Hi all!

I was diagnosed with SCLS in February. I was one of the lucky ones who had an adverse reaction after getting the COVID vaccine. I've since had four IVIG treatments. While I am -- of course -- grateful for the protection the treatment offers, I am still learning how to fit monthly infusions into what I hope will be the next 40-50 years of my life. I'm wondering if anyone else has experienced the same side effects, challenges, or concerns and if so how you've dealt with them. It's overwhelming to me to think that things will be this way for the rest of my life.

Thankfully, I have "good" insurance, at least by US standards. But I will still owe thousands of dollars out-of-pocket every year until I hit catosphrophic coverage. Does anyone know of any resources to help us pay for IVIG?

We have tinkered with the rate of infusion and it takes me about 4½ hours each time, which I've been told is "fast." I am tired at the end of each day, but I am absolutely exhausted for 2-3 days after the second infusion. I also have terrible brain fog. Between the fatigue and the brain fog, it is very difficult for me to complete even simple tasks at work. Everything just feels completely overhelming. Does anyone else experience these side effects? Did you find that they went away over time? Is there something you do to mitigate the effects?

Given my side effects, I'm finding that I'm not at my best at work for almost a week out of every month. I run a school, so my hours and responsibilities aren't flexible. I'm struggling between just staying home until I feel better or being useless at work, neither of which is ideal. I'm wondering if my body would do better with a lower dose of IVIG. Does anyone get less than the recommended 2g/2kg or do subcutaneous IG instead? Have you noticed a difference?

I'm worried about my veins tolerating monthly infusions. Does anyone have a port?

Thinking ahead, how do you manage vacations and travel? If you will be away from home during your regular IVIG window, do you just delay your treatment or do you find an infusion center wherever you'll be? Or do you just not travel anymore?

Finally, there's obviously no research about life-long use of IVIG for SCLS patients. Is anyone else concerned about this?

I appreciate hearing everyone's perspective. This is all so new for me and there's a lot I still don't really understand. It doesn't help that most of my doctors don't really understand this any better than I do. Thanks for your time!

Jen

Hi Jen,

 

i try to answer some of your questions.

First of all: I live in Germany. So some things cannot be compared to your situation, as our health insurance and social insurance systems differ.

i was diagnosed with SCLS in 2013 and get IVIG since 2016.

My rate of infusions is rather low. It takes me three days per month. Each day they start with 30 ml/hour and increase to 120 ml/hour. Each day needs around 6 hours, including the procedures before and after the infusions. Before the IVIG Infusions I only get some infusion against pruritus. I do not have other reactions. Some time ago, I alsodid get several other medicine in advance. But we tried with and without them, and as there was no difference, we stopped it.

Our health insurance pays for the IVIG. I only have to apply for it every 2 or 3 years, as it is not a "standard therapy" but a off-label-use. At the moment that application is no problem, as SCLS fulfil the requirement for this. But sometimes I fear that these requirements or are changed and I cannot afford the IVIG anymore. I try not to think about this too often....

The same is about the place I get the Infusions. It was really hard to find such a place, and I am afraid, that e.g. it will be closed some time. Not every infusion center is willing to deal with SCLS. And in our health system not every center has to pay the same for the IVIG (the differences can be tremendous), so the health insurance prefers some centers. I also try not to think about this too often....

 

Since about two years I have a port. For me, the big advantage is, that it is rather comfortable to get the infusions through the port compared to normal veins. The disadvantages for me are: You need a small surgery to get it. You have something in your Body it and it (includeing a scar) can be seen (but it does not disturb me). Not all medical persons can use it to give me infusions, some are better with normal veins. 
In quite a time, it will have to be changed. At the moment I cannot say, whether I want a knew one ore not. I probably would discuss the vein situation with a specialized doctor (vasculary specialist or so).

Vacations; Actually, I plan them a year in advance around my infusions. As the infusions are recommended once a month, I sometimes get them after four or five weeks, so that it fits to my vacations. All other traveling has to obey the infusions. They have top priority. My family and my colleagues know that.

My first attack (of three) was almost fatal and I have several medical problems and handicaps since them. So I work only part time. So do not have any prpblems with the three days off work for my infusions. I work the other days a little bit more, so that the whole month is ok. 

