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Advocacy Patient stories

Buyer Beware

The cost of health insurance had been a struggle for Diane Steele for years. Even the plans offered by her husband’s employer were too expensive when they had children at home. For a while, the family had to go without insurance. When Diane learned about health share programs, she saw this as an affordable way to protect her family.

Health share programs (sometimes called medishare or healthcare sharing ministries) are faith-based programs in which members pay a monthly “share” (notably not called a premium), which is pooled together with other members to pay for approved medical expenses.

While health share programs have been around for decades, because they are significantly less expensive than traditional health insurance, their popularity has increased in recent years as healthcare costs and insurance rates have skyrocketed. Part of the reason they are less expensive is they are not actual health insurance.

Program guidelines for what will be covered are often limited and may be determined by religious beliefs rather than medical need. Some health shares, for example, specifically exclude things like birth control, addiction treatment, or conditions related to smoking or alcohol abuse. Health share programs may even require members to sign a statement of faith, and some require verification of regular church attendance.

Because these programs have a religious affiliation, they also are not subject to mandates imposed by the Affordable Care Act (ACA), such as coverage for preexisting conditions and no lifetime caps on coverage. Most also specifically exclude drug coverage, especially for long-term treatment of chronic conditions such as diabetes or heart disease. Instead, members are steered to prescription discount cards like GoodRx and NeedyMeds to help pay for insulin and high blood pressure medication.

“We went with the health share because of the cost, and because we liked the idea of helping other people,” Diane says. “The funds go into a universal plan, and as people need it and qualify for it, they cover your costs. The only thing is you have to get approval up front for any kind of major outlay.”

Having to get approval for major healthcare expenses seemed like a small concession for Diane and her family, one that seemed routine when her husband needed major open-heart surgery a few years ago. The health share covered all of the expenses of that hospitalization with little hassle.

When Diane was diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) in 2017, however, she discovered limitations that were much more of a hassle. CIDP is a rare disease of the nerves and muscles in which inflammation causes disabling numbness, weakness, and pain in the legs and arms. It is a chronic condition that often requires a lifetime of treatment.

“I was so sick,” Diane says. “I couldn’t walk. I couldn’t drive. I was basically in a wheelchair most of the time. And I was gradually getting worse and worse.” 

Among the medications her neurologist prescribed for the condition was Gammagard, a form of intravenous immune globulin therapy (IVIG). This treatment, derived from donated human plasma, is very expensive, but it’s also very effective for a wide variety of autoimmune conditions like Diane’s. IVIG treatments did allow Diane to return to a more normal life, one that no longer includes a wheelchair, but the health share refused to pay for it.

“I worked and worked with the health share,” Diane says. “I talked to all kinds of people over there, and often had to ask for the supervisor’s supervisor. It was so complicated. They didn’t even know what Gammagard was.”

The health share required Diane to have a number of different tests to prove the diagnosis. They also required her to try other, less expensive medications (a process known as step therapy) to show that these treatments didn’t work for her condition. And they wouldn’t approve it just based on the doctor’s prescription. Her neurologist had to send them an extensive written report to justify the scientific basis for using IG for Diane’s disease.

To be fair, Diane’s experience with the health share program was not unique. Even traditional health insurance companies require preapproval for expensive treatments like IG therapy. Many also impose limitations on doses or frequency. There is often a large copay, and some will completely deny coverage for the treatment, especially if it is being prescribed off label (for a treatment that is not approved by the FDA for a particular condition).

But even after jumping through all these hoops, the health share only approved the treatment for forty-five days as a test to see if it would work. And the dose they allowed was also lower than what the doctor prescribed. Even when it did prove to be effective, the health share refused to cover the cost.

After the forty-five-day trial period, Diane worked with her specialty pharmacy to access the life-saving treatments she needed. They helped her apply for a special patient assistance program through the manufacturer that provided the medication for free. The specialty pharmacy then provided the nursing services and supplies at no cost. They also helped her apply for Social Security Disability, which would allow her to enroll in the more comprehensive benefits available through Medicare.

