Heaven over Hospital
Julianna Snow is dying of an incurable
disease. She's stable at the moment, but any germ that comes her way, even
just the common cold virus, could kill her. She's told her parents that the
next time this happens, she wants to die at home instead of going to the
hospital for treatment.
If Julianna were an adult, there would
be no debate about her case: She would get to decide when to say
"enough" to medical care and be allowed to die.
But Julianna is 5 years old. Should
her parents have let her know how grave her situation is? Should they have
asked her about her end-of-life wishes? And now that those wishes are known,
should her parents heed them?
Missing milestones,
and a medical mystery
When she was 9 months old, Julianna's
parents noticed she couldn't sit up steadily, something most babies can do
around 6 months. At her first birthday, when children typically start to
walk, Julianna couldn't even pull herself up into a standing position.
This worried Michelle
Moon, as a mother and as a neurologist. "The worst thing in the world
for a neurologist is to not hit your milestones," she says. "But I
tried really hard not to overreact and freak out."
Julianna's
pediatrician said she was likely just a late walker and would catch up soon.
Five percent of children, he noted, don't walk until they're 18 months old.
Michelle wanted to
believe him, but her mother's intuition, or perhaps her neurologist's
training, told her this was something more worrisome. She set out to do her
own research, which was lonely work, because her husband, Steve Snow, an Air
Force pilot, was on a three-month deployment to Korea.
With Julianna's
symptoms -- her developmental delays and her somewhat floppy arms and legs --
this is what kept popping up: spinal muscular atrophy, a hereditary disease
where neurons in the brain and spinal cord are progressively destroyed. Many
children with the disease don't live to see their second birthday.
To get spinal muscular
atrophy, a child must inherit a defective gene from both parents. Michelle
had herself tested and didn't have the gene. She was relieved, but stumped.
By now Julianna was 1½ and couldn't walk without the help of a walker, and
then only for short distances. Her doctors were mystified, too. Then Michelle
remembered her husband's funny-looking feet.
|
Dying = hampir mati
Incurable = tak tersembuhkan
Disease = penyakit
Stable = stabil
Germ = kuman
Instead = sebagai gantinya
Treatment = pengobatan
Decide = memutuskan
Grave = parah, suram (adj)
Heed = mengindahkan
Wishes = keinginan
Milestone = kejadian penting
Steadily = terus-menerus
Even = menyeimbangkan (v)
Pull = menjajarkan
Worry = mencemaskan, kuatir
Neurologist = ahli saraf
Worst = terburuk
Milestones = tonggak
Freak out = ketakutan
Peditrician = dok. Peny. Anak
Intuition = intuisi, naluri
Perhaps = mungkin
Worrisome = mencemaskan
Deployment = penyebaran
Symtom = gejala, tanda
Developmental= pengembangan
Somewhat = agak, sedikit
Floppy = yg terkulai, tdk kaku
Popping = bermunculan
Kept = terus
Spinal muscular = otot tulang belakang
Atrophy = terhentinya pertumbuhan
Hereditary = warisan
Spinal cord = saraf tulang belakang
Progressively = makin, bertahap
Inherit = mewarisi
Defective = cacat
Relieve = meringankan
Stump = tunggul
Mystified = bingung
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A diagnosis, inherited
In
spring 2004, after flying fighter jets over Baghdad and Kuwait, Air Force Capt.
Steve Snow was assigned to Osan Air Base in South Korea. On his first day, not
knowing a soul, he headed to the officers' club in search of company.
That's
when she walked in: Capt. Michelle Moon, the flight surgeon for his squadron.
Michelle
expected Steve to be wary of her, as pilots often don't trust the doctors who
have the power to ground them. But unlike some other pilots she'd met, Steve
was friendly. As they got to know each other, she found him dependable and
trustworthy. She sensed in him, she says, a genuine goodness.
The two
fell in love and were married in 2006. Two years later, their son Alex was
born, and Julianna came along two years after that on August 25, 2010.
