Daratumumab 3-months update

I'm still improving!  

Hi everyone, good to see you back here! 🌺 

First of all, apologies for being quiet for quite some time. It's been a while since my 7-week daratumumab update. Somehow, I just couldn't get in the writing-flow over the past weeks. I guess it's because I'm doing a lot better and I'm finally able to do so many things again, which I haven't been able to do in the past year. So, sitting at my computer during my time off didn't sound too tempting. On top of that it's been really nice to have a bit of a break from constantly thinking about my small fiber neuropathy. 

But even though I've been a pretty bad blogger over the past weeks, I'm definitely planning on continuing my blog, so bare with me! ☺️  

After I wrote about my treatment success with daratumumab in my last post, I've been contacted by quite a few people with small fiber neuropathy or similar illnesses, who had questions, were interested in trying daratumumab, or just wanted to hear more about it. 

I'm very happy to see, that writing about my experience may be helping some others as well. It's also great to see that that there is a community out there where you can exchange experiences and help each other with tips, tricks, and knowledge. When it comes to rare diseases, patients often know at least as much, if not more about their illness than their treating doctors. But getting to this level of knowledge is really hard work, so exchanging experience with others is so important. 

This was one reason why I decided to start writing this blog (besides obviously keeping my family and friends updated), so I'm happy to see that people actually find it and read it. Even though there is still a lot of room to improve my appreance on Google.....but oh well 🙃

That's me writing my last blog post while we were staying at the country side.

And because this photo is already about a month old, I figured... 

It’s time for an update on my symptoms and therapy! 

...and given that it's a rainy day today, and Steve is away for a bike tour through Germany, today is a good day to sit down and write.🤓

So, how am I doing?

In  short, I’m sooo much better and I'm still slowly improving. So I’m veeeery happy with my daratumumab therapy. 

On Friday, I had my 10th (3 months) daratumumab injection, which went pretty event-free. I also finally managed to take a pic, so that you can all see how it looks when I get my injections (below). They inject the liquid into the subcutanous tissue of my stomach over a time period of about 10 minutes or so. The injection leaves a bit of a bruise for a day or two, but other than that it doesn't hurt. 

I told you before, that I'm getting my injections at the hospital in Lucerne, which is about an hour away from home in Basel. That's just because the only neuro in the German speaking part of Switzerland, who is specialized in small fiber neuropathy, is in Lucerne. 

I've learned over the years that it's easier to just stay within one institution as opposed to organizing getting my inejctions in Basel. Coordinating treatments between institutions (I've done this before for IVIG treamtents) has potential for all sort of confusion and complicated logistics, and it can easily turn into more effort than just sticking with the original institution, where they know me and they have all my labs etc. 

After all, you can't just go for injections somewhere, but you need to involve new physicians at the new institution, who look after you there and who might have different opinions on your treatment plan and who knows what. So given that I'm quite happy with the hospital in Lucerne, I decided to just travel to Lucerne. 

And because Steve is away on a little holiday, I went to Lucerne by myself by train for the first time since I started therapy. I must admit it's a lot more convenient (for me, not for Steve obviously 😂) to be driven directly to the hospital and back and to have company by Steve, but I was also very happy to know that I'm finally able to be more independent again and to manage trips like this without being in discomfort and stress.🙏🏻 

How have my symptoms changed since my last update?

In my 7-week update, I wrote that I had hypersensitivity symptoms, mainly in my upper body. They started during dartumumab therapy and they're pretty uncomfortable. Thankfully, they have slowly been getting better over the past weeks. I still have some residual hypersensitivity on my lower right arm. I mainly feel it whenever I wear short sleeves and I'm typing on my computer. When my bare arm is lying on my desk or when it touches the edge of my notebook, it just kind of feels as if the skin in this area was bruised. But, thankfully the hypersensitivity to clothes on my entire upper body has disappeared.🙏🏻

Sometimes, when I wear shorts, my legs feel scratchy when they touch each other. It's a lot better than it used to be; I used to not be able to wear any pants that were not made of super soft fabric, because it literally felt like I was wearing sanding paper. Now, it just kind of feels as though my legs were a little hairy, even though I do my best to keep them smooth.😂🙈 So overall, just a bit of occasional discomfort, but compared to before it's really nothing, and it seems to still be getting better slowly. 

Another thing I still notice are occasional fasciculations, mainly after I do physical exercise. Fasciculations are unvoluntary muscle twitches, which I can  feel and sometimes I can also see them (I've tried to take a video before but i always missed them). We all get them occasionally, but with small fiber neuropathy you tend to get them a lot more. These days, I mainly get them in my legs, but sometimes also in my stomach, my back or my neck. They dont hurt, so if they were to stay I can totally live with them. I think it must be something to do with the signal transmission between nerve and muscle, and whenever there is lots of signals  (i.e. after exercise) the transmission gets a bit overexcited or something.

Over the past few weeks, I've also gotten an occasional weird feeling of restless legs in the evening when I was sitting on the couch watching TV. This has been happening mainly on days when I'd been physically active. It's a weird kind of electric overexcited feeling that is very hard to describe, but it forces me to move my legs because keeping them still is very uncomfortable. It wasn't super strong, and whenever I went to bed it calmed down and was gone in the morning. I know some people with restless legs syndrome have it a lot worse and it keeps them up at night. I've only had this once or twice before starting daratumumab, so I've been telling myself that it must also be a sign of my nerves healing (more signals being transmitted??🤷🏼‍♀️), but who knows really. On the bright side, I exercised 20 minutes on my crosstrainer yesterday and didn't get restless legs in the evening.🪵🤛🏻

One other residual symptom I have is that I can still feel the bottom of my left forefoot getting pretty tender when I exercise. Considering that I wasn't able to wear regular shoes, let alone do any kind of exercise 2-3 months ago, this is massive progress. I can just tell that it's not entirely gone yet, because I can feel a difference between my left and my right foot, and my left foot has always been affected by neuropathy more strongly. Don't get me wong, my feet are doing quite well. I have been for a few hikes and was doing very well, it's just that I can still feel something there occasionally. My interpretation is, that this is probably residual nerve damage, which takes a while to get better. We'll see if it ever disappears entirely. 

