IVIG for autoimmune small-fiber neuropathy

My experience with Intravenous Immunoglobulins (IVIG)

Hi everyone, good to see you back here! 🌺

Many people have asked me if I had tried IVIG (intravenous immunoglobulins) or SCIG (subcutaneous immunoglobulins) to treat my autoimmune small-fiber neuropathy. 

Yes, I had IVIG and SCIG for quite some time 

It worked very well for my neuropathy, but it was quite a ride 

Around February 2019, my autoimmune small-fiber neuropathy had been in remission for 3 months, after my second acute onset episode had been broughtunder control. Both prior episodes started after a viral upper respiratory tract infection, and both went into remission with high-dose oral prednisone. 

However, to control my second episode, I had to take prednisone for almost a year (vs 3 months for episode 1) and taper it very slowly. Symptoms had come back several times when I tried to reduce it faster (I'll write about this another time). So here I was, happy that this resistant episode of SFN was finally controlled. 

But once again, I caught a respiratory tract infection, and 10 days later the exact same neuropathy symptoms started abruptly. I had a few viral infections in between as well, which didn't cause any symptoms, so I don't know what was different about this one. I must react to some specific virus, but I don't know which one it is. First, i developed pain and erythromelalgia in my feet and couldn't tolerate shoes, and within a day my hands got sore too and my nose started to itch. 

Obviously, I was very disheartened by this set-back. I still had prednisone at home and immediately took 50mg to stop my syptoms from getting worse. However,  I knew that taking prednisone for months and months again was not an option without harming my body. 

I needed a treatment that was not predisone and that would not destroy my body long-term

When episode 2 started, I got treated by Prof. Maryanne Walker in Boston (not her real name) where I was living at the time. Back then, she prescribed prednisone, but she also told me that if my small-fiber neuropathy came back she recommends getting IVIG. She also advised me to get in touch with Prof. Wexler in Switzerland (not his real name). He is THE NEUROLOGIST for small-fiber neuropathy in the German speaking part of Switzerland. Apparently, I had to go all the way to Boston to find out about that......

So I got in touch with Prof. Wexler's clinic....

...and was told, that the next available appointment was in 4 months. Exactly what you want to hear when you are in a desperate medical situation. So, I got in contact with Dr. Soland, my local neurologist who is an excellent neurologist and who had seen me through my prior two episodes of SFN. He knew Prof. Wexler and referred me urgently, but we both expected a long wait. 

Much to my surprise, I got a phone call from Prof. Wexler's clinic the next day. Apparently, someone had cancelled their appointment and they asked if I was able to come in the next day. Of course, I dropped everything to make this appointment happen. 

I went to Lucerne and met Prof. Wexler 

I also brought my discharge letters by Prof. Maryanne Walker, whom he knew from different research meetings, in which she wrote that she recommends IVIG if my neuropathy ever needed longer-term treatment.  

Prof. Wexler agreed that IVIG was the best treatment choice

However, he warned me that many health insurances refused to pay this treatment for patients with small-fiber neuroapthy, because obviously it is not very well researched, like everything other possible treatment for SFN. 

The difference between prednisone and IVIG is just that you can get a month worth of prednisoen for around 20 Swiss Francs (20 USD), whereas IVIG treatment costs around 40'000 Swiss Francs or more (approx 40'000 USD) per year in Switzerland. 

IVIG are immunoglobulins (aka antibodies), extracted from donated blood

They have originally been used to boost the immune system of people with antibody deficiencies. Over the years, it has become apparent, that high doses of immunoglobulins can control antibody-mediated autoimmune diseases. The way how IVIG works in autoimmune diseases is not fully understood. 

It is assumed that if you infuse heaps of healthy antibodies into the blood stream, they sort of outcrowd the bad ones (i.e. the auto-antibodies), which also speeds up their excretion. But other mechanisms of action are discussed as well.  

Prof. Wexler wrote a letter to my health insurance to apply for IVIG treatment, and we both prepared for a long back and forth fighting my health insurance. Much to our surprise though, they didn't argue at all. They aproved my treatment within a matter of days. I know, I got very lucky there!

I started IVIG treatment about a week after my symptoms started

Everyone assured me that IVIG caused basically no side effect

...and I believed them. I had to go in three days in a row to get a total loading dose of 1.5g/kg body weight of Privigen®. I was still on 50mg prednisone, which had already controlled most symptoms, and which I was going to take for another week after starting IVIG. 

So I sat in the infusion room of the neurology ward hooked up to a needle and an infusion stand for several hours per day. The staff was very nice, and it was also kind of 'nice' to see other people in a similar situation (although of course I don't wish this on anyone). The infusions went smoothly and I left the clinic optimistically on day 3. I was symptom-free, though I didn't know if this was due to prednisone or if IVIG was already doing anything. I did however notice that my feet started to be more tinlgy in the evening after the second infusion. 

In the evening after the third infusion, I developed a very bad headache...

I had been told that IVIG could cause headaches, but I thought that a little headache would be be nothing to worry about if the treatment helped. And sure enough, noone told me how bad such a headache could get. So I figured I'd just sleep it off, and went to bed hoping I would wake up fresh again in the morning. 

But the headache got worse and worse by the hour....

Sleep was 100% not an option, and by midnight I had a 9/10 headache. I was in pain all the way down my neck and spine right to my tail bone. I couldn't move and could only lie in one sideways position. On top of that I was super nausious. I took all the pain and nausea medication I could find in my well-stocked drug cabinet (I am a pharmacist after all), but even touched the pain. 

Towards the morning the pain easeed up a bit and I finally fell asleep

When I woke up a few hours later, the headache was almost gone. Now that I was functional again, I started to do some online research and found that these types of delayed headaches were actually quite common with IVIG. It also appears that they seem to be more common in patients with autoimmune problems. It is assumed that IVIG triggers some sort of mast cell attack on the meninges, i.e. an aspeptic (non-infectious) meningitis. That made a lot of sense to me, because what I had was definitely not just a headache....

I got in touch with Prof. Wexler and told him about my horrific headache experience. He agreed that I likely had a 'mild' meningitis. If this was mild, I don't want to find out how severe meningitis feels like.... He said that they will reduce infusion speed the next time, which should make it better. Also, I was only going to get 1g/kg per month from now on. Lower doses should also cause less headache. So I was optimistic that we would be able to control this. 

One week later, I tapered prednisone and the neuropathy remained in remission

I was very happy that IVIG seemed to do a great job. My next infusion was planned for 4 weeks after the last infusion. 

However, 3 weeks after the last IVIG infusion, my small-fiber symptoms come back from one day to the next

I told Prof. Wexler about this when I went in for my next infusion and he said to see what happens after this infusion. He said that I likely experience so called 'end of cycle symptoms', where symptoms come back as the effect of IVIG wears off. 

And sure enough, symptoms did disappear again within 2 days after the next infusion. 

