As the world resolves to weather the COVID-19 storm, the primary question amongst those with Ehlers-Danlos Syndrome, Chiari and Comorbids has become, “Do my conditions make me immunocompromised?”

While not all risks are equal amongst us, we do live with several factors originating from our condition(s), that can put us in the high-risk category. Doctors like to downplay our conditions, but the truth is that most doctors have no clue about what we REALLY deal with, and the ones that do admit that there’s still so much that the experts don’t understand. And so it is with COVID-19!

In Appendix A of a recent PDF entitled “Implementation of Mitigation Strategies for Communities with Local COVID-19 Transmission”, the Centers for Disease Control (CDC) included: “Neurological and neurologic and neurodevelopment conditions [including disorders of the brain, spinal cord, peripheral nerve, and muscle such as cerebral palsy, epilepsy (seizure disorders), stroke, intellectual disability, moderate to severe developmental delay, muscular dystrophy, or spinal cord injury].” [1] This encompasses the many EDS related brain and spinal comorbidities (Chiari, Craniocervical Instability, Atlantoaxial Instability, Subaxial Instability, Tethered Cord Syndrome, Syringobulbia, Syringomyelia, or even an intravertebral disc compressing the spinal cord, etc.).

Ehlers-Danlos Syndrome is grossly underdiagnosed, so there are thousands upon thousands of sufferers begging for help who aren’t getting the testing and help with the symptoms that they need from their medical professionals. Even once diagnosed, that’s all that most diagnosed patients get. No further workups or limited workups are done and comorbids (even life-threatening comorbids that would make them immunocompromised) are unidentified and left untreated.

 

SOME OF THE CONCERNS WORTH MENTIONING ARE:

 

1. EDS patients are more likely to suffer from rheumatological conditions than the general population, both autoimmune/inflammatory diseases (psoriasis, PsA, AS, RA, inflammatory eye disease, autoimmune thyroiditis, SLE, Crohn’s disease, pernicious anemia, and TRAPS) and non-autoimmune (fibromyalgia), but diagnosing these conditions require a workup. According to a 2017 study published in Scientific Reports, “The percentage of patients with at least one rheumatological condition was significantly correlated with the level of workup. No workup (physical exam only) = 9.2%; Limited work up = 33.3%, Comprehensive work up = 67.1%.” [2]

2. Many of us suffer from medullary and vagus nerve issues that can labor our breathing and compromise our ability to swallow correctly, which in turn makes us more susceptible to respiratory problems and complicating recoveries when sick. [3]

3. Many of us live with Mast Cell Activation Syndrome (whether we’re diagnosed properly or not), which can increase respiratory issues and can further compromise our immune systems. [4]

4. EDS patients are far more likely to suffer from chronic inflammation (with and without elevated C-reactive Protein (CRP) and Erythrocyte Sedimentation Rate (ESR)); chronically swollen lymph nodes; and chronically elevated white blood cell counts, all pointing to our immune systems being under distress.

5. With our faulty connective tissues, our blood-brain barriers tend to be thin and permeable, thus allowing a virus or viral antibodies to cross over and damage our arachnoid villi. [5]

 

 

ABOUT CoViD-19 & SARS‐CoV‐2

Pandemics are not new to the world or to the United States. Originally deemed “The Wuhan Virus,” because of its location of Wuhan City, China, the virus hit beginning in December of 2019. It then quickly spread beyond the city’s borders. “Viruses are named based on their genetic structure to facilitate the development of diagnostic tests, vaccines, and medicines,” [6] by the International Committee on Taxonomy of Viruses (ICTV). As more information rolled out, it became apparent that while it has shared commonalities with viruses from the past; mainly, Severe Acute Respiratory Syndrome (SARS); it wasn’t the same – it had mutated. Several new names emerged, including Wuhan Coronavirus, 2019 Novel Coronavirus (a novel coronavirus is a new coronavirus that has not been previously identified in humans [7]), 2019-nCoV. Medical researchers seem to have settled on two names that best describe the situation we’re facing.