“Diane’s story is a cautionary tale that underscores the need to know what you’re buying when you purchase healthcare coverage of any kind,” says Michelle Vogel, head of patient advocacy for CSI Pharmacy. “All plans have restrictions and limitation. But when a plan is so inexpensive compared to the norm, you have to be especially careful to understand what they will pay for and be sure it covers the care you need.”

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Advocacy IG Therapy 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.

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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.

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Patient stories

Rolling with It

When Linda Matthews started having trouble standing up out of a chair, she wasn’t surprised. She was 70 years old, about the same age as her mother when she was diagnosed with inclusion body myositis (IBM).

“I was her caretaker, so I knew all about IBM,” Linda says. “As soon as I started showing symptoms, I went to my doctor and I said I’ve got IBM. They did the muscle biopsy and the bloodwork and the EMG. Within a week, it was definitely diagnosed.”

Often when someone is diagnosed with a disabling condition like IBM, they rely on their partner or a family member to care for them as the tasks of daily living become more difficult to manage alone. Linda, however, was already serving as caregiver for her husband Bill, who had been recently diagnosed with a different rare, autoimmune muscle disease: myasthenia gravis (MG).

In March of 2012, Bill had heart bypass surgery. About a month or so later during a routine eye exam, his ophthalmologist noticed his eyes looked a bit droopy. Linda’s brother, who had a friend with MG suggested he see a neurologist. When he did, it didn’t take long for the diagnosis to be confirmed. Bill says his neurologist thought it was the surgery that set it off.

Despite their disabling diagnoses, the Matthews do not despair. In fact, sharing similar diseases makes them more sensitive to each other’s needs. They have many of the same symptoms—muscle weakness, fatigue, mobility challenges—so they each understand, for example, when the other says they need to rest.

“Nothing is as important as getting the rest you need and taking care of yourself,” Linda says. “And it’s good that I know he’s got my back. I can say, ‘No, not right now,’ and I don’t have to justify it.”

“Sometimes you can tell when the care partner doesn’t understand that,” says Bill of other couples they’ve met. “And you can see how difficult it makes it for the person who’s got the disease. In a way, it’s to our advantage that we understand firsthand what the disease is doing to each other. We don’t have to have that fight.”

Having her mother pave the way for her has also made Linda’s journey just a bit easier. She knows what to expect, and she can plan accordingly as her muscle weakness progresses.

When she saw a Hoyer lift at an estate sale, for example, she scooped it up, knowing that one day it may come in handy if she starts having trouble moving from the bed to the chair. She has a motorized wheelchair that somebody gave her. It’s parked in the garage, though, since she still gets around fine with a rollator. The couple also recently traded their car for a wheelchair accessible van.

When they downsized their home earlier this year, Linda and Bill moved to a one-story (stairs had become a challenge) duplex next to their daughter’s family. Before they moved in, they remodeled the house to accommodate their future needs, adding a roll-in shower, raised toilets, grab bars in the bathroom, and wider doors that will accommodate a wheelchair.

The one thing that couldn’t be retrofitted for accessibility, though, was the kitchen. But Linda just winks and says, “Maybe by then I’ll teach Bill to cook, because I’m the one in the wheelchair.”

That’s not a problem for Bill. “We’ve been married for 56 years. We know how it’s done,” he says.

Linda and Bill feel very lucky to have the support of their family. They love living next to their daughter, son-in-law, and nine-year-old grandson and call on them when they need a hand. When they want to give their son-in-law a break—like when Bill needed to get to the hospital recently for cataract surgery—one of their two sons is always willing to step in and help out too.

Being part of a patient support community helps the couple in other ways. They belong to MG Texas, an MG support group in the Dallas/Fort Worth area. And they are active in Northeast Texas support group of The Myositis Association as well as on Facebook forums through Myositis Support and Understanding.

Getting together with others who share their disease helps Linda and Bill learn the medical ins and outs of both diseases, so they are better able to care for themselves and each other. It also gives them access to a whole pool of practical information that’s not readily available elsewhere: things like who is the best neurologist in a certain area, or tips and tricks people have used for managing certain challenges.

