My Baby is not Perfect

(I write this blog in the hope that on May 6th you will support the 2014 Giving Challenge so that parents of newborn children with PWS do not have to go through the same agony as I did.  And I live in a Western country that prides itself on its good health system.

All you have to do on May 6 at noon till May 7th 11:59:59 am (est) is go to: www.givingpartnerchallenge.org  search Prader-Willi Syndrome Association.  Click the button.  There is $440,000 worth of matching dollars out there. The Patterson Foundation will match all our gifts, dollar for dollar, minimum $25 up to $1000 per individual, as long as the funds are still available. 

Please donate early before the matching funds run out!  Let's take this great opportunity to get some matching dollars.  Your click, your donation will help IPWSO continue its work in the world.)

When I was pregnant with my third child I wondered, very briefly, whether my baby would be born perfect.  My other two had, so I put the thought out of my mind and concentrated on eating well, exercising, and looking forward to the new arrival.  I noticed that my baby didn’t move so much in the womb and that there was none of the violent kicking that made my gasp and feel faintly queasy, but, in a way, this was a relief.  Her movements were gentle, like a fluttering, or a slight wobble.    My pregnancy on the whole had been calm, apart from a scare in the third month when there was slight bleeding.  I wondered if I might miscarry; it disappeared for a few weeks but then reappeared to an extent that I rang Emergency and they told me to come into hospital.  There, they made me wait and drink copious quantities of water before taking me in for a scan.  It was incredibly difficult to retain all that water, and retain my dignity.  The scan was normal.  The bleeding stopped, and the pregnancy progressed.

On my due date, I started Braxton-Hick’s contractions, the false labour that can send you into a panic.  They were irregular, just a tightening across the belly as the muscles contracted and relaxed.  Although I wasn’t unduly worried, it seemed prudent to check with the hospital.  They said to come in anyway.  So I arrived at the hospital doors awaiting admittance.  The contractions faded soon after, and in the morning I was sent home.  There was not much longer to wait, though, and that night I was admitted again, this time in labour.

It was a struggle and, because my previous pregnancy had been a caesarean section, the doctor decided forceps delivery was going to be better, so, after a few hours of labour, she was born.  The rush of oxytocin, endorphin and adrenaline hormones made me feel elated and excited.  But there was no cry, no waving of tiny arms, just silence.  I remember the nurse holding her, naked, limp, like a rag doll, arms and legs totally relaxed.  The nurse quickly took her away to a waiting crib and wrapped her in muslin.  Still no cry.  No movement.  To begin with, I did not notice, and was waiting for them to bring her back to me.  The nurse turned to me and said they would just run a couple of quick tests.  I imagined these to be the usual muscle reflex.

It was at that stage that all my unbidden fears, all the things I had pushed to the back of my mind, flooded in quickly taking away the adrenaline and leaving me feeling as limp as my baby looked.  What was the matter?  Where were they taking her?  What was wrong?

My baby is not perfect.  The only thing they could offer was that she had been exhausted by the birth and was hypotonic, limp, no muscle tone.  They wheeled me back to the ward without my baby.  I lay there motionless, waiting for someone to bring her to me, or to tell me what was happening.  Nothing.  I waited.  Hours went by, and a nurse popped her head around my curtains and said – ‘oh, you’re the one with the baby who’s got the speckled head-rash!’  What was she talking about?  I asked her: she said straight away, ‘oh that usually means mental retardation.’

My world collapsed, spinning out of control.  I was icy calm, trying to marshal my thoughts.  What now?  Who can tell me what is going to happen?  I spent the night alone in the ward, separated by a flimsy white curtain from other mothers and their perfect babies.  In the morning the specialist asked to see us.  He said a whole lot of things that simply would not fix themselves in my head.  They entered, then flew away.  I kept asking if he would repeat himself, tell me again what did it mean?  Words jumbled around in my mind.  I could not tell you anything he said.  I felt calm, as though I was not really participating in this discussion, more as though I was hovering above us all just watching. 

They brought me my little red-faced, long-limbed, silent baby to feed.  She would not suck.  I thought she was tired, but she had no power to suckle.  They gave me a bottle with an extra long teat.  ‘Squeeze the teat into her mouth’, they advised.

