Blog 20 - Op #5 for Melissa - 3 1/2 years - VP Shunt - & Speech Therapy

Operation 5 for Melissa - December 2004 - Insertion of VP Shunt

After the dura and skull operation the neurosurgeon told us that the problem had been caused by hydrocephalus. He told us that the build up of cerebrospinal fluid in Melissa's brain was the cause of the brain being pushed up and out of the back of her head. The neurosurgeon told us that she needed to have a shunt inserted as soon as possible to help drain the excess fluid. He was going to do it before we went home but he had to do a baby urgently so we were told to go home and we would be contacted to come back in the next week to have it done.
This was a shock to hear. Though when we thought about it, we remembered that we were told that Melissa had hydrocephalus before her bifrontal orbital advancement at 18 months but the operation was supposed to have rectified it. Obviously it had not. It had not been mentioned since that operation which is why we had forgotten about it.

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What is hydrocephalus?
From my research I've found that cerebrospinal fluid is found within the brain and surrounds the brain and the spinal cord. It is a clear, watery substance that helps to cushion the brain and spinal cord from injury. This fluid circulates through channels around the spinal cord and brain, constantly being absorbed and replenished. It is within hollow channels in the brain, called ventricles, where the fluid is produced. A specialized structure within each ventricle, called the choroid plexus, is responsible for the majority of CSF production. The brain normally maintains a balance between the amount of cerebrospinal fluid that is absorbed and the amount that is produced. Often, disruptions in the system occur. Hydrocephalus occurs when there is a blockage in the pathways through which the fluid normally travels or it results from an overproduction of fluid or a difficulty in absorbing the fluid that is produced. Because the brain is enclosed within the bony skull, the extra fluid, trapped by blocked pathways, has no escape. This extra fluid within the brain will produce increased pressure symptoms: headaches, vomiting, drowsiness and in some cases, confusion. (http://www.neurosurgerytoday.org/what/patient_e/anatomy1.asp)

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We did not hear from the hospital until the Friday of the next week. I had tried during the week to find out from the hospital if an appointment had been made but my enquiries led nowhere. We were just about to get in the car to go to the hospital to see the craniofacial clinic when we got the appointment in the mail. Melissa was to go in the following Tuesday.
We didn't know what a shunt was and hadn't had the operation explained to us, so the neurosurgeon's registrar showed us what a shunt was and explained its purpose and what would be happening in the operation.
We found out that a shunt is a drain. It is placed in the middle of the brain in the ventricles where the cerebrospinal fluid collects and it takes away the excess fluid. The drain takes the fluid down to the stomach. See the diagram.

We were uneasy about Melissa having a shunt but again there was no alternative. If she did not have a shunt we were told she could have more blow outs of her dura and skull, requiring more surgery, more pressure on her brain would occur and pressure would also be placed on the back of her eyes which could lead to blindness.

After the operation the neurosurgeon said that the shunt was definitely needed as it had started to drain before they had finished inserting the bottom end into the stomach.
Melissa was wobbly for a few days and went off her food. She had a very sore tummy for a few weeks but got back to her old self slowly. She had the stitches taken out of her head and tummy 9 days after surgery.

The operation took about 2 hours and she was in hospital for 4 days.
We were given information on shunts and what the warning signs to look for are because shunts can become blocked. These are things like vomiting, headaches, loss of appetite, excessive sleepiness, irritability, dizziness, fever, double vision, clumsiness, change of personality. We will be watching Melissa closely.

Speech Therapy for Melissa and a Question of Hyperactivity

Melissa did not have very good speech. We could not understand much of what she was saying and she would throw very big temper tantrums in response to us not understanding her. No one told us about sign language for toddlers which would have helped Melissa greatly at that time.


Melissa was deeply attached to her dummy and would either try to speak with it in her mouth, or she would not speak at all....so the first step was to get rid of it!

