Those of you who know me will be aware that for many years I have focused some of my charitable fundraising, and giving, on the Multiple Sclerosis Society of Canada.
I didn’t have any particular connection to MS when I first got involved, and I frankly didn’t know a lot about either the disease itself, or the MS Society. I did some homework at the time, and then over the years I learned a lot more.
Through anecdotes and short pieces, I have provided some of you with bits and pieces of information about MS, but I thought now might be a good time to do a more complete piece on the disease: its causes, impacts, treatments, and – one day – cure.
A word of caution, though. I’m not an expert in the field. This is a piece of reporting, i.e. things I’ve learned, rather than things I know well. I am counting on the people I know who are real experts to leap to their feet in outrage if they see anything wrong, so I can correct it. After all, this article is not supposed to be fiction (as much as I might like it to be).
What is MS?
Multiple sclerosis is so named because the disease causes multiple scars (sclerosis=hardened tissue) on the brain and nerves, as seen in autopsies of MS victims. Other “sclerosis” diseases include Lou Gehrig’s disease (ALS – amyotrophic lateral sclerosis), arteriosclerosis (also called hardening of the arteries), and many others. The name is descriptive of a category of symptoms – i.e. the hardened, scarred tissue – not a group of related illnesses. MS and arteriosclerosis are not really similar; ALS, while sharing some symptoms with MS, is not an autoimmune disease; and so on.
First diagnosed about 150 years ago by French physician Jean-Martin Charcot, MS is an autoimmune disease in which the body’s defences attack the protective myelin sheath around nerves, particularly in the spinal cord and brain. Nerves with a damaged sheath, or one replaced by scar tissue, can’t communicate properly, resulting in deterioration of neurological functions.
The cause of MS is not known, but there are a number of important clues.
MS incidence correlates well with distance from the equator. If you were born further from the equator, there is a much higher likelihood that you will have MS. For my friends in Thailand, for example, they don’t need to worry. The likelihood of a Thai having MS is almost zero (1 in 133,333 people), and Indonesia is even less. None of the countries of South East Asia even have a charity devoted to MS. They don’t have enough need for it.
Canada, on the other hand, has the highest rate of MS in the world, 1 in every 344 people. Denmark is second, at 1 in 410. Sweden is third, at 1 in 529. Most of Europe is worse than 1 in 1,000, while most of South Asia and Africa are better than 1 in 10,000. While some of the differences could be the result of local diagnostic practices, and awareness of the disease, it has long been known that MS is a disease of the temperate (northern and southern) climates, rather than tropical climates.
This correlation with latitude is even true within countries. Incidence in the northern USA is twice that of the southern USA, and incidence in southern Australia is twice that of northern Australia.
Although a correlation to Northern European ancestry was once thought likely, the jury appears to be still out on that. On the one hand, some northern peoples, like the Inuit, have very low rates of MS, suggesting that genetics rather than birth location is the tie to MS. On the other hand, some non-European groups, like Palestinians, have higher rates of MS, making the genetic link less clear.
Most telling, though, is that children born in places far from the equator have a much lower incidence of MS if they have moved to a more tropical region prior to the age of fifteen. Perhaps related to that, there appears to be a clear relationship between birth month and MS, with children born prior to the winter at greater risk than those born in the spring. Both of these suggest the possibility of an environmental cause for MS.
That is not to say genetics play no part in it. A number of studies have shown that some patients may be genetically predisposed to MS. However, much of this work – at least at this stage – appears to show only that some people are more susceptible to autoimmune diseases generally, not just MS. One exception is studies showing that identical twins have a 30% chance of having MS if their twin has it, whereas other siblings, including fraternal twins, have only a 2% chance. The connection, if any, between genetics and MS continues to be a matter of some debate in the MS research community.
Another factor in understanding the cause may be the fact that MS strikes women at a rate twice that of men. A further subtlety is that, in women, hormonal changes appear to affect the symptoms of the disease. For example, during pregnancy, women with MS often have a remission of symptoms, then after the birth there is a relapse.
There are basically two types of MS: relapsing and progressive. In the first and most common type, relapsing (called RRMS), there are episodes of symptoms, interspersed with periods symptom-free. In the second type, progressive (called PPMS), the disease slowly gets worse over the person’s life. About half of those with the first type of MS end up with the second type within the first ten years after diagnosis. While both are considered MS, until the cause or causes of MS are known it cannot be said with certainty that they are two versions of the same disease. The fact that there are two things we call MS may, through the study of their differences, end up helping researchers to find the cause of MS.
