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Our youngest daughter, Matilda, was
diagnosed with Non-Compaction
Cardiomyopathy (or Spongiform) when
she was 4 weeks old. We were
referred to a cardiologist because
of a couple of small VSDs which were
detected in her heart in-utero, and
because of a family history of
non-compaction cardiomyopathy.
Nevertheless, we did not expect her
diagnosis. Matilda had been
investigated at birth by
echocardiograph and her heart was
functioning normally at that time.
We did have other healthy children,
and had been advised that it was
likely the loss of our 10 day old
daughter (four years earlier), also
diagnosed with spongiform
cardiomyopathy, was a random event.
Matilda did not exhibit any signs of
heart failure, and believe me, no
one watched for this more closely
than I! We were sent home
from our outpatients appointment
with a prescription for Digoxin, and
instructions to stay in close
contact with our paediatrician.
I remember walking out of the
hospital in tears, holding Matilda
in my arms, feeling history repeat
itself.
I went home, told our family and
immediately started researching this
disease. There isn’t a lot of
information to be found. It is
very rare, but perhaps more common
that it appears. Many children
with cardiomyopathy are later found
to have non-compaction
cardiomyopathy. I inundated my
paediatrician with my findings, and
he patiently read all the articles I
left in his office and answered my
odd (and often neurotic) questions.
Like the time I rung him to tell him
I thought she had fluid retention.
Her wrists were fat and looked like
they had rubber bands around them.
No, he says, she is just putting on
weight (it was true, and he kindly
didn’t make me feel stupid).
From his office, with the approval
of our cardiologist, we introduced
the beta blocker, Carvedilol,
firstly in very small doses and
slowly increased over a period of
months. This did result in
improved heart function (to EF of
42% at 8 months), although
outwardly, Matilda still did not
exhibit any symptoms of heart
failure, and continued to grow and
thrive. She currently remains
in the 75th percentile
for weight and the 90th
percentile for height.
Her heart function recently dropped
back to where she started, at
diagnosis, with an EF of 32% and an
SF of 16%. She still had no
symptoms. This resulted in a
voluntary admission (our first
hospital admission) to Prince
Charles Hospital for an overnight
increase in Carvedilol to more
closely match her body weight and
the introduction of a new drug,
Lisinipril. Once again we hope
her heart will slowly improve on
this increased dose of Carvedilol.
Matilda turned one last month and
tipped the scales at 11kg. She
continues to amaze and inspire us.
How can her heart be so compromised?
She appears so normal. She is
walking, and starting to talk.
She is full of energy, an adorable
and charming little girl. She
hasn’t slept though a full night in
her life, but hey! no one’s
perfect!!
I find the only way to deal with the
question of how long we have her
for, is to realize that we never
know what is around the corner for
any of us or our children. To
spend time worrying what will become
of one child, is a pointless
exercise. I have had five
children, all as precious as the
next. There are no guarantees
in life. It is best to try to
enjoy each day as it comes, and to
appreciate life for the wonderful
gift it is. |