So in summary, 

I'm doing a lot better and symptoms still seem to improve, although at a much slower pace now. So I guess you could say my small fiber neuropathy is in 'partial remission'?

Prof. Wexler and I discussed how to proceed with my treatment. And as always....

it's all trial and error....

The initial phase of my daratumumab treatment was an 8-week trial of weekly injections, which were sponsored by Janssen, because health insurances didn't want pay. 

These 8 injections were completed about 4 weeks ago, and Prof. Wexler and I decided to just apply for treatment continuation with health insurance, to be on the safe side. But we hadn't really decided on how to exactly continue my treatment yet. 

The great news are, that my health insurance has agreed to cover my daratumumab therapy for at least the next 12 months (🎉🎉), so now we are free to plan my treatment without worrying about who pays for it. 

There were a few options on how to proceed with my treatment, and it's kind of impossible to know which one is the right one. There are a few case reports in the literature, where they used daratumumab to treat different  autoimmune diseases, but every report applied a different treatment regimen.

1. One option was to just stop traetment after the first 8 injections and wait and see what happens. This is what they did in some of the papers where they used daratumumab for hematological autoimmune diseases with mixed results. Some people remained stable and kept improving whereas others had relapses...... 

However, I'm just not ready to stop treatment, now that I've finally found something that works with very minor side effects. I'm absolutely mentally not ready to deal with the uncertainty of whether or not symptoms would come back any day. I'm soooo unspeakably relieved to finally have a break from it, and I'm more than happy to keep going with a therapy that's finally doing a great job. 

So I told Prof. Wexler that if possible in anyway, I want to continue getting injections, and he totally understood. We also consulted with my clever friend N, who is our hematology daratumumab expert, and she also agreed that maintenance therapy of some sort probably makes sense.  

2. So we had to come up with a maintenance treatment plan. Problem is, there is not much evidence outthere on how to apply daratumumab over a longer period of time in autoimmune diseases, and it probably doesn't make sense to apply the cancer (multiple myeloma) treatment scheme 1:1 either...

We came across one case series, in which they used daratumumab in patients with a specific antibody-mediated type of autoimmune kidney disease. They also did the 8 week induction period of weekly infusions (they did an intravenous regimen), and then continued for another 4 months with less frequent infusions. After that they stopped treatment and observed patients closely. 3 out of 10 patients had symptoms return within 6 months after stopping treatment, and two of them were then put on a maintenance treatment of one infusion every two months on which they were doing well. 

By and large, this is the study we are sticking with, except that my treatment is subcutaneous and not intravenous. But Prof. Wexler and I also agreed that it's probably pointless to plan too far ahead, so we will take it about 2 months at the time. For now, I'm getting an injection every two weeks for 1 more month and then we will go down to one injection per month. 

We also decided to just put our feelers out, and to contact some of the international daratumumab experts who have published prior case reports, in which they treated patients with different autoimmune diseases. We'll ask them for their experience and their opinion and then go from there. Fingers crossed we get some insightful replys.🤞🏼

I also had a chat with my hematologist yesterday...

and she agreed with this plan. She said that my blood values (hemoglobin, thrombocytes, white blood cells etc) look very stable, so she sees no problem in continuing injections. She also agreed that it may make sense to stabilize my neuropathy for at least 6 months.

One thing I really wanted to discuss with her was my ongoing drug treatment to prevent infections. When I started daratumumab therapy, they put me on a prophylactic drug regimen of an antibiotic 3 times per week (sulfomethoxazol/trimethoprim) to prevent a rare but dangerous bacterial pneumonia (pneumocystis carinii) as well as a daily pill of an antiviral drug (valacyclovir) to prevent me from getting Herpes Zoster. 

Patients with immunosuppression are more susceptible to these kind of infections and she told me that, especially during the early phase of my treatment, when I got a lot of steroids on top of the daratumumab to prevent infusion reactions, I needed to take it. 

However, taking long-term antibiotics is just not something I feel very comfortable with, as it messes with my digestion, and I've read so much about how important it is to keep your microbiome healthy. So I asked her yesterday if I could possibly stop these two drugs and 🎉🎉🎉🎉 she agreed. She said, now that I reduced the frequency of injections plus we also reduced the dose of steroids per injection (from 20 mg dexamethasone down to 8mg dexamethasone) because I never had any side effects, it should be fine to stop them. 

And I'm very happy with that 🎉 

So the only drawback for me at the moment is, that I'm immunocompromised and we are still in the middle of a pandemic with rising numbers of COVID infections in Switzerland. Apparently, up to 40% of people my age in Switzerland (thankfully not my friends and family💕) do not believe in vaccines - but let's not get into this topic - it's mind boggling.🤯🤯

I'm very glad I was able to get the COVID vaccine before starting daratumumab, but I probably don't have the same protection from it at the moment, as someone who is not immunocompromised. So I have to remain careful and just try to live as though I hadn't been vaccinated. However, compared to living in daily pain,  this is a very small price that I'm very happy to pay. 

For now I'm just avoiding crowded places, I don't eat indoors in restaurants, and I only get close to people of whom I know they had the vaccine, which thankfully is pretty much everyone I usually interact with anyway. The rest is out of my control, I guess.🤷🏼‍♀️

And on this note, I wish you all a happy Sunday!

Thank you all for reading my blog and for caring. 🌺 If you want to be notified of future posts, click below to never miss a post❤

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Daratumumab - 7 week update

I'm doing a lot better 🪵🤛🏻 

Hi everyone, good to see you back here! 🌺 

It's been a while since my 3-week daratumumab update, and back then I wasn't really sure if therapy was helping. Four weeks later, I´m very happy to report: 

Daratumumab is doing a great job so far! 

Obviously, I have a lot to update you about, and I'm not sure where to begin. I guess I'll just chronologically tell you how I slowly got better. I'm actually still kind of scared to write about my progress, because over all the ups and downs over the past years and plenty of treatments not working or causing serious side effects, I've kind of become supersticious.🙃 I feel like I jinx my therapy if I go on about how good it is too early. 🙈 But given that I actually went for a hike yesterday, I think it's time to talk about it.