Research into the cause of MS has several directions: genetics (whether as a cause or a susceptibility), environment (for example, vitamin deficiencies), viral (as a direct cause, or as a trigger for genetic or other factors), etc. Much of the work seeks to understand the interactions between factors, and there are many in the scientific community that believe those interactions will be the key to identifying a real “cause”.
However, like much of medical science, research into the cause of MS is characterized by a disheartening amount of “ruling things out”, which while undoubtedly useful is not as exciting as finding “promising leads”.
Impact on Its Victims
Around the world, there are currently about 2.5 million MS victims, with about 100,000 of those in Canada. This is rising fairly quickly, just under 200,000 diagnoses per year, which is about seven times the rate at which the world’s population is growing. There is a good likelihood that the high rate of increase in the known incidence of MS (8%) is due in whole or in part to improved knowledge and diagnostic techniques. That having been said, without knowing the cause of MS, scientists cannot rule out the possibility that environmental or other factors are causing an increase in the absolute incidence of the disease.
People are fond of calling every disease a “deadly” disease, but that doesn’t really describe MS. Although MS victims have a shortened lifespan – perhaps ten years shorter on average – MS rarely kills people.
So, I don’t call MS deadly. You could call it a “debilitating” disease, I suppose, but I prefer to call it a “cruel” disease. MS doesn’t kill you, but it can do a pretty effective job of attacking your ability to live a happy and productive life.
Like most illnesses, the progression of MS is different for every victim. That having been said, because the basic operation of the disease – damaging the myelin sheath – is consistent, there are a number of impacts you will often see.
For example, it is common that MS patients will have pain and burning sensations, as well as muscle spasms. The result can be issues with mobility and balance, including vertigo, dizziness, etc. Many MS victims have problems with their vision, and can exhibit slurred speech, problems swallowing, and other neuromuscular deficits. Many of these symptoms are common in other neurological disorders.
Specific to MS, particularly in its later stages, is the possibility of problems with cognition and memory.
The symptoms have been seen publicly in the impacts on well-known people who had MS.
Annette Funicello, for example, visited the dreams of many a young boy in the 50s and 60s. One of the original Mouseketeers on Walt Disney (the pretty one), she went on to star in a series of “beach party” movies with Frankie Avalon in the early 60s.
In 1987, at the age of 44, she first started showing dizziness and other symptoms. In 1992, faced with rumours that she had become an alcoholic (due to the slurred speech and similar motor disruptions caused by MS), she revealed for the first time that she had been diagnosed with MS. With a couple of exceptions, she essentially ceased to involve herself in acting, or fan events, or any other public activities and appearances. Her symptoms got progressively worse. By 2004, she couldn’t walk, and by 2009 she couldn’t speak. Shortly after that, she was placed under 24-hour care, and died in 2013 at the age of 70. A CTV special on her life, including her battle with MS, was filmed a year before her death. It makes instructive watching.
Another screen star struck by MS was Teri Garr. Most well-known for her roles in Young Frankenstein and Tootsie, Garr had her first symptoms of MS when she was 25. Unlike Funicello, Garr experienced a slower progression of the disease, so she was able to hide it for twenty years. After announcing her diagnosis in 2002, Garr became (and continues to be) a spokesperson for the National MS Society in the US. She continued to act in movies and TV until 2007. At that point, due to MS and other health problems, she ceased to be active in the entertainment industry. She still appears occasionally to speak in support of the fight against MS.
Richard Pryor, one of the most irreverent (at the time, the correct term might have been scandalous) comedians of the 70s, and certainly one of my favourites, was limited by MS for at least the last fifteen years of his life, using a scooter to get around after he lost most of his ability to walk. He also ceased to perform publicly, although he denied being unable to speak due to the disease. He died in 2005, at age 65, of causes likely completely unrelated to MS.
Pryor will forever have a place in MS history, though, for his wry comment that MS actually stands for “More Shit”.
I could go on. Each of these public figures, and the many others we know about, experienced MS in a different way, but for all of them their lives were permanently and negatively altered by the disease.
I have often said that if you choose twenty people you know at random, at least one of them will be been affected, directly or indirectly, by MS: either themselves, or through a relative or friend. I regularly hear stories from friends and acquaintances touched by the disease in some way. In each case, the details are different. In every case, the impact is significant.
MS is not entirely untreatable. Although the overall cause of MS is not known, some of the proximate causes of the symptoms are known, and enough of its physiology is known to allow responses to it. Research has revealed treatments that can, for some people, control the impact of MS while still falling well short of a cure.
In one sense, the most exciting treatments are those that slow the disease down, but they only work on the relapsing type of MS. Even then, they are specific to the patient, and don’t work for everyone. They are mainly fall into two groups.