They let me bath her, and she lay there in the water, limbs outstretched, unable to move, barely able to open her eyes.  Every few hours they brought her back to be fed, and I pretended it was fine.  The nurses volunteered their own information, a cousin whose baby was ‘just like this’ and went on to regain her strength in a matter of weeks.  A son who never walked ‘til he was 3, but was just fine; she’s just like many babies who are born tired but soon recover. 

But the speckled rash was still there, and the words from outspoken nurse who’d frozen my heart, were still tight in my chest.

It was no good, she was losing weight, not crying, not moving; just sleeping.  So they tube fed her and I watched, feeling desperately useless, and wondering what was wrong.  No one could tell me.  Failure to thrive, was the best I could get.  I was terrified, but the terror was now iced in and would not thaw for many months yet.  I expressed milk, sitting in a corner of the nursery, listening to other babies crying, feeling so alone, so sad. 

I was allowed home after – how many days?  I don’t remember, they blurred into one long day, one long night.  And once home the feeding became the only challenge of the day.  She slept endlessly, not waking, not crying, not moving.  I squeezed the bottle’s teat into her mouth and massaged her throat, willing her to swallow.  It took an hour to feed 15 mls.  She became weaker and weaker and went back to hospital where they tube fed her, then left her to sleep.  I would drive to the hospital and sit with her for hours begging her to pull through, to be normal, to come alive.  She lay motionless and pale.  Sometimes when I held her she would open her eyes and engage mine.  That was when I would pray, to what, to whom, I did not know.  They were just words repeated over and over, “let her live, let her be alright”.

She wasn’t, of course.  She did live, but there was something fundamentally wrong; she remained in hospital and the doctors continued to look for an answer.  I came in day after day, asking whether they had found out anything more.  One day there was a plaster on the calf muscle of her tiny leg.  They’d taken a muscle biopsy to see whether she could have Werdnig Hoffman’s Disease.  Spinal muscular atrophy.  A wasting disease that has a child living 18 months or less, then wasting to death.  But they didn’t tell me that.  It was years and years later when Google came into being that I found the answer.  I could then see why they tested for it.  Many of the symptoms and characteristics were there.

After she had gained some weight, I was allowed to take her back home.  I tried to encourage her to stay awake for longer by not putting her to bed, but setting her up in her bouncinette so she could watch the other children, hear household noises, listen to music, watch the dogs.  Slowly, very, very slowly, she started to become more alert, she smiled, she watched our world from her own.  But there was no answer to her medical condition; she still had the label of Benign Hypotonia.

At three and a half years old, when she was finally walking albeit unsteadily, finally talking albeit a language of her own, and we had accepted her as she was, a friend showed me an article in an Australian women’s magazine about a child who was 10 years old and who had started life in the same way as our child had.  This child had a deletion in the 15^th chromosome which caused all sorts of things including hypophagia.  An inability to know when to stop eating.  ‘Obesity and early death usually in their teens, followed.’

The darkness that descended was, again, indescribable.  Our daughter now wore a different label, that of Prader-Willi syndrome.  Again, we were still pre-Google, pre-internet, and pre-information.  This time, the block of ice that had served me so well in the beginning, had melted and what was left in its place was a well-forged, intensely heated rod of iron.  The fight was on, let the battle commence.  I fought for knowledge.  I asked questions.  I was no longer afraid of medical professionals spouting coded messages.  I would not leave their rooms until I had the answers I needed.  It was a long wait and usually ended with one step forward, two back.  The geneticist took down a large medical dictionary, opened the page at “P” and left the room.  Why did he do that?   I still have no idea.  Did he expect me to turn the book around and read the section on Prader-Willi Syndrome (as, of course, I did)?  What then?  Did he think I would understand?  He returned eventually, but threw no new light other than this was a genetic disorder.  The paediatric psychologist did all sorts of puzzle tests with her; finally walking us to the door and saying, ‘Oh look, there’s a young lass with PWS, do you really think your daughter has that?’ 

How the hell did she not see it when I could, so clearly?

It was an ongoing battle.  Friends who knew me well stood by me.  Family worried about me.  No one else mattered.  I had become fanatical about this wretched syndrome, but I kept it to myself knowing how quickly others become bored with fanatics.  Slowly, bit by bit I recalculated my life.  It was a completely new rebuild; I discarded things that no longer held true; fanciful, dreams that would never come true, people I couldn’t be bothered with, certain principles were abandoned in favour of new.  There was a fire sale – a big one – and I closed down the old burnt-out me.  I reopened some months later, just a few doors at a time, just a peep now and again until the paintwork was reapplied, the garden weeded, the fences restored; a completely new façade gave no hint to its terrified interior.

