News About Alex - Excerpts From A Mother's Journal
- POST TRANSPLANT UPDATE - 24 October 2012
- RETRANSPLANT - 2012
- Mom and Alex
- Father Mike Visits Alex
- Christmas Week 2010
- Alexander's Birthday Week
- Relocating to Cincinnati
- Won't Take No for An Answer
- The Transplant
- Enter GVHD
- Alex's Strength
- First Weeks
POST TRANSPLANT UPDATE - 24 October 2012
This ain’t my first rodeo.
Actually, I have never been, just capitalizing on the expression. But more apropos, this is not our first bone marrow transplant (BMT), but God willing, our last.
New transplant, new rules and although Alex entered into the second BMT with an extensive problem list, I was hopeful that it would treat all the preceding problems, and cure him of his primary disease…I was wrong…partially wrong. The chemo prep was hard on Alex…daily injections as well as potent IVs. It took only days to see the effects.
Although eager to receive the second transplant, I was apprehensive. Those who have followed along recall Alex’s stroke, seizures, liver troubles, infections, horribly cruel and lengthy never ending bout of GVHD of the skin, and all the other problems that seem so trivial now.
Because Alex’s GVH was so dreadful, it was the only side effect that consumed us, but our fears escalated to unthinkable heights as just days post transplant, our Alex was fighting for his life.
One day following transplant, I was called into a meeting about Alex needing a liver transplant. Just days later, my son, my Alexander, whom was smiling up to the day of transplant, was in sudden acute respiratory failure.
I begged for my son to be treated in the PICU despite knowing what could potentially happen, but I also knew that if immediate action was not taken, there would be no choice for Alex.
Alex was enormous, full of fluid that backed into his lungs, heart and skin. Alex’s organs shut down…he was in multisysytem organ failure and required immediate life support. He was taken finally taken to the PICU days later.
The PICU was where nearly all bone marrow transplant patients went to die, just before a birthday...and Alex had 2 weeks until turning 3 y/o.
I did not need statistics, I had fact, I had real life fact. I have seen plenty in the 3 years at Cincinnati Children’s Hospital, and have seen far too many to lose their battle.
Every day I sat with baited breath and praying that Alex would make it to 3 years old...and if he could make it to 3, he could make it to 4.
My Alex, who I left days before smiling at me, was unrecognizable. My Alex, bruised from lack of having platelets and adequate blood count, with eyes swollen shut, with a breathing tube down his throat and medically paralyzed, was now requiring immediate and continuous dialysis.
The drugs that we needed to save his life, were on shortage, and the meds that we needed to keep him viable, were not available to us.
I was called several times to his bedside and advised not to come alone. I was instructed to bring family, friends, and our priest.
Then Alex began to recover, and dialysis was on hold as the drug we needed became available. But then as the drug disappeared, so did Alex, and he began to fail and then fail and then fail.
But Alex is no failure…
Alex’s lungs were completely whited out, full of fluid. That fluid soon turned to cement and took multiple bronchoscopies to clear out a fraction of what Alex needed to breath. Alex’s lungs had little room to expand due to all the fluid and the vast size of his liver and abdomen.
Dialysis was inevitable, and Alex was having surgeries and procedures at all hours. I arrived just after a dialysis access was achieved via surgery, only to witness the overwhelming work involved in initiating continuous dialysis. Alex lay there, swollen and beaten from surgeries and procedures, unable to be held as lines from 15 IV poles draped his body and IV access in his neck, chest, arms, wrists, hands, groin, and the back of his knee with tubes is his nose and mouth. The room was overcome by a ventilator, dialysis machine, monitors, and crash cart. Nephrologists, cardiologists, ICU attending, residents, respiratory therapists and several nurses were on hand…and I thought, “That is not my Alexander.” But it was.
How does one hope for the best and prepare for the worst? Especially after witnessing such a traumatic event? I offered Alex the only reassurance I could, holding back tears, but turned away to let myself grieve…yes, I was grieving although my son was still alive.
The dialysis began working, the treatments were effective, the medications were available. Every 72 hours the process of changing the dialysis tubing had to be repeated again with the orchestrated cast of professionals to ensure Alex did not encounter problems.
Slowly, Alex improved, but with new threats…drug addiction, poor line access, and infections.
All of Alex’s IV access was corrupted by infection, and quickly became useless. Surgery, once again emergent, was left to the hands of the surgeon on call that day. Misfortune would have it, all of Alex’s primary surgeons were at the annual conference.