This attack and it's outcome always show me, my family and my colleagues that the infusions and health are top priority and it is not a good idea to play around with that. My family and some colleagues have witnessed me suddenly  go to hospital and stay there for 6 months and come back to work after 11....

I hope I could help you a little bit an did. not disappoint you too much.

Yours

Andreas

 

P.S.: The good thing about the part time work is, that I have much time for my children (9 and 12 years).

Hello Jen,

I am sorry you have been diagnosed with SCLS. But I am glad that you are on IVIG that will most likely save your life many times over. 

I was diagnosed in 2011 when I almost died from my first attack. I have had 2 more attacks that required hospital stays that could have been avoided if I was on the recommended dose and frequency of IVIG. It took 6 months from my initial diagnosis to get on IVIG. At the time I was diagnosed, I had four jobs: mother of four, interior designer, personal trainer, and group fitness instructor. I did not want to have to receive an infusion every month that took 6+ hours that would knock me out for two days following the infusion with horrible side effects. I didn't have time. Because I was always so healthy, I thought I would be fine stretching my infusions to once every three months. Because most doctors do not know much about SCLS, my doctor was fine trying what I wanted. Inevitably I had another serious attack one year after my first episode. I then began monthly infusions and put up with the side effects for a while. I chose not to get a port because I was told with a port I could not swim in the pool with my kids, or lift heavy weights overhead (this is my job). The side effects of the infusions became too much and my doctor decided to break it up to two times per month. This alleviated all of my side effects and cut the infusion time down to four hours. I am even able to teach a fitness class on the same day of an infusion, both before and after.  I had another attack even after all of these adjustments, but I learned that I was actually not receiving the correct amount of 2g/kg of IVIG. I do not know how this was overlooked, but I recommend you be very pushy with your doctors and get what you need. My dose was corrected and even with the extra volume I still do not have any side effects. I contracted COVID last September. It triggered a capillary leak even though I am on the recommended dose and frequency.  I was able to get an extra infusion of IVIG and the leak was stopped. I am convinced that I would not have been so lucky if I was not on my regimen of IVIG. 

When I travel, I am able to push out my infusions for up to 6 days. I rarely travel more than two weeks at a time, so this has worked for now. When I reach retirement age, I may return to monthly infusions for travel purposes. I will cross that bridge when I get there. 

As far as the cost of IVIG, I haven't found a solution. I am lucky that I have good insurance through my husband's job. We have a very sizeable deductible though that is met in January every year due to the cost of IVIG. We plan for the big payment throughout the year and try to reconcile the fact that the money is saving my life. 

I hope you find what works best for you. Please stay on the IVIG and best wishes for a healthy life. 

 

Sincerely,

Cristina 

Thank you so much for your thoughtful responses! Hearing from you all has been incredibly helpful. I am not at all "disappointed." I am relieved to know I am not alone. This is tough; IVIG is not easy for a lot of us. Now I have a better sense of what to expect moving forward and I can better formulate a plan. Thank you!

Hello Jen.... I am from Canada so some of my answers may differ.  

 I was diagnosed in 2012  and have been on IVIG ever since that time. In Canada, IVIG is covered by our Ontario Health Insurance Plan so we are very very fortunate to not have to come up with any out of pocket money for it. I can't imagine the added stress that is for you on top of this new diagnosis.

RATE OF INFUSION: Originally I started infusions once a month, getting about 1.4g/kg(80g total), infused in one day. That worked for quite a while but then in the last few years, I was having more and more chronic swelling episodes every time I caught a virus. My immunologist decided to raise my dosage to 100g every month, which I infused in one day. My body struggled with this amount of IVIG in one day and the side effects were getting worse so we talked about the options and decided to go bi-weekly. Every 2 weeks I would infuse 50g, all in one day.... much easier on my body. I am a slow infuser, never getting above 200 ml/hour, so I am there for about 4 hours every time.

