Old Hospital - New Hospital

Congenital heart disease is one of the most common types of birth defect and in the UK it is estimated to affect around 9 in every 1,000 births or slightly just under 1%.
(Source: http://www.nhs.uk/conditions/Congenital-heart-disease/Pages/Introduction.aspx).

However it is estimated 75% of people with 22q11.2 DS have some form of congenital heart defect and the majority of cases of 22q are diagnosed by paediatric cardiologists.  Cardiac conditions associated with 22q 11.2DS include

VSD (ventricular septal defect)
ASD (atrial septal defect)
Pulmonic atresia or stenosis
Tetralogy of Fallot
Right sided aorta
Truncus arteriosus
PDA (patent ductus arteriosus)
Interrupted aortic arch, type B
Coarctation of the aorta

Further information on these conditions can be found on the website of The Children's Heart Federation
http://www.chfed.org.uk/how-we-help/information-service/heart-conditions/

The severity of these conditions varies greatly and in many respects Sarah was "fortunate" to have an interrupted aortic arch rather than one of the other conditions. As Mr. Austin said at the time all of the plumbing was in place it it just needed to be connected properly. Sadly many other conditions are more complex requiring multiple surgical procedures and unfortunately in some cases although medical knowledge continues to advance currently the condition cannot be treated.

Back to October 2005 and Sarah continued to recover from her surgery, the mood on the ward was fairly relaxed as, where possible, patients were being discharged as the cardiac unit geared up for the its move to the new Evelina Children's Hospital the first new children's hospital built in London in 100 years.

The modern Evelina Children's Hospital

The original Evelina Children's Hospital was founded in 1869 by Baron Ferdinand de Rothschild, in memory of his wife, Evelina who died in childbirth. The original plan was for a maternity hospital but he was persuaded to fund a hospital serving the poor children of South London.  Following the introduction of the NHS, the hospital became a department of Guy's and effectively ceased to exist when services were moved into Guy's Tower in the mid 1970's becoming little more than the name of the children's ward. The hospital was reborn in 2005 with the construction of the new hospital on the site of the former nurses' home at St. Thomas's Hospital.  The hospital is designed around children and in the ground floor waiting area there is a large blue helter-skelter. Now if the hospital was a commercial company I would say that it had be designed to generate new business as having seen how fast children come down the slide I am convinced that it must have delivered a significant number of new "customers" for St. Thomas's Accident & Emergency department !

The day of the move arrived and Sarah and Colleen were in the convey of ambulances making the short journey to the new site. A journalist and photographer from The Times weekend supplement were on hand taking pictures of all the patients. For several weeks we eagerly awaited the Saturday edition of The Times to see if there was an article. The article duly appeared but Sarah did not, typical of the media's obsession with youth there was a younger patient barely two weeks old who stole the limelight !!

One feature of the wards is that built in to the wall beside each bed is a pull down bed which can be used by a parent wishing to stay the night with their child. This is a different world to the one I experienced when I was a child.

I was born with a cleft lip and had surgery at 6 months. By the time I was about four and half a second operation required and I was taken by my parents the the Queen Elizabeth Hospital in Hackney. I was put in an ambulance with several other children and babies and we waved goodbye to our parents as we were transported 20 mile across London to Banstead in Surrey. I think I must have been in hospital for at least a week and was visited once when my parents took the special coach service that ran to the hospital on a Sunday.  This was the latter part of the 1960's when cars and telephones were luxury items and a call to the ward to enquire about my condition would have involved either a walk to the local phone box or finding a neighbour willing to let my parents use their phone in return for a threepenny bit !!

Threepenny bit for the benefit of younger readers

I am not sure if I was traumatised by this event but two things stick in my mind. The first was being put in a bath as soon as I arrived at the hospital. In the 1960's no-one was allowed near a ward until they had been doused in eau de carbolic and secondly being told by a teacher that I could not participate in the hospital school as I was too young.

Fortunately things have moved on in the last 50 years particularly in the world of medical treatment and just 7 days after undergoing surgery Sarah was discharged from hospital and we were on our way home.

