Living with rare illness

Dear keeper of the equilibria of life,

I have a problem, My scales are a little unbalanced, quite like my mind at the moment, having kids is more difficult than any career choice in the universe! In actual fact visiting space would be less scary at times.

No one warned me about trailing off of the normal healthy kid path in to a totally different world from all of my friends.

Before you have a child with a chronic illness your heart is almost virginal to hurt.

I remember when i was told my first born had a touch of childhood asthma i was upset for days even cried. Nothing though ever prepared me for child number 2.

Your children are the most precious things in you're life .. The meaning of your life the substance and core of your being.

So here she was a tiny little baby with big blue eyes and curly hair, I had her in the passenger seat of a mini metro in 20 minutes flat whilst being driven backwards down a one way street (true story). I'm not really well known for doing things correctly, something always has to happen out of the ordinary somewhere, and it did on this night.

Molly bolted out like lightening to be fair I was very ill with cholostasis I think if she hadn't come 3 weeks early on that freezing cold night I would be speaking to you from heaven so really I should thank my moll for having the bottle to expel herself from my being so quickly before my liver engulfed her and myself with poisonous toxins …

She was tiny but strong and weighed in at 5lb 8 and a half, the dr said she was perfect and she was, my beautiful baby Molly blue eyes blond curls and gorgeous

The 'perfect' which the dr spoke of began to unravel at 3 weeks old .. Molly cried so much with awful tummy pain we put down to colic, a hernia appeared in her groin a big lump about the size of a large grape we were told it needed surgery and that it was very unusual for a girl to have one, that was operated on at 4 months but another one appeared the other side not long after.

By 6 weeks old she had caught a cold and ended up in hospital with bronchitis needing oxygen, she was so tiny I had never really been in the hospital before, it was horrible to be fair, Victorian like with big metal radiators and tall cots, the sounds of crying and beeping of machines was quite alarming for a hospital virgin like myself, I also had the most hideous tooth ache at the time but that was the least of my worries there was moll my perfect baby, she couldn't breathe and her chest sounded and looked terrible, this was the first time that horrible scared feeling came over me, a feeling where you feel things are a little out of your control, a scared feeling only a parent could feel for their child .

It was there we discovered she also had a heart murmur, heart scans revealed asd (a small hole) and pulmonary stenosis narrowing of tube / a sticky valve.

Miracle number one her heart actually worked perfectly well. The hole let blood in but the narrowing limited blood flow which meant molly had the exact right amount of blood pumping to and from her heart and did not need surgery.

 

The memories of crying about the childhood asthma were long gone.

My new beautiful baby was ill. She would scream for 6 solid hours a day, her hernia would protrude and need popping back in until she was old enough to operate and she was frequently poorly. I never once left her to scream alone, she was in awful pain, i hugged her, consoled her , sang to her and danced with her for the whole 6 hours each day trying anything to soothe her, sometimes the 6 hours would start at 4 am, She was in so much pain I couldn't bear it. Sometimes i was so tired i couldn't function, my eyes would be blurry I either needed match sticks to hold them open or windscreen wipers to keep them of a visual standard I had once been used to .

When molly was asleep i couldn't sleep, although exhausted i was worried sick why was my baby always so ill? No one else's child caught this many colds and if they did colds would only last a couple of days, molly had a constant cold, once that one had gone bad and needed antibiotics another one would start within a matter of weeks.

By 11 months old Moll had been in hospital many times, watching the nurses squeezing blood from her little hands time after time because they would not give enough blood was hideous i felt powerless, she would scream and just look at me with those beautiful deep blue eyes as if to say why mummy are you letting them hurt me.

She didn't understand, her hands still have many little white scars all over them from the cannulars the scariest time so far was around this time a month before her 1st birthday, she lay there inside an oxygen tent so poorly she didn't even care or protest about the cannula being in her hand or the bloods being taken or indeed being inside this plastic box, her face was so tiny, she was so tiny it wasn't fair.

I said to the dr will she be ok? He said we are doing what we can but didn't say anything else, im not sure to this day if he was trying to freak me out , at this point I nearly threw up. I am really not the sort of person you can say that to.

Moll was ok because she is a fighter and a few days later she was back to her normal self refusing medicine singing songs eating like a horse and being fiesty to the nurses and such like.

But sadly this continued, don't get me wrong in between such episodes moll was just my normal happy baby we did stuff other mummy's and baby's did, the trouble was the in between times didn't last that long

Things kept happening you see, things that would shape my baby's personality her fears her whole future, it might seem silly and over the top to suggest these things but its true, I still know her now and these things affected her, take note professionals who work with kids, give them time, kindness and sympathy a normal child's world is not like your work environment, hospitals are scary places to kids, they are used to being safe at home where nothing scares or hurts them.

There was one occasion about a year later to the oxygen tent episode, moll was in hospital again, another chest infection, she had been taken off of the intravenous medication and she wouldn't take her antibiotics, she wanted to go home to be with joshy and daddy, she really wanted to go home, the nurse said she could go home if she had her medicine, she was a baby you cannot reason with babies. To my horror she actually pinned her on the floor an forced it down her throat.

Molly was two.

This must have been the 10th hospital admission I had seriously lost count by now but know it was in the double figures. Powerless again I'm open mouthed by this action. How dare this big overweight grown woman treat my baby like some kind of gladiator challenge, she's 2 years old she hates hospitals and she hates the taste of that medicine.

