael now is 77.4 pounds and is the heaviest he has ever been in his life. They are hoping for another 7 pounds before surgery to give him a buffer.
We did talk a bit about long term goals in terms of what happens after surgery and he said we needed to keep in mind that the surgery is not going to change anything about his nutritional status and issues and that we are looking at TPN as a long term treatment. His PICC will have to be changed at some point and then they will decide whether to sew in a more permanent central line to the chest. He did say that once he is fully healed from surgery (months) and his nutrition is stable then maybe we could think about doing it 3 days a week instead of every day but that we should consider TPN a long term part of his care. I am glad to know that they won’t just pull it after surgery as I know he will need all the nutritional help he can get to fully heal, but I had mentally thought of it as a month before, month or two after. Still, when I see the difference that just two weeks of nutrition that his body doesn’t have to work so hard to process makes–I can certainly see its importance and we will adjust. We have some….frustration (which might be a mild word) thinking of how his health status would have been so much improved by either continued g-tube or TPN supplementation years ago. I am beginning to understand now why this GI has been almost outraged that he was misdiagnosed for so long or at least the nutritional aspect not questioned and his pancreas tested. Still, we are trying hard to just move forward as it really doesn’t serve any purpose to look back!
SO, moving forward, we have pulmonology in Cincinnati next Tuesday with (likely) pulmonary function tests (we are cringing to see these results) then we have the next week free so far. The week third week we’ll be back to see Dr. H (hem) and Dr. P (GI) for last stop before surgery. The fourth week is surgery week and we don’t have all that worked out yet, it is likely now that he will be admitted a day or so before the 23rd for platelet transfusion/s as his platelets are much too low to go into surgery with. It feels like time is going to fly by and I’m torn between wanting it to SLOW DOWN and wanting to just be done.
Did I mention that he’s gained SEVEN POUNDS?
Our trips are rapidly winding down to the surgery date, a little too rapidly for our comfort in many ways, but we are winding down on pre-surgery appointments (until/when they add more). This week was important as we are seeing both the surgeon and anesthesia and doing pre-testing such as EKG, blood work, and fresh x-rays.
Today, Tuesday, we went in for the EKG (results in a week), blood work (par for the course for him), and new spine x-rays. The x-rays (scans to come when I get home, this camera picture will have to suffice until then) showed an increase in the twist and the curve since February, not too surprising. Unfortunately, some bending and stretching x-rays show the curve as being pretty inflexible and Dr. C. doesn’t feel we’ll be able to straighten him out too much and that the goal of the surgery would now be to stabilize and stop the curving. Obviously they won’t know exactly what they are dealing with until they are in and working on him, but we can’t really expect him to come out with a straight spine. This is discouraging not just because of the straightness factor, but also discouraging for the hope that if we could untwist and straighten the spine significantly that it might have a positive impact on his respiratory problems. Also because of the likely inflexible curve, they will probably be clipping his ribs on the right to allow some give and adjustment of the hump on his back that will allow bone to regrow smoother. Because of this and bone density issues he’ll likely wear a brace (not really a brace as he can’t be restricted, but more of a form that will just help the bone heal in place) for 6 weeks after and then for less hours for another 6 weeks or so.
Regardless, we feel confident that Dr. C will do everything in his power to optimize him to the fullest and to do everything they can do to safely get him through the spine fusion. We went ove a lot of the “what to expect” in terms of time (7 hours are booked), pre-care, what to expect after surgery: he will go directly to ICU because of his various “complications” and likely stay there a bit longer than most, he will have two chest tubes placed to help prevent lung collapse which will stay in “as long as they need to”, a pain management team will start with us at the beginning of the day before surgery and work with us throughout the healing process to keep him comfortable. Long term follow up will be 6 weeks, 3 mo, 6 mo, 1 year, and then yearly for 5 years.
Again, I know some of this isn’t of interest to everyone, it’s just a way for me to keep information clear and have it available for those who do 
We’ve all been cycling through a lot of stress and anxiety and coming up with ways to deal with it all better–we are lucky to have each other to lean on along with our families and friends and all the people around us who care about the amazing young man that is our son, brother, grandson, nephew, cousin, friend, etc.
