Last month I shared a post on my blog’s Facebook page about my IVIG treatments that was viewed by 5529 people, had 2394 engagements, 30 shares, and over 70 reactions. This was despite the fact my blog Facebook page only has just under 200 followers. It was the single most responded to post I’ve ever made, which tells me people on IVIG want a way to tell their loved ones what they are going through. I’ve had many questions of “What IS IVIG anyway?” So, as I prepare to start my 12th month of IVIG treatments, I’m going to share all that I’ve learned in the past year about this amazing, even miraculous for some, and very expensive (on average $8,000 per day) treatment. I’m not a doctor or a scientist. I’m just a rare disease patient that finally relented to adding this treatment to my arsenal to keep my rare disease, Lambert Eaton Myasthenic Syndrome (LEMS) at bay after a year of gentle prodding by my neurologist to start IVIG.
Why did it take a year for me to relent to adding IVIG to my treatment plan? Besides the fact that I knew I would have to spend time a good chunk of time, on a regular basis, having this treatment infused into my body, the side effect profile is SCARY. IVIG message boards are filled with stories of extremely painful aseptic meningitis requiring hospital stays. People who suffer from chronic migraines, like me, are especially prone to this debilitating and very painful side effect.
Warnings and precautions listed on inserts include:
Hypersensitivity (causing anaphylactic reactions)
Renal Dysfunction causing acute Renal Failure, Thrombosis
Hyperproteinemia, increased Serum Viscosity and hyponatremia
Aseptic Meningitis Syndrome
Transfusion Related Acute Lung Injury
Transmission Infectious Agents
In more simple words, all kinds of painful things that could possibly “cause morbidity” are risk factors of the treatment needed to keep the disease a doctor has prescribed IVIG for at bay. Other than LEMS, there are many other diseases that IVIG is prescribed for. Some people whose bodies do not have enough antibodies on their own are given IVIG so that they gain antibodies and can fight off infections. For those whose antibodies are on the rampage attacking their own cells as is the case with many autoimmune diseases, IVIG is used at much higher doses to stop the patient’s own antibodies from attacking. The dosing for autoimmune patients is much higher than those who need to have their immune system supplemented, which can lead to much worse side effects.
Side effects usually include: headaches, fatigue, nausea, fever, flu-ish feelings.
For me, my rare autoimmune disease, LEMS, was progressing despite the original symptoms being successfully treated. My autonomic system was being affected. I was finding it difficult to swallow food, my meds, and even sometimes the process of swallowing my water to take my meds took more effort than one could ever imagine it taking. Then, at about the same time, my voice started losing inflection. The muscle groups for swallowing are right near the muscle groups for the voice box. A different, more straightforward neurologist in my neurologist’s group looked me straight in the eye when I told her I didn’t want to risk the side effect profile of IVIG and said: “Dawn if you don’t start doing something to modulate your immune system, your disease is just going to progress.” That was the only conversation I needed to realize that the path of progression would then lead to the muscles of my diaphragm. When those muscles become involved in any myasthenic disease, that is when things get deadly serious. As we say in Maine, it was a “no brainah!” I agreed to get started with IVIG.
IVIG, in the most simple of terms, is human immunoglobulin G, given intravenously. This product is made from pooled Immunoglobulin G (IgG) of a combined 10,000-15,000 plasma donors. (THANK YOU, donors!) Prior to being separated for its different parts, the plasma from donors is tested for infections and purified. Due to this being a blood product, the infusion is a thicker solution than most IVs. Because of this, and to help lower the risk of side effects, the infusion rates for IVIG products is very slow. For example, an infusion of about 30 g in 300 mls will take many hours. Most infusions are broken down over a series of many hours, over numerous days in a row. This can be problematic with small veins and even the most hearty veins will become sore and sometimes blow out. Some people end up having a port placed to ease the issue of venous access for their infusions. Others change to a subcutaneous route of administering the treatment. However, for those needing the highest doses of IgG, Sub Q can sometimes be a difficult route to gain the most benefit.
How does IVIG work? Literally, there seems to be no absolutely known mechanism of action. Words such as “probable” and “likely” are used more in the description of the mechanism of action with this treatment than any other that I have ever read. I choose to look at it the way my neurologist described it for me (after telling me that the mechanism is really not known). By my body being flooded with immunoglobulin G from 10-15k donors, my antibodies don’t have a chance. They are outnumbered and die. The shock to my system feels a million times stronger than the shock of having a flu shot. I get chills, my chest often times is uncomfortable, my stomach hurts, I’ve had headache pain that is comparable to spinal migraine pain, nausea, chills, alternating with sweats, and am exhausted. I’ve learned to keep lots of fluids with electrolytes handy, and coffee at the ready (to ease the headaches when nothing else will help, even at 2 am). Easy foods are on hand. I rotate between my bed and my recliner as keeping my head elevated helps with the head pain. And, due to the shock to my body, as much as I feel “lazy” I know I can’t put stress on my body and must not push it…no exercising, moving furniture, cleaning. I just have to “be” and let the treatment do its job. I prepare for downtime before my treatment and then let it go and just rest.
