Annie, Get Your Gun

As you know, I’ve been using the Russian roulette metaphor, which I think it rather appropriate.  In this entry, I thought I would provide an example of  what we are dealing with in it’s gory detail.   And in doing so, I must also put a discretionary warning here.  This entry is not pretty. If you are sensitive in nature, I do not recommend that you read on.

I know it’s a bit of a bait.  After all, I have roused your curiosity.  But, I don’t know how else to proceed.  Indira and I have discussed whether we should post such an entry.  One of our goals is to show what it is like to deal with paediatric cancer.   Part of us starting this blog was to have online some information that could be beneficial to others.

Additionally, I also want to highlight the difference between the commercial side of cancer and reality.  There is rather a large discrepancy.  I understand why commercials are necessary.  It helps with funding as well as create a perception that everything possible is being done to help those who develop cancer.  It also makes those who decide to support cancer causes feel good about themselves.  There is nothing wrong with that.  And if that is the end goal for some, that’s great on it’s own right.  We would not be as far as we are without the funding from those who have so kindly decided to help.  James Fund is but one of the great organizations that people can donate their funds to.  We have a great amount of respect for the Birrell family who decided to take up the cause after they lost their son.

In commercials, there are smiling children (and adults) who are triumphant in their “victory” with display of gratitude for those who help beat the disease. Of course, the numbers do indicate that there are more survivors. But truthfully, it’s somewhat misleading.  There hasn’t been any signficant advances in cancer.  Advances in paediatric cancer is almost non existent. WHO expects the rate of cancer to double by the year 2020.  This figure should be of significant concern.  One does not need to look at the stats.  We all know and lost friends and families.  That wasn’t the case some thirty odd years ago. But this post is not about the politics and/or beauracracy.  This is about what it’s like to deal with cancer in an intimate detail.

And lastly, as you know we are in Memphis TN, at St. Judes.  St. Judes is probably the best hospital that we’ve been to.  One of the things that is most impressive about St. Judes is the recognition that paediatric cancer affects the entire family and they have made a significant effort in ensuring that the family is integrated in the treatment.  It is also free for those who are accepted. So, I must also state that what I am stating here is not about a short coming of this particular hospital. Rather, if I had anything to say, I would say that St Jude is a wonderful hospital.  And given the serious nature of Mayas’ disease, I don’t think we could be at a better facility.  I believe without a doubt that our odds are better here than anywhere else we’ve been to so far.

Fasten your seat belt. It will be bumpy.

Staring at T+2, things turned on a dime, as is often the case in cancer therapy.  The symptoms started to appear. The following two video, before and after,  really high lights how tough the therapy is.  These two videos are taken about 10 days apart. This therapy is not as toxic as the one we’ve had before.  We elected to participate in this therapy because it was not as rigorous as other type of transplant.

The symptoms were:

Fever
Fever started and continued for a week. Several times a day, Maya spiked a fever.  When she did, she would shiver violently and asked for warm blankets and hot packs.  Often, we could not give her the warmth that she so desparately needed because we did not want her to overheat.  Moreover, tylenol was held back because the source of the infection was not known.  We did not want to mask the symptoms in case it was some sort of bacterial or viral infection.  Without an immune system, if untreated, the infection could become septic very quickly.  After all, Maya right now is a perfect human petrie dish. So, the fevers were very stressful. What does she have? Is it bacterial, viral or fungal?

The only thing we can do is stand by her feeling completely helpless, running possible outcomes in our minds.  Helplessness is a constant companion of ours.  She is there like an uninvited guest overstaying her welcome.  At all times our thoughts jump to sepsis and losing her through complications of the transplant.  This happens to 2 – 3 % of children.  Is this how we lose her? is always at the forefront of our mind.  For a few days, we could not even offer her the comfort of warm blanket.  How do we console our daughter?  How do we explain why this is happening to her?

Diarrhea

Diarrhea also started around the same time.  Maya was not able to control her bowl.  So we put pull-ups on her.  Again, the cause was not established.  Could be any one of causes, including the new cells that was infused into her.   Diarrhea got progressively worse and around T+5, we were changing her over 20 times a day.  This lasted for about 10 days. A diaper change consisted of:

  • pulling her pull-ups off.
  • clean using a vapor barrier creamed wipes.
  • apply soothing cream
  • put back the pull-ups
  • If she soiled her PJ, we have to change her as well.
  • If she leaked to the covers, entire bedding was changed.
  • Try for a sitz bath or even a shower.  Shower is tough, when Maya can’t stand on her two feet.
  • There were several hand washes in between these steps as well

Again, significant care is required because she is neutropenic and is very susceptable to infection.  The staff is available to help us, but can any parent relegate such tasks to someone else?  Well, I can’t do that.  We take care of our child.

