Emapalumab Induces Remission of MAS

Posted on by Grayson

Amazing news! Emapalumab can help treat Macrophage Activation Syndrome (MAS)!

an illustration of ifny
Source

The brand name for emapalumab is Gamifant. It’s FDA-approved to treat hemophagocytic lymphohistiocytosis (HLH), which is pretty similar to MAS in many respects.

The following paragraph is from a summary on Contemporary Pediatrics:

Emapalumab, a fully human anti-interferon-γ (IFNγ), demonstrated efficacy for inducing remission of macrophage activation syndrome (MAS) secondary to systemic juvenile idiopathic arthritis (sJIA) or adult-onset Still’s disease (AOSD) in patients who failed standard care with high-dose glucocorticoids, with or without anakinra (Kineret; Sobi) and/or cyclosporin, according to a recent study.

This is amazing news. MAS is a terrifying secondary condition. It is most common in SJIA and AOSD, but can pop up in lupus and other conditions. If you want to learn more about MAS itself, I covered a session on the condition at the 2017 ACR annual meeting. You can learn more about the immunology on Frontiers.

Normal treatment involves a ton of gluticosteroids and often Anakinra/Kineret. That doesn’t do it for enough patients, though. It’s important to note that there was a small N (or participant number) of 14. However, by week 8, 13 of the 14 patients had achieved remission. That’s not shabby at all.

Most importantly, no deaths were reported during the trial and the long-term follow-up. That’s amazing for a condition that has a mortality rate between 20-53%.

This was a phase II, open-label, single-arm trial conducted in the US, UK, Spain, Italy, and France. I was also happy to see that Dr. Alexei Grom, who I interviewed several years ago, was a part of this study.

Read more of the summary on Contemporary Pediatrics. Or, you can read the full journal article, Efficacy and safety of emapalumab in macrophage activation syndrome, here.

Seeking Chronic Pain Treatment

Posted on by Grayson

Chronic pain affects millions of people worldwide and can have a significant impact on daily life. Unfortunately, many people who suffer from chronic pain struggle to communicate their experience to their healthcare providers. It’s hard to explain this stuff to people who have no clue! That can lead to suboptimal treatment and frustration.

This won’t be as much fun as my sarcastic post on this topic a few years ago. My hope is that this one will actually be helpful.

In this article, I will provide guidance on how to talk to your doctor about chronic pain. From preparing for your doctor’s visit to developing a pain management plan, I will cover key topics to ensure that you and your healthcare provider can work together to manage your chronic pain effectively.

Understanding Chronic Pain

Chronic pain is defined as pain that lasts for more than six months, even after an injury or illness has healed. Unlike acute pain, which serves as a warning signal to the body, chronic pain is a complex condition that can be difficult to diagnose and treat.

It can be caused by a variety of factors, from injuries to acute illnesses to long-term conditions. Other factors, such as stress and trauma, can also contribute to chronic pain. Note that doesn’t mean that pain is all in your head – it means that there are links between these things and pain that are proven.

There are different categories of pain, too. This can be based on where the pain is located, what type of pain it is, and how the pain came to be. Some types of pain include migraines, fibromyalgia, neuropathic pain, musculoskeletal pain, and nociceptive pain.

Preparing for Your Doctor’s Visit

It can be helpful to keep a pain journal to document the severity, location, and duration of your pain. This information can assist your doctor in determining the cause of your pain and developing a treatment plan. Some of us (waves) dislike doing that as it keeps our focus more on the pain than not, but there are still ways to track pain without being so in-depth. For example, I tend to post on social media about rough pain days. Then, the night before an appointment with a provider, I’ll go back over my social posts in the last few weeks to get a better picture of how my pain has been. It’s a much better set up for me. Your mileage may vary.

Make sure that you provide your doctor with a comprehensive medical history. You’ll want to keep them up to date on any changes, including surgeries, injuries, or illnesses. This can help them better understand your pain and tailor a treatment plan to your specific needs.