Left with no choice, I sent Alex to surgery for him to return with an enormous crater in his chest, from where the port was removed. It was the proper decision as the infected port was liquified and surrounded with pus. But the technique was questionable.
Alex was unable to move his right arm, and had a suture line that would envy Frankenstein, from his ear to his nipple, with a huge gaping hole that peered into his tissue, fat, and muscle.
All lines were removed, and replaced with a double line to the back of his knee.Thanks God for heavy sedation and continuous paralytics.
But in all this, Alex’s transplant was taking. It was actually working. Although Alex required blood and platelet transfusions multiple times daily, his immune system began to recover. In fact, it was his transplant that took too quickly that attributed to most of this mayhem. Unfortunately, with recovery comes oddities…bizarre infections, septic emboli, blood blisters throughout, and so forth.
Nonetheless, Alex is fighting his way back as his kidneys are healing and no longer requires dialysis. After nearly 3 months in the PICU, Alex was finally able to breathe on his own and was weaned off the ventilator. His heart and lungs are improving and his transplant is still 100%.
Yes, there are problems, but are getting through them. From all the sedation, Alex became heavily addicted to his pain medication and paralytics and withdrawing is nothing anyone should have to observe, especially in their baby.
With the help of Ativan and Methodone, Alex is improving.
I have yet to see my son smile, and laugh as unsophisticated as it was, it was nonetheless intoxicating.
But today, I do share with you that our Alex was finally well enough to be transferred out of the PICU and return to BMT (Bone Marrow Transplant).
Alex has overcome far too much in his first three years of his life. Alex turned three, while in the PICU, and has been in the hospital since he was 6 weeks old. In those three years he has already overcome a lifetime of challenges. I can see nothing but good things to come if we can just get him home, and God willing, it will be soon.
Alex has had over 4,500 medical events in the last 36 months, including:
77 Surgeries (yes, 77) 83
195 Hospital admissions
193 Unexpected hospital admissions
375 Inpatient stays (hospitalized days) 425
15 Bone Marrow Aspirations
55 Rounds of chemo
330 Clinic visits
3 Episodes of complete hair loss
5 Times placed on 24/7 pain pump
2 Drug withdrawal complications
7 Central lines placed in his veins, into his heart
4 Line removals (not counting the emergency procedures last week)
1 Port inserted
1 Port removed
500 Medically defined "obstacles" to growth and development 600
115 Days and counting on TPN (liquid food fed directly into his veins)
500+ Blood / platelet transfusions
100+ Days in PICU
RETRANSPLANT - 2012
Alex is scheduled for his second major bone marrow transplant (BMT). Initially set for August 7th, the transplant has been delayed due to cell flight arrival. (The cells are being flown in from an undisclosed area – to protect donors identity.) Nonetheless, I would like to thank Alex's donor, a 23 year old male, for giving us that second chance.
Alex's BMT is scheduled for Wednesday, the 8th of August, 2012 at 1100 EST and I ask that you all join together with us and say a prayer for a successful and uneventful transplant.
Alex started this retransplant process several months ago, after learning the initial transplant and subsequent transplant boosts – the mini-transplants – failed. Not only did they fail and cause Alex a number of complications, including liver disease, stroke, and seizures...the transplant failed in the worst way possible. Exactly two years post transplant, to the day, Alex’s HLH (Hemophagocytic Lymphohistiocytosis) returned. My Alexander relapsed.
Adding insult to injury, over these past few months Alex has had many setbacks. Alex suffered infection after infection requiring him to be airlifted between insitutions. A severe oral infection compromised his life resulting in the extraction of four teeth...all on top. I was unaware that day was going to be the last time I would see that toothy grin...at least for a few years.
Alex continues to encounter setbacks as he tries so hard to advance and reach milestones. Approaching his third birthday, and still unable to walk, Alex started to take a few steps independently...until his body began to fracture. Alex suffers from osteoporosis and arthritis from the combination of high dose steroids and the weight of his body.
Alex is recovering from a fractured rib, a displaced hip, and fractured vertebrae (a broken back) and that is only what we know. Alex is unable to be casted and therefore remains extremely fragile. I cannot even hug my son, my Alexander, or give him basic care without bruising him or breaking his little bones.
Alex is down with OPP – other people’s platelets. Blood and platelet transfusions have been frequent since February, which is when the debilitating nose bleeds began, and continue – and as the chemotherapy and phagocytes (that are responsible for causing HLH) eat them up. Alex bruises from the slightest touch, and yet he still tries to dance, crawl, and play.
