So Here's the Deal...
A lot of people haven’t heard of any of the diseases that my wife has, or maybe have only heard of one or two. I’d like to elaborate and educate those who don’t know what kind of auto-immune diseases my wife has and what challenges and experiences we have had with them. Most of these have been life-threatening or have made life very hard physically for her and emotionally for both of us. We have definitely had our share of struggles. Please remember my wife is a “rare case” with refractory and severe forms of most of her diseases. This is by no means an exhaustive list of her ailments. This is just to give you, the reader, an idea of how things are.
Generalized Myasthenia Gravis
Generalized Myasthenia Gravis (MG) is a chronic autoimmune neuromuscular disease that causes weakness in the skeletal muscles, which are responsible for breathing and moving parts of the body, including the arms and legs. The name myasthenia gravis, which is Latin and Greek in origin, means “grave, or serious, muscle weakness.” (Learn more about Myasthenia Gravis here: What is Myasthenia Gravis?
About a month after Candace and I started dating, I saw her for the first time on a ventilator. She had gone into what is called a “Myasthenic Crisis.” That was the only time I had seen her on a vent with the exception of a couple of surgeries I have been with her for. I have been able to help make it so she didn’t need to be ventilated by being there when she was having issues.
There have been several scary instances where she and I were on the floor for hours with me trying to keep her airway open. For some people, that is one of the things that makes this disease life-threatening. If her airway collapses and nobody is there to open it and keep it open for her or ventilate her quickly, she could literally suffocate and die. I learned from both Candace and her nurses early on how to do a “jaw thrust” which is the technique to use to keep someone’s airway open.
There have also been times, while Candace has been in a Myasthenic flare that she wasn’t able to chew and/or even swallow food. I would have to give her yogurt or rice and cheese or something soft that was easy to eat and swallow without the risk of choking. Not being able to swallow also means not being able to take meds, which presented another problem. I would have to crush her pills and mix them in something (usually water) for her to take.
Another thing with MG that we have to be careful with is that Candace can’t be in the extreme heat because it makes her weak, and makes her symptoms worse. So, during the summer we stay indoors where the air conditioning is, or I go out to the car and start it ahead of time so it’s cool enough for her when she gets in. It greatly limits the time we get to spend outside, and we both love the outdoors which makes it even harder.
After a really bad crisis shortly after our honeymoon, the hospital sent my wife home with a Trilogy machine. It’s a non-invasive ventilator that helps her breathe. She uses it every night and during the day as well if she is having breathing issues. If it wasn’t for this machine, we would have had to make many more trips to the ER.
We have had to make several ER visits for my wife due to her Myasthenic Crises. When we knew it was bad, I would have to help her downstairs and outside and into the car. Having to carry full deadweight down the stairs was very challenging for me. A couple of neighbors helped me one time. They had to literally lift her into the car because I wasn’t strong enough to. I avoided calling 911 at all costs because I needed to make sure to get her to the hospital where her Neurologist was, in order to be able to get better treatment without all the guesswork. The whole way to the hospital, I would worry about her airway closing, which was terrifying.
When we end up at the hospital, we are usually there for 2 days minimum for her recovery and treatments. We have been there for at least a week in some cases. However, prior to meeting me, she used to spend a minimum of 14 days inpatient for each crisis. I would never leave her if I didn’t have to because I am always afraid something will happen to her, like a doctor not knowing what MG was and someone not knowing what to do when her airway closes, and also so I could help her communicate with the medical staff. I have never really trusted her doctors and nurses in ICU, but I have been able to serve as an advocate for my wife, and keep her safe. It was even harder to this when I was working a 40 hour-a-week job, and not yet being married. I tried to schedule days off as best as I could, and there were several times where I called in and put my job at risk.
I can remember several instances when we were in the hospital where she would start having ptosis (drooping) in her right eye, she would be unable to speak, and her words were slurred. On more than one occasion a nurse panicked and thought she was having a stroke. While the symptoms are similar, I’ve been quick to say “no don’t call a stroke alert because she just needs her medication.” After she got her medication and we waited for 15 minutes, the ptosis was gone, and just like that, she could support her neck and speak clearly again. It always amazes the doctors and nurses that see this transformation actually happen. My wife’s MG has served as a teaching opportunity for a lot of her doctors and nurses, especially ones who have never heard of it, or know very little.
Systemic Lupus Erythematosus
Systemic Lupus Erythematosus (SLE) is a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body. The signs and symptoms of SLE vary among affected individuals, and can involve many organs and systems, including the skin, joints, kidneys, lungs, central nervous system, and blood-forming (hematopoietic) system. SLE is one of a large group of conditions called autoimmune disorders that occur when the immune system attacks the body’s own tissues and organs. Learn more about SLE here: What is Systemic Lupus Erythematosus?