Finally, make sure to make a list of any questions or concerns you have about your pain. This can help you address all of your concerns during your visit.

Communicating About Your Chronic Pain

Be specific in describing the type of pain you are experiencing. This information can help your doctor determine the cause of your pain and develop an effective treatment plan. Some words that your doctor will understand include throbbing, radiating, intermittent, constant, and burning.

Use a pain scale to describe the intensity of your pain. That seemingly medieval one isn’t helpful for me, and it’s okay if it isn’t for you, either. Instead, I like to use the comparative pain scale. It’s much easier to use to communicate limitations and discuss how your pain affects your daily life. That includes activities that you are no longer able to do or how being in pain impacts your mental health. All of this information can help your doctor develop a treatment plan that addresses the impact of your pain on your quality of life.

I’ve pulled together a slightly better version of the pain scale, questions your provider may ask, questions you may want to ask, and ways to track your pain to can help you discuss with your providers here:

"discussing chronic pain with your providers" with a photo of two people speaking with a provider

If you’d like to edit this on your own Canva account, click here and make a copy. If you’d like the PDF version, click here.

Discussing Treatment Options

Your doctor may recommend medications like nonsteroidal anti-inflammatory drugs (NSAIDs) to help manage your chronic pain. They might even recommend opiates or similar medications. This is less likely due to ongoing policy issues, at least in the United States.

Medications can be effective in reducing pain, but they can also come with side effects that may impact your daily life. Be sure to ask about potential side effects before starting or changing medications. It’s important to have an open and honest conversation with your doctor about what types of medications and therapies you’ve tried in the past, and what has or hasn’t worked for you. It’s also a good idea to keep track of any side effects you experience so you can discuss them with your doctor at your next appointment.

In addition to medications, there are several alternative or complementary therapies that can help with chronic pain.

Complementary medicine refers to practices that are used alongside traditional medicine to help manage pain and other symptoms. These may include herbal remedies, supplements, or mind-body practices like tai chi. Before trying any complementary medicine practices, be sure to talk to your doctor to ensure they are safe and won’t interact with any other medications you’re taking.

Alternative therapies, such as acupuncture, massage, and hypnosis, may be effective in managing chronic pain. Some alternative therapies may interact with medications or be contraindicated for certain medical conditions. So, again, talk to your doctor first.

Physical therapy can be an effective treatment option for some types of chronic pain. Moving can be helpful to reduce pain levels for some people. As someone with Ehlers Danlos Syndrome (EDS), I work with a physical therapist to strengthen and readjust how to move my body. It’s made all the difference. Your doctor may refer you to a physical therapist to develop a personalized treatment plan.

In some cases, surgery may be helpful or necessary to alleviate chronic pain. Your doctor can discuss surgical options with you and help determine whether it is an appropriate treatment option for your specific condition.

Developing a Chronic Pain Management Plan

When it comes to managing chronic pain, it’s important to have a personalized plan in place. Work with your doctor to craft a pain management plan that takes into account your unique needs and circumstances. This may include a combination of medications, therapies, and lifestyle changes.

When developing your plan, it’s important to set realistic goals. It may not be possible to completely eliminate your pain. That said, you can work towards reducing your symptoms and improving your overall quality of life.

Having a support network can also be an important part of your pain management plan. This may include friends and family members who can provide emotional support, or healthcare professionals who can offer guidance and expertise. This also includes taking care of your emotional and mental health.

Chronic pain takes a toll on everything, but especially emotional and mental health. It’s important to address these needs as well as your physical symptoms. The mind-body connection plays a crucial role in managing chronic pain. Stress, anxiety, and depression can all exacerbate pain symptoms. That’s especially true for conditions like fibromyalgia, EDS, and many types of arthritis.

