Patients Speak Up

Vince: I'm a stay-at-home dad so when you're a stay-at-home parent, you don't have any support. It becomes it becomes harder. Just kind of get to learn on your own and, you know, trial and error and just do it.

When I started developing allergy symptoms and I'd make an appointment with an allergist, an ENT, and they all said the same thing. You know, you're getting develop allergies, the pollen is really high here and that's where everything started. Like, right just in this area. Runny nose, the watery eyes, itchy eyes, you know, the typical allergy symptoms. And then progressively they started getting worse. And now we're in like 2009, going into 2010.

And at that time, I was going back like every three months for checkups. I'm like, Something's off. You know, things are getting worse. I just kept telling them something's off. And then he's like, “okay, maybe we need to do like biopsies and stuff.”

And at the time, the sides of my face started getting really swollen. And it started getting embarrassing to actually go out in public and stuff. That's when like my wife started thinking, “I think you got something else going on.”

I went back to the same doctor and they did scans of my abdomen, my pelvis and my chest and he called me up the next day personally, he said, “I think you have cancer.” And I was like, wait a second. The guy was just doing biopsies on my face like every month and nothing was ever fine. I said, “Something’s not right here.”

At this point, I'm out of options and I got to try something. And now we're in 2013. So I made an appointment with an allergist there because, like I said, it was mainly right up here. And then that's right. When I found out that all my insides were inflamed, inflammation was overtaking my organs.

Soon as I got there, she ran tests, and she ordered scans and like two days later she called back. She goes, “you have an autoimmune disease called IgG4.”

SLATE: Immunoglobulin G4-related disease, or IgG4-RD, is a chronic, systemic, inflammatory disease that can affect nearly any and often multiple organs of the body.

It is characterized by silent, ongoing organ damage and recurrent disease flares causing accelerated and irreversible damage to previously affected and new organs.

Vince: It attacks a lot of organs. It attacks a lot of body parts, and there's a lot of symptoms associated with it. So, it's really hard to pinpoint because there's just so much going on. Just hits like a ton of bricks and then you're just bombarding her with questions. Okay, first question, “Am I going to live?” “Yes, you're going to live. We can treat this, just got to, you know, trust me and let the let the process take its course.” I was like, great, I'm ready to go. What do we need to do?

What I would tell somebody with a newly diagnosis is do trust the system, you know, because when you start getting juggled around from doctor to doctor and pushed around, you start having doubts, you start doubting the system. But, you know, there is a process with this disease because there are people out there that will and genuinely try to help you.

Sabrina: My name is Sabrina. I am a resident of Atlanta, Georgia. I'll be 54 in just a couple of days. And I'm a mother of six. I work in health care. I've been in health care for 31 years.

In 2011, I started to notice that I was feeling a bit underwater in my right ear. I just couldn't hear things. Well, the sound was muffled, and I couldn't really explain why. And so that was a tad bit concerning to me.

The ENT said we're going to do a CAT scan. And during that CAT scan, they discovered a large mass growing in my right ear canal and in my mastoids.

We went to surgery. I was in the recovery room and he came in with sort of a gray look on his face and said, “Sabrina, this looks bad. And I'm just afraid it's cancer.” I was just really confused. After they sent the pathology off, it came back and it wasn't cancer.

When you're in that waiting period to get a diagnosis, I felt so much uncertainty about my life. What if they never find out what's going on with me and I die?

The results of the spinal tap, went back to the rheumatologist, and she says, “I think you have something called IgG4.

SLATE: Immunoglobulin G4-related disease, or IgG4-RD, is a chronic, systemic, inflammatory disease that can affect nearly any, and often multiple, organs of the body.

It is characterized by silent, ongoing organ damage and recurrent disease flares causing accelerated and irreversible damage to previously affected and new organs.

Sabrina: Sometimes there's some concern about who really should treat this disease? And I feel caught in the middle. That doesn't feel so good. I just really want treatment. I really don't care who treats it. I just really want treatment. Before we get to a point where I'm in the hospital again.