I was secretly quite glad when she kicked her in the shins... what a bully of a horrible woman seriously she needed to get another job and not in a caring profession.

I told her Moll was 2! 2 years old not just part of her 12 hour shift of tick boxes! medicine tastes horrible, forcing her like that was hardly going to help me out in the future.... like when she was 6 and they tried to make her drink half a pint of baromeal (liquid white metallic tasting gunk) so they could do x rays of her tummy because it always windy and painful,

Moll was sick of hospitals all they bought was ill and pain, she's my child she's not going to go under the radar she will make her feelings known and im glad about this, children should always stick up for what they believe in, by this point already and again we had been in for a few days , the radiographer got very angry with her and us because she wouldn't drink it, I still have the sarcastic comments he put on the xray form, I kept them purposely because of his rudeness. He had no idea what she had been through with tests scans and hospital visits in the past, I really hope he doesn't end up with a poorly child to this day molly remembers him as the scary man who kept pacing up and down looking at his watch...grown adult v's child again.

I Am lucky to have such a supportive family and amazing husband.

Josh 4 years older than Moll would frequently be staying at nanny and grampy's I've lost count of the times my friend Jane next door looked after him during the day until daddy returned home from work when I had rushed Molly over to hospital yet again because of another chest infection.

Thinking back the Dr who came in to our room when moll was 18 months or so old was on to something.. It broke my heart at the time I felt insulted by the way he spoke to me but he said they were looking in to doing possible tests because Molly's face showed signs of something genetically wrong, he said her facial features were quite elfin like.

This is a term I have heard many times over the years, most of her hospital notes / letters from consultants refer to the same words, I have kept all those too.

Although she was so small and never seemed to grow very much and despite the Dr's thoughts that day she was never referred to an endocrinologist.

To be fair I had never heard of an endocrinologist either but they deal with growth issues.

The focus was always put on just treating the symptoms and not the cause, I just trusted them and was very grateful that they made her better so many times, I was exhausted. So this is what happened. Ive always had massive respect for drs and consultants and trusted them to do the best for my child, myself and martin were only 25 / 26 ourselves we were not drs all we could do was keep saying it wasn't right, at that point she would be referred to someone else for more tests.

There was always this thing in my mind which knew Molly needed more care than your average child, apart from being ill all of the time she needed extra care when she was well to prevent her from being ill.

I'd put this conclusion together even before she was 2.

You know those summer days which are cooler in the mornings ? Well most children would be fine in a cardigan on the way to school. Not my molly she always felt the cold. Her little hands would be freezing on these mornings. I kid you not she had a fur coat on in that pushchair and probably blankets. I'm sure people thought I was completely nuts and way over protective but as she got older they understood. You see if moll got cold Her body would shake, her lips would go blue soon after that she would develop a cold and her temperature would bomb up to 40. This was her bodies response to cold and illness.

This would then progress in to a chest infection where again her temperature would spike 40 and she literally would sleep for hours and hours, this was the 2nd high temp of the cold now infection had set in and it was time to get her to the Dr's where they would send her to hospital. Yet more more antibiotics more needles and more trauma. There were some occasions the infection didn't develop as quickly (as a few days) so we had antibiotics at home but we always ended up there eventually.

I cannot tell You how many times I said and explained this to drs this was not right her body was reacting to illness in a really odd way.

To be fair the consultant organised many tests over many years, and referred her to many consultants.... But maybe just not the right ones.

So lets say now Molly is 2 I'm downstairs she's in her cot. Again she is poorly and is on antibiotics, you could probably say to combat the chest infections she was on penicillin more than she was not on it in the first 2 years of her life.

Back to that night. Molly is crying screaming in pain I rush in turn on the light and she cannot open her eyes they have swollen in to big balls of skin. Her ears were 3 times the size they normally were and she was covered in red blotches (hives) I phoned out of hours immediately and took her straight to the hospital.... Antibiotic allergy number one.

So Molly continues to be poorly, oxygen tents, needles antibiotics and constant illness.

Double pneumonia age 3 found her coming home Christmas eve the tears in josh's eyes age 7 when he thought father Christmas may have to visit the hospital instead of home were heart breaking. My poor little blonde boy his life revolved around hospitals and a poorly sister too.

By the time she was 4 she was allergic to another type of antibiotic this was her bodies way of saying that's just too much. To date she is now allergic to 4 different types this is not good for a child who is ill this much in fact it is very dangerous and has resulted from treating the symptoms and not the cause.

So not to bore anyone this carried on... And on. The concentration was focused on her chest and after the pneumonia again episode she was tested for cystic fibrosis. Now I'm not a religious person but I prayed to God this would show up negative, I had seen the poorly 'regulars' I called them who were in the wards too, these poorly children suffered so much, constant medication and physio with low life expectancy.

I became a regular in the play room and really enjoyed drawing and colouring with all the kids, we played shops and tearooms kitchens and all kinds of games the kids laughed and we had a great time each ones hand bandaged with a cannula in all going through the same thing as molly, luckily for some this would be an out of the blue hospital occurrence hopefully not to be repeated.

Heart breaking sights for Molly too the trauma and screaming was awful. Sleepless nights beeping machines rolls of trolley wheels down the long corridors, The play room is such a sad place really, the kids were laughing and playing but not one of them wanted to be in that hospital, kids are of such a happy disposition though they are very resilient.

