Health Information

Don’t Flush!!

Have you checked your medicine cabinet recently? I was cleaning things out the other day and was stunned to see the shelves behind my bathroom mirror were crowded with bottles I hadn’t touched in years. There were antibiotics I didn’t finish because I had an allergic reaction, an unfinished nausea prescription from the time I had the flu, and pain meds from surgery a while ago. I even had a bottle of ibuprofen from before my mother passed away five years ago.

“Having a shelf full of old or expired medications in the home can be a significant safety risk to family, friends, or even pets,” says CSI Pharmacy Vice President of Clinical Services, Barry Buls, PharmD. 

People often forget why those medications were originally prescribed, or they simply grab the wrong bottle, which could be dangerous. Medications prescribed for one person may make another member of the family severely ill. The powerful pain meds prescribed for cancer patients, for example, could send another person to the hospital or even cause their death. That’s why Barry recommends that each member of the household store their medications in a separate space.

CSI Pharmacy’s Chief Pharmacy Officer, Jack Lemley, PharmD, reminds us that hanging onto those old narcotics that were prescribed after surgery can cause curious kids to get hooked on opioids. And while most medications lose effectiveness over time, some, like that tetracycline in the medicine cabinet, may become harmful if taken past the expiration date. 

“It’s just safer to keep only the medications that are on your current medication list in your home and in a safe place and out of the reach of children,” Barry says.

Getting rid of unwanted medications, however, is not as straightforward as dropping them into the trash. At one time, people were advised to flush old or unused medications down the sink or toilet. Now we know it can be harmful to the environment and even human health when pharmaceuticals end up in our waterways. They can even show up in our drinking water, because most wastewater treatment facilities are not equipped to remove these chemical substances.

So how can we safely get rid of unused or expired meds?

Take Back Programs

The best way to dispose of unwanted prescription or over-the-counter drugs is through a “Take Back” event or at a special drop-off site. Every year, the US Drug Enforcement Agency (DEA) sponsors National Prescription Drug Take Back events. At these times, public safety agencies across the country are available to collect tablets, capsules, patches, and other forms of prescription and over-the-counter drugs, which are then disposed of safely. Medications are accepted anonymously, and the program is free.

Throughout the rest of the year, unneeded medications can be dropped off at special disposal locations, including pharmacies, hospitals, and businesses. Many local police or fire departments also provide year-round drop boxes. Your pharmacy may even offer an on-site drop-off box, mail-back program, or other ways to safely dispose of unused meds.

Home Disposal

If you are unable to get to a Take Back event or drop-off location, many drugs can safely be disposed of at home. These include prescription and over-the-counter (OTC) pills, liquids, drops, patches, and creams. The US Food and Drug Administration (FDA) provides guidelines for how to so this:

  1. Remove the drugs from their original containers and mix them with something undesirable, like used coffee grounds, dirt, or cat litter. This makes the medicine less appealing to children and pets and unrecognizable to someone who might intentionally go through the trash looking for drugs.
  2. Put the mixture in something you can close (a re-sealable zipper storage bag, empty can, or other container) to prevent the drug from leaking or spilling out.
  3. Throw the container in the garbage.
  4. Before throwing away the packaging, scratch out all your personal information on the empty package to protect your identity and privacy.

Special Considerations

Some products, however, require special handling.

Inhaled medications, for example, used for asthma and other breathing conditions, come in aerosol containers that can be dangerous if punctured or thrown into a fire or incinerator. To properly dispose of these containers, whether they are left over, expired, or used up, the FDA recommends contacting your trash facility. These metal canisters should not be recycled.

Similarly, fentanyl patches, used for long-term pain management, require special handling. Even after one of these adhesive skin patches is used, a lot of this high-dose medication can remain. This can be especially dangerous if someone for whom this drug is not prescribed, especially children, touches the patch for even a short time. Fentanyl patches are one exception to the advice against flushing meds. In fact, the FDA specifically recommends promptly disposing of used fentanyl patches by folding them in half with the sticky sides together, then flushing them down a toilet.

The FDA highlights other exceptions to the no-flush rule for medications that have similar safety concerns. Their Flush List includes drugs such as those that are highly sought after for misuse and/or abuse and those that can cause death from even one dose if used inappropriately. They recommend checking the medication label or their online Flush List to see if this form of disposal is recommended.

The FDA acknowledges the environmental concerns about disposing of these and all medications through the sanitation system. The agency recommends flushing for these specific medications only as a last resort. And their 2017 study of the risks posed by such disposal shows there is negligible toxicity to the environment and human health.

Remember, do not flush your medicine unless it is on the Flush List.

Health Information

Feed Your Health Well

During the month of March, we recognize National Nutrition Month. It’s a time for everyone to become more informed about the value of developing healthy eating habits.

Because so many members of our patient communities live with autoimmune diseases, we are especially concerned about how certain foods promote inflammation in the body. Foods that are highly processed, such as high-salt ready-to-eat meals, sugary cereals, high-fat processed meats, and sodas made with high fructose corn syrup are among the products nutrition counselors caution against.

If you’ve thought about how the foods you eat might be affecting your health, this might be the month when you experiment with trying something new. You can use the following ideas as guidelines for making wise food choices.

