Categories
Advocacy CSI Pharmacy stories

Do you have a COVID-19 Preparedness Plan?

(BPT) – If you’re concerned about getting COVID-19, you have good cause.

According to the latest COVID-19 Data Tracker from the Centers for Disease Control and Prevention (CDC), the U.S. alone has already logged over 16.3 million cases and the overall weekly hospitalization rate is at its highest since the pandemic began.

While it’s critical to stay informed about the spread of the virus and know how to protect yourself and others, many people may not realize how important it is to be prepared with an action plan should an outbreak occur in your community or household.

The better informed you are, the more empowered you can feel about coping with the pandemic.

COVID-19 Preparedness Tips from a Professional Patient Advocate Who was Hospitalized

Professionally, Michelle Vogel works in patient advocacy and provider relations, helping other patients navigate the healthcare system. That experience has taught her that sometimes, patients must be proactive and push to find their best options. Preparing as much as possible in advance is paramount.

Have a testing plan. Know the symptoms and if you have been exposed or experience symptoms, know where to get tested in your area, and know how you’ll isolate or self-quarantine if necessary. Relevant information is listed and regularly updated on the CDC website.

Get to know the hospitals in your area. Look up local and regional hospitals in your area and make a plan for where you would seek treatment if you needed to be hospitalized. If you want access to investigational treatment candidates, websites like www.StopStorm.com can help you find research hospitals in your region as they may be enrolling people in clinical trials.

Ask about treatments and clinical trials. Everyone reacts differently to the coronavirus. As such, there’s no one-size-fits-all approach when it comes to treatment. If you’re hospitalized with COVID-19, ask about treatment options and if you may be eligible to enroll in a clinical trial. Since eligibility criteria can change, it’s important to keep asking your hospital and doctor about clinical trials daily.

Throughout this crisis, the biotech and pharmaceutical industries have worked tirelessly to develop vaccines and therapeutics to help overcome this pandemic. However, with over 4,000 COVID-19 clinical trials listed on www.clinicaltrials.gov and the healthcare system almost overwhelmed, the burden to get access to clinical trials is now often falling to the patient or their families who may need to proactively plan, ask and engage.

Clinical trials are crucial in the development of innovative medicines and participating in a clinical trial helps advance potential treatment options for people in need.

“Typically, hospital researchers will discuss clinical trial options proactively with patients who may be eligible to enroll,” said Vogel. “I think that there is so much research going on right now, patients and their families have to be more vocal and engaged in asking about potential clinical trials than ever before.”

What Happens When the Professional Patient Advocate Becomes the Patient?

The expression, ‘when it rains, it pours,’ doesn’t even begin to capture how torrential Michelle Vogel’s experience was with COVID-19. It was the midst of Florida’s hurricane season, the virus was surging in the state and her mom was just diagnosed with COVID-19 in Boynton Beach, Florida, days prior.

While Michelle, a 53-year-old Jacksonville, Florida, resident, waited to get her COVID-19 test, delayed due to heavy storms, her symptoms were getting worse. After her test came back as positive, she went to the research hospital in her area and was admitted to the Mayo Clinic in Jacksonville. She was diagnosed with pneumonia and was treated with convalescent plasma, remdesivir and steroids. Her condition deteriorated and she developed a hyper-response of the immune system that causes harmful inflammation, known as cytokine storm. The condition affects almost 90% of patients hospitalized with COVID-19 and is a leading cause of COVID-19 progression, use of a ventilator and death.

Vogel asked about clinical trials and learned that the Mayo Clinic was one of the sites enrolling patients in a Phase 3 study evaluating lenzilumab, an investigational treatment candidate designed to combat cytokine storm. She pushed to be enrolled in the clinical trial.

The Unsung Heroes

“Patients participating in clinical trials are too often the unsung heroes in the development of innovative treatments and the pandemic has heightened the importance of patient advocacy in the clinical trial process,” said Cameron Durrant, MD, MBA, chief executive officer of Humanigen, the biopharmaceutical company developing lenzilumab as a potential COVID-19 treatment. “The global biopharmaceutical industry has made tremendous strides to develop viable COVID-19 vaccines, but we need treatment options for all patients while we wait for widespread safe and effective vaccine use and availability.”

For Michelle Vogel, her condition improved rapidly after participating in the clinical trial. She had read that lenzilumab could help normalize her C-reactive protein and that is what she experienced. Just days later, she was weaned off supplemental oxygen and discharged from the hospital.

“If I hadn’t pushed to see what other options were out there, I don’t know if the lenzilumab trial would’ve been offered,” she said. “Because of my professional experience as a patient advocate, I knew to keep on pushing to see what my options were. But there are many patients who don’t even know that they can ask what their treatment options are.”

Sadly, however, Michelle’s mom passed away from COVID-19 while Michelle was in her own COVID-19 battle. “I tried hard to advocate for the best treatments for my mom but there were no clinical trial options at her hospital and it’s difficult to even think about whether she would have survived if she had access to a clinical trial.”

To learn more and to find a lenzilumab clinical trial site near you, visit www.StopStorm.com.

