Our Daughter Is Seeing Positive Results From Rituxan
Receiving a diagnosis of Lambert-Eaton myasthenic syndrome can be overwhelming. For many, the journey likely entailed multiple visits to doctors and many years of waiting.
When we received our daughter Grace’s diagnosis, we learned there is no cure, but we were encouraged upon discovering that numerous treatments are available to improve her quality of life.
It took some time to find a treatment that gave Grace significant, life-altering results.
Ruzurgi (amifampridine) or Firdapse (amifampridine) in conjunction with Mestinon (pyridostigmine) is often the first line of defense in helping LEMS patients gain strength and manage symptoms. Ruzurgi has allowed my daughter to get out of bed in the morning. Without it, she would be hindered significantly.
But after these medications, what works best is very person-specific. Many seem to try intravenous immunoglobulin (IVIG) therapy next because it can positively affect the immune system by flooding it with antibodies.
Grace’s neurologist recommended IVIG. It definitely gave her a boost of energy and balance, but she was still hindered in her abilities.
I am grateful that Grace’s doctor has never been too proud to ask for his colleagues’ professional opinions. He first invited Mayo Clinic doctors to help diagnose Grace. He also referred her to a pediatric rheumatologist so he could consult with her about the next course of treatment.
Grace’s neurologist and rheumatologist mutually agreed that it was in Grace’s best interest to start a course of Rituxan (rituximab).
Rituxan is administered via infusion. It suppresses the growth of B-cells, decreasing the amount in the blood, which in turn hinders them from attacking the neuromuscular junctions.
The first round of Rituxan is given in two doses, two weeks apart. The morning of the infusion, nurses start Grace with oral Tylenol and Benadryl.
They then run her IV and give her a drip of Solu-Medrol (methylprednisolone), a corticosteroid that can lessen or prevent allergic reactions to Rituxan. This steroid leaves Grace with a metallic taste in her mouth, so we always have gum or lollipops on hand.
Once the steroid is administered, nurses begin the Rituxan drip. This is when we settle in for the day. Typically, our days at the hospital run about 10 hours long.
Grace has experienced impressive results from Rituxan, including:
She has more energy
Before starting Rituxan, Grace would express how tired she was throughout the day. She now has significantly more energy. Not only does she have more energy, but also it is more consistent, with fewer highs and lows throughout her day.
She no longer slurs her words
Grace usually wakes up in the morning with slurred speech until her Ruzurgi kicks in. Since her second dose of Rituxan, she has been able to speak clearly from the moment she wakes up.
Rituxan kicks in faster
It normally takes Grace about 35 minutes to feel the effects of Ruzurgi, but with Rituxan, she is able to get up after about 15 minutes.
She has a sturdier, faster walk
Most notably, Grace’s ability to walk unassisted has significantly increased. She is able to walk faster, farther, and more sturdily without becoming tired.
She is more productive throughout the day
Because of the increased energy, Grace is able to accomplish more throughout the day, which brings her much hope as she looks to her future.
Although the infusion is an extremely long day, the results of Rituxan have proven to be well worth the effort. We have yet to see any adverse side effects.
This treatment has given us hope for her future and encouragement for today.
***
Note: Lambert-Eaton News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Lambert-Eaton News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to Lambert-Eaton myasthenia.
About the Author
Gisel M Burgos
Hello, my daughter was diagnosed with LEMS September,2020. She is on Prednisone and Ruzurgi. Also taking pgysical therapy 3 times a week. Was getting IVIG treatments at home for awhile which helps alot but, because of change of insurance through her father's work there has been a pause and I see her getting slower and weaker again. Her doctor is not so happy with where she still is. He wants to consider other medications. This post will be very helpful and will show it to her neurologist. And then on the other side of tge spectrum I have been researching homeopathic ways. Detoxing, vitamins, getting rid of sugars, just taking the healthier path because it makes sense of what homeopathic doctors explain about autoimmune diseases. That medications mask the root of the problem. That there was a virus or infection or bacteria in your organs, body that maybe you still have in yoyr system and you need to help your body get rid of it by detoxing. All sounds great but easy said than done. I would like my daughter to try anything before being inprisoned to medications all her life. But, she is an eight year old picky eater and strong hardheaded lol. Many of these natural treatments are nasty tasting natural drinks, pills, etc. And prohibitions of those things she likes. A grown up could go through a detox process and eating lifestyle change. It's so frustrating because regular doctors tend to prescribe many medicines after medicine but I see homeopath's want to get to the root of the cause and work from there. Overwhelming! I pray to God that a cure will arise. That somehow my girl will outgrow this!