About one week into therapy, I noticed the first changes 

when my hands started to do a lot better. Suddenly, I was able to type on my computer without my hands getting sore from touching the keyboard. I was super excited and couldn't believe what an easy ride this was.....

But then about two weeks into therapy new symptoms started

and I started to get these hypersensitivity symptoms in my lower arm and my back. Then my nose started to get itchy and tingly again. I was pretty thrown off about what was going on. It seemed like daratumumab was definitely doing something, but I wasn't sure if my neuropathy was getting better or worse. At that point, my feet hadn't improved yet but had actually gotten a bit worse as well.

About three to four weeks into therapy, I had a few days where my whole upper body was in pain simply from a t-shirt touching the skin, which is something I'd never had before. My skin always kind of felt scratchy and slightly burning at time, but never hypersensitive like this.

The first month of therapy was a brutal mind-game

Overall the first four weeks of daratumumab therapy were kind of brutal on my mind. I was over-analyzing every symptom 24/7, and with these daily changing symptoms and new ones popping up, I had a couple of weeks when I was struggling at keeping it together. 

After all, I'm well aware that daratumumumab is one of the last therapy options I haven't tried yet. If it wasn't going to work there was really no obvious next drug to try and I might be stuck with neuropathy for good... And on top of that it is totally experimental, so nobody could tell me what to expect. So to put it lightly, there was a tiny bit of anxiety in the back of my mind, which I tried to ignore...🤯🤯🤯

I realized that daratumumab was actually doing something, because I didn't have this hypersensitivity before, but what if it made it worse??!! 

I was telling myself that it's possible that healing nerves can cause my body to be hypersensitive. But after all I'm not a neurologist, and even neurologists only make educted guesses when it comes to autoimmune small fiber neuropathy. So what if the hypersensitivity was caused by the drug and was here to stay??? 

But about four weeks into therapy my feet started to get better

But then, about four to five weeks into therapy, I noticed that I was doing better. The itch in my nose disappeared again and I was able to wear regular ballet flats and go for small walks without my feet getting sore and despite pretty warm weather. 

Walking in 'normal' shoes is something I haven't been able to do in a year. As soon as I put on shoes with hard soles, it sort of felt like I was walking on gravel and pain would get worse if I kept walking. I was able to walk in padded shoes more or less for most of winter (probably thanks to rituximab). But for the last 2 months my feet even got sore and hot in soft padded shoes from walking a few minutes. 

But yesterday I went for a hike 🙏🏻

Steve and I are currently on the countryside in a house of my family for two weeks. Yesterday we went for a hike. I had been for little walks in the area over the past week, and I felt like it was time to try and walk a bit further. I couldn't believe it myself, but we walked for a bit over 2 hours in full heat and in hiking boots (about 15'000 steps). After about an hour, my left forefoot felt a bit irritated and a bit like it was swollen, but it didn't get worse and was absolutely tolerable. It kind of feels surreal, because a few weeks ago, even just putting on socks or hiking boots was painful. 

I stopped Lyrica / pregabalin

Another great progress is that I've been able to stop pregabalin /Lyrica, which I started in March. 

Thankfully, my experience with pregabalin / Lyrica wasn't bad. It made me a little bit tired, but it wasn't bad once I was on a steady dose. And it did help with the pain in my legs. So I'm glad I tried it and took it for a while. 

But it feels so nice to be off this drug again. After all, it just masked the pain and didn't solve the root cause of my neuropathy anyway. Plus it didn't control my symptoms sufficiently anyway. 

Hypersensitivity is getting better but is still there

I still get these weird hypersensitive areas on my upper body. The full-upper-body hypersensitivity lasted for about 2 to 3 days. Since then I've kind of had meandering symptoms, which move around on my upper body. Sometimes my shoulder blades are hypersensitive, sometimes my right arm, sometimes my back, and sometimes my hips - and sometimes all at the same time.😂 But overall it´s been getting less intense and it's not too bad anymore.

I talked to Prof. Wexler 10 days ago and he said that hypersensitivity can always mean two things: either nerve damage is getting better or worse.🤯 Apparently, it's something they often see when nerves heal after a physical injury. 

He agreed that in the context of all other progress, these symptoms are likely from my nerves healing, and that he would interpret this as something positive. I was really glad to hear him say this, because obviously he's the expert, but it's what I've been telling myself and really hoped it was the case.

After all, I do have neuropathy symptoms all over my body, which means that small nerve fibers all over my body have gotten destroyed to a certain degree. Obviously, they don't just reappear from one day to the next. And it makes sense that newly growing neurons could cause hypersensitivity.

Now that I finally have a break, I notice how exhausted I am....

I'm definitely doing so much better than a few weeks ago, both mentally and physcially.🙏🏻🙏🏻 Not being in constant discomfort kind of feels surreal and normal at the same time, and I definitely notice how I appreciate little things that I just took for granted before.  

Now that I finally have a break, I also realize just how exhausted I am from all this. I guess that is no surprise....🤷🏼‍♀️ For the past year I've kind of functioned in autopilot, pushing through from one treatment to the next, and from one day in pain to the next, trying not to think about the WHENs and IFs. After all, I didn't really have a choice on whether or not to keep going.

So I guess it's only normal that a year of physical pain, super limited activity, no treatment working, and the uncertainty about whether or not I'll ever be painfree again has paid a toll on my mind. And it's probably a good thing that you can't just turn a switch and go back to the way things were before. I've heard many people say, illness has made them a better person..... I wouldn't claim that about me, but surely there must be something to learn from all this. Anyhow (you can tell I get a bit philosophical about it😂), for now I'll just take it day by day and let it sink in.

I'm super thankful that finally something is working, but I also know in the back of my mind that this is likely not the end of the story. Just because a drug is controlling an illness doesn't mean it's gone, so I'll have to deal with my neuropathy in one or the other way in the future again.....but it's not today's problem and it's certainly good to know that there are drugs that can control it!

It makes me wonder....

It's definitely not a random coincidence that I'm doing better right now. I had to push really hard to get daratumumab treatment. But then again, there are so many factors that are out of my control, and it just kind of amazes me, how coincidental life can be. So to wrap this post up; here are a few deep philosophical thoughts about life from my side...don't worry, I won't go on about it for too long, as I'm definitely better at scientific writing.🙃

It just makes me realize that there are always some things that are in your hands and you need to fight for, and then again other things just happen and somethimes you just get lucky and sometimes you don't. In my case I keep thinking, it's not a given... 

