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Pregnant with autoimmune small-fiber neuropathy
I've been absent for a veeeery long time. You might already guess why...
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.
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....
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.
...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...).
...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.
Finally, 2 months after delivery, a neuropathy flare started...
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....🙈