I was the big bad mum who hid it on top of the fridge and told her that I had thrown it away because she was a big girl now and didn't need it. I decided that 'cold turkey' would be the best method for her - whether it was or not I don't know but it worked.

Melissa cried for a whole day but I didn't give in.

The next day she was over it and had accepted that the dummy was gone.

The speech therapist tried to work with Melissa but Melissa was too active, wouldn't sit still and wasn't interested. The speech therapist suggested that we have her tested for hyperactivity...

We saw the paediatrician to have the hyperactivity question answered. After talking to us about Melissa's behaviours he stated that she was an 'active' girl, not a 'hyperactive' girl.

We delayed the speech therapy sessions for another year until we felt she was able to sit still.

When she went back, Melissa was a different girl. She sat and she learnt and her speech improved out of sight....and so did her temper tantrums!

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission.

Blog 19 - Op #4 for Melissa - 3 1/2 years - Cranioplasty

Operation 4 for Melissa - November 2004 - Repair of Dura and Cranioplasty

We noticed soon after Melissa's bifrontal orbital advancement at 18 months that a lump started to grow beside her ear to ear scar. We brought this lump to the doctor's attention at Melissa's annual craniofacial clinic appointment in the following January. The plastic surgeon was not too worried about it but said he would keep an eye on it. We noticed that it was growing bigger so each time we took Nick in for an appointment with the plastic surgeon we would bring it to his attention. In about April he decided that it needed checking. He sent us to the neurosurgeon for his opinion. The neurosurgeon sent Melissa for a CT scan. It came back as showing that the dura was being pushed out through the weak skull bone. The skull bone had separated and there was no bone protecting the dura. This worried us but the neurosurgeon said that it needed to be fixed but that it was not urgent. He told us that this sometimes happens after craniofacial surgery.
The lump continued to grow and we started to become more concerned. After many enquiries to the hospital as to when this operation would be done we were told that it was scheduled for 4 weeks after Nick's frontal orbital advancement.

They did not want to put the operations too close together so we could rest inbetween (which did not happen in the end). As it turned out the operation was brought forward due to a cancellation. Nick was discharged and Melissa admitted on the same night. I did not leave the hospital for 3 weeks.

After Melissa had been wheeled into the OR I rang my boss to tell him that I would be extending my leave and explained the situation. He was very angry with me. He always told me that 'family comes first'...but I soon realised that if it inconvenienced him, then it didn't. After being berrated by him on the phone, and after what I had just been through with Nick, I dissolved into inconsolable tears.

Before the operation we brought to the neurosurgeon's attention that the lump had gotten bigger since the last CT scan so Melissa had another one done. From this it was thought that the top membrane of the dura had a tear and the bottom part of the dura was pushing up through it. It was obvious on the CT scan that there was no bone covering the area.

When the neurosurgeon went inside the situation was worse then what the CT scan showed. He found that there was a tear in the top dura (Dura Mater) and the bottom membranes (Arachnoid and Pia Mater) and the brain were pushing up through it. This meant that the neurosurgeon had to push the brain back down. He then had to repair the Dura Mater. To do this he took the lining of the muscle in Melissa's thigh and used that to strengthen the membrane. He said that it was weak due to scar tissue from the previous operations.

The second part of the operation was called a 'cranioplasty'. The word cranioplasty is a technical name for the reconstruction or reshaping of the skull. A cranioplasty can be performed using a number of techniques and materials, including bone grafts, metal plates, plastic, and mineral matrix materials.

In Melissa's case the cranioplasty involved the plastic surgeon filling in the gap where the skull was missing with a plate. He then put bone fragments on top of this plate. We have been told that Melissa's body will grow its own bone over the top of the plate so there would eventually be no hole. There is though still a dent.

The neurosurgeon was worried about complications from having to touch the brain. The area that could have been affected was the right hand side of Melissa's body. He was pretty certain that she would not have been able to walk again....but our God is gracious and was watching over her. Melissa is able to walk, jump, skip, swim. 

