Ivy'v Story What care and support is available for those with deletion 22q11 syndrome?


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Max

5th November 1998

MaxMax was born by planned caesarean section at 39 weeks (this seemed like a good idea as Georgia had weighed in at 9lb 8oz). He was a suprisingly small 7lb 12oz but not small enough to worry about and he got 9 out of 10 on his apgar score 5 minutes after he was born. Paul said, "Ooh, look!! small ears!" (This was because Georgia's stick out and I always said that was the reason she couldn't get out!) I thought to myself "Crikey, those are small. For goodness sake; you're never satisfied are you?!"

We were taken up to the ward where it was noted that I was losing quite a lot of blood and that my kidneys had decided to take a short break. The day passed in a haze of drips and proddings and Max was taken to the nursery for the night so that I could rest. I was woken at around 11o'clock by a midwife saying that they weren't happy with his colour when he was feeding, or trying to feed (I'd agreed to bottle as I just wasn't up to trying to breast feeding and he hadn't the slightest inclination to latch on) so they were going to have him checked over in SCBU. I wasn't too worried about this as Georgia had been to SCBU too because she had been snuffly and came back 24 hours later. Special care weren't happy either so they called the paediatrician. It was turned midnight by this time and half an hour later I was informed that Max had a heart defect and that he was being transferred to Birmingham Children's Hospital immediately. I phoned Paul who came back to go with Max and away they went in a blur of blue flashing lights leaving me in my wheelchair at the door at 4am in the morning.

7th November

Max and GeorgiaI visited Max two days later with Georgia and I can't remember much apart from being confused, totally overwhelmed and frightened. Max looked small and very vulnerable and very sick with a big bandages and tubes on each wrist.

9th November

MaxI was discharged after my four pints had been transfused and I felt very perky. We went straight to see Max and sat with him for quite a while, when a man in theatre greens appeared. He said he was going to assist the anaesthetist and we assumed he meant for the cardiac surgery which was planned for Thursday. I thought it was bit strange for him to be there at 9pm and he looked at us as though we were odd when we carried on chatting and generally admiring Max, so I casually said "So when do you think you'll be doing the operation?" He looked confused, consulted his watch and said "In about 15 minutes." I was horrified and blathered something about his heart not being that bad and he said "His heart? No, I'm assisting with his gut operation." I was flabbergasted. "His gut!!! I know he's got an infection but he's having antibiotics!" (oh! Naive fool that I was!). "But it's all been explained to your husband". Paul sat there grinning like a village idiot and as I rounded on him the surgeon arrived in a flurry of white coat, gently palpitated Max's stomach, nodded sagely, hummed a bit and then said that they'd 'have him down straight away'. He then explained about necrotising enterocolitis as only Mr Gornal can. It went something like "We'll open him up I'm expecting to find gangrene I'll remove anything that's putrefying. If it's not too bad then we'll just stitch him up and rest his gut for a few weeks.If it's bad then we'll have to create a stoma. You know what that is don't you? We just get a piece of intestine and bring it to the surface.If it's all no good then we'll close him up and let him go peacefully OK? You may as well go home and I'll call you." A nurse took care of us as we were obviously totally bemused and we went to have a look round ITU. Walking on to ITU at midnight for the first time felt like walking onto the bridge of the Starship Enterprise - all bleeps, technical screens, subdued lighting and completely alien.

We went home and waited for Mr Gornal to phone us. We lay in the dark clinging to each other, terrified, and jumped out of our skins when the phone rang. Paul kept saying "Yes, right, OK" and so I leaned closer and heard Mr Gornal say "He was very rocky on the table, I thought we were going to lose him, so I just pulled out the first thing that came to hand to make a stoma.As I said it's not looking too good and we're doing what we can to keep him alive." Paul eventually put down that phone and I said "So, what did he say?" meaning that I wanted every word, verbatim. Paul replied not too accurately: " He says he's going to be OK".We very quickly learned to hear what was said, not what we wanted to hear.

Max had 12cm of jejunum removed. The jejunum is the bit that comes after the duodenum (which is attached to the stomach) and before the ileum, which comes before the colon. The jejunum is one of the most important pieces of gut for food absorption and as Max only started with around 15cm this was quite a lot to lose and had big implications for nutrition in his future.