To call Mayas void as diarreha is somewhat misleading. It’s more or less water with green tinge (bile) passing through.  Part of the reason why it was so runny is because the lining of her GI track was sloughing off.  Both Indira and I watched in muffled horror as we found strips of her intestine being passed into the diaper.  This is but one of the side effects of the chemo.  We dare not show our internal state to Maya. We dare not show our emotions even to each other.   We simply had to grin and bare it.  Each time we pull her diaper off, we put our “game face on” and keep our heart in check.  Doctors have seen this before and they assure us that it’s not unexpected and the lining will come back.  We can only find solace in the doctors words, although the gap between our mind and our heart is miles apart.
Nausea

Nausea became also a constant companion as well.  For a few days, it was not controlled.  The sources of nausea could be:

  • The new cells comming in.  Watch carefully.
  • Infection
    • Apply antibiotics (Cefepime and Vancomycin).  Vanco causes redman syndrome for Maya. Therefore, apply Benedryl.
    • Apply Antiviral. (Acyclovir and Ganciclovir)
  • Side effects of chemo
    • The GI track sloughing off will make her nauseous.
    • Added Ativan, Bendryl, phenergan at different doses.  Also added Marinol for nausea as well.
    • Is she absorbing these drugs at all?  It is questionable since her GI track is under duress.
    • All we can do is wait for my cells to engraft and help with the repair process.

During these few days, Maya stopped eating all together.  Subsequently, a NG tube was added. Maya hates it. But at least we can introduce some nutrition as well as drugs.  It is questionable whether there is any absorbtion of drugs and nutrition, but nevertheless it is necessary.

Maya also had very thick mucus build up.  It built up around the NG tube that was running down her esophagus. The mucus is so thick that she can not swallow it. It also made her throw up.  The best way to deal with it is as Maya is throwing up, you grab it with your hand and try to pull it out.  It’s not pleasant for Maya, but infinitely satisfying to see it removed from her throat.  For an hour or so, Maya would find nominal relief from nausea.  We start over.

All of the antinausea (anti-emetic) drugs made Maya sleepy.  (Ativan and Phenergan are antiemetic as well as anxiety reducing drugs.  Benedryl is an antiemetic as well as anti-itching drug)

Oh, let’s not forget morphine and hydromorphone to control pain.  Both of these opiates makes Maya very itch.  You can see from the above video clip how itchy it gets.  If left to her own devices, Maya will scrach until she draws blood.   For the itchiness, Benedryl gives her some relieve.
Stressed Organ Function

As we’ve loaded Maya with all these drugs, inevitably, Maya’s liver and kidney objects.  We are hypersensitive to her liver function because of her history of VOD.  We are hypersensitive to her kidney function because Maya has only one kidney.  The left kidney was sacrificed to the god of Cancer in exchange for Maya’s life when we began our journey, some four years ago.

Watching the liver and kidney function is a daily exercise.  How is Maya’s liver?  AST/ALT/Bilirubin are treding up!.  Oh no…. please please stop!  Maya is kept dry to reduce her chance of developing VOD again.  Heparin and Ursodial are also given to Maya prophylactically.   Heparin is infused into her blood system, so no problem. However Ursodial is oral.  Is it being absorbed?  We question it as we see the liver enzymes continues its upward trend.  Oh no… Oh no…  We try to rationalize.  Although Maya is complaining of some stomache pain, clinically Maya does not look too bad.  Portal vein looks OK, no backflow of blood indicating blockage, and her weight is not increasing.  Doctors are not worried yet, although they are keeping a close eye on the liver.  We are defintely worried.  But, stiff upper lip is required here.  There is no sign that she is developing VOD yet.  Please please, stop rising!

Maya’s kidney was doing very well for the amount of drugs that’s floating in her system. That was a relief, until yesterday.  Suddenly, Maya’s creatinine / BUN started to rise very suddenly.  Oh no! Oh no!!!  Within 12 hours or so, creatinine spiked.

