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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.