For Candace, Lupus has affected multiple body systems. She regularly has joint pain, especially in her hips, and usually, there is nothing we can do about it except increase the med she takes for it. Sometimes she complains that all her bones are in pain. When it gets really bad, I have to help her apply ointment on lesions (open sores) on various parts of her skin where she can’t see or reach. On top of that, she sometimes gets low-grade fevers. The fevers come and go in waves. If her fevers ever got too high and stayed that way, it would be an ER trip for us. People with auto-immune diseases who get fevers are in trouble due to already being immunocompromised.
Stiff Person Syndrome
Stiff Person Syndrome (SPS) is a rare, progressive syndrome that affects the nervous system, specifically the brain and spinal cord. People with SPS often have heightened sensitivity to noise, sudden movements, and emotional distress, which can set off muscle spasms. Learn more about SPS here: What is Stiff Person Syndrome?
I have been with Candace during all her bad spasm episodes. When I say spasms, I mean full-body spasms. They affect her feet, legs, entire torso including her diaphragm, the muscles around her abdomen, back, arms, and neck (which of course affects her airway). When she is in the middle of an episode it is hard for her to breathe, let alone swallow pills. When she starts feeling nauseated, which is an indication of a spasm episode, I run for her meds and tell her to take them quickly so that they will help. Her spasm episodes usually last for 45 minutes to an hour, depending on how soon she gets the meds, and how severe the episode is. In my wife’s case, this disease is life-threatening because of the risk of her airway being closed up by her own muscles, as well as her upper torso spasming which prevents her from being able to expand her rib cage for a breath. Sometimes it happens separately, and sometimes it happens at the same time. She has told me when she is spasming, it feels like her whole body is cramping, and she’s being choked from the inside. Imagine a Charlie horse on your calves, or your foot cramping up, but on your WHOLE body.
While she is having an episode, I am completely helpless. I just hope and pray that the medication works fast so that she can have some relief. I stay by her, wherever she is, and keep an eye on her airway and also try to be some encouragement to her. Even after the episode is over, she is still in a lot of pain because her muscles tightened up so much. We also have to keep an eye out for any of her MG symptoms. Spasms make her weak, so essentially we are dealing with 2 diseases at the same time.
Mast Cell Activation Syndrome
Mast cell activation syndrome (MCAS) is one type of mast cell activation disorder (MCAD) and is an immunological condition in which mast cells inappropriately and excessively release chemical mediators, resulting in a range of chronic symptoms, sometimes including anaphylaxis or near-anaphylaxis attacks. Learn more about MCAS here: What is Mast Cell Activation Syndrome?
My wife can become anaphylactic to almost anything, including foods and smells like strong perfumes, and it can happen in between treatments as well. It usually takes the form of hives or rashes. Her blood pressure also drops, she starts to swell, and gets itchy. When Candace is going through an MCAS flare, she “loses” foods that she can eat.
At one point during an MCAS flare, which lasted for months, my wife had lost a significant amount of weight quickly, because she couldn’t eat very much without reacting. At one point, we were down to only 3 foods that she could eat. Thankfully we never had to make any ER visits for this one. We were close to having her get a food tube, but narrowly missed it. Whenever she eats something that makes her react, she could have any type of reaction such as stated above, or something completely new.
Dysautonomia is a general term used to describe a breakdown or abnormal function of the Autonomic Nervous System. Learn more about Dysautonomia here: What is Dysautonomia?
Your Autonomic Nervous System (ANS) is the part of your nervous system that controls involuntary actions, such as the beating of your heart and the widening or narrowing of your blood vessels. When something goes wrong in this system, it can cause serious problems, including blood pressure problems, heart problems, trouble with breathing and swallowing, erectile dysfunction in men. Learn more about the ANS here: What is the Autonomic Nervous System?
Something that we know that helps her with these symptoms is a ridiculous amount of water. I found her a really big water bottle that we take with us everywhere. First thing in the morning, she drinks a full 64 oz bottle of water, along with her morning medications, and helps balance things out better. Another thing she has done is take salt pills. She found them online and they really do help. There have been times where Candace is talking to me while she is standing, and she gets very wobbly and I have to go over to her and keep her balanced or help her onto the bed or onto the floor. There are other times where she stands up from a sitting position too quickly and she gets very wobbly and dizzy and has also passed out. Along with the dizziness, she also experiences extreme shortness of breath both when sitting and standing. It can be heard when she is talking. She sounds extremely exhausted. It’s times like these that I feel the most helpless. There isn’t too much that I can do for her.
Hereditary Angioedema (HAE) is a rare, potentially life-threatening genetic disorder. In most cases, HAE is caused by the deficiency or dysfunction of C1 esterase inhibitor (C1-INH). 1-3. (NIH). Learn more about HAE here: What is Hereditary Angioedema?