Friday & Saturday: A nurse came in about 1:30 and asked if we needed anything, didn’t think much of it as they are the most helpful people here and are constantly asking us that! I said no, thank you, and she said, “Really, how about if I get your husband and daughter??” I think I looked like “um, what are you talking about” and then Katie and Mike walked in around the corner–surprise! We were so excited to see them!! We thought we were coming Tuesday and would be home probably Friday, Saturday at the latest but when we realized it was going to be dragging well into next week—needless to say I was thrilled to see them! Mike held down the fort at the hospital and spent time with Michael and sent me off to relax and spend time with Katie, it was a nice break! Saturday night we were able to get in at the Ronald McDonald house next to the hospital and we were really impressed with it. The people were helpful, the House is just gorgeous and huge, and the rooms were really comfortable and relaxing. Saturday Michael was able to be off his machine for 4 hours so we enjoyed lunch outside and he was glad to be free of it, today (Sunday) he was able to be off for 6 hours, and tomorrow, I think he will be able to be off 8 hours, and etc. until he is at 8 or 12 hours on (depending on which they decide).
Sunday: Not much happens on the weekends, but Mike and Katie left to go back home, which was sad, but we were so glad they surprised us with a visit! Not long after they left, Michael’s cousins came for a visit (with Laura & Ron) and stayed for a long visit–we had lunch outside again and then the boys played video games. It was great to see them and really made Michael’s day! Michael has already gained weight from the TPN/lipids and weighed in at 73 pounds today; 74 pounds is the most he’s ever weighed (when he was on appetite stimulants) and the circles are gone from under his eyes. He’s been coughing a bit more and both lungs sounded a bit gunky this morning and early afternoon, but they are just keeping an eye (or ear) on it for now–he’s already on antibiotics so hopefully that will keep anything from getting too messy. I’m hoping tomorrow we’ll have a more solid idea of when we’ll be home for sure.
Thursday was rather uneventful, his labwork from the morning testing his TPN related levels were fine, a couple levels were low but nothing that was out of the ordinary for him. They’ll being doing a full lab workup including some regarding bone health in the morning (Friday). We had a whole group of GI/nutrition related people come in the room, which Michael wasn’t too fond of as he said he felt like a monkey in a cage. The attending rattled off his history including the fact that there was no air sounds in his lower right, and very little in the upper, and very little movement in the airway itself. The head doctor quizzed the interns on how to spell Shwachman Diamond, one looked like a deer in the headlights and the other confidently said S.C.H.W….and was stopped and informed there was no initial C and that he expected all the paperwork to reflect that. They both looked at each other and said they had some changing to do. I remember all while Michael was growing up under the equally rare diagnosis of Jeune’s Syndrome that his head pulmonologist would always bring in an intern, command him/her to go over his symptoms and give him the correct diagnosis. Of course they couldn’t, and likely he wouldn’t have either had it not been told to him, and there was always a panicked look…or the over confident cocky look…in their eyes. Once he was done I always told them that very few doctors have ever heard of it and not to worry. It’s kind of ironic that all those panicked interns could actually give a sigh of relief as none of his doctors had gotten it right in the end!
Anyway, that was a rabbit trail! We saw endo who are doing some lab work to determine osteoporosis vs osteopenia (less severe) and then what they want to do about it. We also saw a nutritionist who is going to be working in the background following his oral eating in conjunction with the TPN. She sent up an Instant Breakfast shake which he declared much better than mine…I cannot win 
UPDATE, FRIDAY AM: I was pulled out in the hallway for rounds this morning (maybe someone over heard his “monkey in a cage” comments all morning as they didn’t crowd into his room today). They are doing some adjusting of the TPN to balance out his alk phos and sodium and are going up a bit more on lipids but he’s pretty well topped out at his protein and calories (I think) intake and tolerating it well. They are going to get renal panels for the next couple days to make sure that is evened out before we go home. It is much easier for them to make daily adjustments here, versus at home when we get a week’s supply so they want things set before releasing him. His CBC was normal for him, still the downward drop of the platelets, we’re at 42,000 now, his white count was at 3.1, anc at 2, lymphs still low. The earliest we are going to be leaving will be Monday now. Endo came in and said puberty issues are moving along fine for his body size and though his bone mass is low, it is not extremely so when you take into account his physical size matches his bone age–his vitamin D levels are low as well as calcium so their first thing is to get that up, and then they do see a call for him taking bisphosphourous (I know I am slaughtering that) to help increase his bone mass once his calcium and vit D levels are stabilized.