First, various uses (I want to say “types”, or “flavours”, but I don’t know if that is right) of beta interferon have been shown to reduce the number and intensity of relapses in relapsing MS. This drug family is usually taken by injection, and it can have a range of strong side effects. Perhaps the most serious is the potential for liver damage.
Second, there are a series of drugs – some of them very recent – that, through various mechanisms, limit the immune system’s ability to attack the myelin sheath. Usually taken every day, these immunosuppressants and other immune system medications also have severe, even debilitating, side effects. An example is Lemtrada, a drug that breaks down certain immune cells. Although shown to be more effective in limiting MS relapses than other treatments, Lemtrada had a hard time getting regulatory approval because it sports an impressive list of major side effects. It is still used as a last resort when other treatments fail.
Recent studies of this category of drugs, including one released this month, continue to express concern about the balance between drug effectiveness and negative impacts. While not recommending against them, researchers are emphasizing that they are a long way from being perfect solutions.
For some years, MS patients relied mainly on treatments that target the symptoms of MS, and for victims of the progressive version of MS this remains their only drug treatment option.
By way of example, corticosteroids have many medical applications as anti-inflammatories, so the inflammation of the myelin sheath in MS is an obvious area for their use. Drugs like prednisone are used to reduce inflammation in MS patients, and can be effective in limiting the pain and debilitation associated with the symptoms.
Similarly, muscle pain from MS can be controlled to some degree through standard muscle relaxants. As many sufferers have recently found out in Canada, medical marijuana has also proved to be effective for some people in reducing the impact of some MS symptoms. These are just some of the examples of symptoms that can be managed by the same treatments in both MS and non-MS situations.
Treatments also include non-drug therapies.
Recent research is suggesting, for example, that regular physical exercise may reduce the disease’s impact on walking and mobility. For a long time the opposite was thought to be true. For impairment of cognitive functions, various behavioural therapies have also shown good success in particular cases.
For a while, there was hope that a new treatment proposed by Italian doctor Paolo Zamboni (unrelated to the Zamboni of hockey fame) would prove successful. Zamboni claimed in 2009 that he was able to alleviate MS by opening up the veins of some patients through vascular surgery. A number of subsequent studies, including one in 2013 by Canadian doctors, and funded by the MS Society, have apparently shown that Zamboni’s theory is not correct. However, given the controversy surrounding the Zamboni theory, it is likely that the results of a broader study of the Zamboni theory and technique, being carried out in Canada and reporting later this year, will be needed before a consensus is reached on whether it has any basis.
This is not intended to be a full summary of all treatments currently being used. It merely hits some highlights. There are more, and some of them are very promising.
For example, recently Toronto’s CAMH reported success in using myelin peptides to protect the myelin sheath, not by limiting the actions of the immune system, but by strengthening/restoring the myelin directly. Another recent study, funded by the National Institutes of Health in the USA and including American, Canadian, and British researchers, has shown that stem cell transplants may have potential in combatting relapsing MS.
When I am fundraising for the MS Society, I am of course conscious that some of the money goes to make the day to day lives of MS patients better. Just as much, though, I am conscious that some of it is going to fund these kinds of leading edge research.
The Elusive Cure
When you don’t know what causes an illness, it is more difficult to find a cure, but thousands of researchers around the world continue to pursue this disease on both fronts: cause and cure.
One of the least understood questions is why the immune system attacks the myelin sheath. There is research being done to see whether there is a specific trigger for these attacks, either within or outside the immune system. Related to this is the question of whether, whatever the triggering event is, there is a genetic switch that either causes it to be initiated, or allows it to happen.
A significant focus of some research is Vitamin D. Children who grow up closer to the equator produce more Vitamin D in their bodies due to greater exposure to sunlight. Some researchers believe this is the connection between place and time of birth, and incidence of MS. They are trying both to prove the causality empirically, and find neurological explanations that would provide the analytical connection.
Other researchers are exploring the potential relationship between certain viruses, such as measles and herpes, and MS. Since viruses like these often cause damage to, or inflammation of, the myelin sheath, the theory is that viruses may be the trigger that turns the immune system against myelin.
I would be very pleased to say that the cure is just around the corner. That would be lying. It could be. We don’t know. Scientists are exploring many lines of inquiry, and we simply have no idea which one will prove successful, and when.
No, no jokes in this article. I don’t find MS all that funny.
Multiple sclerosis is a serious problem, one that harms the lives of many people.
On the other hand, it is also a problem that we will solve, tomorrow or next week or a decade from now. All it requires is tenacity.
I am confident that, during my lifetime, MS will – like many other diseases before it – be fully understood, and then cured, and I will finally hear the evening news anchor saying that MS has become a disease of the past.
– Jay Shepherd, February 14, 2017