The operation took about 4 hours and she was in hospital for 6 days.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission.

Blog 18 - Nick's First and Second Op - 9 months - Frontal Orbital Advancement

Operation 1 for Nick - November 2004 - Frontal Orbital Advancement

Nicholas was initially introduced to the plastic surgeon when he was five weeks old. The plastic surgeon told us that Nick had trigonocephaly. Trigonocephaly is the name given to the type of craniosynostosis that occurs when the metopic suture fuses early. When Nick was born there was a ridge running down the centre of his forehead which was the fused metopic suture. See the photo above which was taken when Nick was 4 days old. As Nick got older his forehead became more triangular in shape and his eyes looked more and more prominent. He developed a receded forehead and he could roll his eyes up and see us above him.

Nicholas was monitored by the craniofacial clinic and at 3 months had a CT scan. This showed slight pressure on the brain. It was decided that a frontal orbital advancement would be done in the next six months.
At 8 months Nicholas had his first operation. In this operation the plastic surgeon removed the fused bone running down the centre of his forehead. Then he reshaped Nick's forehead and brought the right and left hand sides of his forehead forward to match the middle and then brought the whole forehead quite a way forward. The purpose of bringing the forehead forward was to provide protection for Nick's eyes as well as allowing room for the brain to grow. The doctors also removed a dermoid cyst which was growing under the skin above his left eye. A dermoid cyst is a saclike growth that is present at birth. It contains structures such as hair, fluid, or skin glands that can be found in the skin.
We were told after the operation that Nick had pressure on his brain which may explain some of his behaviours that he was exhibiting before the operation. For a while before the operation Nick was irritable, he vomited, he would pull on his ears and he would cry. We put all of this down to teething. After the operation this behaviour stopped so we think that it was probably due to the pressure.
The operation took about 4 1/2 hours. Nick went to ICU for 24 hours. He was not very swollen, did not need to be ventilated and did not have as many tubes coming out of him as Melissa had so we did not feel as stressed as we had with her. The doctors told us that everything had gone very smoothly so that was even better. But when we saw him the next morning we got a shock. His eyes had become very swollen overnight and turned reddish-purple. The swelling got worse during the day.

Swelling:
Nick went back to the ward 24 hours after being in ICU. I held him up most of the day, every day, hoping that the fluid would drain from his eyes but it did not. Nick's swelling became severe. His little eyes looked like enormous purpley-red over-sized plums. They became firmly swollen shut. The swelling was much more severe then Melissa's. The plastic surgeon said that they were at the very severe end of swelling.

Melissa saw Nick on day three but would not look at him. Even though Joe tried to prepare her for what she would see, she did not cope very well. Melissa did not like 'what was on his eyes' - she didn't understand that they were his eyes. It scared her. She did not want to go back to the hospital to see him. She wanted the old Nicky back.
It took 5 days for Nick's eyes to open. On the Sunday following the operation Nick got very distressed whilst the doctors were trying to take blood for a blood test and this caused him to open them. After that the swelling started coming down much more quickly.
Nick found it hard having his eyes swollen shut. By the fifth day he was starting to feel better in himself but he began to get quite upset with not being able to see. It was a huge relief when he could.

Suspected Meningitis:
Two days after Nick's operation he developed a sudden high temperature and tests were done to see if a reason could be found. The doctors thought it may have been something like an urinary tract infection from having a catheter. No cause could be found but the CRPs in his blood, which fight infection, were up (normal is 6, they were 68) so there was something going on. The Infections Diseases doctor decided to treat the infection as Meningitis. It was unconfirmed as Meningitis. The only way to confirm it was to give Nick a lumber punch which the ID doctor did not want to do. So he was given a broad spectrum antibiotic and the temperature came down. Nick was put into an isolation room which he was unable to leave for 9 days.