My manifestation of IgG4 happens to be in my brain. And so I had full neurological involvement in my brain And these intractable headaches that would almost bring on seizures and multiple hospitalizations and visits to the emergency room. That started my journey to seeing different physicians. That physician, while under his care I had five surgeries.

Those were tough surgeries to get through because all of them involved either my mastoid or my skull base. As long as they were doing surgeries to try to remove some of the invasive areas, everything was fine. But how many more surgeries can you safely have? When those surgeries stopped and there was no treatment to kind of push the disease back, that's when there was more brain involvement,

And also, during that time, I began to experience some facial neuralgia. And so there was this trigeminal neuralgia that would make my face feel like it was literally on fire.

A lot of things have passed, a lot of flares and I'd say that I'm more hopeful. When the when I began the journey, it was completely in the dark. If there is way of saying there's a silver lining to this disease, it is that there are periods of relapse. And when the disease is in a remission state, I’m going to change everything about my life to make the period of remission as long as possible.

So, I'm so hopeful for other treatments that will keep the disease from full manifestation. Everything matters with IgG4-RD and push. If your physician doesn't want to do something, push.

My case is no different than any other IgG4-RD patient. I long for the day when I can say I've been in remission for five, ten, 15 years and I know that it's coming.

Damon: I'm originally from Detroit, Michigan. My parents were both in the military, so I kind of started my first five years of life as a military brat. My mother had multiple sclerosis, so, by the time I was seven, she started having symptoms. And so just kind of a slow deterioration. Until the time I got 20, she passed away from symptoms to multiple sclerosis.

So, I was pretty much raised in Detroit up until I left back in 2008 to come to Atlanta. So 2022, and I came home one day and was taking my shoes and socks off and I noticed indentation in my leg. So the next day I looked down on my feet again and a couple of my toes were swollen I thought maybe, let's see, my primary care physician. Her colleague called me and said, “You need to go to an emergency room.”

They ran bloodwork and the internist came and said, “there's something going on with your kidneys and your liver. I need you to have a CT scan.” And then he came back and I saw his face. Something wasn't right. He was like, “So I get results from your scan, You have masses throughout your abdomen area and we need to rule out a metastatic cancer. You have a lesion on your liver. You have two lesions on your right kidney, one of your left kidney. You have multiple lesions in your stomach area and your pancreas is inflamed.” And they said, “we're going to biopsy your kidneys. We're going to hold off on doing your liver because we want to do your kidneys first.”

But by the July, by the end, this time, he still didn't know what it was. And I start getting really sick again. And I had to go to a hospital and they went in and they did a biopsy on my kidneys. And then they also did a biopsy in my stomach. And that's when I found out it was IgG4.

SLATE: Immunoglobulin G4-related disease, or IgG4-RD, is a chronic, systemic, inflammatory disease that can affect nearly any, and often multiple, organs of the body.

It is characterized by silent, ongoing organ damage and recurrent disease flares causing accelerated and irreversible damage to previously affected and new organs.

Damon: I said, it was impacting my quality of life, just annoying. I would say, because I'm being I used to be an active in doing stuff, but like, I can't you know, I want to go to my office. I want to go to church on Sunday. You know, I want to go travel. And I had planned these trips like, I'm going overseas, you know, now I can’t do the things I want to do.

I will get winded, constantly fatigued. Like just going to brush my teeth in the morning, lean on a sink, be a stand in a shower, leaning against a wall. It's scary not knowing and there's not a lot of research on IgG4 so you Google, you see like the same articles

My nephrologist, she was sharing information with me as long as she found out about it, but don't know can be scary.

Once I realized what the disease was and how it could impact your organs. I go have bloodwork every month, but then they kind of tapered it off every three months. So, between those three months, I was worried like, my goodness, could this be attacking my organs? And when I go back and I'm like, we need to get you in hospital because now is attacking not only your pancreas but your gall bladder. And it moved up to your lungs because I didn't have any symptoms. So, it is like a silent type of monster inside of you. And by the time you find out, it could be too late. And that's what scares me the most.

