As part of our Chordoma Survivorship Initiative, we will be posting a series of guest blogs over the next few weeks about the common — but too-often under-discussed — side effects of sacral chordoma. Written by sacral chordoma patients, the three blogs in this series feature interviews with doctors experienced in treating the challenges experienced by sacral chordoma patients.
Our first guest blog comes from sacral chordoma survivor and Chordoma Foundation Peer Guide Susan Hall. Susan talked with Dr. Stephanie Kielb, a neuro-urological surgeon at Northwestern Medicine, about the impacts of sacral chordoma and its treatments on the bladder, including options for managing bladder-related challenges during and after treatment.
If you or a loved one are experiencing similar issues, remember — you’re not alone. Responses to our Chordoma Survivorship Survey revealed that many of these challenges are common to sacral patients. If you need support or guidance for your specific experiences, we’re here to help.
Guest post by Susan Hall, sacral chordoma survivor
Sacral chordoma patients often experience side effects that impact the critical functions in the pelvis, including bowel, bladder, and sexual. While these functions are critical to the quality of every person’s life and health, they often aren’t discussed publicly.
"Our intent with these blog posts is to take the embarrassment out of these discussions and address real questions from real chordoma patients in a candid, direct, and — we believe — helpful and hopeful manner."
Susan Hall, sacral chordoma survivor
As a sacral chordoma survivor myself, my goal is not just to survive, but to thrive. I sat down with Dr. Stephanie J. Kielb, MD, Chief of Reconstruction and Neuro-urology in the Department of Urology at Northwestern Medicine, to discuss challenges sacral patients often have with bowel and bladder function, as well as options available to improve the quality of life for patients dealing with these issues. While the main focus of Dr. Kielb’s specialty is bladder function, she also shared some insight and support for patients dealing with bowel issues and women experiencing sexual function challenges that will be shared in the upcoming posts in this series.
Dr. Kielb, thanks so much for joining us today. Can you start by sharing what you look for when you see sacral chordoma patients who are dealing with bladder issues?
This is such an important topic. We don’t think about bladder and bowel until there’s a problem. The first thing I talk with my patients about when we talk about bladder function is being sure the bladder is storing urine in a safe way. High bladder pressure can develop in patients when nervous system input is damaged. The kidneys are very valuable organs and they’re pretty “wimpy.” They can’t take a lot of pressure. If you damage the kidneys, you can’t regrow them. So I like to start with a study — a little bladder pressure test — to see if the bladder is behaving in a way that won’t damage the kidneys over time. Above all, we need to protect the kidneys.
What kind of specialist should sacral chordoma patients seek out when it comes to bladder testing and function?
You will need a urologist that specializes in neuro-urology. Usually, most of us are in academic centers. It has to be someone who does this a lot. While they can do a great job, typical urology offices generally manage prostate issues, kidney stones, and urinary infections. You need someone who has a background and experience with patients who have neurological issues to treat chordoma-related challenges.
I get a lot of referrals both in my local area as well as outside the state. I sometimes partner with the patient’s local urologist. I do the studies and come up with the plan and work with a local urologist who can manage the patient locally. If you’re eight hours away from me I can’t see you quickly, but I can consult over the phone and help, particularly with surgical reconstruction issues. So that might be an option for patients as well.
What type of therapies are available and recommended for bladder retention and urine control?
Typically, sacral chordoma patients are dealing with bladder retention. You need to give patients an acceptable way to empty their bladder. There are several options available:
- Intermittent catheter: You put a catheter in the bladder, drain the urine out, and take the catheter out. There’s nothing in there permanently. The benefit is that there is no bag, no tube, no one can see it.
- Suprapubic catheter: Like a Foley or in-dwelling catheter, a tube goes through the lower abdominal wall and stays in all the time. The tube is external to the body, and attaches to a bag for drainage. It’s external to the body but it’s always there. This is done under a local anesthetic or a light general anesthetic.
