My profile

INTRO

I am married to Chris, and Mummy to Jordan-Amy (9), Max (7), and Sam. Baby Sam was diagnosed at our 20 week scan with a rare condition called Congenital diapragmatic herina, and the outlook for him was bleak. We opted for a revolutionary inutero surgery to try to improve Sam's chances of survival, but sadly, he was born on Christmas eve, and died 16 hours later in my arms on Christmas day. My heart was shattered, then i had to smash Jordan and Max's hearts too on Christmas day :O(

We are blessed enough to be expecting a fourth baby. Strangely, this lil one is due 4 days off Sam's due date. He was pre-term but was due on the 22nd February 08. Because of Sam's condition, we were looked after really well and had lots of scans to make sure this lil one didn't have the same problems as Sam. At 13 weeks gestation, we were given the all clear from the fetal medicine experts at Oxford (not our local hospital) and told that our 4th baby was another little boy. I was struck with disbelief as I was so sure I was carrying a girl. Over the next couple of weeks, I was to dream on several occasions of a little girl called Hope. I was convinced I was having a girl!

At 15 weeks, we had another scan, this time at our local hospital. It was a girl! Of course, it didn't any thinking to decide on her name. It was already decided- Hope. Afterall, after losing Sam, she was our hope- our reason to look forward once more.

But it wasn't long before the happiness of finding out we were having a little girl was replaced with fear- once more. There was something wrong. Not the same as Sam, but something wrong with her head/brain. Extra fluid?

We were reffered back to our fetal medicine specialist in Oxford where it was discovered that she had too much fluid on her brain and possibly a small/abnormal cerebellum. We had to have an amniocentisis (sp?) which fortunately came back clear. Next we had an MRI where it was confirmed she had Ventriculomegaly and Cerebellum hypoplasia (small cerebellum- the part of the brain responsible for motor control, balance and speech). We were advised to terminate. We have been told that our li girl's condition is thought to be at the worst end of the scale and that she may never walk or talk. Termination was not an option, I know how it feels to kiss my baby for the last time, to tuck a baby up,not in a crib, but a coffin, and watch your dreams being lowered into the ground in a tiny white coffin. I also believe, we were sent this child for a reason. i think and hope, she'll prove them all wrong, but even if she doesn't, i will love her with all my heart!

HOPE'S MEDICAL HISTORY:

5th August 08 (11 weeks+5 gestation)

Scan report: 'The fetal stomach is not in the chest. This means that a congenital diapragmatic hernia is less likely than if it had not previously happened'.(LI) (Hurrah- she doesn't have CDH-what baby Sam died of. Oh and at this point we were told she looked like a boy. We were reffered back to our local hospital to continue our routine pregnancy.

3rd September (16 weeks)

Scan report: No DH seen (diapragmatic hernia)

This was when we were told that baby was indeed a girl

But our happiness was short lived. AJ rang us later in the day to say she'd noticed possible enlarged ventricles/hydrocephalus? and had reffered us back to Oxford.

5th September (16+3) Oxford

Scan report: 'I consider these apperances to be within normal limits. Rescan in 2 weeks with LI' (PC)

1st October (20 weeks) Swindon

Scan report: ventricles prominent 10-11mm. Cerebellum 16mm. No other fetal abnormality seen. Cephalic presentation.

7th October (18 weeks) Oxford

Scan report: Referred from Swindon- Ventriculomegaly? Previous child NND due to CDH. There is right-sided ventriculomegaly measuring 12mm for the posterior horn and the anterior horn. The left side looks normal. The cerebellum looks small for this gestation. The rest of the brain looked normal. There are no other abnormlities.

MRI arranged (performed at 21 weeks)

Infection screen

Amnio preformed

MRI Report (dated 21st October - though we recieved the findings earlier)

MRI findings 'This shows a cerebellar diameter of 14.4 mm which at 21 weeks is well below the normal range. It also confirmed unilateral ventriculomegaly, but there is no clear unifying diagnosis, particularly as the cerebellar vermis is present and the corpus callosum is normal.'

