I was all set to hit you all with a write up about my recent trip to Iceland. Yep, I went again. I told you when I wrote about it last year that I would be back. I don’t know what to say, I just can’t seem to get enough of Reykjavik. As much as I would love to recap and reminiscence about my most recent adventure, I must devote this blog entry to something more important. While the subject matter at hand isn’t travel related, it is very near and dear to my heart. It’s a subject that receives very little recognition, until it claims the life of someone very dear to us or someone with celebrity status. It has claimed the lives of notable figures like Bernie Mac (actor/comedian), Reggie White (NFL), Sean Levert (singer), and Michael Clark Duncan (actor). And on November 10^th, 2016, sarcoidosis claimed the life of my mother, Belinda Arnett.  April is National Sarcoidosis Awareness Month. So let’s talk about it.

What is Sarcoidosis

My first introduction to sarcoidosis began sometime in 1993. My family and I had just relocated from Chicago to Kansas City. Prior to our move, aside from an occasional cold or a flu bug, my mom had always been generally healthy. She was in her late 30’s and never had any significant health issues. But shortly after our move, she started to develop these random symptoms. They were symptoms you really just couldn’t put a finger on. Initially, the disease presented itself as painless skin nodules. Over time, she developed a hacking cough and shortness of breath that would come and go. Later on, she developed visual disturbances. From the internist to the dermatologist to pulmonologist and ophthalmologist, the road to diagnosis was a tedious one. She was misdiagnosed many times before she ever received appropriate treatment. One physician even concluded that the skin nodules were due to a reaction from drinking too much grapefruit juice. Looking back, I certainly wish that were the case.

When we think of autoimmune diseases, we tend to immediately think of lupus. Auto-immune diseases are kind of low on the totem pole when it comes to knowledge and research, but lupus is the most well-known.  Sarcoidosis is an autoimmune disease that causes inflammation and affects different systems of the body. It is characterized by the formation of tiny clumps of inflammatory cells (also called granulomas), in one or more organs of the body. When the immune system goes into overdrive and too many of these clumps form, they can interfere with an organ’s structure and function. If left untreated, chronic inflammation can lead to “fibrosis”, which is permanent thickening or scarring of organ tissue. Sarcoidosis can affect almost any organ in the body, including the heart, skin, liver, kidneys, brain, sinuses, eyes, muscles, bones, and other areas. Sarcoidosis most commonly targets the lungs and the lymph nodes, which are an important part of the immune system. When it affects the lungs, it is called pulmonary sarcoidosis.  As in my mother’s case, ninety percent or more of people diagnosed with the disease have lung involvement.

So what causes it? Well no one knows exactly what causes sarcoidosis, which is why diagnosis and management is so difficult. There is still so much unknown about this disease and there is no objective test which can easily diagnose it. Numerous exams and tests are required to confirm a diagnosis. However, experts theorize that one or more exposures in people who have a specific genetic makeup can cause cells in the body to react and start to recruit inflammatory cells to involved organs, basically an immune response. Some research also suggests that bacteria, viruses, or chemicals might trigger the disease. There are theories that the immune response may be overactive or in some cases inappropriate, and that this results in ongoing inflammation, the formation of granulomas, and in some cases, for scarring or fibrosis to occur.

Credit: American College of Chest Physicians

What are the risk factors?

Sarcoidosis was once thought to be rare, but it is now known to be common and affects people worldwide.  The disease can affect people of any age, race and gender.  However, it is most common among adults between the ages of 20 and 40 and in certain ethnic groups. My mother was diagnosed at the age of 38. In regards to race and ethnicity, in the United States, sarcoidosis is most common in African Americans and people of European “particularly Scandinavian” descent. The disease is slightly more common in women than in men and manifests differently in different groups of people. While the lungs and lymph nodes are affected in almost everyone who has sarcoidosis, African Americans and people of Japanese descent are more likely to have eye involvement than Caucasians. On the other hand, skin lumps are most likely to affect people of Northern European descent, and those with a Japanese background seem prone to sarcoidosis-related heart problems. Research also shows that risk appears to be elevated to some extent if someone in his or her close family has sarcoidosis, although researchers have not yet found a gene or genes linked to the development of sarcoidosis.

Credit: American College of Chest Physicians

Belinda’s course

Over the years, my mother learned to manage the disease and would go through bouts of remissions and relapses. Treatment initially began with steroids and other medications to suppress the immune response. Most times, the medications would work. And sometimes, the disease would go into remission on its own giving her months to years of relief. But during the last few years of her life, after each remission, the disease would return back worse than before. Because of its mystery, the course of sarcoidosis is unpredictable. My mom’s last battle came out of nowhere and we never expected it would be her last. In fact, we were under the impression that she was getting better. But that was further from the truth.