However, once the side effects drop off, it’s like the rainbow after a storm. I suddenly have more energy than I’ve had in decades. The closet that needs to be cleaned out? Done. Painting the room that I’ve wanted to paint since moving into my home three years ago but could never find the energy to do? Looks great! Take a road trip to anywhere on the back of a friend’s Harley for the day? Let’s go! Go to California for a 4-day Global Genes Rare Disease Summit with jet lag, lots of friends, meetings, parties, and not sleeping in my own bed? SIGN ME UP! Not only have I been able to do all of the above since starting my IVIG treatments this past year; but I’ve been able to do them with a smile and without having relapses needing days of recovery. Sure, I’ve needed to rest. However, upon return from that trip from California, I was able to help rescue my neighbor’s new escaped rescue pup that had evaded the entire neighborhood for days. I was literally with my belly on the ground, utilizing the tricks I learned from “Pit Bulls and Paroles” in order to capture the little wild scared red pup. Prior to IVIG, I would not have been able to have moved, let alone do what needed to be done to capture the pup upon returning from 6 days in California with jet lag.
Case in point, About 10 months prior to starting IVIG, I had visited two of my adult sons in Maryland for 4 days. When I returned home from that visit, my LEMS flared with insane numbness and weakness for well over a week. It took close to two to get back to my “normal.” When I complained to my neurologist and my PCP about it they both reminded me that between my LEMS and another autoimmune diagnosis, my body was going to need time to recover from doing things out of the ordinary. The enjoyment of visiting my adult sons was followed by a huge amount of frustration with my own body reacting that way it did for so darn long after. The IVIG has lessened that recovery time to maybe needing a longer nap than usual the day after returning from one of my trips.
Does everyone gain the same IVIG? Not necessarily. Everyone is different. Some can see an immediate benefit. Some it takes months of treatment. Some find after months of treatment that it, unfortunately, isn’t working for them. There are different brands of IVIG with different formulations, which patients all react to differently. IVIG patients have to find the right brand of IVIG for their bodies as each brand has differing amounts of IgA, sodium, sugar, PH levels, etc. Sometimes infusion centers or specialty pharmacies that set up home delivery for patients who do home infusions have preferred brands. Sometimes it’s insurance that dictates. I even had one situation where the pharmacy alternated brands each day of my three-day treatment (not good!). Each patient’s doctor is as different as well when it comes to being pro-active in doing everything possible to help lessen the chance or severity of side effects. I’m thankful mine orders IV saline for before and after my infusion, as well as Tylenol, Benedryl and Zofran to keep nausea at bay. Some even get IV steroids as a part of their treatment to help alleviate the migraines. I choose to forego the steroids with the fact that weight fluctuates around 10 lbs with each treatment as it is.
IVIG’s half life is 3 weeks, which means the down-slide back to their pre-infusion normal usually starts about then. Schedules differ for everyone. Some need it every week, every two weeks, every three, every four, or more. However, no matter what the schedule, patients need to be on top of it all, during a time when they are feeling at their worst, just prior to infusions. Did premeds get ordered to help lessen side effects? How much fluid should I drink in the days leading up to treatment? (Answer, you can never drink enough!) Do I have everything I need to get through a number of days after not feeling well? Most patients who are on IVIG look like they have absolutely nothing wrong with them when they go to and from the infusion center. We usually look pretty tired on the subsequent days but the reality is, most of us are dealing with invisible illnesses so we go about getting our treatments looking as if we are just tired from a long days’ work. There are no telltale signs of what we are dealing with. So, if you know someone on IVIG, and you have extra energy or time, reaching out with a special treat be it a cup of coffee, a home cooked meal, or even just a text message that you are thinking of them will make all the difference in the world. If your IVIG loved one has young children, offer to take the kids to the movies, or out of the house for a while to use up their energy. As tough a road as recovering from IVIG treatment has been for me at times, I listen to my IVIG online friends with young children and honestly can’t imagine how they do it. Some have mentioned considering NOT doing IVIG because they can’t manage it with their young children. This is simply not okay.
It takes a village to get through the challenges of life; but when the village doesn’t realize what’s going on and how they can help, the village keeps busy with other, more visible things. I certainly hope that your village becomes more aware after reading this post and helps you out with your life-sustaining IVIG journey.