Wednesday, 9 August 2006

It's Another Girl!

Born 16.8.05 - Full Term - 9lb 3oz - It was devastating to hear after our daughter Violet was born, that she had been born with numerous medical conditions. It was last thing we expected, as we already had a healthy 4½ year old daughter, Emily. I had done all I could possibly do, to have what I thought was a healthy pregnancy, but some things you just have no control over! The past year, since our daughter was born, it has been like an emotional rollercoaster, with operations, tests and dealing with seeing your baby go through so much, so young. Accepting that it will be lifelong conditions that will need constant ongoing care. It has already been a huge learning curve for our family.

We spent 1 week in Special Care Nursery of the hospital Violet was delivered in. On delivery, her cord was wrapped twice around her neck and once around her body. The night she was delivered, she was given oxygen for something the hospital calls 'a dusky episode', which means she went blue and was having trouble breathing. She also developed a high temperature, so she was put on antibiotics straight away. At 1 day old, midwives noticed her head circumference was quite big. They asked if they could do an ultrasound on Violet's head and I refused, as I thought they were all nuts and being over the top. In the end, I finally agreed, only because I thought it would prove that nothing was wrong and she was a normal healthy baby and could finally go home. The head ultrasound revealed she had an enlarged left ventricle (we had no idea what this all meant) and the next day we were transferred to Neonatal Intensive Care Unit (NICU) of the children's hospital.

The NICU of hospital did not have the facility for parents to sleep next to their babies, which was very hard. I had to sleep in a parents hostel, which was located 2 floors down from the NICU. I was grateful they at least had this facility for parents, even though it was not free. All I wanted, was to be at her side. What also made it hard was that I was breastfeeding Violet. So,I was on call during the night. I always ran up the 2 flights of stairs instead of using the lift (it took too long) and wondered how long she had been awake crying for. As there are alot of babies in the NICU and few nurses at times to attend to them all at once. I think I could have easily given breastfeeding up this time, as everything was working against me continuing with it, but I am glad I persisted, she needed it more than ever.

I spent all day, everyday by her side. If your were not around when the doctors did their rounds, you would miss them completely until the next days rounds, so it was also a matter of having to stay there to see them, to find out results etc.

It's a weird feeling hospital life, it's like automatic pilot turns on and you seem to float around, trying to remember to feed yourself and try to take in all the medical jargon the doctors speak, and due to the lack of sleep, find it hard to remember anything they tell you.

They performed many tests and scans on Violet. She had an MRI on 24.8.05 and she was given Chloral Hydrate to knock her out long enough to stay still for the MRI or it would have been a waste with any movement. Trouble is by the time a porter comes (they are very busy people) to take Violet down to have it, she is already starting to stir. I had to sit near the MRI Machine, for my own benefit of knowing that she had stayed still, then I would know it had not been a waste of time. There faces said it all, as I was in with Violet I saw only 2 people performing MRI and then 1 going off to get more people and ended up with 5 people. Then when they had finished the scan, they would not tell me anything, even when I asked, so we still did not know what the scan had revealed.

The next day we were told, she had a brain condition called Hydrocephalus. The name originates from the Greek words ‘hydro’ for water and ‘cephalus’ for head.

We all have what's called cerebro-spinal fluid (CSF), it is a clear fluid which is made up of water, protein, sugar and minerals and flows from one ventricle to the next, circulating around the brain and down the spinal cord. The CSF is then absorbed into your bloodstream.

Hydrocephalus is when an event has occurred, which causes the cerebro-spinal fluid (CSF) to accumulate. The pressure then starts to build up within the skull. When the ventricles swell to a large size, the excess fluid can then cause pressure on the brain cells and cause damage.

Violet's Hydrocephalus is Congenital (meaning - present at birth) and Doctors confirmed that sometime during her development, she had many brain hemorrages, from which the blood has plugged up the brain's CSF pathways or ventricles, no longer being able to drain away, as they should.

They measured her head circumference daily. The black texta marks on her head (see photo above)are so they can be as accurate as possible everytime they measured.

...Violet's MRI, showing her Hydrocephalus

As you can clearly see the white area is her left ventricle and that is all a buildup of CSF (MRI makes mirror image, looks like right, but it is the left). You can also see from comparing the right side of her brain, how much her left side of her brain has no room to move or expand. Her right ventricle is still bigger than it should be, due to the left having such a buildup, it has been affected too. But it is still closer to the size a normal ventricle should be.

