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October 30, 2020 | By Dr. Robert Larry Reese-Johnson
Jan. 1, 2020 – It was a new year, and based on the progress that he’d made I emailed Dr. Tomatore regarding this progress and questioning, through my research, exactly what tests results were reviewed, what tests were done and how exactly the diagnosis of CJD was made? Dr. Tomatore never responded to this email, nor did he an acknowledge receiving it.
For the next two weeks, the medical team was re-evaluating Reggie’s care and treatment plan based on the progress he was making and with different symptoms. During this time new MRIs, blood tests, and EEG were ordered to determine what exactly was going on.
I then got a call from the resident in charge of Reginald’s care for that time. Upon review of: the EEG, which showed NO EVIDENCE of the preliminary things they saw earlier; and the MRI, which showed evidence of clearing up the previous spots; and the blood tests, that were still to be determined, they were moving off the CJD diagnosis and agreeing with me that this this was AE. They were also moving forward finally with the next line of treatment, rituximab, and he was set to receive that treatment.
Rituximab had been denied by insurance from the first of December based on the CJD diagnosis, but now it was approved based on the correct diagnosis. There was also a family meeting around this time, at which they recommended sub-acute rehab for Reggie, asking me for facilities that I may suggest in the area for him to go, and the team moved forward with sending referrals.
On Jan. 20, the blood test results came back and the CASPR2 antibody was identified as the cause of his AE. Reggie then went through a full-body CT scan as the creation of the antibody is known to fight off cancer. The results of the CT scan showed no traces of tumors or cancer anywhere, and the team would still try to move him to a sub-acute rehab. Two days later, the nurse case manager called to tell me that he had been accepted to a facility in Lanham, MD. There was concern on my part because I did some research and I was uncomfortable with reports on quality of care by that facility. Then there was a snafu with both time of discharge and dispense of medicine, which he never received.
Upon arriving at the subpar sub-acute center, as Reggie was settling in I was talking to the nurse—there was only one of her for 13-plus patients—he fell twice against a cabinet with metal handles. Prior to his being admitted here, I had let them know he had to be restrained in both his bed and chair with a waist belt, as he attempts to get up and falls. They denied I said that, letting me know they would have never accepted him under those conditions.
Ultimately, I read the reports provided and they only stated that Reggie was agitated at times and would get up, which was a blatant untruth. As a result of two falls over 24 hours, they called 911 and he was transferred back to the PG ER.
Upon arrival at PG, Reggie was evaluated by the attending physician, who was familiar with him from his original admission. I informed this doctor of the AE diagnosis and what caused him to be brought back to the hospital. He let me know he would evaluate him and try to work with me regarding my requests moving forward. Reggie then checked out fine from the falls and that they had no medical reason to admit him.
Knowing the situation however, the physician would try to complete a doctor-to-doctor transfer for him from PG to the attending doctor at Georgetown. While the doctor was doing this, two social workers told me Reggie had three options:
I was opposed to all options and I was not signing for his discharge, so I got a CareFirst case manager to talk with the social workers. They came back to me with the case manager on the line and it was determined that Reggie would return to the sub-acute facility, be evaluated by their medical staff and nurses, so they could determine he’d be readmitted to PG’s rehab. I agreed and he was medically transported back to the facility that evening.
Upon Reggie’s arrival for the evaluation, I was taken into the conference room to meet with their team. They told me they could not do an evaluation, as promised, and could not recommend acute rehabs for a patient. Also, they informed me that they could not keep Reggie, as they could not ensure his safety, as by law they cannot do any form of restraint. They advised for me to take him back to Georgetown ER on my own and that I not him leave there.
I replied that I was unable to take a medically fragile, immunocompromised patient, on a cold night in my car. They then agreed to have Reggie transported back to Georgetown in a van, but without medical personnel, to the ER at Georgetown. I got on the phone again and informed the case worker all of this, and the violation of the agreement worked out. The van then arrived and did not have a wheelchair in which he could be transported. The staff said that if he used their wheelchair, I needed to bring it back the next day. I agreed and he was prepared for transport. Just as he was wheeled out, they told me that the van had left and they could not contact the driver to come back.
