Against All Odds – Beverly & Bonnie’s Story

Imagine finding out that your unborn daughter was having seizures in the womb and her prognosis was poor. Well this was the reality for Beverly Campbell.

But 26 years on her daughter Bonnie has gone on to defy every obstacle put in her way and truly lives up to her name

Bonnie was born full term on 1st November 1992, a healthy 6 pounds 5 and a half ounces. She my second child, but whereas my first pregnancy and labour were textbook, Bonnie’s was far from it.

A Very Different Pregnancy

I was very ill with the mumps in the first trimester and after that the pregnancy felt very different from first time round. Bonnie hardly moved and certainly never kicked, all I felt were these tiny little trembles that lasted thirty seconds or so throughout the day and night, most likely she was having seizures in the womb!

Bonnie was born after an extremely rapid labour, 10 minutes from start to finish, and needed resuscitation post birth. She started having seizures straight away, which at the time I referred to as ” funny turns”. These funny turns were dismissed by clinicians as Bonnie adapting to life after such a traumatic delivery.

The funny turns continued after we left hospital and got worse very quickly, they lasted longer and were more frequent and dramatic, she was having dozens day and night from a few days old.

“I felt utterly helpless as no one was taking me seriously”

And Bonnie never had a funny turn in front of anybody else. I was left feeling as though I was doing something wrong.

I was even asked by a doctor one day to do one of Bonnie’s “funny turns“. I struggled to twitch all over, blink rapidly and thrust saliva out of my mouth. And I certainly couldn’t change my skin colour!

Bonnie by a few weeks old however was an expert.

“Everything I did seemed to trigger an episode “

Learning How to Adapt to a Very Different Life

I very quickly learnt how to handle Bonnie in a way that minimised her funny turns.

Undressing her seemed to be a big trigger, so I got very adept at washing and changing her in stages so that she was never fully unclothed and I only touched or moved her when I had to as she would scream in terror. If I touched or moved her suddenly she would end up having a funny turn.

And, unlike her big brother who gained weight steadily and was interested from day one in his surroundings, Bonnie didn’t thrive. There was no weight gain,  no milestones being met, no smiles or response and she was very floppy even for a young baby.

Some years before I had children and was still quite young, I came across a man having a seizure in the street, I had never seen a seizure before, but it was obvious to me what it was and instinct cut in. I went to his assistance and administered first aid and stayed with him until help arrived. It was equally obvious that despite there being a lot of people around, no one else stopped to help, seeing someone having a seizure is scary, people don’t know what to do, seeing a baby, especially your own baby, have a seizure is even scarier.

Several  times in the early weeks, when by now, these funny turns were lasting five minutes or more, I thought Bonnie was going to succumb to one.

The start of investigative treatment

Events took a  turn at about 3 weeks when the midwife observed Bonnie having one of her funny turns.

“the next few weeks were an endless round of travelling around hospitals in the South of England having test after test with no one really telling us what was going on”

I have a lasting memory of going to a hospital in Wimbledon for Bonnie to have an EEG, and by the time we eventually got home , the blue marker pen and glue they had used to attach the sensors to her fragile little scalp had set and wouldn’t come off. It stayed for weeks and I was glad she was a winter baby as I was able to keep her in a hat to hide her blue scalp.

After extensive tests where completed, the combination of the MRI and CT scans and the EEG results gave a diagnosis and prognosis that was not good.

The worst prognosis

It was explained in a very insensitive way the extent of the damage to Bonnie’s brain and I was given a copy of a medical journal with the part saying ‘babies born with epilepsy were not likely to survive the month’ highlighted with a marker pen.

Bonnie was not offered any treatment, I was told that even if she did survive I would never be able to look after her and I would have to put her into full time residential care.

“But despite the odds stacked against her, she did survive her first month”

And whilst in hospital at 9 weeks with pneumonia, a lovely junior doctor observed one of Bonnie’s seizures and got her started on a course of Carbamazepine.

At the time I was told that Bonnie would have to stay on the medication until she was 2 and I wondered how they would then know if her seizures had stopped on their own. In my naivety, I thought the medication would stop her from having any.

For me the logic was, if you have a headache, you take a tablet and your headache goes. So I was shocked to the core when not only did Bonnie continue to have seizures but they got even worse.

We were told to bring her back if the seizures lasted 10 minutes, so we did. Only to be told bring her back when they last 15 minutes. By the time she was three months old, they were lasting 20 minutes and she was having maybe a dozen a day and we were still being sent away from hospital.