Her left eye's condition is called Microphthalmia, which means small eye. The eye started to develop during pregnancy but for some reason stopped. Due to this condition, the eye is underdeveloped and it has caused Violet's left eye to be blind. This eye also has Leucocoria (White Pupil) and PHPV (Persistant Hyperplastic Primary Vitreous) which means the first (primary) vitreous that grows in the eye fails to become clear. It grows too much (hyperplastic) and becomes hazy and scarred. Usually it would disappear and become clear but instead it stays (persists). The hazy vitreous blocks light passing to the back of the eye. PHPV can lead to other eye conditions developing like glaucoma, cataracts etc.

...Violet's MRI, showing her Microphthalmia.

A huge difference between normal sized eyeball and Micro eye.

I will never forget the day they showed us her MRI showing her left eye and telling us that she would be blind in her left eye, it was the worst day of my life. Words can not describe how it makes you feel to hear your baby is blind, even though it is only 1 eye.

Although her right eye seems okay, it's unknown just how good her vision is in that eye, until she is old enough communicate and respond about it with us. To us, she seems to see quite well with it.

On 25/8/05, The Opthamologist Registrar came to see Violet to examine her eye, which I could not stay to watch, as they use a metal type frame to hold eye open, while they look right behind her eye, the screaming was unbearable enough. They also did a few blood tests today.

On 26/8/05, a more senior Opthamologist came to do the same examination that was done the day before.

On 28/8/05 Violet had an eye ultrasound,to have a further look at her eye condition. She also had a renal (kidney) ultrasound, her kidneys were fine. Did a few more blood tests today.

Doctors also noticed Violet's left side of her body was smaller in general, not the length of her limbs, but the muscle tone in her left leg, arm, bottom and labia were smaller than her right side. Her left side of her face has a red/flushed look and all over her chest and back she has a red veiny/lacey looking rash, which we have been told is a vascular problem.

Although, doctors have confirmed her brain hemorrhage, we still have no explanations what caused this and her other problems, and we may never know. Geneticists have been seeing Violet, but as yet no answers.

These 2 weeks in hospital did at least in some way prepare us for what it would be like when she came back for her operations.....although we were soon to learn it is never easy. They discharged us to see how things progressed over the next month or so.

Things didn't get any better with her Hydrocephalus. With her head size still rapidly increasing, on 24/10/05 she had a CT Scan. This confirmed the need to intervene surgically.

She has had 2 brain surgeries, so far.

Her 1st Operation was on 2/11/05, when she was 2½ months old and involved what's called Endoscopic Third Ventriculostomy. This was to make holes between her ventricles, so that the excess CSF would hopefully escape to her other side that appeared to be draining already and to insert a Rickham's Reservoir, which is a small silicone device inserted into the front of her head. The reservoir is used to access CSF for diagnostic purposes. It stays there for as long as infections stay away.

.....Photo of a Rickham's Reservoir (Photo from 'Hydrocephalus - A handbook for parents and patients')

The day after any of Violet's Operations, she is always like a rag doll and barely opens her eyes. All she seems to do is sleep and cry. I hold her most of the day after, just to reassure her. She develops high temperatures for the first few days after her op's and has to be on antibiotics to try to prevent any infections.

On 3/11/05, she had a CT Scan.

Photos post op of Endoscopic Third Ventriculostomy and Rickham's Reservoir Op.

Unfortunately her Endoscopic Third Ventriculostomy operation, was not successful in controlling her Hydrocephalus and CSF was still building up. Her 2nd Operation was on 18/1/06, when she was 5 months old and involved inserting a Ventriculoperitoneal (VP) Shunt, which is an operation performed to place a catheter into her ventricle and drain cerebrospinal fluid (CSF) from the ventricular system into the peritoneal space (abdomen) This catheter is connected to a pressure valve that regulates the amount of fluid drainage.

....VP Shunt (Photo from 'Hydrocephalus - A handbook for parents and patients')

...Photo of Hakim Shunt (Photo from 'Hydrocephalus - A handbook for parents and patients')

On 19/1/06, she had a CT Scan.

Photos from post op of VP Shunt Op...

Hydrocephalus is a life-long, life-threatening neurological condition, that is treated by a shunt, but it is not a cure.