The child grew up, did not die, was loved, educated, and though there were some dramatically bad times, we all got through them.  The façade of the new me still held good.  However, the interior always needs re-strengthening, redecorating, reviving, and old things cast out for new, It has worked; most of the time it has worked.  Nearly 30 years later, life is different, but it is still good and the interior decorating still goes on.  Nothing is perfect.

Men & Grief

While we've been on the topic of grief recently, I was reminded of an article about men and grief that  I once asked for to go into our newsletter many years ago now.  I wanted to know why some fathers grieved differently from mothers and why men grieved differently from women.  Bear in mind this was a while ago, and I'm quite sure that, generationally, things have shifted significantly.  But if they haven't, for you, then maybe this might help.  You will also notice that in this story diagnosis had been late, as it still can be in many countries and there was no help with feeding.  This family really struggled.

"I had been looking forward to the birth of our first child. For months I had been fascinated by her movements insider her mother; at the beginning little flutterings and then as the months passed, stronger, more vigorous movements. It was exactly what one would expect of a strong, healthy baby.

I was present at my daughter's birth, an experience that defied words All stories and all films that had the birth of a child in them made much of the initial birth cry. I was absolutely unprepared for our experience: she arrived without a sound. I have a clear picture of the doctor holding her up, her little mouth shaped like a cry — but there was no sound. I am unable to describe what came over me at that moment — words like shock, disbelief, do not even hint at what I felt.

My overriding reaction was what I can only call resignation; whatever would be would be and I had no power whatever to change any of it. I was devoid of feeling. That stayed with me for moths. The only thing that broke through my lack of emotion in those early days was a kind of subdued elation when our little girl would actually swallow milk from an eye-dropper which was the only way we could feed her. Eventually our efforts weren't enough and she had to be tube fed at hospital.

At work I was an automaton. I have little recall as to whether I was happy or sad, warm or cold; I had no appetite. I used to enjoy a drink. I never tasted the stuff from this time for months afterwards!

As the hospital testing continued to determine what the condition might be, I became aware how differently my partner was responding. She saw all tests as a challenge. I was indifferent to the various tests and as each eliminated this or that possibility she saw all these as major victories. The diagnosis at this stage was benign hypotonia.

I was conscious that I was unsupportive. My partner's hopes were constantly undermined by my scepticism, actively negative and yet, I could not break out of the "what will be, will be" mindset. While my wife wanted to talk about progress and development and testing, I just wanted to be left to myself. I had closed off — little outside myself held any interest. I "escaped" into Wilber Smith.

A friend was a good listener, but I could only share at a superficial level. Talking was not helping as the overriding feature of "all this talk will not change anything" undermined any chance of healing.

The second stage was marked by my waking one day and noticing the sun was shining. I felt warm for the first time in months. This time pin-pointed another stage: anger. Wild mood swings, optimism (that our little girl would be "normal," that she would outgrow this strange condition) followed by depression caused by the uncertainty of what the condition would mean long term. I cried more now — most nights. Sleep was full of curious dreams and I would wake up exhausted. But there was less of "what will be, will be." By now our little girl was drawing me more and more into her life. I felt I was able to share more of my partner's hopes. The future seemed much less fixed.

Eventually our daughter's strange condition was diagnosed as PWS. It took me a long time to accept this. I read all I could about this syndrome — the information coming from my partner's research; a book here, a paper there, an article somewhere else. After a time I noticed a shift in what I was feeling. The crippling resignation had given way to a hope that what was now, did not have to be in the future. My wife was already planning counter measures for our daughter to run against the classic symptoms — extra help for learning, exercises to firm up the little muscles, games to keep her stimulated.

Sometimes it seems a long time ago; more often it feels like yesterday. A kind of emotional evenness came when she was five and a wholeness at seven. The anniversary is still hard, but my partner eases me through it each year.
Yes, we men do cope with grief differently. Education should enable us to cope better."

After the cat had died...