Multiple tests and surgeries (day after day) were performed in preparation for transplant, including placement of a new central line. Even with the port still embedded in Alex's chest, more access was needed to administer all of Alex's medications...and there are many. Placing that central line almost proved to be fatal, as Alex lost a tremendous amount of blood post op.
Preparation for transplant continued to be difficult as Alex underwent chemotherapy with different types of chemo given both as injections and via IV. Due to chemo, Alex's immune system is now nonexistent. This will give Alex's new bone marrow the opportunity to generate and eliminate those damaging phagocytes.
Unfortunately, chemo has also compromised Alex's nutritional status and healing and Alex now needs replacement therapies (tube feeds, electrolyte replacements, IVs, TPN). The chemo has already compromised his skin causing skin tears and ulcerations requiring hourly diaper changes and wound care. It is painful for Alex...and when he cries, I cry.
But Alex, as Alex, recovers quickly...
But what is not recovering quickly, or at all, is Alex's liver. After nearly two years Alex remains jaundice with glowing yellow eyes and darkened skin, and occasional bleeding/clotting problems. Alex's liver (and spleen) is large and hard and extends into his pelvis.
Regardless of the cause of the liver damage, be it HLH or BMT, Alex is being evaluated for a liver transplant as his liver is compromised. Barely able to grasp having a second BMT, we will be meeting with the Liver Team (doctors, surgeons, psychiatrist, financial advisors and social worker) one day after Alex's BMT to discuss and plan. A preemptive approach, but one that puts me ill at ease at the thought of Alex needing a liver.
There are so many setbacks, problems, frustrations, misery, and heartache that we have been through..and many I simply do not want to recall. But note that although this has been a tremendous challenge, Alex keeps fighting and smiling...so I am going to do the same.
That's our Alex, and God Bless him.
Mother of Alexander
Mom and Alex
I never knew the meaning of tragic until my infant son, Alexander, was diagnosed with such a rare and devastating condition. Our family, especially his sisters, Arielle and Alyssa, ask for your prayers and help in fighting Hemophagocytic Lymphohistiocytosis (HLH), a fatal disease that attacks the organs and tissues.
It has been well over a year since Alexander has been diagnosed with HLH.<br/>
As of December 2010, we have been anticipating better news since the transplant, which occured months ago.
Discharge to Chicago was indefinitely suspended. Alex had a suspicious seizure that prolonged
hospitalization and his GVHD (graft vs host disease) can never seem to stay under control. The
complications upon complications are tormenting.
As of today, it is confirmed that my Alex has permanent brain damage. The stroke has affected him and triggered seizures and has left him with visual impairments and left sided physical deficits. Although it is irrevesible, I am hoping other parts of his young brain will compensate. Time will tell, and as you know...prayer is powerful.
I am actually at a loss for words. It just sunk in about Alex's condition.
Father Mike Visits Alex
Before I begin I would like to offer thoughts and prayers to the children and the families of those children who passed away since my last update. Seven children, suffering from the same insidious disease as Alexander, could no longer sustain the fight and succumbed to HLH. Our hearts and prayers are with them all.
I would also like to thank Father Mike, on behalf of Alexander and his family, for taking the time to visit Alex.
Father Mike and Alex were definitely in sync that day. Alex was on a pass from the hospital that allowed Alex to visit Arielle, who just came to town for a visit, at the Ronald McDonald House. Just as Alex got settled in, Father Mike appeared. Fortunately for everyone, Alex had just gotten a strong dose of oxycodone and nubain for his skin and was in very good spirits.
Father Mike and Alex had a pleasant visit. Alex was happy for the new face and the special attention. It was the perfect compliment to the luck of getting the day pass. Myriam and Gerald fight hard for Alex to get his day passes - they are few and far between. The goal of the day pass is to get Alex out of the hospital and away from the uncountable and insurmountable number of infectious elements present in every square foot of the hospital.
I have not updated anyone for quite some time on Alexander's health, mostly for lack of any new or good news.
As I write this Alex sits in pediatric intensive care, seemingly a world away from where he was just days ago with Father Mike, fighting an infection of unknown origin. Alex has an extremely high fever, likely brought on by what Alex's parents think is a staph infection that spread from something or someone in the hospital, to his skin and then spread to other parts of his body. With Alex's skin constantly peeling, breaking, bleeding and flaking off, there is no question his skin is a major entry point for infection, and why Myriam and Gerald try so hard for those day passes. This one did not come quickly enough to prevent this latest infectious challenge.
A few quick notes on what has transpired since the last update.
At last update Alex started to spike fevers, two or three times per day. Those "spikes" later developed into a steady high fever. Fevers are one of our worst nightmares as they are one of the principle markers for the presence and return of HLH.