Developing strategies for managing these emotions is key for overall health. Some people enjoy meditation or deep breathing exercises. Others find more relief through working with a therapist. If you’ve had traumatic experiences, working with a therapist to unpack that trauma can help with pain levels, too. It’s amazing how much our bodies really do keep the score.

Support groups can also be helpful. These can provide a safe space to talk about your experiences with others who understand what you’re going through. I personally am not the biggest fan, as these can often devolve into “I have it worse” conversations. That said, the groups run by the US Pain Foundation are the absolute best I’ve found. They even have specialized groups for the LGBTQ+ community, the BIPOC community, teens, and more. The LGBTQ+ group is run by my friend Sara Gehrig who is amazingly compassionate and creates a space safe for all members of the community. No transphobia here!

Follow-Up and Continued Care

Managing chronic pain is an ongoing process that requires continued care and attention by you and your provider.

Keep track of your symptoms and progress. Share this information with your doctor at each appointment. This can help you and your doctor make informed decisions about your treatment plan, including any updates or adjustments that may need to be made.

If you’re not getting relief from your pain, it’s okay to seek additional help. Same if you’re experiencing new or worsening symptoms that your provider is struggling to work with. Your doctor can refer you to pain specialists or other healthcare professionals who may be able to offer additional treatments or support.

Now, if you feel like your healthcare provider is not taking your chronic pain seriously? It may be helpful to seek a second opinion. Additionally, you can advocate for yourself by being clear and specific about your pain experience, bringing a pain journal to your appointments, and asking questions about treatment options. It’s also okay to bring a buddy with you to help you advocate, especially if you’re bringing someone who holds identities of power that you don’t. For instance, a Black person may want to bring a white person with them if they’re concerned that their provider isn’t listening to them because of race or ethnicity. A woman may want to bring a man if they’re concerned about sexism.

Remember, you have the right to adequate pain management. Even more than that, you deserve to work with healthcare providers who take your pain seriously.

Takeaway

Talking to your doctor about chronic pain can be challenging, but it is essential for finding the best treatment options. By communicating your pain experience, discussing treatment options, and developing a pain management plan, you can work with your healthcare provider to manage your chronic pain effectively. Remember to follow up with your doctor, track your progress, and adjust your treatment plan as needed. With the right approach, you can improve your quality of life and manage your chronic pain.

Goodbye Mighty Casey

Posted on by Grayson

Have you ever met someone who completely changes your life in the best ways? Who shows you that you can be yourself and change the world for the better, even if you’re not always offered a seat at the table?

Mighty Casey Quinlan smiles at the camera

Casey Quinlan was that person for me. We met on social media and had followed each other for a while. It wasn’t until the ePharma 2016 summit in NYC that we got to meet in person. While she was speaking, a pharma person at my table was grateful she was wrapping up because “she’s scary.” She was challenging the status quo.

I dm’d Casey about this, and she immediately came over to the table and flipped this guy the bird behind his back while dancing and sticking out her tongue. It was the most amazing thing to see.

a group of patients in a photo together

I worried for years about challenging the status quo. In spaces I’d done that before, I was kicked out or not invited back. Casey showed me that it didn’t matter if I was invited back. I had to speak truth to power whenever possible.

Casey and I stayed in touch, chatting regularly. The next time we got to hang out, it was in DC. She took me to her favorite place, the Old Ebbitt Grill. I don’t even remember what kind of cocktail she recommended that I get, but it was good. For the better part of the day between Ebbitt and the hotel bar, we talked about our lives, changing healthcare, and the lack of support for trans folks.

In the years between NYC and DC, I’d come out as trans and begun the process of transitioning. I’d lost several friends, some due to their full on transphobia and others who refused to see they were causing harm. Casey was the one person who I knew worked on getting it right and helping others do so.

a photo of a painted jacket - there is a contract in the background with a hand flipping the bird in the foreground and text "This aggression will not stand, man"

She knew it was important to highlight inequity. On her podcast, she did this regularly, whether that involved speaking truth into the void or inviting folks on to talk about everything that was happening in the world.