  • CoViD-19 – Coronavirus Disease 2019 [6]
  • SARS‐CoV‐2 – Severe Acute Respiratory Syndrome Coronavirus 2 – The virus that causes the Coronavirus Disease 2019. [6]

“Viruses, and the diseases they cause, often have different names. For example, HIV is the virus that causes AIDS. People often know the name of a disease, such as measles, but not the name of the virus that causes it (rubeola).” [6]

As the pandemic has spread across the globe, they are seeing differences from one place to another, but there’s still so much that needs to be known, and in our “everything is microwavable” world, we aren’t good at waiting patiently. We saw the numbers of infections and deaths rise in China and South Korea (both highly populated countries), and then we all heard the news that the curve is flattening and that they’re seeing substantially fewer new infections and deaths. At the same time, we’re seeing the virus wave throughout Europe. Deaths in Italy (who had some hindsight and time for preparation, and a smaller population) have now exceeded the death toll in China. This beckons the question, “is it the same virus or is it still mutating?” Is CoViD-19-China, the same as CoViD-19-SKorea, CoViD-19-Europe, CoViD-19-USA? As they figure things out, they’re going to have to look at data coming out of each location and compare. But we’re going to have to use wisdom and be prudent, while we all await the answers TOGETHER!

Most Coronaviruses only cause mild respiratory diseases (such as the common cold). Two Coronaviruses, Severe Acute Respiratory Syndrome CoV (SARS‐CoV) and the Middle East Respiratory Syndrome CoV (MERS‐CoV) did not follow the “mild respiratory” pattern and instead, they caused prominent upper respiratory tract issues. SARS‐CoV‐2 is believed to be attacking the lower respiratory tract.

 

Prominent Symptoms of SARS‐CoV‐2 (based upon the first‐hand evidence from Wuhan local hospitals): [8]

Fever (83%‐99%)

Dry cough (59.4%‐82%)

Shortness of breath; respiratory distress (~55%), with more than half (~46% to 65%) becoming “acute, highly lethal pneumonia requiring intensive care. About 46% to 65% of the patients in the intensive care worsened in a short period of time and died due to respiratory failure.”

 

The symptoms can occur between 2 to 14 days after being exposed to someone infected with the coronavirus, based on the incubation period of MERS-CoV (Middle East Respiratory Syndrome Coronavirus) viruses.

 

Other Symptoms Include: [8]

• Tiredness
• Aches
• Runny nose
• Sore throat
• Diarrhea

“Additionally, some patients with CoViD-19 have also [shown] neurologic signs, such as headache, nausea, and vomiting.” This is where it may get even more alarming for those of us that already suffer from neurological issues (and the thin blood-brain barrier demonstrated in EDS patients). With SARS‐CoV‐2 bearing so many similarities with SARS‐CoV and MERS‐CoV (and sharing the same beta coronavirus (βCoV) clade), they don’t know for sure where the similarities begin and end. Some coronaviruses are believed to “invade [the] brainstem via a synapse‐connected route from the lung and airways.” Both SARS‐CoV and MERS‐CoV now have supporting data demonstrating their ability to move beyond the respiratory tract and may invade the central nervous system. “The infection of SARS‐CoV has been reported in the brains from both patients and experimental animals, where the brainstem was heavily infected. Some coronaviruses have been demonstrated able to spread via a synapse‐connected route to the medullary cardiorespiratory center from the mechanoreceptors and chemoreceptors in the lung and lower respiratory airways.” [8]

SO, WHAT’S A MEMBER OF OUR COMMUNITY TO DO?

There is currently no vaccine to prevent CoViD-19, so the best way to prevent illness is to avoid being exposed to this virus. The virus is thought to spread mainly from person-to-person from close contact with one another (within about 6 feet), through respiratory droplets produced when an infected person coughs or sneezes.

[Note: We have found an entourage of good ideas that might prove helpful. When the sources are known, they are provided. As information continues to become available, the Center for Disease Control and Prevention (CDC) will be updating the list of recommendations on their site accordingly, so we encourage you all to stay informed by following them: https://www.cdc.gov/coronavirus/2019-ncov/prepare/prevention.html.]

 

 

STEPS TO PROTECT YOURSELF AND OTHERS:

• Wash your hands often (every 20 minutes) with a lathering soap for at least 20 seconds before rinsing. If soap and water are not readily available, use a hand sanitizer that contains at least 60% alcohol, covering all surfaces of your hands (both sides and between fingers), and rubbing them together until dry. [9]

• Avoid touching your eyes, nose, and mouth with unwashed hands. [9]

• Cover your mouth and nose with a tissue when you cough or sneeze or use the inside of your elbow. Throw used tissues in the trash. Immediately wash your hands with soap and water for at least 20 seconds. If soap and water are not readily available, clean your hands with a hand sanitizer that contains at least 60% alcohol. [9]

