Bradley's Myasthenia Gravis

I took him home and made an appointment to see the doctor the next morning.

That wasn't the first time I noticed his eyes, he had been like that all week, but that day was just worse.

I thought it was just something to do with his hay fever or maybe he was tired.

But that day I knew it was something more serious.

The doctor took a look at him, after some time he was just looking at him and thinking, he told me he had to make a phone call and asked me to wait outside.

I knew there was something really wrong!

After about 15 minutes he came out with a letter and asked me to take Bradley to the Local Hospital and give Bradley's name and the letter to the doctor.

His face was changing so fast, he could no longer smile and he was worn-out and terrified of more needles. I felt hopeless as I couldn't do anything to help him.

They told me that there was a chance he had a condition I had never heard of: Myasthenia Gravis, but it could also be a brain tumor.

To be sure there were some tests he had to do, so they were transferring him to Evelina Children's Hospital in London the following morning.

Bradley no longer looked like a normal child, his neck was weak, his mouth was droopy his eyes were getting worse.

He didn't have the strength in his lips to suck a drink through a straw without dribbling down his shirt.

He had a nurse watching him eat just in case he choked on his food as swallowing was difficult for him.

I just wanted to take him away from all that, I wanted my life to be normal again, I wanted to go home and have a good nights sleep.

I just wanted to wake up from this bad dream.

Students from the university were approaching to ask consent to take a look at Bradley and spot the symptoms.I was aware it was a rare disease in children and they had the opportunity to study the symptoms with Bradley. After the tests the doctor came to see me, he told me he believed Bradley was suffering from Myasthenia Gravis and there was a test they needed to do.But that test was done in a different hospital and they couldn't do it until the following week. The problem was that Bradley's symptoms were getting worse too fast and if they couldn't give him pyridostigmine the medication for Myasthenia Gravis there was a chance of a myasthenic crisis; a remote chance but they didn't want to take that risk. A myasthenic crisis occurs when the muscles that control breathing weaken to the point where breathing becomes almost impossible in such cases - if the patient is lucky enough to be in hospital - a ventilator machine has to be used. Therefore they recommended doing a placebo/Tensilon test.But that test was done in a different hospital and they couldn't do it until the following week. The problem was that Bradley's symptoms were getting worse too fast and if they couldn't give him pyridostigmine the medication for Myasthenia Gravis there was a chance of a myasthenic crisis; a remote chance but they didn't want to take that risk.A myasthenic crisis occurs when the muscles that control breathing weaken to the point where breathing becomes almost impossible.In such cases - if the patient is lucky enough to be in hospital - a ventilator machine has to be used.
Therefore they recommended doing a placebo Tensilon test.

I was told they were getting a room ready with a video camera so they could film the all test just to be 100% positive of the result.
In the room there were going to be several doctors.
They would give Bradley three injections; only one syringe would be the Tensilon and only the doctor administering the injections would know which one it was.
All the other doctors and I would have to check if Bradley's symptoms - specifically his droopy eyes - got better after each injection was given.
Bradley cried all the way through the procedure, sometimes his words still echo in my ears, "Mummy help me, Mummy stop them, don't let them hurt me any more, oh God help me please!" Of course I was crying too, I had to hold him tight while they did all the tests.
But after injection number three I was so happy: all of a sudden I could see my son's eyes like they used to be. The doctors and I all shouted the same words at the same time: "it's number three!"
While I realized that he had a serious long-term condition, I was so relieved that he didn't have a brain tumor.
At the time I didn't really know what Myasthenia Gravis was anyway.

Muscles become progressively weaker during periods of activity and improve after periods of rest.
Muscles that control eye and eyelid movement, facial expression, chewing, talking, and swallowing are especially susceptible.
The muscles that control breathing and neck and limb movements can also be affected.

But at least he was taking the Pyridostigmine to help him during the wait, Pyridostigmine is a drug that can improve muscle function by slowing the natural enzyme cholinesterase that degrades acetylcholine in the motor end plate; the neurotransmitter is therefore around longer to stimulate its receptor.

The problem is Pyridostigmine is a short-lived drug with a half-life of about 4 hours.

Dr Heinz Jungbluth, a Consultant and Senior Lecturer in Pediatric Neurology, was Bradley's doctor and he was the greatest I have ever seen.

He was determined to make Bradley better and he came to believe that there was a possibility that there was a tumor in Bradley's thymus gland: a possible cause of the myasthenia.
After the initial MRI scan was done one of the specialists could see what he thought was a tumor.
A CD containing the results was sent to some other experts at another hospital to see if they could verify this conclusion but the disk was found to be damaged and another scan had to be carried out.
After the second scan there was still no consensus amongst the specialists one way or the other but Bradley wasn't doing too well on his Pyridostigmine medication: it was wearing off too fast.
Dr Jungbluth talked to me and said that he wanted to do a thymectomy as he believed it would help Bradley to have a better quality of life.
Even if there was no tumor Bradley would have to have the operation sooner or later as malfunction of the thymus gland is one of the major causes of this condition.
It would be better for him to have the operation while he was young, strong and better able to make a speedy recovery.

By Now I'd Had Time To Read All The Information I Was Given By The Doctors
And Nurses About Myasthenia Gravis.

I learned that Myasthenia Gravis was a condition where muscles become easily tired and weak.
It is due to a problem with how the nerves stimulate the muscles to contract.
The muscles around the eyes are commonly affected first.
This causes drooping of the eyelid and double vision.
The fault is due to a problem with the immune system.