We saw Mr Gornal the next day and he said, "It's good to see you two today, I didn't think I was going to!". Mr Gornal is not one to mince his words which we were later to value most highly. He looked dubiously at the stoma, which he didn't like the look of at all. The nurse tried to convince him that if she shone the pen light on it then she could see pink quite clearly beneath the black. He was still dubious until a big splurge of green slime erupted from it at which he became totally ecstatic and all but hugged both of us before trotting off up ITU with a lively spring in his step. leaving us completely dumbfounded!

14th November

The new carpet was fitted in Max's bedroom.

16th November

Max showed signs of a chest infection.

20th November - 23rd November

Max was re-admitted to ITU with suspected sepsis, treated with antibiotics and ventilated to try to control an 'acidotic' episode.

Acidosis was explained to us and my understanding is that in very basic terms the body works in a very narrow pH range due to the chemical compound that proteins are made from and proteins form enzymes. Air is inhaled by the lungs and transported by the blood so 'gases' are always found in the blood. These can then be analysed and give a good indication of how a persons metabolism is coping. When a body is struggling it cannot use all the air that is inhaled effectively and hydrogen can then stay in the blood (H+ ions being acidic) and when this builds up then the body becomes acidotic.This causes a downward spiral as the person then breathes faster and faster to try to get rid of all the gases but in fact makes the situation worse.
MaxMax got better very quickly and was returned to the ward on Total Parental Nutrition (intravenous feeding) on 13th November.

26th November

A brain scan and EEG are carried out. While we were in radiology Mr Chapman made several attempts to carry out a contrast x-ray study that involved poking a tube filled with barium in to Max's stoma but he became very distressed and when the barium dissipated in to his abdominal cavity a second time the study was aborted.

27th November - 28th November

A quick third trip back to ITU with suspected sepsis or perforated/abscessed cavity in his gut. This was nothing to do with the previous day's debacle I was quite firmly re-assured.

30th November

I stumble across a big pow-wow in the corridor between the cardiac and renal teams. Max's kidneys have stopped working and he's filling up quickly. They think he has a severe bout of sepsis so we go back down to ITU for the fourth time and he's acidotic again. This time his baseline deficit is in double figures and they're worried (+/- 0.5 being the normal range).

2nd December

The upper lobe in his right lung collapses again and we're starting to understand the progression that the respiratory physiotherapist uses, from 'wet', 'boggy', 'soggy', 'consolidated' through to 'collapsed'. His saturations are very poor and he starts to turn orange,indicating cholistasis liver failure. Not quite what the doctor ordered.

4th December

Max's heart function takes 'a turn for the worse' and we're called in again. These things never happen at lunchtime! His blood pressure is very low and Max needs a lot of support. This could have been because he gets upset while Paul Miller (Cardiac Registrar) is doing and echo and thrashes about so much that he pulls his haemofiltration line out. Unfortunately it was attached to his jugular at the time and people were still mopping the floor and looking jittery when I arrived. Better up the sedation, eh? A couple of days later I am collared in the corridor by Paul Miller who quite indignantly informs me "I've never had a baby try to exsanguinate on the bed in front of me before and it's an experience that I wont forget in a hurry!"

6th December

Some of the doctors are looking a bit negative and there are a few sympathetic head shakes. His kidney function might never return, structurally they look fine and the blood flow is good but with trauma and sepsis these things happen... and then miracles do happen too. He passes 18ml of urine never has a nappy been so gleefully examined! Lots of "oohh! Who's a clever boy then!type of cooing noises!

8th December

This was the planned date for his trip to theatre for a cardio-catheter, but no-one mentions it. Also the low platelets are very troublesome.Max is having frequent blood transfusions to maintain his platelet levels at 120 (normal), but he just gobbles them up and within 2 days thay are back down below 10 and as one nurse put it, 'they've taken those out to count them'.