Time Creatinine
5 AM  0.83 Woops!  This is a bit high. Let’s do another test at 2 PM.
2 PM 1.43 OK. This is definitely not good. What’s going on? We need to hydrate Maya more.

Saline Bolus was given and her fluid level was increased.

8 PM 1.60 Oh My God!!!! it’s still going up.
12 PM 1.40 Thank god, it’s trending down!!!!

We feel helpless.  Again, the wild horses are out.  is this how we are going to lose her?  Did we shorten her life by agreeing to this therapy?  If so, she could have lived another year or more.  How did we find ourselves in such a situation?  How will we live without our little girl?  We have nothing but time to run these scenarios in our heads.

For about 8 hours or so, we were on the edge of an abyss.  Of course, the doctors are supportive, but no one can tell us why Maya’s kidney is stressed.  The doctors recommend hydration and Dopamine.   It is thought that the renal dosing of dopamine may be helpful, but not harmful.   We do a quick search on the renal dosing of dopamine.  Even in the scientific literature, the results are all over the place.  Some suggest it works, others suggest it does not work and is not recommended.  Others still suggest it can be harmful. Further, the literature is based on the adult setting and not for paediatrics.  Of course, we take our lead from the Doctors.   But, they are not certain how Maya will respond either.

Yes to additional hydration and let’s wait on the dopamine. If Maya’s kidney remain stressed, we will go head.  We sit and wait.  Please please please… Time is both our friend and our enemy.   Please let the hydration do it’s thing.  We need time for the kidney to respond to the additional hydration.  If the problem is not related to just being dry, we are eating away at the precious time to find the solution.  We envision the worst possible outcome.   OK.  I can give Maya my kidney.  Indira thinks the same.  Since Maya has my blood now, my kidney would be more compatible to Maya at this point.  We don’t want Maya to lose her kidney and that would be very bad.  But, losing her only kidney would not be as bad as losing the liver.  Losing a liver will mean the end.  We could recover from losing the kidney, if we can perform a transplant. We wonder if we just pulled the trigger with the bullet in the chamber. What do we do now?  Only thing to do is wait and watch the wild horses gallop away.  At midnight, labs were drawn again.  We wait another agonizing hour for the results to come back. It is 140.  A wave of relief washes over us.  140 is still very high. But the kidney is responding to the additional hydration.  Click…

We are by no means out of the woods yet.  And there will likely be additional scares in the near future.  But for now, we’ve side-steped a potential catastrophe.  We sigh a sigh of relief.  The adrenalyn running in my body can do it’s part and make me ache. Happily.     Both Indira and I push our heart back down only to notice that it is once again overflowing with love for our child.

 

[Colitis Feb 16, 20:30 update]

Well, that didn’t take that long.  This post was written on Feb 14th. In that time, we’ve run into another complication.  We are in the midst of our current drama.  We will update our current status as we move forward.

When Maya’s liver enzymes started to increase, an ultrasound was performed.  Maya did not enjoy that at all because her liver was slightly enlarged and it hurt to have the ultrasound device pushing down on her liver.  The team here was trying to assess whether we were dealing with VOD.  One of the findings of the study was that Maya’s colon was inflamed (Colitis).  On T+13, MMF was started. MMF is used as an immunosuppressant.  We don’t want my cells to come in too quickly for fear of GVHD.  While all her counts were coming in nicely, Maya had severe nausea as well as persistent diarrhea.  During Indira’s shift last night, she got a big surprise.  Diarrhea had significant amount of blood in it.   So, when I was called, I was somewhat prepared for it.  Yes, there was significant amount of blood in the diarrhea.  But Maya’s counts look good and I saw some clots.  So, no big worries yet.  After all, we have good platelets and that should mediate the bleeding in the colon.

This morning during the rounds, Dr. Leung felt that MMF was the culprit and stopped the infusion.  Further, in order to control the inflamation, Maya was started on infliximab.  Both MMF and Infliximab have wide ranging serious side effects.  What are we dealing with here?  There is a talk of performing a colonoscopy in the morning.  It is too early to determine what the issue is.  And each time we change the diaper, we put our game face on.  It’s not easy to see so much blood in the diaper.  Again, we have but time to look after our precious daughter.  Looking at the side effects of these two drugs is especially heart wrenching for Indira.

Me personally, I reason with myself.  The counts look good.  Maya looks…. OK clinically.  She just needs to heal from the assault.  Too early to  jump to any conclusion.  Perhaps I need to hear myself convincing myself.   Again, the wild horses are running amok.  We wait.