• that I´ve been given the chance to try daratumumab. I talked to many people with small fiber neuropathy in other countries who have been fighting for therapy for years and have still not been treated properly. Some of them are still in pain, some of them paid for drugs out of pocket and used up all their savings. I even talked to a girl from the US who is contemplating moving to Switzerland, because her insurance won't pay for immunoglobulins. Her entire body went numb because of small fiber neuropathy and she could actually show in blood work that she has an autoimmune reaction going on. I got very lucky in my case, because so far, I've always gotten access to treatment. I hope that publishing my case will enable some other people to get proper treatment too.

• to have a lovely friend, who is a smart hematologist, have the brilliant idea to try daratumumab for my small fiber neuropathy. What a coincidence is it, that our paths have crossed and that we decided that we need to be friends.💕 Isn't it crazy, that after all the drugs I've tried, the one that was suggested by my friend and has never reportedly been used to treat small fiber neuropathy is the only one that is finally doing a good job???!! Neither Prof. Wexler nor Prof. Oaklander had heard of this daratumumab when I first mentioned it. What do we learn from this? 

1. you need the right friends in life
2. you need to keep pushing and nagging and do your own research
3. friends are the best for many reasons 
4. girls rule the world😂😂 

• to have a neurologist (Prof. Wexler) who instantly said that trying daratumumab makes a lot of sense and went along with the approval process for this treatment trial. I've talked to many people with similar diseases who haven't found a nice doc yet who is specialized in their disease, or they have super traditional doctors who would never be willing to try something new. Many patients are gaslighted or not taken seriously, and I can't imagine what additional trouble they are going through.

• most importantly, to have someone like Steve, who has been driving me from and back of every single treatment, vaccination, and doctors appointment in Lucerne every week. Obviously, chronic diseases like these are also very tough to deal with for a partner. I know it worried/still worries Steve a lot, but he has been such a big support for me.💕With every situation that came up and with every attack of despair on my side he just tried to find a practical solution for it, he listened to me, and distracted me by taking me to places where I didn't have to walk much. I honestly don't know how I would have managed the past year without Steve, mentally and physically, so I'm very lucky to have Steve (now I'm getting sentimental).💕 

• So let's hope my neuropathy is giving us both a break for a while now.🌺🤞🏼🪵🤛🏻

And on this note, I wish you all a great Sunday. Thank you all for your support and for your interest in my blog! 🌺

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When my neuropathy came back...

My second neuropathy flare after almost 4 years in remission 

Hi everyone, good to see you back here! 🌺 

In this post I'll write some more of my story with autoimmune small fiber neuropathy. I wrote in one of my last posts, that prednisone had gotten my small fiber neuropathy into remission back in 2014. For months after this, I was worried that my symptoms would come back any second. Particularly, whenever I had a cold, because this is what triggered my neuropathy in the first place. 

But time passed and nothing happened. And as the years went by I became more and more confident that maybe this was just a one time reaction to a virus. After all, there were quite a few reports out there of patients with a so called 'monophasic' flare of post-viral small fiber neuropathy, which disappeared after some sort of immunotherapy. 

So I went about my life normally again, and I applied for a scholarship to do a postdoctoral fellowship in Boston. I've always wanted to live and work abroad for a while, and after experiencing how quickly things can become physically impossible, I figured I better not wait too long. 

Turns out I got the scholarship. 🌺 The only downside to this was that Steve and I had originally planned to go to Boston together, but then he got a new job which he liked in Switzerland in early 2016, so we decided that it was better if he stays in Switzerland and we'd both go a bit forth and back. Steve managed to get some prolonged unpaid holidays and I was quite flexible to spend time in Switzerland as well, as I was paid by my own scholarship anyway.  

Flare two started out of the blue while I was in Boston

So in April 2016, I moved to Boston for a 2-year postdoc at a research group at the Harvard Medical School, which is affiliated with some large hospitals in the area. I spent 2016 and 2017 there and I really loved the city. Steve spent both summers there with me and we traveled up to Maine, New Hampshire and Vermont and discovered the area. 

In fall 2017, my time there came to an end and I planned to move back to Switzerland in early December 2017. In mid October, I caught a cold and was feeling a bit under for a few days, but it wasn't anything major and I didn't think much of it. About 4 days into my cold I went and got the flu shot, since I was feeling fine enough and figured I better get that done. 

About a week after my cold, a tingle in my foot started

But then about a week after my cold and about 2 or 3 days after my flu shot, I noticed this subtle tingle and odd feeling in my left forefoot. It was really nothing much at that time but somehow I instantly had a bad gut feeling and it kind of stressed me out. 

I'd had several moments when I had gotten stressed out about some odd sensations over the past years, but usually the symptoms dissolved and I was able to relax again. So I  told myself that surely this is just me stressing and over-focusing, and that this would just pass. 

But somehow I couldn't get over it. This tingle and odd feeling lasted for about 4 days. It was a nice long fall weekend in New England and about 4 days into these symptoms I went on a trip to New Hampshire for a hike to see the nice foliage during Indian summer with friends. 

This is us sitting on top of the hill we climbed looking over the beautiful scenery. We walked up and downhill on hiking trails for about 3 hours, and although I could feel this odd tingle in my foot I wasn't actually in pain or anything. It just kind of felt like something wasn't right.

Symptoms were a bit stronger after the hike

When I arrived back at my apartment and took off my shoes, I felt that the tingle in my foot had started to get stronger and I started to really freak out. I hadn't mentioned my worries to anyone because I tried to tell myself that it was absolutely nothing. But as I sat in my apartment by myself, I realized that something probably wasn't right and I called Steve, who was back in Switzerland at that time. I told him that I was freaking out because I was worried that my neuropathy was about to come back.

Obviously that then freaked Steve out, because we both did not expect this and obviously 2014 was also kind of traumatizing for him. Given that he was back home, there was really not that much he could do at this point, so he recommended I just take a sleeping pill (luckily I had some of those to deal with an occasional jet lag) and go to bed. I think we just both hoped I would wake up and it was just a bad scare. So that's what I did: I went and passed out. 