..We have discussed the fact that babies with these sorts of prenatal findings are almost invariably neuro-developmentally delayed and it is impossible to tell to what degree of severity, but she is aware that it is quite possible that the baby would neither be able to walk or talk. They are nevertheless very clear that they wish to continue with the pregnancy...I do not see that there is any need for her to deliver at Oxford or for us to scan her again , although I have arranged to see her again at 36 weeks. The mode of delivery will need to be discussed if the is marked hydrocephalus, but this is unlikely and i would have thought it appropriate for her to deliver normally without being induced'

...' Clearly the paediatrician will need to be involved after birth, but i do notseethat Oxford's neo-natal unit has any clear advantage over Swindon. I have warned her that the baby may look dismorphic and is likely to have abnormal neurological tone...'

'I cannot see any connection with these 2 major abnormalities and think it is just extremely bad luck. Certainly a geneticist has been involved and she has had an amniocentisis and this has shown a normal karyotype'

(LI) Oxford

5th November (25 weeks)

Ventricles: r=14mm l=11mm. Cerebellum 23mm. Appointment with LG (neonatal) made. Cephalic

26th November (28 weeks)

Ventricles: r=16 l=11mm. Cerebellum 25mm

, Breech presentation.

5th centile for growth HC + AC measurements

10th December (30 weeks)

Ventricles: R=17mm L=11mm. Cerebellum 30mm.

Breech

Estimated fetal weight 2lb08oz

24th December (32 weeks)

Ventricles R=17mm L=11mm

Cerebellum 30mm

Breech

Slipped below 5th centile for HC (255mm) AC 250mm

Estimated fetal weight 3lb4oz

7th January 2009 (34 weeks)

Scan report: Ventricles R=18mm L=11mm

Breech

Estimated fetal weight: just under 4lb - equivalent to a 30 week gestation baby

Head circumference: 275mm Abdominal circumference: 275mm

AJ said the cerebellum appears 'fairly normal'!!!

Oh MY God- my baby, the one Oxford would have 'terminated without question' because of her small cerebellum, could have a normal cerebellum! Only time will tell i guess.

I can't wait to meet my lil girl. To hold her in my arms. But I am also so scared. Scared of it all going wrong and coming home empty handed again- well apart from the souvineers the hospital give you when your baby dies; the hair, the hand and foot prints. I'm scared that, the worse prognosis may be right- that she'll never walk or talk, that i'll come home from hospital and have to leave her there because she's not able to feed, or because she has such poor neurological tone, just because she's too tiny to come home. Hopefully she'll make it to the 5lb mark by the time she's born. I'm eating lots of chocolate to help her in the right direction

She'll be born by c-section on the 28th January at 37 weeks
Obviously c-section is not my fisrt choice, but can you believe it, we are not having a c-section because of her problems (ventriculomegaly and small cerebellum) but because she is breech! Although they've said she may turn, as time goes by, this is becoming less and less likely. I'm pretty certain she's very happy as she is- she's been this way up for quite a while now-despite me trying the all fours postition and asking her nicely- she's not budging
I'm quite used to being able to pat her on the head which i can tell you, with her being my 4th is rather strange.

20th January

On Tuesday we went for our last official ultrasound appointment. Although of recent, we have been encouraged by the growth of her cerebellum and the only slight increase in her ventricles at Swindon' GWH, i knew we were in for some painful truths from John Radcliffe

Here's our reality check:

'This fetus has ventriculomegaly. The right ventricle measures 16mm and the left 12mm ( should be under 10mm). The cerebellum remains small for gestation at 32mm. I cannot see the cavum septum clearly (great so we've got another problem! apparently this was noted at the mri yet is not mentioned on the mri report).
Mrs Matthews is aware that there will be some degree oof handicap and that this is difficult to predict. However, this is more likely to be at the severe end of the spectrum. We have discussed this before and Mrs Matthews would prefer to wait and see (i pressume this is reffering to the fact that i could still legally terminate her )

She is delivering at 37 weeks

Please let us know how she and the baby get on'

