How Medicare/Medicaid’s New Proposed Law Could Harm Millions of Patients
Written by Jennifer Robin Kulik and Arielle Davis
The Centers for Medicare and Medicaid Services are proposing new legislation (CMS-9926-P) that could have devastating consequences for patients nationwide. Here, we explore the nature of these dangerous effects on a specific patient population.
Jennifer Robin’s Story
A wave of intense heat enveloped my whole body. I was on fire. My cheeks turned bright red as my heart beat out of my chest. I grew faint, my blood pressure spiking and then falling. Extreme anxiety and unquenchable thirst ensued as I doubled over with abdominal pain and gastrointestinal distress. This quickly affected my ability to breathe. My throat swelled nearly shut, closing off my airway. In minutes, I was rapidly declining. I quickly grabbed my EpiPen, administered it, and rushed for the Benadryl. Luckily, I survived. Unfortunately, I was far too familiar with this scenario. For years, I had nearly daily episodes without any answers.
What I experienced was a life-threatening reaction called anaphylaxis. Anaphylaxis is a severe allergic reaction that causes dangerous symptoms throughout the body. If left untreated, it can be fatal [6, 13, 14]. I have a condition called Mast Cell Activation Syndrome (MCAS), which puts a person at increased risk for anaphylaxis. MCAS patients can have anaphylaxis to all types of triggers that can change over time. These triggers can include heat/cold, stress, fragrances/odors, exercise, food, and medication [4, 9, 12, 15].
Although I had reactions to triggers like these, I went undiagnosed for years. During my search for answers, I was diagnosed with Ehlers-Danlos syndrome (EDS) by Dr. Peter Holt and Postural Orthostatic Tachycardia Syndrome (POTS) by Dr. Blair Grubb. Finally, in 2014, I was diagnosed with MCAS by a world-renowned expert, Dr. Lawrence Afrin. When I had an anaphylactic reaction in Dr. Afrin’s office, he immediately recognized it and sent me directly to the hospital. Due to MCAS, my histamine levels (and other mediators) were exceedingly elevated above the normal limits. This doctor saved my life. I am thankful to all the medical personnel who have helped me throughout my long health struggle, but I am especially grateful to my primary care physician, Dr. Gary Milles.
My journey to find and manage my diagnosis was a long and isolating journey. I did not want anyone else to have to go through this alone, and I wanted to help other people who suffered from this condition connect with each other. So, I formed a Facebook support group, Mast Movement, which now has over 7,000 members from all over the world. This is where I met Arielle.
It was the second day of my second semester of college when I decided to take out a snack, a protein bar, from my backpack during class. Although I had been diagnosed with a peanut allergy from a skin test in high school, I was an allergist’s nightmare patient. I did not avoid cross-contact, never told servers at restaurants about my sensitivity, and only carried my EpiPen if it happened to fit in my purse that day. After just one bite, I flushed from head to toe, broke out in hives across my chest and arms, and my throat grew itchy. Before I knew it, I was wheezing and feeling faint. EpiPen in hand, I went straight to the Student Health Center as fast as I could. I handed them the EpiPen, terrified to use the auto-injector for the first time. They handed it right back to me, saying, “You cannot be afraid of it! You may have to use it again in the future.” After passing the auto-injector back and forth one more time, I jabbed the EpiPen into my thigh. I felt immediate relief. The culprit was peanut flour, the very last ingredient on the protein bar label.
Since my symptoms occurred immediately after eating a food that contained an allergen, we assumed that my episode was just an anaphylactic reaction to peanut. However, these episodes kept happening over and over again, completely independent of food. Even when I was religious about what I ate, I was still having reactions to seemingly nothing. I was undiagnosed for about a year and a half until a friend encouraged me to ask my allergist about MCAS. I was referred to Dr. Andrew White at the Scripps Clinic in San Diego, CA. Like Jennifer, I was diagnosed with MCAS and began treatment. Later, I was also diagnosed with POTS and EDS. These diagnoses were confirmed at Stanford’s Autonomic Disorders Clinic and the Mayo Clinic in Arizona. Like Jennifer, I finally had an answer and feel eternally grateful to Dr. White and the other physicians who continue to serve as my biggest advocates on a daily basis.
Life with Mast Cell Activation Syndrome
Patients with EDS, MCAS, and POTS often “look” healthy. For this reason, these conditions are sometimes called “invisible illnesses.” However, each day can truly be a struggle to survive. Patients report that symptoms vary greatly, sometimes changing from hour to hour. This variability can make it difficult for others to understand. For example, POTS, one form of dysautonomia, seems to be particularly confusing to those who are not familiar with it. Common comorbid conditions with EDS include Mast Cell Activation Diseases (MCAD) and dysautonomia; this combination is being deemed a new disease cluster by researchers [8,11]. “The Trifecta” is the term used to describe the association among these three conditions by some in the patient community.
