Giuliana Grossi

Wonder Drake, MD, is a professor of medicine in the Division of Infectious Diseases at 

Vanderbilt University Medical Center (VUMC)

. She's also the director of the university's Sarcoidosis Center of Excellence, as well as a member of the World Association for Sarcoidosis and Other Granulomatous Disorders (WASOG) executive committee.

Drake chose sarcoidosis research to be the focus of her career because she wanted to further the understanding of sarcoid pathogenesis. When she realized that a patient she was treating had the disease it made her wonder, "could we develop something that would have cured it?"

"It just really impacted me that a disease could just take this young person who, you know, was married and working hard with 2 little kids, and I'm about to put them on disability," she said.

 that's characterized by the growth of granulomas, or collections of tiny inflammatory cells, throughout the body. They often present in a patient's lungs and lymph nodes but can also affect the eyes, skin and heart.

The incidience of the disease within the US falls among African Americans, with a particularly heavy burdern on African American women. According to Drake, this suggests that not only are genetics an important factor, but hormone status is too.

Because there's no straightforward test to determine a diagnosis, it can be difficult to detect. When Drake's patient first presented with fatigue and weight loss, she initially suspected HIV but it turned out that he had sarcoidosis involvement in his kidneys and was going into renal failure. 

"It's tough," she said, "because the disease is a disease of exclusion."

Drake approaches a suspected case of sarcoidosis by ruling out other possibilities like infections, cancers and lymphomas that can also cause granulomas.

"So, you really just rule out all these other diagnoses and then if the clinical picture fits, then you tell the patient that they have sarcoidosis," she said.

Videos

Sarcoidosis expert Dr. Wonder Drake got into the field after diagnosing a patient with the disease early in her career. In this interview she explains the best way to approach a suspected case of sarcoidosis. 

Wonder Drake, MD: Sarcoidosis is a Disease of Exclusion

California Sickle Cell Disease Mental Health and Wellness Initiative

 to provide mental health services to those with the disease, at no cost. In addition to offering services on site, part of the initiative is to provide these services virtually so any patients in California can participate in treatment. 

Carolyn Rowley, PhD, is the executive director of the Cayenne Wellness Center, which is devoted to supporting people living with 

, Rowley reflected on her extensive experience in the field and shared the story of a patient who helped her realize just how imperative mental health support is.

When she got a call from a hospital nearby about a teenage patient with sickle cell disease experiencing a vaso-occlusive episode, or a pain crisis, she made the hour and a half drive and Bobby became one of her first patients.

While Bobby's condition appeared to improve, he was refusing to leave the hospital. Rowley explained that essentially, he didn't want to live any longer. 

"My role was to convince him that life is worth living," she said. "So, I was on a mission, and I was going at least twice a week. And I'm also an adult person with sickle cell disease. So, I'm here to, you know, to be a model and say that 'life is so great and yes, yes, there is pain, but life is great, and you can have these wonderful peak experiences,' etc, etc."

Unfortunately, medical appointments and episodes of severe pain are not the only challenges these patients face. Stigma and bias are still prevalent for those with the condition, not only in society but within the healthcare community as well. 

So, when Bobby asked her questions like, "Dr. Rowley, can you guarantee that if I decide to live that I will no longer be called outside my name as a 'sickler'?", and "Can you guarantee that I'll be believed the next time that I come [to the emergency department], and that I won't have to wait a long period of time, and that I'll get what I need?", she was honest.

"No," she said, "there's these biases and prejudices and we're here to advocate. That's what we do. I can't guarantee that it won't happen, but I can say that we'll make sure that it doesn't happen as long, and that we'll get involved."

Unfortunately, knowing his experience was not the last time he would "be called outside his name, knowing that he'll be seen as a 'frequent flyer', as a drug seeker, etc. -- it wasn't good enough for him." 

"So, as I continued to see him, I realized that he had already resigned, and he was going to allow his body to atrophy," Rowley explained. "So, pretty soon I had to just accept and just go there to be friends."

The initiative is setup to enroll patients in 15 or more cognitive behavioral therapy (CBT) sessions because, according to Rowley, it's effective with depression, anxiety and sleep disorders and its shown to help patients improve shortly after they begin. 

Also, the basis of CBT is for patients to develop tools to manage their own mental health and become aware of thinking patterns, irrational thoughts and other intrusive mental behaviors in order to address them. 

"So, I do this for Bobby," Rowley said. "I don't want anyone else at 18 to say, 'I'm done. I'm done. I can't do this anymore, it's too much to not be believed, too much to have to suffer this way.' And that's why we do it, and that's why mental health support is so, so important."

Dr. Carolyn Rowley talks about the Cayenne Wellness Center's mental health initiative for individuals with sickle cell disease and shares the story of one of her first patients.

Carolyn Rowley, PhD: Patients with Sickle Cell Disease are Born into Pain

 that's characterized by the growth of granulomas, or collections of tiny inflammatory cells, throughout the body. Typically, granulomas present in a patient's lungs and lymph nodes. Among other organs, they've also been known to affect the eyes, skin and heart.

. In an interview with HCPLive, she explained how incidence and presentation of sarcoidosis can vary by region.

In terms of worldwide incidence, the Netherlands has the highest rate of the disease, followed by Japan and the southeastern US. Drake's focused her career on deepening the understanding of why some patients with the disease progress while others don't.

"Even though the highest incidence is in the Netherlands, they're also the group that is more likely to resolve their disease," she said. "And then the Japanese, their disease is more sarcoidosis of the eyes and the heart, more so than sarcoidosis of the lungs and the skin, which is predominantly what we see here in the United States."

The incidience of the disease within the US falls among African Americans, particularly African American women. According to Drake, this suggests that not only are genetics an important factor, but hormone status is too.

