Myasthenia Gravis

Watching countless doctors next to him, doing various tests was the scariest time of my life. 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 St Thomas' 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 nightmare.

We arrived at Evelina Children's Hospital, Department of Pediatric Neurology, St. Thomas' Hospital, they were all ready for Bradley, and doctors started tests right away. 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 the right medication 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. Tensilon is the trade name for edrophonium chloride. The Tensilon test is an injection of edrophonium chloride used to diagnosis myasthenia gravis. He told me 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, 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.

Bradley still had the other test, (intramuscular) EMG, the following week and was diagnosed positive for myasthenia gravis. The hallmark of myasthenia gravis is fatigability. 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 right medication to help him during the wait, he was given pyridostigmine and that drug 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 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.

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.
People with myasthenia gravis have a fault in the way nerve messages are passed from the nerves to the muscles. The muscles are not stimulated properly, so do not contract well and become easily tired and weak.
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.

A thymectomy is an operation to remove the thymus gland. It usually results in remission of myasthenia gravis with the help of medication 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 remember looking for something on the internet to give me hope. All I could find at the time was information that I already had. 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 lens 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!