After my daughter had to make a heart-wrenching decision to have her cat of 10 years put to sleep, she showed some interesting behaviours and some that I'd not seen from her before.  It was difficult enough to handle the behaviours before the event (and I am eternally grateful to her residential staff who supported her, at times, courageously, and also very lovingly, through the entire procedure).  Her anxiety levels had risen considerably once she knew the cat was terminally ill.  Predictably (for her), she became angry, tearful, and violent (bashing a hole in a door, while crying uncontrollably).  All of us tried to talk her through the whole affair, saying that she was doing the cat a kindness, was brave in making the right decision, the cat will never recover, and so on.  Of course, all of you reading this will know that this was rather like water off a duck's back, because all that mattered was the actual moment she was in at the time.  There's no reasoning with someone (with PWS) when they are in such an emotional state.

But somehow we needed to keep pushing forward the idea that what she was doing, was the very best thing.  I apprised the vet beforehand, and told him that as far as my daughter was concerned, he was the "cat specialist" and she would trust and believe him.  I had good knowledge that this vet was not only an excellent diagnostician, but also incredibly kind and gentle.  He asked how much my daughter would understand and comprehend (everything) and conducted the whole procedure in a humane, thoughtful, and utterly gentle manner.  My daughter conducted herself with poise and grace throughout the whole time (and I think that her favourite TV channels, TV vets and Animal Rescue, probably played a part here too).

And now we come to afterwards.

She phoned me immediately from her caregiver's phone (she lives some way from me and I was unable to drive, having had a recent hip operation, otherwise I would have been there), and told me what had happened.  Both of us were very tearful.  And then she said she thought she would like an ice-cream.

She was allowed that (and, stupidly, I didn't stipulate what kind.  So she had one that probably had the calorific value of two meals combined).  Later that evening, she asked her caregiver if she could go to McDonalds for a coffee and muffin.  The caregiver said that she wasn't able to do this.  So there was a medium-sized melt-down.  The caregiver left the room until my daughter calmed down a little.  What she did next was to gather up 10 of her tiny incense cones, go outside onto the drive-way, sit down and put them in a ring around her and lit them.  Each cone represented an animal in her (and our) lives that had died.  She spoke to each of them individually, and collectively, telling them stories, reminiscing, and introducing them to each other.  This lasted an hour.  The caregiver, not wanting to interrupt her, could only finally entice her inside with the temptation of a plate of jelly.

The day after (she'd arisen at 2am to play music), she was calm, collected, and spoke easily about her cat and... how she's going to get a new one very soon.  After she's finished grieving...in about a week, or so, she thought...

***

This blog is a bit longer than usual and the general story finishes here, but if you want to read further, I was so interested in this behaviour that I wanted to find out more.  So what I did was to google behaviours in the intellectually disabled group.  There have been lots of papers written, but I didn't find much anecdotal evidence which is always much more interesting to parents.  I also wondered whether, because our sons and daughters with PWS are living longer, and well past their previous life expectancy, this was an area that we may need to look at for future reference.

Professionally, there was a lot of stuff like:" Individuals with intellectual disabilities as such may have limited understanding of causality.   There also may be cognitive reactions.  In the initial phases of grief, people may be in shock – disbelieving or denying, unable to process information.  Throughout the grieving process, individuals may find their concentration or span of attention is impaired.  They may process information less effectively.  It is not unusual for health professionals or others to be asked the same question or to provide certain information repeatedly."  (Of course, we must understand here, that the subject of the grief  in this instance is not just a cat, but someone close to that person.)

This kind of information, to me, seemed pretty  normal for anyone in a state of grief.  This article went on to say that it wouldn't be unusual for "people with intellectual disabilities to experience sleep disorders", or engage in "risky behaviours such as substance abuse.  They may be angry, lashing out, or seem withdrawn."  They may experience "impaired judgement" and theses changes may be profound.

Some other articles described stages of process for dealing with grief, like, preparing the person, instructing caregivers, modelling emotional support etc.  All of which is necessary, and it's good to know that such work has been done on this kind of preparation already.  Wanting to know more, I asked Professor Tony Holland (Professor of Psychiatry, Cambridge University, and President of the UK PWSA) what he knew.  He led me to Professor Sheila Hollins' work. A Professor of Psychiatry and Disability, she has a son with an intellectual disability; Professor Hollins is also a Peer of the British Realm and as such, one would think has some influence.  Professor Hollins has written some excellent books about loss and grief, mainly in pictures, for people with disability which you can see here "Books Beyond Words" 

To his knowledge, Professor Holland did not know of "anything specifically written about grief in people with PWS, but I could imagine that loss could have other implications that lead to high anxiety - eg, it's a change in routine", and he also advised that it is best to try to explore the mental experiences so as to understand what "might have started as grief, has now moved onto something more."

