This is among the most painful updates I’ve had to write. The emotional and physical suffering our family has endured over the last week and a half is unbearable. At times I can barely keep my composure – It takes every last bit of strength I have to stand next to John’s bed and see him suffering this way.
A timeline is probably the best way to describe the events that have taken place:
John was doing so well. He was healing from his splenectomy, his engraftment was rocking & his MRD was on the way down. For a moment we actually felt like “we CAN do this!”. With transplant day +100 right around the corner I even let myself start to think of going home.
Feb 15th – 18th
We were settled back in at the Target House. John was weak but we all attributed it to his lack of nutrition – we increased his feeding and waited for improvement. His weakness progressed. His speech was slowed, he was no longer able to stand or walk without assistance and he lost the ability to lift his arms. This was when sheer panic set in (for all of us – including John) and we bolted to St. Jude.
Thursday morning/afternoon was organized chaos. John experienced RAPID ascending paralysis and was immediately admitted to ICU (Martha May was literally running while pushing John’s hospital bed to the ICU). Most of the day was spent assessing his condition and giving him medical support to stabilize his deteriorating status.
Thursday night was one of the hardest days of this journey – John’s breathing was increasingly labored (paralysis was effecting his diaphragm) and he was suffering terribly. I held his hand tightly and we had a brief heart to heart (I poured my heart out and said everything I’ve every wanted to say to him incase it was the last time I spoke to him) then the ICU Intensivist and his team rushed in to intubate. We stood outside the room and sobbed. Bella, who has been very strong during this journey, finally became overwhelmed with emotion and wept inconsolably – both of my children were suffering and I felt helpless, heartbroken and terrified. After John was stable and comfortable we were allowed back in the room (he is on a Dilauidid pump for pain and Versed pump for sedation). I couldn’t get to him fast enough – all I wanted to do was hug him but I couldn’t, he was covered in tubes, so I kissed and held his sweet hand. Loving Bella, once again, didn’t shy away in fear, she put her hand on John’s head and told him he was ok and she was going to spend the night next to him (as she always does). Rich was quiet – he stood at the foot of the bed, looking at John on life support, in total disbelief.
The day started early – we attended “rounds” with doctors from all disciplines: ICU, Leukemia, Transplant, Neurology, Infectious Disease, Respiratory, Pharmacy, Nutrition etc- all hands were on deck! Pui wanted swift action and he made it very clear he wanted answers. John had an MRI of the spine & brain (checking for lesions, and/or inflammation), Lumbar Punction (checking for pressure, protein in the spinal fluid, bacteria, viruses, fungus and Leukemia) Bone Marrow Aspirate (checking for Leukemia) and labs (checking for everything imaginable – truly everything!).
Although John was very sedated, he had moments of alertness and was able to cry and move his head slightly in response to questions. We tried to only ask questions that would aid in a diagnosis – he was VERY anxious & aware of what was happening to his body.
Feb 22nd – 26th
Results from his Cerebral Spinal Fluid and Blood cultures started to stream in. His spinal fluid had high levels of protein (consistent with Guillain-Barre Syndrome), high levels of white blood cells (consistent with infection although all cultures showed no bacterial or fungal infections) and negative for Leukemia. Blood cultures were all also negative for bacterial infections and Leukemia (MRD 0.000%). Also, Neurology performed a Nerve Conduction Study, the finding were consistent with Guillain-Barre Syndrome. Given that information, his clinical status & his CNS results, a diagnosis of Guillain-Barre Syndrome (GBS) was established. GBS is a rare disorder in which your body's immune system attacks its own nerves. The exact cause of GBS is unknown. But it is often preceded by an infection or illness. John’s doctors feel, in John’s case, it may be due to post transplant immune-reconstitution, especially since he had VERY robust engraftment after his Splenectomy). There is no known cure for Guillain-Barre syndrome, but several treatments can ease symptoms and reduce the duration of the illness (high dose IVIG treatments and Plasmapheresis). Most people recover from GBS, though some may experience lingering effects. John is, once again, a unique patient in that his body is already compromised from years of therapy and his stem cell transplant so, no one is speculating on his recovery or prognosis.
