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Dr. Jones: Multiple sclerosis is a disease that's more common in women than men. It's a complicated disease, it's a neurologic disease, but it affects many parts of the body and today we're going to talk about the bladder, and MS, and your health, on The Scope.

Announcer: Covering all aspects of women's health, this is the Seven Domains of Women's Health with Dr. Kirtly Jones, on The Scope.

Dr. Jones: Today in The Scope studio, we have Dr. Sara Lenherr, who is a specialist in neurology, she's trained as an urologist, but she's pretty clearly interested in the way the brain talks to the bladder. And today we're talking with her about MS, patients with MS, and problems they might have with their bladder, and what might be done. So talk a little about the brain talking to the bladder and multiple sclerosis.

Dr. Lenherr: In normal patients that don't have neurological problems, the brain is designed to tell the bladder to store urine for as long as is reasonable, and then when you're near a bathroom, then you volitionally go ahead and void out your urine. Unfortunately in multiple sclerosis and a lot of different types of neurological disorders, the communication between the brain and the bladder is disrupted by the nervous system problems that happen in MS.

And so specifically, sometimes that bladder becomes over-active and receives too many signals from the brain, and then it also, the sphincter that's supposed to keep you from leaking doesn't necessarily relax when you want to go ahead and pee.

Dr. Jones: So what happens? So a woman who has MS and has neurologic symptoms in her bladder, what would she experience?

Dr. Lenherr: So usually they'll present with urinary frequency and urgency, but sometimes they just don't empty their bladder at all, so they'll feel like they have to go, they try to go, and then they can't empty out their bladder completely. Either just a little bit comes out or none comes out at all, and interestingly, sometimes we catch these cases of multiple sclerosis before they're even diagnosed by a neurologist, because women will present when they're a little bit younger, and they have no reason to be in urinary retention to not empty their urine.

Dr. Jones: So let's back up, so urinary retention. You mean if they go a little bit, then their bladder gets fuller, and fuller? I see a balloon in my head.

Dr. Lenherr: Exactly.

Dr. Jones: How full is too full and what happens?

Dr. Lenherr: Well, if you have too much urine in your bladder, especially for women, usually when you have too much in your bladder and you're a female, you have what's called overflow incontinence, where the urine just comes out even though the sphincter is nice and tight. And so those women will notice that they just leak, and they can't empty out all the way. They feel full.

Dr. Jones: Well, so a lot of women leak, so how would you know that it's overflow? What test would you do?

Dr. Lenherr: So we can do either a catheterized volume to see if there's urine left over after you pee. Or we can just do a little bed side ultrasound to evaluate whether or not there's any urine leftover in your bladder.

Dr. Jones: So a urologist might actually pick up MS before the patient shows the other neurologic signs of MS.

Dr. Lenherr: That's correct.

Dr. Jones: And these are young women.

And young women being wet all the time is devastating, well it's what, it's devastating for any woman of any age, but for young women in particular, they don't want to be wearing pads, and Depends. So what kinds of things do you have to offer for women with MS?

Dr. Lenherr: So once we identify the problem, then we need to discuss with the patient what drives their quality of life, and what is a safety issue. So safety issues would be if your bladder doesn't empty all the way, and it ultimately causes the bladder to stretch out and cause damage, and sometimes could impact kidney function.

Dr. Jones: Oh, so it backs up and backs up?

Dr. Lenherr: It can back up all the way and it could cause the kidneys to have damage which is a bigger issue. The other thing that can happen with the urine sitting in the bladder for a long time, it can lead you to get urinary tract infections. So there's multiple things that we can address with a safety issue, and then we need to look at quality of life, so quality of life is impacted by leaking all the time, or having to go to the bathroom all the time.

Dr. Jones: So do women have to empty their own bladder with a tube? I mean do you give them medication to make their bladder squeeze a little harder?

Dr. Lenherr: So depending on how their bladder works when we evaluate it, we frequently have to have these patients go use a small catheter to empty their bladder on a timed basis. And that generally treats them very well because it empties the urine when they want to, and they're able to control risks of urinary tract infections and kidney damage, and then they also don't have the overflow incontinence that we discussed before.

