November 2017

As mentioned, Alina had always been a bright girl, very sociable and with an excellent sense of humour. She was loved dearly and she loved life.

In November 2017, the week following her 18 month MMR vaccine, things changed dramatically. She became a significantly different child. Unsociable, she stopped eating solids or feeding herself, became unusually irritable, poorly and with horrendous tonsillitis, wheeze and the distinct smell on her breath became worse. Serious consideration should be given to Cross Immunity (The MMR immune response coupled with a pre-existing condition of a “nested” Chronic Virus)

There can be cross-reactivity between the immune system and the antigens of two different pathogens, or between one pathogen and proteins on non-pathogens. Simplified; if you catch one virus, it can alert the immune system to over react and develop the immune response to another previous virus simultaneously

Cross immunity also likely happened for the second time in Hospital when she had a horrific seizure on the 12^th January when Alina had contracted an adenovirus that was spreading across the ward. She had significant brain damage as a result and from there on in a state of constant heavy seizures until the trial of an autoimmune drug.  

6 days following the MMR, the family was invited to a party and Alina’s friends and their parents also noticed how quiet she was, as typically she would mingle and play, especially enjoying and reacting to adult attention. Being a party with a variety of foods on offer, she finally ate some solid food that week: a piece of cake.

That evening, shortly after her formula bottle feed, Alina started drooling heavily, so much that mum had to change her top. She then had a seizure in her throat, which spread to her lip on her left side (the same side she had a dancing eye). She was rushed to the local GP’s where the doctor believed it may be a seizure and called the paramedics. The paramedics believed it was something trapped in her throat, as she was fully conscious, since it was twitching, more like an irritation.

She was taken to Canterbury Hospital, where it was noticed she also had croup and was given steroids. The seizure continued in her lip and throat. Midozolam and adrenaline stopped the prolonged seizure immediately.

It is worth noting the heavy drooling, seizures in the throat and mouth, croup, and recovery with midazolam, as being the predominant features of this initial seizure episode. Ali had Hand, Foot and Mouth Disease (HFMD) aged 11 months.

All of this clearly pointed in the direction of a chronic viral illness, information regarding HFMD and Herpangina and Laryngeal Croup, below.

Over the next few days both parents were very cautious, in disbelief that their daughter had had a seizure, since there was no history for either side of the family (other than a maternal uncle who electrocuted himself in his teens). Alina became very lethargic and irritable. Within 3 days (on the following Monday) she had another seizure and croup occurred again. Again, she was taken to Canterbury Hospital, then Royal Prince Alfred Hospital, then on to Sydney Children’s Hospital where Dr Hugo Sampaio was assigned as her Neurologist Paediatric Specialist. Her brain MRI was shown to be clear.

The full and detailed history of Alina’s illnesses was given, as seen on this list of GP visits (Excel, 13kb), without focus on any single aspect as the parents did not know the relevance but they were very thorough: repeated virus; HFMD (at 11 months); vomiting of blood; predominant and frequent night cough; head sweats, rashes and wheeze that occurred simultaneously with eye deviation. Post vaccine MMR illness. Being very clear that this was a repeating and chronic virus, confirmed from a large number of visits to the GPs.

Dr. Sampaio prescribed Keppra and gave his contact information, with a follow up appointment 3 months later. Many questions were asked by the neurology department: unusual birthmarks; contact with anyone returning from overseas; does she play in the park or been to the zoo even? The parents gave every answer fully.

For the next month, Alina again had a persistent metallic smell on her breath, strong left eye deviation, sweaty head and wheeze. The night coughing became worse. By coincidence, her normal formula (Karicare, given prior to bed time, but incorrectly documented in the hospital notes) was unavailable due to daigou, so another brand was used. She was otherwise mostly eating the same as her parents and they were suspicious that the new formula milk could be associated with the increased wheezing and coughing.

The parents contacted Dr. Sampaio twice in this period. The first email was as follows:

“Often we were asked if she [Alina] had any birthmarks, and up until Sunday we didn’t think she had. On inspection we found this on her back, left mid trunk, about the size and shape of a Dairy-Lea/ Laughing Cow processed cheese triangle. Very feint and white in colour.

After asking mum – Indian descent, she too has a large dark birthmark on her neck, that in fact until asked I just thought was a collar tan line on the back of her neck. She says its sensitive to bright sunlight and often covers it when outside.

Secondly, Alina is not eating solids in any form, she’s excited to see food, plays with it and puts on spoon, into mouth, but seems unable to swallow and so spits it out. This started about 3-6 days before her first seizure on the 11th Nov. Lastly she has terrible bad breath”

3 weeks later, after a very difficult night, Alina showed familiar symptoms experienced many times before, but this time all together rather than separately. They were also now accompanied by hives and the concerned parents contacted Dr. Sampaio with the following images and email.

“Further to the observations, to add, Alina has developed small spots (hives?) around mouth & chin, a few on chest, backs of knees. Images attached.
She is slightly wheezy also and when asleep she twitches quite frequently, like small body hiccups. Much more tired than before the seizures
We saw our GP on Saturday, she observed that Alina has sore/bleeding spots on tonsils. She said she will be in contact regarding a discharge report:
Dr.Zenifa Peesa, Victoria Tower Medical, Burwood. 8322 9099
We have reverted back to pureed food/soup which she can swallow.
Thank you again for your correspondence and your concern
best regards”

These are the very unique symptoms or red flags for an enterovirus, specifically EV71, especially when combined with the history to include HFMD. They are specifically detailed in medical information and papers such as below.

Do also note the effect of loud noises. A feature described frequently by the parents to the Neurology Doctors. Regardless of how asleep she was, Alina would display “sudden jerks” when she heard certain noises, in particular car or motorbike engines (and also plastic bags). The parents had noticed them from birth. Noise sensitivity was noticed by her nursery care worker also, 5 days before her first seizure.

Combined with Alina’s clearly deviated eye, you could even summarise this was “grade 1 and 2” EV71, as defined in the 1998 Taiwan epidemic (see Clinical features of EV71 above).

It was also in line with development and summary of all the individual components of nearly 40 visits to the GPs, as seen in Alina’s medical history i.e. it was a chronic and progressive variation of EV71, and had been developing since a week after birth. This was a very clear clinical presentation in every manner except confirmation by testing.

Alina had a seizure 2 days later and was taken to Sydney Children’s Hospital, now with continuous focal EPC seizures and the MRI showed brainstem inflammation.