I woke up in pain and full body discomfort

But the moment I woke up the next morning I knew I had a problem. My feet were tingly and sore and my hands felt like they had this electric layer all over them. On top of that my nose was really itchy and basically my whole body felt itchy. Within a day, all my symptoms I'd had in 2014 were back full blown. 😩😩😩😩😩😩

• My feet were sore and tingly and putting on shoes was painful
• The bottoms of my feet felt like I was walking on gravel
• My fingers were sore and felt like they had an electric layer on them
• My nose was itchy and felt as though I had to sneeze
• My arms and legs just felt itchy all over and scratching didn't help at all
• My feet would turn red (erythromelalgia) whenever I walked or was a bit warm, like you see in the picture below. This is literally after walking for a couple of minutes and its really sore.

I had never been to a doctor in the US

It's one thing to have neuropathy coming back, but it's a whole other story to have this happening while living abroad by yourself with travel health insurance only. I had kept my main health insurance in Switzerland and planned to just go to the doctor whenever I was there. So obviously I had never been to a doc in the US. Had I been back home in Switzerland, I would have called Dr. Soland and could probably have seen him the next day to figure out how to go about this. 

For a second, I literally contemplated to book a flight and to just fly back the same day before it got worse. But then I figured that this would just get super complicated, as I was going to move back home in 6 weeks anyway and would have to clear my apartment etc.

After all, I sort of knew what to do; I had to start prednisone treatment as soon as possible and hopefully symptoms would resolve soon. Being a pharmacist, I actually carried a load of prednisone with me wherever I went anyway since my first flare in 2014.  

Plus, I was in THE medicine city. Boston is probably the city with the highest density of medical specialists in the world, and I was working at one of the best hospitals in the world. So if anywhere, this was the place where I could get really good care by specialists. I just had to make an extra effort to find my way around the system in a moment when I already felt super beat down. 

I knew one of the neuropathy experts worked at my hospital

During my first flare in 2014, I had to do a lot of literature research to find out what I had. During this literature search, I came across some publications by Prof. Maryanne Walker (not her real name), who is one of the few neurologists who is specialized in autoimmune small fiber neuropathy. 

Coincidence had it, that I actually worked at the same hospital as Prof. Walker, so I thought surely it must be possible for me to see her. BUT.....I underestimated American bureaucracy.....🤯🤯 If you think Switzerland is a bureaucratic country, let me tell you, the Americans have taken bureaucracy to the next level.😂 

Naively, I just called Prof. Walker's office and asked if it was possible to see her. There was a lady on the phone who told me that generally Prof. Walker was not accepting new patients and that if I was an emergency, I'd have to get referred by my primary care physician.

I had to find a primary care physician

Fair enough, it's standard in Switzerland as well that you have to get referred to a specialist by your primary care physician, but the problem was that I had no primary care physician and turns out it's almost impossible to find one. 

I asked some of my work friends and called their primary care clinics to see if I could come in, but all of them told me that they didn't accept new patients, or that I could get an appointment in 3 months or something.  

After a day of research, I found a walk in primary care clinic, which is run by one of the large hospitals. This clinic was downtown and it was a lot more fancy than any primary care clinic I had ever seen in Switzerland. I was brought into my own shiny room where I even had my own huge TV to entertain me while I waited.

After a while a nurse practitioner came in and asked me why I was there. So I told her about my symptoms and my history of post-viral small fiber neuropathy. She did some very basic neuro exams, and like always could find nothing wrong. She had obviously never heard of my illness, so I asked her to prescribe prednisone and to refer me to Prof. Walker, which she did. 

I never actually saw a physician during this visit but later got a bill of about 400 USD for a 10 minute consultation and a prescription....oh well🙃

I was told that my referral should be through within 2-3 days and that I will get a call by the hospital. I was explicitly told I did not have to call and that I will be contacted at the latest within a week. But experience taught me that these things tend to not work as promised, so I was ready to stalk the c..... out of this hospital until they give me an appointment....and let me tell you.....it was A HASSLE but I managed.👻 

Alright, this post hast gotten kind of long already, so I will continue next time. Spoiler alert: I did actually manage to see Prof. Walker and in hindsight I'm very thankful for the fact that I did stay and fight through the bureaucracy jungle. 

And meanwhile I wish you all a good start into the week. Thank you all for reading my blog. 🌺 

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Daratumumab - 3 week update

3 weeks into daratumumab therapy 

Hi everyone, good to see you back here! 🌺 

It's been 3 weeks since I've started daratumumab (Darzalex) as an off-label treatment trial for my autoimmune small fiber neuropathy, so I thought I'd give you an update. 

In short, I can tell you that I still don't know a whole lot more. Some symptoms have definitely changed but my neuropathy is far from gone. I'm still having trouble figuring out what's going on and making sense of my symptoms / change of symptoms. But I'll try and summarize what's happened so far. Maybe that'll help me making some sense of it all myself as well. 

The main problem is that nobody can tell me what to expect, so anything that happens throws my mind off onto a wonder..... First, I don't know what exactly is going on in my body, so I don't know if daratumumab is going to work at all.  Second, nobody has been able to tell me how long it would take until I could feel a change and how it feels when small fiber neuropathy heals. My small fiber neuropathy has been poorly controlled for about a year now, so there would be quite some damage in my small nerve fibers by now. The good thing about small nerve fibers is that they grow throughout our lives, but I have no idea how long it would take for them to regrow, how severe the damage is, and how it would feel when they repair themselves. 

The longest I've ever gone without proper treatment was for 3 months before I started my prednisone treatment in 2014. Back then my symptoms were restricted to my feet and hands. Back then it took two to three months until symptoms were gone, so I definitely don't expected an immediate resolution of symptoms. But because my neuropathy has spread since then I also don't expect it to be the same as back then.

So let me try and summarize what I've noticed so far.