“The myositis Facebook site is wonderful, because people ask questions, and a lot of times it’s a question I have too,” Linda says. “And then in the MG group, we love it when we are able to go to the meetings. They’re always interesting, with new speakers and new ideas. It’s also nice getting to know other members who are in the same situation as we are.”

While living with conditions they know can cause significant health concerns, Linda and Bill remain upbeat.

“My mother was a wonderful example for me how to live my life with this disease,” Linda says. “You just roll with it and try to figure out another way of accomplishing your tasks.”

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Patient communities Patient stories

Partnering with Patients to Solve the Mystery of Myositis

When Lisa Christopher-Stine, MD, MPH and her colleagues formed the Myositis Center at Johns Hopkins University 13 years ago, they wanted to create a place where those affected by this collection of rare autoimmune muscle diseases could receive the very best care possible. The Center is a patient-centered, multidisciplinary clinic in which specialists in rheumatology, neurology, pulmonology, and rehabilitation come together to collaborate in the care of these very complex patients.

The Center also aims to better understanding myositis diseases and help develop new, more effective treatments. One way they are doing this is by partnering with patients. From the very beginning, the Center’s clinicians and researchers invited all of their patients to be part of a large, long-term registry, a research database that included blood samples, DNA, and clinical information acquired during clinic visits. This database now includes information from about 2,500 patients.

“That clinical care–research interface is an important way to think about rare diseases,” Dr. Christopher-Stine says. “You need lots of data points in order to see patterns that you just can’t see in caring for one, two, or three people. Especially when you follow people over time, you can look back and compare that data with their blood samples and DNA and find things that you weren’t even sure were true when you saw the patient in real time.”

Recently, this database facilitated one of the most significant discoveries in myositis. For many years, Dr. Christopher-Stine and her colleagues heard from patients that their muscle weakness and fatigue came on after they started taking statin medications, a widely used drug to lower cholesterol and prevent heart disease. The weakness didn’t go away after they stopped taking the drug, and the cardiologists who prescribed it said that meant their symptoms were unrelated to the statin. Scientists at the Center proved that wasn’t true.

“It’s a great example of how patients drive what we do,” Dr. Christopher-Stine says. “After a while, you hear that story enough times and you say that’s really curious.”

She remembers vividly the evening a young research assistant came up to her after clinic and said, “This is an unusual antibody here. What do you think this is?”

The research assistant, Grace Hong, had been working with Dr. Christopher-Stine in concert with the Myositis Center team, including Dr. Livia Casciola-Rosen PhD, an expert on autoantibodies, to understand how autoimmune diseases work in the body.

What Grace had first noticed turned out to be a new myositis-specific autoantibody that had not been described before. After comparing a number of the patient samples from the Center’s database, it became clear that many of the patients who said their weakness started after taking statins were the same people who had those antibodies. Now we know that that antibody—Anti-HMG-CoA reductase (HMGCR)—is associated with a form of myositis called necrotizing autoimmune myopathy (NAM), which causes muscle cells to die.

This was a valuable discovery, but there is still much more to learn about myositis diseases and how we can help improve patients’ lives. Among the first tasks that must be achieved, says Dr. Christopher-Stine, is to get more drugs approved by the Food and Drug Administration (FDA) for treatment of myositis diseases.

While a number of medications are very effective in treating myositis, most of these are used “off-label,” meaning outside the official approved indications. Insurance companies often challenge these uses, causing delays in treatment as patients and providers fight for access.

Along with this goal is finding an effective way to treat inclusion body myositis (IBM). Currently, the only treatment available for this chronically debilitating form of muscle disease is exercise, which only serves to slow the progress of disability. Those who live with IBM are understandably desperate for any therapy that can improve their condition.

