Mikis Story By Amy, Mikenzee's Mum

Well tomorrow My beautiful little princess turns 2, I cant believe the last two years have gone so fast, It seems like only yesterday when she was born, yet I can hardly remember life before we had her, Miki is my hero, my strength my everything I love her so much, and I have learnt so much from her, Miki is such a fighter and never gives up no matter what is thown at her Miki wil always bounce back and surprise use time and time again.

mikis journey has been hard on her, her struggles started shortly after the birth she went into respiratory distress, at the time we had no clue why assumed it was due to her being 4 weeks early but we now know it was due to her Laryngomalacia, she had some breathing assistance for a few days and all seemed good at the time she was being nasogastric fed, Then the hard part came trying to feed her.

First I tried to feed her my self but she was unable just screamed and arched away, and each time I was told she is just to weak to feed so we topped her up with the tube, then after speaking with the nurses and trying to nurse her I resailed that if I wanted her home she had to feed orally and the only way to do that was to try her on a bottle so we did, she still couldn’t feed so I started to squeeze the milk into her mouth and she would choke and cough and splutter every time, at the time I didn’t know what was rite and what was wrong I just went with the flow as Miki choked and coughed and puked (all babies reflux I was told ) Then we found miki had tongue tie and needed it fixing and thought once that was fixed she would be able to feed properly, I remember the morning she was getting her tongue tie clipped, I remember thinking my poor baby that’s just dreadful, I really was devastated and thought that the clipping would be the worst thing our beautiful baby would have to go through (little did I know )

Coming home from hospital We arrived home with our beautiful baby, after being in the hospital with nurses to show us what to do we now all alone with out little baby, feeding was a constant struggle and the peeking and choking and her breathing worsened and she has the most horrendous heat breaking cry, we took her to the local emergency and was told by one nurse she had colic and I was a typical first time mum, however she did get the pead down to have a look at her, this was Dr Naidoo her pead from neonates he checked her over and told us that she did in fact had reflux and had aspirated her stomach content onto her lungs so Miki was admitted and treated for the aspiration, when we left she was still not able to feed well but we thought we was getting by. The x-ray to confirm the aspiration also showed an enlarged heart, we knew of her heart murmur but was un aware of the size

Things started to turn really bad her feeding again got worse and the vomiting got terrible she would 4ft across the room and what seemed to be her whole feed, we persevered a few days in fear of looking like paranoid first time parents, but we then realised that she needed to go to hospital as she was not keeping anything down.

We went to Princess Margaret Hospital, and she was admitted straight away to ward 5d, she continued to vomit all the time but we still didn’t now why only that she had reflux, the nurses knew there was something wrong more then the average reflux, but again didn’t know what and the puking continued to get worse and worse.

Then the day came where we found out what was wrong the Dr she was under Dr martin had been around to see her and told us he still was unsure as what was going on despite the nurses saying she was projectile vomiting, just after he left the room I started to attempt to feed her as she screamed and pulled away then all of a sudden she started choking really badly I called out to the nurse and her and Dr martin walked in to the room to view Miki vomit from one side of the room to the other, at the time alarm bels must have rang as the dr and nurse stepped out side to have a chat about her, on returning I was told that it appeared as though there was a blockage from her tummy to her intestines but she would need an ultrasound to confirm it, as she was really dehydrated and loosing weight they put in an IV line for her to get fed though whilst they figured out what was going on, we then went to the x-ray dept and she had an ultrasound on her tummy that confirmed she has Pyloric stenosis the pylorus being the opening at the bottom of her tummy, The reason this was not though of earlier as it is far more common in boys then girls we was then told that they needed to operate to fix the blockage.

That night the anethasist cam around to talk to us about her going under a GA at this time I panicked as I knew about her heart being enlarged and that her pead had booked her for further testing, on telling the anaesthetist this she ordered an ECG which confirmed Mikis heart merman, so she contacted the cardiologist who instructed them not to operate until he had a good look at her heart via an echo and Ultrasound, as if she needed the heart correcting then that was to take priority to her tummy.

We got great mews from the cardiologist that her heart murmur was caused by a kink in the valve to her lungs and I didn’t need to be surgically fixed which great news was.

Then we was introduced to the Surgeon Dr Owford she explained to us what pyloric stenosis was and how she was going to fix it, my husband I was heart broken at the fact out tiny baby was about to have an operation on what was supposed to be her Due date she was still so tiny and frail tinnier then the day she was born, we did however know that it had to be done.

The moment came where we had to take he to the operating room I held her as they put her to sleep and then I had to go so they could get started, It was the most horrible thing I had ever done, I walked out to my Mat her daddy and he was heart broken we both just stood there crying not really understanding what was going on, back then we was so new to all of this.