Michael was frustrated at having to stay but he’s doing better about it so we’re trying to just roll with it, he slept a lot better last night.
NOTE: Latest update is that we will be staying until around next Friday. Monday I’ll be trained in PICC line care, Tuesday refreshed and tested on PICC line care and some TPN & lipids and wed. through fri. on TPN & lipids. I’m hoping it will go quicker than that, but that is the latest word.
TUESDAY: We headed out for Cincinnati on Tuesday to meet up with my friend Kristin at the Garfield Suites (love this place–it’s old, smells a little bit, but it is spacious and has a full kitchen and a Skyline Chili in walking distance). Literally just as we got to the hotel, I felt something snap under my feet in the van and it felt like the power steering was going. I finished pulling into the garage and got up the first ramp and it was gone–I *had* to get out of the way as it was right as the garage was emptying out. So started a horrid process of tugging and pulling and backing up and inching forward and hanging off the steering wheel, and–yes, Michael–I’ll admit to some yelling and swearing. Car after car after car moved around me when I got it at least enough so that people could get out but not one person stopped. The first guy who was held up for quite a while while I tried to maneuver was in a truck and he literally sat and watched me for 5 minutes, didn’t offer to help, anything. I stepped out towards the truck finally to explain my power steering was gone and see if maybe he could turn it better than I and he saw he could get around and off he went. That was disappointing. I was amazed at how many people, men and women, who edged around the van and not one single person asked if they could help, call someone, offer a “sorry, bad situation”–nothing. Humanity redeemed itself when my husband (and hero, even all the way from Temperance) called the hotel and had them send three men out to help and then he called a tow service to come and get it. These guys were fabulous, they had to work their butts off to get me out of the predicament and backed into a parking space–yanking the wheel in place and having me hold it, then pushing back and forward and repeat over and over until they were just dripping with sweat. They had great attitudes, they were encouraging to me who was frazzled by that point, and stopped me later that night in the lobby to make sure things were taken care of in terms of getting it fixed. The man who came and picked the van up was one who had helped us before and he called the hotel later to let me know he had it, he was very sweet and said he had thought of Michael a lot and that he hoped that everything went well at the hospital and not to worry about the car. It’s fixed now (cheap and easy fix) and they are going to bring it to the hospital for me. Luckily my friend Kristin was driving up from Tennessee for moral support so we had a ride to the hospital on Wednesday and I had someone to laugh about it with. So to all those multitudes that Michael was texting saying I was going crazy and swearing, LOL, I make no apologies, I was throwing everything at that steering wheel to get it to turn. Michael gets very, very stressed under situations like that, but we had a good long talk later in the hotel about the fact that it is okay to flip out a bit and get upset, then you shake it off, laugh at the situation, and move on. We ended up having a nice evening watching Ghostbusters I & II (25 year anniversary), enjoying a great thunderstorm, and spending time with Kristin.
WEDNESDAY: The PICC line went in very well. Once again I was extremely impressed with the team at Cincinnati, they truly went out of their way to make it a smooth, efficient, stress free experience for Michael. While they had one person there just to focus on keeping him relaxed, every one of them was kind, courteous, and gracious. Michael said there were hard pinching feelings and pressure, but that it was so much better than he expected. Kristin had brought a movie for him and they popped that in a portable DVD player and it was great help in having him focus on something else and keep his arm relaxed. Anyway, very impressed, they were a fantastic team and I said so on the “rate your experience” survey.