Positive changes: 

• The skin on my hands and feet seems less dry and more smooth. For the first three days into therapy my hands felt a lot more dry than usually and then about a week into therapy I noticed that it got better. Below is a pic of my hands on a day last fall when they were really dry. They're not always that peely, but since my neuropathy got out of control they have felt a lot more dry and scratchy than they usually do, and once in a while they would get peely like this. I will spare you a pic of my feet, but I can tell you that they get very dry too.🤨People with small fiber neuropathy may have increased or decreased sweating, because the neuropathy causes sweat glands to malfunction and they may die off too. In my case I think that I have reduced sweating, which causes the dry skin. During my autonomic function testing back in 2014, the doc did notice reduced sweat response in my most sore foot. 

• My fingers feel less sore. When I'm typing on my computer, I always wear a tennis sweat band around my wrists, because the edge of my notebook irritates the bottom of my hands and wrists. It's a weird hypersensitive electric kind of feeling. About one and a half weeks ago, I noticed that I can work quite a bit without waearing the sweat bands. 

• The strength in my pinky is back. This also happened about a week into therapy. My pinky gets weak and clumsy from my small fiber neuropathy. My neurologist has previously told me that he also noticed reduced strength in my toes, but I don't really notice this myself. I usually notice that my pinky is weak for instance when I rub face cream onto my face and I notice that my pinky kind of drags behind. About a week into therapy I put on face cream in the morning and noticed that my pinky was functioning properly when I put on face cream, just from one day to the next. During the first week it felt a bit more weak and then suddenly it was gone.  

• I reduced the dose of pregabalin/Lyrica by 50mg without worsening of burning pain on my legs. I started pregabalin / Lyrica about 2 months ago, because I developed more severe burning pain in my legs and feet. Overall I don't have much of the burning pain in my legs at the moment. I'm currently on 175mg / day. 

• Reduced redness on my legs after showering: my legs get these weird red spots in the area around my knees after showering. I think this is a form of erythromelalgia, which I get in my hands and feet when I get hot. About a week ago I noticed that I have these spots a lot less, even after a hot shower. Below is a pic of the erythromelalgia I get around my knees after showering. It's not always that bad, sometimes it's also just a few red spots, but I thought I'd pick a good photo for your🙃
  
  
  

Negative changes: 

• The most prominent negative change is that I've developed a patch of pretty severe neuropathic pain on my lower right arm. This developed about 4 days ago and is super weird: my lower right arm has been affected by small fiberneuropathy for about a year now, much more than my left arm actually. However, it always felt more like a dull ache and mild weird sensations for instance when it touched the desk while I was typing. It never actually hurt when clothes touched it or anything. For the past four days my arm really hurts from any kind of touch, even if I just slide my finger across the skin. I also have to pay attention that clothes don't rub against it because that is very irritating. It's a clearly defined area, which is most severe around the bone of my wrist and runs down the top part of my lower arm. I drew it onto my arm in the pic below, so that I can remember where it is. It's not the end of the world, but it's just so odd, because I've never had this before and of course this sends my brain 🧠 on a wonder what this means. Does it mean that nerves are growing back and they are over-reacting?? (🪵🪵🤛🏻🤛🏻) or is my neuropathy getting worse??? Does this happen because I over-focus on my pain?? Or is it just coincidence???🤯🤯

• Over the past three days the skin in other areas of my body has been hypersensitive. Most prominently the skin on my back and torso feels sore. It's not too bad, it feels like a dull ache when I lean against my back on the sofa or when I touch it otherwise. It also feels hypersensitive when I put on clothes for instance.....

• I developed an itch in my upper nose about 10 days ago, which feels as if I had to sneeze. I've had this itch during previous neuropathy flares and it's really annoying, but it had actually been gone for the past half year. 

• Generally I've been more itchy in my hands and feet and less intensely in various spots all over my body. 

So, what do all these symptoms mean? WHO KNOWS!!?? 

How long will they last? WHO KNOWS!!??

I know that a few others with small fiber neuropathy read this blog. So if any of you has made a similar experience or has any other advise please do reach out!  

Bottom line is, I need to be patient, and I'm really not good at that anymore. I'll try to take it day by day and not overthink things, but that is kind of a brutal mind game at the moment 🤯🤯 because it's impossible not to overthink for me at the moment. Either way, I'll find out and I'll keep you all posted as always. Thank you all for keeping your fingers crossed for me.🤞🏼🌺

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SARM1 inhibitors: a cure on its way?

SARM1 inhibitors - a future cure for small-fiber neuropathy? 

Hi everyone, good to see you back here! 🌺 

Today's post is for my fellow pharma-nerds, treatment optimists, and for everyone who is interested in drug development. 

I'll write about a drug class called SARM1 inhibitors, which are in early drug development, and which could potentially treat autoimmune small-fiber neuropathy and many other neurodegenerative diseases in the future. 

I like reading about such drugs in development, because it makes me optimistic that in the future there will be much better treatment options for many patients with rare nerve diseases. It's amazing how much we can fix, as long as there are enough money and intelligent minds going into it. 🌺  

Disarm Therapeutics

The company, which is working on these drugs is called Disarm Therapeutics, a spin-off from the Washington University in St. Louis, Missouri, USA. The founders of the company are researchers, who have worked in academia for decades, during which time they discovered the molecule called SARM1 and its functions. 

I think they are really onto something!✨ 

SARM1 inhibitors are a completely novel therapeutic approach, which may potentially treat a huge range of devastating neurological diseases. 

What does SARM1 do?

The molecule SARM1 is present in all our nerves and functions like a switch, which controls whether or not a very important part of our nerve fibers (called the axon) degenerates/dies off. Axonal degeneration is the main driver of many neurological diseases. But let's take a step back...

What is an axon, and what is axonal degeneration?

I'm not a neuroscientist, so if you find that I write something incorrectly, please let me know and I'll fix it. Basically, we all have nerves running through our bodies, which originate in our brain (called the central nervous system, CNS), and run through the spinal cord, and then out into the periphery (peripheral nervous system, PNS) all the way into our toes and fingers. 

The peripheral nerves are roughly divided into large peripheral nerve fibers, which control motor functions, and into the small nerve fibers, which control pain, vascular function and other autonomic (unvoluntary) functions in our bodies. 