A Move for the Better

Thankfully, we moved to a different county when Bonnie was 6 months old and our journey took a different direction. We ended up in hospital the day we moved for emergency surgery, which turned out for the best as we had a much more positive experience.

A young consultant with children the same age as my own was very understanding and very keen to help, arranging for Bonnie to see a neurologist .

For the first time since Bonnie had been born , I had hope that we would finally have some answers and that Bonnie’s seizures could be controlled”

The neurologist was very blunt but he made some recommendations, starting off by adding Sodium Valproate to her Carbamazepine.

After a brief honeymoon period on the new medication her seizures returned with a vengeance. This was to become a pattern over the years which I’m sure this will resonate with many.

After this came a succession of various other first generation anti-convulsants in various combinations, all with awful side effects and still no seizure control.

Becoming an Expert on How to Best Treat Bonnie

Over time, the newer second generation anti-convulsants started to become available, some hailed as wonder drugs with much less side effects and fantastic results in seizure control, but still Bonnie continued to have intractable seizures.

Intractable Seizures
Intractable means not easily managed or
relieved.
This is also known as uncontrolled
or drug resistant epilepsy
Can also be referred to as Refractory Epilepsy
1 in 3 people with epilepsy will develop
Intractable (refractory) epilepsy
Patients with intractable epilepsy can have
partial or generalised seizures and in some
cases both partial and generalised seizures
Intractable epilepsy does not always remain
intractable. There are many different treatment
options, from medication, to the Ketogenic diet,
to VNS and even Brain Surgery that may provide
control to seizures. Even some changes to lifestyle
can help improve some people’s prognosis.

In time, with the use of the internet , I would research any new medication Bonnie was put on. I would read up on the latest research into epilepsy, often reading medical journals way over my head but getting a better understanding of the condition that controlled my daughters life.

I read about a very small trial of playing a particular piece of Motzart , k448, piano sonata for 2 in d major , as being effective in treating patients with intractable seizures, I played it so much that the music has implanted in my brain and I could hear it no matter what I was doing.

26 years on Life is Still a Challenge But We Remain Forever Hopeful  

So fast forward to today Bonnie has tried most drugs available in various combinations she has tried doses of Biotin and Pyrodoxine, Vitamins B1 and B6,  as they have been known to reduce seizure frequency and to treat the adverse affects of the anti-convulsants.

She has been on the Ketogenic Diet which is a high fat low carb controlled protein diet that is a treatment option for children whose seizures are not controlled by medication.

But still Bonnie continues to have seizures.

“Caring for someone with epilepsy is not easy, there is a lot of uncertainty and stress, and the major seizures are often unpredictable and with no obvious warning signs”

But advances over Bonnie’s life time have helped. Medications have improved to give fewer side-effects and better seizure management, VNS surgery has become more widely available as a means of a complimentary epilepsy management and more recently for those of us who are not keen on an invasive VNS surgery, there is the tVNS (Transcutaneous VNS).

tVNS
(Transcutaneous Vagal Nerve Stimulation)
Transcutaneous VNS is a non-surgical version
of the traditional VNS treatment.
Traditional VNS treatment consists of a pacemaker
type box implanted via surgery in the chest and attached
to a wire that is coiled around the vagas nerve in the neck.
This provides an electrical stimulus every few seconds to
help control electrical activity in the brain and reduce or
control seizure activity for the user.
An extra charge can be administered during a seizure by
swiping a magnet over the box in the chest to help stop or
reduce the length of a seizure.
tVNS takes advantage of the fact that the small area of skin
on the outer ear is connected to  the vagas nerve.
Gentle stimulation of the skin has been shown to produce
similar fMRI brain activity  changes as the traditional
surgical VNS helping reduce and in some cases control
seizure activity.
For more information on tVNS therapy click here
https://cerbomed.com/en/tvns-technologies-gmbh/

Research into epilepsy is ongoing and new medications and treatments come onto the market periodically so I feel that we still have options.

However ,every day life is still a challenge for Bonnie but equally it is for me as her carer. I have to remain alert and vigilant at all times of the day and night, and this is a strain both mentally and physically.

The frequency and intensity of seizures that Bonnie experiences put’s her at the highest risk of SUDEP (sudden unexpected death in epilepsy) about five times higher than the usual SUDEP risk.