It presents us a lot of other different diseases. And that's why I physicians are not aware of it. So, it's very important that you follow up with your health care providers to make sure you get tested frequently and do the blood work so you can see what these is doing inside of your body.

I would say, make sure you surround yourself with positive people And I would say, be honest with what you're going through and make sure you translate that and make sure you're honest with your providers about what you're going through and hold them accountable. And just don't give up. Don't give up.

Mahsa: My name is Masha. I am a wife, I am a daughter. I am a granddaughter. I love my family. Love my pets. Since I was born, I started having presentations in my neck and face glands. I was showing signs of parotitis, inflamed glands, ear pain and infections, and no one could figure out what was going on.

It was just another thing that, you know, they say kids get sick and it was just put on their allergies and the symptoms kept spreading. I had bruises all over my arm, down my back, on my legs. At the age of 16, I missed a lot of school. It was about six months of my high school that I missed, and from there went on, we kept going, you know, like my gallbladder was next on the list, which it got affected.

But for six years I was in and out of hospitals and from there more symptoms kept happening with no diagnosis. You know, nobody reached out to me for the pathology of my gallbladder to let me know if they found anything or not. From there, I was still in and out of hospitals as my face glands and neck glands were being affected.

I woke up one day a couple of years ago and my eyes were just puffed shut and I couldn't see anything. The doctors do their tests and at the end of the day they don't find anything. They would always just get some antibiotics and a round of steroids and, you know, a couple of years later, come back full blown. Doctors just couldn’t figure it out.

You think you're going to go to school, you're going to graduate, get a job, get married, settle down, have a family right. All of those things fell apart.

I asked a few doctors to, you know, biopsy anything and everything that was inflaming, and I started doing research from there. And I spent hours online. But even with all the hours that I spent, it took me one week to figure out what I have. One week it took me so. And now I have to go forward and prove why I have what I think I have. Thankfully, I found the right specialists who were willing to biopsy different parts of my body.

So, for three months I went forward biopsy everything. And they took all that information. And after 30 years being misdiagnosed as allergies, they said, “well, you have IgG4-related disease.” And I said, “I know.”

SLATE: Immunoglobulin G4-related disease, or IgG4-RD, is a chronic, systemic, inflammatory disease that can affect nearly any and often multiple organs of the body.

It is characterized by silent, ongoing organ damage and recurrent disease flares causing accelerated and irreversible damage to existing or new organs.

Mahsa: Getting a diagnosis is very validating in our daily struggles. The feeling of validation is very important for us patients. I feel we don't get it from a lot of people, especially if it's an illness that invisible, that feeling of validation. It's everything. It's everything.

This process taught me how to listen to my body. It's made me strong enough to want to advocate for myself and advocate for other patients, you know, to kind of help bridge these gaps that are missing and connect the right doctors to the right patients.

When a disease takes over your body, it becomes a part of you. So, I will always be a patient, but at the end of the day, I'm still human.

One day at a time. One day at a time.

Mitch: I'm from Montana originally. I live there now. Pretty much a jack of all trades done construction from the ground up, farming, had my own businesses. My dogs are my children. We've got four in the house.

2020 I was working 60 hours a day, I was doing a second story remodel on an office building. And every step up and down the stairs and doing the floor hurt and my legs burned. And weeks later it didn't go away and a week later it didn't go away. And I just kept thinking, the next week I'm going to get enough energy.

I got all kinds of things I'd have hadn't had heart burns and my body went weird for sure. At that point, I was throwing up a lot and I just associated it with stress and nerves. My feet of swell to the point I can't walk. Hips, knees, elbows, shoulders, all my fingers, my spine from my tailbone all the way up feels like somebody hit it with a hammer.

The inflammation has been consistent for 20 years. I developed psoriasis with it, so from my waist down was covered in scales and psoriasis. And so with all of that, I had constant itching, constant pain. I've laid on the ground and wished I would die, couldn't get up, didn't want to get up.

And finally it got to the point where I walked into a doctor's office. and I begged for help. I needed to know what was going on.

