How Weird Weird Can Be

Case here. The optimism of two days ago has largely passed, and reality is beginning to set in. I think this is true both for Wanda, our family, as well as the doctors. On Monday, we were told that chemo was probably the best option and generally the atmosphere of the discussion was very upbeat and hopeful. However, the Vanderbilt doctors still needed to look at the results of the bone marrow biopsy and still needed to confer with the neurologist (for head bleeding) and hematologist (for the hemophilia - factor viii inhibitor antibody production). I think these discussions led to a much more stark reality.

From my perspective, the biggest problem is that the biopsy slides seem to show that this AML is caused by a progression from MDS - so, related to her previous disease. Outcomes of people who have MDS-turned-AML are not good. Here's a quick recap of the numbers they cited us - note that these numbers are for a typical MDS-to-AML patient:

• overall, there's a less than 5% chance of a big picture cure - this is after chemo, remission, lymphocyte infusion, and transplant - lots of hurdles
• left untreated, she probably has a few weeks - could be more, could be less
• death from complications due to chemotherapy in the 4 weeks after starting chemo are somewhere around 10%
• with just chemo, it's expected that the AML would return within 6 months - so the good effects of remission are relatively short-lived

Of course, right now, mom is not a typical patient. She has an active bleed in her head and leg. She has hemophilia caused by the production of factor viii inhibitor antibodies. They would guess that the the these two problems would give her something like a 30% (rather than 10%) chance of having complications from chemo in the first 4 weeks, but it's anyone's guess. She is not typical patient, but instead "challenging" and "unique" (after all, she is still Wanda ;-))

So - what's next? Initially, we thought that we were going to have to choose heavy (induction) chemo or no chemo this morning (Wednesday), but the plan has changed. After consulting with each other, the doctors think the best course of action is to get the hemophilia under control by itself, and then start to work on the AML. In order to do this, they're going to use a common outpatient chemotherapy drug that is usually used to treat lymphoma (Rituxan). The hope is that this will knock out the production of the factor viii inhibitor antibodies and that will then lead to her bleeds being sealed up and healed. Of course, through all of this she will continue to get a good amount of platelet and red blood transfusions. They think they'll wait about 7-10 days after this chemo to ask questions about where we're going next. They see this chemo as relatively low-risk, so mom signed off on it without much thought.

It's anybody's guess as to what treatment Wanda will choose next, but it seems that our conversations are guided by how Wanda wants to spend her time and what the effects of treatment are on quality of life. We all know that Wanda is all about living the good life, and we trust her decisions will be made in a way that supports how she defines Life to be. As the days pass, she has become more lucid and more understandable as the effects of the pressure in the brain start to subside. We've had reasonable conversations with her - you just have to understand that only about 75% of the words she uses are the words she intends. Sometimes she gets stuck on a word and you have to start asking questions to try to figure out what word she is intending to say. While it is frustrating, she still feels like mom. It's maybe a bit unsettling that after spending a bit of time trying to get the right words out and failing, she can say in perfect Wanda intonation and facial expression - "oh come on, Case!" Haha.

Ignoring the prognosis and what is coming next, Kit, Pat, and I had a great day with mom yesterday. She got the EEG stuff removed from her head, took a shower, and was very excited about us brushing her hair. She has proven to have a good appetite for her normal food, but she's also shown a voracious appetite for orange sherbet, which we have an unlimited supply of. As I said, she feels like her normal self. The inability to easily express everything she is thinking may actually be a great thing, because it's meant that we've all been a bit more reflective than maybe we would be otherwise.

Callie, Mike, and Davis will come in town today. Demian (Kit's fiancé) will come in town on Thursday. We'll all be returning home by Sunday except for Callie & Pat, who are going to stay for longer. We've received a tremendous amount of support from friends - food, housing, conversation, swanky hotel rooms - and we are so thankful and think that the cards have meant a lot to her. (Please let us know if you'd like to send one to the hospital - I can provide the address. Just send an email to casetaintor@gmail.com)

There are a few words that mom has been quite fond of using to describe the situation - "crazy" and "weird." It's amazing how many complex thoughts you can express solely through the use of 'crazy' with changing intonation. Perhaps one of the more profound things mom said is that "it's crazy how weird weird can be."

Walking through Fire

Case here. I got in from Tel Aviv yesterday morning around 9:30am and hitched a ride with a friend to the hospital. When I first got in, Kit, Callie, and Pat were all here with Wanda and she was pretty lucid and was able to communicate and I spent the night here with her last night.