I went to collect her from theatre and here she was covered n tubes and wires and machines alarming and breathing for her and feeding her and keeping her warm basically doing everything for her, I was so scared but happy she was out of theatre and was on the mend, over the next few days we attempted feeding again things had not improved she still didn’t want to feed but at least she was not projectile vomiting across the room she was peeking but not as before the operation (having said that she was on 20 malls feeds) We was allowed to go home and the peeking and the screaming in pain continued, we had an appointment booked with her Pead Dr Naidoo he checked her over and was really not happy with how she was doing so he put her on medication called Lusec witch was to help stop the acid production in her stomach so that the reflux wouldn’t hurts as much and she would start to feed better, at this point we started feeding her with a Syringe about 10mls ever half hour 24 hours a day she could tolerate anything more and was struggling at that amounts eh continued to reflux and scream and choke as before.

Miki then got worse again and started to vomit up blood and was admitted back to hospital at this point the doubled her lusec dose and hoped over a few days things would settle down they didn’t they got worse she started to not only vomit blood but poo it was well, at this point we was instructed to try her on a formulary called neonate a dairy and soy free formulary that is broken down to make it easier to digest, over the next few weeks the blood and mucus seemed to slow down and so did the projectile vomiting but the pain and choking and poor feeding continued to go on.

At this point we saw the feeding team who ran some tests and told us that miki not only had reflux but also a problem with her suck and swallow coordination and her food going the wrong way, she was readmitted and her nasogastric tube reinserted and we was to do continuos feeds at night and attempt bolus feeds in the day we soon realised the bolus feeds was a night mare and not working she was them put on continuos feeds all the time, surgery was suggested at the time to stop the vomiting but we decided to avoid it was long as we could, various tests were carried out and all confirmed that she has server GERD, we tried everything to avoid Surgery, we went through food trials that were horrendous the first few days we thought this is ok she would try a mouth full but as the days progressed she ate less screamed more and reacted worse each time, At this point we was ordered to stop the food trials and more tests were run including a gastric emptying study that confirmed she not only had reflux but also server delayed gastric emptying. She was started on domperidone to help with that.

Miki wasn’t getting any better and at this point her Gi that is very antifundo suggested that we go ahead with the fundoplication so called antireflux surgery) as miki could no longer go on how she was between the pain and aspiration and the FTT Miki was really suffering so we agreed to the surgery.

Miki then went for the fundoplication surgery she was un for 5 long hours, this was her 4th operation as she also had two operations on her airways to correct another problem that’s she has which is laryngomalacia, so we was no longer new to all of this but still didn’t like it and each time as scary as the last, Miki came out of surgery and I went to get her from rectory as with all the times before she was covered in wires and tubes and alarms after improving she was able to return to the ward to see her daddy who was waiting patiently for her, she was still in a lot of pain but at least she was out of theatre.

Miki still had her NG tube in at this time as the hope was she would start eating well and digesting her food after the fundo and would not need a tube, we tied her on oral feeds and it was night mare what we couldn’t get in they had us put down the tube as bolus feed (she had only been on continuos) each feed the screaming and gagging worsened and she was in unbearable a pain, the nurse called the dr from the ER up to see what to do as the pain was obvious the Dr ordered then to aspirate the tummy and see how much had been digested and what was left upon doing this they realised she has not digested any and in fact had more content in her tummy as she now not only had feed but stomach acid, We then stoped the feeds and waited to speak with the GI in the morning she ordered to get her back on continuos feeds we had to start of slower then before as she wasn’t tolerating them we attempted bolus feeds again and again but never succeeded.

miki was then discharged with the Nasty NG tube still in place and still on continuos feeds and gagging an retching all day long, cut along story short we then consulted with another Dr the same surgeon that corrected her tongue tie as baby, he told us that he has no idea why miki was like she is or how to fix her but he would make her comfortable in the mean time that day he organised her Gastrostomy to be done a few weeks later to get rid of the NG that was causing yet another lots of problems he also introduced us to another Gi that agreed with his treatment plan, over the next few months we tried to persevere with the G feeds and even tried to get her onto bolus feeds all failing miserably , we also attempted food trials again they all failed miserable, We was still seeing her pead as well Dr naidoo he was coordinating all her care and was much more confident with the new drs involved which was helpful, at this point her tolerating of feeds was not getting any better and she has numerous lots of blood and mucus stools and was put on a med called Intal to be administered gastricly its a non steroid medication to treat gastritis, esophagitis, and colitis its seems to help with the inflammation which is good, we also consulted with a metabolic dr who prescribed enzymes to help her tolerate and absorb the feeds he also agreed to keep up with the Intal or the allergic GI system, but also agreed that both meds will not have full effect until they are administered straight into her intestines.

