Nia’s story
I have always wanted
to tell Nia’s story, but up to this point, I’ve been far too emotional to do
so. It is early Sunday morning, and Nia just walked over to our bed, cuddling
alongside us and falling asleep again. Looking at her, sleeping soundly and
happily, fills me with pride and enormous happiness and that I am finally waking
up from a nightmare that started nearly a year ago…
Nia was one day short
of 10 months old on
Tuesday, April 17th, 2001
. My husband John and I took Nia to visit
my parents in
Germany
for a few days over the Easter holidays.
I had booked a routine check-up with a German paediatrician during our stay
because I was not happy with Nia’s eating habits, her low weight gain, and her
lack of mobility development, (she still did not roll over onto her tummy or sit
up by herself). She also just recovered from bronchiolitis. I did not have much
trust in the
UK
’s NHS system—the health visitors kept
telling us that Nia ate as much as she needs, and since John and I were quite
slim children, Nia was just the same. They explained her lack of movement was
sheer laziness, and we should encourage her more to lie on her tummy. I always
felt classified as the notorious first time Mum, which is a feeling I believe I
share with most first time Mums. The German paediatrician, a female doctor, did
all sorts of checks, and was generally content until she listened to Nia’s
heart. The doctor closed her eyes and I saw her concentrating. I feared
something was wrong. She then asked me if somebody already told me that Nia has
a heart murmur (something that is quite common in babies and does not necessary
mean that there is a problem). No, I said, nobody had mentioned it. She advised
me to eventually get it checked by a paediatric cardiologist. I did not know how
to get hold of one in the
UK
(we were planning to fly back two days later). The doctor kindly
arranged for us to see the paediatric cardiologist in the regional hospital.
Two hours later, my
Mum, Nia, and I were in the hospital, waiting for the doctor to come (John had
stayed back at my parents house since he was not interested in the shopping trip
I have planned for the rest of the day). Nia had an ECG and an echocardiogram.
The echo took more than an hour. The doctor did not say a word while poor little
Nia screamed hysterically. It was the first time I have ever heard her scream in
this way. I got more and more nervous, and after the longest hour in my life (up
to that date) the doctor asked us to dress Nia and come down to his office. I
then called John to come to the hospital, since I knew something must be
terribly wrong. By the time we entered his office 10 minutes later, the doctor
had already arranged for us to go to the heart unit of the children’s hospital
in the city of
Giessen
(about 60 miles away). He suspected that
Nia had a rare heart condition called ALCAPA (Anomalous origin of the Left
Coronary Artery from the Pulmonary Artery). With this condition, the left side
of a baby’s heart muscle doesn’t get the rich oxygen concentrated blood from
the aorta, but the nearly oxygen-free blood out of the pulmonary artery.
Therefore the heart muscle and the left side of the heart cannot function
properly (it took me several months and a lot of studying to understand her
condition). The doctor told us to drive directly to the children’s hospital,
and not to drive by my parents’ house first to pack anything. It took us
nearly two hours to get to
Giessen
. We were greeted by another paediatric
cardiologist, Dr. Bauer. He did another series of tests and told us Nia was
booked for a catheter examination the next day. That was the only way to see
exactly what was wrong with her heart. He also added that if Nia indeed had
ALCAPA, she would require open-heart surgery the following day. At that time, I
luckily still had no clue of all the events to come.
On Wednesday, April
18th, Nia’s 10 months birthday, we had to sign the consent form for
the catheter examination. During the procedure, a tube is inserted the flow of blood
through in thetinto a vein in the thigh. The tube is pushed through her
veins into her heart. A contrast fluid is fed through the tube and squirted into
the heart. This fluid appears on x-rays, and enables the doctors to see the
structure of the heart, the connections of cardiac veins and arteries, and the
flow of blood through in the area. Sadly enough, the examination confirmed her
condition, and later in the day, we had to sign the consent forms for the
surgery the next day. Again, I was luckily still very unaware what open-heart
surgery really meant—I was under the assumption that it must be nearly a
routine operation by now, maybe slightly more risky then an appendectomy. To the
contrary, we were told that the survival rate for open-heart surgeries is about
90 % (sounds high initially, but if you think that out of 100 babies, 10 die –
it sound very scary).
On Thursday, April 19th,
at
7:30 am
, nurses helped us roll Nia’s bed to the
theatre. Nia was already drugged to go to sleep, but she stayed wide awake. She
was pulling my hair, not wanting to let go. However, we had let go of her—we
were not allowed to stay with her in the theatre. There is no way I can describe
this feeling of totally losing control of my daughter, my life, and all of the
things that are happening. For the next 5 hours, John and I walked around town,
but every minute felt like hours. At
noon
, we walked up to the intensive care unit
and after 30 minutes waiting, were at least told that she is out of the theatre
and on her way up. At about
1:30 pm
we were allowed to don green theatre
gowns and ife, totally losee her.