I informed the staff that as a last resort I would take Reggie to Medstar Georgetown in my car as HE COULD NOT STAY HERE ONE MORE MOMENT because this facility WOULD NOT BE A PLACE I WOULD WANT ANY LOVED ONE! Also, I informed them that if Reggie, because of his compromised immune system, got as much as a sniffle and this caused his recovery to be extended, I would be suing the facility. He was placed in my car and I began the took him to Georgetown. All this time, Reggie wasn’t provided with the crucial medication that was prescribed for his condition.
As I was transporting him to Georgetown ER, I called ahead to inform them of the situation. I also called the supervisor of case managers and the case worker so that everyone was fully aware. During three conversations with the supervisor, I was told the sub-acute rehab hadn’t known about Reggie’s needing restraints on his waist and an alarm on the bed, prior to or when making the referral.
He was admitted to the ER, where he stayed in a room for two days, despite needing an individual room because of his agitation and need of a sitter. I was also informed at this time that referrals would be made to Encompass Health in Virginia or Capital Region Physical Therapy at PG for acute rehabs. The director for Encompass called and let me know they would turn him down, but would re-evaluate if Reggie required only an alarm on the bed as an alert.
Jan. 24-Feb. 5 – Reggie received a room assignment in the post-surgical unit. I was impressed with the care as he was receiving PT and OT every day, and he was walking and progressing with less agitation. They were actually working with Reggie to devise a plan for him not to be restrained by the waist belt, and he was right outside the nurses’ station and they available to assist him immediately.
The social worker and the rehab physician for the unit, though, had no clue that Reggie was a returning patient to the hospital until I told them. The rehab doctor actually offended me by saying, “I would suggest or recommend a sub-acute rehab because that would get the most bang for your buck from insurance.” This was based on getting things out of insurance and not on the patient’s quality of life. The last time rehab was suggested and tried, Reggie went to acute rehab then to sub-acute rehab, both of which were cleared by insurance, and this would have been a total of 10-12 weeks or less, if they would have done it correctly?
Feb. 5 – Reggie moved back to the neurology unit and the care varied as they attempted to get him to be less agitated so he’d be accepted by acute care rehabs. There was no family meeting regarding this plan or any plans moving forward once Reggie was readmitted to Georgetown. When he was there previously, he received PT, OT and speech daily, and the nursing staff was walking him and getting him up to the bathroom daily, as he showed the interest. This no longer happened in the neurology unit. Most everything that Reggie did and attempted to do was prompted by me when I visited, or when I showed the nurses who agreed to do it and tried when I was not able to be there.
Feb. 15-22 – While visiting him in the evenings over two weekends I assisted him in eating of his meals. I then noticed he was not chewing as well as he had been previously. I inquired of the nurse and tech if this is what they had experienced during the previous meals, and they confirmed it.
I later noted that Reggie’s bottom row of dentures were not in his mouth because someone had removed them and placed them in a green cup. I alerted the nurse of this on Feb. 16, as my concern was that this could have caused choking or other problems with his lungs. Her response was that she did not know he had a bottom row of dentures, but said she’d write it in his record chart. Imagine my surprise when I came in the next weekend and discovered the same thing was happening and his bottom dentures were removed again; when I was assured that all would be informed? So, I took the action of writing on the chart, in his room to ensure dentures are in before eating, and that he has both top and bottom.
Feb. 27 – I was contacted at 2 pm by the attending physician that Reggie had experienced a fall while trying to get out of his chair. Because the footrest was in the up position, he got stuck and fell. Though the chair’s alarm sounded, the nursing and tech staff didn’t arrive in the room, and he fell on his knee and head. It was our experience that the nursing and tech staff were inconsistent in arriving in the room after Reggie or I hit the call button. All staff were aware that if Reggie tried to move or grabbed himself, he was letting them know he needed to use the bathroom and attempts should be made to get him up. The connections within his brain are being encouraged to promote independence. Right now, though, he needed assistance because I didn’t want the habit to form of just urinating or having a bowel movement on himself while trying to go as he normally would.