Categories
Advocacy Patient stories

IG Shortage is Coming

Walter “Kip” Caro started noticing tingling, numbness, and pain in his feet back in 2007. Gradually these symptoms progressed up his legs and into his body to the point where he had to be hospitalized because his torso was affected and he couldn’t breathe. It took three years for doctors to diagnose him with chronic inflammatory demyelinating polyneuropathy (CIDP). In that time, Kip suffered permanent nerve damage in his feet.

For the last 11 years, however, Kip has been treated with intravenous immune globulin (IVIG), which has worked like a charm. He never has to worry about his symptoms, unless he can’t get his treatments every three weeks. If he doesn’t, as happened last June when there was a shortage and his infusion center couldn’t get immune globulin (IG) products from their distributor, his disease starts to relapse.

“It was scary,” he says of this experience.

“Over the years, we’ve met people said they could make a change to their treatment schedule, and their symptoms would not be affected,” says Kip’s wife Bonnie Joslin. “But we’ve learned, for Kip, his body just can’t do that.”

Kip feels lucky that Bonnie is such an advocate for him. By the time his infusion center called and told him they weren’t able to get his medication, Bonnie had already been reading about other patients’ experiences online. She immediately contacted Michelle Vogel, who administers a Facebook page for people who depend on IG therapy. Michelle, who also serves as vice president for patient advocacy at CSI Pharmacy, was able to get Kip hooked up with home infusions within a week.

“He was already starting to relapse,” Bonnie says. “But Michelle pushed it through, and Natalie Edwards in the insurance department worked to get approval. I know it wasn’t easy, but it was like a miracle happened.”

By early 2021, however, nearly all IG manufacturers expect to see shortages again. This time it’s because pandemic shutdowns earlier in the spring have decreased plasma donations. Plasma is the raw material from which IG and other protein therapies are made. The manufacturing process takes seven to nine months to create IG from donated plasma, so unlike last year’s shortage, this time we know it’s coming and can be prepared.

When IG products are in short supply, rationing will happen, Michelle says. “When the market tightens and we have shortages, what tends to happen is that certain sites of care like hospitals may stop treating outpatients. If they do treat outpatients, they will limit treatment to primary immune deficiency, Kawasaki disease, and transplant patients.”

That’s because supplies of IG are not universally distributed. Hospitals and infusion centers have contracts with certain manufacturers or distributors, so they can only get specific brands. Last year, patients like Kip who were served by these sites of care sometimes found their treatments delayed or dropped completely.

Specialty pharmacies like CSI Pharmacy that offer home infusion services, however, have access to more brands of IVIG as well as subcutaneous immune globulin (SCIG). In 2019, CSI Pharmacy was able to work with patients, their healthcare providers, and their insurance plans to allow them to continue IG treatment in the home.

At times this meant switching to a different brand of IVIG when certain brands were not available. Some patients even switched to subcutaneous (meaning under the skin) infusion after being on the intravenous (IV) form.

While changing to a different product can be scary for patients who have confidence in a brand of IG that is working well for them, please be assured that  a switch can be relatively comfortable if you work closely with your doctor and pharmacist. The pharmacist can find a brand that is close in formulation to your current brand, for example. If side effects become a problem, the infusion rate can be reduced and pre-medications prescribed. 

If you depend on IG therapy, now is the time to prepare. We suggest you have a conversation with your provider and current site of care.

  • Ask your provider what other brands are good options if your brand is in short supply, and ask your care site if they are able to obtain one of these brands if needed.
  • Ask the site of care if they will prioritize patients if there is a shortage. Will it be based on disease, IgG levels, and/or rationing product by the number of grams or frequency?
  • Ask how the site of care handled past shortages.

If it turns out that your site of care does not prioritize patients with your disease or they are unable to obtain your brand of IG, you may want to consider how important is it to you that you continue IG therapy. (Some patients feel comfortable skipping their treatment for a month or two if it means they don’t have to make changes.)

If you’re like Kip, however, and can’t function without regular IG infusions, you’ll need to think about what options are available to enable you to access this treatment. Are you willing to switch brands? Are you willing to move to home infusion? Are you willing to switch to SCIG?

If home infusion becomes your alternative, we urge patients to make that move now. Specialty pharmacies are more flexible and were less affected during the last shortage, but that may not be the case this time.

If you are a patient of a specialty pharmacy when this crisis hits, you may be able to get access to IG since they do not ration based on disease and have access to more brands. However, we do not know how the shortage will affect sites of care. 

“We want to see all patients have access to all brands in their preferred site of care,” Michelle says. “But we just don’t know what the impact is going to be. We expect this shortage will be across all manufacturers. Patients should understand that this is not going to be a perfect scenario. You may still run into hurdles like access to your preferred brand.”

CSI Pharmacy’s patient advocates are available to discuss your options related to IG therapy, regardless of whether or not you are our patient. If we can’t service your needs, we will gladly help you find other resources. No patient should suffer alone.

Categories
Advocacy Patient stories

Disability Benefits in Danger

Andrea Williams had a panic attack when she opened her mail earlier this year. Her hands were shaking even before she tore into the envelope from the Social Security Administration (SSA). The letter inside informed her that, according to SSA, she was no longer disabled and would no longer receive disability benefits, including Social Security Disability Income (SSDI) and Medicare.

Andrea is one of thousands of American citizens who are unable to work because of severe, chronic health problems who can expect to receive these notices in the coming year. This is because SSA wants to cut $2.6 billion dollars from SSDI and its sister program Supplemental Security Income (SSI) by changing the rules they use to terminate people like Andrea, who have depended on the disability safety net for years.