That's one good strategy, sometimes if the over-activity is really bothersome, and they still have irritation even though there's a small amount of urine in their bladder, we put them on other types of medication and we also can offer them chemodenervation, which is called botox, which is similar to the botox that you put on your forehead for wrinkles, we can inject that in the bladder to relax it.

And we can also put in nerve stimulators that help act like a bladder pacemaker. So there are multiple different options we can offer women with multiple sclerosis to help them manage their bladders better.

Dr. Jones: Well that's great news, because for MS it's a condition that waxes and wanes through a life time. It often begins in women's early 20s, or 30s. So giving somebody the qualify of life so they can be the persons that they want to be, is a really important service that you guys can offer.

And I think for many women with MS, they feel like their life and their agency has been taken away, and empowering them to have a little more control.

Dr. Lenherr: Exactly, and also considering that we follow them for the rest of their lives, and sometimes their bladder conditions change, so we need to adjust the strategies that were working five years ago.

Dr. Jones: And here at the University of Utah, we have a medical record that helps our doctors talk to each other so you're not doing this just in the urology clinic. You talk to their other MS doctors.

Dr. Lenherr: Exactly.

Dr. Jones: Because often they're on a lot of meds.

Dr. Lenherr: We coordinate all their care and make sure that we're all working together to improve the quality of life and keep them safe.

Dr. Jones: That's great to know because I've had a lot of patients over the years with MS. It's very discouraging. Knowing that there's things that they can do is very helpful, and Sara thank you for joining us on The Scope.

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Interviewer: ALS, what is it and what do you need to know? We'll examine that next on The Scope.
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Interviewer: You may have heard of ALS, or Lou Gehrig's disease, but do you really know what it is and how it could affect you, or somebody you love? We're going to find out more with Dr. Mark Bromberg. He's Director of the ALS Clinic at University Utah Healthcare.
Let's talk about ALS for a second here. I'm not even going to try to attempt to say what those initial stand for. I'm going to let you do it.

Dr. Mark Bromberg: ALS stands for Amyotrophic Lateral Sclerosis and it's the term that was coined by a French neurologist in the 1800s when they were just beginning to understand the diseases of the nervous system. Actually it's a very descriptive disorder, because the amyotrophy indicates that there's atrophy or shrinkage of muscle and that in turn we feel is due to fewer nerves from the spinal cord going to the muscle.
And the second, "lateral sclerosis", refers to a second set of cells that send their fibers down the lateral sides of the spinal cord. And when they degenerate there's a bit of scar tissue that forms, which is hardening. And so sclerosis is a general pathologic term for hardening. So that indicates there are two sets of nerve cells that degenerate in ALS; one that goes out to the muscle, and one that starts up in the brain and sends their fibers down.

Interviewer: So as a result of that, what are the symptoms that somebody would have?

Dr. Mark Bromberg: The primary symptoms of ALS are weakness. And so ALS, for unclear reasons, tend to start locally. In other words, people will have difficulty or weakness with functions. For example it may start with difficulty with speech, in other words they're not able to move their tongue easily and so the words are slurred, and with that can be difficulty with swallowing. Or it may start in a limb. When it starts in a limb it tends to start in a hand, if it's in the arm or in the foot if it's in the leg.
The problem with ALS is that this weakness progresses. No matter where it starts, it moves to other areas. Unfortunately, at this time we do not have a cure for the disorder. The weakness does progress, and eventually it involves the nerves that go to the diaphragm muscle, so people do pass away from respiratory failure.

Interviewer: So people can die from it, but more likely they just become so weak they can't function anymore? What exactly happens, you end up in a wheelchair?

Dr. Mark Bromberg: Well, yes, weakness does progress. Yes, people do have difficulty with walking and need a wheelchair. But unfortunately everybody does pass away from the disorder.

Interviewer: Just end of sentence.

Dr. Mark Bromberg: Correct.

Interviewer: Gotcha. That sounds pretty scary.

Dr. Mark Bromberg: Well, it is scary when you stand back and look at it. To have a condition that starts, and we don't know why it starts, and it progresses and we do not have a therapy, and people pass away from it. Yes indeed, it is a frightening prospect.