This is not something I like to acknowledge but it is what it is and I’ve had to accept it and remain as vigilant as I can. I have adapted and learnt over the years the best way’s to treat Bonnie and how to try and minimise the risks to her and keep her as safe as possible.

Bonnie still has many and varied seizures throughout the day and night the seizure activity is always there with peaks and troughs of Intensity.

Night’s pose the biggest risk to Bonnie

Night time monitoring presents its own problems, it is when Bonnie is most at risk as I need to sleep too, however, her seizures don’t switch off just because she is asleep, so therefore I never fully switch off either.

In the past I have relied upon using A/V monitors to keep an eye on her and alerting me to the noises that signify the onset of a seizure. I looked at dedicated seizure monitoring systems but I didn’t feel that any at the time was suitable for Bonnie.

Over the years Bonnie seizures have changed and now not all her seizures that require rescue medications are precipitated by any sound.

How PulseGuard Changed our Lives

Whilst trawling the Internet one evening a few years ago I read an article about a new device that was soon to be launched that monitored the persons heart rate and alerted carers to seizure activity when the heart rate changed above or below individual thresholds.

I was instantly interested. Bonnie is very good at having very subtle, sub clinical, seizures where she is very still but her heart races. I would say to her carers “ please go by her heart rate and not her Sats”. I  knew that I wanted this device.

I have been using the PulseGuard for about 4 years now with Bonnie, it took a little while to get the optimal values for the alarm settings right for Bonnie as she is so complex. I did this by monitoring Bonnie’s heart rate over the initial few weeks and observing the changes in her heart rate as activity happened, whether it was sub clinical activity or any other type of seizure she experiences right up to a full blown biggie.

“I know Bonnie so well I can tell how bad the activity in her brain is just by glancing at her”

So with a combination of what the monitor read,  what I observed myself and a few Tonic Clonic seizures, the monitor was set so that it would alert me when it was necessary. It has never let me down and I trust the
PulseGuard™ 100%. It has alerted me to seizures I would not have been aware of, particularly overnight that needed rescue medication. It has saved Bonnie’s life, of that I’m convinced!

The PulseGuard confirmed everything I knew and thought about Bonnie‘s epilepsy which in a way has empowered me, I have been accused in the past of being paranoid about Bonnie‘s epilepsy and seeing seizures that weren’t there, this is not so, I just wanted to be believed and the
PulseGuard has given me the evidence as well as the reassurance.

My nights are easier, even though I am awoken every night by her monitor going off to a lesser or greater extent, I can sleep soundly knowing that when the alarm goes off, it’s gone off for a reason.  

“My sleep is deeper because I can relax with the
PulseGuard doing my night duty”

I’ve learnt that it really is quality that counts with sleep rather than quantity.

The PulseGuard has also enabled me to better predict Bonnie’s major seizures by observing her heart rate throughout the day and night so I can titrate her medication more effectively.

We are eagerly awaiting the arrival of our PulseGuard Mark II. We are perfectly happy with our original PulseGuard, but new technology is being invented all the time and we live in a day and age that such technology is reasonably cheap, portable and accessible. A far cry from 26 years ago when Bonnie was born!

And So Our Journey Continues

Bonnie and I have been on a long journey together and it has taken me many years to understand the complexity of her seizures and to work effectively to minimise the impact they have on her life.

I have to be both resourceful and adaptable, I’ve had to gain expert knowledge quickly and have learnt how to adapt to Bonnie’s seizures as they have changed over the years, learning the proper first aid for the seizure type as well as keeping abreast of all the latest treatments, developments and technological advances to help me better understand, detect and treat Bonnie’s epilepsy.

There has been no real change for Bonnie over the years with regards to her epilepsy. What has changed though is my understanding of her condition and the affect it has on Bonnie. And as her mother and carer I know the most effective way to treat her symptoms to minimise the chances of her succumbing to a seizure.

If you have been affected by anything mentioned in Beverly and Bonnie’s story and would like advice or support, or would like your story featured to help raise awareness for others. Please contact Kate on 01622 321111 ext 1011 or kate.farmer@pulseguard.org

Author Info

Kate Farmer

Senior Clinical Advisor for PulseGuard International Ltd. I have 15 years experience working frontline A&E in the UK NHS Ambulance Service. I have gone on to train and research extensively around epilepsy, to provide the most up to date information, clinical advice and support to PulseGuard customers and followers as well as living first hand with the personal effects of Dravet Syndrome within my family

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