Six years ago, Andrea was diagnosed with a rare, debilitating disease of the muscles called myositis. She had trouble lifting her head, she couldn’t pick up her newborn baby, she couldn’t climb stairs, she couldn’t even lift her arms enough to wash her hair. For months she went back and forth to the doctor saying, “I feel like I’m dying.” It wasn’t until she was referred to a specialist and ended up in the hospital for a week that she was finally diagnosed.

Myositis causes chronic pain, disabling weakness, and extreme fatigue. Treatment has helped Andrea, but she has lost too much muscle tissue that she will never regain. She can’t stand for any length of time, she drops things, and the brain fog from her medications makes it difficult for her to think. Her doctor told her she would never work again. When she applied for SSDI benefits in 2016, her application was accepted on the first try, which almost never happens.

“I’m scared to death,” she says. “My biggest fear is not having the medications, and I already can’t afford the doctors.”

An “Explanation of Determination” letter like the one Andrea received is a notice an SSDI or SSI recipient gets when they have been identified by SSA for “Continuing Disability Review” (CDR). This is the agency’s review process to see if beneficiaries are still medically eligible for the program. After this review, if SSA believes the person no longer meets their criteria for disability, their benefits are terminated.

A medical CDR is done at least once every three years, unless the SSA expects your medical condition to improve sooner. Those who have a medical condition that is not expected to improve undergo a CDR every seven years.

A rules change that took effect earlier this year, however, dramatically increases how often a person must undergo a CDR review. This change adds a new category for those whose condition is “likely to improve.” Hundreds of thousands of people now in less frequent CDR categories will be moved into this new category to be reviewed every two years.

This new rules change is especially disturbing because it targets people like Andrea who have chronic conditions that flare up unpredictably. Those who are approaching retirement age and those with mental illness are some of the other people this rules change aims to remove from benefits.

“If you get one of these letters, the first thing you need to do is notify Social Security that you want to appeal this decision,” says Michelle Vogel, CSI’s vice president for patient advocacy.

To appeal, you need to submit a Request for Reconsideration form within 60 days of receiving the notice of denial of benefits. If you want to continue receiving benefits while your case is being decided, you will need to submit this form withing 10 days of the denial, and you need to specifically ask that benefits continue. Be aware, however, that if your case is unsuccessful, SSA can require you to return the overpaid benefits.

Andrea has followed this advice and is awaiting a response from SSA. She is hopeful that, with the support of her doctors and her patient advocate, her appeal will be granted and she will once again be able to sleep at night.  

For others like her, Andrea offers this advice:

“You have to reach out and get some help from someone like a patient advocate,” she says. “You have to have your medicine. You have to see your doctors. You can’t take no for an answer.”

CSI Pharmacy has patient advocates who can help you navigate this and other health care access challenges. We offer this service regardless of whether you are a CSI Pharmacy patient.

Categories
Advocacy CSI Pharmacy stories

CSI Pharmacy’s Plasma Donor Superhero

Maddie was in high school when she developed juvenile dermatomyositis, a rare autoimmune disease of the muscles that made her so weak she couldn’t walk. Bill developed myasthenia gravis, another autoimmune neuromuscular disease, after having anesthesia for bypass surgery. Amanda and her daughter have immune deficiency diseases that make them susceptible to all kinds of infections. Immune globulin (IG) has been a life-saving therapy for all of these individuals.

IG is one of a number of treatments that can only be made from donated human plasma. This year, as a result of coronavirus restrictions, plasma therapeutics companies have experienced a significant drop in donations, which will limit supplies of plasma products such as IG by the end of the year. For patients, this is their worst fear. It means they may not be able to get the medications that allow them to live a normal life.

When Justin McNeill learned that plasma donations were down by as much as 40%, he thought of patients like Maddie, Bill, Amanda, and her daughter. Much of CSI Pharmacy’s business involves providing home infusion services for those who depend on IG therapy. As a delivery technician for CSI Pharmacy, it’s Justin’s job to pack up shipments of immune globulin and the supplies needed to administer it and make sure it all gets to the patient’s home in time for their infusion. 

In the spring, CSI Pharmacy joined the Immunoglobulin National Society in an effort to raise awareness about plasma donation and to inspire more healthy donors to contribute. As part of that effort, the company initiated an internal contest to encourage employees to become plasma donors. Justin was among the first to respond.

“We were told that with all the coronavirus restrictions, people aren’t donating plasma as much,” Justin says. “That means patients aren’t going to be able to get the medicine they need. I figured I’m able to give, so there’s no reason not to.”

Justin started donating in May and has given twice a week ever since—the maximum weekly donations allowed. To date, he has donated plasma 24 times. And even though he works full time and goes to school in the evenings, showing up at the BPL Plasma donation center is part of his weekly routine. He plans to keep on giving as long as they’ll let him.

Justin may have run away with this contest, but he’s not the only CSI Pharmacy employee to participate in the plasma donor drive. Eleven other members of the staff have also donated at least twice. (Regulations require two donations before the plasma can be used to make plasma protein therapies like IG.)