Over
the years, Michelle had never given her husband's feet much thought, except to
occasionally notice that they were sort of odd looking, with high arches.
But as
Michelle searched for the cause of their daughter's slow development, Steve's
funny-looking feet became the key to solving their daughter's mystery.
High
arches, Michelle remembered, can sometimes be a sign of Charcot-Marie-Tooth
disease, a neurodegenerative illness awkwardly named for the French and British
doctors who discovered it nearly 130 years ago.
Poor
reflexes can be a sign, too, so Michelle took her reflex hammer to her
husband's Achilles tendon. His foot should have responded with a little jerk.
But it didn't move at all. "I thought, uh-oh. You can not have reflexes at
60 or 70, but in your mid-30s?" she remembers.
Michelle
brought her husband into her office during lunch hour and attached electrodes
to his arms and legs. She found his nerves were slow at sending impulses to his
muscles. Another neurologist confirmed her findings and diagnosed Steve with
CMT.
At
first it seemed unbelievable -- Steve had been athletic his entire life and now
flew fighter jets for a living. Countless doctors had examined him over the
years for flight physicals. How could he possibly have nerve damage in his arms
and legs? But CMT can sometimes be so mild that it goes unnoticed, even by the
person who has it and the doctors examining him.
By some
awful quirk of genetics, Steve's mild case of CMT had manifested as a severe
case in his daughter.
In fall
2012, just after Julianna turned 2, Steve and Michelle brought her to the
University of Iowa to see Dr. Michael Shy, one of the world's leading experts
on CMT. He explained that the myelin sheath covering her nerves had never
formed correctly, and so underneath, her nerves were degenerating, as a wire
frays if the insulation around it breaks down.
The
severity of CMT depends on the specific genetic mutation that has caused it.
Shy tested Julianna and couldn't find anything amiss, so he sent her DNA to a
specialized genetics lab at the University of Miami for a wider search. They
couldn't find anything definitive, either.
This
didn't mean Julianna didn't have CMT; scientists just haven't discovered her
particular variation. Without knowing what genetic mutation was causing her
disease, it was hard to give Michelle and Steve a prognosis.
Maybe,
just maybe, she would walk by age 3, in about a year. He had seen it happen.
Watching
her daughter's decline, Michelle doubted it. But she prayed he was right.
The 'slow-motion horror story'
By
longstanding tradition, when an Air Force pilot takes his final flight, his
colleagues douse him with champagne in celebration as he emerges from the
cockpit. Steve had decided to leave the Air Force and his "fini
flight," as it's called, was set for Davis-Monthan Air Force Base in
Tucson, Arizona, where the Snow family had recently moved from Texas. It was
January 30, 2013, about three months after their visit in Iowa with Shy.
Steve
didn't want to stop flying, but being an Air Force pilot means working long
hours and transferring frequently from base to base. Steve's family needed him
and it would be much better if they could live in one place, near extended
family who could help. Julianna was getting sicker and sicker and required more
attention. Alex, a 4-year-old bundle of energy, needed attention, too.
Later,
Michelle would write in a blog that Steve "sacrificed a career he loved to become
Julianna's main caregiver. ...(He) is simply the most decent human being I
know. ... He is my rock star."
As the
last bottle of champagne rained down, Steve became sad, thinking about how he
would never again fly the A-10 jet, his favorite aircraft.
He
looked out into the crowd for a supportive smile from Michelle, but he couldn't
find her.
He did
see Alex and his babysitter, who explained to Steve that Julianna was in the
hospital. Steve ran inside to change out of his wet uniform and drove straight
there.
Inside,
Julianna was having trouble breathing. What had started as a cold had turned
into something more sinister. The doctors assured Steve and Michelle that after
a round of antibiotics and some supplemental oxygen through a tube in her nose,
she should be back home soon.
But
that's not the way it turned out. In Michelle's words, a "slow-motion
horror story" unfolded as Julianna spent 11 days in the hospital
struggling to breathe, most of it in the intensive care unit. She needed more
than just a tube of oxygen in her nose; she needed a pressurized mask pumping
air into her.