MCAS, the most prevalent Mast Cell Activation Disease, is an immunological disorder that also has hematological and allergic components [1, 20]. MCAS is currently classified as a rare disease, but some experts believe that it may affect up to 17% of the general population . This percentage is equivalent to 1 in 6 people or over 55 million individuals being affected by MCAS according to the 2019 United States Census Bureau report . The severity of the illness can vary greatly among patients, and symptoms can manifest within all organ systems including, but not limited to, the respiratory, neurological, gastrointestinal, cardiovascular, and immunological systems as well as in the connective tissues and the skin . Some common symptoms include rashes or hives, flushing, nausea or vomiting, shortness of breath, fatigue, brain fog, and abdominal pain . It is important to note that each patient presents differently and not all symptoms are required to suspect a diagnosis.
In the body, mast cells release chemicals (mediators), such as histamine and prostaglandins. In MCAS, a normal amount of mast cells release mediators at inappropriate times and trigger an allergic-like response without the presence of a classic IgE mediated allergen . As a result, patients with MCAS can experience allergic reactions, including anaphylaxis.
Reports from members of Mast Movement, a Facebook community for MCAD patients, suggest that MCAS is often underdiagnosed. This appears to be due to a lack of provider awareness (MCAS was newly introduced into the literature as its own disease entity in 2007) and due to testing/diagnostic challenges [2, 5, 16, 19, 21]. MCAS falls under the MCAD umbrella and is an accepted diagnosis at top research institutions such as Harvard, NIH, and the Mayo Clinic. Although Mastocytosis is a rare but more well known MCAD, it differs from MCAS in that Mastocytosis is caused by the presence of too many mast cells while a normal amount of mast cells are present in MCAS; as a result, MCAS can be difficult to diagnose and has different diagnostic criteria . Testing requires very specific time-and-temperature-sensitive procedures . There is currently a lack of consensus on diagnostic criteria. According to patient and some expert feedback, a common misconception among providers is that a negative tryptase test can adequately rule out a mast cell disease . Further testing must be performed. Since there are over 200 known mediators released by mast cells and only a few can be commercially tested for, a clinical diagnosis is often made if all other diagnoses are excluded and the patient responds to treatment .
CMS-9926-P and its Implications for the EDS and MCAD Community
Patients in the EDS and the MCAD community have expressed concern in regard to Medicare’s CMS-9926-P. This proposed law would allow insurers to make mid-year changes to their prescription drug formularies. This means that Medicare may not cover certain brands of medications even if the brands are medically necessary for patients. In addition, CMS-9926-P would also exclude manufacturers’ co-pay coupons from being applicable towards annual cost-sharing limits, increasing the financial burden patients face in obtaining prescription medications to meet their cost-sharing limit. This would also increase the demand on the already time constricted medical staff to review and process these medication requests more than once a year.
MCAD patients are particularly vulnerable under this CMS-9926-P legislation because it is not uncommon for them to have sensitivities/allergies to fillers, dyes, or byproducts in prescription medications . In fact, medications are often one of the most common triggers cited among Mast Movement members. It is essential for many patients with MCAD to take specific brands or generics of medication to avoid reactions that may include anaphylaxis. Patients may need to go to several different pharmacies to seek safe excipients. According to MCAD expert, Dr. Lawrence Afrin, author of Never Bet Against Occam, “In Mast Cell Activation Disease, the dysfunctional mast cells not uncommonly are triggered by assorted medication product excipient ingredients (i.e., fillers, binders, dyes, preservatives) to even more heightened states of (inappropriate) activation, driving even more multisystem sickness.”
Although MCAD patients may never experience anaphylaxis, nor is it required for diagnosis, these patients are significantly more susceptible to anaphylaxis than the general population . According to the American Academy of Allergy, Asthma, and Immunology (2017), “anaphylaxis is an acute life-threatening systemic reaction that results from the sudden, rapid, systemic release of mediators.” The release of these mediators, chemicals that affect body functions, can lead to symptoms such as itching and swelling of the mouth and throat, vomiting, shortness of breath, and weakening pulse, among other symptoms. Anaphylaxis occurs as the result of rapid mediator release.
These “misbehaving” mast cells can lead to drug hypersensitivity reactions, including anaphylaxis . Compounding the issue is that medication is one of the leading causes of anaphylaxis worldwide. Medication is a widely known trigger for anaphylaxis in mast cell patients. MCAD patients can and do react to inactive ingredients or drugs themselves, sometimes leading to anaphylaxis. Although generic medications may have identical active ingredients to a brand name medication or other generic equivalents, the other ingredients like dyes, fillers, and other excipients often vary. Specifically, the AB rating used by the FDA to show equivalence among brands and generics does not protect MCAD patients from reacting to inactive ingredients. Dr. Afrin states, “It is frankly dangerous to many MCAD patients to be forced to switch from medication formulation they have identified as safe to new formulations whose excipient packages likely are at least somewhat different from the patients’ established safe formulations.”