10th December

Further echoes are carried out and the severity of his heart condition is made clear to us. No rising pulmonary vessel, phased prosthetic implants, can't do a major operation like that in one go and it will need changing as he grows. A VSD the size of Europe, can't do a repair like that all in one go and it will need to be altered as he grows. No pulmonary valve,that will need to be replaced periodically. MAPCA's, inoperable until very recently. Luckily we've got Mr Braun

It was also around this time that Andrew Tappman, one of the anaesthetists on ITU, took us in to a little room and gave us the prognosis with both barrels that no parent wants. It went something like "Max is juggling too many balls. He hasn't got a single fully functioning organ or system in his body. He won't die today, he probably won't die this week, it might not even be this month but he is going to die."

12th December

The jaundice is getting to be quite a problem with his billirubin levels now over 350 (around 15 is normal). There's a bit of a hoo haa as to whether it's the conjugated or unconjugated counts that are too high and he's started on oral feeds. We don't get too excited as it's not like we can feed him with a bottle. He's put on 1ml per hour continuous nasal gastric feed of pregestimil (semi-digested milk). The platelets are still worrying and it's decided not to transfuse him unless he's under 10 or actually bleeding - he's completely cleaned out the West Midlands Blood Bank and the last lot of compacted irradiated cells had to come from Derby or Nottingham so he's had too many and is in danger of developing anti-platelet antibodies which wouldn't be too helpful!

Then the haemofiltration canular fails and he hasn't got another blood vessel big enough to cope with a canular that size. What do we do now? Everyone stands around for a few hours looking uncertain

During the whole of this time Max has had 50% of a paediatrician (Elena Brooks shares the paediatrician with Max), a nurse to do the usual ITU things and another nurse to look after his haemofiltration so when things stop happening the change is very noticable we wait for the plan...

"We'll withdraw the morphine and the paralysis and the hypnotic drug (these are used to induce a coma-like state so that the baby can't feel, think or move and it's easier for the medics to take control of the metabolism) as sometimes babies just don't want to come out to play again after they've been through the sort of traumas that Max is going through. We'll give it a couple of days and see if there's anyone in there.."

13th December

Max opens his eyes and starts to cry (silently) which is more than wonderful for the first few minutes but then upsetting as he starts to try to extubate himself with arms that are angry purple windmills and has to have chlorolhydrate to quieten him down. It also causes a few problems as this wasn't supposed to happen. What do we do now? Max has beaten the odds again.

14th December

MaxMax goes to theatre to have a peritoneal dialysis canular inserted 'under sight' and a quick laparotomy while they've got him in there to see if his gut is 'viable' (we didn't ask the 'what if it isn't viable' question.). This reveals that he has lost 75% of his peritoneum (the bag that hold the bowel together) so peritoneal dialysis might not work.But he has three free floating bits of gut that just might be enough to join stomach and bum.He passed 100ml of urine but it grew staphylococcal and enterococcal bugs. We don't know whether to laugh or cry so we ask if they'll give him another platelet transfusion so that we can give him a 'good smacked bottom'!! My mum always said that it worked a treat with us!!

Suzie Hutchinson, our cardiac liaison nurse, puts us in the picture: "They can do bowel transplants, they can do liver transplants, they can do kidney transplants and they can do heart and lung transplants but not all on the same baby." And really what Max needed was a full body transplant...

19th December

PD cannula is taken out - urine output is shaky but steady.

20th December

Extubated

21st December

Returns to Ward 11 to a hero's welcome weighing a bone dry 4.1kg

25th December

has to go to ITU (5th visit) due to an acidotic episode and breathlessness - suspect sepsis once more but then again maybe he just wanted to say Merry Christmas to his friends in ITU! He is put on some hefty antibiotics again.

27th December

MaxReturns to ward 11 - and it felt like the whole world passed through, stopped mid-stride, did a double take, came closer to the bed and said "Oooh? Is that Max? Ahh! I'm so pleased because I really didn't think he was going to make it this time!"

1st January

'Lost' the long line. Phoned up from a friends house party to say Happy New Year and had a bit of an 'eppy' down the phone - "Did it just jump out all by it's self, or what?!"

2nd - 3rd January

Another trip to ITU (he obviously gets bored with the scenery in Ward 11) this time due to apnea and hypocalcaemic seizures. The fits are not nice and unfortunately I have a cold and am banned from getting anywhere near him as respiratory physio is a thrice daily event and we have heard mutterings about chronic lung disease.