Myasthenia gravis is an 'autoimmune disease'.

This means that the immune system (which normally protects the body from infections) mistakenly attacks itself.
In most people with myasthenia gravis, antibodies are made which block, alter or destroy the acetylcholine receptors on muscles.
The acetylcholine cannot then attach to the receptor and so the muscle is less able to contract.
The reason why the body's immune system starts to make abnormal antibodies against muscle receptors is not known.
Abnormal antibodies are made in various parts of the immune system which include the bone marrow; blood; lymph glands and the thymus gland.
However, the thymus gland is thought to be a main source of the abnormal antibodies.
Myasthenia gravis can develop at any age but most commonly affects women under 40 and men over 60.
It affects approximately 1 in 20,000 people.
Women are three times more likely than men to have the condition.
It is not inherited although it can occur in more than one member of the same family.

Dealing with Bradley's myasthenia gravis only six weeks after having a baby made it all the more difficult.
I wanted to be with Bradley but I also felt the need to be with my new baby.



Evelina Children's Hospital was incredible: they understood the situation and let me have the baby with me all day long.
I remember one night; my husband took the baby home as I wanted to spend the night with Bradley.
About 3 hours later I was crying on the phone, I felt so empty I needed to be with the baby as much as I needed to stay with Bradley.
It was very late but my husband drove all the way back to Evelina Hospital just so that I could spend a few moments with our new baby.
It was quiet at Evelina Hospital at that time of night so I asked the male nurse in charge if was OK to let my husband and the baby come in just for a little while.
As he looked at me I had tears in my eyes and he said, "It's not easy for you having such a small baby and dealing with Bradley's myasthenia gravis too.
I can do better than that.
Room 33 has a cot and it's empty.
Why don't you spend the night there with the baby and your husband can stay with Bradley.

That way you can check on Bradley anytime you want."
I can't even describe what that meant to me that night.
For some reason I really needed to be with both of my children that night.
I'll never forget that nurse: he knew I appreciated what he'd said but he didn't realise how much it meant to me.
And even better the next day, since the cot next to Bradley's bed was empty he told me to put the baby in it just so I could sleep between both of my children.
I'll never forget that!
The doctors and nurses at that hospital truly care about the patients but they also care about the families who are suffering.
But Bradley was doing well on his Pyridostigmine medication and after a few days they told us we could take him home until the operation was booked.
It felt so good to get back home and have the family together again.
It felt like we had been away from home for a long time.
I was a bit scared about having Bradley at home: it was a big responsibility making sure he had his Pyridostigmine every three hours, looking out for the symptoms constantly in case the Pyridostigmine wasn't effective for all the time between doses. Sometimes, if he had too much excitement, the symptoms would come back before he was due to have more medication so I had to make sure he would go to bed to sleep, not even to watch TV in bed.
Sleep was the only way to fight the symptoms.
He was also allowed to go back to school.
At the time Bradley was going to Temple Hill School in Dartford.
Going back to school meant more worries as I feared his symptoms may not be spotted.
Bradley's nurse Debbie Clark went to his school to educate his teachers and all the school staff about this illness as they all had to watch out for the symptoms.
She was a good nurse and gave us lots of support and information.
After that I had to drive to school to give Bradley his Pyridostigmine on time as he needed it every three hours and the school policy didn't authorise the staff to administer it until they fully understood his illness.
It worked out quite well in the end: the school was also great at dealing with this.
They keep their eyes on Bradley's eyes and if they noticed any droopiness they would put him in a room to rest or sometimes call me to pick him up.
This meant Bradley wasn't allowed to do PE or even go outside at playtime; he couldn't do anything physical at all.
It was very frustrating for him as he couldn't understand the reason why he wasn't allowed to go out and play with the other children.
He had to stay in the school library while the other children played happily outside.
But his teacher, Miss Donovan was so good, she asked a different classmate each day to stay with Bradley, just so he wouldn't feel so lonely.
That was nice of her to give him and us so much support.
So until the Thymectomy Bradley still had a bit of a life, going to school meant so much to him as he loves school.

A thymectomy is an operation to remove the thymus gland.
It usually results in remission of myasthenia gravis with the help of Pyridostigmine including steroids.
However, this remission may not be permanent.
A thymectomy is mainly carried out in an adult.
This is because the thymus loses most of its functional capacity after adolescence, but does retain a small portion of its function during adulthood.
This is shown in the decreasing size of the thymus with increasing age after adolescence.
The thymus is considered to be essentially an organ of the immune system child but not in the adult.
From the arguments put forward, the hypothesis is put that the role of the thymus is to store and propagate the stem cells required for the organ growth which takes place subsequent to their initial development.
The thymus gland is located in the upper chest under the breastbone.
It is composed of many small lobes and is shaped somewhat like a butterfly wing over the windpipe.
Hormones produced by the thymus gland are thought to affect the immune system and the neuromuscular transmission although the exact role in Myasthenia Gravis is not completely understood.

I looked for hope, I wanted to find someone who actually dealt with Myasthenia Gravis on a day to day basis and was fine.
We knew that even if the operation was successful we'd be living with this for the rest of Bradley's life.
I found plenty of medical essays but I couldn't find that hope on the internet at the time, so I just had to believe and trust the doctors.
I made this article just so someone in the same position as me back then can believe and feel hope living with this rare condition.

Bradley is living a normal life and his remission may not be permanent, but so far he is just like any other child. No medication and no restricted play time, the only thing that reminds us of all this nightmare is the scar in his chest. I hope this story can help you!