The way that I know this disease is affecting Candace is because she experiences an obscene amount of swelling in the body. This is another disease that could affect Candace’s airway. Different parts of her body swell up suddenly and very quickly. She usually swells in her face or abdomen, to begin with, and then it will move up into the throat if it starts to get bad If we don’t get her medication to her in time, her throat can tighten up and she can literally suffocate to death. A jaw thrust won’t save her. Our first indication that she is swelling is that her cognitive issues become worse. She has a hard time speaking, like her brain and mouth are not making a connection, she has a hard time finding the right words, and she is also more forgetful. Sometimes she has no idea that she is experiencing HAE symptoms and if I realize this, I go get her medication as fast as I can.
There have been numerous times where I have had to give my wife her medication for this disease very quickly. It can be very severe at times. I have watched my wife turn blue while trying to push her IV medication into her body fast enough. It was terrifying I have a stocked med bag ready for these emergency cases with supplies.
But Wait, There’s More!
On top of all these things, Candace deals with photosensitivity from the sun. She literally cannot have any skin exposed to the sun for a prolonged period, or she breaks out in rashes or blisters, or lesions. Sometimes even if she is only exposed for 30 seconds to a minute, she still has reactions. Not only that, but she has also had systemic reactions, meaning she gets fevers. This is not always the case for people with photosensitivity, but in Candace’s case, it is. In fact, her photosensitivity is so bad sometimes, we had to get special light bulbs in our house and a blue light screen cover for her computer. Her cognitive abilities also diminish, and she gets very dizzy. So, to protect her, we got useful (and expensive) tools such as cooling towels, a hat, special clothing, and an umbrella that are all SPF 50. She uses these whenever she is outside. I even got all the windows tinted dark on our car, even the windshield, as well as multiple sunshades that attach to the windows from the inside.
Candace has cognitive issues regularly. It’s a combination of her medications along with her auto-immune disease symptoms. There are days where she will ask me multiple times what day it is. I manage all her medications because I just want to ensure that she gets them when she is supposed to. Some are given every 2-3 hours, some are given every 4 hours, some are given every few days, and some are given as needed, and I have had to learn what she needs to take for what. I have picked up on certain symptoms that indicate which disease is affecting her, and I am able to give her the right things to make those symptoms lessen or go away. Together, we are also able to think ahead and try to prevent any issues, especially when we leave our house.
There has only been one time that all of these diseases seemed to hit her in one day. It was one of the scariest days of my life. I was pretty shaken up after, and so was she (naturally). It started with her Dysautonomia, then shifted to HAE, then moved onto MG, and then finally SPS. It was one right after the other, and it really put me to the test physically and emotionally.
There were a couple of scary instances during this episode where her airway started closing. The first time it happened, she started turning blue because her airway was closing up and she was starting to suffocate. I was able to administer her medication quickly and it worked within 10 seconds. I stood there helpless, giving her medication to her, trying not to panic, and watched her take her first breath, gasping for air, once the medication was in her system. Thankfully I knew which medication to give her. The second time it happened I had to do a jaw thrust quickly and I had to hold her head up so she could breathe while her medication was working, which was about 15 minutes. I needed help from our oldest to give her med to Candace, which I’m sure was scary for both of the kids to see their mom going through.
There was a lot of emotion going on during this time. Both of us were so baffled by what was happening. Altogether it lasted for about 2 hours. I remember seeing her struggle cognitively. She was getting upset and yelling at me because she thought I wasn’t listening to her. I remember trying to stay calm so that I could help her the way she needed me to. I got frustrated and it made my hands shaky which made it hard to do things.
Since she and I have been together, we haven’t had to deal with anything like that before. There have been times where I have had to get her to the ER quickly. We always try to prevent that, but on this day, I had our oldest have the phone ready in case we needed to call 911. That’s how bad it was.
This piece was co-written with the help of my lovely wife, Candace. Thank you my love for helping me with this, and making sure that the information I share with others is accurate and true. Thank you for your patience with me and for your understanding during this process. Thank you for your willingness to teach me about you and your diseases and for letting me share our stories with everyone. Everything that I have learned is from you, and you are a great teacher! You have taught me everything since the beginning of our relationship, and we have also learned things together along the way.
CITATIONS (Myasthenia Gravis) https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Myasthenia-Gravis-Fact-Sheet
(Stiff Persons Syndrome) https://www.ninds.nih.gov/disorders/all-disorders/stiff-person-syndrome-information-page
(Mast Cell Activation Syndrome) https://www.aaaai.org/conditions-and-treatments/related-conditions/mcas
(Autonomic Nervous System) https://medlineplus.gov/autonomicnervoussystemdisorders.html
(Hereditary Angioedema) https://ghr.nlm.nih.gov/condition/hereditary-angioedema