But the headache came back as well.🤯 Thanks to the lower dose and the slower infusion speed the headache was a bit less intense, but still intense enough to make me absolutely miserable. And worst of all, this time the pain lasted for almost 2 weeks.

On top of that, I developed an allergic whole body rash, which lasted about 10 days and was super itchy. But in light of the severe headache, the rash seemed like a very minor nuisance. 

So here I was, neuropathy-free but with a headache that was probably worse than having neuropathy. It felt like chosing between Sodom and Gomorrah. And as the headache eased up, the 'end of cycle neuropathy symptoms' started over. 

Initially, we had hoped that 1 or 2 IVIG infusions may put my small-fiber neuropathy into remission for good. But that was obviously not the case, and it seemed that I needed long-term treatment.

However, we were far away from a good treatment plan

Prof. Wexler was also surprised about the severity of my headaches, which made me pretty much non-functional in daily life. So with each round of infusions, we further reduced the dose, the interval between infusions, and the infusion speed. This did make my headaches less intense, but unfortunately, even less intense headaches were still severe enough to make me miserable.

We reduced the dose as much as possible

At some point, I sat in the infusion room for 8 hours straight every week to be infused with a small dose (15g or so) of IVIG. Nonetheless, I still had a 24/7 headache accompanied by nausea that didn't allow me to enjoy my life. 

Antihistamines helped to take the edge off the pain

Because it's assumed that this headache is due to a mast cell reaction towards the meninges, regular painkillers don't work, but antihistamines can help to reduce the pain. Interestingly, the newer antihistamines, which do not make you tired didn't work for me at all. I tried levocetirizine and fexofenadine and had absolutely no improvement. 

The antihistamine that worked for me was diphenhydramine. It's an old type of antihistamine, which makes you very tired, because it crosses the blood brain barrier. Given that the headache stems from an inflammation of the meninges, it makes sense to me that a drug that crosses the blood brain barrier works better. That is just my hypothesis though. 

In the US, diphenhydramine is frequently used as an anti-allergy drug and is sold under the brand name Bedadryl. However, in Switzerland, it is only sold as a sleeping pill under the brand name Benocten. At first, I felt pretty stupid going into the pharmacy and asking for sleeping pills several times. And of course, when I told them what I needed it for, they just looked at me with big eyes...I started to worry they would put a note into my patient profile about potential drug abuse. But Prof. Wexler gave me a prescription for it, which took care of this. 

But you can imagine, it's not ideal to take 'sleeping pills' during the day all the time. You might have an improved headache, but you also just have to go and take a nap. You do get used to them a bit, and after a while you don't get so tired from them, but nontheless, this was not an ideal long-term plan, as the headache was far from gone anyway. 

After 3 months of this torture we decided that IVIG wasn't for me...

...and applied to switch to subcutaneous immunoglobulins (SCIG)

SCIG is essentially the exact same thing as IVIG, you just administer it yourself at home and it goes under your skin instead of into your blood. And because you don't give it directly into your blood stream, it arrives in your body much slower and causes much less side effects, including headaches. 

The problem was just, that SCIG had only recently been licensed in Switzerland, and of course not for autoimmune small-fiber neuropathy. We didn't know if insurance would approve it, although the cost to them are pretty much the same or maybe even a bit less because they don't have to pay for hospital staff doing the infusion. 

Thankfully, health insurance agreed to this change and I was able to start SCIG. I will write a separate post about my SCIG journey next time.  

So all in all, yes IVIG worked very well to control my autoimmune small-fiber neuropathy. Symptoms were in complete remission for 3 weeks after each infusion. But IVIG wreaked havoc on my body. 

I think doctors just don't really worry about side effects that are not directly dangerous, i.e. that can't kill you. Even if that means you can't live your life. When I started my immunosuppression, doctors discussed the risk of infection with me over and over. However, this risk of infection has affected my life so much less than the torture I was inflicted on by IVIG. 

This is totally not to discourage anyone to try IVIG!!

I'm still glad I tried it, and I would do it again, because it led me to a treatment that worked and that I tolerated better. Also, almost everyone else I know, who tried or is getting IVIG tolerates it much better than me. I just got unlucky there. However, I feel like it's important to share this experience, becuase noone had warned me sufficiently of these potential side effects. 

If you plan to get IVIG, it might be better to start with a small dose at a very slow speed and see how you go. It also make sense to take a lot of antihistamines before and after the infusion and if you don't tolerate IVIG well maybe even some steroids (usually dexamethasone). 

This is a very helpful paper, which discusses exactly this problem, and which provides a raodmap on how to best start IVIG inpatients with autoimmune small-fiber neuropathy. They do also write, that in their experience, this type of headache is more common in young patients with autoimmune SFN. Make sure you bring this to your pre-IVIG meeting with your neurologist and make sure they have read it. Prof. Wexler totally agreed with this paper, and we adjusted my treatment according to it, which was successful in the end.

I will tell you more about my journey with SCIG (Hizentra (R)) in my next post. And until then I wish you a good weekend!

Thank you for your continued interest in my blog and for your support. 

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Pregnancy and small-fiber neuropathy

Pregnant with autoimmune small-fiber neuropathy 

Hi everyone, good to see you back here after a long time! 🌺

I've been absent for a veeeery long time. You might already guess why...  

we had a baby last October... 

In this post I'll let you know how my small-fiber neuropathy behaved during and after pregnancy. I hope this will be of help to many of you. I always wished I had more information on what to expect, before I was pregnant, but I couldn't find any information. Thankfully, I knew some women from social media who had been pregnant with small-fiber neuropathy.  

I had daratumumab in early January 2022

In my last post in January 2022, I reported how I restarted daratumumab. After two daratumumab infusions, I could quickly feel my neuropathy improving and I was almost symptom-free by the end of January. 

In early February, I found out I was pregnant

Having a baby had been on our minds for a while, so obviously I checked beforehand, whether any of the drugs I was taking could be problematic if an unborn baby was exposed during early pregnancy. My OB reassured me, that any type of immunoglobulins (including monoclonal antibodies like belimumab or daratumumab) do not cross over to the baby until week 13-15 of pregnancy at the earliest. 

I had also discussed with my OB, which treatment for my small-fiber neuropathy is possible during pregnancy. Drug treatments are always tricky during pregnancy. Generally, pregnant women are excluded from clinical trials, which is understandable from an ethical point of view, but it results in a complete lack of safety information for most drugs. 

We agreed that I would continue belimumab until week 12 and then stop any treatment because continued treatment could cause immunosuppression in baby later during pregnancy. This is not to say you absolutely can't have belimumab during pregnancy; belimumab is not super toxic to baby or anything and there are many reports where women took it throughout pregnancy. But it's better not to take it if you don't have to. Although not much is known, Prof. Wexler as well as Prof. Maryanne Walker from Boston reassured me, that the few SFN patients they had seen were doing fine during pregnancy with minimal to no treatment. 