• Clean AND disinfect frequently touched surfaces DAILY. If surfaces are dirty, clean them with detergent or soap and water, prior to disinfection. Then disinfect with a bleach solution (recommended ratio: 5 TBSP (1/3 cup) household bleach per one gallon of water OR 4 tsp household bleach per one quart of water. *Note: Never mix household bleach with ammonia or any other cleanser.), alcohol solutions using a minimum of 70% alcohol, or other common EPA-registered household disinfectants with EPA-approved emerging viral pathogens (https://www.epa.gov/pesticide-registration/list-n-disinfectants-use-against-sars-cov-2). For more on disinfection guidance, visit: https://www.cdc.gov/coronavirus/2019-ncov/prepare/cleaning-disinfection.html [9] Some research is encouraging that you pay particular attention to cleaning metallic surfaces (refrigerators, toasters, doorknobs, etc.) thoroughly as these surfaces might be allowing pathogens to remain and stay viable for up to 9 days.

• Cancel all non-essential appointments and stay at home, even if you have no symptoms of the virus unless you’re having a medical emergency. If you are having a medical emergency, call 911 and let them assist you in properly assessing the situation and helping you get help if it is a genuine emergency (this is a very different day in time and many local 911 centers ARE NOT dispatching for non-emergent calls).

• If you must leave the house for essentials (groceries, pharmaceuticals, etc.), assume that everyone that you encounter as potentially infected and protect yourself (masks, gloves, hand sanitizers, etc.) and practice social distancing (maintaining a 6 ft distance between yourself and others, as much as possible). If possible, designate one person in the household that is not in the high-risk category (under 60 years old with no pre-existing conditions that might increase their risk) to do the essential errands of the household. When they venture out for these essentials, they too should protect themselves (for their sake and that of the family that they will be returning to) with masks, gloves, hand sanitizers, etc., whenever possible. Whoever leaves the house should lather up in a shower or bath and wash their clothes immediately after returning home (BEFORE sitting down or touch things around the house). Thoroughly clean and sanitize the bathroom afterward.

• If you have symptoms, DON’T PANIC. Stay home and put distance between yourself and other people (even those in your own household). Avoid common places in the house as much as possible. Use a separate bathroom if you can. If you can’t, try and clean things behind you each time that you use it. DO NOT GO DIRECTLY TO THE EMERGENCY ROOMS UNLESS YOU ARE HAVING A LIFE-THREATENING EMERGENCY. If you fear that you need to go to the Emergency Room and you have time, call them first for advice and that will also allow them time to prepare for your arrival in a way that considers your safety and the safety of everyone that you may come into contact with while there. If you don’t think it’s emergent, contact your primary care doctor by phone or email and inform them of your symptoms so they can assess your risk factors and advise you accordingly. Everyone that gets sick shouldn’t expect a test kit, because they ARE in short supply. If you’re healthy enough to be treated at home and recover without complications, you might not ever know if you had CoViD-19 or not.

• Try to avoid the use of all anti-inflammatory treatments right now (including NSAIDs), without checking with your doctor first. There are studies coming out indicating that anti-inflammatory treatments might counter antiviral treatments. NSAIDs, such as aspirin and Ibuprofen, can take up to 10 days to pass out of the body. If you end up needing an antiviral treatment, you might not have the 10 days to wait. [10]

• Don’t smoke or vape ANYTHING, especially right now, as they can further compromise your airway and lungs, abstaining won’t stop you from getting the virus, but it might help reduce the possibility of complications if you do.

• Drink lots of hot (but not scalding) liquids (coffees, teas, soups, warm water) and try to take a sip of warm liquids every 20 minutes to help keep your mouth moist and help wash viral pathogens that might be in your mouth (some reports indicate that they can stay in your mouth for 3-4 days before entering your lungs) down into the stomach where the gastric juices can help neutralize it before it attacks the lungs.

• Gargle once a day with an antiseptic in warm water (like saltwater, lemon water, vinegar water).

• Eat fruits and vegetables and try to safely elevate your vitamin C levels and zinc levels to help your immune system as much as possible.

• It’s okay to listen to the news, but don’t let it absorb you. Check-in for an hour or so and then turn it off or tune it out and decompress your mind. Stress can manifest in our central nervous systems and that usually includes an increase in pain levels. So, turn off the televisions and put on a song or two that makes you hopeful and happy, even if it’s short-lived.

• Stay connected with others. Use this time of isolation as an excuse to call an old friend and see how they’re fairing through things.