I didn’t grow up with a supportive family. I haven’t talked to my mother in almost a decade and never truly felt at home there anyway. But, I did feel at home wherever Casey went.

Back on the 10th, my partner and I drove out to visit Casey in hospice. The two of them hadn’t met and, while hospice wasn’t the best meeting place, they got along well. I told Casey what she meant to me, who she was for me. I will never forget how she turned that around to remind me that I helped her and others, too.

Ever the snarky human, she told me that she loved me to death as we squeezed each other’s hands and I kissed her forehead.

I hate that you’ve gone where I cannot follow, my friend. But I know that you’ll make sure that place gets its shit together before those of us following in your footsteps to change the world get there.

I love you to death, Mighty Casey.

Casey with a QR code on her chest

ACR Endorses Strengthening Medicare for Patients and Providers Act

Posted on by Grayson

The following is a press release issued by the ACR as of yesterday, April 24, 2023:

Today, the American College of Rheumatology (ACR) commended the introduction of the Strengthening Medicare for Patients and Providers Act (H.R. 2474), a bipartisan bill intended to update Medicare physician payments to reflect the impact of the broader economy on physician practices.

The legislation, introduced by Reps. Raul Ruiz, M.D. (D-CA), Larry Bucshon, M.D. (R-IN), Ami Bera, M.D. (D-CA), and Mariannette Miller-Meeks, M.D. (R-IA), would adjust the Medicare Physician Fee Schedule (MPFS) based on inflationary updates measured by the Medicare Economic Index (MEI).

“For too long, specialty providers, like rheumatologists, have faced considerable uncertainty regarding their ability to continue providing needed care to patients,” said Douglas White, MD, PhD, president of the American College of Rheumatology. “The Strengthening Medicare for Patients and Providers Act represents a long-overdue adjustment to the Medicare Physician Fee Schedule that will help stabilize physician practices and ensure that beneficiaries have timely access to rheumatological care.”

The MPFS is the only major fee schedule without an automatic inflationary update. Consequently, Medicare provider reimbursement has failed to keep pace with broader economic realities. Recent analysis from the American Medical Association (AMA) demonstrates that when adjusted for inflation, Medicare physician payments declined 26% from 2001 to 2023, as consumer and practice costs rose.

“This legislation would allow Medicare to more accurately reflect the cost of practicing medicine, which has increased dramatically in recent years,” said Christina Downey, MD, chair of ACR’s Government Affairs Committee. “ACR looks forward to working to advance this important policy reform that will help build a sustainable payment system and protect access to care for patients with serious, chronic diseases.”

Taiwan: Actemra Approved to Treat COVID-19

Posted on by Grayson

The following is a press release issued today by Chugai Pharmaceutical:

Chugai Pharmaceutical Co., Ltd.(TOKYO: 4519) announced that Chugai Pharma Taiwan Ltd., a wholly-owned subsidiary of Chugai, obtained an import drug license from the Taiwan Food and Drug Administration (TFDA) for Chugai’s Actemra® (tocilizumab) intravenous (IV) formulation for the treatment of COVID-19 in hospitalized adult patients who are receiving systemic corticosteroids and require supplemental oxygen, non-invasive or invasive mechanical ventilation, or extracorporeal membrane oxygenation (ECMO).

This approval is based on the results from clinical studies evaluating Actemra in hospitalized patients, including an investigator-initiated, randomized, open-label, platform overseas study (RECOVERY study) and three placebo-controlled, randomized, double-blind, multicenter, global phase III studies conducted by Roche (COVACTA study, EMPACTA study, REMDACTA study).