Alex then became lethargic, would not hold up his arms, would not participate in therapy, did not want to have mom hold him and dance, did not want to sit in the jumper -- all things that were daily activities until a month ago -- he just buried his head in mom or dad's shoulder and did not move.
After aggressive coaxing by mother and father, and getting Arielle and Alyssa to visit for a couple of days, Alex started to come around. That last fever was put in check with multiple and continuous IVs of antibiotics and non-stop attention from mom and dad. At that time no tests were done to check for HLH. We're still holding our breath about that.
A few days after that feverish episode, the family noticed Alex would not let anyone touch his left leg. Our fear was that he had a broken leg or hip or back -- which is not that unusual for a BMT baby, nor anyone on large amounts of steroids and other anti-inflammatory medicine. Alex had his legs checked by x-ray and there were no visible fractures. We are still awaiting the ultrasound of his hips and feet and lower back.
This brings us to today.
As noted, Alex is being treated for an exceptionally high fever due to an infection they cannot control. Myriam and Gerald have asked for a specialist, an infectious disease doctor, to have a look at Alex before things become even more dire.
Alex has been nauseous and not able to hold down food, however, he had that PEG installed three months ago and he is being fed while he sleeps. We are hoping that the reason Alex cannot hold food during the day is simply because he is being over fed during the night. It will take a couple of days of experimenting to figure that out
Otherwise, we are still in the dark regarding the most important issues, which are controlling his GVHD and his engraftment level. Engraftment has not gone well at all. We are nearly two years out and Alex has no trending engraftment numbers that are positive. Until he is stable and engrafted at 25%, he will not be coming home.
I have been remiss in issuing updates because every day presents a new challenge -- by the time I draft an update on one incident, another has occurred. Plus, some people hate to hear about what is going on, others can't hear enough. I am at a loss as to what to present. In the interest of satisfying both camps, below is a bulleted update with the most recent information. If anyone would like to hear more about anything at all, just drop me an e-mail and I will be happy to respond; others can simply skip or scan what follows.
- One positive sign is that Alex's T-Cells are improving, but we have not checked those, nor engraftment, for nearly three weeks because Alex has been so sick. In the coming weeks, those tests will be priority.
- Alexander's nurse missed his port with the needle and ended up pushing the meds under his skin. Those meds are toxic and cause cellular necrosis -- Alex had an unknown amount pushed under his skin, which spread throughout and under the skin of his chest. Two nurses rushed in after being summoned, and gave Alex a dozen large injections of antidote to prevent his tissue from being consumed. Alex screamed in pain for about 17 hours before collapsing with exhaustion.
- Myriam drove out here last month to pick up Arielle and took her to Cinci for her birthday - she turned five at the end of the May. Arielle was very happy to be with her parents and brother for her special day, even though she had to come back for her "graduation" from pre-K.
- In June, Myriam returned for the girls and they were all out there for three weeks, and Alex could not have been happier.
- Sorry to say that Alex has been in the hospital for 22+ months, and is himself 23 months old. We are ever hopeful that Alex can come home soon.
- It is a very harsh environment for Alex and difficult for his parents. Only three children out of nearly 14, from around the world, have made it out of the hospital with allogenic transplants, like Alex has. It is a dire burden to bear, to come to know and love those other children, then to know they have moved on, to that place past life. These kids were Alex's "friends" whom would be wheeled past Alex's infusion room, only to pause and wave at Alex, who so happily waved back through the window with the wire mesh. The wire mesh was a good thing to have, because Alex would smile so proudly at his friends I think his smile could have shattered the glass, so happy he was to see other kids.
- We do not expect Alex to be "home" but we are ever prayerful that Alex can get transferred to the University of Chicago. Once there he will be allowed to spend weekends home, and perhaps one other day every other week. From there we'll take it one day at a time.
- Alex continues to fight staph infections, and gets them recurrently, each seemingly more serious than the last.
- Alex has a rash on his face and arms that does not fit any profile, and has therefore resisted treatment.
- Alex's liver function is noticeably impaired. Alex's liver has caused jaundice. The proper course would be to take a liver biopsy, but due to his other issues, he is not healthy enough to undergo that procedure.
- As Alex approaches two years old, he is the same height and weight as he was nearly a full year ago. The medications Alex is taking to GVHD also suppress growth, as do the immunosuppressants.
- Alex is now consuming approximately 40 medications per day, some multi-times per day, some via IV, some via PEG tube. Simply managing and administering his meds takes up to six hours per day. His nubain, for instance, must be administered every three hours, 24 hours per day, including over night. The nubain controls itching and were it not for the nubain, Alex would be in a never ending cycle of pain and itching over every square inch of his body.