The rules governing who can qualify as a plasma donor are very strict. Justin, who is 24 years old and healthy, had no problem qualifying. When several other employees attempted to donate, however, they were turned away because they have chronic health conditions or other restrictions. This only made Justin more committed to continue donating.

“I knew a lot of the people here in the office couldn’t donate because of various health issues or medications, so I said, why not me?”

“Justin is very modest,” says James Sheets, CEO of CSI Pharmacy. “I know he doesn’t like to call attention to himself. But for us he is a superhero. We are pleased that our employees take this so seriously and are willing to donate plasma. And we’re extremely proud of Justin for his ongoing commitment to making plasma donation a part of his life.”

For Justin, it’s all about Maddie, Bill, Amanda and her daughter, and others for whom he packs up the products and supplies for their home infusions. He urges anyone who qualifies to consider becoming a plasma donor.

“We’re probably about to get hit with a really bad shortage of IG products,” he says. “Our patients need this medicine that’s made from human plasma. We’ve got a lot of people who are really sick and really need this medicine. Even donating just twice will help save lives. You can make a big difference.”

As the winner of CSI Pharmacy’s Plasma Donation Incentive Program, Justin McNeill was presented with a trophy and a monetary gift during a ceremony in September.

Find a plasma donation center near you.

Categories
Advocacy CSI Pharmacy stories Health Information

Be an Advocate

COVID-19 was the last thing on Michelle Vogel’s mind the day she raced to South Florida to care for her elderly mother who had fallen in her home. Her mom hadn’t been feeling well that week. She thought she had a urinary tract infection again. As it turned out, Judy Vogel had COVID pneumonia. She died a week later in ICU.

Michelle is the head of CSI Pharmacy’s Patient Advocacy team. Since the beginning of the pandemic, she has tirelessly insisted that everyone needs to wear a face mask, wash their hands or use hand sanitizer, and maintain social distancing. And she walks the talk. She knows that the lives of the immune compromised patients she cares for depend on this.

It never occurred to her, however, that she would need to protect herself from her own mother. Judy lived alone. She rarely left the house. And she took precautions. No one knows how she might have contracted this highly contagious condition, but she gave it to her daughter.

Five days after learning of her mom’s diagnosis, Michelle herself tested positive for COVID. As someone who lives with several rare, chronic conditions, she knew her chances of developing severe COVID pneumonia were high, and over the next few days she did become very sick.

“I’ve had migraine headaches, but they’re nothing compared to COVID headaches,” Michelle says. “And I’ve never been so tired. I’ve never had so much pain in my legs that they just give out. The coughing is worse than any bronchitis. And then it’s just odd to lose your smell and taste. My stomach, the diarrhea, the chills and fevers…it just hits every part of you. I even have skin lesions.”

With the support of daily telemedicine checks, Michelle battled the disease at home for a week. Her doctors started her on a corticosteroid (prednisone), which has been shown to reduce lung inflammation in COVID patients. They tried azithromycin (Z-Pak), which has antiviral properties, although it has not been reliably tested in COVID patients.

They also ordered cough suppressant medicine and a portable nebulizer that helped her to breathe in a bigger dose of medication to open her lung passages. From an online supplier, she ordered a pulse oximeter (a medical device that fits over your finger) to be sure her blood oxygen levels stayed adequate. Still, she ended up in the hospital with COVID pneumonia.

“There’s a lot doctors can’t see on a telemedicine call,” she says. “They don’t see you when you’re gasping for air. They can’t listen to your lungs to hear how congested you are. And how do you get a chest x-ray or labs drawn when you’re too exhausted to drag yourself out of the house?”

Michelle is the person many rare disease patients turn to for advice on navigating the maze that is our healthcare system, overcoming health insurance obstacles, and accessing the expensive therapies that keep them alive. She is an expert who loves sorting out these challenges.

So during the week she spent in the hospital, Michelle became her own advocate. She knew, for example, that remdesivir (an antiviral medication) and convalescent plasma (blood serum with antibodies from COVID patients who have recovered from the disease) had shown some positive results, so she requested these. She also asked about other treatments and was offered a clinical trial to test a new biological therapy.

While no one expects to come down with COVID—or any other disease, for that matter—Michelle’s experience shows the importance of educating yourself about whatever condition you find yourself burdened with. Know what drugs and therapies are used to treat the disease and ask if they might be right for you (or your loved one). And if you don’t understand what the doctors are saying, ask questions until you do.

“COVID-19 has affected all of us in one way or another,” Michelle says. “I have stayed vigilant in wearing my mask and isolating to stay safe. I never imagined that it would touch my family, take my mother, and leave me battling with COVID pneumonia. Please stay safe so this doesn’t happen to you or someone you love. And if it does, be an advocate for yourself or your loved one.”

Categories
Advocacy CSI Pharmacy stories Health Information

Can’t be Complacent with COVID

Judith Vogel had lovely hands, always perfectly manicured, and beautiful, beautiful eyes with long lashes. She taught fifth grade for most of her life in Montville, New Jersey and saved every letter her daughter Michelle wrote to her from camp as a child.

At 81, she lived alone, but she enjoyed a full social life: playing canasta and mahjong, going to movies, eating out. She had lots of friends. With this year’s pandemic restrictions, though, Judy lost all that. All she could do was sit at home and watch the news and fret. She was afraid she would get sick. And she worried about our country, all the hatred she saw. All the fear.