Mar. 2 – A meeting with the medical director and others of Georgetown was held regarding “next steps” and where they were planning to send him. The doctors gave me a great deal of information and I respectfully allowed them to speak. But they contradicted previous statements from other doctors. I stopped them from talking and said, “Reggie will not be going anywhere except Medstar National Rehabilitation Neurorehabilitation Acute Rehab or Encompass Health and Rehabilitation in Virginia!” If they proposed anything else, they would hear from a team of lawyers that I had contacted with all the prior information. Within the next hour they had a new PT physician evaluate Reggie and he was then set to move to Medstar NRH on Mar. 5.
Mar. 5-Apr. 1 – Reggie was admitted to Medstar NRH and made great progress there receiving an hour everyday of speech, OT and PT. I was there daily until I wasn’t allowed to because of COVID-19. Reggie finished his 30 days in acute rehab and was transitioned to sub-acute rehab at Largo Nursing and Rehabilitation Center.
Apr. 1-28 – Reggie was admitted and continued sub-acute rehabilitation at Largo Rehabilitation and Nursing Center and even though this facility was just a 10-minute drive from our house I could not visit him, because of the Covid-19. I was able to talk on the phone or Skype with him daily. This absolutely killed me, as I knew what we had gone through in the past and I wanted to be with him to ensure the maximum was being done. As we were battling AE, limited knowledge of healthcare workers and therapists have of both it and COVID-19 caused concern.
Apr. 28-Jun. 28 – Reggie was discharged from the sub-acute rehab for home-based rehab through the Medstar VNA. He received 10 sessions of PT and OT and 14 sessions of Speech throughout this time, and he improved every day.
Jul. 27 – Reggie was taken to Georgetown because he had a two-minute seizure at home. It was determined this “breakthrough” episode was caused by Reggie’s not following his medication protocol, i.e. not taking anti-seizure medications as prescribed. Even before his bout with AE, Reggie was non-compliant with medications and argumentative about taking anything, even vitamins. While in the hospital, he completed his second round of rituximab infusion.
Aug. 13- Oct. – Reggie is now an outpatient who receives OT and speech therapy at least twice weekly. He is making great strides and progress and I know will continue to recover with these therapies. I am impressed by these therapists, as they are trained in the field and have a neurological background. The OT and the speech therapists work together to help Reggie with his aphasia and apraxia toward reaching his stated goals. At the time of this writing, I am hopeful and joyful of what is going to be a better than before recovery!
Conclusion – This is the entire saga to this point with Reginald Johnson. As you can see, we have been through much that is both heart-wrenching and frustrating. At times, we not only have had to fight this disease, we have to fight the medical community so that they would not stop searching, treating, and taking proper care of him.
Reggie appears to get better every day. I have faith that full recovery is possible and will happen. I wrote this so all who choose to read it can be helped and assisted in any way. More importantly, understand to fight for your loved ones—especially when they cannot fight for themselves!
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.
September 12, 2020 | By WhereAreMyPillows.com for the International Autoimmune Encephalitis Society
A few weeks ago, I celebrated the 1 year anniversary of my discharge from hospital.
There’s been tons of ups, as well as some downs in the past year—but ultimately, I write this to provide a message of hope to those that are having a hard time with autoimmune encephalitis (AE) right now.
My journey began in 2014, when I suffered a flu-like illness after which I was never quite the same. For 5 years I lived with a mystery illness that was slow to settle in and occasionally remitted, but progressively robbed me of my key cognitive abilities. I dropped out of university (twice) and suffered countless periods where I felt like the living dead.
The future became increasingly bleak, as I lost trust in my basic ability to think—let alone the capacity to ever make a living for myself.
Then summer 2019 came.
Through the haze, there’s one memory that sticks out: laying in a hospital bed for the first time, and looking over at my mom. I told her I thought this hospital stay was my last hope of ever getting better.
Imagine the heartbreak she must have felt in that moment.
To her credit, she channeled my ominous words into sharpening her advocacy efforts. While my mind was adrift in a catatonic stupor, she fielded questions from a bevy of unhelpful specialists who looked at her with scrutiny and disdain. Being the determined woman that she is, she held her ground and didn’t let anyone push her around.