About Actemra

Actemra is the first therapeutic antibody created in Japan by Chugai. It is designed to block the activity of IL-6, a type of inflammatory cytokine. First launched in June 2005, the intravenous injection is approved for seven indications in Japan: Castleman’s disease, rheumatoid arthritis, systemic juvenile idiopathic arthritis, polyarticular juvenile idiopathic arthritis, cytokine release syndrome induced by tumor-specific T cell infusion therapy, adult Still’s disease, and SARS-CoV-2 pneumonia. In addition, Actemra subcutaneous injection is approved for three indications in Japan: rheumatoid arthritis, Takayasu arteritis, and giant cell arteritis. Actemra has obtained regulatory approval in more than 110 countries worldwide.

As you may know, Actemra is one of a small number of medications FDA-approved for both SJIA and AOSD.

"Things to ask before okaying medication changes" with a photo of a provider discussing a topic with two people

Evaluating Medication Changes

Posted on by Grayson

Before making any changes to your medication regimen, it’s important to carefully consider the possible risks and benefits. Your doctor may recommend a medication change to better manage your symptoms or improve the effectiveness of your treatment. However, it’s crucial to fully understand why the change is being made and what the potential side effects or interactions may be.

In this article, I’ll outline some of the key things you should ask before okaying medication changes, including understanding the risks and benefits, discussing alternatives, and monitoring your health. By being an active participant in your healthcare decisions, you can make informed choices about your medication and ensure the best possible outcome for your health.

Continue reading “Evaluating Medication Changes” →

"What is patient-centered care?" with a photo of a provider and a patient speaking

What is Patient-Centered Care?

Posted on by Grayson

Patient-centered care has become a buzzword in healthcare in recent years, but what does it really mean? At its core, patient-centered care is an approach to healthcare delivery that prioritizes the needs, preferences, and experiences of patients. It emphasizes the importance of communication, respect, and shared decision-making in the patient-provider relationship.

While the benefits of patient-centered care for patients and providers are well-documented, implementing this approach in healthcare systems can be challenging. In this article, we will explore the definition and core principles of patient-centered care, its benefits for patients and providers, challenges and barriers to its implementation, and examples of successful patient-centered care programs.

Defining Patient-Centered Care

Patient-centered care is a healthcare approach that prioritizes the needs and preferences of patients, placing them at the center of their healthcare journey. It is a collaborative process where clinicians work with patients to address their unique needs, values, and goals. This approach emphasizes the importance of communication, respect, and individualization in healthcare delivery. It is a holistic approach that considers the patient’s physical, emotional, and social needs. This approach recognizes that patients are unique and have different preferences, values, and goals.

The concept of patient-centered care has been around for decades. It wasn’t until the 1980s that it gained mainstream attention, though. This was in response to patients’ dissatisfaction with the traditional, doctor-centered approach to healthcare that focused more on diagnosis and treatment than on their needs and preferences.

Core Principles

Patient-centered care is built on core principles that guide healthcare providers in their interactions with patients. Patient autonomy and shared decision-making are central to patient-centered care.

We know that patients have unique needs and preferences. This approach involves tailoring care to the individual patient, rather than a one-size-fits-all approach. It involves providing care that is responsive to the physical, emotional, and social aspects of a patient’s life.

Effective communication and active listening are key to patient-centered care. Healthcare providers must listen attentively to patients to understand, not to respond. Then, they work together to develop a care plan that addresses the patient’s needs and preferences.

Benefits for Patients

Patient-centered care has several benefits for patients. It leads to higher patient satisfaction rates. Patients feel heard, respected, and involved in their care, too, which leads to a more positive patient experience.

There are also a ton of links between this type of care, improved health outcomes, and reduced health disparities. When patients are involved in their care and feel empowered to make decisions, they are less likely to run into barriers around treatment. That results in better health outcomes overall and a lower likelihood of visiting the ER or being admitted to the hospital.

Most of all, this setup empowers patients to take an active role in their healthcare journey. Giving patients who have can’t control their conditions a sense of control and ownership in their care makes a huge difference. In fact, it leads to increased engagement and motivation to manage their health.

