Please note, this is my personal experience of living with Ehlers Danlos Syndrome, the treatments and path to diagnosis and should therefore not be taken as medical advice. I am aware that some complaints are only indirect EDS symptoms but these also contribute to the overall picture and my experience with the condition, EDS.
Over a period of more than 10 years I have undergone various treatments. Here is a summary of my experiences.
Vesicare and Betmiga
With trembling knees and after continuing to walk around with my bladder complaints for far too long, I finally found the courage to go to my GP to indicate that I had incontinence complaints. He was very understanding and first prescribed Vesicare, and when this did not help, Betmiga. Both medications should reduce the urge, but after trying a few doses it was clear that this would not help. A known side effect is a dry mouth, and I still remember that well.
Flow-residue measurement
Then the medical mill started. From waiting time for intake, to waiting time for evaluation, to waiting time because the urologist went on maternity leave, to waiting time for the examination, would a Flow-residue examination finally take place. After waiting so long I was excited and nervous that something was finally going to happen. The examination would have to take place with a bladder that was as full as possible. In the meantime my bladder was constantly tense. I no longer had a sense of urgency, but about 20 times a day I had severe cramps followed by urine loss. My bladder and pelvic floor only tried anxiously to keep my urine in. On the way to the hospital I had already lost urine several times and I was afraid and tense that nothing would come of the examination. During the flowmetry measurement I could only squeeze out a few drops. Drops that felt good and gave relief. But of course this was not a full bladder and I was afraid that I had ruined the examination. Then came the ultrasound to measure the residue. This was done with an ultrasound device that is similar to the one used for pregnancy ultrasounds. The probe is provided with gel. Then it is firmly pressed against the abdomen at the level of the bladder. This was so painful that at that moment a jet of urine escaped. Despite this, to the great surprise and panic of the nurse I still had a residue of 700cc. This was let out of my bladder with the aid of a catheter.
Indwelling catheter
The conversation with the urologist was chaotic. I was stunned and had no connection with the doctor. My concerns were only increased by his helplessness. Because this amount of residue was dangerous for my bladder and kidneys, I had to get an indwelling catheter on the spot and then learn to catheterize myself. The urologist was not specialized in this subject and he would refer me to a colleague for further treatment. Self-catheterization would be taught by a specialized nurse for whom a separate appointment had to be made. So I went home with an indwelling catheter, a whole box of leg bags, night bags, brochures and so on. I had no idea how to work, walk or deal with this. The thought of self-catheterization was also a horror. The feeling of a constantly empty bladder was unfamiliar, pleasant but strange. No hassle with absorbent material and once I got used to the catheter, no worries. There was still tension, and the nurse had great difficulty inserting the catheter into my bladder. I also still had cramps that were more severe than without a catheter.
Self-catheterization
Fortunately, it didn’t take long before I was given instructions to catheterize myself. The nurse removed the indwelling catheter from my bladder. This was very painful and too sensitive to insert a catheter myself at that moment, so it was just an explanation, not an exercise with the nurse watching. I was surprised at how thin catheters were, and once I got home I managed it after a few tries and it was even okay. Unfortunately, I still couldn’t do without absorbent incontinence material. I catheterized about 6 times a day, and the feeling of a completely empty bladder was pleasant. However, this feeling didn’t last long. I still got cramps, followed by urine loss, but catheterization reduced the amount of urine, which was a small improvement.
Cystoscopy
Then I was put on a waiting list again. This time to discuss a follow-up treatment plan. First, they would look in my bladder by means of a cystoscopy. Of course, there was a waiting period for this as well. The cystoscopy was not too bad. The device that the urologist came with was scary and at first sight impossible to insert. Some numbing gel was applied to the glans and then the scope was inserted. I could watch on a monitor and the urologist told me what could be seen. It was painful along the prostate and it was slightly enlarged. There was something irritated in the bladder, but this was probably due to the self-catheterization.
Urodynamic examination
Since no major abnormalities were visible in the bladder, a urodynamic examination followed. Of course, there was also a waiting period for this. In the meantime, self-catheterization became increasingly difficult. I catheterized at set times, but often I had such a loss of urine beforehand that only a few drops came out of the catheter. For the urodynamic examination, a flowmetry and a residue test were performed again. Then a catheter was inserted anally and a tube with a catheter in the urethra. This looked like the scope of the cystoscopy but slightly thinner. The aim was to measure the tension in the bladder. The bladder was emptied while the sensors measured the tension in the pelvic floor and bladder. The device then filled the bladder with a liquid. I had to indicate when I started to feel a strong urge to urinate. It is normal for one to be fixated on the feeling of the bladder during this examination and a doctor also takes this into account. I was constantly asked if I felt anything yet. At 600cc I still didn’t feel the urge. The doctor stopped and emptied my bladder and another attempt was made. This time I got a cramp and the fluid escaped from my bladder before I could indicate that I felt the urge. Then a 3rd attempt was made. Now the fluid that was injected into my bladder immediately leaked out again. For some reason my bladder didn’t close. During the evaluation interview the urologist said that it wasn’t good, but that it wasn’t clear what was happening. A detailed treatment plan was therefore not obvious, but there were options.