My mom tells me at least 4 neurologists came by—and all were absolute crap. They either thought I was fine, or that I was a crazy malingerer. But thanks to her insistence that something was medically wrong, a specific psychiatrist was sought out as a consultant to my case.
I consider that one psychiatrist my angel: she fought for the PET scan crucial to my diagnosis, as well as the Rituximab a medical committee tried to bar me from accessing. I’m fortunate that my mom and psychiatrist never gave up on me, even after my inadequate response to the steroids and IVIG initially administered. At one point I felt like my life was over, but lo and behold: 7 weeks after my first Rituximab infusion, I started experiencing significant gains. By week 10, I had gotten about ~95% back to baseline. The end of 2019 and early 2020 are some of the best months I’ve ever experienced.
I wish the story ended here, but the fact of the matter is that some challenges remain. I’m back on the mend via Rituximab again, after experiencing a relapse this spring. You can read more details about what’s happened since then at www.wherearemypillows.com.
Despite the setback, I’ve improved considerably from my worst points last summer, and consider myself lucky to have parts of my brain back I thought I had permanently lost. Though my recovery has stretched on longer than anticipated, the important thing is that it progresses upwards nonetheless.
In the meantime, I’m grateful to have a community of fellow AE warriors to fall back on. I want to shout a huge THANK YOU to the International Autoimmune Encephalitis Society and their lively Facebook group, which has provided me with endless emotional support and a pool of knowledge with which to fight my personal AE battle. It’s humbling to be in a position now to pay it forward and help others touched by AE—and to further this personal mission, I’m delighted to announce that I’ll be sharing my experiences in a new #WhereAreMyPillows monthly column. These will be published right here on the IAES blog!
While none of us want to be running this marathon, I hope that the burden of AE is a bit lighter knowing that you’re not alone. I got back on my feet after 5 years of misdiagnosis— a reality that seemed a mere pipedream just a year ago. Don’t give up and don’t lose hope!
WhereAreMyPillows is a seronegative AE survivor from Canada. Her favourite activities include writing on her health blog, taking photos, doing yoga, and finding her next spot to take a nap.
Join her on the IAES Facebook group, and on her WhereAreMyPillows Facebook Page, Instagram, and Twitter pages #wherearemypillows
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.
June-24-2020 | Carolyn Keating, PennNeuroKnow
As the name suggests, autoimmune encephalitis (AE) is a group of diseases in which the body’s immune system attacks the brain. To treat it, there are a variety of therapies that target different aspects of the immune system. The goal of these immunotherapies is to reduce brain inflammation and the resulting symptoms, as well as maintain these improvements by preventing relapses1.
Immunotherapy is most successful in patients with antibodies against cell-surface proteins (such as NMDR, LGI1, and Caspr2). These diseases tend to be caused by B cells and autoantibodies. In contrast, when antibodies are directed against molecules inside of cells (such as Hu, Ma, or GAD65) the disease is usually mediated by T cells, and these patients typically do not respond as well to immunotherapy. It should also be noted that removal of any disease-associated tumors, such as the ovarian teratomas frequently seen in NMDAR encephalitis or tumors seen in patients with intracellular antigens, should be an early treatment priority as removal quickly produces improvements2. However, there are currently no standardized treatment guidelines; at present, different regimens are used based on the patient’s particular condition and clinical status, as well as the opinion of their doctor.
The first treatment for most patients is typically steroids, also calledcorticosteroids. Corticosteroids act to broadly inhibit inflammation in multiple ways, which results in the depletion of mainly T cells. They offer the additional benefit of restoring the blood-brain barrier (BBB), which can be impaired in
AE. However, corticosteroids aren’t perfect. They have many side effects, and can aggravate or even induce psychiatric symptoms associated with AE such as depression, insomnia, agitation, and psychosis. What’s more, corticosteroids do not target B cells, the cells that go on to produce the antibodies that cause many of the symptoms of AE3.
