brothers

brothers

Wednesday, February 16, 2011

Brochial lavage

Quick update...Todd reports that Ethan came through the procedure just fine.  He's in recovery and sleeping.  His O2 saturation is a little low, so they've got him on "blow-by" oxygen.  They'll likely be another couple of hours getting recovered and discharged.
~Sarah

Saturday, February 12, 2011

On Again -- Off Again

Well, it's been something of a topsy-turvy week here.  I'll try to catch you up.  What follows is a little confusing and fairly technical.  Those of you playing along at home might want to get a pen and a piece of paper so you can take notes or draw Venn diagrams or something. 


You may recall, that when last we left our hero, Ethan, Dr. McMillan (one of the general transplant docs in the rotation) had come by with the biopsy results and said that the granuloma was the result of thromboemboli; that it was non-infectious; and that she would recommend to Dr. Lund (the head of Ethan's team) that we proceed to transplant. 


Sarah and I e-mailed Dr. Lund late last week, and he said that he agreed that we might move to transplant, but he wanted a follow up CT scan to confirm that the granuloma weren't growing--which would be a sign of an active infection.  


Ethan had a sedated CT scan Wednesday morning.  That went really well, and Ethan did great. They just used a light IV sedation; they didn't have to intubate him.  The whole thing took less than a half an hour from the time they took him back to the time they brought me to be with him in recovery. 


Thursday morning, Ethan and I were over in the infusion center for his weekly enzyme treatment, and Dr. Lund came around.  Dr. Lund said that the CT looked pretty good.  He said there was one spot where the granuloma appeared to be 3mm in the original scan and was 6mm in the follow up scan, but that this was likely due to where the scan had bisected the granuloma.  Dr. Lund wanted us to meet with pulmonary to get their sign off on the CT scan, but expected that we would be admitted this coming Thursday, the 17th, to start chemotherapy. 


Late yesterday afternoon, we met with Dr. Laguna in pulmonary at which point she put the brakes on the whole thing. 


Here's where things start to get technical.and a bit confusing.


It turns out there are three sites in Ethan's lungs that they're looking at.  (This was news to me, I thought there were only two, but whatever.)  Dr. Laguna doesn't feel that the thromboemboli (negative infection) result is necessarily accurate, because (and here's the fun part) she's not sure that the site they biopsied was actually a granuloma. 


Okay, here's how it breaks down.  When Dr. Lund went over the original CT scan results, he highlighted two granulomas: a small nodule at the bottom of Ethan's right lung; and a larger, "lacy looking" granuloma along the base of Ethan's left lung. The original pulmonologist (not Dr. Laguna) and the surgeon had decided to take the biopsy sample from the base of Ethan's left lung because the right side granuloma was very small and would be difficult to locate and sample.


According to Dr. Laguna, it turns out that there's just one catch with that: the lacy looking granuloma in Ethan's left lung might not actually be a granuloma.  What she said was that when one takes a CT scan of a patient under general anesthesia (as Ethan was for the first scan), the machine is breathing for them and the lungs may not fully inflate.  When the lungs don't fully inflate, you sometimes get a result on the CT scan at the bottom of the lung that looks like the "lacy" area in Ethan's first CT scan.   So it doesn't matter that the biopsy came back negative, because they may well have been looking in the wrong spot. 


Okay, everybody got the first piece?  Lung biopsy may have been a big waste of time.


Here's the second piece.  It turns out that there's a third granuloma site that they're looking at, in the upper middle of Ethan's left lung. This granuloma appeared to be 3mm in the original scan and appeared to be 6mm in this week's scan.   When Dr. Lund and I talked about this in the infusion center on Thursday, he said that the growth might be a result of where the CT scan bisected the granuloma.  (I didn't understand that at the time, but talked through it with Dr. Laguna.  The way she explained it to me is that Ethan's CT scans were set to take an image every three millimeters along Ethan's chest.  They do these snap shots to limit the amount of radiation the patient is exposed to.)  What Dr. Lund was saying is that it's possible that the original CT scan bisected the granuloma mass at a narrow point and that this follow up scan simply bisected it at a larger point. 
Dr. Laguna didn't put a lot of stock in that.  Her take is that the granuloma is growing, and it's growing even though Ethan's been on an anti-fungal medication for two weeks now.  


Unfortunately, the granuloma that appears to be growing is in the upper middle of Ethan's left lung, so they can't go all the way in there to biopsy and get a sample. Instead, they're proposing a bronchial lavage. A lavage has been described to us as a fairly non-invasive procedure during which they'll sedate Ethan, then feed a tube up his airway and squirt a small amount of fluid in his lung and suction it back out.  When they capture the fluid coming back out, it will bring with it a sample of some of the infected cells which can then be cultured to determine the specific type of infection. 