- Mitrofanoff (or Monti) procedure: This is a surgical procedure where we use the appendix or intestine to create a small channel through the belly button and sew it onto the bladder. This way, the patient can catheterize through the belly button. Especially for women who aren’t ambulatory, transferring from a wheelchair, laying down, you can’t be out in society and do that. With a channel, they can do that in their chair. They don’t have to lie down and don’t even have to undress. There is no external bag or tube. And they can cath anywhere.
Can you talk a little about the Foley catheter? You spoke at the 2019 chordoma Community Conference at Northwestern Medicine and didn’t sound like a fan.
The Foley is an in-dwelling catheter, and it’s definitely another option. A big concern is that they are uncomfortable. But let’s say that a patient doesn’t have good sensation — even if they can’t feel it, the in-dwelling catheter is constantly putting pressure on the urethra. This can cause damage to the urethra over time and enlarge the tube, which can then result in leakage around the catheter. Often what we see is larger catheters are then placed, which over time causes more breakdown and once again leakage. The problem is that the urethra can expand and expand and, all of a sudden, where once the urethra used to be the size of a pencil, now it’s the size of a golf ball.
There’s also a higher probability of infection in the urine with in-dwelling catheters. There is a higher risk of kidney and bladder stones forming. Stones form when there is a build up of calcium. Stones can form with intermittent catheterization too. Anything going from the outside to the inside automatically means there will be bacteria on the inside. Some of these bacteria help make stones, and the stones can cause infections and catheter clogging. We know that in-dwelling catheters can also increase the risk of bladder cancer, double that of patients who don’t catheterize. So it’s not an exceptionally high probability, but there is important surveillance that needs to happen.
How do you determine which procedure you’re going to use with a patient?
It depends on the patient, their situation, and what they want to do. I talk with my patients about all of the options, discuss their mobility and lifestyle, and together we come up with options that will be best for them. If they don’t have very good hand function, for example, they may still be able to catheterize through the belly button. It also depends on their support at home and personal preference.
A suprapubic catheter is a ten minute outpatient procedure. We make a little tiny nick in the skin over their bladder and insert a small introducer, place the catheter in, and take the introducer out. It’s a very low-risk procedure.
If I’m making a channel with a Mitrofanoff Procedure, it’s a much bigger commitment. It’s an intra-abdominal procedure. Sometimes we’re also making the bladder larger at the same time, which is called a bladder augmentation. This procedure requires a week-long hospital stay and a 4-6 week recovery. It carries a whole set of risks. But depending on the patient’s situation and goals, it can also be life-changing. You can cath anywhere, any time. It’s a lot more convenient. But it’s not without risks.
With all of these procedures, we need to talk about it and make sure that the bladder has stabilized. I won’t do this surgery for 18 months after surgery or injury because I need to know what the bladder is going to do. And patients really need to think about it. I have a list of patients willing to talk with other patients about what to expect, what the recovery is like so they get the information from a patient level.
"There are a lot of things we can do to help. There are options that are low risk and very effective for helping you get control and get your dignity and your life back."
Stephanie Kielb, MD, Northwestern Medicine
While bladder retention is common among sacral chordoma patients, I’ve talked with a few of my fellow patients and survivors who have the opposite problem — bladder spasms and extreme leakage. Dealing with adult diapers can be inconvenient and stressful, especially when traveling or in social situations. Any advice or help that you can offer people who are dealing with these issues?
There are a lot of options. It goes back to the bladder studies and looking at the reasons for bladder leakage. There are basically two reasons for leakage. When you think about bladder function, I look at both storage and emptying. So the bladder doesn’t want to store urine. Why not? Is the problem with the bladder or the urethra?
There is a type of muscle around the urethra called a sphincter that squeezes when a nerve signal tells it to. This sphincter is always on squeeze. It’s like an always closed faucet. When you want to pee, a signal from the brain stem gives permission to the sphincter to open, the bladder gets a signal to squeeze, which pushes the urine out.