About two weeks before my mother’s death, she complained of a sudden severe headache. Then she experienced generalized weakness and loss of appetite. These were vague symptoms that even the healthiest of people can experience. We usually chalk it up to a flu bug or “maybe I ate something bad.” With no improvement in her symptoms, we made the decision (against her wishes) to take her to the emergency room.  My mother was stubborn and hated hospitals. After being examined, having lab work drawn, and a chest x-ray, the doctors discovered she had abnormally elevated calcium levels, a condition referred to as hypercalcium. Because this condition requires IV therapy and monitoring to resolve, she was going to be admitted for a couple of days. No biggie right? She’ll be home back in time to watch her favorite TV reality shows right? Wrong. While waiting in the ER for a bed assignment, my mother began to complain of the same severe headache she experienced a couple of days prior. Except this time, the headache returned with greater intensity. The nurse promptly came in to assess her and found her blood pressure to be in 200/120 range (yikes, NOT good). As I tried to comfort my mother, she whispered to me, “make it stop” as she touched her head. I said, “it’s going to be alright” and tried to comfort her. But it wasn’t alright. Seconds later, she went into a seizure. I promptly pressed the code button on the wall and yelled for the ER staff. The seizure never resolved so she was intubated (put on a breathing machine) and transferred to the Neuro Intensive Care Unit for further evaluation.

The first week of her course seemed promising. She was followed by an extensive team of doctors and kept in a medically induced coma. Initially we didn’t know what we were dealing with. Was this related to the sarcoidosis? Was this a new issue? After ruling out infectious diseases like meningitis, it was speculated that my mother had an reversible neurological syndrome that causes seizures in response to elevated blood calcium levels. The treatment plan involved keeping her sedated for a couple of days to give her brain a chance to “quiet down” while they corrected it. She would go on to receive a couple of rounds of dialysis. It didn’t work. The calcium levels remained elevated. After further evaluation by the endocrinology team, it was theorized that the sarcoidosis was more than likely the culprit. She was started on high doses of steroids to suppress her immune response, the process that was causing the elevated calcium levels. But, by the time that intervention came into place, my mother developed an entire new set of problems. While her calcium levels improved, she would go on to develop pneumonia, a blood infection (sepsis), and went into complete multi-organ failure. Each organ system shut down one by one. Even after stopping the meds that were used to induce the coma, she never regained consciousness. She had minimal brain activity. Because of her very poor prognosis and quality of life, we were forced to make the difficult decision to end any extreme life saving measures and allow her to pass peacefully. Mom didn’t come home this time. Sarcoidosis finally won.

What the hell happened?

Looking back, I’m not sure what we could’ve done differently. My mother kept up with her doctor’s visits and treatment regimen as instructed. Sure, my mom was stubborn and sometimes frustrated, but she was never negligent. For myself, I often question if getting her to the hospital day sooner would’ve made a difference. I question if I should’ve been more involved in the treatment plan she had with her physician. Yes, I blame myself sometimes. But I guess its human nature to question if we could’ve prevented a loved one’s death. And, I carry the blame burden the heaviest because I’m a nurse.  I have a health care background; I should’ve been able to see this coming. But the reality is, we don’t know and will probably never know.  Sarcoidosis is a sneaky disease. We thought she was getting better and meanwhile, it was quietly destroying her organs, so much so that they couldn’t fight or bounce back once compromised.

How can you help?

Most you reading this will probably never be directly impacted by this sarcoidosis. But if you have this disease, the best advice I can give as a person who has been affected (and also a health care provider myself) is to be very proactive in your care and treatment plan.  Be very vocal and persistent with your physician(s) if it feels like your symptoms are not being managed well or dismissed all together.  If your needs are not being met, change you provider if possible. Everyone’s situation and circumstance are different, so I know this isn’t always easy or possible. And talk to your family. Keep them in the loop regarding your symptoms and treatment plan. If you have a loved one with sarcoidosis, try to educate yourself as much as possible (see resources below).  Be sensitive to your loved ones needs and symptoms. Auto-immune diseases present very differently than other common diseases. A person can feel completely miserable, even when they “look” very well.  And on the other hand, some people can look and present very well, but there are NOT well at all.

Credit: American College of Chest Physicians

Resources

• Foundation for Sarcoidosis Research: Based in Chicago and provides basic information about sarcoidosis, research information, patient information, patient support, and other resources.
• National Sarcoidosis Resource Center: Based in New Jersey and provides links to other sites, videos and books for purchase, and microscopic images of sarcoidosis.
• Sarcoidosis Network Foundation: Based in California and provides general information on sarcoidosis and related events and meetings.
• Sarcoidosis Networking Association: Based in Oregon. Provides basic information on sarcoidosis, links to support groups and other sites, upcoming sarcoidosis meetings and events, and a newsletter.
• FSR Physician Directory: A directory to help patients identify a specialist best equipped to develop and manage sarcoidosis.
• Sarcoidosis | CHEST Foundation: Organization raise sarcoidosis awareness through clinical research, community service, and patient education.
• The Bernie Mac Foundation: A nonprofit organization established by the late comedian and actor, Bernie Mac, to raise awareness and raise funds for sarcoidosis research.

Hopefully my personal story can help someone and raise awareness to bring this disease to the forefront. I implore you to also share your stories and experiences. Until we find a cure, be well.

My beautiful mother, Belinda Arnett (August 13th 1955 – November 10th, 2016). Until we meet again…rest well. #findacure

@urbantravelista