Nice eh? I suppose i sometimes try to think, and do convince myself that she might be fine, but in reality...well..only time will tell. Not long now though. I'm having a c-section on the 28th January. Part of me is sad because she is my last baby, i'll miss having her in there, even though her head being under my ribs is causing me great discomfort. Also, im not too thrilled about the prospect of having a c-section after having 3 fairly normal deliveries.
However, I have had enough of the 'morning sickness' (yes, aren't i lucky), the heartburn, the worry about whether or not i'll get to keep this baby, worry about whether she'll go to special care, worry about whether she'll look 'dysmorphic' (oxford's term), worry about whether she'll be able to feed, whether she'll have 'poor neurological tone'. i have had enough of the uncertainty. Bring it on. Im ready to meet my little girl!

In the words of Barack Obama 'WE'VE CHOSEN HOPE OVER FEAR!

(where text is in 'speech marks' this refers to what has been said or written by the medical profession- of whom i am having less and less faith in).

Day 0- 28^th January 09

9:59 am Baby Hope born screaming! She came out bottom first by elective classical c-section weighing 4lbs 14oz

She scored 9/10 on apgar at 1 minute, and 10/10 at 5 mins. This is not what we were expecting as we were warned that she's likely have 'poor neurological tone'.

11. Hope's blood sugar was reported to be only 0.8 (should have been over 2)

11. 'Aptimil given, baby not interested. Baby became blue and floppy. Taken to resucitare. Administered facial 02. Paed called. In view of baby's condition transferred to SCBU'

Day 1- 29^th January 09

Hope is being fed by a nasal tube every 2 hours. Her blood sugar levels are improved.

We met with Dr. Zengeya . He had done an ultrasound on her head and found that her ventricles were measuring 17mm and 23mm (a scan just 2 weeks earlier has said they were 12mm and 17mm). Dr. Zengeya wasn't very positive about her prognosis. He wanted to see if she'd drink from a bottle today though didn't expect she'd be able to.

That afternoon, Hope came out of her incubator and into a 'hot cot' with breathing monitor.

At 4pm she took a feed from a bottle.

After her bottle, she had her first trip in a pram...for an MRI :O( I was gutted watching someone else take her for her first outing.

Just before midnight, Hope breastfed! Dr. Zengeya- WRONG!

Day 2-30^th January 09

'3 hourly feeds of 44ml. Combination of bf/ng tube /bottle'

Kicked out of intensive care into special care (nursery). I'm now able to do all her feeds, nappy changes etc :O)

Day 3- 31^st January 09

4 hourly feeds. Breastfeeding with bottle top ups of between 15-30ml. Ng tube only used at 3am when nurse fed her.

7pm, taken off hot-cot :O)

Day 4- 1^st February 09

I requested that nursing staff didn't use the ng tube for her 3am feed (they use it because it's quicker). When i arrived for the 7am feed, the ng tube had been removed! This was such a huge milestone for us because we'd been warned prior to her being born that she may need to be tube fed for life! WRONG AGAIN!

The evening of that day we moved to the 'mother stay' unit, which is basically a home from home, but where they could check her over when needed.

Day 5- 2^nd February 09 (worst snow for 18 years! :O) )

Trial demand feeding.

Weight 4lbs 9oz

Met Dr. Zengeya who was pleased with her progress. He discussed with us and showed us the MRI findings. The ventricles are very large :O( Apparently, they are now questioning whether she has a corpus callosum, which is odd since it stated on the prenatal MRI that 'the corpus callosum is present and normal'. I have to admit, the ventriculomegaly looks severe on MRI images...big black spaces where there should be brain :O(

He also stated that her cerebellum was 'Fine. Maybe a little bit small. Not a concern' WTF- this is why were advised to terminate!

Day 7- 4^th February 09 – DISCHARGED!!! :O) :O) :O) a day i never allowed myself to even dream of- taking home a live baby!

Neonatal discharge:

'An MRI confirmed antenatal findings of ventriculomegaly and a small cerebellum. The ventriculomegaly was bilateral but more pronounced on the right, with dilation of the third but not fourth ventricle. There was a marked CSF space surrounding the cerebellum but also surrounding the cerebral hemispheres. A corpus callosum could not be identified, but had been normal on the antenatal MRI. There was evidence of porencephalic change in the right frontal lobe.