We now have to always carry a 'Hydrocephalus Shunt' Card. This shows that she has a shunt, what kind it is, what shunt valves and reservoir have been inserted. Also, lets other people know symptoms of shunt


Some problems with Shunts are:

*Shunt Blockage
*Shunt Infection
*Shunt Disconnection

Some of the symptoms associated with the above Shunt problems are:

* Vomiting
* Loss of appetite
* Seizures/Fits
* Irritability and changes in mood or personality
* Drowsiness
* Headache
* Vision Problems
* A persistent fever
* Redness or swelling along any of the shunt system
* Swelling or tenderness of the abdomen (with VP shunt)
* Changes in cognitive ability

There are many other problems that can be associated with Hydrocephalus in general and vary for each person, some problems are:

* Learning disabilities
* Motor skill disabilities
* Memory deficits
* Behavioural problems
* Psychological deficits
* Vision problems
* Hearing difficulties
* Seizures/fits
* Hormonal imbalances
* Sensitivity to sound, pressure and light

So far we know Violet is delayed developmentally. The right side of her body has very limited function. It has increased tone or muscle spasticity, making it difficult for her to use. She has to wear arm and hand splints for weight bearing, to keep her hand open and her arm stretched out. She has been attending Physiotherapy and Occupational Therapy since she was 4 months.

As for her eye condition, it is in-operable and she has to wear a scleral shell, which she has been wearing since she was 4 months. This will stimulate the tissue around the eye to make the eye socket grow, so she can look as normal cosmetically, as possible as she gets older.

She has to have an EUA (Examination under Anesthetic)once a year until she is about 5 years old, for the ocularist to take a mould of her eye, to get an exact fit, from this he can add to the shell as she grows in between the mouldings. She has only had 1 moulding so far. She has clear shells for now, but in the next year she will get a hand painted shell to match her good eye. She is on her 3rd clear shell, so seems to be going well.

Photo's above are of eye inflammation from conformer - Her conformers at times make her eye inflammed and closed for a couple of days, then once it has had a couple of days rest and has opened again, we can try to put it back in again.

I would love to hear from parents of children with either of the conditions my daughter has, as it would be great to hear your experiences and knowledge


Roxy said...

I am so sorry to hear about that.
I think your daughters are absolutely gorgeous....and
i will be praying that you have the courage and funds to make it through. Good Luck..and peace to you.

Courtney said...

I read your story and my heart goes out to you. I have a four month old son, Tyler, who was born with PHPV and microphthalmia in his right eye. He was diagnoised with the condition the day after we left the hospital.
Since then we have visited many doctors and hospitals. All with the same bad news, being that nothing can be done.
I can't help but feel guilty, like I did something wrong! I had a healthy pregnancy, and did all the right things.
I get fearful to have another child, I don't want the same thing to happen, yet I can't help but want another joyful little one around.
The only thing that I can say, is that my heart goes out to you, I remember the feelings that my husband and I had, when we found out Tyler was blind in his right eye. I have never hurt so bad in my life. He was our first child.
It is amazing though how happy and joyful he is.
He is my little blessing

Corrie said...

what a beautiful little girl you have and thank you for sharing your story and so much information

she is a credit to you and your family and must charm everyone she meets

I just discovered this condition on EB when someone had terminated a pregnancy and I had found it so upsetting...seeing your beautiful little girl makes me realise how special life is and how God sends us here with a purpose and your little daughter is living proof!

I'll be coming back to check out all the cute photos and so glad she's walking! my little one is walking and I love watching her little legs and bottom move all around the house!
thank you

Anonymous said...

I also have a litte boy who has a vp shunt and he is blind my heart goes out to you.

Lisa said...

Thank you for sharing your families journey. I have just begun reading and will come back to check progress reports. I admire your courage, your strength. What a treasure Violet is!

bluelady said...

hi I just want to say hello
I too have hydrocephalus and i made it to 35 without intervention. I had a third veent op done in 2003 and so far i am doing ok

sending you and your family warm healing hugs and best wishes for a long healthy life

hugs from
kathy robson aged 38

lithgow australia

Josephine said...

My daughter was born in February 2006 with hydrocephalus, so reading your blog about your time in the hospital brought back some memories for me. We found out about the hydrocephalus about 2 weeks before she was born, and she had her shunt placed when she was 2 days old. I was sorry to hear about your experience with the hospital not being as communicative and helpful as they should have been - we were blessed with some wonderful nurses and doctors who did an excellent job of keeping us informed and feeling looked after during that horrible time.

allie_Jade said...