Up
until that point, the Charcot-Marie-Tooth disease had weakened only Julianna's
arms and legs. Now it was attacking the nerves that controlled her breathing muscles.
The
Snow family will always remember Steve's fini flight as the beginning of a new
and horrible chapter in their lives.
The
worst was yet to come.
The start of tough choices
Dr.
Sarah Green was nervous to meet with Steve and Michelle. Just 33 years old and
only four years out of her training, she was relatively new to the delicate
task of talking to parents of dying children. For difficult discussions like
this one, she and her colleagues usually worked in pairs, but on that October
day in 2014, there had been a scheduling problem and she was on her own.
Julianna
was now 4 and in her third stay in 10 months at Doernbecher Children's Hospital
in Portland, Oregon, where Steve and Michelle had moved to be close to Steve's
parents.
Up
until this point, the family had been action-oriented, getting Julianna fitted
for a back brace to fix the scoliosis that had developed as a result of her
weak muscles, arranging for braces on her ankles and feet in hopes that she
might walk one day, making appointments for physical and occupational therapy.
But
none of this was helping her get better. In fact, she was getting worse. At 2,
she could sit up unsupported and walk with a walker, but now, she couldn't do
either. She once had nearly full use of her arms, but now couldn't even hold a
small toy without help. At one point she ate food, but now her chewing and
swallowing muscles were so weak, she was fed through a tube in her stomach.
Most
significantly, it was getting harder and harder for her to breathe and cough.
Repeatedly, mucus was settling in her lungs and causing pneumonia.
The
doctors had been able to pull Julianna out of it each time, but by definition,
because CMT is a progressive disease, her breathing muscles would only get
weaker and weaker. If she got another infection, her doctors weren't sure
they'd be able to help.
Green
proceeded with the conversation slowly and carefully. She asked Steve and
Michelle: If Julianna were to get another infection, would they want to bring
her back to the hospital? There was a reasonable chance Julianna would die
there after being subjected to painful procedures. Even if she lived, it would
likely be for a short time, and she'd likely be sedated, unable to think and
talk as she could now.
The
other option would be for her to stay home and forgo treatment, in which case
Julianna would most certainly die. But she would be dying at home and without
painful medical interventions.
There
was no right or wrong answer, Green told them. The choice was up to them.
Enduring difficult treatment
For
several months after Julianna was discharged from the hospital, her dolls kept
needing to be admitted. Julianna assured them there would be no shots or IVs
there, just Band-Aids and new toys.
It was
the wishful thinking of a 4-year-old, and even she knew it wasn't real. Over
and over, she told her parents how much she hated the hospital, especially
"NT," or naso-tracheal suctioning.
Several
times a day in the hospital, a nurse would put a tube down her nose and throat,
forcing it past her gag reflex and into her lungs to suck the mucus out of the
tiny pockets in her airways. It was too dangerous to sedate her for the
procedure, so Julianna had to feel everything.
The
task of doing NT suction often fell to nurse Diana Scolaro, who took care of
Julianna during all three of her intensive care unit stays at Doernbecher in
2014. Stronger children usually scream and have to be restrained when someone
tries to put the tube down their nose. But Julianna was so weak all she could
do was cry.
When
she was done, Scolaro would say to Julianna, "Rest now, baby. Maybe you
can make it two hours before we have to do it again."
But she
seldom lasted that long. "It's not for the faint of heart to take what she
took," Scolaro says.
When
Julianna told her parents how much she hated NT suctioning, her mother tried to
make her understand why they'd done it. "I told her that even though it
was really hard in the hospital, it let her get better and come back home to
us, so wasn't it worth it?" Michelle remembers.
Julianna
would never answer. That's when Michelle decided to have a conversation about
heaven.
Michelle
asked Julianna, then 4 years old, if she were to get very sick again, did she
want to go back for more treatments, or did she want to die at home?
Julianna's
answer was loud and clear. She chose heaven over the hospital.
Now
Michelle and Steve had to decide: Would they abide by her wishes?