Most autoimmune diseases are less active during pregnancy

During pregnancy, the immune system changes. It typically calms down in order not to reject the genetically 'half-foreign' baby. Thus, many autoimmune diseases like MS or rheumatoid arthritis often improve or even go into remission during pregnancy. This is also why you are more susceptible to infections during pregnancy. However, in the end you never know. Some auto-immune diseases, such as lupus, are also notorious for flaring up during pregnancy and might require intense treatment. 

Around week 5 of pregnancy I had an acute neuropathy flare 

One week after I found out I was pregnant, I was in pain all over from one day to the next; my feet and hands were very sore, my legs were burning, my face and neck were itchy..... and I was frustrated! I was just doing really well after the daratumumab infusions and was happy about our news. I was also worried, that if my immune system went crazy now, it might abort my pregnancy. But thankfully it didn't.... 

Symptoms gradually improved again... 

From about week 6 on I was so nauseous, which effectively distracted me from my neuropathy anyway.🙈 A few weeks later, after I had found a drug that controlled my nausea, my neuropathy symptoms had already improved quite a bit. I was still on belimumab at that point, so I'm not sure whether I improved thanks to belimumab or due to pregnancy hormones. However, by week 15 my neuropathy symptoms were pretty much gone, despite me stopping belimumab at week 12. So the hormones were definitely helping.

I know it was week 15, because we went for a 10 day vacation in Mallorca then and I was able to wear my hiking boots and go for hikes along the coast. Occasionally I could feel my feet getting a bit warm, but I had no other neuropathy symptoms. I was more starting to deal with pregnancy-related swollen feet, shortness of breath, and exhaustion, which prevented me from doing bigger hikes. 

Of course I also did my usual PubMed search...

...and I found an old study in which they measured overall IgG concentrations in pregnant women over time. IgG are the most common antibodies we have in our body (not sure on that one actually....), and Prof. Wexler suspects, that my neuropathy is driven by some unidentified aberrant type of IgG, which my body produced in response to a viral infection and which attacks my own nerve fibers. This study nicely showed an increase in IgG in early pregnancy, which then decreased over time until delivery. This might explain my symptom flare, but who knows in the end....surely it's more complex than that too.

Between week 14 and 30, my neuropathy was in complete remission without any treatment

It was such a nice break from the constant UPs and DOWNs with my SFN over the past years. Finally, we had something exciting and beautiful to focus on and the hunt for new medications and the constant negotiations with health insurance were on hold. 

I expected my small-fiber neuropathy to be quiet until delivery....

...but around week 30 I could suddenly feel the trigger spot on my left foot. Within a matter of 2 days I was in full neuropathy pain again without any obvious trigger. And I guess you can imagine what mood that left me in....

Steve had Covid the week after my symptoms came on

I'm still wondering whether I might have had asymptomatic Covid-19 before my symptoms started and passed it on to Steve. This could explain the onset of my neuropathy symptoms. However, I did test myself regularly from the moment Steve was ill, and I never had a positive (we did gear up with masks even in the house and all, given I was pregnant...). 

But I don't think I had Covid....

...even though it would make sense. I was immunocompromised AND pregnant, so out of everyone, you wouldn't expect an asymptomatic infection in my case. Plus I was super careful not to contract COVID all the time, and had basically not been in any risky situation in the time before. It's much more likely that Steve caught it at work and I was just lucky not to catch it from him. But after all you never know.

Most likely my small-fiber neuroapthy just came back randomly

I had read about other autoimmune diseases being most quiet in trimester 2. Maybe my body was just getting used to being pregnant and deciced to act up a bit?....Or maybe daratumumab had still been helping before and was starting to wear off? 

So now I was in pain, but could not take the drugs I usually take 

My options were: 

1) do nothing and just sit it out...

...I wasn't keen on that, since I didn't want to go into delivery on my last leg and then having to care for a newborn whilst having a big struggle with neuropathy. Additionally, nobody knows if the antibodies, which cause my symptoms cross the placenta and would cause the same symptoms in the baby. 

2) high-dose steroids... 

...steroids are usually fine during pregnancy, but given that my neuropathy had previously become resistant to steroids I was not keen on trying around with that. Plus steroids drastically increase your risk to develop gesetational diabetes, which comes with its own set of problems. 

3) intravenous or subcutaneous immunoglobulins (IVIG/SCIG)...  

...IVIG/SCIG are human immunoglobulins, so they are fine to take during pregnancy, but like for steroids, my neuropathy had previously become resistant to IVIG/SCIG. Plus I can only tolerate SCIG anyway because I get terrible headaches from IVIG. 

4) plasmapheresis to wash out the bad antibodies... 

Luckily, Prof. Wexler and I had a chat at the beginning of pregnancy about what our backup plan was, in case my neuropathy came back during pregnancy. We agreed, that plasmapheresis will be the treatment of choice for above reasons and because all other immunosuppressants would either not work fast enough or would come with risks for the baby. Back then we were both optimistic, that we wont have to use this backup plan, but I was happy we had a plan when I needed it. 

So we got started and applied for cost approval with health insurance, which we luckily received quickly. We also got in contact with the treatment center in Bern, which has most experience conducting plasmapheresis in pregnant women. 

Together with the experts from Bern we decided to go for plasmapheresis and not immunoadsorption. Both are similar procedures, but immunoadsorption specifically filters out IgG from the blood, whereas plasmapheresis exchanges the entire blood plasma including all antibodies and inflammatory proteins which float in plasma. Thus, you have a higher chance to catch the culprit with plasmapheresis if you don't know what's exactly causing your disease. So while immunoadsorption is a bit less heavy on the body, plasmapheresis provided the higher chance to work because we are not sure what exactly causes my neuropathy in the end. 

I had several rounds of plasmapheresis

I could write an entire post about this treatment, but this post is already getting long. Long story short, plasmapheresis is not a walk in the park, but it's doable. It's definitely not a desirable long-term treatment if you have other options. You are hooked up to a machine and you have to lie there while your blood is running through a machine, and if you get unlucky your blood pressure acts up during the process...you can imagine how pleasant that is.

Feel free to let me know in the comments if you'd like an extra post on my plasmapheresis treatments, but I'll leave it at that for now. 

Did plasmapheresis help my small-fiber neuroapthy?  

Yes it helped; my symptoms improved after every procedure. After procedure 3, I was even in complete remission for a few days. However, the problem was that my symptoms returned only a few days after every treatment session. So after about 7 treatments we decided to stop, as it was not feasible, and I was not willing to continue plasmapheresis several times a week right up to delivery. 

My initial hope was that plasmapheresis could induce remission that would last well past delivery, but obviously that was not the case. However, my symptoms were somewhat milder than before. 