Interviewer: How common is it?

Dr. Mark Bromberg: Well, statistically it's considered to be a rare disorder. But practically speaking it's not that uncommon. If you look at numbers, about 2 per 100,000 people develop the disease in any given year. If you stand back, in the United States, that's about 5,000 or so new cases per year.

Interviewer: So not a lot really, unless you're that person, I suppose, or know that person, or love that person.

Dr. Mark Bromberg: That is correct. It's somewhat of a quiet disorder in that about 20 years ago when we did not have the clinics, doctors generally said, "This is what you're diagnosis is. There's nothing we can do about it, so no need to come back." Under those circumstances people tended to retreat into their homes and were unable to get out and so it was very must a quiet disease.
But when you hear about it and talk to your friends and neighbors about it, you find that more often than not that there's somebody in your locale, or sometimes several people in your locale, who do have the disorder.

Interviewer: So you talk about the clinic. You've said it's a multi-disciplinary clinic. What the advantage to having that type of a clinic treat somebody who has this disease?

Dr. Mark Bromberg: Well, as I mentioned earlier it may start in one particular place and move to other places. In some sense no two patients have the same difficulty at the same time. So in the multi-disciplinary clinic, the disciplines are people who can provide specific care.
For example, we have speech pathologists to help with improving and enhancing speech output. We have nutritionists in case swallowing becomes a problem and people lose weight. We have occupational therapists to aid with hand function, physical therapists to aid with leg function. We have respiratory therapists to help when the breathing becomes shallow.
We also have a social worker, because this obviously, in addition to having a physical toll, does have a psychological toll, and not only for the patient, but also for the caregiver. We have a clinical pharmacist. We have perhaps the most important person, which would be a nurse. And then we have the neurologist.
This particular clinic is sponsored by the Muscular Dystrophy Association, so we do have a representative from that association. And they can help with community resources and the lending out of equipment at times.

Interviewer: So it's very obvious by all the people on this team that, as you said earlier, there's no treatment, there is only managing of the disease, it sounds like, and this is all part of to maintain somebody's life.

Dr. Mark Bromberg: That is correct.

Interviewer: What kind of drugs? Are there drug treatments to slow this down yet?

Dr. Mark Bromberg: Well, at this point there is only one drug that is found to have any clear effect on the course of ALS. The problem is that the effect is very modest, and that drug is called "riluzole". All of the other medications we use to help manage symptoms as they arise.

Interviewer: And is this hereditary? Is ALS hereditary?

Dr. Mark Bromberg: Well, it turns out that in about 5% of people; they will give you a very clear family history that it is in the family. But as we determine genes that are associated with it, the number of genes is fairly large. At least 12 different genes have been associated with this.
We are also finding that in some families they do not appreciate that there's an underlying gene, but we are beginning to find that. So overall, perhaps 8-9% of people have a gene associated with the disorder. Some we think is directly causative and some of it may be contributory.

Interviewer: What's the worst part of ALS in your opinion?

Dr. Mark Bromberg: Well, I've seen a lot of people with it and it's very difficult for me to say from their perspective. But what I hear from my perspective and I what I put together is that as the weakness progresses, people can kind of get used to the degree of disability and manage at that time. But as time goes on, just as their managing, they become weaker yet and weaker in other areas. And they manage that, and then they become weaker and in other areas. So it's sort of the relentless progression that to me, I think, would be the most challenging.

Interviewer: Do you have any final thoughts or anything that I forgot to ask you, or something that you would want a listener to know?

Dr. Mark Bromberg: Well, I don't think people should be overly concerned about contracting ALS, because it is a rare disorder. But do be aware of it, and if there are any people in your family or in your neighborhood or your other social circles that do have the disorder, you may inquire or is there anything you can do to help them out or help the caregiver.

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Interviewer: After receiving stem cell therapy, mice with a multiple sclerosis-like condition go from being nearly paralyzed to walking. My guest and co-lead author of this study, Dr. Tom Lane, talks about these exciting results just published in the Journal of Stem Cell Reports. Dr. Lane, what is multiple sclerosis?