It's easy to stay positive if we're only thinking about the head problems, so let me start with that.  The blood on her brain has most definitely affected her ability to say what she wants to say. The nurses come in every hour or two and ask her a few questions followed by telling her to do various things like clenching her first, moving her legs, etc. The typical questions are 'what's your name,' 'where are you?,' and 'what year is it.' Depending on how sleepy she is, she will almost always eventually get her name out, but the other two questions are anyone's guess much of the time - "Pain City USA" was one of the responses to the location query (even though she says she's not in much pain right now). She never has trouble with the movement-on-command parts. It seems that as the day wears on, her responses become more difficult. For instance, our friend Elizabeth who is a pediatrician at Vanderbilt came by around 1pm with lunch for us and mom definitely recognized who she was and greeted her with a fully formed sentence that maybe had one or two words wrong. We took about an hour or two to eat and talk and when we came back, I asked mom who had come by to see her and she emphatically responded with 'Elizabeth!' Fast forward 30 minutes, though, and mom couldn't answer her name nor who I was. The brain is a crazy place indeed. Overall, her night was not-that-great when it comes to having coherent responses, but when 6am rolled around, she was demanding food and we were having a good conversation - obviously labored and confusing, but more complicated than just yes/no/gee. She's had a good appetite this morning and has been showing off her mental state to the slew of doctors that have come by and even went so far as to remind Dad that he needed to get the registration renewed by the end of the month on her S2000. To sum up all of her head issues - there's blood on her brain, it's stabilized, her mental function is getting better.

Now we get into the nitty gritty - why has her blood not been clotting and what about AML?

So - they know that her blood hasn't been able to clot due to her B cells creating factor 8 inhibitor antibodies. This inhibitor keeps her immune system from orchestrating blood coagulation, which means she wont stop bleeding even if she had a plethora of platelets. As far as I can tell, they have done two things to fix this, both considered temporary. First, they are giving her Novo 7 which orchestrates coagulation through a different means (??). Second, they have given her prednisone, a steroid, which should keep the B cells from producing the inhibitor. This has worked at stabilizing everything, but it's not a permanent fix.

Her bone marrow biopsy shows that she has 30% blasts, which is how they determined that she has AML. They are currently looking harder at the results of the biopsy to determine what has caused the AML (evolution from MDS, donor cells, her cells, etc), but the result of that shouldn't affect their next, immediate, course of action. She's going to need chemo to put the leukemia into remission and get us into a place where she can heal as we figure out the next steps.

OK - all of the above sounds reasonable, except there are a few problems that the doctors see. First, they don't really know why her body is producing the inhibitor. Their best guess is that the leukemic cells (T-cells, I believe) are somehow communicating with the B cells to tell them to produce the inhibitor. They tell us that they have never heard of this specific thing happening - typically they associate the inhibitor production with something like GVHD. It's not very comforting to feel like we're living an episode of House here. Second, chemo with bleeding in the brain is risky. If things go wrong, fixing it is complicated. Finally, she's already in a weakened state - far different from the last time she had chemo.

So - here's the plan, subject to change:

She'll go on chemotherapy tonight or tomorrow. Typically for AML, chemo consists of 7 days - 7 of one drug and 3 with two. Due to her current situation, they are probably going to go with 5/2 instead. Two weeks after starting chemo, they will do another bone marrow biopsy to see if the AML is in remission. If not, then more chemo. Unlike her previous chemo, this chemo will likely involve lots of transfusions of platelets and red blood cells. The doctor described this as being like "walking through fire." When the doctor asked mom whether she had any questions she laughed and said no and he responded by saying "well, I'm glad, because I probably don't have any answers."

As I said, their best guess is that the leukemic cells are causing the production of the inhibitor, but they could be wrong. If they're right, chemo will wipe out the leukemic cells which will also stop the production of the inhibitor - a good thing. If they're wrong, then she'll be very low on platelets and even if they add lots of platelets, her body will still be producing the inhibitor so she's at a high risk of bleeding. This could lead to blood building up in her brain which may mean surgery - a risky endeavor to say the least.

If all of this is successful, then we'd start talking about next steps, which may involve another transplant or something involving the lymph nodes... but the doctors aren't ready to talk about that yet. They want to get through the next 2 weeks before discussing the next steps.

So - them's the facts. For all the negativity you see above, I think there are reasons to be hopeful. First, mom looks good aside from the bruising and head issues - she has color and smiles (the permanent eyeliner was a good move for her.) Second, she reacted to the doctor's news with laughter in a sort of "well, that's where we are, let's get on with it" sort of way, which I will interpret as good of a response as can be hoped for. Finally - my mom is incredibly strong and positive and has an amazing support network of family and friends. Please continue to send your thoughts and prayers our way.

Hungry Wanda!
(The head wrap is for an EEG which has so far shown that we shouldn't be worried about seizures.) 

In Nashville

Thank you all so much for your love, prayers and support. We are so sad to be writing to the blog again, but I guess that's life.

Wanda was officially served a shit sandwich this morning.