There is a study going around (Olsen et al.), which reports a series of 17 or so patients with presumably autoimmune small-fiber neuropathy in the US. All reportedly profited from plasmapheresis. Patients initially got 5 or so rounds of plasmapheresis in 2 weeks and then one monthly procedure. This report was one of the main reasons why we went for plasmapheresis, and why I hoped it would result in a lasting effect for months. 

When I read this paper again, after I had my plasmapheresis, I realised how vaguely they defined their outcome. All they said was that all patients self-reported improved symptoms or slowed disease progression. No numeric or qualitativ details on this.... This can basically mean anything. They might still have gotten worse by the day but just a little bit slower... So I guess I can also report 'improved symptoms' but this small improvement was probably outweighed by the invasive an unpleasant procedure. So personally, I cannot recomment plasmapheresis....but then again, everybody is different.

After plasmapheresis, I had to substitute the good antibodies they washed out

I had a very nice hematologist who supervised my plasmapheresis. He explained that I should replace my lost IgG after finishing plasmapheresis to boost my own immune sytem as well as baby's immunity. He also encouraged me to keep SCIG going for a bit to see if it would help my symptoms after all. He managed to get my health insurance to approve expensive ongoing SCIG treatment in record time somehow. He said sometimes treatments work again, even if they stopped working before, and he may have had a point. 

So that's what I did for the remainder of pregnancy. I tried to apply individual SCIG doses as high as I could tolerate and luckily I didn't develop any bad headaches. 

Neuropathy did not resolve prior to delivery....

...but it did improve over time. By the time delivery came around it was quite mild. I could feel it a bit when walking but apart from my feet being a bit sore I didn't have many symptoms. Ironically, I did develop pretty bad carpal tunnel syndrome in my hands from edema in the last two weeks of pregnancy, which was definitely also very painful, but felt very different to SFN.

Again, was it SCIG or the pregnancy hormones that helped? 

Who knows.....but I'll take the win!

So we had our baby who was doing very well and we got to enjoy family time without worrying about neuropathy too much....which was lovely. I was worried I would have a BIG BAD flare shortly after delivery, when hormones start changing back. But thankfully this didn't happen. The first month I continued SCIG therapy, but after I started to have a few bad headache days I decided to stop it. 

Finally, 2 months after delivery, a neuropathy flare started...

This was not unexpected. I had discussed with our pediatrician before, which drugs I could take while breastfeeding. After consulting with the pharmacolgoy team at the children's hospital we agreed that it was ok to re-start belimumab while breastfeeding. So that's what I did and am currently still doing. 

How am I doing 5 months post-partum? 

I'm doing better than I feared. I expected a bad flare to come on soon, which would make me miserable and require daratumumab, which in turn would force me to stop breastfeeding. Thankfully, this didn't happen (yet🪵🤛🏻). The flare I had 2 months post-partum improved quite quickly after I started belimumab. 

 

I did have two symptom-flares following viral infections in January. After new year's I caught an annoying stomach flu, which gave me a fever and GI symptoms for about a week. And of course, 5 days into this process SFN symptoms popped up all over. Same thing again, but a bit milder 2 weeks later. But thankfully both flares calmed down again over about 2 weeks. 

Neuropathy is not gone, but it's currently pretty mild

My only limitation I have right now is that my feet get sore if I wear the 'wrong' shoes. But as long as I stick to soft padded shoes I am doing well, and I'm veeery thankful for that. This way I get to enjoy this beautiful time without the whole neuropathy hassle. 

I've been wondering what role breastfeeding hormones play in this....I guess we find out when I stop....🙈

And of course I'm always worried something will trigger neuropathy to come back full force, which could basically happen any day. With all Covid mitigation measures gone, it is hard to protect yourself from catching it. So far I've still managed to dodge the bullet. Who know's maybe it wouldn't be too bad, but given how I react to most infections, I'm not so keen to find out how I'd do with COVID....but I guess we'll find out one day.  

Anyway, I'll take it step by step, and we'll see how it goes. 🪵🤛🏻 Right now, I'm doing well, and thats GREAT🙃

And on this note, I thank you all for sticking around while I was absent for so long. I hope it won't take a full year until I manage to update you all again, and until then I hope you all stay healthy. 

I hope this was helpful to many of you. If you have any experience with small-fiber neuropathy or similar during pregnancy, please share in the comments! 

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Daratumumab restart

Daratumumab here we go again 

Hi everyone, good to see you back here! 🌺

It's been a while since I told you that I'd had a flare of small fiber neuropathy after a viral infection in early December. 

In the first half of January, I got 2 doses of daratumumab to get this flare back under control. In this post, I'll talk about how this has been working so far. 

So lets start where we left off!

My neuropathy flare had calmed down a bit by the end of December... 

but I still had trouble with my feet as well as some other symptoms. In my diary I wrote (it's impossible to remember symptoms otherwise), that the bottom of my hands and my lower arms were sore when typing and that I had to wear sweat bands on my wrist to avoid discomfort. Also my nose was itchy and the tip of my tongue felt as tough I had burned it. 

The burning tongue symptom can occur with small fiber neuropathy. I don't think it's really known how often this occurs. I have recently talked to a friend who has the same thing and who has SFN as well. Apparently she knows quite a few others who have it too. It took me a long time to realise that this symptom correlated with the severity of my neuropathy and, thus, must be related to it. It's not terrible in my case. It just kind of feels as though I had drank a tea the day before without waiting for it to cool down first. 

Over New Year's Steve and I spent 5 days in the mountains 

which was lovely and relaxing. We did go for some walks, and I was surprised that it went better than I had feared. I did some 10'000-step walks in the snow in my Ugg boots. I did notice that my feet would get warm after a while, and whenever I took of my shoes they were a bit red, swollen, and sore, but they would calm down again. However, I could still not wear normal shoes without my feet getting pretty sore. Before my flare up, I had just started wearing other shoes than Ugg boots and had started exercising on my crosstrainer (bare-foot). 

The continued symptom improvement was likely due to belimumab (Benlysta) 

which I've been taking once a week since October. Whenever I had a flare of symptoms before, it would never calm down much on it's own. Doctors would always tell me it might just pass, but over the years I've learned, that this doesn't happen for me. Without some sort of drug treatment, symptoms would always get worse or at least stay the same. So far, belimumab has not been able to prevent the flare due to my viral infection, but at least I could see a steady slight improvement over time. This is a lot actually, because even a slight improvement in symptoms is worth a lot!

Plus, who knows how bad this flare could have been without it?? 

However, belimumab is not a drug that has an immediate strong effect, so its best to combine it with something else and/or to use it as a stabiliser once the neuropathy is more or less under control. Thankfully, I tolerate belimumab well, so it's planned that I keep taking it for the unforeseable future. 

To speed things up, I got 2 doses of daratumumab January 7 and 14

In an earlier post, I wrote about my literature search and that Prof. Wexler and I had decided to go for a maintenance therapy with belimumab and to only use daratumumab in intervals. This is pretty experimental, but given that I had a relapse while on maintenance therapy with daratumumab last summer, this was the best plan we could come up with. 