- Alex has many moments of happiness with his family present, and tries to follow Pie in everything, right down to eating her Barbie doll heads and stealing her markers.
- What continues to make Alex happy are people and faces and expressions...not things. Alex would much rather interact with a lively person, as he did with Father Mike, than play with a toy or watch cartoons -- Alex is a people person!
- Alex so desperately wants to stand, but his weakened bones will not allow him that accomplishment.
- Alex is undergoing neurological testing to re evaluate his stroke and to try to figure out why he does not bear weight on his left leg, which they also thought may have been a fracture due to loss of bone density.
Myriam explains with no hesitation that Alex's family surely gets angry at Alex's lack of progress and constant setbacks, but she explains how they are consumed more by disappointment than anger...especially for the girls, whom miss Alex terribly so, and of course, Alex, whom we just want to be a healthy normal boy.
Despite this all, Gerald and Myriam, Alyssa and Arielle, can still see Alex shining through the sickness. Though in constant and sometimes protracted pain, Alex will gladly offer you his hands and wait for you to pick him up to dance around the room - by far his favorite activity. Despite his skin once again hardening, peeling, bleeding, and ultimately falling off, despite itching so badly that he scratched his scalp so deeply as to cause a major laceration requiring first aid, despite all of his hair falling out for the third time, despite consuming enough meds in a day to fill up a cereal bowl, despite daily events like that, Alex still will catch your eyes with his smile, will engage you in Alex conversation, will beg to be held up to the window, will marvel at seeing a car pass by on those rare days he can see daylight. Doctors, nurses and strangers alike often refer to Alex as "that Little Man", or the "Iron Man", or "Super Boy" -- due to the fact that at least until today, he has fought off all manner of infections, disease and immune related complications. But we sadly remind everyone that Alex is not a Super Boy, nor an Iron Man. Alex is a fragile little boy fighting every moment of his life to be around for the next moments of his life. Despite it all, Alex is still Alex and loves life as much as we love Alex. Alex is our mission, above all else.
Lastly, Alexander Maximus Hilton will be two years old in five weeks. I can no longer refer to the updates as updates on "Baby Alexander" -- from now on, he will be "just" Alexander.
If anyone wishes to send birthday greetings to Alex, old-fashioned birthday cards can be sent to the contact address on this web site, or you can simply drop Alex an e-mail, at firstname.lastname@example.org. Several people have expressed an interest in sending flowers or a stuffed animal. As much as Alex appreciates the kind thoughts, due to being a BMT boy he cannot be around live plants of any kind, including fresh fruits and vegetables, and cannot be exposed to any plush toys -- but do accept our sincere thanks for asking.
MERRY CHRISTMAS TO ALL, FROM ALEXANDER AND FAMILY
Merry Christmas and Happy Holiday's to all. Our family would like to take a moment to wish you all a Holiday Season of Peace and Joy. Through adversity and strife, we will surely find our way.
As some of you know, I had been predicting that Alex would be transferred to a local, Chicago-area hospital in the first week of December. I had the occasion to listen in to one of Alex's checkups several weeks ago and I heard a boy whose voice rang through my phone with such clarity and happiness that I was for the first time filled with joy and anticipation - certain that Alex was but days from discharge.
Before I tell you any more, I will tell you now, Alex's discharge is not an option.
Three weeks ago Alex's parents, Myriam and Gerald, were told to get ready for a soon to be ordered discharge, first to the Ronald McDonald House, where the parents stay while in Cincinnati, then to a Chicago area hospital, where treatment would continue. The idea was that Alex would be discharged to the Ronald McDonald House for a few weeks - he would stay nights at the McDonald House, and come every day to "day hospital," where he would receive his usual six to eight hours of drug infusions and several hours of therapy, but then have the opportunity to sleep with his parents every evening, and all weekend.
Alex was actually discharged to RMH three days ago. Alex's eyes were as wide as saucers, as he peaked and peered at every little thing - his first time outside of the hospital for well over a year. He babbled on and on for hours, no doubt trying to ask his parents what all these new and wonderful things were that he was seeing for the first time. Mom and dad were grinning ear to ear as their boy took his place on his big-boy bed at RMH.
Yesterday Myriam called to tell me that at his second visit to day hospital, less than 48 hours after discharge, Alex was back as an in-patient, back in his 10 x 12 room, back to the white walls and cold stainless steel bed rails. While in clinic, Alex appeared to have another neural episode, a stroke, a seizure - God only knows what or why.