Among the MCAS patients of Mast Movement support group, a variety of different adverse reactions were described when members switched brands or generics of the same medication with changes in inactive ingredients. These reactions include increased orthostatic intolerance, dizziness, nausea, tachycardia, rashes or hives, swelling, blood pressure fluctuations, diarrhea, vomiting, flushing, wheezing, and anaphylaxis as well as neurological and neuropsychiatric side effects. Patients who are forced to try other brands or generics of medication due to shifts in insurance coverage may suffer from these negative responses, sometimes requiring emergency medical attention. According to Dr. Afrin:
Especially given that even trace amounts of a triggering excipient can cause
severe clinical illness (even sometimes life-threatening anaphylaxis) in an MCAD
patient, it seems the epitome of the ‘penny wise, pound-foolish’ decision for a
third party (insurer, pharmacist, etc.) to change an MCAD patient’s established
safe medication formulation to an alternative formulation simply to save a few
pennies. The cost of a single emergency department visit and/or hospitalization
caused by such a change forced on merely a single MCAD patient might easily
completely eclipse the savings the insurer would reap from such a formulation
change across the insurer’s entire client base.
CMS-9926-P legislation jeopardizes the safety of any person who takes prescription medications. Adverse reactions, including anaphylaxis, can occur in any individual at any age. It is not uncommon for individuals to develop new allergies over a lifespan. Furthermore, the population of individuals with MCAS is growing. Even if you don’t have a mast cell disease or allergies, it is likely that you know someone who does. Dr. Afrin states, “Physicians and pharmacists and insurers alike must begin recognizing and accommodating the unique needs of the (large!) MCAD population if they hope to stem the financial hemorrhage their uneducated actions cause the system to unnecessarily suffer in providing care for this population.”
We hope that Medicare will recognize the significant risk that CMS-9926-P poses and identify safer and more effective ways to reduce costs. The patient communities will continue to work together as a united front to advocate for all.
For more information on CMS-9926-P please visit: https://www.federalregister.gov/documents/2019/01/24/2019-00077/patient-protection-and-affordable-care-act-hhs-notice-of-benefit-and-payment-parameters-for-2020#addresses
For more information about Mast Movement Facebook Support group please visit: https://www.facebook.com/groups/MastMovement/
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 Valent P. (2013). Mast cell activation syndromes: definition and classification. European Journal of Allergy and Clinical Immunology, 68(4), 417-424.
 White, A. A. (2015, February 17). A Tale of Two Syndromes – POTS and MCAS. Retrieved February 17, 2019, from http://www.dysautonomiainternational.org/blog/wordpress/a-tale-of-two-syndromes-pots-and-mcas/
Medicaid’s New Proposed Law Could Harm Millions of Patients By Jennifer Robin Kulik and Arielle Davis (FINAL) – Article PDF
The Mastocytosis Society (Website)
Continuing Medical Education/CMEs (EDS Awareness)
2016 Wellapalooza Physicians Learning Lunch (EDS Wellness)
2017 Physicians Learning Conference Recordings (EDS Wellness)
‘Never Bet Against Occam: Mast Cell Activation Disease and the Modern Epidemics of Chronic Illness and Medical Complexity’
Jennifer Kulick’s Mayo Clinic Article
Listing of Dr. Lawrence Afrin’s Research Publications
Mast Cell Research Course (Compilation of MCAS-focused Webinar & Presentation Recordings)
‘Ask the MCAS Expert – LIVE!’ with Dr. Tania Dempsey (Monthly Webinar Series)
Mast Cell Research YouTube Channel
Core Brian Journal Podcast with Dr. Lawerence Afrin
Evan Brand Podcast with Dr. Tania Dempsey
Better Health Guy Podcast with Dr. Theo
Patient Support Groups:
Mast Movement (a Facebook group)
Never Bet Against Occam (a Facebook group)/Mast Cell Research FB page
Mast Attack (a Facebook group)
DC/VA/MD Local MCAS Support Group (link to related Facebook Group)
LEAD EDS (Patient Leadership & Advocacy Training & Education Program)
Article Published and Shared with Permission. Opinions expressed are solely those of the article authors and do not represent the views or beliefs of EDS Wellness, our Executive Director, members of our Board of Directors, organizational volunteers, sponsors/donors, and/or affiliates. Edited by Liz Murphy. Medical & Healthcare Content Reviewed by Lisa Klimas, Lawrence Afrin, M.D. & Other MCAS Community Experts.