We decided to do the first infusion as an intravenous (iv) infusion. So I had to sit at the hospital for a whole day with a drip in my arm. Last summer, I got all my daratumumab doses as subcutaneous injections, which is a lot easier to handle. 

Why did we opt for an iv infusion instead of a subcutaneous injection this time?  

Basically, our hypothesis is that most of the reaction to daratumumab (i.e. killing of plasma cells, which produce antibodies) happens very soon after the drug gets into the blood stream. Plasma cells express the highest level of CD38 proteins on their surface before they are exposed to dara, and they start to downregulate them once they get in contact with the drug (this is very simplified). 

Daratumumab binds to CD38 expressed on the surface of plasma cells. So in order to get the most interaction between dara and my plasma cells, we figured we want to make sure that there is a high concentration of daratumumab in the blood stream right at the beginning (i.e. we wanted a high peak concentration). 

With subcutenous injections the compound is injected into the fatty tissue (usually of the belly) and then slowly diffuses into the blood stream over 1-3 days. With an iv infusion, the drug is right there as soon as you let it in. 

There are several benefits of subcutaneous injections, which is why they are generally more popular. Most importantly, they cause less allergic reactions, because the body doesn't have to handle all of the drug at once. But given that I'd never had any adverse reaction to dara so far, we figure this is a risk we can take. Subcutanous injections are also easier to apply because you can inject them within 10 minutes. So they're less work for the hospital staff and less time intense for the patient. However, after the first subcutaneous dose of daratumumab after a treatment free interval you have to sit at the hospital for several hours after the injection anyway to make sure you don't have an allergic reaction. So it didn't make a huge difference in terms of logistics in my case. 

For multiple myeloma, daratumumab is pretty much always given subcutaneously these days. However, there are probably some other/additional mechanisms at play when giving daratumumab to treat multiple myeloma vs autoimmune diseases. I discussed this question with my hematology friend as well, and she confirmed that they give dara iv to patients with autoimmune diseases for this reason as well. So, Prof. Wexler and I decided we might as well do it properly and go for iv. 

So, I spent a day at the hospital and got the infusion over 9 hours 

This sounds worse than it was. I actually had a bed for the day and was able to binge Netflix all day. An hour before the infusion started, I got pre-medicated with steroids and an antihistamine, which made me super tired. So, I was kind of dosy all day watching silly reality TV shows. 

Thankfully, it all went smoothly. I didn't have any adverse reactions and felt fine as I left the hospital. I was also a bit worried to spend the whole day at the hospital, because COVID numbers are very high in Switzerland at the moment. So if you spend a day in a room with some other people, chances that one of them has it are currently pretty high. 

Catching COVID on the day I get immunosuppressants loaded into your blood stream was not exactly what I wanted. But I managed not to catch it (I did have my FFP2 mask on at all times and only took it off to sip on my drink or to have a bite to eat). 🪵🤛🏻

The only thing I noticed is that I must have developed some sort of allergy to bandaids over the past months. After they freed my from the iv drip, my arm looked like this and stayed like this for about 3-4 days. I didn't use to get this last year, but it wasn't itchy or anything, so that's no drama.🙃 

One week later I went in to get my second and currently last dose of daratumumab, which I did get as a subcutaneous injection this time. I had to sit there for 2 hours after the injection to make sure I didn't have an allergic reaction, but then I was good to leave and done with dara for now. 

Did daratumumab work again??

I was quite anxious about this actually, because after all, I did have a neuroapthy relapse while on dara therapy last summer. After reading all the literature, Prof. Wexler, N (my hematology friend), and I agreed that it's likely that dara will work again. But who knows, right? This is all experimental, and things often play out differently in clinical practice than one expects. So you just have to wait and see and hope for the best.

After my first infusion, I was quite nervous about what would happen...

But I noticed quickly, that it was definitely doing something again!

Within a day I could feel that my symptoms increased in intensity. Strangely, the same thing happened when I first started daratumumab in May 2021. Symptoms got worse for about 2 weeks before they started to get better. 

My hypothesis is, that when dara kills plasma cells, it sets free an extra load of autoantibodies, which were trapped in the plasma cells before. I picture this like a little 'plasma-cell-explosion'.💥 This is probably not what's really happening, but it helps me to make some sense of what's going on. 

It's also known that neuropathy symptoms can be perceived as worse when nerves start to heal, because you have more feeling in your nerves again. However, I doubt that nerves would start healing within a matter of a day, because antibodies usually take about 1-2 weeks to clear from the blood stream.

So who knows what's going on....🤯 

But this is the change in symptoms I noticed: 

The day after the infusion, I noticed that I felt as though I had a strange band over my nose. This wan't painful, but it just felt as though someone had put tape over the back of my nose. Over the following 1-2 weeks I also noticed that I developed erythromelalgia (red, hot, sore skin) a lot faster. I said to Steve, it just felt as though my skin was a lot more nervous than usually. 

This is a photo of my legs 3 days after the first infusion after having a moderately warm and not very long shower. The same thing happened to my feet and hands. They kind of felt warmer and more hypersensitive than before the infusion and were a lot quicker to play up and develop erythromelalgia whenever I did any sort of activity. I spare you a photo of my feet, because I really need a pedicure (which I don't dare getting atm due to extremely high COVID numbers) and you've seen plenty of photos of them anyway.😂

About a week in, I also noticed that I was more itchy all over and I had more of these weird muscle twitches (fasciculations) mainly on my legs but also all over my body. Overall, it was pretty similar to what I had experienced during my first round of dara. 

I was very happy to see a clear reaction to dara, so I was more than willing to put up with some increased symptoms for a few weeks. It's weird how you can be happy about symptom aggravation. After all, it's always about hope and future outlook. 

About 10 days after the first infusion I started to notice improvement

The first thing I noticed (like last time), was that my pinkies did not feel weak or clumsy anymore. Losing some strength in my pinkies is always one of the first signs I notice whenever my neuropathy takes a turn for the worse. It's not like they are paralysed or anything, but I just notice that they have less strength than the other fingers. About 10 days into treatment, I put on face cream in the morning and noticed that my pinkies cooperated with me perfectly. 

I was also slowly able to do longer walks in my Ugg boots again without my feet playing up. One day I did a very short walk (5 minutes) in 'normal' (non-padded) shoes, just to see how this goes. I managed without feeling discomfort. After about 5 minutes I did feel some pressure on my left forefoot, but before dara I would feel uncomfortable right away from the first step on. 

I was able to type for a while without dyscomfort on my hands/lower arm. I started doing small rounds of exercise on my cross trainer (15 minutes at the time on a low setting of resistance), which I managed without major hiccups. My toes would still get a bit red, but they would calm down quickly. Before that, a round of exercise would result in a payback of sore feet all evening.