What she really wanted was to see her grandson get married (his wedding was postponed twice because of the pandemic). And on her birthday, November 3, she wanted to celebrate by voting for Joe Biden. He could turn things around in this country. She was convinced.

Judy will enjoy neither of these dreams. She passed away on August 20. She died, as she feared, of COVID-19.

It was a Thursday morning. Michelle hadn’t been able to get her mom on the phone for two days. She threw some things in the car and drove the 300 miles to South Florida. She made it in record time. On the way, she called her cousin who lived closer and asked her to go check. They found Judy on the floor. She had probably lain there for a full day.

When the paramedics arrived, they decided she was OK. She was conscious. She didn’t have a fever. She wasn’t coughing. They didn’t want to risk taking this elderly lady to the hospital where she might catch COVID.

When Michelle got there, her mom was sitting comfortably in a chair, sipping fluids. She made an appointment for Judy to be seen by her primary care provider the following morning. Judy had a history of urinary tract infections (UTIs). Michelle assumed that’s what was making her confused and fatigued. That and the fact that she’d lain on the floor for the last 24 hours.

Michelle, who serves as CSI Pharmacy’s Vice President of Patient Advocacy and Provider Relations, took her mom to the doctor and got her started on treatment. But that afternoon, when Judy was too weak to stand up from a chair, Michelle knew something more serious was going on.

She had to call 911. It was the only way to get her mom to the emergency room. But she couldn’t go with her; no one is allowed in hospitals these days except the patient. That evening when she called the hospital, she learned that Judy had tested positive for COVID. Judy was in ICU. Soon she would be on a ventilator. Michelle would never see her mom again.

“I was shocked,” Michelle says. “I thought she had a UTI. I didn’t put the symptoms together. I never thought of COVID.”

Looking back, Michelle realizes there were a lot of signs she missed when she talked to her mom every day. She thought, for example, that her mom’s decreased appetite was related to the isolation and depression Judy was feeling. But maybe she wasn’t eating because she couldn’t taste or smell. These are symptoms of COVID.

Judy complained of headaches and muscle aches, but she didn’t have a fever. She just thought she was coming down with a cold. When Michelle talked to her mom’s best friend, though, she said Judy had been coughing for weeks. Maybe she’d been sick for weeks, but no one realized it. Judy didn’t like to bother anyone.

The thought of her mother lying on the floor alone all day and all night before she was finally found will never leave Michelle. But even as she moves through her own grief, Michelle wants her family’s experience to serve as a lesson for others.

“Isolation is hard on everyone,” she says, “but it is especially difficult for our seniors. It affects us both physically and emotionally. It can be really horrible. But as much as people want to be more socially active and get back to their lives, this virus is going to go on for years. And the more complacent we are, the more severe it will be. We can’t assume COVID hasn’t affected anyone in our personal circle. We still need to take precautions. We need to be safe.”

Secondly, she wants people to be aware that COVID is a threat that is with us everywhere, and that coronavirus should be at the top of our minds at all times.

“We don’t really understand all the symptoms of COVID-19,” she says. “A lot of patients never present with a fever, but they have all these other symptoms: severe headaches, body pain, diarrhea, rashes, weakness, tingling toes…all kinds of things. We need to understand that there are many more symptoms than just the cough and fever that you always hear about. And if you are feeling bad, you need to get yourself to the doctor.”

Categories
Advocacy Health Information

Tips and Tricks for Managing MG

We recently spoke with neurologist Charlene Hafer-Macko, MD from the University of Maryland’s Myasthenia Gravis Center about ways myasthenia gravis (MG) patients can keep themselves healthy and stay out of crisis. Here are some of her tips and tricks:

Communicating about your condition with healthcare providers, especially in an emergency situation, can be a challenge.

  • Wear a medical ID bracelet, such as the MedicAlert, that identifies you as having myasthenia gravis.
  • Know what medications MG patients need to avoid, and carry a list of them in your wallet for easy reference.
  • Download the MyMG app from the Myasthenia Gravis Foundation of America. The app also has a list of medications to be avoided with MG.
  • Document your symptoms, treatments, and how they affect your daily life so you can have these data readily available when your doctor asks, “How have you been feeling?”
  • Always ask your healthcare provider or pharmacist if a newly prescribed medication is on the list of drugs MG patients need to avoid.
  • Use online resources to look up conditions and medications. Just be sure the source is reliable and informed by science.

Weak muscles can make breathing difficult, but there are things you can do to make breathing more efficient.

  • Use pursed-lip breathing, a technique in which you breathe out through puckered lips. See this demonstration.
  • Learn how to breathe into your belly by lowering your diaphragm. Learn how here.
  • Explore mindfulness practices that focus on breathing. This is also helpful for stress reduction. Find a guided exercise here.

MG symptoms tend to get worse with heat for many people. To avoid melting when it’s warm:

  • Take tepid showers. If you really like a hot shower, finish it off with a cold splash.
  • Avoid being out in the sun for long periods of time.
  • When the weather is hot, plan big activities for the cooler part of the day, and take advantage of air conditioning as much as possible. Ask your electric power company for a form that will ensure you are a higher priority for power when the electric goes out a storm.
  • Consider using a cooling vest if, for example, you want to sit in the hot sun for hours at the baseball game. Here is a sample of some available models.
  • Sporting goods stores also sell cooling towels and reusable, freezable gel packs.