She was lying there, still knocked out from all the drugs, on the ventilator
(the machine that does the breathing for her), with over a doze tubes going into
or out of her. Strangely enough, this all did not scare me too much. I was just
happy to see her and thinking she is the most beautiful little girl. I was
hoping the worst was over then, but learned that the next 48 hours are indeed
the critical ones. It took until Saturday for her situation to be called
stabile. John and I spent most of the time waiting to get to see her in
intensive care—we were usually only allowed to stay with her for short periods
of time. This is very different from the
UK
, where parents are allowed to stay with
their children for nearly most of the time. On Sunday she was taken off the
ventilator and was again breathing on her own, waking up on and off. On Monday
she was moved out of the intensive care unit onto the ward.
Back on the ward, Nia
was recovering, but also crying a lot. One of the nurses explained that Nia is
“on cold turkey” – a condition drug addicts are on when they stop taking
the drugs. Since Nia had been on morphine for nearly a week, her body had to
adjust being without it. I slowly started to regain my confidence again, hoping
this nightmare would be over soon. On Wednesday, April 25th, I met
the head of
Giessen
’s paediatric cardiologists, Professor
Schranz. He told me that Nia’s condition was discovered very late (it is
usually picked up in the first three months). Most babies with the condition
untreated will not celebrate their first birthday, and when treated as late as
Nia, very often need a heart transplant since the heart muscle is damaged and
cannot be repaired. Nia apparently suffered from various heart attacks before
(very often mistaken for colic – and Nia has been a very colicky infant) and
her mitral valve (the valve between the left chambers of the heart) was
seriously damaged and did not recover as expected after the surgery. In
addition, the surgeon had not been able to perform the planned surgery, which
had been to re-connect the left coronary artery to the aorta. Instead, he had to
perform a bypass operation, very common in adults with coronary diseases. We
were told that they had to do another catheter examination in a few weeks time
to verify that the bypass is indeed functioning. On May 3rd, 2 weeks
after her open-heart surgery, Nia underwent her second catheter exam. It showed
that there was good blood flow in the bypass and that this would likely persist,
but her mitral valve was still severely leaking and could require further
surgery in the future. We flew back to the
UK
with several medications for Nia (8 times
a day) and a referral to the Birmingham Children’s hospital (BCH).
Nia had her first
appointment at the BCH in June. The consultant there, Dr. Stuemper (also
German), doubted that the bypass performed in
Germany
could indeed ensure enough blood flow to
the left heart muscle. He suggested doing another catheter exam later in the
year to see how the bypass developed. We saw him again in July and then were
scheduled to come back in October. In September, I had to go on a business trip
to
Germany
. I took Nia along to have her checked
again in
Giessen
, were she had her first surgery. Prof.
Schranz he was still not happy with the mitral valve and urged us to get it
repaired or replaced within the next three months. Strangely enough, he
recommended that Nia have the surgery done at BCH since he considered Mr. Brawn,
a paediatric cardiac surgeon there, one of the best in the world. Prof. Schranz
promised to send a letter with his recommendation to BCH.
At our next
appointment with Dr. Stuemper in October, I expected to discuss the upcoming
surgery. However, Dr. Stuemper did not concur with the German recommendation. He
wanted to perform another catheter to see how the heart and bypass were doing.
On December 4th Nia had her 3rd catheter done at BCH.
Sadly, it confirmed Dr. Stuemper’s concern—the bypass was closed and did not
supply the left side of the heart with blood. This explained the poor state of
the mitral valve. Dr. Stuemper
strongly recommended another surgery for Nia using a technique called Takeuchi.
This is a complex operation that is rarely performed and was classified as too
dangerous from the consultant and surgeon in
Germany
(we were told by the German site that
they have only seen fatalities with this type of surgery). But Dr. Stuemper was
convinced this surgery would be Nia’s only change to save her heart and if we
do not give it a go, Nia will need a heart transplant in the near future. A
heart transplant might sound like a long-term fix, but sadly it is not—in
babies with a successfully performed heart transplant, the survival rate is
about 50 %, and the heart is expected to last for an average of 5 years before a
new heart transplant is needed. Dr. Stuemper said he would try to book a surgery
appointment with Mr. Brawn for two days later (December 6th). John
and I were totally devastated, especially since we felt we were caught between
two systems and not really knowing whom to trust. The next day, we were told to
go home, since the surgery slot was taken away for an even more urgent case and
we should expect to get an appointment in January. Dr. Stuemper said we should
try to enjoy Christmas with daughter. A part of me feared that this decision was
done because they have already given up on Nia and wanted us to at least
celebrate Christmas together. Nearly 8 weeks of long waiting passed and we then
got the appointment for
Monday, January 28th 2002
. In the meantime, I contacted the
Giessen
hospital again. Prof. Schranz again urged
us not to go ahead with the Takeuchi procedure, and only go for a mitral valve
repair. He promised to discuss this with Dr. Stuemper, but never did. I do not
know exactly why, but we decided to give our full trust to
Birmingham
. We met with Mr. Brawn, the surgeon, on
the evening of January 27th. He is the most remarkable man I ever met
– he sat down with us for more than an hour on that Sunday night. He told us
that he did not know of any case where the Takeuchi procedure was done as a
second attempt. Also, he said the Takeuchi procedure indeed is quite rarely
used. He had only done a few (a decade prior) while working in
Australia
and did not know about the long-term
outcome of these patients. Given his knowledge, sincerity, and honesty, John and
I fully trusted him.