Its so difficult as a parent, we know our children have to be there, we know they have to hurt so they can be made better, but what ever you do and how ever you explain it they are babies they are not going to get it.

Winters were always worse. I spent hours and hours with her over my knees in bed banging her chest to relieve the congestion. I became the worlds best physiotherapist

 

I didn't want my child to think pain and suffering medicine and screaming was normal I struggled with that a lot.. I had friends who had children they used to say how they kept a bottle of calpol in the cupboard like it had been there for years, I was buying calpol as part of my regular shopping list.

Molly started school at 4. It soon became very apparent that her health was going to have a big impact on her school years.

Her primary school were amazing especially Her teacher Mrs Hawkins, she totally understood the way Molly was and knew exactly what to do, coats / hats / gloves at play time no pe if she was poorly there was absolutely no problem with attendance officers because the school had explained molly's absences and health problems to them without worrying us further.

By this time we had also been introduced to physiotherapy which made controlling this a little easier.

Molly would continue to catch every cold and virus going and was still ill every 3 to 4 weeks. So she could go to school in the run up to a chest infection or post chest infection Mrs Hawkins would do physical percussion physiotherapy on Molly's chest at lunch time so that she could breathe in the afternoons. I will never forget Mrs Hawkins for this and appreciate her efforts in helping Molly so much to this day.

Not many teachers would do this. Sometimes she would call me in fact it was a lot of times.

I remember one time in particular when I went to the school, moll was slumped over her desk solid asleep with a raging temperature. She could be relatively normal you see, playing, happy etc. etc. then all of a sudden she would just go down hill. This became the known term for moll which is still used now by drs ... She goes down quickly.

This wasn't a particularly special day it happened all the time but it was the first day I ever cried in public.

I remember putting my head in my hands sat down at her desk and just broke down.

How many times could she be this ill?

I remember saying through wobbly chin and exasperated tears.

'Here we go again' quickly rectifying myself I sucked it up and carried on as I always did. Scooping up Molly and rushing her to my car.

Thankfully I had called Martin he was already on his way home from work to make sure Josh was taken care of before joining me later at the children's hospital. We had open access by now. So it goes on she was 5 years old at this point and still tiny in 3 year olds clothes it seemed that nothing had changed in 5 years.

It was decided that moll should repeat her 1st school year again because she missed well over 50% of it through being in hospital.

Infections grew a bit less due to constant physiotherapy every time she had a cold.

It sometimes would take hours of physio to cough up the gunk, I knew though if she didn't get rid of the congestion it would turn bad and get infected again meaning more hospital. Me and Molls developed our own physiotherapy shop where we'd blow bubbles, do something called autogenic drainage I learned from great ormand street and our physios

 Laughing is also great for coughing up gunk so it's a good job I'm really funny ... Joke.

 

To be honest despite all of the trauma upset and constant illness my way of coping was to just try to be happy at all times. This was a little difficult when we had our 3rd baby and I developed pnd.

 

The Dr thinks it was probably connected to the constant worry and stress of poorly molly, I was 6 months pregnant and sleeping in a chair at the hospital when Molly had another chest infection. I tried to leave her once, Martin was to stay so I could have a rest, but I cried all the way home and didn't sleep all night.

 

When moll was 7 we were in the hospital again it was near Christmas we were all going to see Cinderella I'll never forget her tears as Josh and Martin left the hospital to see the pantomime without us. Remember the song wires? Well that's the song which reminds us of our Christmases we always had a visit to hospital somewhere. Holidays were the same.

 

We have never been away for more than a week in 17 years. Most times moll has either been close to a chest infection or post chest infection or she gets one during, chronic asthma has also played a massive part making activities very limited, Molly was still in a pushchair sometimes when she was 11.

 

Holidays were never abroad and we have been known to turn up at out of hours hospital at 7.am to get her checked by a Dr so we can just take her down to Cornwall... Imagine what this is like for our other children.

 

Moll was sent to a chest specialist he was great but could not explain her constant chest problems he said they were secondary to something else. Moll also spent forever with a tummy ache this would also get worse when she was poorly and she would always be yellow, darker when poorly.

 

When moll was 9 she was tested for addisons disease. I googled addisons and was terrified This was found to be negative and investigated no further as apparently her adrenal glands worked.

 

It was around this time that she was in hospital for a different reason. Her appendix burst. That was also very dodgy too, I thought she had a sickness bug from her little sister but the dog would not leave her alone, to be fair the dog had seen her in all sorts of states but this time she kept nudging molls face, trying to move her and kept barking to the door, it was weird I cannot explain her behaviour and am not exaggerating it to you, Moll can remember this clearly and Lilly our Labrador was the reason I rushed her to the dr.

 

Dr Baghaver I believed saved molls life this day, by the time we got there moll was very dehydrated and was going down hill fast, he felt her tummy and said, she has an appendicitis, I was shocked and she was rushed over to the hospital... again.

 

We got there and a surgeon came to assess her, Moll was used to being ill and in pain and had this amazing ability to just carry on until her body could do no more. She would run around the playground with unknown pneumonia them boom she'd go down, so the surgeon examined moll and said can you jump up and down. Moll stood up and jumped up and down, although she wasn't fond of them she respected drs as a good girl would and so did what she said. Molly will tell you now it took all her strength to jump that day and it was agony.