4th - 5th January

Max goes to theatre to re-site his triple lumen (long line) and to have a Broviac line inserted. The latter is a main arterial line that is stitched into an artery in the chest and is basically 'permanent'. He comes out of ITU quickly.

7th - 11th January

Max finally has his cardiac shunt operation. This is normally a very low risk procedure as it involves a conduit being put in to allow blood to flow from the right sub-clavian artery to the pulmonary artery. The ductus can be allowed to close and Max can finally come off the prostin. This is a hormone derivative and in Max caused prostaglandin bone deposits in his hips and knees, which would need to be removed before he could move freely. The only hitch was that Mr Sethia couldn't find Max's right sub-clavian artery so they just dug a bit deeper and made it a central shunt instead of a BT shunt. His 8th visit to ITU is nice and easy and he comes off the ventilator with out too much of a problem.

13th January

Max is noticeably stronger and more alert and happy - that is until he goes to radiology for a bone scan as it is thought that he has osteomyelitis in his right shoulder - this is a nasty infection that lingers.

The bone scan involved being put on his back and held still for 10 minutes, then put on each of his sides for 10 minutes and held still and then on his front and held still for a further 10 minutes. This is a baby that has spent nearly his entire life flat on his back and doesn't like being fiddled with as it normally involves a lot of pain, so it didn't go down too well. When his saturations dropped to 40 I had to call it off as he was suffering and I certainly couldn't endure that any more.

14th January

Another middle of the night crisis call - spot the working organ again for his ninth spell in ITU - as he does the acidotic baby trick just a bit too well. The normal baseline for a blood gas is +/- 0.5. The first time he went to ITU his baseline deficit was -9 and that was severe. This time he topped out at -25.4 - a record for Birmingham. It transpired that the shunt was too big and he developed a periocardial effusion. Dr Wright tries to tap it but is driven back by the angry purple windmills. Dr Giovanni tries his hand and drains 100ml of haemo-fluid from around his heart.

18th January

Max is started on immunoglobulin and B & T cell counts are sent for. With all the transfusions he had had it was unlikely to reveal an accurate picture. He goes back to the ward.

26th January

MaxA group of students are brought to have a look at Max and try to decide what's wrong with him.I thought this was a bit mean of Dr Wright really as they obviously hadn't seen a sick baby before. One asked if his necrotising enterocolitis scar was where his nappy had been put on a bit tight and another one asked whether his stoma was some sort of a nasty sore or rash! And then they couldn't decide if his heart murmur went 'wish woosh' or 'woosh woosh' or just constantly 'swoosh'.





MaxBut I didn't care really as Max was the best he'd ever been, he started to smile and he was happy.We start to think about how long he is going to be in hospital for... a long time, possibly more than a year? We would organise ourselves around our 'new life'. I would come in in the morning and leave at 2.30 to pick Georgia up from school, she can't be pushed from pillar to post for ever then Paul coul come in for a couple of hours after work, fabulous.Things are starting to look so good we can't believe our luck, he's really going to make it Even warnings from Paul Miller, like "Don't get too carried away, Max is always on the brink of ITU, he's never well" fall on deaf ears...

27th January

His long line fails and he doesn't have another available spot to put one in so Dr Wright manages to re-wire the old one in theatre and is very pleased with himself.

28th January

The neurophysiotherapists do an eye scan and various tests on Max and are pleased with the results - he does have one bit working OK we found it at last it's his head! His abdominal scan looks good - if he carries on like this then he can have his stoma closed soon...

29th January

MaxHis first bath, I'm so nervous and so is Paitra. Paul had played a trick on Paitra a couple of weeks before: she had been looking after Max when he collapsed with the hypocalcaemic seizures and had been very upset by the fits, so when Max came back up to the ward Paul got Amanda, one of the other nurses, to say "oooh Paitra! Come quick Max is having one of his fits again." At which Paitra ran off the ward crying saying that she couldn't face Max again because he'd been through too much for any baby to bear.

Paul bought her a bottle of wine to apologise but she was still nervous of Max. I felt the same as every time I plucked up enough courage to hold him it seemed as though he'd promptly collapse and end up in ITU. But between us we managed, despite the audience that had gathered to witness the BIG event that was Max's first proper bath like a big boy!