Yesterday, after getting his eight hours of drug infusions, which he gets every other day - Alex was carted off for an MRI, an EEG and his ninth spinal tap - to again test to see if the HLH has returned. Alex's skin also took a turn for the worse, blistering only hours after his last meds. As of today, Alex is still in the hospital, awaiting the results of his MRI and spinal tap.
But that is not even the part of the story that pains me most. It is this, that swells my eyes with tears. After 13+ months in the hospital, and nearly six months post bone marrow transplant, Alex's transplant has not yet engrafted, it has not yet "taken" and Alex's body is still in a state of rejection. Without the transplant engrafting, Alex is in jeopardy of the HLH returning - until the engraftment is stable and at least at 20% (it is currently at 12%) Alex cannot come home. As long as his skin, via the graft versus host disease, is not healed, he cannot be discharged - not to RMH, not to a Chicago area hospital, and not home.
The family and I all agreed, including four year old Arielle, that the only present we needed this year, the only thing that would make it okay that there was no money for a tree, no money for presents and no money for Christmas dinner - we all agreed that Alex coming anywhere closer to home, even to the Ronald McDonald House, was the only gift we could possibly hope to receive.
With that gift not in Santa's bag, Alex's mother and family have asked you all to pray for Alex, pray for his health, his will and that God's love will see him through and bring him home.
Your prayers and kind words have helped the family through so much, and Alex has never been better than on those days following your collective prayers.
I will ask you all to pray for Alex once again, to please grant Alex your Holiday prayers - the gift that we happily return in favor, as we all pray for those whom support Alex and his tribulations, and we ask you all, on Alex's behalf, to pray for all those less fortunate than us, those without support, without family, or whose health is failing - including all those on this update list whom we may never meet.
Let's join hands by joining our hearts and minds, and pray, yet again, for health and good fortune for Alex, and all those like him.
HAPPY BIRTHDAY ALEX ! ! !
Alex was admitted to the BMT ward the day after his birthday for what they said was to "keep an eye" on his GVHD. However, I suspect the drug Alex was given to prevent him from catching the virus that had an outbreak in his ward, whether contaminated or not, led to his poor health and to the admission.
The drug is known to cause hypotension, nausea and vomiting - similar to septic shock.
During the stay, the "keeping an eye" was of no help to Alex, as he had a third and very significant flare of the GVHD. For days and days he was in constant pain, crying, unable to eat, and nasty sores erupting all over his body. We had to start him back on the morphone pain pump, but it did little to help and he is still miserable.
While GVHD was being treated, now with new and different drugs, which cause new and different side effects, he developed hemolytic anemia and required nearly daily transfusions of blood and platelets. Alex developed antibodies to his donor's blood, as they are two different types, so that issue also had to be treated. Basically he was rejecting the blood that was being created in his bone marrow by the transplanted cells.
A spinal tap revealed that Alex had a relapse of the original HLH disease and this time it was in his cerebral spinal fluid. The HLH attacked his brain and latched onto the right rear hemisphere. The resulting infection of his brain caused lesions on his brain, which in turn caused Alex to have a stroke at 13 months old.
Alex's stroke resulted in a physical deficit (not able to use his left side), and recurrent seizures, noted by bizarre eye movements.
The HLH not only returned, but to a place that was previously disease free. And since it was in his brain and spine, he required weekly infusions of a potent chemo drug under anesthesia and in the OR. The infusions were administered via lumbar puncture, three times per week. Because of anesthesia, Alex was intubated. With the multiple intubations, he lost the ability to make sounds and eat.
Medications and side effects continue to be issues, as they send him into lethargy and compromise his cardiac status.
He developed HHV6, a type of herpes virus, that too affected his brain and liver causing hepatitis (clinical sense). His liver numbers as well as the viral numbers were astronomically high and this required Alex to start to take a potent antiviral that again affected his bone marrow (suppressed the engraftment of the donor cells.) By disturbing the transplant and engraftment process, Alex's recovery can be permanently impacted, closing the window to engraftment and requiring a second transplant - thankfully we are not yet ready to call the transplant a failure.
Alex is recovering from the stroke and HHV6, but still has pain and remains on pain pump. Fighting another bug, not sure what it is, and his skin remains an issue - flaring up into blisters and sores with no warnings or symptoms, and within seemingly minutes of looking fine.
Alex remains highly immuno compromised and is highly susceptible to everything out there.
