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.

Comments

Anonymous said…
I can understand your concern and I hope the lavage gives you the info you need. How
frustrating that two different doctors can interpret results so differently! Hang in there, and hopefully things will be more definite soon.

Melissa Thompson
Y said…
Really praying that the lavage comes up with the right answers and you can kill the fungus/bacteria or whatever and move on with the transplant. Also really hoping that nothing else goes wrong ever; or at least for the duration of the transplant year.
Wendy B. said…
That is quite a lot of medico-techo-crazo speak to ingest, Todd. Even my head is a bit twirly from all the blah-blah-blah. It is a very good thing they are so very cautious about Ethan. And, yes it is so very frustrating to have him poked and prodded over and over again (there must be a better word for "frustrating" but it eludes me at this moment). So many cooks in the kitchen!!! You are an amazing family, without a doubt. Say, I missed the address we can send stuff to for the Waddell-Asurude clan. If you get a chance to come up for air send it to me. Everyone at Doernbecher sends their love. Always in my thoughts and prayers.....Wendy
Cheryl Garris said…
It still amazes me the strength you all show daily in dealing with the many things that cross your road. You are such an inspiration to us all. I hope that the lavage is able to give you answers to get things taken care of and get the show on the road for Ethan. As always, prayers are being sent up. Thank you for the updates.

Hugs to you all,
Cheryl
It seems our boy's like to take in turns! Disappointing that the docs have to go in again but hopefully you'll get an answer and be able to treat it and proceed to a successful transplant. By reading your words it always brings back to me the real reason we all go through this process and it's highs and lows, to give our kid's the best life possible however long that may be. You are all in our thoughts, lots of love Teresa, Shane and Corey x