• Nerve fibers are long strands of nerve cells, also called neurons. 
• Neurons transport electric stimuli through our bodies. 
• If you touch something with your finger, this triggers an electric stimuli in a neuron, which then travels up to your brain so you can feel it.
• Axons are part of every neuron. They have the job to transmit information from one neuron to another, or from a neuron to muscles or glands. 
• Axons can be up to meters long. 
• Axonal degeneration means that axons die off.
• When axons die, neurons can't function.
• Depending on which type of neuron is affected (CNS, PNS, large fibers, small fibers etc), patients have different diseases.
• In small fiber neuropathy, axons of the small peripheral nerve fibers are affected. 

Many diseases are caused by axonal degeneration 

Axonal degeneration in the CNS:

• Amyelotrophic lateral scrlerosis (ALS)
• Altzheimer's disease
• Multiple sclerosis (MS) 
• Parkinson's disease

Axonal degeneration in the periphery:

• Diabetic neuropathy
• Chemotherapy induced neuropathy
• Small-fiber neuropathy
• Traumatic nerve injuries 
• Glaucoma 
• Genetic forms of neuropathy
• and many more.......

All these diseases have different causes, like in my case an autoimmune reaction. For some of them we have no idea what the actual cause is. But they all have the following in common - that something induces the axons in specific neurons to die off, which causes detrimental symptoms. This is why these diseases are called neurodegenerative diseases. 

SARM1 controls whether or not an axon dies or lives

Researchers have observed, that in many neurodegenerative diseases, axons die off much earlier than the rest of the neuron. They suspected that there must be a specific mechanism, which triggers this. However, researchers have tried and failed to identify what causes this axonal degeneration for decades.

During their academic careers, the founders of Disarm Therapeutics have identified SARM1, a molecule, which seems to trigger axonal degeneration once it gets activated. Here is a link to an scientific review on SARM1 inhibitors.

We all have SARM1 in our neurons, and it is usually kept inactive by our bodies. However, SARM1 gets activated by different pathological stimuli, and from what I read it's not entirely understood how. However, it was observed, that once it's activated it causes a cascade of reactions, starting with calcium influx into the cells, which eventually leads axons to die off. This process is called Wallerian degeneration (don't worry I've never heard about that before either 🧠🧠).

So those researchers figured that if you could inhibit SARM1 from activation, you may prevent this Wallerian degeneration and thus you could prevent axons from dying off. They developed a method to produce many different molecules, which may inhibit SARM1 from activation. 

Where do SARM1 inhibitors stand in the development?

It is still very early days in the drug development process for SARM1 inhibitors. They have developed a bunch of molecules, which do inhibit SARM1 in lab tests. First pre-clinical (so not in humans) studies on cell lines and mice are looking promising. They were able to show that their drug candidates do prevent axons from degenerating when exposed to chemotherapy.

Of course it is still a very long way; only 1 in 10 drugs that are ever tested in humans eventually make it onto the market. But these ones just sound so fascinating, and I think given that they are developing a whole range of molecules they want to test, they have a valid chance. Big pharma has recognized their potential, and Eli Lilly bought them in fall 2020 for 135 million US Dollars. 

We need a biomarker to measure axonal degeneration

Until today, there is no easy routine lab test that you can do, to test for axonal degeneration. However, it's impossible to develop a drug to fix axonal degeneration, without a means to measure if it works. So these researchers are about to fix this problem as well. 

They found that whenever axons die off, they release a molecule called neurofilament light chain (NFL) into the blood stream. Neurofilaments are parts of the neuronal cytoskeleton, and are especially abundant in axons. The problem was though, that concentrations of this molecule are so low in blood that it was really hard to pick it up in a blood test.  

However, they have now developed a test (a SIMOA assay), which can pick up tiny concentrations of NFL in the blood. And they were able to show that measured concentrations correlate with disease severity and prognosis in case of multiple sclerosis.

I'd love to be able to take this test

Unfortunately this test is not available routinely yet, because I'd really like to do it. It's suspected that in small fiber neuropathy, the axons of the small nerve fibers die off. But in reality nobody really knows what is going on. 

At the moment I'm undergoing this new daratumumab therapy. The only way to see whether it works or not, is for me paying attention to my symptoms. And like I told you before - that can be very stressful for by dear friend BRAIN. 🧠🧠🧠🧠

With this test, I'd be able to see whether there is more/less NFL in the blood before / after treatment, which would show if the treatment is successful much before I'd notice a change in symptoms. I'm sure some time in the future this test will be a lot cheaper and available as a routine test, because it just makes so much sense to me. I think its a genius invention.

So let's keep our fingers crossed (🤞🏼🤞🏼) that some SARM1 inhibitors will roll through the drug development process smoothly. And that they will become available as powerful weapons in the fight against disabling neurological diseases in the semi-near future. 

Now I wish you all a great weekend and hope this post was not too technical. After all, it's your fault for reading to the end, in case I bored the c* out of you.😂

In the next post I will give you an update on my daratumumab treatment again. 🌺 

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Daratumumab therapy started

My daratumumab therapy started 

Hi everyone, good to see you back here! 🌺 

Last Tuesday, my daratumumab therapy started, so I thought I'd give you a quick update. Actually, there is not much to report yet anyway, but I'll just tell you how the first infusion went.... 

I spent all day at the hospital on Tuesday 

On Tuesday early morning, Steve drove me to the hospital in Lucerne and I went to the hematology ward where I get my treatment. I'm usually treated at the neurology ward, but nobody there knew daratumumab, so we decided to do the therapy at hematology, where they are very familiar with the drug.✨  

The hematology ward is in a brand new part of the building and everything is modern and nice looking. I first had to go for a blood draw and to get this nice little iv access put in place. Daratumumab is actually incjected under the skin, so I didn't need the access for this,💉 but the docs need it in case you have any adverse reaction and they need to give you drugs quickly. 

First I got some drugs to prevent infusion reactions

After that I was sent to my infusion room. I had a nice room to my self almost all day and I was sitting by a big window. An hour before I got the actual daratumumab injection, I got some drugs to reduce the risk of an allergic infusion reaction. 

They gave me 20mg dexamethasone, which is a pretty high dose of a drug similar to cortisone/prednisone. Dexamethasone is also given to people with severe COVID, but that just as a side note. I also got an iv antihistamin called clemastin, an asthma medication called montelukast, as well as some paracetamol. 