The rest of the day was mostly waiting as they figured out what they wanted to do in terms of the TPN (the nutrition support). We hit a large snafu when the insurance said they wouldn’t pay for home TPN…which is a little funny given that they pre-approved inserting the PICC line for the purpose of home TPN, that makes a lot of sense. I was stressing about that but one of the GIs came in and said they were “siccing our bureaucrats on their bureaucrats” and would take care of it, so we shall see. They started him on 24 hours of TPN with blood work taken this morning (Thursday) to see what adjustments need to be done–other than going to the bathroom every 2 hours like clockwork, no problems. No one will give me any time frame for being here, the GI we have seen the most just said that Michael was complicated (no surprise there) and they will see–a “few days” is the best I’ve gotten. It was really fantastic having Kristin here, she went above and beyond the call of friendship duty running around (she even ran to where the van was to pick up a bunch of misc. things I forgot to grab before it was towed) and we were sad to see her go last night. So, a day at a time, we’ll see what Thursday brings.
We did (are finishing) Michael’s fourth dose of IVIG today at the hospital and so far so good. He hasn’t gotten a headache yet like he did last dose. They did, however, redo his labs as the ones in Cincinnati were so low and he’s had three doses of the higher GCSF shots. Good news, his white count went up to 4.9 and his anc to 3.9 (absolute neutrophils are the part of the white cells that are really doing the work and that we watch the closest), his platelets were worse, but that seems par for the course the last few months. His lymphocytes are the lowest they have been since I started tracking it all in September, they are the brains of the white blood cell line and not affected by the shots he gets. Still, all in all we were hoping for a better ANC and at least the higher dose worked! Not so good news, he lost 1.5 pounds from last month–not a lot for most people, for him, no good. I’m glad we’re getting the IV nutrition started next week.
We had thought we would be zipping back this week but the coordinator called yesterday and the earliest she could get him scheduled was next Wednesday, so we’ll be heading up Tuesday to spend the night and head in early that morning if I can’t shift it later in the day and just drive right in…or I’ll hit the road at 3am, but that doesn’t sound like a good idea as it’ll just be him and I.
We met with Dr. Harris, Michael’s hematologist in Cincinnati and also a specialist on SDS. He is starting him on voriconazole (an anti fungal medication) and azithromycin (antibiotic) two months before and two months after the surgery, we are continuing the IVIG, and we will probably be going to daily GCSF shots a few days before and after the surgery as well. They are doing everything they can to bolster his mess of an immune system. They took labs and while we were on our way home Theresa (Dr. H’s incredible coordinator) called with bad news on his counts, she hasn’t emailed them yet so I don’t have the full workup, but his white count was only 1.2 and his ANC (absolute neutrophil count) was only 220…220! And this on on GCSF every other day, he has never been that low on GCSF shots. I remember once he was at 150 but that was off GCSF. Anyway, that is very discouraging, she is consulting with Dr. Harris to see what he wants to do.
So, that wraps up this trip to Cincinnati and despite having to have the PICC line placed and the horrid counts which are giving me a stomach ache thinking about–I am, as ever, grateful to the doctors in Cincinnati who are being so thorough and careful to try and think of every way they can to give him any edge possible going into this. We truly are aware of how lucky we are to be working with them and that we have them in Michael’s corner.
Coming up we will be heading back to Cincinnati in the next week to have the PICC line placed, start the IV nutrition through the night, and stay a couple days to keep an eye on things and get trained in care. Next month we have a slew of appointments to start the next phase of pre-op things.
Today was a quick day, we had an appointment with gastroentronology, Dr. Putnum who was the first doctor back in September who thought Michael was misdiagnosed with Jeune’s and really had Shwachman Diamond Syndrome. I pointed that out to him today, that he was the one who started it all, and he said it was quite obvious and started to go off about the misdiagnosis and stopped himself. We saw him today as all of the doctor’s involved are concerned with Michael’s nutritional health and weight and he said that as things stand he doesn’t have the physical reserves to do all the tissue healing that will be necessary after the surgery. We had three choices: a) have a feeding tube placed down his nose, past his stomach and etc. into his large intestines–it couldn’t go into his stomach as risk of reflux compromising his already compromised airway was too high; b) surgically have a feeding tube placed into his side, again, bypassing the stomach and going down into the large intestine; or c) have a PICC line placed into his arm for IV nutritional support. We are going with the PICC line for a number of reasons: they want to do IV antibiotics for at least a month before surgery and they can use the PICC line for that, they can do his IVIG treatments through it rather than starting an IV every month, they can use it for an IV line at the hospital surgically, and it can remain in for months–which Dr. P felt was a good idea as he doesn’t feel that his nutritional/antibiotic issues are going to be short term after surgery.