 

To my absolute disbelief the woman said, nope she hasn't got an appendicitis she just jumped up and down!!.... ffs you couldn't make it up.

 

In the middle of the night she was getting worse and a full sized hard lemon shape appeared in her tummy, they rushed her down to theatre, I mentioned the fact that we did not have the addisons results through from Bristol yet ….. this was unknowingly my first encounter with Addisons disease /adrenal insufficiency and its misunderstanding of knowledge to its seriousness ,

 

I didn't know the true risk then but giving surgery without cortisol (steroid) replacement cover could mean death, they never had the results but operated anyway, … I suppose it had already been left very late they had no choice, afterwards she was weak but seemed to be getting a bit better. But then her body did the same sort of thing as usual and developed a raging temperature. Miracle number 2 by the way, knowing what we do now, she got through surgery,

 

miracle 3 would be the way her body cocooned her appendix in a wall of fat to protect her body from its poison. The surgeon was impressed by this cocoon spun in fat and so was moll. He said he had never seen anything like it!! … I said welcome to the world of our moll.

 

She had pockets of infection all over her body, again I was so worried, at first they couldn't work out what was wrong, Dr's talking about your child in front of your child is also very scary for them this happened time and time again. I am surprised the poor girl does not have paranoia.

 

Molly just Looked at me upon learning that the even the drs didn't know what was wrong, with frightened eyes she said mummy what's wrong with me?.

 

This hasn't been the first time I just did not know what to do or who to turn to.

 

She was under some of the best consultants in our area and yet she still kept getting poorly. Nothing seemed to follow a normal pattern. Nothing I did could prevent these colds.

 

That feeling of desperation just makes you look up to the sky and say what the hell do I do who in this world can make her better.

 

Molly was referred to immunology it would then take a further 5 years to get a diagnosis.

 

Five more years of ill and the said above.

 

The first sign that something wasn't quite right was a severe vitamin D deficiency.

 

There are also 2 areas of molls immune system which are low, she also suffers low mbl, making her more susceptible to illness and she had less resistance to fight it off, but we were assured absolutely nothing could be done about that.

 

Therefore she would keep catching every virus going.

 

This was now damaging molls chest and taking its toll on her and over the next few years she began slowing down a lot.

 

Molly started to put on an extreme amount of weight on up until now she was 2 or 3 centile lines below average for weight and height on the charts.

 

One side of her body was also bigger and much more developed than the other and before this time we were getting well periods in between the illness.

 

 

 

In these well times she was actually like any other child and would run about with the others playing, dancing, singing etc this was all about to change. The In between times had gone and Molly was more tired each day. I remember talking to Martin worrying about the fact I could no longer carry her down the steps to the car, things were be coming more difficult because she no longer fitted in the pushchair. Martin basically spent until about this age with her on his shoulders that was her form of transport. He walked miles with her on his shoulders. Moll was 3 when she named it a Hocoruss ride! And so he became the Hocoruss! We still aren't sure what a Hocoruss is . but he still is one.

 

 

 

Thankfully to nurse Lizzi and Dr Finns team at bch they spotted molls problem because of the weight gain, I am so grateful to them because if they had not done thyroid tests and referred her to endocrinology that day I truly believe she may not be here now. Miracle number 4

We had an appt very soon with an endocrinologist (I call them endos nowadays) who suspected a problem although it was very rare.

 

I was told not to google it.... that alarmed me, luckily I was not given its actual exact name otherwise google would have been on fire.

 

Whilst Molly was in the appointment she was put on hydrocortisone replacement immediately, Martin literally was sent to the pharmacy there and then. I still did not know the real seriousness of this condition. Moll was put on steroid 3 times a day.

 

 

 

We went from May to September knowing that Molly could potentially have a very serious condition. My 40th birthday party seemed stupid and insignificant now and was over tinged by the not knowing, we felt on eggshells and I really just went through the motions of living my life pretending stuff was normal but inside I was scared witless, even then without knowing exactly how scary it was I was just on auto pilot.

 

 

 

We went on holiday in the July and Molly fell asleep at every opportunity. On the beach in a cafe at our holiday home.

 

She really was not well her body seemed to be going in to some kind of shut down mode.

 

In the August the hideous tests began.

 

Because of the rarity of the suspected condition I feel that the tests have not been improved since the dark ages to say the least they were barbaric.

 

Molly was basically injected with insulin and we just had to watch her fall. It was an induced coma if you like to see if her body would kick in and save her life in times of stress. It did not.

 

 

 

Molly was pumped with orange squash and digestive biscuits every time she started falling asleep and passing out, she felt as if she was blacking out. The whole thing was hideous and I seriously cannot believe she had to be put through that, its like giving someone cyanide and then slapping them about the face as they start dying to try and revive them.

 

 

 

The test showed that Molly's pituitary gland in her brain does not send messages to her glandular system. She has no thyroid function , no growth hormone production and no natural defence mechanism against stress or trauma .. Her adrenal glands did not work.

 

 

 

Cue massive life at risk problem.

 

 

 

They did not produce enough cortisol to save her life in a stressful situation, she would basically go in to adrenal crisis which would cause cardiac arrest and all of her organs to shut down. Followed by coma / death.