The problem with the lavage is that it's kind of catch as catch can; they only capture infected cells about 30% of the time. That means it's possible that the granuloma could be infectious, but the lavage just doesn't capture and return any cells that can be cultured to determine the type of infection. 


After the lavage, Dr. Laguna also suggested that we change the anti-fungal medication that Ethan's been taking as a precaution. The doctors say that there's a higher probability that the granuloma are fungal (as opposed to bacterial), so it's hoped a broad spectrum anti-fungal medication will help kill it even if they don't know the specific type. Dr. Laguna said that if the granuloma are in fact fungal, then it doesn't appear that the current medication, Voriconazole (or vFend), is catching this particular strain.  She's recommended that we meet with the docs over in infectious diseases to get their thoughts on what types of infection this might be, and that we anticipate moving to a different anti-fungal after the lavage.  (They don't want to change before the lavage because they want to maximize the chances of getting a sample from the lavage in the hopes that they can get a positive identification of the infection.)


So, t looks like we're going to do the lavage, meet with infectious diseases, change the anti-fungal, then wait a couple of weeks, do another CT scan and see if the granuloma is smaller.


Okay, so that's all the technical stuff.  I hope everyone kept up with that.  


How do Sarah and I feel about all of this?  Well, fundamentally, everyone agrees that if there's an active infection when we go to transplant, it will kill him.  We're very happy that the pre-transplant workup was so thorough and that the processes here are set up so that each specialty department reviews the particular findings at each step and can catch these things.  So, we certainly want to take the time to deal with this.


Having said that, I am a little ticked that they didn't discuss with us the fact that the "lacy" granuloma in Ethan's left lung--the site that they biopsied--might have been due to lack of full inflation of the lung from the general anesthesia. If Ethan were a normal kid, I think I'd be fairly incensed that they moved ahead with a lung biopsy in light of this uncertainty about the site they'd chosen. However, we all recognize that Ethan's condition is very complex and that there's not a lot that's black and white with this kid, and that we're getting the best information and recommendations that we can and erring on the side of caution.  I hate to say it, but that may mean that Ethan's sometimes going to be a medical or surgical pin cushion as everyone tries to do the best they can. 


It seems to me that it's more important that we move ahead with caution but as quickly as possible and reasonable.   One of the main reasons that Ethan is in reasonably good shape today is that he's had mixed chimerisms from the first transplant, so that over the last year his body has been producing some of his missing enzyme and that has gotten into his brain to slow the neurological degeneration of this disease. If he had not had the benefit of that, it is almost certain that we would already see significant, irreversible neurological effects to the point where it wouldn't even be worth it to go to transplant at this point. 


His last chimerisms in late December showed 8% engraftment of the original transplant. Given the rate at which it has declined over the last year, I believe that by now his first transplant has completely failed.  I don't think that we will continue to see the same pace or level of neurological development until the second transplant engrafts.  This is not a fast spreading lukemia where it is critical that he go to transplant tomorrow irrespective of possible complications.  But every day that we delay does matter and does have an impact on him.  I want to take the time we need to take, but the process needs to move forward as quickly as possible.  Every day that we don't take positive steps to stabilize his condition is a small step backwards for him. 


All of this just came to light late Friday afternoon, so it was difficult to get things put on the schedule for next week.  As of now, he's scheduled to meet with someone from infectious diseases on Wednesday.  It's my hope that they can schedule and proceed with the lavage Monday or Tuesday and that we won't have to wait for the Wednesday eval before getting that done.


Outside of all of that, life at the Ronald McDonald House is moving along.  We are all enjoying spending time together.  Sarah and I agree that my quitting my job was a very positive step.  Mentally and physically, it's allowed us as parents to focus on the kids and our family.  We both feel a lot better not having to keep all of those plates spinning.  It's even allowed Sarah and I to find some time to exercise, something that we haven't been able to do in the 20 months since Ethan's diagnosis. 


A couple of weeks ago, we had a funny experience which showed us that it would have been very difficult for us to manage both jobs organizationally as well.  I had scheduled a phone meeting with the wonderful part-time coordinator that we'd brought in to work on my projects while I was on leave. Caleb was at school,  Ethan was taking his nap on the couch in the living room, and Sarah was on a phone conference in the bedroom.  The only quiet space that I could find for my phone conference was in the walk-in closet in our bedroom.  There I was, sitting on the floor in the closet with my phone and my laptop for half an hour taking care of some work stuff.  It pretty much drove home the point that it would have been very difficult for Sarah to work 40 hours per week and for me to try to work 20 hours per week when my leave finished while effectively caring for Ethan and Caleb.


Anyway, that's the latest from here. Thanks very much for checking in on us.  And as Sarah said last week, thanks a bunch for all of the wonderful posters and care packages.  They've made our room here lovely and cozy and homey.