Benefits for Healthcare Providers & Systems

Patient-centered care benefits not only patients but healthcare providers as well. Provider-patient relationships improve due to better, more effective communication and collaboration. Having a more positive and trusting relationship between the healthcare provider and patient leads to better ratings.

Providers who practice patient-centered care often report higher levels of professional fulfillment and job satisfaction. This approach allows them to build meaningful connections with patients and truly see the positive impact of their work on patient outcomes. Accounting for barriers and working around them also helps providers worry less about their patients. Let’s be real – being a provider is hard. They take their work home, too.

Speaking of that, patient-centered care is linked to reduced burnout and turnover rates. A more positive work environment encourages collaboration and communication outside the exam room, too. Stress levels drop and work can feel less chaotic.

This manner of care can also lead to better ratings and fewer costs for the healthcare systems overall.

Implementation in Healthcare Systems

This isn’t just a concept. It’s a practice that healthcare organizations can incorporate into their culture. Here are a few ways healthcare systems can implement patient-centered care:

  • Create a culture that values the patient’s voice and experience. Empowering patients to be active participants in their own care can also help systems uncover barriers and issues internally. Involving patients and their families in quality improvement projects, such as Patient and Family Advisory Boards institutionalizes that work, protecting it moving forward and leading to even more improvements.
  • Offer training on cultural competency and health literacy to help providers better serve diverse populations.
  • Offer regular training to providers that is focused on patient-centered communication skills. This can improve their ability to listen, understand, and respond to patients’ needs.
  • Adapt to new technologies that allow for improved patient engagement and communication. For instance, telemedicine, remote monitoring devices, and electronic medical record portals increase engagement across the board. These technologies provide a more personalized approach to care delivery. Telemedicine in particular offers the clinicians the ability to see what a patient’s home life may be like, which can lead to addressing other barriers or socioeconomic issues.

There are many examples of successful patient-centered care programs. Some of these include the Mayo Clinic Model of Patient-Centered Care, patient-centered medical homes and primary care redesign, and the Patient-Centered Outcomes Research Institute (PCORI).

Challenges and Barriers

While patient-centered care has many benefits, there can be challenges and barriers that affect its implementation. Some insurance and reimbursement models may not incentivize patient-centered care. While this is ridiculous, it can make it difficult for healthcare providers to prioritize patient needs over financial considerations.

Language and cultural differences between patients and providers can cause misunderstandings and hinder effective communication. You can’t provide patient-centered care if you can’t relate to or center the patient in discussions.

Some healthcare providers may also be resistant to change, prioritizing traditional medical models over patient-centered approaches. This paternalism limits the adoption and success of patient-centered care. That’s especially true when healthcare systems don’t address paternalistic providers.

Future of Patient-Centered Care in Healthcare

As healthcare continues to evolve, patient-centered care will become increasingly important. As we think ahead to the future, here are some ways that this practice may show up:

  • Conducting clinical trials and further research of patient-centered care programs. This will be critical to improving outcomes and expanding successful programs.
  • Scaling of successful programs, adjusting them for different settings. What works in New York City likely won’t work in Appalachia, but we can imagine what changes we could make to improve and institute a successful program in Appalachia.
  • Increasing access to technology. This could create new opportunities for patient engagement. It may even help us to work through how changes in insurance and reimbursement models could create new challenges.
  • Developing better tools to measure patient engagement. These need to take into account issues such as socioeconomic status, oppression and discrimination, and equity issues.

Patient-centered care plays a critical role in improving the quality of healthcare delivery and patient outcomes. While it may not be easy to implement, the benefits of this approach for patients and providers are clear. By prioritizing patient needs, preferences, and experiences, healthcare providers can build stronger relationships with their patients and ultimately provide better care. As the healthcare landscape continues to evolve, it will be important to continue to prioritize patient-centered care and find new ways to meet the unique needs of patients.