About 3 weeks after the first infusion I put on jeans for the first time in a long time, and it was fine. I have to say, they were soft stretchy jeans, but this was a major achievement for me. I used to always be a jeans and t-shirt kind of person. But ever since my neuropathy got worse in summer 2020, I haven't been able to wear jeans, because the fabric just felt like sand paper rubbing against my skin. But I'll wait a bit longer until I try some proper jeans on. I remember, even last summer, when I was in 'remission' they felt very scratchy when I tried some on.

 

So how am I doing now? 

Once again, it's all about being patient. I'm definitely much better than before daratumumab, and very much better than just after my viral infection in December. But I'm still not symptom-free. Last spring it took about 2 months until symptoms were more or less gone. The weird thing is, that nerve healing comes with a lot of symptoms on its own, and it's so hard to tell apart, which symptoms are symptoms of healing and which are classical neuropathy symptoms. Unfortunately, it's not possible to take a drug and symptoms disappear all at once, because nerves need to heal first and that takes time. 

So far, I have not developed any bad hypersensitivity like last time. My theory is that this may be because my neuropathy was not as bad before the dara infusion this time around, so the nerve damage was not as extensive. Moreover, I was also on pregabalin before starting dara in spring 2021, and then reduced pregabalin gradually, once I started to see improvement. Maybe this could also had something to do with hypersensitivty, so once again, who knows. 🤷🏼‍♀️

Either way, I'll leave it at that and will keep you posted. For now I can say that I'm very happy that our hypothesis has played out and that daratumumab seems to be doing something again. I was worried that I had built up a permanent resistance to dara last summer, and at least this doesn't seem to be the case. I hope that we are one step closer to figuring out a long-term treatment plan for my small fiber neuropathy.🪵🤛🏻

Thank you all for your interest in my journey and all your thoughtful comments and inputs. If you want to be notified of future posts, click below to never miss a post❤

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Neuropathy flare caused by a virus...🦠

I caught a viral infection... 

Hi everyone, good to see you back here! 🌺 

I hope you all had a happy and restful holiday break. I'm checking in with an update on my small-fiber neuropathy. 

I've been dealing with a neuropathy flare since November

In one of my last updates, I told you that my neuropathy was only mildly active and overall didn't limit me too much. I had started belimumab in October to further improve and stabilize it, and things were stable. But as always, things change unexpectedly...  

So what exactly happened?

In late November I caught a bad cold (or whatever it was)

I was watching TV on a Sunday evening, and I could feel a scratch in my throat, which quickly developed into a very sore throat. The next morning, I woke up feeling under the weather with a headache, a very sore throat, and a whole-body muscle ache. I didn't have a fever, so I was hoping that it wouldn't get too bad and would pass within a few days. 

However, three days into this infection I started feeling sick to my stomach and spent a night throwing up...(sorry for the TMI🙃). 

The next day, I woke up with a 39°C fever and felt terrrible. We did take Covid tests, which were negative (yes, Steve was pretty sick as well....). I also tested my COVID antibodies two weeks ago and didn't have any, so we definitely didn't have COVID. 

I spent the whole day in bed, and the fever gradually improved, but it kept lingering between 37 and 38 degrees for several days. This is when I started to get worried... 

Viral infections make me nervous... 

because my neuropathy was orinigally triggered by a cold. And pretty much every neuropathy exacerbation since then was preceded by some sort of respiratory infection. Don't get me wrong, not every cold causes my neuropathy to flare up. I've had many colds, that passed without any drama.  

However, it does feel like playing Russian roulette every time...🦠🦠🦠

One week into this viral infection, I was still running a high temperature and I was up doing something on my computer. Suddenly, my lower right arm felt really uncomfortable while I was typing and it touched the surface of my desk.

It's a really weird type of pain, that I've dealth with all of last winter; it's a strong hypersensitivity to any objects touching my skin. It feels kind of electric, scratchy, and achy at the same time, but strangely it's not sore to touch when someome touches the skin with their hand. 

I knew this wasn't good news....

I tried to talk it down and hoped it was just a brief flare up due to my fever. But the next morning I woke up, and I knew this stupid virus had triggered a pretty big neuropathy flare. My whole body was in pain and pretty much all of the fun symptoms I dealt with last winter were back.😡 

I was so bummed out... just when I was improved enough to exercise a little, this happened. 

The day before my sore throat started, I had done 30 minutes on my crosstrainer for the first time in several months, without my neuropathy playing up. I was telling Steve how excited I was about this, and that I wanted do this a few times a week to get fit(ter) again....but this obviously hasn't happened now.

Here are a few impressions of my neuropathy flare

The morning after my arm got sore, I woke up to something that looked and felt exactly like a bad sunburn on my neck and chest. My skin was really hot, burned, and itched. I kept scratching, which only made it worse, so I really had to concentrate on not touching it and to wear clothes that didn't touch the red area of skin. 

This was a new level of annoying!

I'd had irritated skin around my neck and chest before, but never like this. Last winter, the skin around my neck and chest always felt a bit scratchy and I couldn't wear woolen cardigans for instance. However, it was never visible and certainly never that itchy.

I applied cortisone cream several times a day, but that didn't really calm it down. Two days after it started, I swallowed a pretty high dose of dexamethasone (20mg) that I still had at home from my daratumumab injections. This did actually calm it down, but only to come back two days later.

Thankfully, this 'sunburn' started to calm down 10 days later. By now, I have some remaining red spots, which feel warm to touch, but they are not itchy or sore anymore.🙏🏻

I wonder if this rash around my neck had something to do with the fact that my throat was pretty badly infected. I've never had the focus of my symptoms on my neck before.🤷🏼‍♀️ But that's just one more untested hypothesis...

Of course, whenever neuropathy flares, my feet get really sore. Before this infection, I was able to go for pretty long walks in soft shoes, such as Ugg boots, and I could also wear 'normal' shoes as long as I didn't walk too far.

The day before I develeped my gastro symptoms, I had actually gone for a 1 hour walk in the cold, which probably wasn't the smartest idea in hindsight. I thought that getting some steps in might help me to get fit again, but I was probably sicker than I admitted. Walking in the cold certainly didn't help my body fighting down this virus...

At the same time as my 'sunburn' started, my feet got really sore 

They felt really hot and got sore pretty much as soon as I tried walking in any sort of shoes. They were also really sensitive to heat, and I had erythromelalgia from the smallest physical exertion or warm temperatures. They also felt crampy as though I had a tight band around my feet even when I was bare foot, especially at night or during rest.

Also my hands got sore again... 

although they had pretty much been symptom free prior to the infection. These are my hands about a week after the flare started. Looks great right?! They were not always that red, and thankfully my hands have calmed down a fair bit by now again, but I still get hypersensitivity during some activities.

So how am I doing now?

Thankfully, symptoms have calmed down a bit over the past 2 weeks. I've been on belimumab since October. I'm not sure if my improvement is due to belimumab, or due to the fact that the infection has calmed down by now. It's probably a mix of both.