Side Effects: I have quite a few side effects and they have not improved to be honest. I have developed chronic migraines from the IVIG treatments and SCLS episodes. I have treatment on Fridays. Saturday and Sunday I feel flu-like, brain fog-like, anxious, and like I have cement in my boots... By Monday these symptoms are starting to subside. However, a migraine cluster sets in Monday evening that will last 3 days. By Thursday evening, I am feeling better.

How to Mitigate Side Effects: Before Treatment- I am given reactine (cetrizine hydrochloride), x-tylenol, & solumedrol i(n IV form) to help me not react to infusion. During treatment, a bag of D5W runs with my IVIG to hopefully help me not get as many migraines. I make sure to drink lots of water. After Treatment: As soon as I get home, I take another cetrizine hydrochloride and keep up with that for the weekend, more tylenol or advil, and drink lots of water to try and flush my system. I try to behave myself for the weekend and not overdo it. I have migraine medication that I take to get through the migraine cluster.

My Dosage: My immunologist did not want to start at 2g/kg because he said that was our upper limit. We will work our way up to that if needed. I see him every 3 months and he monitors my episodes, treatments very closely to ensure that they are working. I did try SCIG last year during COVID as my immunologist didn't want me exposed to the hospital. I LOVED it and the freedom it gave me. I was on it for 6 months. However I was not able to infuse enough product to keep the disease behaving. We are going to revisit the idea of a hybrid model once COVID settles down where I can do SCIG and then a monthly top up at the hospital. I have such respect for my immunologist as he is willing to try whatever I need to make this work.

Does anyone have a port? No and again my immunologist is reluctant to put one in yet because it opens me up to the the risk of bacterial infection.  My veins are getting harder and harder for the nurses but they are pros. I try to drink lots of fluids the day before treatment and make sure my hands are nice and warm when I arrive to have my veins in the best shape possible. So for now we are steering clear of the port. If I need it for other medical issues, we will use it for IVIG but so far that is not necessary.

Travel: My immunologist has encouraged me to travel and live life as normal as possible. We have been to Europe 2X and to Cuba 2X since I was diagnosed. This has been a real wake up call for our family to appreciate the time we have and seize the opportunities to enjoy our time together whenever we can, wherever that is. I have been able to plan the trips around my infusions. I just made sure I took lots of medications with me in case I went into a swelling episode when I was out of Canada.

WORKING: I worked full-time up until a year and half ago but then had to go on medical leave. I was a teacher for 29+ years and was finding it progressively harder to bounce back from treatments, get back into the classroom, and teach with the migraines. I was put on some very strong migraine medication that affected my language skills and cognitive skills. It was disheartening, so frustrating and exhausting. Up until then, I had just been able to push through and keep going.... But for some reason, my body just said that enough was enough. I am not sure if I will return to teaching. My husband says that it is time to retire and just enjoy our grandson. However we still have a mortgage and our last child to put through university. Me being sick has put enough stress on my husband, I so don't like putting more on him by not working. But I am so enjoying having the time to heal and not rushing back to work... In the perfect world, finding a job where I could work from home would be ideal.

CONCERNS ABOUT LONGTERM EFFECTS OF IVIG: There are a lot of things that scare me about SCLS.... but I am just so thankful that IVIG is keeping me alive to be with my family and have these years to play a role in their lives. I met a woman in the clinic the other day who has been on IVIG for 20 years for another disease and she is still mobile.... That seemed encouraging to me. I hope that these answers help and make sense.... Please feel free to reach out and ask anything... or ask for clarification. Know that you are not alone... Take Care, Lisa

Hello all,

I also would like to give some input of my situation. I receive every 3 weeks 1g/kg a dose of IVIG in a local hospital (650 bed capacity). The biggest part of the treatment is paid by the social security of Belgium. My blood is also analysed before receiving the IVIG and I have an evaluation with the hemathologist of the hospital. But he is, of course, no specialist of SCLS.

After my first atack (which took place during our vacation in France), I was transferred to the University Hospital of Leuven as soon as I was “stable” and out of coma.  Also in that big hospital doctors are not very familiar with the disease (understandalbe because of the rarity of SCLS).

My IVIG treatment takes about 5 hours and I do not have major side effects; sometimes some headaches but not very heavy.