4th February

MaxPaul gives Max a bath - Max is a lovely baby. You have to work hard for your smiles but once he starts even a blood gas being squeezed out of his foot won't wipe it off his face. We are the original stupid, dopey, doting, parents.
MaxGeorgia gets to have a hold and sit him on her lap and declares that it's the best day of her life... Someone (I can't remember who) mentions that he's growing another bug - candida - and does it so that I'm suppose to understand that this is significant. But he's doing too well and I prefer not to pick up on it...

4th February

Yet another crisis call - ITU are angry. "Why have they let him get into this state? It was spot the working organ again and we knew it was his brain because he cried when we picked him up!" This is our tenth time in ITU and we've been in just about all of the 19 bed spaces by now having been moved a couple of times when he needed more space. And we know people well and they know us and they know Max so when two of Max's favourites, Jo and Nicky said they didn't want to look after him this time we were upset. I tackled Nicky about it. "We can't cope with seeing him suffer anymore, I can't sleep at night, I worry about him, I'm too close to him, neither of us can do it anymore."

10th February

Max's ITU partner Elena Brooks dies in theatre due to MRSA endocarditis. Max and her shared the title of sickest babies in the hospital for some time We feel sick and we try to help Mary and John but it's just a bit too close to home.

11th February

Max's closure is cancelled. Devastated. Angry someone else had his ITU bed how dare they... they shouted louder than us. But that baby isn't half as needy as Max..etc. etc. .. but it doesn't change anything..

12th February

Max starts with a cold. He seems to be coping, so we leave him and see if he can manage to deal with it on his own. It's only a cold after all. But the SHO's have changed and we've lost Tammy and Emma who are our stalwarts, they know Max inside out and we trust them. I'm not happy, I'm reassured that the new ones have been briefed but they avoid us...

13th February

Max is not well... I just know it,he's not happy... he's developed an oxygen requirement over night and if the mask is moved his saturations drop instantly... he is not moving well.. I'm feeling frantic but I go home to fetch Georgia from school anyway and just phone up later. Paul visits but Max is asleep so he just sits with him for while... he's worried too.

15th February

Max starts with a rash. He's done this several times before, in fact it's one of his specialities.The renal team drop by " it's nothing to do with us.". the liver team drop by "it's not our bit", haematology drop by."Not us." General surgeons take a quick look. "That looks nasty but it's not us you need." So when Dr Stumper (Cardiac Consultant) peers at him and says "Oooh! That doesn't look very nice. What is it?" I'm afraid I'm a bit sarcastic: "Well, it looks like a rash to me but I'm only a parent. You're a bloody doctor so you tell me!"

Eventually the dermatologist gives an official diagnosis of 'generalised, non-specific, blanching purpura'. That means a red blotchy rash that isn't meningitis and from the general sighs of relief I know why they were so worried this time!

His chest is bad, it's a pseudomonia, a very nasty infection, so he's started on a cocktail of super strength antibiotics, vancomycin, gentomycin and teicoplanin and something for the candid

21st February

Max is doing well.It's been a hard pull but he's rallying and the decision is taken to put him on a short tube with a view to doing the closure immediately i.e. without completely extubating and popping him back to the ward. Ward 11 refuse to take him anyway as they've got rota virus and don't want to expose him to the risk so he's got to stop in ITU.

22nd February

ITU is chocca, so Max is extubated and put in a head box but kept downstairs in ITU while a plan is made and then he slides a bit and has to go back on short tube.

24th February

Something is wrong, his platelets are all to cock again, but they want him fit for theatre so he has a platelet transfusion and then results come back showing that the candida is rife and flourishing.He is swabbed everywhere and samples are taken from every line and vein that they can find.

26th February

Max is back to being fully ventilated.. it's not looking good.. he's on liposomal amphotericin which is the most effective drug for candidal septicaemia but they didn't want to resort to this as it damages the liver function and Max already has a 60% liver dysfunction. Mr Gornal wants to carry on and do the closure and change the Broviac line at the same time as the candida has colonised the line. But, Mr Grey the microbiologist, says the candida must be treated through the line (never met him even though he makes this decision). We're horrified and plead with Mr Gornal as he walks away from us and out of ITU: "but you don't understand, that's never worked, the line always has to come out" I feel bereft and that day our best ally, Registrar Catherine Penrose, left the hospital. It's all going very badly wrong.