SLATE: Immunoglobulin G4-related disease, or IgG4-RD, is a chronic, systemic, inflammatory disease that can affect nearly any, and often multiple, organs of the body.

It is characterized by silent, ongoing organ damage and recurrent disease flares causing accelerated and irreversible damage to previously affected and new organs.

Mitch: My internal organs, kidney, bladder, urethra, scrotum, anus, all that is been affected and flares up. And through that process, the dealing with the pain is one of the hardest things because of the system and the way it works and the pain issues that go along with it.

It's almost every part of my body. I believe it's affected as far as diagnosis and doctors seeing it there.

I can remember when I was healthy, and I can remember when nothing hurt and everything worked for the most part. And I can remember for the first few things when the joints started swelling or and I thought I could fix it. I thought I could make it go away, you know, I thought it would get better.

As bad as it is to go through all the things, I'm still alive. I think they keep me alive. And then it's got to be a point where I have answers to my disease. I know what it is now. And I can, for me, knowledge is the power that I need to understand. I might not know, say, take that knowledge and go down the same path as everybody. But it helps my mind understand what's going on. So now I have the knowledge. I know what it is. I can face it, tackle it, you know, it's hard to fight an invisible demon.

And I'm excited to give it a go and then take that energy and go out in the world and see where it takes me.

Just spread that love and energy and positivity and message of hope to people that are out there that, you know, you can it can all be horrible and it can all be miserable and it can get better.

Donna: My name is Donna and I live just outside of Boston, Massachusetts. I have been married for about 18 years. I have a 17-year-old daughter and I'm a nurse. Before that, I worked with people with disabilities, and I used to run group homes for people with developmental disabilities.

So, it's only been a little over a year that my entire, I guess you would say journey, started. I was just starting to notice that I was having some pain from my stomach area after I was eating. I went for the CAT scan and on the CAT scan report it was not a stone. They noticed that there was like a mass and on the CT report it said it was highly concerning for malignancy, which is cancerous.

And it was just nothing that I was even expecting. You know, I'm thinking it's very simple. It's a stone, you know, they'll blade it'll, you know, work itself out will be fine. I saw the word malignancy and my heart dropped. Long story short, he presented my case to the tumor board at the hospital, which is just a collective of physicians.

And the consensus was that that they really needed to take out the entire kidney and ureter, for a full biopsy. Two weeks later, pathology came back. It was not cancer. And he said it was IgG4-related disease.

SLATE: Immunoglobulin G4-related disease, or IgG4-RD, is a chronic, systemic, inflammatory disease that can affect nearly any and often multiple organs of the body.

It is characterized by silent, ongoing organ damage and recurrent disease flares causing accelerated and irreversible damage to previously affected and new organs.

Donna:

The challenge with me, as he said, was none of this showed up on any scans to begin with. My urologist were coming to see me. He'd like. So you have no symptoms? You know, like nothing. You have no pain. I don't feel, and I. I couldn't tell you right now that my right kidney is dying.

Like I have no pain. I think it's just having something that's just kind of part of your every day. Now. Like, I think about it a lot. I'm always paying attention to it.

I just realized that for the rest of my life, if something shows up on a scan, I'm going to have to be poked, prodded, biopsied, because no doctor is ever going to say, "I see something there. It's definitely IgG4. It's not cancer."

Looking forward for me is really just focusing on getting my IgG4 into remission.

My first advice would always be go see the people who know the most about this. I'm very lucky that I work in a part of the country, I work in a hospital where we do rare, we do complex all the time. There are a lot of people out there that are struggling with this autoimmune disorder that live in parts of the country that don't have access to that.

It needs to be something that all doctors are aware of, because that's probably not the first person you're going to see in your encounter depending on how you manifest. The focus needs to be for them and for all of us on getting the education out there to other clinicians, other doctors, NPs, PAs, anybody that you come in contact with.

I'm very hopeful and very excited about what's to come. As a nurse, I focus more on the patient, and so it grounded me a little bit more and really appreciated where I was at and, stronger empathy for the people who were on the other side, because I know what they went through. I was just lucky.