The size of a walnut

The news that Dr. Hobbins had detected a heart murmur was a bit of a surprise but there was no obvious sign of a heart problem and I was fairly optimistic that it would turn out to be a false alarm so the following day I went off to work as normal.
At around 11a.m I received a call from Colleen which confirmed my optimism had been misplaced. The echocardiogram had shown an anomaly and Sarah's doctor was on the phone to the specialist cardiac team at Guy's Hospital.  At that point I was unable to concentrate on anything and sat there waiting for more news. Around half an hour later I received a call from my sister-in-law telling me that Sarah and Colleen were in an ambulance on the way to Guy's.

In 2005 I was working in central London close to Tower Bridge and as soon as I received the call I was on my way to Guy's Hospital which was a brisk 15 minutes walk away.

Dr. Owen Miller, consultant in paediatric and fetal cardiology, performed another echocardiogram and gave us the diagnosis that Sarah had an interrupted aortic arch (IAA) and a large ventricular septal defect (VSD) which is more commonly know as a hole in the heart. She was experiencing heart failure and surgery was the only option. It was also mentioned that as many congenital heart defects are caused by genetic conditions it was advisable to do a blood test.

Around 50% of all babies with this type of IAA and VSD have 22q11.2 DS.

At that point there was little we could do but wait and contemplate the fact that our 17 week old baby was about to undergo major surgery. Around 48 hours after Sarah had been admitted to Guy's the medical team were discussing whether or or not to proceed with the operation as they thought Sarah may have had an infection which could cause complications. That afternoon we received the news the operation was going to take place the following morning.  I must admit we had mixed feelings as on the one hand we wanted the problem fixed as soon as possible but on the other we were apprehensive about the forthcoming surgery.

That evening we met Mr. Conal Austin the surgeon who was to perform the operation. He described in detail how he would undertake the repair and helped to ease the anxiety we were feeling at that time.

As a general rule of thumb the size of a person's heart is about the size of a fist. In the case of a 17 week old baby the heart is about the size of a walnut. I still find it unbelievable that life saving surgery can be successfully carried out on such a small object and we are very privileged in this country to have surgeons with the knowledge and skill to perform such intricate surgery.

After listening to what was about to take place I was asked to sign the consent form which included the potential risks associated with surgery of this nature including the 5% risk of death during the operation.

When thinking about risk human nature is to focus on the downside. However in such a situation my recommendation is to look at the risk equation from the other side and I told myself instead of a 5% risk of death look at it as a 95% chance of surviving which sounds much better.

Thursday 13th October  (Luckily I am not superstitious)

Sarah was scheduled to be the first operation of the day but due to an administrative mix up another patient went down to theatre first. This not only prolonged the agony for us but Sarah had not been fed since the previous night and was becoming quite agitated. At about 11a.m. we got the call to go, carrying Sarah down to theatre, and leaving her in the care of the surgical team was probably the worst thing I have ever had to do and the memory of this will live with me for ever.

The next few hours were the longest I have experienced, we took a walk along the South Bank and went for lunch but had little appetite.

Fortunately we had been offered a room at the local Ronald McDonald House which provides accommodation for families of seriously ill children and went back to the room to rest and wait. Around 3.30 p.m. we got the call to say Sarah was on her way to intensive care and we rushed back to the hospital to see her.  Although she was covered in tubes, drips and drains she had come through the surgery and Mr. Austin told us that the operation had gone well.

The body's powers of recovery are amazing and hour by hour we could see an improvement. Often we would go out to get something to eat or drink and when we got back to intensive care we found that another tube, drain or monitor had been removed.

Barely two days later we were back on a normal ward feeling confident that the worst was over.

The Phoney War

For as long as I can remember I have always been interested in history, eagerly devouring books, magazine articles and TV documentaries. The next part of my 22q story resembles a period of the Second World War which in Britain is referred to as "The Phoney War" covering the period from the initial declaration of war in September 1939 when thousands of children, including my late father, were evacuated from major cities until April 1940 when Denmark and Norway were invaded. A period marked by limited military activity in Western Europe.

Colleen's pregnancy continued without incident, she had a couple of scans and nothing untoward was detected. Colleen was keen to know the sex of our baby but successfully keep the news from me as I preferred to wait until the big day.

The due date came and went and twelve days later at around 3 a.m. Colleen went into labour and we set off on the short journey to the local hospital. Labour was progressing nicely during the morning and early afternoon but by early evening progress had slowed and the baby was showing some signs of stress.