Then I had to sit there for an hour to let those drugs do their job. After about half an hour I suddenly felt super tired. I already expecte that, because it's a typical reaction to the iv antihistamines, and I have had them before with the same reaction. 

I made sure I brought my iPad so I could watch some light-weight Netflix shows. I also don't really mind being tired and dosy on a day when you just have to sit in a reclining chair with a drip in your arm anyway. 

Then daratumumab was injected into my belly

Daratumumab is given as a subcutaneous (SC) injection. It can also be given as an infusion into the vein, but it's easier and better tolerated if you get it subcutaneously. In the pitcure below you can see what a SC injection is. Basically, the drug is injected into the fatty tissue under your skin, typically into your belly or an upper leg or so. 

Many drugs are given as SC injections, such as insulin for diabetes.💉 Usually it's  very small amounts of liquid that are injected over a time period of a few seconds. 

In case of daratumumab, it's actually a pretty big syringe of 5 ml. The nurse put the needle into my belly and then sat there for about 5 minutes slowly pushing in the liquid. There was another nurse there too, who was being trained on the ward, so the two of them tried to distract me a bit and asked me all sort of questions about my neuropathy. 

We had a chat about my illness, and obviously they both had never heard of it too, but they were interested. It looked a bit strange having this syringe stuck in my belly for 5 minutes,💉 but the injection itself doesn't actually hurt. So if you ever need daratumumab, I can tell you - the injections are totally managable. I'll try to take a picture of the syringe and/or the injection next time.

After the injection I basically just had to sit there for 6 hours, and every 15 minutes or so a nurse came in to measure my temperature and blood pressure to make sure I didn't have an allergic infusion reaction. Thankfully I didn't.🙏🏻 

This was my companion for the day, following me everywhere. 

When I left the hospital I noticed some side effects

Around 5 pm I was ready to go and Steve was waiting with the car. As I walked through the hospital I noticed that my vision was super blurry and I had a hard time finding my way around.👀👀

The nurse had already warned me that this is a frequent side effect of the antihistamine, but I was actually fine all day. I also don't recall having had this the last time I got these drugs - but as always you have to stay flexible, right? 🙃

After we got home we ordered some food and were watching 'Home and Away' on TV. Home and Away is an Aussie soap opera that we like to watch. I told you before, Steve is Aussie, and a few years ago he found this soap on one of our TV channels and started watching it for some homey feeling and beach views.😃And now he got me totally addicted to it too. 🌺

Luckily this soap is totally predictable, because I sat there and actually couldn't even recognize who was on screen at all. I could see sharp within a radius of about 10cm, but anything in further distance was just one big blur. I was still really tired from the drugs anyway💊, so i just went to bed, ready to wake up with vision in the morning.

But turns out I was basically blind for almost two days 

When I woke up the next morning I could still see absolutely nothing.😩 In the search for some entertainment, I started organising my blog, holding the screen of my notebook about 10cm in front of my face. 

This was probably not the smartest thing to do, because my eyes didn't seem to recover but actually got worse. So in the afternoon I even had trouble reading my phone and I parked myself on the lounge and listened to some podcasts for the rest of the day. FINALLY, towards the end of the day I noticed that objects started to have contours again. And on thursday when I woke up my vision was back👀🙏🏻

How am I doing now?

I didn't have any other side effects from daratumumab so far, except for a slight bruise on my belly and a very mild headache occasionally.🤞🏼🌺 Compared to the IVIG (immunoglobulin) infusions I've had previously, it's been a walk in the park so far. IVIG infusions have actually traumatized me a little, because they've regularly put me to bed for days and weeks with the worst headache ever and pretty severe nausea. I'll write about these infusions another time.  

How is the neuropathy going?

That's a difficult question 🙃The tricky thing is, that daratumumab has neven been used to treat small-fiber neuropathy, or at least not that I know of. So nobody can tell me what to expect at all. My neuro keeps telling me that he finds it very interesting. 👻

I appreciate that he finds it interesting and stays tuned to my treatment, but everytime he says this it just hammers in the fact that there is nobody outthere knowing what will happen. But that's just how it is and I've been dealing with this for a while now.... 

I try to tell my brain not to analyze every single symptom all the time, but obviously BRAIN🧠🧠 has it's own agenda and it likes to over-analyze.🧠🧠🧠 So far, BRAIN has not come to a conclusion yet though. 

For the first two days I didn't notice any difference at all. Then, from Thursday on I felt like my symptoms got a bit more intense. But then again this could just be because BRAIN likes to focus on them. Realistically, I will not see an immediate effect anyway. 

With autoimmune small-fiber neuropathy, some immune cells distroy the neurons of the small nerve fibers. So even if you stop this immune reaction, the nerve fibers would need some time to recover. And the more destroyed they have been the longer it will take for them to recover. The one good thing about small nerve fibers is that they actually regrow throughout your life. This is not the case for large nerve fibers. 

When I had prednisone treatment, it took about 3 weeks until I could definitely notice a difference - so this is kind of my reference for now as well. So I guess I'll just have to wait and hope (🤞🏼🤞🏼) that it will do something. You'd think that these infusions sound unpleasant, but for me the wait after is a lot worse than going to get some infusions....🌺

Either way, I'll keep you posted on how it's going (🤛🏻🪵🪵) and hope you have a good Sunday!

BTW: For those who subscribed to my blog, you probably noticed that the email design has changed. This is actually what I did semi-blind on my computer on Wednesday morning, so I hope you appreciate it.😂 Many of you told me that my old emails looked like spam, and I totally agree. I think the new ones look a lot better - so 🤞🏼they will work as they should.🌺

For those of you who have not subscribed yet and would like to experience my excellent email design as well - feel free to subscribe below! 🌺

I've you've missed my previous posts and want to know what daratumumab is and why I get it: here are the links to read up. 

New treatment option?

https://mysmallfiberneuropathy.blogspot.com/2021/02/new-treatment-option.html

Insurance didn't approve daratumumab 

https://mysmallfiberneuropathy.blogspot.com/2021/03/application-daratumumab.html

I will try daratumumab

https://mysmallfiberneuropathy.blogspot.com/2021/03/daratumumab-for-small-fiber-neuropathy.html

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