Not getting enough sleep will make anybody more fatigued. For those with MG, insomnia can be related to corticosteroid use, anxiety, stress, and other effects of chronic illness. Good sleep habits can help. Here are some tips:

  • If you take prednisone, do so early in the day.
  • Schedule your bedtime so you get at least 7-8 hours of sleep.
  • Make a habit of going to bed at the same time every night and getting up at the same time each morning, even on the weekends.
  • Keep your bedroom quiet, dark, relaxing, and at a cool temperature.
  • Create a relaxing bedtime routine.
  • Ban electronic devices, such as TVs, computers, and smart phones, from the bedroom, and avoid using screens during the hour before you go to sleep.
  • Avoid large meals, caffeine, and alcohol before bedtime.
  • Exercise regularly to help you fall asleep at night.

And finally, Hafer-Macko advises that those living with MG watch their bodies, learn how their treatments affect them, and plan activities accordingly. For example, if you (or those you live with) notice that you start to slow down as you get closer to your next pyridostigmine (Mestinon) dose or IVIG infusion, that may not be the best time to schedule a big day with the kids that will use up a lot of your energy.

Similarly, if you take pyridostigmine, notice how it affects you. If it starts to wear off too soon, mention it to your doctor; you might need to adjust the dose. Also, be aware of scheduling high-energy activities like shopping or cleaning during times when your meds are wearing off.

The following organizations offer additional resources, including support groups, education, and research:


Categories
Advocacy Patient communities Patient stories

Being There for Those in Need

Rebekah Dorr never set out to become a myasthenia gravis patient advocate. It started with her Facebook page, Myasthenia Gravis Unmasked, and just sort of evolved. That’s where, in 2014, Rebekah first shared the story of her own harrowing journey with myasthenia gravis (MG) and some of what she learned along the way. She wanted to bring hope to those who live with the disease by raising awareness about how it affects individuals and correct some of the misconceptions even the medical community still endorses.

When someone messaged her on the page asking for help, she wasn’t sure what she could do. “But I’d advocated for myself,” she says. “So I thought, let me see if I can help this person. I think she was indebted to me, because she turned around and started sharing about me in other groups.”

Since that time, Rebekah has lived on her phone. She posts educational content about living with MG and provides a platform for the personal stories that give voice to the challenges myasthenics face every day. She also responds to every comment and private message—sometimes dozens a day—from those with questions or who need her help to get the care they need.

“I was frustrated with what was available at the time,” Rebekah says of the MG support system. “There was research, there were support groups, and that was it. There was this huge no man’s land for what was happening for the patients. And I was like, who’s taking care of patients the way I needed to be taken care of? So that ended up becoming my passion.”

At least part of this passion for walking with patients in their time of need comes from her own experience. She knows what it’s like to be alone and afraid, not understanding what was happening to her, and not trusting the medical community to make the right decisions for her care.

Rebekah’s symptoms began one summer when, out of the blue, she started feeling really tired, like she had the flu. Very quickly, however, those symptoms escalated to significant shortness of breath. She had trouble chewing and swallowing, and her legs became so weak she couldn’t walk. It seemed like one minute she was playing on the beach with her cousins and the next she was unable to get out of bed.

The next two years was a terrifying odyssey that included countless ER visits, hospitalizations—including several stays in ICU and more than one time when she had to be resuscitated—lab tests, scans, surgeries, spinal taps, and specialist consultations. It was a time when, looking back, she wished she’d had someone she could have called upon to help her know what to do.

Doctors refused to believe that a woman of 22 could develop MG, despite the fact that her grandmother also has MG and other evidence to support the diagnosis. Instead, they said she was faking the fact that she couldn’t walk and couldn’t breathe, and diagnosed her with somatic conversion disorder (meaning she was mentally ill, making it up). This label, together with the disrespect with which she was treated, did more damage to her health and spirit than MG ever could. It made her question her own truth and made her terrified to seek the care she desperately needed.

“I didn’t know anything,” Rebekah says. “I didn’t know blood tests for antibodies had to be sent to a special reference lab. I didn’t know my shortness of breath wouldn’t necessarily make my oxygen saturation go down. They didn’t explain the drugs to me. I had no idea I was being overdosed. I had no idea what any of it was.”

But she learned. Having people who depended on her for answers forced Rebekah to dig into the research and understand all she could about MG. She quizzed her own neurologist, listened closely to conversations she heard in hospital hallways, and read everything she could get her hands on. She also listened to the stories of patients. And she became the expert others needed.

“Word of mouth was spreading about me,” she says. “I don’t think the word advocacy was ever used, but it was just, hey, contact this woman, she’ll help you. And so I started getting flooded with messages. It became a job for me. It became my life.”

Rebekah now has clients all over the world, some of whom she works with for months or years at a time, sharing knowledge and awareness. More often, however, she’s there with patients—in-person for local clients, but by phone for most—when they need to go to the ER or are admitted to the hospital to help them navigate a system that often doesn’t understand this rare disease.

By 2016, however, Rebekah realized she was not receiving the kind of respect she needed from the healthcare community. She didn’t have credentials or the backing of some authority that would make medical professionals take her seriously. So she started her own nonprofit organization: The Myasthenia Gravis Hope Foundation.