Sunday, February 6, 2011

Biopsy Results`

The amazing harmonica duo:
Sarah and Caleb
(after a harmonica teacher
came to the house to
teach the kids)
 Hi everyone,


Sorry it's been a while since we updated.  I try not to bother you all unless we've got news to share, and, well, we haven't had any news.  For the purposes of this blog, if you haven't heard from us, just figure that no news is good news.


For those of you who didn't get the Facebook update, I should let you know that Ethan's recovery from the biopsy went pretty well.  He had some pain and discomfort Wednesday afternoon and night, while the chest tube was in, but the morphine did what it was supposed to do and allowed him to get some rest.  They took a chest x-ray the next morning and pulled the chest tube out about 9 a.m.  Ethan perked up immediately after the pulled the chest tube out, and five minutes later we were out of bed and walking around the ward.  Ethan had his ERT infusion inpatient that day, and we went home right after that.


We just received the biopsy results last Thursday when we were in for Ethan's infusion.  Dr. McMillan, the clinic doc this week, said the biopsy results don't show evidence of any type of infection.  The biopsy showed that the granuloma was the result of thromboemboli (or pulmonary thrombo embolisms), which, the doctor says, is a generally benign clot or clots possibly caused by his central line. McMillan went out of her way to say that these types of embolisms are generally not the type that can break off and kill the patient.  But why, and what the difference is, I can't say.)

Dr. McMillan characterized this as a positive result that most likely indicated that the granuloma were not the result of either bacterial or fungal infections.  She said that given this result it was possible that Ethan might start his chemo before completing the full four week vFend course, but that it would be up to Ethan's primary transplant doc, Dr. Lund. 

Dr. Lund reviewed the results and confirmed that the granuloma do not appear to be infections.  However, he's ordered a follow up CT scan for early next week to confirm that there hasn't been any growth in the granuloma.

If the CT scan doesn't show any growth, then they'll draw some additional labs to ensure Ethan hasn't picked up any viruses in the last few weeks.  Then we'll do another "exit interview" to review Ethan's condition and the transplant protocol before we start.  If the CT scan's okay, I'm guessing that Ethan could start chemotherapy as early as the end of this week, but more likely some point next week.

Ethan digging his new hat.
In the meantime, this has been fairly easy time for us.  Caleb is enjoying school and both boys are enjoying playing around the house.  Sarah and I just had Caleb's regular parent-teacher conference here at the house's charter school.  The teachers say he is doing really well both in meeting and exceeding the Oregon academic goals and standards and in interacting with the other kids in the class (who range from age 8-14).  

Sarah and I have both noticed that Caleb is learning lots.  Last week he read a book to Sarah, which is a huge milestone!  And his numbers are getting better, too.  Caleb has also told me that he likes this school a lot because it's challenging.

Ethan is doing really well, too.  He's energetic and active, and he's learning how to do and say new things.  When I look at him, he's doing so well that I wonder what in the hell we're doing here.  It's really hard to believe that he's got a serious degenerative disease, and that even with all the progress he's making how, he'll lose it all if we don't move forward with this.

We may even have solved the problem we've been having with Ethan's dressing.  After talking with a number of other families, we think he's developed sensitivities to the dressing and skin preparation agents we'd been using.   We moved him from alcohol preps and cleaners to iodine cleaners and to a hypoallergenic dressing.  Unfortunately, scratching his dressing had become habitual for him.  But we've finally focused in on the vest that Grandma Lilianne made from him as the best option to keep his hands out of that area.  I think he'll probably be in that vest for at least a couple of months, and we've asked Lilianne for a couple more.  So far, I think we're on day 5 for the current line dressing, which is the longest we've gone since early December.  None of us like dressing changes, so we're all really happy and hopeful!

Ethan's only real problem at the moment is that he's having a really hard time sleeping.  It often takes him two - three hours to get to sleep, and then he usually wakes up in the middle of the night.  We think the Corteff, which is a steroid that was prescribed to stimulate his adrenal response, is screwing with his sleep.  He's going to have enough other things screwing with his sleep once he starts transplant, so we'd really like to figure this one out soon.

But for today, the big decision is do we try to go sledding or just hang out and watch Scooby-Doo movies and play with Ethan's new Cozy Coupe car and the new laser pistol Caleb won at Bingo Thursday.  A fairly normal Sunday for the Waddell/Aaserude clan, and we plan to enjoy them while we've got them...to savor each other's company in these quiet times in anticipation of the more stressful days to come.  Many thanks,again, to all those who have sent posters and care packages..warm hats and chocolate pretzels, games and toys, Utz potato chips and books galore, posters with tropical birds, fanciful animals, cars, and trains.  We are truly blessed to be surrounded by such a warm blanket of well wishes and positive thoughts and prayers.