So we are going to come back to Cincinnati next week sometime to have the PICC line placed and be in the hospital for a couple days to get the nutrition started and train me on using/caring for it. Michael seems okay with it, a little worried about it going in, but otherwise at the moment he is just glad he isn’t going to have a tube down his nose for 6 weeks (which he was dreading). I have mixed feelings about it (surprise surprise) just in the fact that he needs to have a line placed like that drives home all his extra issues leading into the surgery. But for the most part I am just glad that there are things they can do that will give him ANY edge up to get through the surgery/post surgery successfully.
Tomorrow we see Dr. Harris to discuss plans in regards to his immune/bone marrow issues going into surgery.
On a fun note, we went to the zoo afterwards with Amanda and Jayden (Jayden has SDS as well) and had a really lovely time with them. The kids said our zoo (Toledo Zoo) was better but that it was fun to go somewhere different. Although I have to say that their train ride is pretty cool and you can’t beat the white tigers and manatees! A couple pictures from before my camera died are on Flickr and Facebook.
[I know as a writer that there is a fine line between honest speaking and melodrama and that the following can probably be read in either way--but the truth is, I think that sometimes we do the world a disservice in not speaking honestly, that there is something not quite healthy about our societal pressure to say "I'm fine, how are you" whenever we are ritually asked, "How are you today?" Sometimes we aren't fine and that is okay.]
While Cincinnati has been life changing for us in terms of finding out the information we needed to know, proactive in Michael’s care as a complete and complicated individual–to Michael, it is the worst place on earth and we’re heading back there tomorrow. Last night we were talking about it and getting ready for his shot and he said he wasn’t sure any more that he wanted to have the surgery, that maybe his back wasn’t that bad. I had to explain to him that the surgery really wasn’t a choice because if we do nothing, he will, at the very least, end up completely bent over. It was a difficult conversation and, I think, one of many to come over the next two months as we kick into gear getting ready for July 23rd. This week’s appointments have driven it home that it’s moving into “game time” and we’re all feeling the stress. My stomach is a constant ball of knots and sleep is starting, again, to come only at the point of exhaustion with music or a book playing loudly through my iPod so I can’t hear my heart and my brain saying over and over like I was three “I don’t want to do this, I don’t want to do this, I DON’T want to do this.”
I am thankful that we have this option, that we have people willing to do this surgery, but I don’t want to do this. It is terrifying, all the possibilities and the “what ifs” and the doctors “concerns” and I am glad they are thinking over every avenue and I am glad they are covering every base but every time I hear “there are concerns” or “this doctor has concerns” or “he is a complicated case and there are concerns” my stomach gets tighter and I wonder how I will breathe through 8-10 hours of surgery.
So, that’s the unsugarcoated (most definitely not a word) truth. We don’t want to do this, we are tired and scared and time has shrunk again and two months sounds incredibly close.
When Michael was a baby I played the role of superwoman very well, I had it all together, I was strong, doctor’s were impressed, etc. ad nauseum. The truth is, I’m older and I’m wiser and while my first instinct is still to play the superwoman and say “oh, everything is fine, we’re holding together, one day at a time, it will all work out, let’s go plant a tree, and how is your day???”–life is messy. It is glorious and amazing–but it is messy, and that is okay. So, tomorrow I may actually be superwoman for a day or even two or six, but maybe not–regardless, today, I’m not, today I’m a tired and scared mom who only wants one thing, what we all want for our children, that they be happy and healthy and I am sad that the road to not even healthy but ”maybe more healthy” for my son is one that includes this painful experience.