[/Colitis]

 

This account is but a sample of what we the paediatric cancer family deals with.  Of course, it’s not like this for all families.  It depends on the disease, the stage as well as the histology of the disease.  If you are stage 4 with unfavorable histology, while the details may vary, but helplessness, the fear are common.

Still, we are blessed to have Maya with us.  Yes, there are hardships.  But hardship is just hardship.  There are many things that are greater than hardship;  Love, devotion, compassion, inspiration and human spirit all rise above hardship.  These human traits are all still with us.

If  a time comes when we feel that it’s time to let go, we will do so.  Hesitantly, but nevertheless, we will let go.  It is not about fullfilling our needs at all cost. But that time has not yet come. There are still hopes and dreams for our little Maya.  Where does our road head?  It’s impossible to tell.  But our future is only friviolous meandering from our current position.  Thankfully, we have a sick child to attend to.

And thankfully, again, the ringing sound  in my ear was that of the hammer hitting an empty chamber.  Click!

Diary of a Wimpy Parent

Cancer world is a strange world. So far, we’ve been in active therapy for the majority of the past four years. Once you are here, you are submersed in it. It consumes all of your energy. Nothing exists outside it.

Social conventions often are stripped away from us. We live a subsistential life. How do we keep Maya alive takes most of our energy. There is little room for anything else. Battle fatigue is but one additional burden we carry on our already overloaded shoulders.  Could we ever go back to a place where we would post what I ate for breakfast on facebook? Not me.  But it would be nice to have such luxury of mind.

The most profound difficulty we deal with is the uncertainty from which we are asked to make decisions. How many times have we made decisions purely by the seat of our pants? How many times have we “pulled the trigger” not knowing if we are making the right decision?  How many times have we “pulled the trigger” knowing that longer we are at it, inevitably the bullet will fire? Is this it?  Have we finally run out of options? Of course, the gun is pointed at Maya. For example:

  • Will you perform the primary tumor bed irradiation? Irradiation will cause additional stress to an already inflamed liver and it may cause additional “complications”.
    • If you agree, you can proceed with the protocol.
    • If you do not agree, you will be off the protocol.  Maya’s chance of survival decreases.
  • Many of these therapies can cause secondary malignancies, such as lymphoma, MDS. We got MDS.
  • Would you like to participate in the Fenretinide clinical trial? It has been shown to work with maturing the neuroblastoma cells. No thank you. Why? Just did not feel right. We’ve learnt to trust our instinct.

We’ve made tough decisions not based on reason and rationale, but rather on gut instinct. In many cases, there isn’t sufficient data from which to make a sound decision. MDS for example is not a disease children get.  There are only handful of children who got it, most as the result of therapy. Most of them are dead. We make these decisions based on what literature we can find on a specific therapy or a study. In the case of MDS, there aren’t any.  Although we have learned quite a bit along our journey, we do not have the background to digest enough information to make the optimal decision. So, gut it is.

And why would you not trust the doctors in these matters? Very very few doctors will make recommendations. Why? I don’t know. However, I suspect some of the reasons are:

  • Unless you are at a larger and more reputable centers, oncologist themselves are not aware of the bleeding edge therapies. You are on your own.
  • There may also be egoes involved. “If we can’t help your child, no one can…”
  • Legal liability? I can’t tell you which therapy you should try because I may be sued.  No one gets fired for following protocol.

These are just my guesses. Whatever the reason may be, the outcome is the same. We are put into an impossible situation, expected to make life giving/taking decisions without the necessary tools or support. When we are forced into a situation where a decision is absolutely necessary, we try to understand to our best ability, we make a prayer, with our hearts in our throat, pull the trigger. The pressure that comes with such a decision is ineffable. It is overwhelming.

Given the circumstance which we find ourselves in,  after all that we’ve been through, we are often gob smacked when some doctors or nurses, typically a plebe, who insists on text book approach to Maya’s care.  They are too eager to flex their technical prowess. They have not yet understand that above anything else,  it is their compassion and empathy that will make them a good health care providers.  Often they balk at our questions and requests with the air of paternalism which is so misplaced.  To them, I say this:  Please please save my child.  If you do, or even if you just try your absolute best with personal investment in Maya, I will do whatever you ask of me. If you can’t, then perhaps you should reassess your significance in the lives of these desparate people, such as ourselves.