Lori Dunham
Hello Gisel. My daughter was 14 when she was diagnosed. I understand how hard it is to adjust their eating habits and taking the homeopathic route when you have a picky child. We have been able to go gluten free mostly because she was having alot of digestive issues which the gluten free definitely helped. My main goal with the GF is trying to protect her from developing another autoimmune disease on top of this one, so thought gluten free would be worth trying. I have also thought to try the homeopathic route but need to look into it more as well. The rituxan has made a huge difference for Grace. IVIG helped but it was inconsistent and she was still exhausted all the time. Rituxan has given her so much energy and she is able to walk so much stronger and faster. I hope you can talk with your daughter's doctor and hopefully try some other treatment options. I also pray for a cure!!!!
Joyce Crawford
Hi Lori,
Thank you for posting Grace's treatments. I have not had good results with my current treatment and poor communication with my neurologist. Due to all that, I just found a new neurologist that I think has treated LEMS patients before. Your information gives me hope that I will still be able to get better, as I have been sick for about 10 years now, but was only diagnosed with LEMS 6 months ago. Please continue to keep us up on Grace's condition.
Lori Dunham
Hi Joyce. I really hope your new neurologist will listen well to you and you can find a treatment plan that gives you more of your life back. The rituxan has made a huge difference for Grace.
Veerle Van der Eecken
Hi Lori,
My daughter Emma was diagnosed with LEMS more than 10 years ago. She was 12 years old at the time. In August 2010 she was cycling easily 20 kms and in October of that same year she couldn’t walk anymore. After 2 months of hospitals and a misdiagnosis of Myasthenia Gravis, we got a final diagnosis: LEMS. With 3,4 DAP and Mestinon she got better, but for outdoor transfers, she is in a wheelchair. Unfortunately IVIG didn’t work that well for her and is not really an option here in Belgium, as it is not covered by any insurance.
The last 10 years have been a journey with ups and downs and one of looking for answers really everywhere. And it still is because LEMS itself is also an “up and down” story, sometimes with good moments and sometimes with (very) bad moments. Today my daughter is in a wheelchair for most of the time and is suffering from chronic pains (joint pains and severe stomach issues) mostly due to lack of movement for such a long period as 10 years. Even with a lot of physical therapy, it was not possible to avoid joint-issues...
Very recently, our neurologist mentioned Rituximab as a possible treatment for Emma (as she had no success with immunosuppressants either). So, I was very interested in reading your post about Rituxan.
However, our neurologist said that all Rituximab-treatments in Belgium were cancelled due to Covid. Were you able to start Rituximab-treatment during Covid and if so, did your daughter have to take special precautions after the treatment?
Lori Dunham
Hi Veerle, thank you for reaching out. I am so sorry to hear of the struggle and pain your daughter is going through because of LEMS. I know as a mom how heart wrenching it is to see our children in pain or diminished abilities. Our doctor did want to try rituximab for Grace right as covid hit but they held off for six months because they didn't want to start her on an immune suppressant in the middle of a pandemic. She got her first dose this past August. The doctor felt like it was worth it to try the rituximab despite covid and we committed to being extra careful with exposure. She has done extremely well. She can walk better, has so much more energy, is more balanced in her walking. She still struggles in the mornings getting out of bed on her own and slows down in the evenings. We are very diligent in keeping her exposure low for covid. I hope it can bring some added mobility for your daughter once the option is available again. Please let me know if I can answer any other questions.
Veerle Van der Eecken
Hey Lori,
Thanks for your quick reply! Coming May, we have our first appointment with the neurologist to discuss the pros & cons of Rituximab for Emma.
I do have some additional questions:
-When did you notice the first positive effects after rituximab?
-Have you been able to reduce your daughter's first line medication: amifampridine, mestinon? or does she still take the same doses?
-I suppose your daughter hasn’t been vaccinated against Covid. Is a Covid-vaccination still possible after rituximab and are you considering vaccinating her later on?
Lori Dunham
Hi Veerle. I am happy to answer any questions I can. We started noticing an increase in energy almost immediately after rituximab but full effects took about a month after the initial dosing. The doctor says she should continue to see increased results throughout the first 18 months of treatment and then level off from there. Of course everyone is different. We definitely see her slowly make progress with balance, walking, and she isn't near as tired as she used to be. We have not been able to reduce her medicine although we haven't tried either. She takes mestinon (180 mg a day) and rizurgi (60 mg a day). I know her neurologist said maybe we would try to reduce it, but in my opinion she still needs the same dose, although we see much better results because of the addition of rituximab. We have not had any negative side effects with the rituximab. Grace has not yet been vaccinated because she just turned 16 a week ago. We had an appointment with her rheumatologist this week and she did say now that she is 16 she will be eligible for the pfizer vaccine which, here in the US, is approved for 16 and over. Because she just received a dose of rituximab in February, she wants us to wait until July to get her vaccinated. So we have to time it correctly for her to receive the benefits of the vaccine but she is able to get it soon. I hope that answers some of your questions. I'm here for you as you pursue this option.