Two other first-line therapies do target autoantibodies. One is administration of intravenous immunoglobulin (IVIg). IVIg is a blood product prepared from the serum of more than 1,000 donors that contains a broad range of antibodies. Some of these antibodies target a patient’s autoantibodies and neutralize them, along with other pro-inflammatory aspects of the immune system3. The other first-line treatment targeting autoantibodies is plasma exchange (PLEX, also called plasmapheresis). PLEX “cleans” the blood of autoantibodies by replacing the liquid plasma portion of a patient’s blood with that of a donor. PLEX also changes T and B cells in favorable ways. A more refined form of PLEX called immunoadsorption has also been used to treat AE, and selectively removes antibodies from the blood, instead of all the other components that are also in the plasma3. However, both PLEX and immunoadsorption only remove antibodies from the blood, not from the brain; although decreasing antibodies in the blood can lead to a decrease in the brain4. Furthermore, all first-line treatments but especially PLEX require a good deal of patient compliance, which can limit their use if the patient is agitated or displays other behavioral problems5.
Different subtypes of AE respond differently to treatment. For instance, patients with LGI1 antibodies who are diagnosed early are often responsive to corticosteroids alone. In contrast, only about 50% of patients with NMDAR antibodies are responsive to first-line treatments, and the remaining require second-line therapies6.
Second-line Treatments
There are two main second-line immunotherapies for AE. The first is a drug that destroys B cells called rituximab. Rituximab is actually an antibody that targets B cells, which normally go on to become antibody-producing cells. It is expected to work particularly well in patients with LGI1 and Caspr2 autoantibodies. However, because B cells can cross into the brain and become antibody-producing cells, but rituximab cannot cross the BBB, its effects may be limited3.
The other second-line treatment is a chemotherapy drug called cyclophosphamide. Cyclophosphamide directly prevents T and B cells from multiplying, but it affects the ability of many other cells to multiply as well. For that reason, it has some potentially serious side effects including infertility, and instead rituximab is usually the preferred second-line therapy3.
Alternative Treatments
Sometimes second-line treatments are also not effective at treating AE. When that happens, options include re-administration of first-line therapies, extended use of second-line therapies, or use of other non-steroid (steroid-sparing) drugs to suppress the immune system. For instance, the steroid-sparing drug mycophenolate mofetil prevents T and B cells from multiplying and has a better side-effect profile than cyclophosphamide3.
Other alternative treatments are also available. One option interrupts the inflammatory effects of a molecule called interleukin-6 (IL-6). Normally, when IL-6 binds to its receptors on immune cells, it causes B cells to multiply and mature into antibody-producing cells, and causes pro-inflammatory T cells to mature. The antibody drug tocilizumab targets the IL-6 receptor and prevents these inflammatory processes. A molecule related to IL-6, IL-2, is also a target. Instead of inhibiting this molecule, giving patients low doses of IL-2 activates a “good” type of T cell called regulatory T cells that help the body shut down autoimmune responses. Another option, bortezomib, directly targets antibody-producing cells, instead of their immature B cell precursors3.
Maintenance Treatments
Even if AE is successfully treated, sometimes the disease can relapse. Relapses could be caused by some antibody-producing cells that can survive for many months, which are not targeted by treatments. Many of the therapies described above, including the first-line treatments, steroid-sparing agents, and rituximab, have been used as maintenance therapy to try and prevent this from occurring. However, the length of time patients should continue to receive treatment is unknown, and can range from 6 months to several years depending on the patient’s condition and doctor’s opinion3.
In addition to immunotherapy, other important aspects of treatment include supportive care (particularly while in the hospital); treatment of symptoms such as seizures, spasms, and psychiatric issues; and rehabilitation1. While responses to tumor removal and immunotherapy are often seen within a few weeks, it may take years for patients to return to normal7. As more is discovered about which aspects of the immune system are involved in each subtype of AE, hopefully more directed treatments will become available.
J
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
1. López-Chiriboga, A. S. & Flanagan, E. P. Diagnostic and Therapeutic Approach to Autoimmune Neurologic Disorders. Semin. Neurol. 38, 392–402 (2018).
Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society cannot provide medical advice.
International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.
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