Besides new therapies, a consistent treatment protocol is needed that has been scientifically verified, rather than based on “what we’ve always done.” Currently, there is no such standardized formula for deciding which drugs to try first when a patient is diagnosed with dermatomyositis (DM), for example. Providers differ widely on how they use corticosteroids and other treatments, how they evaluate effectiveness, and when they add to or change the regimen. Patients often suffer prolonged or worsening symptoms because of ineffective protocols.

Dr. Christopher-Stine also suggests that even the way providers refer to these diseases is confusing and not based on the science. Specifically, she challenges the term polymyositis (PM), calling it a diagnosis of exclusion. When myositis diseases were first classified more than 40 years ago, someone with the typical pattern of myositis muscle weakness but without the rash associated with DM was identified as PM. Modern science has refined the picture of all forms of myositis, yet old terminology remains, causing confusion and possibly hindering further progress in understanding these diseases.

“We need to put people into the right category so that they’re studied properly,” Dr. Christopher-Stine says. “The way the disease works is very different between NAM, DM and PM. If you put too many people in one box who have entirely different disease states, you’re going to bias the results.”

If a drug company has a new drug, for example, they need to test it on a fairly similar group of patients so they can tell if it is effective. If the group they study includes both DM and PM patients, the results may be mixed rather than showing a strong positive effect. This may mean that a treatment that worked well for, say, DM patients shows statistically that it isn’t effective because it didn’t work well for PM patients. 

None of these challenges are insurmountable, however. The myositis research community is one of the most collegial communities in academic medicine. Myositis experts from the Johns Hopkins Myositis Center are working together with colleagues from around the world to solve these and other questions with the goal of improving the lives of those who live with myositis diseases.

“I dream that one day we can take care of people with targeted therapies that are personalized just for them,” says Dr. Christopher-Stine. “When I retire, I want to leave the field knowing that I and others made a significant contribution to this personalized approach for all myositis patients.”

With the collaboration of the myositis research community along with data from patient registries like the one established at the Center, Dr. Christopher-Stine is optimistic they will achieve this goal.

The Johns Hopkins Myositis Center is one of the most highly respected centers in the country. It brings together a wide range of clinical expertise in rheumatology, neurology, pulmonology, and physical medicine rehabilitation along with basic science research. Patients with a suspected or confirmed diagnosis of myositis from across the country can be evaluated at the Center, with follow-up consultations with local practitioners.

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Patient stories

Service Dog Brings More than Self-sufficiency

Bitsy Anderson was literally teetering on the edge of a cliff when she started taking seriously the muscle weakness she had been feeling off and on for a while. She and her husband Terry were hiking in Utah, and she was stuck. She had lost her sense of balance, kept tripping over nothing, and needed help to get down off that mountain. In hindsight, symptoms like these had been creeping up on her for years.

That was twelve years ago, and Terry had just retired. The couple was planning to spend their golden years traveling. They had just bought a pair of matching bicycles. They had taken some paddling lessons and were about to buy a couple kayaks. But when they returned home after the hiking trip Bitsy was diagnosed with a rare muscle disease called inclusion body myositis (IBM), a disease that causes disability through increasing muscle loss in one’s legs and arms, difficulty grasping objects, and often trouble swallowing.

“IBM has pretty much changed our whole life,” says Bitsy, who leads the Maryland/Delaware/DC/Northern Virginia support group for The Myositis Association.

“It’s had an impact on both of us,” Terry says. “But it’s an impact we share, and we’re doing the best we can.”

Perhaps the hardest change for Bitsy was adapting to the loss of her independence. As her disease progresses, she’s had to give up driving, has trouble putting on her shoes and managing the housework, and she is always dropping things. She does not like having to constantly call on Terry just to get through the day. Fortunately, a fair-haired helper recently arrived.

Maya, an English Labrador retriever and Bitsy’s service dog, officially arrived in January of this year. Now it is her job to bring in the newspaper, help with the laundry, open the freezer, find things Bitsy has dropped, and dozens of other little tasks that make Bitsy more self-sufficient. She even helps Bitsy on with her jacket and off with her shoes.