Feeds was still not going well at all and Dr gollow instructed them to insert GJ tube which bypassed the tummy and feeds into the jejunum, at this point she is still fed into her J tube and will continue to for the foreseeable future.

On top of all the Gi issues Miki also suffers with hypotonia, hyper mobile joints, ataxia, and central sleep apnea, and absent seizures, her original pead Dr naidoo who was fantastic recently referred her to the head of paediatrics at PMH Dr scurlok the first time we went to see her she told us that one way or another she would figure out DD and treat her the best way possible, Unless you have lived on the reflux roller coaster (as Roni he owner of IRD calls it) you couldn’t understand what it meant to s to have dr that was determined to figure her out, she told me that she didn’t believe all miki issues was caused by her Gi issues and that she thought there was a connective tissue disorder or a Neuro disorder going on.

She ordered an MRI and when we went in for the results she informed us that Miki has Chiari Malformation in her brain and it could likely be the cause for allot of her issues, we are now waiting on seeing the neurogist to discuss our options on how to treat this, as well as this she also has muscular issues but we are not surgeon her exact diagnosis as yet but hoping that this year will be the year to figure out our beautiful daughter and try and make her more comfy.

All the drs that have seen miki have worked the best they could with the information they had, she wasn’t misdiagnosed and it was no ones fault, It is just one of them things that was missed, but as I said no ones fault the main thing is we now know more and can help her more.

Mikis is still on J feeds and has been for a while now and is doing really well in the weight department she is 7.7kg which is great for even her pead is very impressed we are complelty ignoring charts for miki now as she has never been anywhere near it for very long time we are working on the thinking that as long we she has consistent overall month gain we are happy KWIM, her hight is also doing well she is not 74 cm so still a shorty for her age but she is tall for her weight, her head circumference is WAY above average im not sure on the numbers but I know the pead said that her head on the 90th percentile, so is a bit concerning

Miki is still continuing to gag and reflux on J feeds but thankfully she isn’t refluxing formula on a regula basis mainly just bile so that’s not affecting her growth over night we have her on drainage still which has helped amazingly she is getting about 150 to 200 ml of gastric output over 12 hours all that she would usually reflux and choke on, the drainage is completely green which indicates there is nothing happening in the tummy at all because of the paralysis hence why the G feeds was so dam useless and the g tubed meds was also useless by the time they moved though the stomach acid had made them near on inactive,

Oral feeding is still at nothing but for us its not really an area of importance due to the fact she cant digest it anyway and she is intolerant to everything KWIM, miki doesn’t have oral aversion she will pretend to clean her teeth etc (only with dry tooth brush loll ) but can not and will not drink or eat a thing (I wouldn’t either if she is lucky enough to get it into her mouth she then has to swallow with out gagging she then also has to struggle with aspirating then once in her gut she has to deal with reflux while the tummy takes its sweet *** time to start moving then as it hits the bowels they react and attack the food before it attacks them then her bowel produces mucus and starts to bleed then she has to try and poo it out so I don’t blame her for not eating )

She has also been diagnosed with epilepsy and is medication for that hopefully the seizures will completely stop but we should also see a change in her stress levels as they believe some of that is Neuro related and also the intense screaming periods she has they also believe there is seizure activity going on there as well,

With the MRI results in usual miki tradition there is nothing straight forward, yes she has the Chiari malformation and the cysts but they don’t believe this is the cause for all her Neuro issues the are addiment there is something going on in the mid section of the brain which is responsible for the gut they just not 100 percent sure what it is, but at least the pead and Neuro are tying to figure her out and no making me feel like a failure because my child is unwell

Miki has many issues to do with observe compulsive disorder and sensory integration disorder, and has major anxiety episodes allot of this they do believe is to do with the seizures and what ever Neuro condition is causing it, it will be up to the Neuro weather or not he diagnosis miki with ceramal palsy ataxia as she does have the traits of it but because of her other diagnosis we are not sure its basically onion based KWIM

Speech is where miki excels at she is great with her talking im so proud of her, she is also great at colouring she has heaps of words and uses them well

Physio, mikis gross motor skills are still delayed she is great at walking with her walker and also can walk by her self buts eh gets really tired and falls allot and her balance is major issue for her with the ataxia, she still has a extremely wide based gait and has some what of a limp one foot seems to step lighter and briefer KWIM you can here it when she walks, we are starting hydrotherapy soon which will be great for her we cant wait