The scan was reviewed by the neurological team at Oxford who advised that no neurological input is required at present. (This is good news as there was talk of her needing surgery to insert a shunt).

Hope's parents are aware that some developmental and or motor delay is likely but that the degree of this is hard to predict. (this slightly more positive than prenatal reports which stated that she'll likely be severely handicapped and may never walk/talk).

She will be followed up in Dr Zengeya's clinic and will need twice weekly measurement of her head circumference in the meantime. Dr Chinthapalli has been asked to advice re: specialist neurological follow-up'. It's felt that no shunt is required as it was not the appearance of high pressure.

19^th March 09

Hope is reviewed by Dr Zengeya. He seemed surprised by how well she seems to be doing, and said she's doing 'extremely well and hopefully she'll continue to do so'.

His report:

'Problem: Bilateral ventriculomegaly, IUGR (inutero growth restriction), a small cerebellum and possible absence of corpus callosum, small stature.

It was a pleasure to review Hope in my clinic today at aged 7 weeks with both her parents. She has made significant progress since discharge. She is breastfeeding on demand and receiving supplementary bottles feeds of 10oz of Aptimil per day. She has been gaining weight, although slowly. She appears to be very hungry and cries a lot. She smiles socially. She has good head control. She is now fixing and following with her eyes through greater than 90 degrees. She startles to noise and is beginning to respond to her mother's voice.

On examination she looks well. Her weight is 2.51kg (5lbs 8oz) which is below the 0.4^th centile but she's beginning to approach it. Her head circumference is 33.4cm (0.4^th centile and following it). She has a high arched palate but the rest of her physical examination is unremarkable. (I am inclined to disagree with him on this last point; he forgot to mention she's absolutely perfect and soooo beautiful, but I suppose in their terms unremarkable is good, especially since prenatally we were advised she may have dysmorphic features).

Hope remains small with a poor weight gain. She was seen by our dietician following the clinic visit who suggested that we should switch from aptimil to infatrini to boost her calorie intake. I will review her in 3 months time to monitor her progress. (3 months is great because it shows he's not too concerned at present).

Today. 24^th April 09. aged 12 weeks

Hope is now 7lbs 4oz and has just moved into newborn clothes. She' spoilt! We never put her down. She has a tantrum if we ever try. If jobs need doing, I put her in her sling. She now sleeps about 4 hours at night, from 12-4, then comes for cuddles for rest of morning. Every time she wakes me at 4am I count myself lucky. She can roll onto her side. We have lil conversations. I think she may be slightly delayed, perhaps I'm just paranoid, but I don't think she smiles as much as a baby of 12 weeks should, but that just makes her smiles even more special. She really is doing so well. I'm so very proud of her. So far she's pretty much proving the medical profession wrong, and if and when the time comes that she stops proving them wrong..that's fine. I do think that she'll be delayed in some respects. Those big black spaces in her brain are bound to have some consequence, but I'm pretty certain that in her own time she will both walk and talk. Until then, she is my reason to smile again, the light of my life! My lil Hope!

8th June 09 -The Truth about Hope

(excuse the lack of capital letters. i am typing 1 handed; hope is in the other arm feeding)

ok, so when i say 'the truth', it's not because i haven't been telling the truth in the previous updates, but i suppose for the first 3 months or so, things were going so well; we were sticking our fingers up to the doctors who had predicted the worst, she was proving them wrong, my own little miracle!

however, more recently, it's becoming clear that things aren't quite as well as they should be. i'll include the recent overview from the health visitor because i do think this sums things up quite well. then i'll fill you in on the last 24 hours, and the hospital appointment today.

report to Hope's consultant by health visitor:

Hope has been visited at home by the health visiting team weekly since birth. the nursery nurse has taught mum baby massage which Hope and mum both enjoy.

CHILD GROWTH
weight has been measured weekly and is following a line just below the 0.4 th per centile. The most recent weight is 4.310 kg ( that's 9lbs 8oz) on 01.06.09. Hope is having mainly breas tmilk and 10oz of Infatrini (high cal -meant to give her a boost) in 24 hours.

length is 57cm and on the 2nd per centile.