“Our whole focus is advocacy,” she says of the Foundation. “I define that as clinical advocacy. We’re not just doing awareness or education. We’re actually coming in for the patient when they are most vulnerable to challenge the stigmas and misconceptions about MG that severely affect how they are perceived and treated.”

Beyond Rebekah’s lifesaving advocacy, MG Hope also provides funds for patients to travel for care and to cover the cost of critical medication until they can get enrolled with manufacturers’ assistance programs. The organization also helps patients access medical and specialty care and emergency medications.

For Rebekah this work—none of which she is paid for—is all about helping others avoid the hell she went through. She remembers sitting in a tiny closet of a hospital room which she had occupied for thirty-five days. She’d gone in for a thymectomy, but never got it. Instead, she experienced anaphylactic shock as a reaction to blood products, endured two resuscitation codes, went through cholinergic crisis because of titration mistakes, and so much more.

She remembers thinking if only somebody had educated her about these possibilities, she could have prevented nearly all of them. As a person of faith, that’s when she vowed to be the one to help others overcome or avoid these challenges. Now as the founder and CEO—and the only active member of the staff—of the MG Hope Foundation, she’s doing that work.

“I’m passionate about focusing on the patient experience,” Rebekah says. “I think that honesty and vulnerability is where we have the power to transform things, to actually step into somebody’s life and to maybe change it for the better. Whether they need emotional support or education, I want to show up for them in whatever way I can. That’s just where my heart is.”

Rebekah’s grandmother, Doris (95) was diagnosed with MG more than 60 years ago. This photo of Doris and Rebekah was part of MG Hope Foundation’s project called The Humanity Behind MG, designed to capture the essence of the human experience of those who live with the disease.

Categories
Advocacy

Standards Still Apply

Recently, a patient we will call Angela posted a note on a private Facebook page describing a troubling situation she encountered with her home IVIG infusions. She wrote to the group of fellow immune globulin (IG) patients that her infusion company decided that the nurse no longer needed to stay with her for the duration of her infusion.

Because of coronavirus infection risk, some infusion companies are apparently trying to limit the time the nurse spends in the patient’s home. The nurse is instructed to set up the infusion and stay until it had been increased to the scheduled drip rate, then she is to leave, for the rest of the day. Another patient in the group posted that her nurse didn’t leave, but she spent most of the time of the infusion sitting in her car outside the house.

“My infusions take basically eight hours, and she will be here for two of them,” Angela posted. “She’s going to teach my husband how to draw and administer diphenhydramine [an antihistamine used to counteract an allergic reaction, also known as Benadryl] in case of an emergency. I am nervous for sure. What will we do if air gets in the line? What will we do if something goes wrong?”

Angela’s concerns are not unfounded. According to Michelle Vogel, Vice President of Patient Advocacy and Provider Relations at CSI Pharmacy, leaving the patient during an infusion violates strict standards of care established by the Immune Globulin National Society (IgNS), an organization of Ig therapy professionals.

“Not only is this unacceptable, but it is extremely dangerous,” Michelle says. “The nurse needs to constantly monitor the patient for infusion reactions. This is crucial and cannot be done over the phone or if the nurse is not present.”

“As nurses our duty is to provide safe and effective nursing care,” says Brittany Isaacs, RN, IgCN, Director of Nursing at CSI Pharmacy. “Our nursing judgement should not be clouded by situations that place a patient or their safety in jeopardy. Our duty is to do no harm, so we need to protect both the patient and ourselves during any encounter. Ensuring proper personal protective equipment is doned to keep everyone safe and following the guidelines outlined by the CDC, WHO, IgNS, and the Infusion Nurses Society allows a nurse to continue to provide safe and effective nursing care during home infusions.”

While COVID-19 has caused many changes in healthcare protocols, patient safety should always be the ultimate guiding principle. The following guidelines are drawn from IgNS’s Immune Globulin Standards of Practice and COVID-19 Resource Guide and FAQ.

During the COVID-19 pandemic, patients receiving in-home immune globulin therapy can expect the following from their specialty pharmacy or home infusion company:

  1. Pharmacy personnel will wear personal protective equipment while packing the medications and supplies that are sent to the patient’s home.
  2. The home infusion nurse will be screened by their company for COVID-19 symptoms to ensure they will not carry infection into the patient’s home.
  3. Patients will be screened to ensure they do not have COVID-19 symptoms before being infused.
  4. Nurses will wear personal protective equipment, including masks, gown, gloves, and face protection, while in the home.
  5. Social distancing should be maintained to the extent possible, except when providing direct patient care.
  6. Patients should wear a mask or face covering while the nurse is in the home.
  7. Patients can request that their specialty pharmacy include masks, gloves, and hand sanitizer in their IG shipment.

The following practices are not acceptable, even during COVID-19 restrictions:

  1. Neither the patient nor a family member should be taught to self-infuse IVIG or to remove the IV after the infusion is completed.
  2. The infusion nurse should never leave the home for any reason while the infusion is in process. This includes sitting in her/his car outside of the home during the infusion.
  3. Family members should not be asked to leave the home during the infusion.
  4. Nurses should never refuse to wear personal protective equipment.

