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When I was pregnant
with my third child I wondered, very briefly, whether my baby would be born
perfect. My other two had, so I put the
thought out of my mind and concentrated on eating well, exercising, and looking
forward to the new arrival. I noticed
that my baby didn’t move so much in the womb and that there was none of the
violent kicking that made my gasp and feel faintly queasy, but, in a way, this
was a relief. Her movements were gentle,
like a fluttering, or a slight wobble.
My pregnancy on the whole had been calm, apart from a scare in the third
month when there was slight bleeding. I
wondered if I might miscarry; it disappeared for a few weeks but then
reappeared to an extent that I rang Emergency and they told me to come into
hospital. There, they made me wait and
drink copious quantities of water before taking me in for a scan. It was incredibly difficult to retain all
that water, and retain my dignity. The
scan was normal. The bleeding stopped,
and the pregnancy progressed.
On my due date, I
started Braxton-Hick’s contractions, the false labour that can send you into a
panic. They were irregular, just a
tightening across the belly as the muscles contracted and relaxed. Although I wasn’t unduly worried, it seemed
prudent to check with the hospital. They
said to come in anyway. So I arrived at the hospital doors
awaiting admittance. The contractions
faded soon after, and in the morning I was sent home. There was not much longer to wait, though,
and that night I was admitted again, this time in labour.
It was a struggle
and, because my previous pregnancy had been a caesarean section, the doctor
decided forceps delivery was going to be better, so, after a few hours of
labour, she was born. The rush of
oxytocin, endorphin and adrenaline hormones made me
feel elated and excited. But there was
no cry, no waving of tiny arms, just silence.
I remember the nurse holding her, naked, limp, like a rag doll, arms and
legs totally relaxed. The nurse quickly
took her away to a waiting crib and wrapped her in muslin. Still no cry.
No movement. To begin with, I did
not notice, and was waiting for them to bring her back to me. The nurse turned to me and said they would
just run a couple of quick tests. I
imagined these to be the usual muscle reflex.
It was at that stage that all my
unbidden fears, all the things I had pushed to the back of my mind, flooded in
quickly taking away the adrenaline and leaving me feeling as limp as my baby
looked. What was the matter? Where were they taking her? What was wrong?
My baby is not perfect. The only thing they could offer was that she
had been exhausted by the birth and was hypotonic, limp, no muscle tone. They wheeled me back to the ward without my
baby. I lay there motionless, waiting
for someone to bring her to me, or to tell me what was happening. Nothing.
I waited. Hours went by, and a
nurse popped her head around my curtains and said – ‘oh, you’re the one with
the baby who’s got the speckled head-rash!’
What was she talking about? I
asked her: she said straight away, ‘oh that usually means mental retardation.’
My world collapsed, spinning out of
control. I was icy calm, trying to
marshal my thoughts. What now? Who can tell me what is going to happen? I spent the night alone in the ward,
separated by a flimsy white curtain from other mothers and their perfect babies. In the morning the specialist asked to see
us. He said a whole lot of things that
simply would not fix themselves in my head.
They entered, then flew away. I
kept asking if he would repeat himself, tell me again what did it mean? Words jumbled around in my mind. I could not tell you anything he said. I felt calm, as though I was not really
participating in this discussion, more as though I was hovering above us all
just watching.
They brought me my little red-faced,
long-limbed, silent baby to feed. She
would not suck. I thought she was tired,
but she had no power to suckle. They
gave me a bottle with an extra long teat.
‘Squeeze the teat into her mouth’, they advised.
They let me bath her, and she lay there
in the water, limbs outstretched, unable to move, barely able to open her
eyes. Every few hours they brought her back
to be fed, and I pretended it was fine.
The nurses volunteered their own information, a cousin whose baby was
‘just like this’ and went on to regain her strength in a matter of weeks. A son who never walked ‘til he was 3, but was
just fine; she’s just like many babies who are born tired but soon
recover.
But the speckled rash was still there,
and the words from outspoken nurse who’d frozen my heart, were still tight in
my chest.
It was no good, she was losing weight,
not crying, not moving; just sleeping.
So they tube fed her and I watched, feeling desperately useless, and wondering
what was wrong. No one could tell
me. Failure to thrive, was the best I
could get. I was terrified, but the
terror was now iced in and would not thaw for many months yet. I expressed milk, sitting in a corner of the
nursery, listening to other babies crying, feeling so alone, so sad.