However, even though my neuropathy has stabilized, it's still worse than it was prior to the infection. It's mainly my feet that bother me a lot. They get sore and hot when I walk, so it's not easy to get around. And when I try to exercise on my hometrainer they get warm and red, which makes them sore as well.

What's the plan to get this back under control? 

I spoke to Prof. Wexler on the phone last week. We decided to go ahead with two daratumumab infusions in January. This should theoretically get this flare under control, and then we aim so stabilize it further with belimumab, which I'm already on.

But we'll have to wait and see how it plays out in practice

The thing with belimumab is that it works very slowly, so we added the two daratumumab infusions to speed things up.

Before I hung up the phone, I wished him a good start into 2022. Then he said to me, that he really hopes that next year will be less trouble for me. He said '2021 must have been a lot for you'...And I couldn't agree more! 

It was reassuring to hear, that even he thought that 2021 had been a lot. This thought had definitely crossed my mind several times, but I've had nothing to compare my situation too. 

Prof. Wexler sees many patients with small-fiber neuropathy, and apparently I must have stuck out with particularly many ups and downs in a short period of time. But on the bright side, at least it was not only downs, but we have found ways to beat my neuropathy back under control when it was really bad...  

So let's not jinx 2022 - I'll take it as it comes and don't expect smooth sailing...🤞🏼

I know that many patients with small-fiber neuroapthy experience many continuous downs, and are unseccessfully fighting for drug treatment or proper diagnosis. When I hear this, I know that I can consider myself lucky with regard to the fact that I've so far gotten all possible expensive treatments. 

If you are one of those patients, I want you to know that I've got my fingers crossed tightly for things to start moving in the right direction in 2022 and that you find some answers that start moving the path upwards. 

I hope that my blog can at least show that it is possible to treat this very frustrating disease, even after some treatments failed. Even if I'm also still trying to figure out the perfect long-term treatment. 

And on this note I wish you all a good start into 2022 

My new year's resolution for 2022 is to try and live more in the moment. If chronic illness can teach you one thing, then it's that things can change quickly and that you shouldn't postpone things that you could enjoy today. 

I have to admit, I definitely haven't mastered that discipline yet, and I'm still very much the same old control freak I've always been. But I promise I'll work on it.🙃

As a first step, Steve and I have booked a lovely holiday appartment in the mountains for 4 days over new years eve, which I'm looking forward to a lot. And after that it's almost time for my daratumumab infusion. 

Thank you for following my journey.💕 I'll keep you all posted...

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My neuropathy workup in Boston

I finally met a specialist in 2017 

Hi everyone, good to see you back here! 🌺 

In this post I take you back to 2017, when my second small-fiber neuropathy flare started, while I was living in Boston doing a postdoc at a large hospital. 

This is just a bit of Boston spam. A photo that Steve took while he was over visiting. 

I recommend to first read part 1 of this post, in which I wrote about how my neuropathy symptoms came back in 2017. Like in 2014, it happened about 2 weeks after a 'harmless' viral cold, this time after almost 4 years in remission. 

I left off writing about how I had gone to a fancy walk-in primary care clinic in downtown Boston, where I got a prescription for prednisone from a nurse practitioner. She had obviously never heard of autoimmune small-fiber neuropathy, and just did a few basic neurological exams to make sure I'm not having a stroke or anything immediately dangerous. 

I asked her to refer me to Prof. Maryanne Walker (not her real name) who is one of the leading international specialists on autoimmune small-fiber neuropathy. Coincidence had it, that Prof. Walker practices at the same hospital in Boston, where I was doing my postdoc. 

The clinic reassured me that they had sent out a referral, and that I would be contacted within a week.  

So I went home and started my prednisone treatment...

I was pretty freaked out, because first of all, I had never expected symptoms to come back at all, and secondly, I was living abroad by myself, not knowing my way around the local health care system. I was kind of in denial, and all I wanted at this time was for symptoms to disappear, so that I could pretend all this never happened. 

I was prescribed 1mg of prednisone / kg body weight, and in my weird state of mind, I just added another 20mg daily. Of course that was absolutely unnecessary, because 1mg/kg is a high dose, but I just wanted to make sure it worked and people do weird things I guess.....OH WELL.... 🙈

Prednisone did its job once again...

Luckily, prednisone did work very well again, and after about a week of treatment, I was able to walk home from work (about 30 minutes) in regular shoes without any major foot pain. During my first flare back in 2014, it took more than 3 months to do all the diagnostic workup, so after I finally started prednisone, it took about 3 weeks until I could feel a slight improvement and over 2 months until I could walk pain-free. It makes sense to me, that the longer you let nerve damage happen, the longer it takes for it to heal. 

This is a photo of my feet after a shower a few days into prednisone treatment. You can see that they were a bit red after being exposed to heat, but not as bad as they had been during my flare in 2014. I also bought my first pair of Ugg boots, which facilitated walking a lot, because the soft padding reduces pressure being applied to the bottom of the feet. 

But I did feel a bit beside myself during this time

Prednisone is a synthetic stress hormone, and it basically puts your body in a constant state of stress. At high doses it can even induce psychological side effects like mood swings and sometimes even psychosis. 

I generally tolerate prednisone quite well...

...but in hindsight I have to admit, that I was in a bit of a weird head space during this time. It's a weird feeling somewhere in between being super energetic, very moody, and totally exhausted at the same time. The tiniest things upset me a lot more than they usually would. On top of that, I could constantly feel my heart beating quite fast, and I was up every day at around 3 am without a chance of going back to sleep. 

Thankfully, I had told the nurse practitioner that I had trouble sleeping with prednisone the first time and she prescribed some anti-histamine sleeping pills. So at least I was able to fall asleep and get a few hours every day. 

So my neuropathy was doing better, but what now??? 

Obviously, I needed to be checked out by a neurologist. Prednisone was helping, but it's not a drug that you want to take long-term, especially not without any medical supervision. So I needed a plan of how to go forward with my treatment. 

Days past and nobody contacted me...

The clinic had promised that the hospital would call within a week, and that I did NOT need to contact them. However, after more than a week had past, I decided to give them a call to check on the status of my referral. Let me tell you, bureaucracy can drive you absolutely crazy anywhere, but they sure know how to in the US. 🤯🤯🤯

Turns out, they never received my referral...

I got through to some receptionist of the patient coordination center of the hospital, who told me that I needed to get my primary care provider (PCP) to resend my referral. 

So I called the walk-in clinic again, and they promised to resend it. But even though I paid about 400 USD for a 10 min appointment, they didn't manage to send it through, even at the second try. They insisted they had sent it, and the hospital insisted they had never received it. 

I asked the clinic to send the referral to me, so that I could send it or bring it in, but of course, they were not allowed to do so for legal reasons...🤯😡😡

I started to get really frustrated and was giving the hospital daily calls. But all I ever heard was that they can't help me without the referral, and that the referral was not there. 