If you are uncomfortable or do not feel safe with your infusion company’s changes in protocol, please do not stop treatment! Staying on therapy is vital. If your company is unwilling to adhere to these standards of care, you may want to consider changing companies. If you need help with this, CSI Pharmacy’s patient advocates can help, even if you are not our patient.

Additional resources can be found here:

Infusion therapy standards of practice. Journal of Infusion Nursing

Immune Globulin National Society – Standards and guides

The role of an IG infusion nurse. IG Living Magazine. August/September 2013

National Home Infusion Association

IDF guide for nurses: Immunoglobulin therapy for primary immunodeficiency diseases Immune Deficiency Foundation

Categories
Advocacy Patient communities Patient stories

Paying It Forward

Ten years ago, Karon Faught started slurring her words and having trouble lifting her arms to blow dry her hair. She was only in her thirties, but some months later while at work, she couldn’t find the right words she wanted to say. She thought she was having a stroke. An MRI disproved this possibility, but it did nothing to alleviate the overwhelming fear she and her husband Jerry felt about what was causing these symptoms.

When she went to her neurologist, he had a list of conditions he wanted to test her for. Among them was not myasthenia gravis (MG).

“He said he was going to test me for MS and ALS and a couple other things,” Karon recalls. But nobody gets myasthenia gravis, the neurologist said, so I’m not even going to test for that.

Ironically, MG was the thing Karon’s primary care provider specifically suggested she be tested for. Also ironically, while she was in the office, the neurologist agreed to give Karon a magnesium infusion, a treatment she’d had before to treat her chronic migraine headaches.

What Karon and Jerry didn’t know at the time was that magnesium is one of a laundry list of drugs that makes myasthenia symptoms worse. That infusion persuaded the neurologist to do the blood test, because there in his office, Karon lost her ability to speak and move as a result of it. Three weeks later, the tests came back positive for anti-acetylcholine receptor antibody-positive MG (AChR MG).

This was just the beginning of a harrowing ride for Karon and Jerry, one they are grateful to the MG community in their home state of Texas for helping them survive.

“Ten years ago, there was really no good information about MG,” Jerry says. “Even at the top hospital in the region, one of the only hospitals in the country that was doing an MG clinical trial, people didn’t understand it. Their nurses were still giving IV Benadryl to MG patients, and their medical school was still teaching that kids couldn’t get MG. When we came into this, the best knowledge base was those that came before us.”

So when Karon was facing surgery to remove her thymus (a procedure that is often part of treating MG), she wanted to talk with others who had the disease and could help her understand what to expect from this major chest surgery. She and Jerry attended their first Myasthenia Gravis Foundation of America (MGFA) national conference that year and connected with the only MG support group in Texas, which was in Lubbock, about 300 miles from their home north of Dallas.

The group was led by Coleen Shinn. She and others from the group took the couple under their wing. And when Karon was in the hospital after surgery, they were a lifeline for Jerry who guarded his wife’s treatment from healthcare professionals who repeatedly tried to treat Karon with medications on that laundry list of drugs that make MG worse.

“After my surgery, Jerry was on the phone with Coleen almost 24/7, asking her questions and gathering information,” Karon says. “When the doctors wanted to give me a medicine, he would call Coleen and say, is this okay? And she would say yes or no. A lot of times it was no, don’t give her that medication, because she’ll go into crisis.”

Jerry chokes up when he remembers this time. “I owe a huge debt of gratitude to Coleen and Marvin and Lowell and Margaret and all the people in Lubbock who took us in. There’s no way I can explain how they helped us. There are just no words that can describe it.”

After Karon’s surgery, she and Jerry continued to attend support group meetings in Lubbock. They needed that companionship and the knowledge of others who lived with MG. But it was a five-hour trek, and they knew there had to be other MG patients closer to home. So armed with contacts they met at their second MGFA conference, they pulled together a support group in the Dallas-Fort Worth area.

Since then, the couple has created a nonprofit support network, MG Texas, whose only mission is to empower people by sharing knowledge about this rare neuromuscular disease. The organization now supports honorary Texans all over the world, and through the work of many others, there are now support groups in Dallas, Lubbock, Austin, San Antonio, Corpus Christi, San Angelo, and Beaumont. And because they live only 20 miles from the Oklahoma border, it was easy enough to restart the groups in Oklahoma City and Tulsa after the MGFA reorganization. They’ve also started, MGKids.com, the only nationwide MG support organization for children with MG.

“There are so many fears when a person comes into this,” Jerry says. “If we can alleviate any one of those fears to help them get to the next stage of the healing cycle, then that’s really what drives us.”

This support group network has also been part of the healing cycle for Karon and Jerry.

“The support group has helped us get through this,” Karon says. “Helping other people has helped us heal from what we’ve lost. Having a diagnosis like MG, you have to look at life a little bit differently and make some adjustments. But when you help other people, you don’t think so much about what you’re going through.”

“I’ll be honest with you, at the beginning, I was angry, Jerry says. “And the only way to battle this disease that we can’t fight is to educate so that it doesn’t hurt others. That was the why we started the support group in Dallas. The bonus was, if we get more people in the group, it will help us.”

But healing the fear and anger is only a part of Jerry’s motivation.

“There’s a debt we have to pay forward,” Jerry says. “We’re going to have it for a long time. And that’s really what drives me.”