Head circumference has been measured weekly as requested and for the last 4 weeks has dropped off the 0.4 th per centile. i am concerned that the anterior fontanelle appears to be small and almost closed; i am unable to palpate the posterior fontanelle.

VISION
Hope's vision and eye contact are causing a concern to me annd her parents. i am concerned that her eye contact and ability to follow has worsened in the last few weeks. mum reports that she has witnessed side to side nystagmus most days. i am concerned this may be a symptom of her underlying neurological problem. as her vision has appeared to have worsened her parents would like an assessment by an opthalmologist and or neurologist.

GENERAL DEVELOPMENT
Hope's head control is weak for her age and she has a preference for the left side. she appears to have a full range of movement in her neck and no obvious torticollis. she is able to lift her head momentarily when lying prone but is not able to move her head from side to side (well actually she does sometimes).

Hope will grab objects when they touch her hands. She puts her hands to her face.

Hope is able to roll over from her front to her back by flipping herself over. she initially disliked being placed on her stomach but now seems more settled in this position.

SUMMARY
Hope is a delightful little baby who is growing slowly but consistently and seems satisfied after feeds.
In my opinion she is now showing signs of generalised delay and main areas of concern include her vision problems and static head circumference measurements.

:O(

that really came as a blow when i read the report. although i already knew it all, seeing it all in black and white, it seemed so...i dunno...sad? i was upset. chris just didn't get it. i shouldn't expect a man to understand. he said 'are u just upset because she's not perfect like jordan and max? we knew she'd have problems..' to which i replied, 'she IS perfect!' and she is. she always will be. hope is hope, and she's just adorable. but i do worry for her. surely that's allowed. right?


anyway, i fully admit to being completely over protective. i wont leave her- at all. even in the car, i'll sit in the back so i can keep an eye on her (it's that or i'm constantly getting the kids to check her which; bless them, they do). i guess, i've always had in the back of my mind that i needed to be there incase something happened, or she had a seizure. well, it breaks my heart to say, she has. she's had a few :O(

last night, her lil arms both shot up in the air like she was doing the dance from micheal jackson's thriller video. her beautiful blue eyes shot up into her head and were flickering at a hundred miles an hour. it only lasted a few seconds, but about a minute later, happened again. i knew it was a seizure :O( i came downstairs and typed infantile seizue into youtube. my fears were confirmed.

this morning Hope and i were in bed, thinking about getting up (as you do). she was full of smiles and happiness. they got her again. this time probably about 10 times in as many minutes. fortunately they don't seem to distress her at all, and she was smiling inbetween them.
after i'd dropped jordan and max at school, i was pushing hope home in her pram, and i could see it was happening again.

we already had an appointment today to see her consultant. good job really, i'd have taken her to a+e otherwise.

today, at 19 weeks and 5 days, she weighs 9lbs 14oz.
her consultant, like me is obviously concerned. he's going to arrange for an EEG asap. that should be within a week he said. in the meantime, i am to keep a diary of anymore seizues, to try and capture on video, and he's arranged for us to have open access to the children's unit; which basically means, if they get worse, become stronger or more frequent, then i can take her straight to children's unit rather than mess around with a+e. he said they could get worse, or disappear. let's hope it's the latter!

her consultant is arranging for her to see an opthalmologist, and to have another cranial ultrasound.
today we had to go and have lots of blood taken for various tests.
my head is spinning!

i know this isn't what you all wanted to hear. but i also know that you'd all want to know. please don't feel sorry for us; we will be fine. please don't be disappointed that she isn't that miracle baby. BECAUSE SHE IS! She, along with the other kids, make me so happy; happier than i ever though possible after lil Sam died.

september 12th

it turned out that Hope has severe epilepsy :O( took a while to get dr z to listen, he thought it was colic. as if!

anyway, things are notstraightforward; Hope is now being tested for a rare metabolic disorder :O(

please have a look at her blog for updates www.honkeyplonk.blogspot.com