Last check, transplant numbers were at 17% - he cannot be considered for discharge to Chicago Heights with numbers below 23%. We are hoping the sorted engraftment studies show other improvement that can get Alex home.
He has now been in an 8 x 12 foot hospital room for 13 straight months.
Thanks to everyone for keeping Alex in their prayers.
JUNE 2010 - RELOCATING TO CINCINNATI
We temporarily relocated to Cincinnati for Alex’s life saving treatment and care of his HLH, and have left behind Alyssa and Arielle at home with relatives.
Since Alex was 2 months old, he has been battling this rare and fatal blood disease, and as part of his treatment, he underwent chemo. But to ultimately save his life, he would need a bone marrow transplant (BMT).
There was no match in the immediate family – we had to search the National Donor Marrow Registry, and waited for an answer with baited breath.
The difficult task of finding a donor was complicated by the limited number of ethnically diverse donors. Alex was very fortunate to find a donor, until we learned that she changed her mind.
As we waited, Alex underwent more invasive testing and four operations for placement of a central line to his chest. He was crying, always in pain, rarely slept. Alex’s HLH was advancing – then we recieved word that the chemo was ineffective. Simply put… he was dying.
My son was dying and the person whom could save him simply changed her mind.
My heart was broken and I was too stunned to cry. I suppose I was angry, but I had to believe that everything happens for a reason; but I wanted to know that reason.
News arrived of a second donor, but she too was not able to be found.
At this point I am distraught as not one, but two donors failed me, but still hopeful. But I wondered if I should bury him in the little white satin Christening suit he wore just weeks ago. No.
That life saving transplant came by way of a 31 year-old man, whom without hesitation so generously gave my son that second chance. And I am eternally grateful to him for ending that hurt and that stage of uncertainty. He will never know what he has done for me. And so it seems, third time’s the charm.
Alex received a two week prep of harsh chemo drugs that wiped out his immune system; he had zero immunity. The drugs ate away ate his mouth and nose and stomach. He was started on a morphine drip and all this before the actual transplant.
Alex lost the rest of his beautiful black hair and took on another form. Swollen from meds, Alex could hardly keep his head up from the weight of his face. He tried…he always tried. My arms nearly collapsed under his weight.
He was still in pain and fentanyl was added for pain management.
Despite the pain from mouth sores, he still preferred to try his bottle than receive nutrition from a tube. The tube (NG/NJ) inserted into his nose, irritated more than his nose and throat, but his spirit. Rather than assist with feedings, it caused him to vomit throughout the day, and paralyzed his vocal cords…he could no longer make sounds, and I was speechless.
To date, Alex has had 5 NG/NJs, all of which he successfully removed. He is working to regain his voice. Slowly but surely.
The cells arrived and was a rather ordinary day. It infused like a bag of blood, but with an odd offensive odor, but a smell I have come to love. The staff believes it smells like cream corn…frankly, I have no reference point.
After four hours, the transplant was complete. That was it. A few days later the screaming returned.
As the cells grow and differentiate into bone marrow, the effects are powerful. It is intense and painful and mimics shin splints…shin splints throughout Alex’s tiny body. As I watched my baby boy vomit 20 times a day, stop eating, and communicating, I wondered if transplant was the best option. As told time again, death was the other option and dying is not an option. I would come to question that decision time and again.
At this point, Alex is on 33 medications, that I give him, and I have just about lost my mind.
One month post transplant Alex was doing well. His voice was returning, although the grunting was difficult to interpret. The pain was under control, and Alex was happy. However, one month and one week later, he was not.
I was unprepared to deal with such nasty complications that created more uncertainties and heart aches. I was on the verge, once again, of losing my son. To date, I do not know whether that feeling will ever go away.
We have gone from one life threatening situation to another. Alex suffers with GVHD (graft vs host Disease). GVHD occurs when the engrafted donor cells attack the patient's organs and tissues – and it has grossly attacked my son.
Alex is dying again. I do not know if I can ever get over this, because it is still occurring. For Alex to survive this, he will need high engraftment levels. But as engraftment increase, his GVHD worsens.
My Alex, my poor Alex cried incessantly from the pain of his ripping skin. He blistered violently, he wept terribly, and he bled from his eyes, his nose, his mouth, his fingers.
The skin was so taught and mimicked scleroderma…and nearly severed his left arm and fingers. His skin was so tight; he could not close his eyes or mouth. Alex was contracted at the knees, and would not bend his arms. He kept them straight out rather than try to bend; it was too painful, and his skin would rip.
He looked like a severe burn victim. It was beyond severe. It was near fatal.
Treating GVHD would compromise his transplant, but not treating it compromised his life.