27th February

We go shopping first thing and come back home to two messages - "Hello - ermmm this is ITU Max has taken a turn for the worse.. it might be a good idea for you to come in - soon". I leap in the car and race to Birmingham - Paul arranges for someone to have Georgia again (I think some people thought that we were making it up when we kept saying that we were summoned to ITU!)

It's worse than bad Max is being hand bagged (manually ventilated) and is massively acidotic. The scale on ITU stops at around 180 H+ (hydrogen ions) in a blood gas as the body is so acidotic at this level that enzymes and proteins are denatured. Normal is about 40 and at around 80 children start to die. Paul Miller says that he's never seen a baby come back from more than 200 Max is at 247. "Even Max can't survive this."

The vicar is called and we have a bed-side christening with both eyes firmly fixed on the monitor the second he's done, Max is being hand-bagged again then someone says "it's very stiff, what if the tube is blocked off?" It's risky, very risky - if they can't pass the tube again then he dies.. But they can't hand bag him forever and they've been doing it for hours already... It works and the elation is incredible. Paul Miller eats humble pie... Max has beaten the odds AGAIN!!

28th February

MaxMax starts the day as well as can be expected. They re-pass the tube, hoping that it was blocked again and that is why his ventilation requirement was going up and up. But his chest is very grim and his lungs are oozing and this time the tube wouldn't go down his nose. It's in his mouth and for a baby who loves his dummy this is not funny at all Out of all the horrible things that happen to Max, the absolutely most heartbreaking was watching him try to suck his ventilator tube.

He gets upset and has to have chloral again as he is really mucking up his numbers by having a paddy even his soothing music box is a very temporary solution.

Paul Miller does an echo "How is it looking?" I ask "Pretty bad. With the amount of adrenaline he's on his heart should be jumping out of his chest and he's on very large doses of calcium for ionotropic support. I know I've said it before but I really think you ought to prepare yourselves this time." And somehow this time I know he's right.

1st March

MaxWe phone ITU before we set off to ask questions. What's his ALT and ALP? Not on the scale anymore.. What's his billirubin? More than 550 and then the big one: Has he come round at all? No.

We know that chloral only last a few hours at the most. We know he doesn't want to come out to play anymore and who can blame him? It hasn't exactly been a barrel of laughs for him...

My dad comes along to see Max one more time.
MaxWe go to the hospital and clear his bed space on the ward, which they've kept open for him, because they wanted us to know that they were looking forward to him coming back. It's the grand round and I don't want to speak to anyone this is a private thing and part of our grief...

We go to ITU. We have a meeting with a very upset Dr Wright and Gale Pearson who knows how to say and do the right thing at the right time.

Max is moved in to a side room so that we can say our goodbyes with privacy and Max can die with dignity.

We have all day and we need it because the hardest thing we shall ever do in our lives is say; "Ok, let Max die now."

10th March

MaxMax is buried at 2pm - we are staggered by the amount of people at the funeral - over a dozen from the hospital. John Lennon's "Beautiful Boy" is playing as we walk behind his coffin. I feel small and insignificant without my brave boy, like somehow his death has diminished me.

We had cards galore, including a beautifully hand-painted one from Lorraine, one of Max's nurses.

It felt wrong for a long time when the sun came up, just like it did before, and the world carried on just like it did before, only now my baby was dead. I didn't cry a lot, I could just smile or talk to people it felt like a sort of facial paralysis I would look at people and be thinking "oh dear! They're going to need a reply soon now how do I work my face? How do I smile?"

But we were lucky. We had 115 days with Max, and saw him smile, and know who he was, and we learned about ourselves and the people around us. We were lucky that Max was able to go to Birmingham Children's Hospital, where, if just caring could keep babies alive, then we'd still have Max today.

We decided that Max's life meant more than his death and so we made two fundamental decisions:

We're going to have another baby: Archie was born on 4th January 2000
We're going to set up the support group: so Max Appeal! came in to being.