ACR: Medicare Drug Pricing Negotiation Methodology Should Include Real-World Experience

Posted on by Grayson

The following is a press release issue by the American College of Rheumatology on April 14, 2023:

The American College of Rheumatology (ACR) today submitted comments to the Centers for Medicare and Medicaid Services (CMS) administrator Chiquita Brooks-LaSure urging that Medicare’s drug price negotiation program balance cost with innovation, incorporate real-world experience from patients and providers, and improve transparency in the program’s methodology.

In its comment letter, the ACR urges CMS to:

  • Ensure that drug pricing timelines and eligibility requirements for negotiating high-spend drugs do not impede innovation and the development of new treatments.
  • Allow greater public participation as the process evolves, including public comment periods of at least 60 or 90 days.
  • Incorporate patient and provider perspectives throughout the process.
  • Make drug negotiation methodologies transparent, accessible, and understandable to all stakeholders.
  • Publish subsequent policies regarding drug price negotiations using the customary regulatory process including a more appropriate public comment period.

“While negotiations toward a maximum fair price are largely between the pharmaceutical industry and CMS, the implications of these negotiations are far-reaching,” said Douglas White, MD, PhD, President of the American College of Rheumatology. “We firmly believe that the real-world experience of patients and prescribers must be integrated into any attempts to identify and negotiate fair drug prices. The ACR appreciates the opportunity to contribute its experience to CMS’s process.”

Michael J. Fox and Rebuking Ableism

Posted on by Grayson

Person off-screen: “The sad sack story is Michael J. Fox gets this debilitating disease and it crushes him.”
Fox: “Yeah, that’s boring.”

On May 12th, Apple TV is releasing Still: A Michael J. Fox Movie. I am SO excited to watch this look back at not only his career but his early Parkinson’s journey.

Like many people, Fox felt like he had to hide his condition from the public. No one outside of his family knew for a long time. I’m sure there were many factors to this, but chief among them is ableism.

People won’t hire disabled folks for roles, assuming they can’t do specific actions or out of worry about what to do if the star’s condition worsens. Instead of being able to show up authentically in the workplace, many actors with disabilities have to hide them in order to continue working. In the meantime, they get to deal with tabloid commentary about their weight, appearance, family life, and more.

Perhaps what I’m most excited about is the interaction I opened this post up with – that focusing on a pity party or rooting this film in ableism is boring. It is! “Pity is a benign form of abuse,” Fox has said. He’s not wrong. More than that, films that lean that way act as though disabled folks are already dead, that our lives hold no more meaning.

As he says in the below around the 1:50 mark, “I love the idea that disabled people can be assholes, too.”

It’s beyond refreshing to see someone so well-known rebuke that idea and focus on sharing what real life with his disability is like. We’ve seen that with celebrities who have recently come out with their disabilities, including Selma Blair. It’s nice to see it coming from someone who played such a pivotal role in many of our favorite shows and films from the 80s and 90s.

That’s doubly true when the Michael J. Fox Foundation does such amazing work. This week, they announced a breakthrough in the search for a biomarker for Parkinson’s – “a biological test for Parkinson’s disease that demonstrates high diagnostic accuracy, differentiates molecular subtypes and detects disease in individuals before cardinal movement symptoms arise.” This will lead to early detection and treatment as well as a better understanding of this condition. It will save lives, and it could very well be an important stepping stone to a potential cure or at least better treatments.

Fox had this to say about the discovery: “There are many ways I am involved with the work of the Foundation, but I come to this result first and foremost as a Parkinson’s patient. I am deeply moved by this breakthrough and endlessly grateful to the researchers, study participants and funders who have endeavored to bring us this far. When we started PPMI, we weren’t casting about for fish — we were going after a whale. Now, here we are. Together we are making a cure for Parkinson’s inevitable.”

Fox has won multiple awards for his activism, but I missed him receiving the Jean Hersholt Humanitarian Award four months ago. If you did, too, it’s worth a watch:

I am so excited to see Fox being more present and better recognized for the work he has done and continues to do.