Operation 2 - Insertion of PICC Line

Two days after being on the antibiotics Nick's IV line stopped working. Three doctors tried to find a vein where they could put another one but no one was able. That was a very traumatic ordeal to witness. Having your baby being used as a pin cushion is difficult to watch. Your motherly or fatherly instinct is to get your baby out of there but you can't. So on the Saturday afternoon Nick went back into theatre to have a PICC line put in. It is a internal semi-permanent tube that went in through Nick's foot all the way up his body to an artery in his chest. This operation took 1 1/2 hours. The waiting was stressful. The operation being 4 days after his first one was stressful enough in itself and then we weren't told how long this operation would take. We were really starting to get worried as the time dragged on. The PICC line lasted for 9 days and then stopped working. Nick was to have the antibiotics for one more day but it was decided that they could be stopped, otherwise he needed to have injections in his bottom which was deemed too cruel if it could be avoided.
Nick was given a blood test two weeks after his operation, and his CRPs had gone down to 13. We were told that that was satisfactory and that he could go home. That was nice to hear, except that Melissa had been admitted during that time so I wasn't going anywhere... But Nick got to go home.
It must be noted that the plastic surgeon did not think Nick had Meningitis. He believed that the temperature was caused by the body's reaction to the severe swelling. The neurosurgeon said that he didn't think there was any way that the Meningitis could have gotten to the brain during the operation but he said that the infectious diseases doctors knew what they were doing. We also heard on the grapeview from other parents that there had been other babies come down with the same symptoms and that it may have come from the operating theatre. We will never know but the main thing is Nicholas doesn't seem to have suffered any ill-effects from it.
The photo below was taken 12 days after Nick's operation. His eyes were still bruised and slightly swollen.

Emotional Roller Coaster:
A roller coaster ride of emotions was what I experienced leading up to Nick's operation. There are the initial emotions when the plastic surgeon tells you that he is going to operate. Then you get another pump of adrenalin when you receive the booking letter in the mail. Then your mind goes through all of the 'what ifs' and 'will he be ok?' etc. You get yourself psyched up for the operation - you know it will be a long wait, your baby's head is being cut open, they will be going to PICU, they will have swelling...it tears your heart apart and circles in your mind. 
At our hospital there is no guarantee that the operation will be on until the morning of the op, due to limited PICU beds, so even though you are in hospital (the baby is admitted the day before for blood tests and general observation) you still are unsure as to what's going to happen until that morning.
You then give your precious baby over to the surgeons which is one of the hardest things in the world to do. Even though you know they are competent there are always those things called complications... Then the wait happens. You worry for hours until you hear that your baby is out of theatre. At our hospital there is a parent waiting lounge. As the hours tick by you watch a lot of other parents come and go as their children come out of theatre and that in itself is stressful. When the operation is over there is the relief that everything went well (or more stress if there were complications) and then the maternal instinct to want to get into PICU to see your baby, but having to wait until they are ready.
When you are allowed into PICU you see your baby lying there and all sorts of emotions race through your head. This time it was not too stressful and we felt even relief as Nick looked good and we knew that everything had gone well. The next morning though when we saw that Nick's swelling had increased we were a little stressed but more disappointed as we had thought he was going to miss out on the swelling. Of course at that time we did not know that he would develop such severe swelling and stay so swollen for so long.
Being released from PICU is good because you know that's the next step in the recovery. You know that once your baby is in the ward that you are on the downward side of the hill.
But when Nick had the temperature our stress levels rose sharply mainly from the unknown. Waiting for the test results, waiting for the doctors to decide what they were going to do, having the temperature keep rising, and then be told it might be Meningitis which is a terrible disease with serious consequences if not caught in time. The stress then came down when Nick started the antibiotics and his temperature did come down. You put your faith in the infectious diseases doctors.
After that you are so very tired but have to keep on being mummy. You run on lack of sleep and maternal love. Nick only wanted me during his fortnight in hospital which didn't give me much of a break. But even though I was so exhausted and stressed I would not have not been there for him.