There could be problems with the sphincter or the bladder, or both. You could have a problem with bladder spasming and contracting or a sphincter problem so the urethra is not closed. So the bladder may be holding just fine, but when you bend over or cough, pee is getting pushed out because the “faucet” is open. 40% of women in the general population leak some urine; it’s very common. A lot of women have stress incontinence from having babies or being athletes. Men typically don’t leak as much because they have such a long urethra and it takes an angle through the prostate. But they can have a bad sphincter. Men can also have stress incontinence. They could also have had prostate surgery before and now the sphincter isn’t as good. Obviously history and exams are very helpful to know what’s causing the problems and what the bladder and the sphincter are doing. Bladder studies (urodynamics) help us figure this out.
As far as bladder spasms, there are a lot of treatment options:
1. Medications: There are two classes of bladder medications. Anticholinergic medications are an older class of drugs that we’ve been moving away from quickly. They have problematic side effects, like dry mouth, dry eyes, and constipation. There is increasing evidence that long term exposure can increase incidents of developing dementia later in life. This class includes medications such as oxybutynin, solifenacin, darifenacin, tolterodine, and fesoterodine.
The second class of bladder medications are called beta-adrenergic medications. The FDA has approved two drugs in this class, virabegron and mirabegron, which work by a completely different mechanism and don’t have any of the same side effects. They can give you a stuffy nose and mirabegron has an increased risk of high blood pressure but mostly for people who have blood pressure control problems. They don’t cause any cognitive issues. This class of drugs is our first go-to these days. We are actively switching patients from anticholinergic to beta-adrenergic.
2. Botulinum toxin (Botox®): Botox has revolutionized treatment for bladders affected by nerve damage. On the plus side, it’s an outpatient procedure that takes just minutes in the office and typically lasts 4-6 months in the bladder. And there are no side effects anywhere else in the body.
It’s very potent at paralyzing the bladder. We do different doses depending on the goal. Usually the standard starting point is 100 units, but we can start lower or go higher if the patient wants to be really dry. The tradeoff is that you won’t be able to pee; you’ll be catheterizing all the time. Once again, it’s individualized. I have to ask the patient about their goals. You can do botox and medications at the same time.
3. Surgery: If botox and medications together are still not enough and the patient really wants to be drier, there’s a more extreme surgery called a bladder augmentation. We take a piece of intestine and sew it onto the bladder to make it bigger. There are a lot of considerations for this. It’s a week-long hospital stay and a 4-6 week recovery. Most patients will have to catheterize and there are risks. Another, more drastic option is a urostomy, where we remove the bladder and give people a bag. It’s like a colostomy only we remove the bladder, not the colon. Again, that’s a big surgery. But for people who can’t or don’t want to catheterize, and want to be dry, it’s an option.
Are there any other options patients should consider?
There are more options than a lot of people realize. Let’s say the bladder pressure is fine, I refer a lot of patients to pelvic floor physical therapy. This can help with bladder and bowel. If you want to see how much you can improve without medication or surgery, start there. Sometimes physical therapy gets them to a certain point, then we add a med and they’re happy. If they are not relaxing their sphincter when they go to pee, pelvic therapy may help. However, if nerve function to the bladder is interrupted, physical therapy won’t help that.
What resources can you recommend for sacral chordoma patients to learn more about these procedures and options?
There are a few organizations in the U.S. that have patient materials, although they’re not specific to nerve damage issues. The Society of Urodynamics, Female Pelvic Medicine, and Urogenital Reconstruction has good patient handouts and treatment road maps, mainly for overactive bladder. The American Urogynecology Society also has good patient information and videos that are helpful.
What about sacral neuromodulation?
Sacral neuromodulators have been around for decades, but they are not approved for neurogenic bladder disorders (those caused by nerve damage), mostly because they attach to the S3 nerve root and sacral chordoma patients may not have the S3 nerve root.
Posterior tibial nerve stimulation, also referred to as PTNS, is also not approved for neurogenic bladder disorders. Most data suggests you have to have some intact nerve to get signals up to the brain for these modulation therapies to be effective.
Is there anything else you’d like chordoma patients to know as we close out today?
There are a lot of things we can do to help. There are options that are low risk and very effective for helping you get control and get your dignity and your life back.
This interview has been edited for length and clarity.
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