Day +34 Christmas Day

John is in REMISSION.  Results from his most recent peripheral blood MRD test show that he is MRD NEGATIVE 0.000 (there are no leukemia cells present at that sensitivity)!  Woo Hoo! Typically the test is taken from a sample of bone marrow but John is still too compromised for sedation.  As he starts to recover, a follow up bone marrow test, we pray, will confirm his remission. 

His chimersim from last week was 100% (100% donor cells).  However this week is 99-100%; hematology was not able to differentiate if the other 1% was donor (me) or patient (John).  As always the chimerism will be drawn again on Monday and if the results are the same, a “boost” of my cells may be necessary.  I pray that is NOT the case and he is a very strong 100% donor next week.  Although a 100% chimerism is not a guarantee that he won’t relapse – having a 100% donor immune system gives him the best chance at remaining cancer free.

We have so much to celebrate & be thankful for!  But, still so much to be concerned about.  We are not only fighting cancer we are fighting very serious liver damage.

John’s liver is extremely compromised and it is causing a multitude of obstacles in John’s recovery.  Years of therapy have caused severe liver scarring.  The blood flow to his liver is backed up, increasing the pressure within the large portal vein that carries blood to his liver. This pressure is forcing the blood to seek alternate pathways through smaller veins, like those in the lowest part of his esophagus causing John to have esophageal varices (the thin-walled veins in his esophagus are ballooned with the added blood; which can rupture and bleed).  After consulting with an outside GI doctor, John was placed on a medication that helps with portal vein hypertension (it doesn’t fix it and has not alleviated all of symptoms).

The obstructed flow of blood through his liver’s portal vein is also causing John’s spleen to enlarge.  His enlarged spleen is trapping his platelets.  The result, the platelet and blood cell count in his bloodstream continue to fall dramatically.  Due to entrapment, his spleen grows and grows in size, and as it grows, it is trapping more and more blood cells and platelets.  These blood cells and platelets are clogging his spleen and interfering with its normal function.   His new marrow is successfully producing blood products but his spleen is hoarding them, not allowing them to flow into the bloodstream therefore his blood counts are not able to fully recover post transplant - worrisome.  So, what can we do in the short term to help?

After much consideration his medical team recommended an additional 6-10 session of radiation to the spleen.  The hope is that the radiation will improve the function of the spleen.  As of today, he has completed 6 sessions and there has been little to no improvement.  We will give it a few more days then we’ll need to discuss the possibility of removing his spleen (this is not ideal because it would increase the pressure in his portal vein).  

Because of his liver damage he also continues to experience ascites, fluid build up between the lining of his abdomen and his organs.  It is managed for the most part with daily diuretics (but that is very hard on his kidneys – and we don’t want to add kidney damage to the mix).  However quite a bit of fluid continues to pool around his lungs; making it difficult for him to take deep breaths.  Because he can’t take deep breaths his alveoli (the tiny sacs within the lungs that allow oxygen and carbon dioxide to move between the lungs and bloodstream) are not fully open.  He requires oxygen throughout the day to ease his breathing burden.  Various breathing exercises are helping but the fluid buildup and lack of mobility are preventing him from having a full pulmonary recovery.

So, if you haven’t noticed already the liver has a systemic effect – reeking havoc.  There is no quick fix for John’s liver.  Only time will tell if the stem cells from his transplant will help heal/regenerate what few healthy cells he has left in his liver.  They may or may not compensate for the damaged cells.  Unfortunately time is not on our side.   We are investigating other options, temporary options (possibly a shunt).  It is also possible in the future John will require a liver transplant – I would be the donor since we now share the same DNA. 

Last week we were discharged from the transplant unit and headed out into the “outpatient” world.  It was ridiculously overwhelming.  I say ridiculous because if we didn’t laugh at ourselves carrying a portable hospital on our backs we would have cried.       