Recovery at Home:
It was good to get Nick home after the operation. In the second week in hospital he began mixing up night and day and I wasn't able to get much sleep. Melissa and Joe would come during the day so I would spend time with Melissa, giving her attention, and then Nick would be awake for half the night. It became like torture. On one night I became very upset. The nurse had attached an alarm to Nick because he was sleeping on his stomach. The alarm went off but no one came to check on him or to turn it off, for what seemed like an eternity. Nick got more distressed the longer the alarm went on and I got more distressed, the more distressed he got. Anyway I cried and cried and cried which I think I needed. I had been so strong for so long. Anyway the alarm did not get turned back on. Melissa's nights in hospital were so much more easier.

Outcome:
This operation changed the shape of Nick's forehead and because his forehead has been advanced, he now has protection for his eyes and they look smaller then they did. His left eye though is more prominent then his right, as it was before his operation.
Once Nick was home and back in his normal routine we noticed a great improvement in his behaviour. He ate and drank so much more, he was more happy, his speech improved and his general development progressed more quickly. Nick had an overactive tear duct in his left eye before the operation, it too was fixed. We were happy. The one thing we did notice was that Nick still had a slight ridge down towards his nose. You can see it in the photo below. We were told that this was the fused metopic suture bone. In the operation the plastic surgeon had flattened the bone (a bit like panel beating). He said that he was not able to cut out the ridge because the bone was too thin.

Fused Lamboid Sutures:

We didn't see the doctors for about 5 months prior to the operation and in that time Nick's other sutures had fused. The fusion of his lamboid sutures at the back of Nick's head made the back of his head flat. We brought this to the doctor's attention but he said that doing the front was much more important and that Nick's hair (when it eventually grows!) would cover the flat spot. Time will tell if anything will need doing. This photo was taken at 10 months looking down the back of his head.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission.

Blog 17 - The Birth of Nick and Op #3 for Melissa - 3 years - Delta Hallux

We took Nick to see the plastic surgeon when he was 5 weeks old. Nick had a metopic ridge down his forehead which would need to be re-opened. It was decided that they would do his frontal obital advancement first (the opposite to Melissa). Interestingly, because he metopic suture had fused early meant that his eyes were not wide set which is usual in Crouzons. The fusion had pulled his eyes together. He was different. Genetic testing was done and as expected Nick had the same gene mutation as myself and Melissa. More of this, and Nick's operation, is explained in the next blog.

I did not suffer from depression because I felt more in control. Nick also could not breastfeed well because of his facial shape. I managed to get him to 6 weeks and then he went on the bottle.

Nick suffered from silent reflux which was picked up by the child health nurses. He kept his head tilted to the left and we needed to place him on his right side to lengthen those muscles in his neck.

During this time Melissa's curved big toes were being monitored. That had worried us from birth. They looked to us like she had hands on her feet when she was born. Over time Melissa gained more control over her toes and could bring them together and they did not look as weird. The big gap between the big toe and 2nd toe stayed.

We had more concerns with Melissa's feet when she started to walk. They didn't look too bad when she was sitting as she could pull them together, but when she walked the pressure made her toes really curve outwards. We do not have a photo of how bad they were when she walked. We took her to see the orthopedic surgeon who diagnosed her as having delta halluxes. This meant that the bone in the big toe was growing curved. She had plastic inserts in her shoes for a year but they did nothing to help. Melissa was fine when she was wearing shoes but as soon as she went barefoot she would trip over her big toes, hold them and cry in agony.

Something needed to be done. Surgeons do not like to perform operations on growing toes unless absolutely necessary because the growth plates can be affected.