“I don’t think she could have come to our home at a better time,” Bitsy says. “Maya makes it possible for me to maintain my independence and also adds an element of fun. If she hears the click of the brakes on my walker, in two seconds she is right by my side to see what she can do. She is very devoted.”

Aside from the help she provides for Bitsy, Maya has brought an added dimension of joy to the Anderson’s lives. Both Terry and Bitsy love her like a family member.

We love to play with her,” Terry says. “We take her out to the backyard. I will throw the tennis ball, and she always brings it back to Bitsy. It is a good opportunity for us to be outside and enjoy each other. It’s good exercise for her, too.”

“She loves to have her belly rubbed, but I can’t get down on the floor anymore,” Bitsy says. “So, when Terry comes along, she jumps all over him, so he’ll scratch her belly.”

Maya came to Bitsy from Fidos for Freedom, a local volunteer organization that trains service, hearing, and therapy dogs. Fidos is a member of Assistance Dogs International, Inc., a worldwide coalition of nonprofit programs that creates quality standards for assistance dog acquisition, training, and partnership. Fidos also educates the public about individuals with disabilities and about the benefits of assistance dogs and therapy dogs.

As Bitsy and Terry learned, getting a service animal is a big commitment. And the process is far from simple…or easy! Years of training are involved, and not just for the dog. Maya spent a year with a puppy trainer, becoming socialized and learning basic commands before coming to Fidos to find a partner.

Bitsy was accepted into the program in September 2017 and spent more than two years participating in twice-weekly training sessions before she was matched with Maya and allowed to bring her home. Even now Maya’s stay is probationary for a year before her adoption is final, allowing Bitsy and Terry to be certain the arrangement is working out. And even when their probation is over, Bitsy can still consult with a trainer to help her figure out how to help Maya adapt to her needs.

“The program is intense,” Bitsy says. “There was a time I almost quit. There were days when I came home from training saying, I am not doing this anymore. But you must have perseverance. You have to hang in there.”

Terry agrees. “You have to go into the program with your eyes wide open, knowing that there is a commitment there.”

Both Terry and Bitsy are thrilled to have Maya in their lives now and cannot even imagine giving her back at the end of the year. And once the limitations of the coronavirus pandemic have passed, they look forward to getting out into the world again. While they may not be traveling internationally again, they do hope to flee to Florida for winters again and to explore some or our beautiful National Parks. And they will have Maya right beside them.

“This has been a great experience,” Bitsy says. “You just have to go with the idea that I’m going to do this no matter what it takes, that it’s going to be a benefit. It really is well worthwhile.”

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Patient stories

When You Need a Little Help from Your Friends

Madison Davis had been planning her career in the military since she was seven or eight years old. Living near the Naval Academy in Annapolis, Maryland, she joined the Naval Sea Cadets and was intent on moving up through the ranks. She strategized her high school resume to help her get accepted into the Coast Guard Academy, as a soccer player, if possible.

“She was obsessed with the Naval Academy,” says her mom, Christine Davis. “She always wanted to visit there and wear the uniform and work on advancing her ranks. She was a big creator, too, a lego girl, up until she was 13 or so. She always talked about wanting to work on Lear Jets, fixing planes. She always knew what she wanted to do, and she was excited.”

In May 2017, however, gazing down at her daughter lying limply in a hospital bed as doctors gave their diagnosis, a strange, rare disease that seven weeks earlier had weakened Maddie’s muscles, given her a rash over large portions of her body, and paralyzed her into the worst pain she’d ever experienced, Chris cried. Not only was her daughter suffering, but she knew all Maddie’s dreams had just been evaporated by an autoimmune disease they couldn’t even pronounce: juvenile dermatomyositis (JDM).

“I was so numb to everything in the hospital. It really didn’t hit me,” Maddie says. “I wasn’t actually facing all these things I had just lost. I was just on to the next thing. Like, okay, so what do I do now? What schools do I apply to now? I didn’t really give myself a lot of time to deal with it in the moment. And believe it or not, I felt like I was handling everything very well.”