In 2016, the Sarcoidosis Center of Excellence was established at VUMC and has been under Drake's direction since. She's also a member of the World Association for Sarcoidosis and Other Granulomatous Disorders (WASOG) executive committee where she serves as associate chief editor.

Living with a rare disease, or treating one, is associated with various challenges. Overcoming a lack of awareness is a fundamental obstacle surrounding sarcoidosis. Drake thinks the creation of networks within the field, like the 

Foundation for Sarcoidosis Research (FSR)

, is an important resource for providers and patients. 

"One of the things that I've been really impressed with the Foundation for Sarcoidosis Research is that they've done a really good job of connecting general practitioners, or even specialists not familiar with sarcoidosis, to providers who are very comfortable taking care of patients with sarcoidosis in each state," Drake said.

"So, no matter where you are," she continued, "they have a listing and you can find providers that are quite comfortable taking care of patients with sarcoidosis."

In this interview, Dr. Wonder Drake shares physician resources and explains the unique presentation of sarcoidosis and how it varies by region.

Wonder Drake, MD: Understanding the Progression Patterns of Sarcoidosis 

A new retrospective cohort study evaluated opioid-naive patients who were prescribed at least one opioid during an inpatient hospital visit to determine baseline factors associated with a new diagnosis of opioid use disorder (OUD). The ultimate goal was to better understand factors associated with the disorder in order to inform pain management strategies in this setting.

The team of investigators, led by Elizabeth C.S. Swart, BS, UPMC Centers for High-Value Health Care and Value-Based Pharmacy Initiatives, UPMC Health Plan, alluded not only to the 2.5 million individuals living with OUD in the US, but also the economic impact of this epidemic.

In the US, the cumulative societal cost of opioid use disorder is nearly $80 billion and has been associated with the rise of illicit drug use. Investigators acknowledged the numerous methods of response to this crisis, including policy and treatment approaches aimed at harm-reduction, medication-assisted treatment (MAT), Good Samaritan laws and increased availability of naloxone.

“Other efforts to curtail the epidemic have mainly focused on reducing inappropriate prescribing,” they wrote, “which have led to decreased opioid prescriptions; however, rates of OUD, nonmedical opioid use, and opioid-related overdose deaths remain prevalent.”

While many studies have evaluated inpatient opioid administration, this study explored baseline characteristics including medication use pre-admission, during hospitalization, and at discharge to identify associations with a new diagnosis of opioid use disorder in the year following hospitalization.

"To our knowledge, this is one of the first studies to evaluate patient- and medication-related risk factors in the development of OUD in opioid-naïve inpatients exposed to opioids during hospitalization," investigators wrote. "The inpatient setting is a critical setting for opioid exposure and can serve as a conduit to long-term opioid use."

Investigators developed a dataset of electronic health records (EHR), pharmacy claims, and medical claims that reflected data of patients aged 14 or older who were discharged between 2014-2017 from UPMC Health Plan, a vertically integrated healthcare delivery and finance system. Patients who were diagnosed with OUD, or prescribed an opioid prior to the study onset were excluded.

Out of the final sample of 23,033 opioid-naive patients, 2.1% were diagnosed with the disorder within a year of receiving their first opioid in the hospital. Within that population, investigators discovered that a substantial number of those individuals were young and white, residing in high opioid geo-rank regions and had a history of nonopioid related drug disorder, tobacco use, and gabapentin use.

When examining the 6 months prior to hospital admission among those with a new OUD diagnosis, investigators observed that the average number of prescriptions filled were higher, the number of days’ supply was higher, and prescriptions filled for gabapentin and skeletal muscle relaxants were higher, compared with those without a new diagnosis. 

"Notably, although a baseline history of skeletal muscle relaxant use was more common in those who were diagnosed with OUD, in our multivariable model it was not associated with subsequent OUD" they wrote. "This is contrary to similar studies which looked at factors influencing long-term opioid use among opioid-naïve patients."

Adjusted multilevel mixed-effects logistic regression models were used to indicate the factors associated with a new opioid use disorder diagnosis within 12 months of discharge.

“Overall, presence of chronic liver disease and being discharged to a long-term hospital, rehabilitation center, or special needs facility were significant factors for patients in the surgery cohort who developed OUD (

< .0001). For patients who did not receive surgery, opioid geo-rank was significant at 

< .0001 for those diagnosed with OUD compared to those who were not.”

General characteristics that were associated with higher odds of a diagnosis included being between the ages of 25–34, compared to those 65 years of age and older. When looking at race, White patients were associated with higher odds of a diagnosis.

Medical history and comorbidites played a significant role. Previous history of chronic liver disease was associated with higher odds of an OUD diagnosis in the surgery cohort. Those with nonopioid-related drug disorder (excluding cannabis and cocaine), tobacco use disorder, and mental health conditions also demonstrated higher odds of diagnosis.

Investigators noted that the use of benzodiazepines or skeletal muscle relaxants in the 12 months prior to index hospitalization event was not associated with an OUD diagnosis; however, use of gabapentin 12 months prior to index date was. 

Additionally, patients discharged to long-term hospitals, rehabilitation centers, or special needs facilities instead of going home were more likely to receive a diagnosis. 

"Future research is needed to evaluate if identification of high-risk patients can mitigate risk of subsequent OUD through proactive effective pain management strategies that include reducing opioid exposure when feasible," investigators concluded.

Patient and medication-related factors associated with opioid use disorder after inpatient opioid administration

Articles

Presence of chronic liver disease, prior use of gabapentin and being discharged to a long-term hospital or rehabilitation center are among some of the factors associated with a new opioid use disorder diagnosis.