The medical team took Colleen into theatre and I was directed to the room where the medical scrubs were kept and told to get kitted out. So there I was, someone who had always been squeamish about the sight of blood, needles and anything medical, dressed up ready to go into an Operating Theatre.
To my immense surprise I stayed upright and didn't pass out there and then !!

On my way to theatre I stopped off to collect a nappy and babygrow from the bag we had taken to the hospital. I unzipped the bag and my eyes were immediately struck by an explosion of pink. The secret was out we were having a baby girl !

In theatre there was an initial sense of panic although this quickly subsided and the team got to work on delivering the baby by caesarean section. I had always thought new born babies cried the minute they arrived and was therefore concerned when a saw a limp silent Sarah being passed to another doctor. The silence seemed to last for ages but in reality was probably less than a minute. I then heard the doctor say "I have a strong heartbeat" followed shortly afterwards by Sarah's first cry. I felt an immediate sense of relief as Sarah was whisked off to Special Care Baby Unit where she stayed for several days receiving antibiotics as there was concern that she had ingested meconium.

After 6 days Colleen and Sarah left hospital and we could start being a family. The initial excitement had passed and we quickly got into the routine of feeds and nappy changes. I am please to say I very quickly developed the knack of sleeping through the night undisturbed by night feeds !   Sarah was a very good baby and cried very little. Her cries were more like little squeaks which resulted in us giving her the nickname "Squeaky" which we still use today.

Sarah fed well, slept well and continued to put on weight. The only unusual thing was when she brought up her milk she was sick thought her nose as well as her mouth. The weeks passed uneventfully and we even had time to spend a relaxing week on holiday in the Cotswolds.

Chipping Campden, Gloucestershire where we spent a relaxing week

Around three and a half months later we were at a family christening and Sarah was very restless, unable to sleep and constantly wanting to be carried. For the first time we noticed a reluctance to feed and our GP referred her to the local hospital. The hospital diagnosed bronchiolitis and Sarah was admitted. Several days later Sarah was still in hospital and although she was not getting better she did not appear to be getting worse. Imagine our surprise when following an examination by a senior paediatrician we were given the news that she had noticed a slight heart murmur, we were told that it was probably nothing to worry about and as a precaution she would undertake an echocardiogram the following morning.

Like the people of Britain in Spring 1940 who I mentioned at the start of this piece we were just about to experience our first bombshell.

The First Scan

Do you know what 22q 11.2 Deletion Syndrome is ?

If you had asked me this question nine years ago I would not have known the answer. Was it some kind of chemical formula or part of a mathematical equation ? To be honest nine years ago did I really care ?

In spring 2005 my wife Colleen and I were more focused on the arrival of our baby. We had got married the previous year and not being the youngest of newlyweds had decided to start a family straight away. With the biological clock ticking the big concern was the risk of Down's Syndrome which as everyone knows is more common where older parents are concerned.

So early in Colleen's pregnancy we went off to King's College Hospital for a nuchal translucency scan which measures by ultrasound the fluid at the back of the neck of the foetus. This measurement is then used to predict the risk of Down's Syndrome.

We were told the risk was within the normal range and the scan was OK.

Like all prospective parents we were hoping for a healthy baby. Having had the nuchal translucency scan we had ticked the box for Down's Syndrome, so no need to worry about a chromosome abnormality!!

King's College Hospital, London

Welcome !!

Inspired by my friend Mike Woods who writes an excellent blog http://rulesfoolsandwisemen.blogspot.co.uk/ I have been thinking of writing my own blog for some time.

To date, despite my best intentions, I have so far not managed to get round to it.

Now I have no excuse.

22q Awareness Days is a campaign which aims to enhance the general public knowledge of the genetic condition 22q11.2 Deletion Syndrome over a period of 22 consecutive days from 27th April 2014.

https://twitter.com/search?src=typd&q=%2322qAwarenessDays  https://www.facebook.com/22qAwarenessDays

22q11.2DS is believed to be the second most common genetic condition after Down's Syndrome.

Most people know something of Down's Syndrome but very few have ever heard of 22q11.2 DS.

This is a condition which affects my daughter Sarah and I thought it would be a good idea to share my family's experience of 22q11.2DS and hopefully bring knowledge of this condition to a wider audience.

My objective is to write a post each day for the next 22 days and I hope you will join me on this journey.