Jacob Treebeard Brown was born on August 18, 2003, his Daddy’s 30^th birthday.  And what a gift.  He was a fighter from the moment he sucked oxygen, bellowing through initial attempts at breastfeeding with a winsome enthusiasm.  Our little man was baptized at St. John’s Episcopal Church six weeks later.  Though Pastor John performed the sacrament for four children that morning, as he held our son he paused for a quiet moment, then said that that this boy Jacob Treebeard, this one was special.  We glowed in a Hallmark sort of way and didn’t even begin to fathom how true these words would prove to be. 

Jacob entered the hospital the week after the ceremony for a different sort of baptism.  From that day to the present, he’s been on an incredible journey – from Trumbull Memorial Hospital to Tod Children’s Hospital, a brief respite at home, back to Tod’s, and finally to Cincinnati Children’s Hospital Medical Center.  Along the road there’s been infection in his urinary tract and bloodstream, spinal meningitis, PCP pneumonia.   IV lines in both arms, IV lines in both legs, an IV line in his head.  Feeding tubes in his stomach, feeding tubes in his intestine.  CAT scans, PICC lines, reflux studies, lung studies, bone scans, bone marrow extracts, brain scans, ultrasounds, oxygen masks, X-rays, bloodwork. Failed IV lines, failed studies, allergic reactions, chemotaxis problems, unscheduled nights in Intensive Care.  During these days the doctors nodded when asked if Jacob’s life was in danger.

But we said his verse over him every night and he continued to fight bravely.  Little by little he got better.  Completely better.  January 9, 2004 was his Homecoming Night and everyone hoped that the adventure was over, that the “happily ever after” part would now commence.  The short-term medical game plan was to continue IVIG therapy so that he would be protected by the combined immune systems of more than 40,000 blood donors.   Meanwhile, doctors would continue their investigations as to the root cause of Jacob’s early infections but the voiced hope was that these investigations would be fruitless.  Every three weeks, the family made the five-hour trek to Cincinnati.  Eventually, it seemed these trips too would end.  However, on his last scheduled visit, doctors arrived with news. They had sent Jacob’s DNA to a laboratory in France and had reached a definitive diagnosis. 

Jacob has a genetic condition called Anhidrotic Ectodermal Dysplasia with Immunodeficiency.  Sometimes it’s called NEMO because this is the name of the protein that doesn’t function correctly.  The condition was discovered just recently; the doctors estimate that there have been fewer than forty cases ever seen in the world, and only a handful of these children are still living.  Usually, mothers of affected children miscarry.  The condition affects hair, teeth, and sweat glands, but the most dangerous part is the immunodeficiency.  These children are extremely susceptible to the kinds of life-threatening infections Jacob experienced during the first months of life.

Since one can’t “outgrow” a genetic condition, we knew treatment would be a lifelong process involving daily prophylaxis antibiotics, IVIG infusions, and selective isolation. The team in Cincinnati was frank:  this was uncharted medical territory. They speculated that a bone marrow transplant might offer a potential cure for the immune system problems.  However, as one doctor put it “Bone marrow transplant is a terrible treatment for terrible diseases.”  Another doctor told us never to consider BMT unless there was no other hope offered.  Instead, a preventative treatment plan was put in place and we returned home, praying every day for Jacob’s continued health.

And family life at home was good.  In fact, as Jacob grew strong, Jodi grew back into her maternity clothes.  When we told our doctor at the National Institutes of Health that Jodi was pregnant, he exclaimed, “Oh no!”  There was a 50% chance that our second child would be born with the same genetic condition.  We had a different perspective and welcomed our “faith baby,” Justice Major River Brown, on April 27, 2005.  Genetic tests revealed that Justice was 100% healthy.

Life was sweet for a long season.

Then in July of 2006 Jacob developed a dime-sized sore on his leg. Because this looked curious, the cautious doctors ordered a biopsy. A few weeks later, a lab in Texas discovered that the biopsy had grown out a very rare, life-threatening fungal infection.  Doctors called Jodi at home and said to bring Jacob to Cincinnati immediately.  They wanted Brian at the bedside too; they were afraid that the infection may have spread to Jacob’s brain, lungs and other internal organs. Surgeons cut it out of his leg while we prayed and awaited test results.

Once again, God smiled on our boy.  All tests of the lungs and brain were negative.  The fungal infection had been isolated to one site.  However, this was a stark reminder of the bizarre unknown risks Jacob faced with NEMO.  The doctors were stunned.  This type of fungus is used to kill insects and has only been reported in humans three other times in the literature.  One doctor described the infection as a “sentinel event.”  Another spoke of “altered calculus.”  All said that the risks of waiting for another infection now outweighed the known hazards of a bone marrow transplant.  It was time to be proactive.

We met with Jacob’s Immunologist, Dr. Lisa Filipovich.  She told us that without a transplant, children with primary immune deficiencies like Jacob have an average life expectancy of ten.  But because he’s young and has never endured chemotherapy before, she gave Jacob a better than 70% chance at a successful transplant with no lifelong complications.  She used the word “cure.”  He could have his chance.  We said yes.

It will be a long and difficult journey.  We have taken professional leaves of absence and moved to Cincinnati for the next 8-12 months.  Aunt Rebecca turned down gainful employment to offer our family much-needed help.  As of October 23, Jacob began the “workup” phase of the transplantation process.  This involves bloodwork, multiple tests of the heart and kidneys, a full-body CAT Scan, etc.  Following this exhausting week, there will be a reprieve for gathering strength.  On November 17, Jacob is scheduled for surgery to insert his Central Line.  On November 24, he’ll be admitted to the BMT floor.  On the 25th, chemotherapy will begin to wipe out his own immune system.  The transplant day, Day Zero, will be December 5, 2006.  We’re told that many BMT recipients refer to Day Zero as their “2^nd Birthday” because of their new chance at a new life. 

At the outset however, this new life will be extraordinarily challenging.  He’ll be in the hospital in a double HEPA-filtered room from Day Zero through Day 30 at the least.  It will take two to three weeks for the new donor cells to engraft, during which time Jacob will have no immune system whatsoever. These are critical days since he’ll be extremely susceptible to any virus, bacteria, fungus, etc. and at the same time the side effects from chemotherapy will be at their worst.

Further, doctors worry about Graft-Versus-Host disease, a scenario in which Jacob’s body begins warring against the donor’s cells.  He’ll be on a host of medications but we’ve been told to expect “predictable bumps in the road.”

If all goes well, Jacob will be discharged sometime after Day 30 to spend nights at our Cincinnati apartment.  This phase lasts a minimum of 100 days.  Jacob and Justice must be separated, both an emotionally and logistically difficult proposition.  We will become nursing staff, administering IV medications and feedings, performing line cleanings, etc.  Our collective background in liberal arts promises to be entirely unhelpful.

But of course, this is the longwinded adult version.  With the wisdom of a child, Jacob simply says, “Jacob get a BIG immune system!”