A five-day course of high dose IVIG treatment began. IVIG is an immunoglobulin containing healthy antibodies pooled from over one thousand donors. The theory is that the high dose of immunoglobulin would block the damaging antibodies that may contribute to GBS.
At this point we were hoping to see, at the very least, his symptoms plateau but instead we saw a steady decline in his autonomic nervous system functions (most notably in his cardiac regulation). We also noticed more significant paralysis in his face. After another neurological exam in the afternoon, performed daily by our new friend, Dr. Khan, it was decided we would proceed with Plasmapheresis (five plasma exchanges – one every other day). During this process the liquid portion of John’s blood (plasma) is removed and separated from his blood cells. The blood cells are then put back into his body. Plasmapheresis is thought to help BGS by ridding the plasma of certain antibodies that contribute to the immune system's attack on the peripheral nerves.
John was quickly brought down to Interventional Radiology where our old friend, Dr. Gold, placed a Central Venous Line in his neck (for apheresis) as well as an arterial line in his wrist (used to monitor more closely and accurately his BP). By the time we rolled back (late evening) the apheresis team was in place and ready to begin. (only at The Jude could this be arranged so quickly)
Since John is now a high risk for a “cardiac event” they began with only a half plasma volume treatment. During the exchange John’s blood pressure and pulse continued to skyrocket then plummet nevertheless, his vitals were managed very well.
At this point our only form of communication with John was with his eyes (opening his eyes if the answer to our question is yes etc.) he was no longer able to move his head or neck. He does, however, continue to shed tears. We continued to spend most of our time with him just talking, reassuring him that he is safe and informing him that a large team of doctors and nursing are working tirelessly to help him.
We started the morning with Plasma Exchange #2 – this time it was total plasma volume. It was an effort keeping John’s vitals stable during and after the procedure but the apheresis was accomplished as planned.
John no longer responded to us. His eyes opened and closed as a reflex only. When they were open, they were glazed and looked right through us. Tears, however, would roll down his face without explanation.
Given John’s gaze, Neurology ordered a lumbar puncture to re-access his cerebral spinal pressure and protein levels. Sweet Martha came in on a Sunday to perform the procedure (again, only at St. Jude). The results showed an exponential increase in protein (consistent with GBS) and an increase in cranial pressure.
He is somewhat more responsive, nodding slightly when asked a question, crying when in pain or anxious but overall still completely paralyzed.
I can’t end this post without giving a very special thank you to the STJ staff attending to John’s every medical and emotional need. Dr. Pui (unwavering – he is and always has been about treating the child not the disease) Martha May (faithful and loyal – taking it one step further by treating our family not John’s disease), the ICU staff (rockstars – continuity of care has been seamless), the nurses & doctors from Leukemia (as always very compassionate), the Neurologist, Dr. Khan (while new to the Hagen journey, devoted to John’s care), the BMT staff (just the 4 who like us J - invested and genuinely concerned), Psychology (consistently making sure our mental health is in check!), Interventional Radiology and Imaging (completely accommodating to John’s urgent needs), line nurse (gently caring for John’s central lines even though the nurses up here in ICU are capable), food services (bringing Bella a milkshake every day even though she’s not the patient), the many others at STJ who stop to hug us and tell us our family is in their thoughts and prayers and last but not least our family and friends who carry our family in their hearts.
John is in critical condition. His life remains entirely dependent of life support.
How does that look?
Scary – he has a tube coming from his mouth connected to a stand alone ventilator bedside, a double lumen central line coming from his chest, a double lumen central line coming from his neck, an arterial line in his wrist, a catheter and a pole with 8 chambers and 3 CADD infusion pumps attached. His face is swollen but his body is thin and his muscles are atrophied.
What does it mean?
We are uncertain. He is receiving all known treatments and supportive care. We are letting John’s body and his doctors guide us (read between the lines).
How are we?
We are terrified and heartbroken. We aren’t ready to let go but our hearts are heavy seeing John suffer.
What do we need?
Please join us in praying for healing and peace – in heaven or on Earth.