It was decided to aggressively treat him. HLH was a threat once again. If HLH does not take my son’s life, the transplant will; and that is an awful place to be, but this is where I sit.
As I pray for this delicate balance, my Alex suffers with uncontrollable pain and misery. Worse yet, he has lost his spirit. He was not my Alex and he has not smiled in over a month.
I can no longer trust my emotions as they often betray me and taking one day at a time is a luxury I seldom have.
I do not own them…these emotions. When Alex is happy, so am I. When he hurts, I hurt too. At times I can barely breathe.
Alex and I share secrets, as I cannot hide my emotions from him.
It was during a moment of despair that I could no longer contain my tears. I sat rocking him, confined to that uncomfortable chair, to ease what pain meds could not.
My child was dying and I cried, baptizing him with my tears. I felt something touch my lips, and it was Alex, offering me his pacifier, “No worries mom…I’ll be OK.”
Stunned, but content, I accepted. The pacifier did little for me, but his actions spoke volumes; he offered me hope, solace, and strength.
Alex had done that to me before. At 2 months old, he was intubated and suffering with multisystem organ failure. He was to die that night, but instead raised his right hand as I sat quietly in the dark looking for life. I have that image forever imbedded in my memory (and on this website.)
He kept his promise, and I know that this will be no different.
Days passed, and as he gathered more strength, I did too. With closed fists, but with more mobility, Alex sat on my lap and distracted my tearful episode. He compassionately stroked my arm, just as I have done to ease his pains countless times.
I told Alex that I was not crying because I hurt, but because my heart hurts. He placed his head on my chest… and for a moment the pain went away.
But the pain never completely vanishes. These are my rare moments, but truth be told, I often feel like I am falling apart.
I think back to all the invasive and painful procedures, and surgeries that went awry, and medications that simply did not agree with Alex. I think of when the complications and the endless nights will be a memory. And yes, I wonder if I made the right decision.
Alex has been hospitalized 6 times with lengthy and complicated stays by the time he was 8 months old.
100 days post transplant, and we are still here.
He is very vulnerable and an infection of any sorts can end his life. His medications are somewhat disfiguring, but hopefully temporary.
Alex continues to suffer with GVHD and still searching for that delicate balance.
I remain hopeful, but there are days that I am less optimistic.
Today is not that day.
As for Alex’s hospitalization and this process…it can be considered as being put on “Death row.”
Alex and the rest of these children are given death sentences.
What crime have these children committed? Faulty genetics and mutated genes are to blame for such misery.
The treatments are harsh, disfiguring, debilitating, and yes, fatal.
We rotate rooms every 30 days and have no exposure to the outside. Sunlight poses too great a risk to his frail condition. I am a prisoner to my son, and he a prisoner to this is disease. Alex and I share confined space. What makes it tolerable…simply put, I love my son and by the Grace of God, he will be healed.
I am surviving on the kindness of others and the prayers of many. I will go broke trying to save my son’s life, but thanks to family and friends, Early Childhood preschool, Infant Jesus of Prague and fellow parishioners, St James Hospital and coworkers, countless others, and especially the Mulliganeers, it eases the current financial burden.
You all have afforded me the opportunity to be at my son’s bedside and care for my girls, even at such a distance. And to the 31 yr old man who donated his bone marrow, I am eternally grateful.
Alex is still very ill and the medical team is still searching for ways to treat his complications. The medications are costly and at 9 months, Alex has met and exceeded his lifetime insurance cap.
I never knew the meaning of tragic until my infant son, Alexander, was diagnosed with such a rare and devastating condition. Our family, especially his sisters, Arielle and Alyssa, ask for your prayers and help in fighting Hemophagocytic Lymphohistiocytosis (HLH), a fatal disease that attacks the organs and tissues.
HLH has ravaged Alex’s little body causing high fevers, central nervous system breakdown, cellular breakdown of his blood and bones, digestive, muscular, and visual problems, and multisystem organ failure resulting in intense pain and constant crying.
It was a difficult and near tragic five weeks in the pediatric intensive care unit, but Alex is now home. He is improving, but still very ill, not eating much, and in constant pain. His condition is further complicated by the negative effects of his treatment and requires around the clock medication and care.
Alex undergoes weekly chemotherapy to help manage his HLH, but to cure him and ultimately save his life, he will need a bone marrow or cord blood transplant. His sisters unfortunately are not a match, and need your help and prayers to find a donor match in order to save my Alex.
We are running out of options, and still have a long way to go and ask that you all continue to help me save “our” Alex.
Mother of Alexander