Operation 3 for Melissa - May 2004 - Delta Hallux Surgery

When she turned three, the orthopedic surgeon said that she needed surgery to straighten the bones in the big toes. He took out a triangular wedge of bone from within the bone to straighten it. This operation also shortened the big toe. Melissa was in plaster for 6 weeks with pins in her toes. The physiotherapist put shoes on her casts so she could walk. These casts did not slow her down. The physio was flabbergasted by Melissa as she told us that Melissa was the first child she had seen who as soon as they were up took off without any hesitation. Melissa had made cookies out of playdough and she wanted to get to the play oven to bake them! And she did.

One of the pins in her toes came out prematurely at about a month and the other was taken out after 6 weeks, in theatre. After having the casts off Melissa needed physiotherapy to help build up her calf muscles and to help the development of her gross motor skills. Her toes are now a lot straighter and she can go barefoot with no real problems. She still has the gap and will probably only be able to wear certain types of shoes. Time will tell.

In February 2005 we took Melissa back to see her surgeon who was very happy with the results. One of her big toes looks like it is slightly twisted but a x-ray showed that the bone is straight. Melissa still kicks her big toes occasionally which we told the surgeon. His suggestion was that she wear shoes to help. That was Melissa's last appointment with him.

Nick's big toes were also examined by the doctor. His toes are not as severe as Melissa's but he still has the gap and the curve. When Nick tries to walk his big toes curve outwards just like Melissa's did. The surgeon said that Nick does have the same thing and will need to have the corrective surgery when he is 2 1/2 years old.

Post-script. Melissa's and Nick's toes are monitored each year. Unless their toes cause them pain they will not be operated on until the toes have stopped growing.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission.

Blog 16 - Another Miscarriage, Then Baby Makes Two

When Melissa was a year old I found that I was pregnant again. We once again were ecstatic.

Everything was going well and we saw the baby and the heart beating on an ultrasound. A week later though whilst at school, I started to bleed again. Another ultrasound. This one revealed that the baby had died.

We were devastated again. It was doubly devastating as my father had passed away a few weeks before. It wasn't fair I wanted to scream. Two deaths too soon.

I went into hospital the next morning and had another D & C. Again no counselling. We dealt with it on our own. Another baby whose cry we would never hear.

As part of my grieving I made a memorial plaque for my two precious angel babies and hung it up in my bedroom.

After this pregnancy I couldn't conceive. The obstetrician put me on fertility drugs and I had to fill in temperature charts each month. Everything was timed to maximise the possibility of pregnancy occurring. This was not a pleasant time. Each month waiting, hoping, then no pregnancy.

Eventually we did conceive and we celebrated cautiously.

This baby though was strong and caused no problems. Again he started off as Down syndrome with the thick nuchal translucency and again like Melissa, this was discounted. By the time Nick was born the technicians were pretty certain that he had Crouzons too.

Nick was born in 2004. He came 3 1/2 weeks early in the middle of the night, in the middle of a thunderstorm after a stinking hot day. Mum couldn't come to babysit so Melissa was taken to the hospital. My husband had to look after her so I went into the operating theatre on my own.

When Nick was born he didn't cry. I panicked thinking he must have been stillborn. I cried out to the doctors to tell me if he was alright. They told me he was ok...but he did need oxygen and a massage. Until I saw him moving I was not convinced that he was alright.

When they showed Nick to me I saw his bulgy eyes straight away so I didn't need to ask....I knew he had Crouzons too and I fell in love with him instantly.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission.

Blog 15 - Op #2 for Melissa - 18 months - Bifrontal Orbital Advancement

Operation 2 for Melissa - November 2002 - Bifrontal Orbital Advancement

By about 12 months we noticed that the sagittal suture was nearly fused. Melissa would cry a lot and hold her head as if she had a headache, and her brain had started to grow out of the back of her skull where the doctors had left soft spots, like it did out through the top last time. We alerted the doctors to this and Melissa had another CT scan. This scan showed that her skull was too small for her brain again and that she had pressure on her brain. Holes had started to be worn in the skull from the pressure. She also had mild hydrocephalus (fluid on the brain).