Veerle Van der Eecken
Hi Lori,
I’ve read in your post of April 26, that Grace is struggling in the morning because her last dose is at 6 pm the previous day. I recognize very much this situation, we had the same problem until our neurologist suggested giving Emma a delayed-release 3,4 DAP 30mg at midnight. This was not available in our country, but we could order it abroad via our neurologist/hospital (in The Netherlands). This was really a life changer for us, as Emma was able to sit up in the morning en get out of bed, she didn’t have to lie back in bed anymore and wait until her medication started working.
I noticed that I cannot comment on your recent posts anymore (I see no comment button), so I did it via this older post...
Lori Dunham
Thank you so much for that information. I had no idea there was a delayed 3,4 DAP on the market. I will look into that. That would be incredible for her to have that help overnight.
I am not sure why you cannot comment on my more recent posts. I will definitely look into that.
Thank you so much!!!
Veerle Van der Eecken
Hi Lori,
I read your latest posts about “Open dialogue with doctors” and “Changing medication schedules” with great interest (still cannot comment on your latest posts, so I’m using this post instead to communicate with you ;)).
We also have a lot of reservations concerning the COVID-19 vaccine. Emma’s disease manifested shortly after the second shot of the hepatitis B-vaccine back in 2010 (when she was 13 years old), so we are very wary when it comes to vaccines. Could it make LEMS worse? Could another autoimmune disease be triggered? I have been asking around, but no doctor can give me a clear answer. On what grounds are you going to make your dicision? What did Grace’s doctor advise?
It took us about 1 year to get a medication schedule that worked best for Emma (trial and error based). Since 2011 (she was 14 years old at the time) she sticks to the following schedule:
• Morning: 20mg DAP + 60mg Mestinon
• Noon: 20mg DAP + 60mg Mestinon
• Mid-afternoon: 20mg DAP + 60mg Mestinon
• Early evening: 15mg DAP + 60mg Mestinon
• Midnight: delayed-release DAP 30mg
Next week we have our first appointment with our neurologist to discuss Rituximab for Emma. We are very curious about his opinion...
Lori Dunham
Hi Veerle. I'm sorry I have not written back until now. I was following up with our Tech people to ask about the comments problem you are having. They suggested you try logging in to the website with a different browser. Please let me know if that works. We definitely want you to have the ability to comment.
Lori Dunham
Thank you for sharing with me your daughter's dosing information and also your concerns about the covid-19 vaccine. My daughter was 13, about to turn 14, when she received a flu shot vaccine. Shortly after she started exhibiting symptoms of what we now know as LEMS. I have brought my concerns to both Grace's neurologist and rheumatologist because I didn't know if it was "just a coincidence" that it happened after a vaccine or if it was something that was triggered by the vaccine. I only share what I gleaned from our doctor's here and what her doctor shared with me made sense. When I asked if the flu vaccine caused her LEMS, she explained to me that most likely Grace was predisposed to an autoimmune disease and the flu shot did not cause LEMS but triggered an immune response. Getting the flu would have also done this so although I wanted to "blame" her illness on the flu shot, it made sense to me that it was her body having an immune response, which would have happened with the actual flu or a flu vaccine. As far as the covid vaccine, I have been very concerned because, like you, we do not know if it will cause her LEMS symptoms to increase. Will it trigger a different autoimmune disease? Unfortunately, no doctor can give me a clear answer on these questions. We have one doctor who says the benefits of the covid vaccine outweigh the risks of it, the other doctor says he doesn't think the vaccine would protect Grace because we don't know if she could build an immune response. So we are kind of left to make the decision for ourselves. Grace is unable to get the vaccine until July anyway because we have to carefully time it between her IVIG and rituxan treatments so we have some time to gather more information and make the decision. I understand your concern completely. Please let me know what you decide and I'll share any information I base our decision upon. So many similarities in our daughter's stories.
Veerle Van der Eecken
Hi Lori,
I tried Chrome, Safari and Firefox browsers to log in, but with the same result: I only see a “print this article” button, but no “comment” button. I tried it on different computers as well.
Lori Dunham
Ok, I will pass this message along to our tech team and see what else they suggest. Thanks for letting me know.