The only cure to prevent crisis is to give a 100ml cortisone injection within about 30 minutes of a trauma, this can also be caused by serious diarrhoea and vomiting.

 

And yet I still at this point did not know this serious information

 

So it turned out after 14 years It was not me walking on eggshells because of molls ill health it was Molly! ... Anything could have happened to her at any time no one due to the rarity of it would have known she needed a simple injection of cortisol to save her life she was a ticking time bomb and that is why I believe despite all this that we are all the luckiest people alive.

 

So I had a phone call from molls endocrinologist explaining what the tests had revealed and that Molly needed a brain scan to eliminate a brain tumour as the cause of this pituitary failure.

 

I said to to the consultant but Molly was tested for addisons age nine and it was negative (addisons is one of the causes of adrenal insufficiency) I knew this because every day for five years an addisons information website some how became part of my computer start up process . I must have bookmarked the page whilst researching it at the time she had the test.

 

 

I could not believe that they had nearly stumbled across this 5 years before and did no further investigations. Her adrenals do work you see but they are just not told to by her brain.

 

Result the same thing a life threatening disorder called adrenal insufficiency.

 

From the mention of brain tumour to the horrible Mri, through to the results I basically held my breath the diagnosis was hideous enough but a brain tumour? I probably lost a stone in weight. Everything seemed to be going out of control . my baby girl who I'd taken so much care of all of her life has suddenly been diagnosed with a potentially life threatening and

threatening life long condition. I was in pieces total shattered pieces this was terrible I couldn't understand why the hell people around me were smiling how dare they smile when my world was collapsing around my feet. It felt like it did when my little brother died in a car accident age 21. I couldn't believe the sun was coming up everyday and life was just going on around me.

Everyone else's problems faded in to insignificance, I thought People around me were pathetic and Selfish moaning about how they had to wait five minutes in the rain because the kids got let out late from school or how little suzie was crying because she'd had a bad recorder lesson how would they feel of their child had something which could kill them in 30 minutes flat. There's a problem for you!

 

The truth though was I was probably being pathetic and Selfish myself by falling in to this big hole of doom and only thoughts of myself and Molly.

 

Our next appointment revealed that Molly did not have a brain tumour ! That night I cried the relief was immense I was sure I could not cope with anything else ….like ever again .

 

We saw the endocrine nurse and she showed us how to administer the 'emergency ' injection you know the one, the one which needs to be given immediately at a time of trauma to prevent death.

I could not believe what I was looking at and it was at this time I thought right that is it!

This is hideous! if my daughter has to live with this thing after all of those years of being sick she is going to have better than that utter crap!

My normally active take no for an answer brain was fast coming back out of the hole of doom.

Clarity!

Finally My child has been diagnosed! Surely she Will get better now and she deserves the best chance of a normal life as everyone else.

No illness and to be able to manage this condition on her own. Well this was not going to happen with the contraption before us. Martin looked at me as if to say wtf?

 

This 'emergency' I kept repeating emergency in my head .

This emergency injection came in 3 pieces . a sealed glass vial with no lid a syringe and a needle. I immediately said to the nurse is this serious?

She agreed it was totally inappropriate for it's purpose but there was not an epipen / quick pen injection for this treatment probably because it's so rare. She said that some guys at the pituitary foundation had tried to get one before but had not been succesful.

Great so now we have a chance of moll getting better she has this to cope with!, Molly would never in a million years fathom this injection and in a comatosed state after a trauma there would be no chance.

Molly was also put on thyroid replacement and growth hormone injections. Of course the growth hormone treatment came with its own designated nurse a choice of devices and even dvds!!

I could not believe that the actual life threatening part of her condition came with nothing! NOTHING AT ALL! but a contraption of an injection from Victorian times!. No instruction, no DVD , no information, no choice of quick pen device, no counselling nothing !

Nothing at all! I was totally flabbergasted! …. I still am.

 

We were then told Molly must have a medical alert bracelet in case anything happened and she required the injection. And yes of course we would have to sort this out ourselves to.

It all seemed totally unbelievable something this serious was just abandoned by any sense, awareness decent resources and medication. I literally had my hand out waiting for some kind of information pack.

This was when my brain starting turning cogs at a million miles an hour !! How in sods law is that right? You have basically kept your child alive for 14 years. Picking up the pieces after every test and hospital visit, the constant physiotherapy. I'm sure I haven't slept a whole night since moll was born I must have put her back together 100 times. And now ! Now I tell you it was becoming very apparent that the condition she has is totally ignored by the 21 century ..

 

My friend Vicki has addisons disease and came round to see me in my month of shell shock, it soon became very apparent that there is a major problem in this failing adrenal world of endocrinology.

 

Vicki nearly died before she was diagnosed it took 5 years of going back and forth to the Dr's . eventually she went to a private consultant who had tests done and she was immediately put on the life saving drug hydrocortisone.

 

Since this time she nearly died again and had been refused the emergency injection when in crisis by a paramedic when in the ambulance, She literally lives 5 minutes from my home and this news terrified me.

 

Things were to get a lot lot worse when Molly was to return to school after the summer holidays, 2013 the worst summer in the history of the world.

 

 

 

At first all seemed good, I thought I had better pop in to school to notify her teachers of this new diagnosis in the holidays.

 

 

 

              

 

 

 

Her form tutor school nurse and head of year were brilliant ! I couldn't fault them.