Botox in the bladder
The first option was botox in the bladder. Botox paralyses the bladder muscle and since I had urine loss due to cramps I had high hopes that this would help me. Of course after another long wait it was time. First the bladder was emptied again and with the help of the cystoscope it was checked whether there was no abnormality in the bladder that would prevent botox. That was not the case and the botox could be injected. Another tube was inserted and the urologist injected 100 units into my bladder wall at different angles. This felt like pricking, annoying but not really painful. The optimal effect would last a few days. After a few hours I already felt something that was encouraging. I felt a firmness of my bladder that I was not used to. Cramps were still there but without urine loss. I had not been able to initiate urination myself for a long time, all urine flowed away uncontrollably. Not initiating urination myself caused a problem when the botox started to work. I got cramps but the urine did not flow away, which was extremely painful. I could catheterize but I got cramps 20 times a day that could not be overcome by catheterization. The botox could work for 3 to 6 months. I was happy when it had worn off after 3 months. I have used a lot of catheters and had a lot of pain.
Pelvic floor physiotherapy
The next step I went through was pelvic floor physiotherapy. The treatments and examinations I had undergone were quite drastic and physiotherapy initially sounded like a preliminary treatment. To get minor complaints under control again. Nevertheless, I went there with good courage. After having seen so many doctors and medical specialists who only looked at my bladder, it seemed good to see a therapist who looked at my body and complaints as a whole. The therapy initially started well. The region that was affected was completely involved in the treatment. From lower back to anus and bladder. Pelvic floor was a well-known but abstract concept and unknown what it meant for the body.
Tensing and releasing was practiced and here I noticed that releasing tension was a very unpleasant feeling. Relaxing has a counterproductive effect on EDS patients, but at that time I did not know this yet, and I could not put my feelings into words. The therapist did not understand at all why relaxing did not work for me and did not understand it either. It frustrated her and although she didn’t say it out loud I noticed that I was a difficult patient for her. I went home in pain after every therapy. I felt like I not only had no control over my bladder but also over my bowels.
I had pelvic floor therapy for about 6 months. Although it didn’t improve my symptoms, it did give me insight into my body.
Gentamicin bladder rinses
The urologist at the regional hospital had exhausted all options and I was referred to a university hospital. A new doctor, a new perspective, so this doctor wanted to do the same tests again, also because time had passed. Of course with the necessary waiting time, intake interviews, blood tests and discussions about the treatment plan. In the meantime I had had several bladder infections. These delayed the tests even more. Getting the bladder infections under control was the first priority from then on. After several courses of antibiotics an infection remained. Then I was given antibiotic bladder rinses. This was such a high dose that it was administered in the hospital. For 3 weeks I had to go to the hospital 3 times a week. The fluid was introduced via a catheter and had to remain in there for about 20 minutes before the fluid was removed again. This was sucked out, which was a bit painful. Then the bladder was rinsed with a rinse fluid. However, an infection still remained and the treatment was repeated 3 times. The values were still not completely good then, but it was decided to leave it at that.
Bladder rinses NaCl
I did get a maintenance dose of antibiotics to take orally. However, this caused a lot of intestinal complaints and I stopped after a few weeks. The last option was to rinse the bladder myself. I was given bags with bladder rinse bags and catheters to take home. The bladder rinse bags were not actually intended to be connected to a single catheter, but the urologist thought she knew that I would somehow manage to do this, which I thought was funny, but was true. I did this for about a year and a half, twice a day. Insertion, warming it a little to body temperature was no problem and not annoying. The fluid could remain in the bladder until urination. However, it was often after a few minutes that my bladder opened the gates, and although I had no problem with rinsing, I still found it a lot of hassle to insert fluid that ideally does its work in the bladder for at least 2 hours, but was urinated out again so quickly.
Cymbalta
That my bladder problems were complex was clear at the time. Cause and effect could no longer be traced. A new treatment was proposed to me that would be supervised by a psychiatrist. Of course, an intake interview followed, again after a long wait. Then a number of sessions were needed to determine whether I was eligible for treatment with an antidepressant. On the one hand, it was assumed that the drug would only help if one was open to the treatment of the nerves, on the other hand, one had to be mentally stable enough because the drug can also have side effects on the psyche of a person. The sessions went well, this psychologist was a very nice man with whom I, as an introvert, enjoyed talking. He was very human and I remember a conversation in which he grumbled about the senseless waste of money on computers and such in the hospital, while there was no money for a good salary for his nurses. His way of thinking and frustration was very recognizable and I experienced the sessions more as good conversations than as medical treatment. After some time I was finally prescribed Cymbalta. But unfortunately this also had no effect on my bladder.
Sacral Neurostimulation
The only therapy that could still be tried was neurostimulation. I had been hoping for this since I was referred to the university hospital, as this was one of the few hospitals that offered this treatment in our country. When the urologist suggested the treatment I had mixed feelings. I knew that there were not many options left, and this scared me. What if this also did not help? First a test neuromodulator would be used that would remain in place for 8 weeks, and if the result was good then the definitive one.