Every night before bed, Rich, Bella and I sit at the kitchen table and draw up the next day’s 30+ syringes of medications (so we would have the entire day’s medication with us at the hospital).  This entails Bella making the labels (medication name, dose & time it needed to be administered) and placing them on the right size syringe – Rich & I organizing the pills, cutting them, crushing them and melting them in sterile water then drawing them up into the correct syringe (to place in his NG tube).  Some need to be refrigerated so they are placed in a “to-go” cooler along with his outpatient IV meds that are in eclipse balls that we administer directly into his central line.  We double and triple check our work so no mistakes are made (we are exhausted). 

Then Bella, Rich or I get John’s feeds ready for the night (pouring the protein formula into a bag, priming it, clearing the pump and attaching it to John’s NG tube).  

We change the batteries in his food pump, his hydration pump and his Dilaudid pump.  They are all attached to a pole that we can push around the Target House/hospital. 

Then off to bed, not to sleep.  John is up at the very least 6 times a night.  No one is getting any rest.  

Next I pack up the hospital supplies we need to have with us throughout the day, in a very oversized and overstuffed backpack – it looks like I’m climbing Mt. Everest.  Half the time I can’t even carry it because I’m pushing John’s wheelchair and maneuvering the pole – trying to get from appointment to appointment.   Little Bella carries her school backpack and the hospital supplies backpack.  I call her my little Sherpa.  Again if it weren’t such a funny site we’d be crying.

Since John requires oxygen (and there is no physical way we can carry around one more thing let alone a tank of oxygen) we race from place to place finding an oxygen hookup.  Its very stressful and a huge problem.  

We spend the better part of 8-10 hours a day in the medicine room getting IV medications and blood products to support John’s recovery.  That’s after all of our other appointments.  We start our day in the hospital early and end it very late.  By nightfall we are all dragging ourselves into the parking lot.  It’s a true team effort! Thank goodness Rich & Bella are still here.  

There is so much to remember I have to keep a daily spreadsheet of medications, labs, appointments & questions for the doctor.  If it’s not written down I probably won’t remember it.

After four days of endless hours in the hospital and frantic searches for an available oxygen hookup we opted to get readmitted to the transplant floor for at least the holiday weekend.  It will give us a chance to regroup and come up with a much needed and much better outpatient plan!  

Another reason we need to regroup is because we are not familiar and not content with the “team” approach surrounding John’s care.  The transplant doctors/nurses operate as a team.  There isn’t one “go to” doctor responsible for John.  If we have a question during hospital hours we are just supposed to call the clinic and talk to whatever doctor is in the clinic.  If we have a question after hours we are supposed to call the on-call doctor.  WTF!  That’s not going to happen.  Rich, John and I are accustomed to the Dr. Pui & Martha approach – which is, one doctor and one nurse practitioner totally invested in caring for our child, accessible whenever, wherever.  It’s also known as continuity of care!!!!  Caregivers striving for excellence!  After multiple requests, via scathing emails, for a 24/7 contact (that would know every detail about John so we wouldn’t need to reiterate the last 3 years) we found ourselves at a standoff.  We need feel comfortable with John’s “team” and if we can’t find some middle ground then the Hagen’s are going to be a permanent fixture up in the inpatient transplant floor.  

So here we are, inpatient on Christmas Day.  Today was supposed to be John’s first day off-treatment.  We had so many plans for how we were going to celebrate.  It’s heartbreaking to see John where he is vs. where I dreamed he would be.  For this reason, other than celebrating the religious significance of the holiday I’m just trying to get through the day.  Don’t worry; I splurged on gifts for Rich & the kids (Santa also came to see the kids this morning in transplant, bringing some additional amazing gifts).

 

A special thank you for all of the many thoughtful and generous gifts sent to us at the Target House!  (some came without cards so I’m not sure who to thank - sorry)  We appreciate everyone’s kindness and well wishes.

Merry Christmas.  I hope you all have a very blessed day with your families.  

I pray the New Year that is upon us brings renewed Health, Hope and John’s forever cure!  

So I end this post, watching John sleep in a hospital bed, while in my Christmas jammies, sipping my coffee, pretending for a moment that everything is ok. 

Love, Kathy