Let me tell you....the combination of high dose predisone and the growing frustration was not a great combination. I remember one time, after I hung up the phone, I literally slammed my phone on the floor and then had to go pick it up in pieces....luckily it still worked.🙈   

Long story short...it was impossible to get a specialist appointment

They also informed me, that Prof. Walker was generally very booked out, so even if they did get my referral, they cannot guarantee a timely appointment with her.

This is actually a worldwide problem

I have now heard from so many more people with rare diseases all over the world, that they have to wait several months and sometimes even YEARS 🤯 to see a specialist. Specialists are few and far in between and all of them have really long waitlists. 

So I decided to give it one more try, and if that didn't work I would just go home to Switzerland and see Dr. Soland. Nowadays, I would probably just ask Dr. Soland for a Zoom call, but prior to the pandemic this was not something I thought of.... 

What a coincidence, that I worked at the same hospital as Prof. Walker...

I started to look into whether I could get an appointment via some internal processes for employees to receive accelerated treatment. 

Surely these hospitals wouldn't turn down their own employees in need??

Two days later I was notified that I could go see Prof. Walker the next day. 

I first went to see a nurse practitioner, who took another skin biopsy 

She took a skin punch biopsy sample from my upper thigh as well as a sample from my lower thigh from 10 cm above my ankle. When I had my skin biopsy done in Bern in 2014, they only took a sample from my lower thigh. 

I didn't receive the results from my skin biopsy until I was back in Switzerland, as the lab analyses these samples in batches once every few months. I later got the result below, showing that I most likely had small-fiber neuropathy. Density of my small nerve fibers was on the 10th percentile, and morphologically it looked like I had polyneuropathy. I find this quite impressive considering that I had only had symptoms for about 2 weeks by that time.

After the nurse practitioner had taken the biopsy, I went to see Prof. Walker in her office.

For the first time, I met a doctor who actually knew what I had...

...and it started to dawn on me, that it was probably a lucky coincidence that my second flare started while I was in Boston. This way, I actually had the chance to meet one of the few experts in autoimmune SFN worldwide. What are the odds that I would be working under the same roof as her...

And in hindsight, I'm so thankful that I didn't pack up and leave 

Prof. Walker had seen and published about patients with autoimmune SFN from all over the world, who had flown to Boston because they could not find medical treatment at home. And here I was, randomly working at the same place, sitting in her office...sometimes life really has its own ways. 

She was very kind and understanding. She also told me that her mother immigrated from Switzerland and that she is half-Swiss and that she collaborates on different scientific projects on autoimmune SFN with neurologists in Switzerland. 

She knew Prof. Wexler (not his real name), who I'm seeing today in Switzerland from conferences and research and pointed me in his direction in case I ever needed a specialist in Switzerland. I may not have found him without Prof. Walker, and I'm very thankful for that, because Prof. Wexler is great. 

I got a thorough small-fiber neuropathy workup

She did a detailed physical examination and asked me about all my symptoms. Given that I had been on prednisone for almost two weeks at this point, most of my symptoms had already subsided, but I showed photos of my feet from when it started and explained my symptoms.  

I also told her everything about my first flare back in 2014, and how we treated it successfully with prednisone, thanks to her publications. 

She agreed that all this definitely sounded like typical sensory small-fiber neuropathy. She also agreed that it must be autoimmune given that it responded so well to prednisone and that it happened after a respiratory tract infection both times.

Then she said, that I was an interesting patient, because she had never seen a patient with a relapse of symptoms after so many years.🤯 

On one hand it was great to hear that I was an interesting case to her, but at the same time, here I was with one of the few experts in the field and I had to hear that my case was unlike anyone elses.....🤯🤯🤯

After that I went and had a whole bunch of tubes full of blood drawn. By the way, all tests came back clear once again, but Prof. Walker said that this was expected as SFN is often a rule-out diagnosis. 

The day after, I went in to do autonomic function testing 

Small-fiber neuropathy can affect the small autonomic nerve fibers, which control things like blood pressure, heart rate, sweating, digestion, and bladder function. I never noticed any symptoms of autonomic dysfunction. Back in 2014 I had some autonomic function testing in Switzerland, and all they found was some mildly reduced sweating in my right foot. 

Of course, Prof. Walker's specialized clinic had all the fancy equipment, and I had my first tilt-table test, to measure if my heart rate and blood pressure can keep up with changes in position. I was strapped onto a stretcher and they patched electrodes and all sort of devices on me. Then I had to lie still for a while and they recorded my blood pressure, my heart rate, oxygen saturation, and other things. 

After about 15 minutes, the strecher started moving into an upright position, while my body functions were being monitored. Finally, after another 15 minutes, it slowly went back down to a horizontal position. Thankfully, they did not find any abnormalities in my heart rate and blood pressure.🙏🏻 

I know that people with autonomic dysfunction often feel terribly ill for days after a tilt table test, but given that my autonomic system seemed to be ok, I didn't really notice any side effects. 

Then they measured my sweat response in a QSART sweat test

Once again I was treated with electro shocks....🙈

I remember that they would induce electric stimuli (as they called it - I call it electro shocks😂) to my arms and legs while I had my hands and feet on some surface that would measure my sweat response. But also in this test, they could not find any abnomalities, which may also be due to the fact that I was already on treatment. 

I remember they tested a few more things, which I can't remember exactly, because it's been a while ago now, and I don't think I ever asked for the medical notes of the autonomic testing. However, if you want to know more about autonomic function testing, this youtube video by a neurology professor at the Mayo clinic gives a nice overview on what to expect. 

After all the testing was done we discussed treatment...

I told Prof. Walker, that I was currently on 80mg prednisone per day, and she looked at me with a slightly concerned face.🙈 She wanted to drop the dose down to 40mg per day immediately, but then I got worried that symptoms would come back... so we bargained a little and agreed on 50mg/day.

The plan was to complete a total of one month on 50mg/day (including the 2 weeks I had already been on 80mg/day) and then to drop by 10mg every week. If symptoms came back when I dropped the dose I could go back up for a little while. However, she said, if I can't drop down to zero within reasonable time she recommends switching to intravenous immunoglobulins (IVIG) for a while once I'm back in Switzerland. 

And now this post has already gotten very long, so I'll leave it at this with a few nice impressions of my otherwise great time in Boston. 

And on this note, I wish you all a good rest of the weekend. As always, thanks for your interest in my journey.💕

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This is me sailing in Boston harbor with a great native Boston friend. I blanked out my face, just because I have no idea where these photos end up, once you add them to a public blog....

I loved the foliage during fall in New England. These photos are from New Hampshire. 

A beatiful photo, my husband took during one of our lovely trips through Maine in summer. 

This is Jamaica Pond close to where I used to live. I loved walking or running there (when I didn't have neuropathy obviously). 

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