The next few years were difficult for Maddie. She is fortunate that she lives near one of the world’s leading Myositis Centers at Johns Hopkins University, so the medical treatment she received, including prednisone, methotrexate, and intravenous venous immune globulin (IVIG) therapy, was excellent and effective. Her mental health, however, was a different story.

Maddie missed out on much of the excitement of her senior year of high school because she was too weak to participate in many of the activities. Not only was her athletic strength gone, but the prednisone, a steroid medication, made her gain weight and gave her what she calls “’roid rage,” making her emotionally erratic. She lost friendships, because she missed a lot of school and couldn’t go out on weekends, and her friends just didn’t understand why she was behaving so strangely.

“She suffered a lot in many different ways,” Chris says. “We were just trying to survive it every day and trying to seek answers to make it better.”

Maddie and Chris did find answers, mostly from others who live with rare diseases and know what Maddie was going through. On her first outing after spending three weeks in the hospital followed by two weeks in rehab to relearn how to walk, Maddie and Chris attended a local support group meeting of The Myositis Association (TMA). The speakers at that meeting happened to be three members of the CSI Pharmacy team, talking about immune globulin (IG) therapy and home infusion.

During the presentation, a representative from CSI Pharmacy shared her own journey with myasthenia gravis—another rare autoimmune disease—and IG therapy. In her talk, she described how her disease had ended her career as a Navy nurse, and how hard that was to deal with.

Maddie remembers glancing at her mother and whispering, incredulous, “Did she just say she was in the Navy?”

After the talk, Maddie and Chris approached the speaker and shared Maddie’s similar regrets about losing her own dream career in the military. After that, The two not only became good friends, but she also became Maddie’s home infusion nurse, allowing Maddie to receive her IVIG treatments at home rather than in the hospital infusion center. Later, when Maddie went off to York College of Pennsylvania, her new friend helped her learn how to give her own IG treatments subcutaneously (SCIG, under the skin, rather than in the vein).

The Davises also found out about Cure JM Foundation, a group more focused on children with myositis. They attended the Cure JM annual patient conference, and there Maddie befriended a group of other teens who also lived with myositis. It didn’t take long for Maddie to become a leader in this organization, one of the “older kids” who, the following year, helped lead the panels she had learned so much from the previous year.

Maddie discovered a passion for helping others through the rough spots of living with JDM. She had a Facebook page and website, Maddie Strong, where she talked about her challenges with her disease. Once she even posted a video demonstrating how she gives herself SCIG treatments.

Maddie may have powered through at the beginning of her disease, but eventually the walls started crumbling. During the summer before college, she started looking around and realizing how much had been taken away from her.

“That’s when I started dealing with depression,” she says. “I was thrown so much off course. It was really, really hard. I know everything happens for a reason and I’m really I glad to be where I am. I’m glad I’m at York. I’m actually really happy I’m not playing soccer. But it took a lot out of me.”

It’s the friends she met along the way, the other kids who share this disease and understand the journey, who have helped her get through that dark time.

“They have always been the first families that we’ve reached out to,” Chris says. “If she was feeling really low or like life isn’t worth living anymore or the medicine is just too much, she always had people to turn to who could walk her through it, because they’ve already been there.”

Now as she prepares to start her junior year of college, Maddie is strong. She’s an education major, making the dean’s list, works with K through eighth graders at the York City schools, and is an education technology entrepreneur with a fun teaching app for kids she’s hoping to market. Best of all, her disease is in remission.

Still, kids who are struggling with coming to terms with the life-altering experience of a disabling rare disease diagnosis like JDM are never far from this young advocate’s mind.

“The one thing that I wish I could tell parents is, for kids who are going through this, the mental health aspects are a really big deal,” Maddie says. “I would say focus your attention more on the fact that your kid is going through a lot of mental stuff right now. They are confused and they don’t understand and its really scary. Just talk about what’s going on.”

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Patient communities Patient stories

Meeting Patients Where They Are

Like most neurologists, Dr. Charlene Hafer-Macko treats patients with a variety of neuromuscular conditions. Her focus, though, is myasthenia gravis (MG). It’s MG patients who provide her with the intellectual challenge she loves.