 

 

 

They knew Moll well and knew her history of illness, I went through her new medication with them and showed them the 'emergency' injection and how to use it, although they too agreed it was unbelievable a quick pen didn't exist they were very supportive.

 

 

 

I felt really happy that I could leave moll in school and she would be safe I was really grateful to these teachers, just the support from school made us feel less worried about the situation.

 

 

 

Moll packed her bag up with all of her books and was really excited to start back to school, she loved school.

 

 

 

My happiness and relief was short lived, The head at the school had decided she would in no way sanction any of her staff to do the injection if Molly Needed it.

 

 

 

The chances she would need it were very slim (she had gone 14 years without such incident) but no she would not even entertain the idea.

With such a serious illness it would have been decent of her to contact me to reassure me that moll would be safe in school and that they would take care of her. In fact I should have hoped for the exact opposite.

 

 

 

She did not call me write to me or speak to me in anyway at all, instead she had Molly's head of year phone me just to say that they would not be doing the injection .

 

 

 

I feel quite sorry for the woman now to be honest I was not a pleasant parent in receipt of that news. I asked what they would do if Molly was unconscious on the floor and needed her injection to survive... The answer was we would not be allowed to do it.

 

 

 

In fact at the time of they had bothered to check it out this medication is exempt from any legal implications because it is an emergency life saving medication ... I did not know this at the time either, no one had told me..

 

 

 

So my daughter has been diagnosed with this hideous problem. She loves school and couldn't wait to get back there.

 

 

 

It was like someone taking the shattered bits which were left from my summer from hell and stamping all over them whilst they were on fire, crushing me in to the ground all over again.

 

 

 

I literally could not believe what I was hearing.

 

 

 

They would stand back and watch her die because they were not trained! I had shown them what to do, they were happy to do it!

 

 

 

I phoned molly's Endocrinologist nurse in tears . Well it doesn't surprise me she stated we have this with schools all of the time, we try to dumb down the seriousness of it all because they start worrying about insurances and being sued wtf? That is wrong too!!

 

 

 

I could not possibly have responsibly allowed Molly to go to school until I knew she would be safe. It would have been irresponsible of us as parents to send her in until this was resolved, I arranged for a local endo nurse to go and train the teachers and to be fair they were all trained after school one day. I was extremely grateful for each one for doing this but surely it could have been dealt with in a more sympathetic way, there should be blanket protocol in place, written out for these children and all of their carers whether they were in school college or nursery.

 

 

 

The fact I had to keep Molly off until this happened upset her immensely. Molls was upset . she had the enormity of her diagnosis, the feeling of being so ill and now the school . to be fair how would you feel if your head teacher blatantly laid out the fact they would let you lie there and die rather than give an injection because of legal repercussions. My poor child what could I say to her ? Again I tried to protect her from this bureaucracy but she's not stupid and asked me herself if they would withold her inhaylor if she was having an asthma attack? Well what do you say to that. She loves school this was absolutely hideous for her her life was falling apart and they basically just stamped all over it. If a teacher or pupil ate peanuts in the canteen and went in to aniphaxic shock Would they stand back and not bother to give adrenaline ? Let them die? ... Same thing.

 

 

 

The head had made our terrible time 100 worse it was awful, as if we didn't have enough to deal with at that time.

 

 

 

Molly was ironicly quite well and ready to return to school after the horrordays but by the time the injection training was done she was ill again this was September. By October Molly was just asleep. Asleep every single day. All day long. Her headaches were so painful she could not even move her head.

 

 

 

I desperately wanted some normality in her life.

She was so poorly again I felt in complete dispare I was sure no one knew how hideous we were feeling this was my girl ! My baby !

We all have dreams for our kids they have dreams themselves and now things had got so bad she was alienated from any normality. She just wanted to go to school be with her friends and She was as far away from herself as I could ever imagine, at this point I literally felt like banging my head against the wall. My baby girl had been saved ! She had a diagnosis an explanation for her life long illness, I was obviously smacked to the ground with this news but a small part of me was relieved because surely now she would get better.

 

 

 

Things stayed this way for for sometime I was angry scared and loosing control of any impact my help would ever have in make moll better. I'd always made her better. We'd sing songs at 4 in the morning whilst steaming her out in the kitchen (i can't count how many kettles we got through when she was small) I'd spent nights on end holding an oxygen mask wafting near her nose because she couldn't tolerate a mask on her face.

Night upon night of physiotherapy getting no sleep because if that gunk took over her lungs she would be really poorly. All of that effort and now it seemed there was nothing anymore I could do to help her.

 

 

 

To be fair I walked round like a zombie eating Breathing and living making Molly well .

She had all of this to deal with and too was completely gutted that her school would rather let her die than give her the medicine she needed.

I mean I tried to shelter her from the enormity of it all but she's a bright girl, I tried in my mind to think of the positives and the things I could do to help moll.

So she had this thing but it's liveable she would be ok. Surely at some point she would start to get better. And when she was better what was she going to do?

How could she be safe walking about with a Victorian mechanism to save her life of it needed to. How would her 14 year old friends cope with that?

We had already seen the reaction of the adults . If there were an epipen a quick injection pen moll could do that , her friends could do that, her teachers could do it even a toddler could click a pen in to someone's thigh.

 

 

 

My anger soon replaced the upset how the hell could this be right?