Mr. Brawn saw us
again the next morning together with Dr. Stuemper, who seemed extremely nervous.
We signed the consent forms and at
noon
and carried Nia into the operation theatre. We were allowed to stay with
her until she was asleep. Again, we had to let go of her, not knowing if and
when we will see her again. After the first experience in
Giessen
, We were aware that we not only needed to
wait for the surgery to finish, but also endure the following 48 hours after
which she will hopefully be out of the danger zone. The surgery was estimated to
last 6 hours, but one of the nurses must have misunderstood something and told
us to check back again at 4 pm. At
4 pm
, Nia was still in the theatre. John and I moved our bags into a room at
the Edward House, next door to the hospital. The ward promised to ring us as
soon as Nia came out of theatre, but by
6 pm
I thought I would go nuts and had to go back to the ward. I literally
ran into Mr. Brawn, who just came out of theatre to see us. Somehow he looked
very sincere and I feared the worst – but for a change he had good news. The
surgery was successful; he was even able to repair the mitral valve to a degree
(something he doubted he would be able to do). He walked with us to Nia’s
bedside in Intensive care (PICU). I was allowed to stay with her the whole time
she was there. I never had to leave PICU, even when they took the ventilator out
at
3am
– only 9 hours after the surgery (last
time it took 4 days). Seeing my baby on a ventilator, knowing she is not even
breathing by herself and a slight mistake in medications or drugs could mean her
end, is just pure horror. It was especially true since this second time, I was
more aware of what can go wrong than I was the first time. But the staff members
in PICU were just wonderful – they constantly kept me informed. As soon as Nia
was taken off the ventilator, a nurse placed her in my arms, with all the rest
of the tubes still attached. Nia then settled down (she was quite active, trying
to pull her tubes out). She woke up early in the morning, and by the afternoon,
she was transferred back to the ward – not even 24 hours after her surgery.
Going back on the
ward meant she was out of the critical phase. I had not eaten in nearly 24 hours
and boy, was I hungry. I had a big breakfast, one of the best breakfasts I had
had in several months. Everything went uphill from then on – she went through
her “cold turkey” phase where she even pushed me away. On Monday, February 4th,
Nia had to undergo her 4th catheter examination. Dr. Stuemper
performed the catheter and saw us right after he left theatre. I will never
forget the look on his face – he was so happy! Everything looked fine. Blood
was flowing into the left coronary artery, and her mitral valve leakage was
classified down to mild-moderate (before, it had been classified as severe). If
he and I weren’t both stiff Germans, I truly would have kissed him! And then,
even more amazingly, we were allowed to leave the next day, on Tuesday, February
5th.
Today, Nia is still
takes plenty of medication to help her heart recover. Nobody can predict how
successful the long-term outcome of the surgery will be, or how the blood supply
to the heart will establish in the long-term. But nobody who sees our little
princess can believe what she has gone through. The only constant reminder is
the big scar on her chest (looks like a big number “1” – how
appropriate!). We were so lucky to live close to
Birmingham
, one of the leading paediatric heart
units in the world. The care there was supreme. They absolutely fulfilled their
promise that the parents are part of the team. We were constantly involved in
Nia’s care; one of us was allowed to sleep next to her bedside; and every time
a medical professional approached her, we were fully informed and could ask any
questions. Dr. Stuemper and Mr. Brawn are more than my personal heroes, and I
know there is nothing I can say or do to fully express my gratitude. Being so
far away from our families in the
US
and
Germany
, John and I are also grateful for all the
love and support we received from our colleagues and friends here in
Milton Keynes
. Without them, I doubt I would have
stayed sane during the last year.
I must apologize that
this article got a lot longer than intended. I must stress that Nia’s
condition is very rare, so I do not want to worry any of you. ALCAPA
occurs once in about 300,000 live births—so far, only 300 known cases
world-wide. I just want to share one lesson with you that I have learned: always
trust your instincts. Whenever you think something might not be right, say so.
Enjoy every single
day with your little ones—don’t worry too much about tomorrow, or be too
upset about the past.
Thank you,
Corinna