I still wanted to know more so I turned to my e-group of older parents with older kids with PWS  (PWS_Older@yahoogroups.com  if you're interested).  We've had this group since our kids were in their early teens and shared a lot of 'stuff'.  I was wanting to know about responses to grief in anecdotal terms.  And this is what I was told:

"My Dad passed a couple of years ago and a friend was killed in a house fire 7 yrs ago. My son handled all that amazingly well. But let one of his chickens die or be killed by a predator and he goes off the deep end. He screams and hollers for hours…..then he has to bury it in the back yard. We have our own little pet cemetery….."

"My son is very spiritual, as am I.  He attends church and loves being an acolyte, a server and in the choir.  He loves the ritual and pageantry.  We have had many discussions about God, and such.  Sometimes he's so very deep.  He grieved for our pets and his granddad in a strong, but deep way.  He also seeks solace in food, but then again, don't many of us?"

"A friend who was also a home help, had given our daughter a kitten for Christmas.  Some years later, this friend died.  She had an open coffin and viewing before the serviced.  We explained to our daughter as best we could, and gave her a choice to go and view.  The cat had to go as well.  So off we went, four of us, my husband, daughter, me, and the cat.  No one questioned the cat being there, and in a way she was an ice-breaker for everyone.  They loved seeing the cat there, and how much she had grown since kitten-hood. "  My daughter looked at our friend in her coffin, talked to her, and then we said a prayer.

She has coped with death in the family really well,  and the death of animals as well, since we live on a farm.  But pet dogs, or cats, or lambs - she just has to replace them straight away and is constantly looking for one til it is replaced."

"Yes, my daughter felt grief, but handled the reality very, very quickly.  She was very strong in that area.  She could face life 'square on' but felt very deeply for her animals.  Replacing them seemed to be the answer for her as she then enjoyed them young again and it took her mind into a new experience.  It could be that she was able to cut off after a certain point in her grief pattern."

"I knew of someone who was 'engaged' for a long time to a lady (with PWS).  They hoped to marry.  They lived in close proximity, possibly in the same house, I don't know.  However, she died.  I asked the provider how it had affected him and how he was doing.  She said that it didn't affect him at all and that he was just living as if nothing had happened.

"Another lady died recently, she'd lived with three other ladies for at least 12 years.  She died on a Friday.  The other ladies were concerned about whether the group would be going garage-saleing the next day as previously planned.

"With my daughter, there's no emotion, but only questions about the person's/animal's whereabouts... for years!"

"When his paternal grandmother passed away a few years ago he handled it really quite well. His main upset was that Dad no longer had a mother or father. A few months later when it was Mothers' Day he didn't want to see me at all - he just wanted to take Dad out for lunch because he didn't have his mother."

While we're on the subject of death - how about the after-life?  My daughter had an experience in her new home whereby some chimes began tinkling for no reason, and a small pot of handcream leapt off her table across the room.  To cut a long story short, the caregivers had a priest come in to bless the house, but my daughter asked that the priest allow her friend (who had died a few years ago, and who my daughter thought it was) to continue to visit.  And, apparently he does - the chimes ring, and my daughter holds a conversation with her friend.  In answer to this, one of my e-friends said this:

"My son has frequently been visited by family members who have passed. He mentioned my Mom a few times and his other Grandmother from his Dad's side. But mainly it's a young cousin of ours, she has visited many times and my son would tell me about what she was doing, what they talk about and about heaven. Sometimes I hear him laughing in his room and later when I ask what was funny he'll tell me that he was talking to his young cousin. Recently a mom on facebook who just lost a child (non pws) mentioned that her child with PWS is the only one in the family who is visited by the one who passed. I believe our kids know and see things we are unable to."

This is only a small sample from six mothers, and you can see that grief in PWS may run the whole gamut of expression, just as it does in the normal world, but perhaps there is a link to understanding grief that our guys have that can help us understand them even better.  I just wanted to write about this in case there were other parents wondering the same thing.  I'd be happy to hear any comments.  Quite possibly, it's the behaviour before the event that is more challenging than afterwards, but we need to be aware of both sides of the coin.

Linda


References: http://www.ddhospicepalliativecare.org/forum/ciwid.pdf
http://bma.org.uk/about-the-bma/who-we-are/biography-sheila-hollins