I was allowed home after – how many
days? I don’t remember, they blurred
into one long day, one long night. And
once home the feeding became the only challenge of the day. She slept endlessly, not waking, not crying,
not moving. I squeezed the bottle’s teat
into her mouth and massaged her throat, willing her to swallow. It took an hour to feed 15 mls. She became weaker and weaker and went back to
hospital where they tube fed her, then left her to sleep. I would drive to the hospital and sit with
her for hours begging her to pull through, to be normal, to come alive. She lay motionless and pale. Sometimes when I held her she would open her
eyes and engage mine. That was when I
would pray, to what, to whom, I did not know.
They were just words repeated over and over, “let her live, let her be
alright”.
She wasn’t, of course. She did live, but there was something fundamentally
wrong; she remained in hospital and the doctors continued to look for an
answer. I came in day after day, asking
whether they had found out anything more.
One day there was a plaster on the calf muscle of her tiny leg. They’d taken a muscle biopsy to see whether
she could have Werdnig Hoffman’s Disease.
Spinal muscular atrophy. A
wasting disease that has a child living 18 months or less, then wasting to
death. But they didn’t tell me
that. It was years and years later when
Google came into being that I found the answer.
I could then see why they tested for it.
Many of the symptoms and characteristics were there.
After she had gained some weight, I was
allowed to take her back home. I tried
to encourage her to stay awake for longer by not putting her to bed, but
setting her up in her bouncinette so she could watch the other children, hear
household noises, listen to music, watch the dogs. Slowly, very, very slowly, she started to
become more alert, she smiled, she watched our world from her own. But there was no answer to her medical
condition; she still had the label of Benign Hypotonia.
At three and a half years old, when she
was finally walking albeit unsteadily, finally talking albeit a language of her
own, and we had accepted her as she was, a friend showed me an article in an
Australian women’s magazine about a child who was 10 years old and who had
started life in the same way as our child had.
This child had a deletion in the 15th chromosome which caused
all sorts of things including hypophagia.
An inability to know when to stop eating. ‘Obesity and early death usually in their
teens, followed.’
The darkness that descended was, again,
indescribable. Our daughter now wore a
different label, that of Prader-Willi syndrome.
Again, we were still pre-Google, pre-internet, and pre-information. This time, the block of ice that had served
me so well in the beginning, had melted and what was left in its place was a
well-forged, intensely heated rod of iron.
The fight was on, let the battle commence. I fought for knowledge. I asked questions. I was no longer afraid of medical professionals
spouting coded messages. I would not
leave their rooms until I had the answers I needed. It was a long wait and usually ended with one
step forward, two back. The geneticist
took down a large medical dictionary, opened the page at “P” and left the
room. Why did he do that? I still have no idea. Did he expect me to turn the book around and
read the section on Prader-Willi Syndrome (as, of course, I did)? What then?
Did he think I would understand?
He returned eventually, but threw no new light other than this was a
genetic disorder. The paediatric
psychologist did all sorts of puzzle tests with her; finally walking us to the
door and saying, ‘Oh look, there’s a young lass with PWS, do you really think
your daughter has that?’
How the hell did she not see it when I
could, so clearly?
It was an ongoing battle. Friends who knew me well stood by me. Family worried about me. No one else mattered. I had become fanatical about this wretched
syndrome, but I kept it to myself knowing how quickly others become bored with
fanatics. Slowly, bit by bit I recalculated
my life. It was a completely new rebuild;
I discarded things that no longer held true; fanciful, dreams that would never
come true, people I couldn’t be bothered with, certain principles were
abandoned in favour of new. There was a
fire sale – a big one – and I closed down the old burnt-out me. I reopened some months later, just a few
doors at a time, just a peep now and again until the paintwork was reapplied,
the garden weeded, the fences restored; a completely new façade gave no hint to
its terrified interior.
The child grew up, did not die, was
loved, educated, and though there were some dramatically bad times, we all got
through them. The façade of the new me still
held good. However, the interior always
needs re-strengthening, redecorating, reviving, and old things cast out for new,
It has worked; most of the time it has worked.
Nearly 30 years later, life is different, but it is still good and the
interior decorating still goes on. Nothing
is perfect.
They also nearly always say the same thing about how much she loves me and what a great mother I am and my apparent amazing attributes are freely listed. What this does is put my own life into perspective. It doesn't matter that most of my friends have 'normal' kids, it doesn't matter that I might have missed out on various outings, or feasts, or had to stay home and deal with stuff that wasn't expected. It doesn't matter that the word 'dysfunctional' may have been used to describe my family - words, after all, are just words. What does matter is that my daughter can see past her own disability and see her life as "fantastic".