Veerle Van der Eecken
Indeed, many similarities in our daughter’s stories! I’m really grateful that Lambert Eaton News brought us together. What are the odds... It’s nice to know that there is someone out there understanding the struggles as a parent and also fighting for the same good cause (=giving our daughters hope and the best care possible). About the vaccine: I got the same answer from my neurologist: that Emma probably has a genetic predisposition for autoimmune diseases and that the vaccine possibly triggered a faulty immune response. Emma’s 1st COVID-shot is planned the 1st of June, so I don’t have much time left (but I probably can postpone the date). I reached out to a well-known Belgian vaccinologist today and hope that he can give me some answers. I’ll keep you posted if something interesting pops up. In several of your posts, I read that Grace is treated by a neurologist AND rheumatologist. Emma is only treated by a neurologist so I was wondering what the role of the rheumatologist exactly is in Grace's treatment?
Lori Dunham
I am so glad Lambert Eaton News has brought us together as well. It's so nice knowing we are not alone. I would love to hear what you learn from the vaccinologist. Please let me know what he thinks.
So Grace was first seen only by her neurologist. He is the one who diagnosed her and prescribes the ruzurgi and mestinon as well as IVIG treatments. When we decided to add in the Rituximab he referred her to a pediatric rheumatologist who specializes in treating autoimmune disease. He wanted the rheumatologist to be the one to order the dosing of rituximab because it was her specialty. She has been a welcome addition and is very knowledgeable in "manipulating" the immune system. I hope your appointment goes well as you talk about the possiblity of rituximab as well. I would love to hear about it.
Veerle Van der Eecken
Hi Lori,
I heard back from the vaccinologist. I’m paraphrasing his answer below.
First of all he confirms that there is no contraindication to the administration of COVID19 vaccines in people with autoimmune diseases, based on the millions of vaccines that have already been administered, and the safety data that have been monitored and maintained mainly in the EU, the UK and the US. He says that rather COVID itself could create a flair-up of an autoimmune disease and refers to the fact that this is one of the reasons why MS liga in several countries in the world, including B and the US, strongly recommend COVID19 vaccination for their target group. He says that vaccinologists have more than 15 years of experience with RNA vaccines in the therapeutic world and he is therefore convinced about the safety of these vaccines. I also asked about 1 or 2 injections (I was thinking that maybe 1 injection would be enough for some protection and we didn’t have to boost the immune system again a second time), but he says that the 2° injection is mainly intended for long-term protection, and wider protection against the variants.
We still have doubts, but I’ll keep you informed about our final decision.
Lori Dunham
Hi Veerle. Yes, thank you so much for the information. I was trying to track down a solution for your comment issues. It looks like our tech guys can comment on all my posts so we aren't sure why you aren't able to. I'll keep digging.
In the meantime, thank you so much for the information about the vaccine. It was valuable information that he offered you. When do you have to decide by? I hope you feel at peace either way. Please let me know what you decide, I will let you know what we decide as well. We have until July to decide before her next rituxan. Have you had the appointment discussing rituxan yet? If so, how did that go?
Veerle Van der Eecken
Hi Lori,
I sent you the reaction of the vaccinologist a couple of days ago, but I can't see it in this column.
Have you received it well?
Veerle Van der Eecken
Hi Lori,
We had our appointment last week with the neurologist about Rituximab and he is convinced that it is worth trying as a LEMS treatment. However, he warned us that the chance of success is only 50 percent for Emma due to the duration of the disease (Emma has LEMS for more than 10 years). Apparently the chances are better at an early stage of the disease. It will also take longer to see the first effects (+/- 1 year). But we definitely want to give her that chance and decided to start Rituximab therapy mid August. According to the neurologist, it is important to have a Covid vaccination before starting Rituximab and he is very confident that it is safe. So, last week Emma had her first Pfizer shot! No problems (side-effects) so far...
He also mentioned interesting options for LEMS in the future. He is quite a famous Professor in Belgium and involved in the research and clinical studies of future biologicals for rary diseases.
Lori Dunham
That is wonderful to hear she has done well with her first covid vaccine shot. Please let us know how she does with the rituximab. I really hope she sees positive results. I would love to hear more about the possibilities of future treatments as he shares them with you.
Veerle Van der Eecken
I have noticed that the “comments” problem has been solved. I can now see a “post comment” button below each of your posts. Super!
Lori Dunham
Wonderful. I'm happy to hear that!
Veerle Van der Eecken
Hi Lori,
I sent you an email a week ago, but I don't know if I got your email address right. I was wondering if you have received it well? The subject was: news from Belgium...