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We had our hopes raised and then dashed today 
and for an hour or two we thought we would be heading home today. Michael had a rough night last night with coughing, a splurt of hives and a dash of mild fever, and being up all night going to the bathroom from the TPN. This morning his hem (blood doctor) came in and said that nothing had grown and they believed the first culture must have been contaminated and that she was comfortable with him going home. I talked to the nurse as I felt uneasy as *something* was obviously up for him to be doing any kind of fever and his coughing was really awful although everyone agreed it was keeping stuff up and out of his lungs. We hadn’t met with his pulmonologist yesterday so she called and he stopped over to see Michael. He said his lungs sounded good for him and, like the hem, was comfortable sending him home after they did a different culture way down his nose into his throat and that he would call something in tomorrow if any infection was running around there. I still felt uneasy about it but I understand it is important for him to *not* be in the hospital if at all possible so as not to catch something there, so I told Michael that it looked like he would be going home. He was half asleep and said he didn’t want to go home (a first time in 19 years), shocked, I asked him why, and he said he was too sick to go home and went back to sleep. Ten minutes later, his hem Dr. zipped in and said, “Um, sorry, but you aren’t going anywhere.” Apparently Michael knows his own body, the culture from yesterday (which was *after* he was nailed with some heavy hitting antibiotics) had grown more than one type of germ/bacteria/infection whatever you will.
So they did a culture down his nose and into his throat for his lung doctor, we’ll see what that shows tomorrow on that end. The plan is to hit him again with the “big gun” antibiotic that they hit him with Monday and changed up the secondary antibiotic to one they hope is more effective killing the bacteria. As I understand it, the goal is to hit hard and knock it out of his system before it can overwhelm his system and put his body in shock (sepsis). His nurse said not to expect to go home until sometime next week, so we’ll just take it a day at a time.
Hopefully he gets more sleep tonight since it isn’t a TPN night (which makes him go to the bathroom all night), we could use a good night sleep!
Yes, today was a very very crappy day indeed, and exhausting and I’m joining Michael in hating Cincinnati, at least for the day (yes, I love Cincinnati Children’s and appreciate everything they are doing, just, for today, I hate Cincinnati).
Our first visit was to Infectious Disease who are going to work with the doctor’s to make sure Michael is well covered in the right antibiotics to keep him healthy and infection free after the surgery. They gave us an antibacterial mouth wash to use all week leading up to the surgery, some kind of antibacterial nose solution to help staph not colonize in the nose, and some more antibacterial wash to use 5 days before. They also recommended two antibiotics to give by IV just before the surgery itself. We told them our issues with Michael’s poor arm, he really has no solid skin left on his arm due to an allergy to tegaderm that we never knew he had and so they called in the Vascular skin/PICC unit to see what they could do. Unfortunately a combination of the tubing being out too far and the condition of his skin made it so they had to decide to pull the PICC line and reinsert it in the other arm tomorrow. As much as Michael didn’t want to and as much as I wanted to avoid that for him, I completely agreed with the decision as that arm just cannot even hold a patch long because his skin just falls off and with it the patch. It made me sick to even think of sticking something else on it! I explained how we found on brand that didn’t bother his skin as much (Sorbaview) but that we could not get it around us anywhere. They had some! They are in the process of giving it a trial run.
Anyway, they pulled the PICC out (luckily it was painless, just not pulling up the dressing and cleaning his poor skin) and we headed over to see Dr. P (GI), who they had consulted on pulling out the line. He said we definitely did not want to leave it out until surgery as a) we want to maximize him to his fullest and not loose anything and b) they can use the line for pre surgery uses as well. He was pleased with his weight gain (he’s at 80lbs 12oz up from a around 14 pounds in February according to Dr. H’s!!!!) SO, the plan is to put the PICC in his other arm, use a less abrasive cleanser, and use the Sorbaview dressings and hope we don’t get into the same situation.
Next stop was Dr. H, the hematologist that specializes in Shwachman Diamond Syndrome. They drew pre-GCSF shot labs to get an idea how his white count (among other things) looked off the shot and then gave him his shot as I hadn’t brought it thinking we would be home tonight. Theresa (nurse coord) called and said his white count was 2.7 which they were happy with as it will go up with the shot. Hims renal/liver panel looked normal as well as his hemoglobin was 12.4 which they are satisfied with, but his platelets have dropped even lower to his all time low of 32,000. Blech, so, the plan is to admit him on the 22nd so they can do platelet and plasma transfusions and get him up to an acceptable range before the surgery on the 23rd.
Last stop was for an echocardiogram, results of which we do not have yet.