When Melissa was 18 months old she underwent her second operation. This was a 'bi-frontal orbital advancement' where the doctors reshaped her asymmetrical forehead and advanced it. Her forehead was an odd shape due to the coronal and metopic sutures being fused from birth. If you look at her pre-op photo which was taken the day before her operation you can see that she has a receded forehead and the left hand side of her face is different to the right hand side. The doctors brought her forehead forward to be infront of her eyes. The left hand side of her forehead needed to be brought quite a way more forward to match the right hand side of her forehead. Melissa had a strabismus in her left eye which meant that her left eye was very off centre (you can see this in her pre- photos) and this operation helped bring the eye to face more to the front. You can see the difference in the two photos below. The operation took about 6 hours.

We had problems getting this operation done. After the neurosurgeon saw the CT scan he said that she needed the operation but he did not write it down in Melissa's chart or communicate it to anyone so it wasn't scheduled. When it was finally scheduled the operation was cancelled due to hospital strikes. The neurosurgeon told us that he was not operating. So it was re-scheduled only to be cancelled due to lack of an PICU bed. This was a real roller coaster ride. You psyche yourself up for an operation and then to have it continuously cancelled was very frustrating and stressful. We did not know what was going on in our little girl's skull and we were worried. I ended up writing to our state Premier (the leader of our state) to try to get some help. Finally the operation was done and afterwards the plastic surgeon said that the skull had holes worn in it from the pressure of the brain and that they should have done the operation sooner....

The operation was to consist of two parts. The plastic surgeon thought that he would first fix up the soft spots at the base of Melissa's skull where the brain was pushing its way out through by putting bone grafts over them. And then he would do the frontal orbital advancement. When the skin was peeled back a blood vessel in the dura (the membrane between the brain and skull) burst. We were told that it was in some scar tissue from the previous surgery. Melissa lost 3 times her blood volume. This part of the operation was cancelled and just the frontal orbital advancement done.

I cried again when Melissa had to go into theatre. We worried about complications. I again felt guilt and had to deal with seeing her in PICU again all swollen, with her head wrapped in a bandage, ventilated and tubes coming out of all orifices. We also had to deal with the fact that we could have lost her. That was scary but made her even more precious to us.

Melissa's eyes were swollen shut soon after the operation. She was in PICU for 24 hours and then went to the ward. Her swelling was more severe then in the first operation. One of her eye lids rolled under during the operation and became scabby and crusty. It took at least a month for the eye lid to turn itself back to where it should be as the swelling went down. Melissa was in hospital for 2 weeks.
The operation made a big difference to the top of Melissa's face.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission.

Blog 14 - Post-Natal Depression & Op #1 for Melissa - 9 Months - Posterior Cranial Expansion

We took Melissa to see the plastic surgeon when she was a week old. Because she had webbed feet, curved big toes, broad thumbs and big toes, he questioned the original diagnosis of Crouzon Syndrome. He thought we might actually have Pfeiffer Syndrome. Even that was a bit of a slap in the face to me as I thought I was losing my identity.

Bloods were taken from both of us in 2002 and sent away for testing. They both came back as the same gene that produces Crouzons, so even though we were not typical, we go under the name of Crouzons.

Post-Natal Depression

At the age of 32, and a professional with three degrees, I thought looking after a baby would not be too difficult. Little did I know. I had had no experience with babies so really knew nothing except for what I read in books and I learnt about the prenatal classes.
I had the difficult baby first. Melissa cried a lot and had a lot of trouble breastfeeding. I tried my best. I did not know how to stop her from crying. I would do everything that was written in the baby books and she would still be crying. This stressed and depressed me and I often cried when she did.
A clinic nurse came to visit me at home and she found that Melissa was not gaining enough weight and I told her of my problems. I went to the baby clinic for a day and it was discovered that Melissa was not sucking deeply enough to get the hind milk and therefore I was not producing enough milk to satisfy her. So she was supplemented with formula. After 6 weeks my patience had worn out and we put her  just on formula. Once the feeding issues were sorted Melissa slept and was happy, and I settled down and began enjoying her.