• Stay encouraged and try to encourage others around you. For many of us with chronic pain conditions, self-isolation is a way of life, but for those around us, it may be a new thing and that can be frightening. It is okay to tell them that you understand because this has become much of your reality, but don’t just leave it there. Remember how hard it is/was for you to adjust and tell them about things that you found to help and pitfalls to avoid, and maybe you can make plans to try and get out together once this is all over. This might create an ideal opportunity to better understand one another and heal broken relationships if you are willing to rise to the occasion.

 

References: 

1 “Implementation of Mitigation Strategies for Communities with Local COVID-19 Transmission.” Center for Disease Controls, <www.cdc.gov/coronavirus/2019-ncov/downloads/community-mitigation-strategy.pdf>.

2 Rodgers, Kyla R., et al. “Ehlers-Danlos Syndrome Hypermobility Type Is Associated with Rheumatic Diseases.” Nature News, Nature Publishing Group, 4 Jan. 2017, <www.nature.com/articles/srep39636>.

3 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” Wiley Online Library, John Wiley &amp; Sons, Ltd, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/full/10.1002/ajmg.c.31549>.

4 Cruse, Glenn, and Peter Bradding. “Mast Cells in Airway Diseases and Interstitial Lung Disease.” European Journal of Pharmacology, U.S. National Library of Medicine, 5 May 2016, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4637266/>.

5 Driscoll, Diana. “The Driscoll Theory: The Role of External Communicating Hydrocephalus, Mast Cell Disease and CCSVI as the Cause of POTS (Postural Orthostatic Tachycardia Syndrome) in Ehlers-Danlos Syndrome.” The ILC Foundation, Apr. 2012, <www.theilcfoundation.org/wp-content/uploads/2012/04/Driscoll_Theory_EDS_-_Online3.pdf>.

6 “Naming the Coronavirus Disease (COVID-19) and the Virus That Causes It.” World Health Organization, World Health Organization, <www.who.int/emergencies/diseases/novel-coronavirus-2019/technical-guidance/naming-the-coronavirus-disease-(covid-2019)-and-the-virus-that-causes-it>.

7 Commissioner, Office of the. “Coronavirus Disease 2019 (COVID-19) Frequently Asked Questions.” U.S. Food and Drug Administration, FDA, <www.fda.gov/emergency-preparedness-and-response/mcm-issues/coronavirus-disease-2019-covid-19-frequently-asked-questions>.

8 Li, Yan‐Chao, et al. “The Neuroinvasive Potential of SARS‐CoV2 May Play a Role in the Respiratory Failure of COVID‐19 Patients.” Wiley Online Library, John Wiley &amp; Sons, Ltd, 11 Mar. 2020, <www.onlinelibrary.wiley.com/doi/abs/10.1002/jmv.25728>.

9 “Prevention of Coronavirus Disease 2019 (COVID-19).” Centers for Disease Control and Prevention, Centers for Disease Control and Prevention, 18 Mar. 2020, <www.cdc.gov/coronavirus/2019-ncov/prepare/prevention.html>.

10 Stebbing, Justin. Published:February 27, 2020DOI:doi.org/10.1016/S1473-3099(20)30132-8 PlumX Metrics, et al. “COVID-19: Combining Antiviral and Anti-Inflammatory Treatments.” The Lancet, 27 Feb. 2020, <www.thelancet.com/journals/laninf/article/PIIS1473-3099(20)30132-8/fulltext>.

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Bridges are built as a means to assist people in getting over the things that obstruct their path. We are constantly facing obstacles when it comes to our health, so we decided to build a bridge! Chiari Bridges is a non-profit network built by Chiarians for Chiarians. Our goal is to provide information, substantiated by published studies, in a way that is easy to understand. We encourage readers, to not only read our articles, but look up every study for themselves and read that too. Print it out and sit down with a highlighter; read it as many times as necessary until you understand; then use it to talk to your doctors. Our doctors are under no obligation to read our articles, but they should be willing to read officially published studies from journals, especially those specific to their profession. As many of our authors are patients as well, all authors sharing original pieces will be writing under pseudo-ghost names, to protect them from being black-listed by their doctors. Chiari Bridges is not affiliated with any physician or medical group. You should in no way use this site as a replacement for diagnosis, treatment, or medical from a qualified medical professional. The information provided on our website is strictly for educational purposes, in order to help further the understanding of Chiari, and its associated conditions. Our goal is to share our collective experiences and the information we have gathered, with those in need of answers, that it may increase your knowledge and guide you in conversations with your doctors.

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