Surely someone had to change things. I formed an awareness page and set up a petition my life was now focused on making Molly better and giving her the best chance at life as anyone else, realistically she had been poorly all of her life and hasn't really had the chance yet to have a normal life.

Within 3 weeks I had 3000 signatures there was obviously a big problem here. I started receiving emails from parents all over the world with similar problems. Also many problems with care, awareness and dangerous encounters at hospital when people were not being given their emergency injections because Dr's or paramedics .

One incident sticks in my mind of a beautiful little 2 year old from Texas he had died because he was not given medication soon enough, he was the most beautiful little boy it was heartbreaking to talk to his mum. To be honest I have known about this now for just over 2 years and have heard of at least 7 people including children who have lost their lives due to Dr's not recognising the problem. Last year our community, my new family had lost 4 children. Many more have had close escapes, one friend was left in a corridor on the floor in full adrenal crisis this is in the uk, .. no on would give her the emergency injection, I have read 4 inquests which state that more awareness has to be raised to this condition one judge even recommended that NICE look in to this and have some guidelines drawn up. By February my petition had reached 10,000 people I have them all here now printed out and ready to use. Reasons for signing are so powerful many people are totally in disbelief that this sort of thing goes on in this day and age, It was really quite amazing I have met so many new friends. I have friends all over the world now who totally understand what we are going through. Which is good because some of my closest friends did not realise the severity and enormity of what was happening to us... I haven't seen them for dust .

 

 

 

Molls was upset . she had the enormity of her diagnosis, the feeling of being so ill and now the school . to be fair how would you feel if your head teacher blatantly laid out the fact they would let you lie there and die rather than give an injection because of legal repercussions.

 

  

 

 

 

 

I met a new contact who was a dr he had a baby girl with the same condition as molly, we met through my petition and became friends, he had some great contacts / colleagues in the medical field who had many years of experience in these things, an amazing chain of events starting with my will power on one afternoon a petition and my new dr friend actually led us to the missing piece of jigsaw.

 

 

 

Someone who worked in the industry who wanted to help, he had worked on many major medical breakthroughs in the 80's and was part of a team who had bought some much needed medication to the market also in the field of endocrinology.

 

 

 

He set up meetings with relevant people and also visited the two of us, he spent quite some time talking to us and really wanted to help. I could not believe what was happening it was like winning the lottery we were ecstatic ! At our meeting he showed us a device his company already have in use, he was amazing he really cared about this and spent a long time going through what they could do and the processes they would go through. The difference here from previous times is that they have the device already in use in hospitals its a matter of putting the medication and device together and more importantly he wants to help, no profit will really be made from this because of the numbers involved but he seems very committed to seeing the project through, and has a factory ready to produce it.

 

 

 

I cannot wait for the day that this happens so many lives will be saved, and so many people will be able to live safely, I still have the petition and will give it to them to use it if any difficulties are met along the way, so for now I have taken a step back and shall let them get on with it and await good news.

 

 

 

Martin was under the impression that all of the work involved in the epipen campaign would kind of finish now, it had taken a lot of effort and time, from other people too who were also getting paper signatures from different parts of the country and sending them to me. We had many stories in the press there is massive interest in this and complete disbelief of the stories being told regarding awareness and medication.

 

 

 

I could not possibly leave it alone I was now getting emails every day from parents who were

 

 

 

having awful problems with schools not understanding the condition, unwilling to do the injection and not taking the sick day rules seriously. To be honest with you a sickness bug could kill our children.

 

 

 

Molly's school didn't seem to understand this either and once refused to tell me if a sickness bug was going around, something had to be done about this, also advice on medication and treatment from professionals varied so much, and advice on what to do if your child is sick, some say double medication some say triple it. Some consultants do not even prescribe the emergency injection and the patient has never heard of it never mind the fact they need a medical alert bracelet, also not one parent I have spoken to feels 100% safe when taking their child to A&E because of the non understanding of the whole thing.

 

 

 

At Christmas time 2015 in the states a two year old's mum was begging drs to give the emergency injection to their child, they did not, she died. There are some great organisations who are making sure protocol in the states is updated, the emergency services there do not even carry the emergency injection never mind administer it, thankfully they have managed to change this in a lot of places but awareness has a very long way to go.

I could not believe the amount of stories be in sent to me regarding very late diagnosis, understanding and general worry. People being denied there life saving medication. Surely every person diagnosed with this should be given a pack of information detailing the risks of adrenal insufficiency, a medic alert bracelets. A detailed letter from your Dr, a red flag emergency protocol for anyone to be able to follow. I contacted Jeremy hunt who told be to contact nhs England.

The guy there spoke to me for quite some time and agreed it was something they should provide. This is what I had in mind http://rachelpeglerartworks.wix.com/savinglives#!the-simple-ai-pack-/c19u6 nothing expensive just the basic requirements I would have thought would be provided for a condition of this kind.

There were as I said also huge problems with ambulance protocol. Even they would not give the injection. An ambulance trust from the south east of the uk had actually lost a patient due to this and so has written a protocol with The patients mother to insure everyone is now updated on adrenal insufficiency and crisis. There seemed to be no pattern in the UK of a localised problem it was quite general, I wrote to each ambulance trust in the UK and asked that their protocol be updated. I had some really good responses and just hope has made a small bit of difference. The pack I sent out contained the south east's protocol following the death of the young man, also contained a paper by one one our top endocrinologists who advocates listening to the patient. And a simple poster which have been circulated, as for the nhs they say they are looking in to providing the packs to newly diagnosed patients but I have heard nothing since. It needs pushing by someone in the medical profession,

I put together a pack for schools for download and print which just had basic information about what to do in an emergency situation , what to avoid , this should be standard, every school or work place should have all of the information needed provided by a health professional.