You have 2 very beutiful kids. My heart goes out to you guys. I was born with Microphthamia. Although its hard to get through at times. I truly believe its made me a stronger person. We are special individuals. ;-)

Melissa said...

My Son was Diagnosed with Hydrocephalus due to two hemorrages that he acquired at birth. He was born 14 weeks premature and had both a grade 3 and four bleed in his brain which than blocked the flow of his csf. he couldnt get a shunt until about 2 months after he was born because of his very low birth weight he was 1lb 13ozwhen he was born and they wanted him to be atleast 4lbs before they could do it. First they put a Subgaleal shunt in that didnt work and two weeks later converted it over to a VP shunt which worked much much to quickly the first night.
his head went down 3 cm in an hour it was completely sunkin in it was awful. they feared he would have developed more bleeding in his brain because of the change in size was so quick. but thank the lord they found nothing.

Now he is 3 months and 1 week old. Monday Aug 25 is his ACTUAL due date. and they are thinking about sending him home On the 27th of Aug.

His head isnt shaped perfectly yet. His bones in his head shift a lot because they arent fused yet but they will we just have to rotate his head a lot.

god bless your family and your beautiful little girl.

Tina said...

my daughter was diagonised with hydrocephalus and hemihypetrophy.
she was done an ETV at 4mths and luckily it worked so there was no need of shunting.
thank you for sharing your story with us. makes me feel stronger to keep up the faith and allow what cards life is dealing me.
God bless you all and happy new year.

Jude said...

I'm speechless and in tears after reading this... having a child is an absolute blessing, the love you have for them overwhelming, I cannot even imagine the pain you must feel watching Violet have to deal with all this. I look at my two boys and thank my lucky stars they are healthy, and I send all the love, light and luck imaginable your way for you and Violet to overcome the challenges you face. I have so much respect for you, for all parents in a similar position... xoxox

Shannon said...

Thank you so much for sharing your story. My son , Dominic, is 7 weeks old and was born with Microphthalmia and PHPV in his right eye. We were completely devastated when we heard the news. Thank God we found a wonderful Ocularist who answered all of our questions and really helped releive some of our sadness. Dominic just got his first Scleral Shell yesterday September 1, 2009. I know we face a long hard road ahead but I pray every day that he stays healthy and the sight in his left eye is good. You have beautiful children and I wish nothing but the best for you and your family. God bless you all!

Anonymous said...

wow this is an amazing story. i am 14 years old and found out my little sis has hydrocephalus. i've been crying and crying and your story gives us all hope that god is with us. To all of you who read this may you please pray for my little sis anf to all the others struggling with this disease.
god bless you all

skye martin said...

you have a lovely daughter,thank you 4 shearing your story i know i am not alone anymore,there are lots of children with son has dandy walker syndrome (DWM)witch caused his hydrocephalus he had a vp shunt at a day old he is now 22 weeks and his shunt has already failed.

skye martin said...

i feel fir you my son has got dandy walker syndrome witch has caused his hydrocephalus he had a vp shunt at a day old.

Jennifer said...

I'm sorry to hear of all the hard things your daughter has had to deal with due to her hydrocephalus. I was born with this condition and, due to the Lord's great blessing, have had few additional physical complications to due with. Please learn to draw near to Him and beg His help on a continual basis. It really does help!!! I pray you're able to deal with this and that she is able to lead a near normal life. I feel so bad for you . . .

Mattías Flachsland said...

Hi, thanks for sharing your story. Hopefully it will help to maintain people's awareness and a need in scientist to keep working to preserve lives. I just wanted to say that today doctor Hakim passed away. He was the inventor of the Hakim-valve-shunt that saves lives everyday.
He was 89 years old. He is survived by his 4 children and wife. Three of his children are in the medical field and are constantly fighting to develop more treatment and better equipment. Hopefully we will all leave to see better days and better lives for people with hydrocephalus. God Bless you! and thank you doctor Hakim, Little Violet is a happy little girl and a prove of your courage and persistence.

Vangie Cintron said...

Hello my name is Vangie and Im a 28 yr old with hydrocephalus. I had a VP shunt put in twon months when i was born. I have had 12 surgeries throughout my lifetime and now Im a mother to a 1 yr old healthy baby boy. If you want to read my story find me on facbook under Vangie Cintron. thank you

Big brother, Little sister. said...

Sara h, I think this is the first time I have read all this story, and yes it has me crying again. wow what a journey you and Violet and the family have had. I am just so grateful to have met you and Violet in real life and online xx love to you both x

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