When she was 3 months old I had to go back to work full-time. My husband became a house husband. I thought I would enjoy it but I hated it. I would miss a lot of Melissa's firsts and would get really angry when I found out what she had done during the day. In the end my husband stopped telling me what she was doing so I could see them for myself. Looking back I was still suffering from the post-natal depression.

At 6 months of age she had a CT scan.. Melissa had fused metopic, coronal and lamboid sutures. We had noticed that at about this time that she would hold her head and cry a lot, as if she had a headache. The CT scan showed that she had pressure on her brain and that her skull was thinning from the pressure. It was decided that the first thing that needed doing was to open the lamboid sutures. This meant a 'cranial vault expansion' of the back of her head. The purpose of this was to give the brain more room to grow as by then there was pressure on the brain and it was growing out through the top of her head, through the sagittal suture.

Melissa was 9 months old when she had this first operation called a posterior cranial vault expansion. The purpose being to make more room for the brain to grow.

Operation 1 - February 2002 - Posterior Cranial Vault Expansion

In this operation the doctors took a section of bone from the top of Melissa's head from ear to ear. This bone was then repositioned into the back of her skull to make the back of it larger and rounder. Absorbable plates and bolts were used to join the front of the skull to the newly expanded back. The doctors left a soft spot at the base of the skull for expansion and for a place for her brain to grow out through if necessary. The operation took about 5 hours.

When Melissa went in to have this first operation I had to deal with this thing called Crouzon Syndrome. I realised very quickly that I had not dealt with it at all. I had pushed a lot of feelings and hurt down into the lower regions of my heart and a lot of them re-surfaced when I saw my baby lying in PICU with purple swollen eyes, a bandage wrapped around her head, being ventilated and tubes coming out of every orifice of her body. I had cried when Melissa went into theatre from the uncertainty of what was going to happen and I cried when I saw her in PICU. Her little body looking like someone had belted her with a baseball bat. Nothing prepares you for that. The plastic surgeon told us beforehand that she would have swelling of her eyes and face even though he was working on the top and back of her skull, but they were empty words until we saw her and the emotions took over.

All sorts of emotions bubbled to the surface, with the main one being guilt. I felt guilt because I had made a decision to bring this child into the world knowing that there was a 1 in 2 chance of her having the syndrome though I prayed and hoped before and while I was pregnant that she would not. I was the one with the gene. I gave her the gene though if I could have not I would not have. It was my fault.

And I had to watch my child, and still do watch my child, go through operations and pain because of me.

I had to deal with this and work through it and talk it out with my husband.

Watching your child go through craniofacial reconstructive surgery is very difficult and only those people who have done it, know how tough it is. I did not realise when my husband and I made the decision to try for children what the surgery/ies would entail or how they would affect me or the children. You can't possibly.

My father helped me a great deal. He held my hand and told me that I looked worse then Melissa did when I went through my operations. That in some type of crazy way helped. He gave me hugs and talked to me about my operations. I took comfort in knowing he had been there for me and was still there for me and Melissa.

Melissa was in PICU for 24 hours and then went back to the ward. Her eyes became swollen shut soon after the operation. When she was back in the ward I held her upright for a day and the next day she could open her eyes. The swelling had drained away. It was like a miracle. Melissa was in hospital for a week.

We did not take any photos of Melissa's first operation as we decided that we did not want her to see what she went through.

After this op, life continued on. We loved Melissa and so did her grandparents. We would take her out to the beach and to the park, enjoying all of her milestones. Her Gramps would love to play with her. He fed her her first bowl of chocolate ice-cream. Melissa would get lots of cuddles and kisses.

My wonderful dad passed away on New Years Day the following year (2003), before Melissa's next operation. With my dad's passing our life changed.

© 2012 by Jenny Woolsey

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