| Wellness |
JULY , 2023 | THE HEALTH
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That rings true to O ’ Connor . Initially , doctors thought she might have a different rare neuromuscular disorder similar to MG . Another issue that made her diagnosis tricky is that she was so young - only 10 years old - when her symptoms started . In women , symptoms of MG more typically start in their 20s ; in men , symptoms are likelier to start in their 50s , according to a 2023 report in the Cleveland Clinic Journal of Medicine .
The first symptom , says Dr Sun , is typically an eye droop or other visual problem like double vision . Often , symptoms then progress to include difficulty swallowing , weakness in the arms , legs and neck and extreme fatigue . The condition can also interfere with a person ’ s ability to breathe . “ We call that a ‘ myasthenia crisis ,’ where patients have to be intubated ,” says Dr Sun .
Another characteristic of the condition is that symptoms tend to get worse as the day goes on . People with MG “ may feel better when they first wake up , but the more they use their muscles , the more fatigued they get ,” says Dr Sun .
In order to function optimally , O ’ Connor naps when her schedule allows and takes breaks throughout the day . “ And if I don ’ t get nine hours of sleep a night , my eyelids droop ,” she says ( as shown in photo ). “ My life is guided by my health ; it ’ s all about listening to my body and constantly adapting to what it needs because those needs are always changing .” 2 . Myasthenia gravis is an autoantibody disease , which means the immune system turns on itself When the immune system functions normally , it attacks foreign invaders , keeping us healthy . With MG and other autoantibody diseases , “ something goes wrong with the surveillance system — that is , the immune system — and the body begins to develop autoantibodies attacking itself ,” says Dr Sun .
One sign that O ’ Connor had MG was that her blood test results revealed high levels of autoantibodies attacking a protein known as acetylcholine receptor ( AChR ), located on the muscle membrane of a particular site called the neuromuscular junction . “ AChR is a critical protein that normally binds with a chemical messenger called acetylcholine ( ACh ), triggering muscle contraction ,” says Dr Sun .
“ When the receptors are bound by autoantibodies , not enough ACh binds to trigger normal muscle contractions . In MG , this disrupted communication between the nerves and muscle result in muscle weakness and other tell-tale symptoms .”
O ’ Connor filling her pill organiser with the additional medications she takes to treat her myasthenia gravis .
With MG , however , the autoantibodies interfere with that process , disrupting communication between the nerves and the muscle , resulting in muscle weakness and other tell-tale symptoms . 3 . There is no cure for myasthenia gravis , but surgery and medication can make a difference After her diagnosis , O ’ Connor was hospitalised during spring break of her eighth-grade year . She underwent a procedure known as plasmapheresis , along with intravenous immunoglobulin therapy , a process designed to remove the offending antibodies from her bloodstream and replace them with “ good ” immunoglobulin antibodies . The procedure is “ very invasive and time-consuming , and tends to be used when patients are at risk for an MG crisis ,” says Dr Sun .
In O ’ Connor ’ s case , the procedure was done to help prepare her for surgery to remove her thymus gland , which is thought to generate the offending rogue immune cells . “ Normally , the thymus gland tends to be bigger when we ’ re children , but shrinks once we get older ,” says Dr Sun .
In some 50 to 70 per cent of patients with MG , however , the gland can become enlarged ( called thymic hyperplasia ) and another 10 per cent develop tumours on the thymus gland . “ Surgery to remove the thymus can improve symptoms over the long term ,” says Dr Sun .
Indeed , a 2019 study in The Lancet Neurology found that people with MG who had their thymus gland removed showed significant improvement in their symptoms after three years . “ The thymectomy
definitely helped me , but it wasn ’ t enough ,” says O ’ Connor . “ I was still experiencing symptoms .”
In addition to the muscle stimulants she was already taking , her physician prescribed steroids to tamp down her overactive immune system . “ I have a love-hate relationship with steroids ,” O ’ Connor says . “ They have significantly improved my symptoms , but the side effects are incredibly debilitating .”
Says Dr Sun : “ We ’ ve used these immunosuppressive drugs [ i . e ., steroids ] for years , but they can cause adverse effects on blood pressure , cardiovascular health and bone marrow density .” For O ’ Connor , taking the medication twice a week versus every day or every other day and tapering the dose has made a positive difference . 4 . New potential treatments are on the horizon One problem with current immunosuppressive medications is that they suppress the entire immune system , making people with autoimmune disorders vulnerable to infection , as well as other problems . But a new treatment being investigated by researchers at Janssen is believed to act more selectively , blocking a protein called FcRn which , in turn , potentially helps lower the level of dangerous autoantibodies so muscles can function more normally .
“ The investigational therapy is almost like a portable plasmapheresis machine in that it is believed to remove the pathogenic autoantibodies from the bloodstream , only it does it more quickly and easily ,” says Dr Sun .
“ It ’ s not a cure . But , so far , trials are showing that it gets to the cause of the problem in a very targeted and effective way , and it has demonstrated a safety profile that supports further development of the treatment .” 5 . Myasthenia gravis is not yet a household word , but it ’ s on more people ’ s radar With more effective treatments and promising new therapies in the pipeline , the future for people with MG is looking brighter every day .
As for O ’ Connor , with her most troubling symptoms under control , she is feeling happy and hopeful . “ I ’ m getting married in July of 2024 ,” she says . “ My mother likes to say it will be more than just a wedding . It ’ s a celebration of how far I ’ ve come , and it feels wonderful .” – The Health
This article appeared recently on the website of Johnson & Johnson
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