To close this out, I’ll share another one of my favorite lines in the Still trailer – one that likely won’t surprise you, given the name of this site.

Person off-screen: “What did it mean to be still?”
Fox: “I wouldn’t know. I was never still.”

graphic with the title 'orthostatic hypertension' featuring an illustration of a dizzy person with a heart on their shirt

Orthostatic Hypertension

Posted on by Grayson

Back in December, I had a long day of tests at the Cleveland Clinic. After speaking again with the provider who ordered these tests, she shared that, while she did not think I had POTS specifically, she did think I had another, less common form of dysautonomia.

 

Orthostatic Hypertension

Orthostatic hypertension is when someone’s blood pressure suddenly shoots up when they stand. Generally, this requires a rise in your systolic BP (the top number) of at least 20 mmHG. We don’t know what causes this, but some conditions that are related are anorexia, inflammation of the aorta, narrowing of the kidney arteries, and type 2 diabetes.

According to a 2008 study, this condition affects just over 1% of the population. This condition isn’t super well known, meaning that there aren’t necessarily concrete treatment regimens for it.

Now, does this mean I don’t have POTS? Actually, no! People can have both. Some providers would still cause this POTS, but specify that it is hypertensive. There’s not a lot of agreement on how to diagnose or label this correctly honestly. Furthermore, this is a form of both orthostatic intolerance and dysautonomia, so it’s still in the same genre. To me, orthostatic hypertension makes far more sense as a diagnosis based on my symptoms and labs – and that’s true looking back a ways.

 

Cardiology + Treatment

Ritalin – which I’m on for my ADHD – can also help treat forms of orthostatic intolerance. We see this specifically in conditions like POTS. However, we know it can also cause hypertension on its own. So can testosterone.

Thankfully, the cardiologist that I saw is amazing:

I immediately was put at ease. I can’t think well without my Ritalin. Going off testosterone would literally cause an uptick in my chronic pain. Plus, I’d feel awful from experiencing the effects of estrogen again. There’s no way I was going to budge on these things anyway.

So, we’re starting with a medication to lower blood pressure called doxazosin. There is some evidence to suggest that this is a helpful medication for this condition. This medication is also an alpha-adrenergic blocking agent, a class of medications that is used to treat PTSD and nightmares.

I’ve also stopped my increased salt pills and diet, something that a previous provider recommended when he gave me a differential diagnosis of POTS based on symptoms and comorbidities. (No, he didn’t want to put me through a tilt table test at the time. I’m annoyed I didn’t press further.)

I’m testing my blood pressure at random times to be able to show how things are going at my next appointment. So far, though, I’ve had a general lowering in my systolic numbers which is great!

 

Outlook

Dean and Hank - black and white mix dogs - look out a window while leaning on a couch
Dean and Hank watching the UPS dude grab my Kineret this morning

Well, since this condition isn’t well-known, it’s not as well-studied as I’d like. Orthostatic hypertension is associated with higher risks of heart attack, stroke, and early death. Obviously, the goal is to not have those things happen. If we decide this medication isn’t working as well as we want at the end of this trial period, we’ll re-evaluate medications and see what else we might need to do.

I’m paying more attention to my both my pain and stress levels, as these can send blood pressure soaring. I am historically awful at balancing both of these things. Being in the middle of countries around the globe attempting to pass a record-setting number of transphobic legislation is not helping. This is part of why we talk about how stress kills marginalized and underrepresented folks.

I also had to fight all year to re-obtain access to Kineret after an insurance switch. (It’s been a whole thing.) My month-long shipment of meds arrived today. That along with having re-started physical therapy will both be beneficial.

If I could share one thing with folks, it’s this: get a tilt table test done before you accept a differential diagnosis of POTS. I’m kicking myself for taking steps that would’ve been great for POTS but probably ultimately will have taken years off my life because I received incorrect information. I don’t want that to happen to other people.