“I really like this population,” she says. “There are so many things you can do to help them stay in control of their disease. And this is a group that really uses the information they have to help themselves. Helping people through the journey is the part I find the most fun.”

As an associate professor of neurology at the University of Maryland School of Medicine, Dr. Hafer-Macko serves as director of the university’s multidisciplinary Myasthenia Gravis Center. This clinic pulls together all the services an MG patient needs in one coordinated package—including an infusion suite that provides intravenous immune globulin (IVIG), plasmapheresis service, and thoracic surgeons that focus care for thymectomy for myasthenia gravis.

“Our team is very well versed in myasthenia,” Hafer-Macko says. “So not only are they providing care, but they’re also monitoring for side effects and providing education and support at the same time.”

For Dr. Hafer-Macko, it’s the education and support part of working with MG patients that she finds most satisfying. Several years into her career, she realized that she wasn’t feeling fully fulfilled by her interactions with patients. She would assess their weakness and check their blood work and tweak their medications, but these exchanges with mostly stable patients felt flat. She needed something more.

She discovered that something more in the stories her patients told about their daily experiences. They reported, for example, that even when their double vision was controlled or they were back to walking normally, they still had trouble reading or watching TV, and they felt exhausted after a trip to the grocery store or just walking across the room.

“Even when many patients are well controlled, fatigue is an element that just stays with them,” Hafer-Macko says. “Fatigue is such a tricky thing. It’s something that is not often addressed effectively. So really understanding what’s driving that fatigue was something that I got very interested in.”

She teamed up with occupational, physical, and respiratory therapists to develop a better understanding of fatigue and the needs of MG patients. Together with this team, Hafer-Macko developed a toolbox of techniques for helping patients avoid or overcome fatigue and other challenges.

Listening to her patients’ stories has also helped Hafer-Macko become a better doctor.

“I learned how to ask questions differently, questions that gave me better data,” she says. “And then once I’d ask them differently, I could coach individuals on how to give me better information.”

When she would ask a question like, “Are you better,” for example, she found the patient’s response—“Yes, I’m better now”—didn’t provide much in terms of measurable outcomes. If, however, she asks about how long the patient can read before their eye symptoms make them put the book down, she has a benchmark that she can compare to a previous exam. It’s data that shows a meaningful response to treatment.

Stories of her patients’ fatigue also inspired Hafer-Macko’s research. She is part of a group at the Baltimore Veterans Association Medical Center that is exploring exercise, nutrition, and fitness in older adults. One of the things they have learned is that, because of their weakness, those with MG must work extra hard to accomplish even minor tasks like walking to the bathroom. This leaves far less energy for all other activities.

“It’s like every time they walk to the bathroom, they’re running a marathon,” Hafer-Macko says. “They have very little reserve. They’re just working very hard because of that weakness.”

Dr. Hafer-Macko has been recognized by the Myasthenia Gravis Foundation of America (MGFA) for her outstanding work with the MG community. She has served on the board of directors for the organization and currently serves on two of their committees.

Ironically, Hafer-Macko’s greatest inspiration is not a patient at all. It’s her mother. At 82, Charlotte Hafer still teaches dance—these days remotely by zoom. In 41 years of teaching elementary school during the day and dance at night, she never took a sick day. She continues to work as a math and reading specialist by day and teaches dance at night. As a devoted theater fan, her mother saved up her sick leave compensation so she could go to shows in New York City and in the Pennsylvania/Maryland/DC area to see shows. This year, Charlotte engaged the brave new world of Facebook to win a contest in which she was named Broadway’s Biggest Fan.

“She’s actually my inspiration,” Hafer-Macko says. “To deal with my mom and her medical hang ups, I’ve learned so much about taking care of people. You’ve got to meet folks where they’re coming from. They’ve got nuances, and that makes such a difference in working with a patient to find a care plan that will work well for them.” 


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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.

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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.”