2 weeks ago, 2 days after my IVIG,  I had a bad fever of over 40° Celcius (more than 104 Fahrenheit) that would not go down.  So we decided to go to the emergency of our local hospital.  They acted very well and transferred me to the Universitty Hospital of Leuven where I was monitored constantly to check that the fever and possible infection did not result in a new episode of SCLS.  Luckilly there was no attack and after 4 days I could go home. 

It was good to know that both hospitals took the mater very seriously and did not take any risk. I was the first time after my life-threatening attack in September 2019.

I found the item "travel" of “lisamccoleman” interesting while me and my wife are not yet to the point of real travelling.

Psychologically, we are not yet ready to travel. The fear of another attack is always there. This is probably also due to the fact that we were on holiday during my first attack.

Of course after my recover of my SCLS attack, Belgium and Europe went in all kind of lockdowns, so travelling was not really possible (Covid 19, you know). Next week we will go on holidays at the Belgian coast but only 120km from where I live. So later on we still are not keen on travelling too far from home also while my severe attack was on holidays. Luckely is was in France and we do speak rather good French. The year before we were in Croatia. We can not imagine having an attack there also given the language barrier. So travelling is for us physchologically still difficult and travelling by airplane will be excluded. We are also afraid that in case of a new episode we end up in an hospital that is not at all familiar with SCLS and no IVIG is available.

I started IVIG about 2 years ago at full dosage of 2g/kg over 2 days at a local infusion center, and had migraines and felt 'hungover' for about a week after.  I did realize about 2 weeks in, though, that my constant leak of 10 years was completely gone, so I contacted my Mayo doctor and asked about stopping, but he preferred to be proactive instead of reactive, and we compromised by stretching that to 8 weeks.  After 2 or 3 more treatments with a week to 10 days of recovery each, we adjusted to 1g/kg every 4 weeks. I've been on that schedule for almost a year now and am still not leaking and have not had any kind of episode since beginning. I do so appreciate the willingness of my doctors to listen and respect my opinion -

For prep, the week of my Friday treatment I increase fluid intake, (attempt to) eat cleanly, avoid alcohol, and get extra rest. I'm lucky to have my treatments at home, my nurse shows up about 9 and is gone by 1. I am a network engineer working from home, and able to work thru my treatment. In the beginning, I was missing at least 1 day/week after treatment with headaches & 'hangover', but the last few months I have been very close to not even noticing I've had a treatment. My husband has concerns about this being something we have to do/schedule/figure out for a lifetime, but I have come to look at it as luxury to slow down and take care of myself, listen to my body, eat and rest as I feel the need - and I am a strong believer that IVIG is worth it until something better comes along.

I am very lucky to have good veins, but I do have concerns about them becoming difficult with so much use.  I'm not interested in a port right now. I have been talking a bit with my nurse about the subcutaneous IG, maybe at a later time.

As far as travel, we will plan around my treatment. I, like Hans, am petrified of having an episode in another country, but we will bring Mayo info and contact wherever/whenever we go.

As a sort of side note, I have been getting Gammagard, but my insurance will not pay for it after January 1, and I have heard that the different brands can have very different reactions, so am a bit afraid that I may have to start figuring out how to cope post-treatment all over. I am going to look here on Rareshare for other patients' reactions to them. This site has been a blessing, you will find so much help here - good luck to you :-) 

kmiclon,

On your last issue, about having to switch from Gammagard to some other brand of IVIG after the turn of the year, you've got nothing to fear.  There can always be a bad batch once in a million, because after all IVIG is a plasma product and not an artificial, lab-manufactured compound, but the product is generally obtained in a similar manner following stringent quality controls -- no matter the brand.  Over the past dozen years, I have been given Privigen, Octagam, Gammagard and Gamunex-C, and I've had no adjustment problems at all.

The reason why different health systems, hospitals, and clinics use different brands is because they shop around and make their own deals with different manufacturers.  And I think the most common reason why they sometimes "run out" of a particular brand is probably because they are wanting to impress upon their current provider that they are willing to switch brands if they can get better pricing.

aporzeca - thank you for your input, it is (as usual) helpful and encouraging.  This site continues to be the best resource and truly a comfort when it sometimes feels like I'm navigating blindly thru a snowstorm!