Dr. Lane: Well, MS is an autoimmune disease and essentially it's a misguided attack of the immune system against proteins within the central nervous system, proteins that are primarily enriched within the myelin sheath. And so this is why many people that have MS have impaired motor skills or problems with vision as well as a variety of other clinical problems.

Interviewer: Can you explain what myelin is?

Dr. Lane: Myelin, a simplest explanation is it's analogous to the rubber sheath that encircles an electrical wire. And so the myelin does a very similar thing. Myelin wraps around axons. The axons extend from the nerve cell body and similar to an electrical wire, impulses are sent down the axon to allow us to do what we're doing right now-talk, move. And so the myelin sheath is necessary for this to occur.
Now, if that myelin sheath is damaged or destroyed in any way, that disrupts the conduction of the nerve impulses. So the primary area of research right now in my laboratory is focused upon trying to repair the damaged myelin.

Interviewer: And so you look at this problem in mouse models. Why did you choose that way to research the problem?

Dr. Lane: Well, the mouse recapitulates many of the clinical and histological features that we see in MS patients, inflammation of the central nervous system and demyelination. They have some impaired motor skills. A very small percentage of animals will be completely paralyzed, but we provide the necessary food and water for them because ultimately they would not be able to do so, because animals will have to stand on their hind legs to acquire the food.

Interviewer: Oh, right. So you injected them with neural stem cells. Correct? What were the main findings in your study?

Dr. Lane: Well, it was kind of surprising. Science works in funny ways and this was a happy accident for lack of a better word. We had started these experiments using human neural stem cells, and these were provided by my collaborator and good friend, Dr. Jeanne Loring, who is the Director of the stem cell center at the Scripps Research Institute in La Jolla. And within the central nervous system, rejection of foreign tissue has been somewhat controversial whether they would be recognized as foreign or not. So that was the original idea behind doing these experiments.
And so we surgically engrafted these human neural stem cells into the spinal cords of animals and my post doctoral fellow, Dr. Lou Chen, who is first author on this particular paper, came to me and said the mice that we transplanted are walking. And I didn't believe her. And it's not that I didn't believe her, but I was surprised.

So we went down and I looked at the animals. And sure enough, the control animals had what I would have expected, severe paralysis, whereas the animals that we had transplanted with the human neural stem cells within a remarkably short period of time, about 10 to 14 days, had regained motor skills.

Dr. Lane: That's a great question. The drugs that are available, the main three are called the ABCs: Avonex, Betaseron, and Copaxone. What those drugs are designed to do are to stop the infiltration of myelin reactive lymphocytes into the central nervous system. So, in other words, to stop the progression of the disease. None of these drugs as we know them function to restore or promote remyelination.
So MS, once you're diagnosed with it, about 85% of people that have the initial form of the disease have the relapsing, remitting form of the disease. Ultimately though they will progress into what's known as the progressive form of the disease. And the progressive form of the disease, there are no FDA approved therapies that are available to MS patients.

Interviewer: Okay. And so is that where you're taking the research now?

Dr. Lane: Yes, but we report in this paper is that our human neural stem cells secrete TGF-beta 1 and 2, that's transforming growth factor beta 1 and 2.

Interviewer: That's a molecule.

Dr. Lane: That's a molecule. That's exactly right. What's interesting is we can take TGF-beta 1 and 2 in vitro using our particular cells that we draw from our animal models of MS, and they function the same way. They convert bad T cells to these regulatory T cells. So we're very excited by this. But I think inevitably there have to be other molecules involved, so we have to just take it one day at a time and slowly work through the problem.

Interviewer: Do you see that this work might lead to clinical trials at any point?

Dr. Lane: I hope so. That's the goal of most people doing translational type of therapy and it's one of the reasons I wanted to come to the University of Utah, because they have such an outstanding record of this. So that's where we're moving. We want to try to move as quickly and carefully as possible. The emphasis being on carefully to make sure we have something safe. I would love to have something that could promote repair and ease the burden that patients with MS have, and hopefully see some type of therapeutic recovery.

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