Perhaps it is because we have challenged the system that Maya is still alive.  Nevertheless, the system has worked for us thus far.  Maya  is alive and full of beans.  We will never discount that and we are grateful that we have had opportunities to keep Maya with us.  Of course, the cost has been exacting in many many levels.

After four years, we are but a handful of parents who are still left standing. We stand with our daughter with gratitude. We also feel guilt towards those who have fallen along the way. Why us?  Will we be next? I still see Connor – the first neuroblatoma child we met and lost-  walking the halls at Sick Kids, whenever I see a five or six year old bald headed boy. When we meet the surviving families, there is an intimacy that can only be shared between us.  It’s hard to put into words what that is.  I suspect it is not too different than what soldiers – brothers in arm – must feel. There is a deep level of connection, happiness in seeing them as well as sadness and guilt in being alive.  We know that the battle scars are all there.

At the end of the day, those who stand and those who faced the horrors have nothing more to do than the luck of the draw. A close (neuroblastoma) friend we know of figuratively dodged a bullet in flight.  After four years or more, we don’t really know why we still stand. Perhaps it’s a combination of luck, timing, some lucky decisions on our part, support from some incredible doctors, and the constitution in our children to weather the greatest storms of their lives.

We did not choose our children to be ill. It isn’t anything we might have done or even genetics. I don’t know if there is a lesson to be learned. I don’t care for one really. What I do know is that it has challenged and tested our love towards our child beyond what I thought was possible;  that part of our success as a species necessitates such dedication and devotion towards our children in all of us. Therefore, one can be assured that if an unfortunate circumstance arises in life, everyone is capable of withstanding the demands of it, although the outcome may vary.

There is nothing remarkable in this story aside from the fact that we are given the opportunity to express our love. That and the realization of human spirit and kindness.  If there is a realization in me, it is just that I love my children more than I could have ever imagined. And that is good.  We do what we must.  And presently, we can still enjoy our daughter.  That is enough for me today.

Time Warp

It’s hard to believe that we’ve been here in the Bone Marrow Transplant Unit for two weeks already. I don’t know where the time goes.  We’ve known that.  It’s a time warp.

It’s just a jump to the left

We have the chemo.  While the regimen is not myeloablative, still it devastates the marrow.  Maya’s count has dropped to a point where moderate transfusions were necessary; in particular, the red blood and the platelets.  And as you may know from facebook, we have had to deal with pretty significant allergic reaction from the platelets.

There were also prophylactic application of antibiotics.  Vancomycin has also caused thered man syndrome.  That’s not been so easy to deal with.   Maya becomes very red in the face, neck and the upper torso.  It is also incredibly itchy for her.  In order to counter the red man syndrome, Maya has been premedicated with Benedryl, which knocks her out.  Vanco is infused every 8 hours to fight infection and so, Maya has been knocked out for a good part of the day.

And then a step to the right

is the fevers.  Since T+2, Maya has been dealing with the fever.  Both blood and fecal cultures are done on a daily basis, but nothing has turned positive.  So, at this point, the source of the infection is a mystery.  Vancomycin is the goto drug for possible bacterial infection.

We all know what it’s like to deal with a fever at home.  It’s worrisome to deal with fevers at home when our children are sick.  The main difference here is that Maya has no immune system to fight off the infection.  If you will, Maya right now is a perfect human petrie dish.  And infections can and do cause death in this place, although the chance of that happening is not too great.  Nevertheless, we worry.   She’s been running 39 + degrees for three days now.

But it’s the pelvic thrust

that gets both Indira and myself wiped.  Maya has not been eating since T-3 or so.  On T+1, Maya got an NG tube. Through it she is getting both the nutrition as well as her meds.  She needs all the energy she can get.  But for some reason, whether it is the infection or the antibiotic or whatever, she’s also been dealing with diarhea.  We need to change her a dozen times or more a day to keep up with her.  Since she is immunocompromised,  we have to be extra careful.  We are all tired.

You bring your knees in tight

Today T+5 is the Natural Killer cell collection day.  In a few hours, I will head down to the donor room and get two pokes with these giant needles again.  I will sit for 3 or 4 hours and have my blood get drained, where the NK cells will be selected out.  These cells will be infused into Maya tomorrow.  The NK Cell infusion will cause additional fevers as well.

And all these moves makes for a timewarp!

But !  Both Indira and I will do it a thousand times and over.    Yes, I feel a bit goofy.  But it’s the time warp.