Doctors have confirmed from the bone marrow biopsy that she has AML (acute myeloid leukemia). They were very quick to say that the prognosis is not the prognosis for a relapse from a transplant, which would be pretty darn bad news. Instead, because she is 3.5 years out, the prognosis is the same as for a new leukemia patient. Which they think is good.  As soon as she stabilizes here, they would begin aggressive chemo to get the AML in to remission and then begin the stem cell transplant party again.  In the next few days they will determine whether the leukemia originated in the remnants of her blood or from her donor blood. This does not change anything except whether or not we need a new donor. If the AML leukemia originated in her blood, they will use the same donor. If it originated in his blood, then we will be on the hunt for a new donor.

This news was hard to receive. The pressure in mom's brain has made that even harder. She understood (mostly) all that her dr presented us, but she was not able to articulate questions or reactions.

Which leads me to the hurdles she has between now and chemo. Unfortunately, those hurdles feel very significant right now.

They have not been able to 100% address the issue of her bleeding. The coagulant is working but not perfectly. This means that they will not be able to perform surgery to relieve pressure in her brain even if they wanted to. It seems like there is a lot of blood in her brain, and what we are praying for right now is that we do not have a situation soon where the neurologists need to perform surgery and the hematologists don't want to.

So we have two prayer requests today. First, that her bleeding in the head has stopped so that we aren't faced with the potential of having to decide whether or not to risk potentially necessary brain surgery at a time when we are unsure that we can control bleeding.

And second, that she is not losing heart. It is hard to know, and that terrifies me. She has (short) periods of alertness followed by extreme tiredness and general non-responsiveness. In the alert periods she can often muster a smile. We all know she is a fighter, but I can't help but worry.

Case will be joining us here in Nashville in the morning. We tried to make mom choose which of us (me, Kit or Dad) should go with her to get the most recent CT scan, and she said Case.

Lots of love-

A Bump in the Road

Case here. It's been quite a while since any of us have written an update on Wanda's progress. This is mainly because she'd mostly returned to her former, spunky self. Sophie and I just had our first baby, Patrick, in February and we were able to spend a lot of time in Chattanooga. Since we were there, Kit, Callie, Mike & Davis all came down and our whole family was able to spend a weekend together at the beginning of April. The weather was great and we had a wonderful time.

Sophie, Kit, Wanda, and Patrick

It's been a few weeks since then, and we've all returned to our respective homes (for Sophie and I, this means Tel Aviv). Wanda was planning on coming to Israel for three weeks starting next week in order to take care of Patrick during the day. Unfortunately, we seem to have hit a sizable bump in the road.

Wanda hadn't been feeling well the past few days, with a cough, a headache, a toothache, and pain in her shin. She went to her doctor, who took blood to perform a standard CBC and sent her home with some medication for allergies. Things took a turn for the worse on Tuesday night, when Wanda started vomiting and generally feeling really terrible. Pat & Wanda decided to go to the ER at Erlanger on Wednesday morning when they saw that her leg was very swollen and she was in immense pain.

Erlanger admitted her and began trying to figure out what was causing the extreme swelling, starting with an ultrasound to try to rule out a blood clot (well, deep vein thrombosis or DVT). They also did a CBC and found that she was anemic, had low white blood cell counts, and was dangerously low on platelets, so they gave her a blood transfusion. They did an MRI on her leg which confirmed that there really weren't any clots or tissue damage in the leg. They also did a CT scan which found that she had excess blood in the brain, so they scheduled an angiogram to rule out an aneurysm. Finally, they wanted to get a bone marrow biopsy and, in doing so, they found that the wound was taking a long time to close up - so, something was wrong. I believe it was this that led the hematologist at Erlanger to run some more tests which showed that mom had a coagulation factor inhibitor - basically, her body was producing something that was preventing her blood from coagulating - thus, the bleeding wasn't going to stop quickly. This has led them to give her a "Novo 7" shot(?) which helps the blood coagulate.

Through all of this, they have wanted to transport her to Vanderbilt, where she will get the best care for her situation - Vanderbilt was where she had the stem cell transplant done. Unfortunately, each one of these problems has meant that the doctors have been hesitant to transport her since she wasn't stable. With the addition of the Novo 7 and an angiogram with a negative aneurysm result, they have now determined that she is stable and is ok to be transported to Vanderbilt by helicopter. Callie and Pat are waiting with her and will travel to Nashville once she gets on the helicopter.

So - that's where we are. Callie, Pat, and Kit will be in Nashville with Wanda tonight. Mom was talking (a bit) and forming coherent sentences, which is an improvement from earlier, but she has many obstacles in front of her. There is a strong possibility that this is MDS or something in a similar vein, but we don't know for sure and won't know until the biopsy results come back. I do know that my mom is an amazingly strong woman, always has a great, positive attitude, and has some of the best friends out there. Your support would be greatly appreciated while we figure out what's up. Love to all of you!