On the way out I was called by Dr. B (lung)’s nurse coord who said that oxygen has been prescribed for Michael for both night time and activity. I wasn’t surprised by this given the results of his walk test, he simply can’t be dropping that low and forcing his heart rate up that high (77 O2 and 169 heart rate). I hadn’t heard the results of his overnight O2/HR but obviously he was dropping at night as he qualified for oxygen. She’s going to have Dr. B call to go over the results in more detail [SEE NOTE BELOW]. While, again, we are glad that we have this information so that he isn’t continuing to struggle, it is a very difficult step to go back on oxygen after all these years–I feel like we have come back full circle to his first three years and it is discouraging.
Needless to say, Michael’s spirits are very low right now, he is tired, he is frustrated, he feels like his body is letting him down every where he turns and it is painful to watch not only his physical problems, but his spirits bowing under the weight of all of this.
Tomorrow we have the PICC line placement and some special x-rays of his spine.
UPDATE: I just spoke with Dr. B (lung) [I might hate Cincinnati today, but I still love Dr. B] on the phone (7pm) and his overnight O2 was fine, well, maybe not normal, but good enough that he doesn’t need to have oxygen at night. His walk test def. showed a problem getting oxygen with even mild activity so he will have a portable oxygen tank to use with any activity (even walking in the house). The nuclear scan showed pretty much what we expected, no air flow in the lower and middle right (didn’t know about the middle) with only the top portion getting air flow. His top right accounts for 20% of his lung function, which means that his left lung is having to make up 80% of the work. His left lung shows patchy airflow, which other scans have shown and this confirms, so it has some issues as well. They can’t say exactly why without doing biopsies, which they are not willing to do, they don’t want to mess with that lung at all. His echocardiogram was fine, he’s never had an issue with his heart, but it was still good to see.
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?
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.
Monday: The surgery went very well and very fast. The followed the tube down, stitched the stomach closed, then cut the tube away and pulled it out, then closed the layers up to the skin. He spent the night and has had some pain, but the Tylenol with Codeine works pretty well. He was most irritated with not being to eat until Tuesday.
Tuesday: The hospital wanted to make sure he was eating fine, breakfast and lunch, which worked out well as then we could just go downstairs to the orthopedic appointment. They re-did all his spinal x-rays and a couple we hadn’t had done before, including a traction one that was a bit painful on his stomach and put his pain up there pretty good. We dosed him with some more Tylenol+ and by the time we got back to the hotel his pain level was down. Unfortunately, the orthopedic consultation was very difficult and he seemed to have been listening well. His spine has curved significantly since September and he has moved “past the point of no return” (as the song from Phantom of the Opera kept going around in my head). Whether they can do anything to fix the airway or not becomes, while not irrelevant, no longer the deciding factor. The doctor said that at the conference the biggest issue was trying to figure out where the pressure was coming from that is pinching the airway and how they could relieve it. There doesn’t seem to be a good way to separate the spine and the front, but they are going to try to tweak things as they go in the surgery and hope that it will have an impact on his airway.
The surgery will be in early summer, most likely, I should hear more in a week or so–it sounds like June or early July. There are, of course, a lot of concerns and issues to be dealt with…consults with pulmologists and anesthesiologist to try and come up with the best way to get him safely through the surgery process despite his serious lung issues which will be about 8 hours long. His nutritional (too skinny) factor is a problem as well and 6 weeks before the surgery (give or take) he will have a feeding tube placed down his nose, past his stomach, and directly into his intestines for maximum nutrition absorption. We’ll do feedings through that to pump him up and bulk him out, it will also be used after the surgery when he will not feel like eating due to drugs and pain issues. We’re to start iron and vitamin C build up now and work to a certain level by surgery. We’ll be seeing Dr. Harris tomorrow who will help us make a game plan from the immune system end of things which will probably include the IVIG infusions before and after surgery.
Seeing his September scans next to his scans from today was heartbreaking, along with thinking about the surgery and recovery ahead. Michael was terrified, his words, and very overwhelmed, as we all were. We all had a good cry before we could leave and the nurse was very compassionate.
I’m not sure what we expected from today, I think I expected to hear that because it probably wouldn’t help the airway, there was no point in going through the spine surgery. There hadn’t been a big shift from Sept 2007 to Sept 2008 so we were not expecting to see such a dramatic shift. Although, seeing him without a shirt is becoming very painful for me, so I had some idea that things were not right.
Anyway, it was a difficult day for all of us and a difficult a difficult few months ahead as we do whatever we can to get him in the best health possible going into the surgery.
Tomorrow is an appointment with Dr. Harris and an attempt to get our van home. As some may have heard, right when we got off at our exit here our van’s transmission gave out on us. Luckily we were only a few miles from the hotel and they sent a shuttle to get us. We had the van towed to the hotel and have been able to use the hotel shuttle to get back and forth between the hotel and hospital. Mike’s friend can fix the transmission for more than half what it will cost here and thinks if he can get it on 75 he can get it home if he doesn’t stop. A transmission guy here agrees, so we rented a vehicle for me to drive my mom, kids, and myself and all our luggage home while Mike makes a run for it. Life is certainly never boring!
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!
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.