Nevertheless, there were and are many patients who benefited from a neurostimulator. A process was started to see if I would be eligible. From the perspective of the urologist this was the case, but my psyche was also (again) looked at. In the meantime I had urinary tract infections again and together with waiting times for each examination a lot of time passed again. A second urologist, with his vision, had to give a definitive answer as to whether the urinary tract infections would have a negative effect on the effect of the neurostimulator and he saw no obstacle. The psychologist and medical social worker also gave the green light.
Standard for an intervention is the consultation with the anesthetist. He used the reports of the psychologist and medical social worker, and his conversations with me to determine which anesthesia would suit me best. He thought I was stable enough to undergo this under local anesthesia. This gave me a strange feeling and I indicated that I did not want to feel any pain and he indicated that I did not have to worry. They only chose a heavier anesthesia if a patient could not mentally cope with the pushing and pulling during a medical intervention. A full anesthesia sounded tempting, but the choice had actually already been made by the anesthetist. The waiting time for the intervention would be considerably longer with a full anesthesia and this was the deciding factor to agree to a local anesthesia.
During the intervention there was only 1 doctor who did everything. The anesthesia, the placement and the stitches. I lay on my stomach and the neurostimulator was implanted in the lower back. Leads are connected to the nerves at the exit of the spinal cord. The anesthesia went well at first. Injections left and right, wait a bit like at the dentist. It took a long time before I noticed a difference but if you are focused on feeling, and to be honest, are also a bit scared, then feeling is not black and white. At some point it had to start and this went well at first. I did not need to know what he was doing and tried to shut myself off. However, the deeper he went, the more it started to hurt. The first cut may not have hurt, but I had felt it. But deeper the pain became unbearable. I indicated this and he started to inject more anesthesia. Again we had to wait for the anesthesia to work but every test, even after waiting for minutes, indicated that I still had feeling. More injections were given and I suspected that this was the last anesthetic he had at his disposal. Wait again and he started to reassure me which did work a bit. I became calm and after a few minutes he continued. However, I felt every movement and I screamed. He stopped a second time and I could tell he was in disbelief. He tested my pain reflexes again in the numbed area and it was clear that this was not fear or anything on my part, but that I was definitely feeling pain. There was panic in his voice, we waited a bit more and he said he was already so far along that it wouldn’t be long before the stimulator was implanted and he asked if I could handle it. I then felt needles going deep into my flesh and pain from the stimulator being implanted under my skin. I left the operating room dejected.
I could activate the stimulator myself after a few days using a smartphone with the app installed that connected to the stimulator. I could set the strength of the pulses myself and this was recorded for evaluation by the doctor. A certain target setting was indicated, this was a pulse strength that worked for most people. The low setting that I started with gave a pleasant feeling. This gave feeling, deep in my body, where I lacked feeling. It activates the region where the complaints are. lets you constantly feel your pelvic floor. But unfortunately it also remained a feeling and did not help me get my bladder under control. A higher pulse strength, about half of the target strength, was unpleasant. It felt as if the current made a tense nerve vibrate like the string of a violin. I could tolerate this for a few hours but ultimately did not help me.
After 2 months the stimulator was removed again, by the same doctor. He still recognized me and said that he would remove it as soon as possible. Again I was in a lot of pain, but removing the leads and the stimulator went a lot faster.
Conclusion
With that, there was no further urological treatment option. The urologist advised me to start a course of treatment to control the bladder pain. I had to learn to live with the pain and the thought of being urinary incontinent for probably the rest of my life. Bladder pain is a constant in my life. A belt is painful, I feel every bowel movement near my bladder. Even abdominal breathing pushes on my bladder and the pain radiates to my genitals, anus and prostate. Yet I have not yet started pain treatment for my bladder complaints. Over the years of urological treatments, my general physical condition has deteriorated. Joint pain, stiffness, nerve pain, tremors and much more came to the surface. While the acute pain stabs of urge decreased. I hardly feel the urge anymore and although I constantly feel my bladder, I have pain with a slight internal or external pressure, my bladder has become a lot calmer.
A urologist gave me an option early in the treatment period was a bladder removal. At the time, it was a shock to even consider this. During bouts of pain, I often thought: I’ll pull my bladder out with my bare hands. Now, the pain in my bladder is just one of many complaints and a bladder removal is too drastic and will not significantly improve my quality of life, unfortunately.
Bladder issues, bedwetting, and incontinence have had a major impact on my life. The despair caused by shame and lack of understanding of my own body has shaped who I am. My self-image has been negatively affected, and I often wonder what kind of person I would have become if I hadn’t experienced these problems growing up. Everyone is different, of course, but if a child today grows up with these problems, I hope they know and realize they are not to blame. I hope there is more understanding among parents, teachers, peers, and doctors about this issue and how much it can burden a child and adolescent. A few incidents have always stuck with me, which I describe here to illustrate their impact and to show the impact bedwetting and incontinence can have on a person. I hope this will be helpful to someone struggling with this today.
Living with Ehlers Danlos Syndrome 