I have so many stories emailed to me , sent to my awareness page or messages sent through the petition detailing terrible incidences of miss diagnosis, late diagnosis, and very poor understanding in emergency situations, deaths just seem to go under the radar its like some underground forgotten world, The page I started was really just an awareness page, im not a medical professional but am an artist, I have worked in advertising and know what catches peoples eyes, visual stuff! I just began producing awareness posters which hopefully would attract and interest general people. It seems to be working, I have over 2,100 members now and in just one week one of my posters reached 26,000 people. I do not want any thanks for this or recognition If I can save just one life that's enough. I started an awareness day on june the 6th each year and for 2 years running have held worldwide molly's picnics, last year we had well over 100 picnics all over the world. I just feel so so passionate about the whole thing, no one should ever have a childhood like molly and if one person can be diagnosed because of the reach we have made well that will better better than anything.

I contacted a really well respected professional I had heard a lot about, I really wanted to do something useful to help with this obvious problem and although have a team of people who back me and spur me on I felt I needed someone solid to work with, unfortunately they did not appreciate what I was doing and I feel got the entire wrong end of the stick and presumed I was somehow attacking the profession, offending them personally, to be honest this was as far from the truth as you could imagine, egos I have found really take over the need for talking, one nurse I told about the petition just said pah! Good luck with that in a sarcastic manner. I cannot be assed to email her with the 10,000 signatures I managed to get. I find the whole thing really weird there is a patient side and a professional side …. I am a black and white kind of person if something is wrong I have to put it right and this is wrong. I have on one side literally 100's of people contacting me with these awful problems and on the other side people who could help with this thing treating me like i'm some kind of thick dr hating idiot … the conversation turned quite nasty, I tried to explain I was not insulting anyone I simply wanted to help raise awareness, money for research in to better medications and treatments if I can reach 26,000 with one poster surely im not that stupid and really could make a difference here. This conversation still upsets me every single day and has really limited what ill do now, not that ill ever give up because I have to many people who cannot also be wrong behind me.

So thats it really we are up to date, Moll as we speak is lying on the sofa, she has another cold and im monitoring her oxygen levels, each time she has a cold now she seems to be getting worse, close to Christmas this year we had to call an ambulance because her oxygen levels we so low and her heart rate so high, we again barely made it home for Christmas, it was only because she broke her heart in front of a dr on Christmas eve that we were allowed to leave the hospital on the full understanding of if she deteriorated in anyway we would call 999 and go back. To be fair this was toi worst Christmas we ever had. Although as well probably the luckiest, at least we were all together I cannot imagine my little primroses face if father Christmas had not come on Christmas morning and she had to go to the hospital to see molls, I suppose though having a poorly molly has made us the most together strongest family unit I have ever known. Primrose 4 does molls zips Pop 12 does her shoelaces and josh 21 pushes her until his arms are numb up hills in her wheelchair, we all know moll needs a bit of extra care and I think this has made my other children in to caring thoughtful non shallow individuals who know what is important in life, selfishness and meaningless trash doesn't even enter their psyche and I thing that's a good foundation for life. We still do not have a full diagnosis for molly but will keep pushing on, we have just met a new consultant who has a lot of experience and seems to suspect a few conditions which could be responsible for her overall unwellness. Molly no longer attends school she is too unwell and is in a wheelchair, her school were not able unfortunately to understand her condition, they ignored every single email I ever sent them regarding the epipen campaign or Mollys awareness day, other schools in the area and all over the country held picnics and events, also raised awareness in assembly and did non uniform days' they could have done a lot in the way of support but molly ended up being pushed out of school because I believe she was bad for attendance records and was not any good for them grades wise.

Its ironic out of all of my children she is the one who enjoyed school the most, because they did not read my letter of notification that I was to home educate properly she did not even finish year 9,

she turned up at school after the Easter holidays to be met with oh molly we thought you had left . (this was the teachers)

I then complained to the school and told them my letter clearly said she would be leaving at the end of summer term they then foolishly backtracked and said they didn't know when I said she was leaving …. it made no sense at all because the teachers were told she was leaving at easter and they had told molly that that very day before I complained, I wondered at this point if they did in fact have any qualifications at all. Moll walked out of tht school that day with no goodbyes no prom no nothing, it broke my heart no one even noticed and we have not heard a word from them since, apart from a snotty letter from the head who blamed me for it ! Unbelieevable. So This is the story of rare disease and how it affects a family, I really hope if you have read this that you may take the time next time to try and understand someone's life if it is different to yours, because without bringing normal unaffected by this people e have no chance of making a difference in this world. And if you know any wizard please send them over here because more than anything in the world I want my molly to be well.... Thanks for reading and if you think you can help or support please join our story here https://www.facebook.com/rachelpeglersavinglivesforsterioddependants

 

 

 

 

 

 

 

 

 

17. Apr, 2016

Published for Rare disease day 2016

http://www.rarediseaseday.org/stories/5359

17. Apr, 2016