  • Avoid processed and fast foods, including those with high fructose corn syrup, artificial ingredients, preservatives, and pesticides. Instead, opt for fresh food. Eat a variety of brightly colored fruits and vegetables and foods that don’t contain a long list of unpronounceable ingredients.
  • Reduce the number of foods made with refined white flour and sugar, especially bread, pasta, and most packaged snack foods. Instead, choose foods made with whole grains, such as brown rice, bulgur, and quinoa.
  • Try to eat less saturated fat by cutting back on animal products and foods made with palm kernel oil. This will help your heart as well as your immune system.
  • Use extra-virgin olive oil and expeller-pressed canola, sunflower, and safflower oils.
  • Experiment with adding avocados and nuts to your diet, especially walnuts, cashews, almonds, and nut butters made from these nuts.
  • Eat more vegetable protein, especially from beans and soy, and choose fish, cheese, and yogurt more often than you choose meat.
  • Avoid drinking soda and choose tea instead of coffee.
  • If you drink alcohol, red wine may be preferable.
  • And here’s the good news: dark chocolate (in moderation), can be a healthy choice! (Choose the kind with a minimum of 70% cocoa.)

If you take corticosteroids (prednisone, for example), especially in high doses and for long periods of time, you may develop side effects that have implications for what you eat.

  • Prednisone increases appetite. To avoid weight gain, try to avoid high-calorie foods. Eating frequent, small meals can help maintain steady blood sugar levels so you don’t feel hungry. Getting plenty of exercise will also help.
  • To reduce the risks of high blood pressure and fluid retention, try to keep your salt intake at less than 1,500 mg per day. You can do this by eating fresh rather than processed or canned foods and hiding the saltshaker.
  • If you take medication for high blood pressure, you may need to eat foods high in potassium, such as bananas, apricots, cantaloupe, baked potatoes, and tomatoes.
  • Prednisone can irritate the stomach, so it is important to take it with food, not on an empty stomach.
  • Diabetes is also a risk when taking steroids. To keep blood sugar levels within the normal range, avoid foods high in simple sugars, such as fruit juice and those gooey pastries, and keep carbohydrate intake to between 45 and 60mg per day.

It may not be possible to get all the nutrients you need to be healthy from your diet. That’s where eating specific foods or taking supplements may be useful. The following suggestions are recommended for those who have autoimmune diseases.

  • Bone health is a concern for those who take prednisone. That’s why it’s important to eat foods rich in calcium, such as milk, yogurt, cheese, leafy green vegetables (kale, bok choy), almonds, and broccoli. Calcium supplements may also be needed to minimize bone loss and osteoporosis.
  • Vitamin D is a hormone produced in the skin in response to sunlight. It is also important for bone health as well as controlling inflammation. Because most people do not (and probably should not) spend enough time in the sun, most people need to take Vitamin D as a supplement.
  • Folic acid (also called folate) is a B vitamin that is found in leafy green vegetables, such as spinach, kale, broccoli, and other foods. Because the drug methotrexate interferes with the way folic acid is used in the body, if you take this anti-inflammatory medication, you may need more folic acid than you can get from food. You may want to ask your doctor about folic acid supplements.
  • Omega-3 fatty acids, which are anti-inflammatory, and omega-6 fatty acids, which cause more inflammation, should be in balance in the body to keep us healthy. Most Americans, however, eat much more omega-6 (found in vegetable oils, safflower oils, meat, poultry, and eggs), which causes a more pro-inflammatory state. Eating more foods containing omega-3 fatty acids, such as salmon, mackerel, sardines, leafy green vegetables, flaxseed, canola oil, walnuts, and enriched eggs, can help bring this back into balance. Omega-3 supplements are also available.

Of course you should always avoid foods that you are allergic to. And as always, it’s important to discuss diet with your healthcare providers to be sure they know that you are concerned about this important lifestyle habit and can guide you in making the best choices.

Health Information IG Therapy

When Your IG Therapy Makes You Feel Worse

As with most medications, immune globulin (IG) therapy can cause side effects. If your IG infusion leaves you with these uncomfortable symptoms, the good news is most of the time there are things you, your nurse, or the prescriber can do to make side effects less likely and relieve them if they happen.

Infusion reactions are most common if you use intravenous immune globulin (IVIG) rather than the subcutaneous form (SCIG). IG infusion nurses and pharmacists are usually very familiar with the types of reactions that can occur during or after your infusion, and they know how to respond to them. Rest assured that your nurse will be with you throughout your infusion to monitor for any reactions and respond to them.

Premedication can be one way to avoid reactions from occuring. Along with IG infusions, prescribers often prescribe over-the-counter pain medications, such as acetaminophen (Tylenol) or ibuprofen (Advil), and an antihistamine such as diphenhydramine (Benadryl).

Drinking lots of fluids (several liters a day) before, during, and after the infusion will also help. Be sure to hydrate with water not soda, coffee, milk, or other beverages.

Common Symptoms

Headache is one of the most common side effects with IVIG therapy, but usually this is mild. It’s why the nurse will start out running the infusion slowly and increase the rate gradually over the first hour or so. If a headache develops, the nurse will likely run the infusion more slowly to see if this will relieve the pain. Drinking more fluids may also help. If the infusion needs to run so slowly that it will take all day to infuse, it may need to be divided into smaller doses and given over several days.

Other milder symptoms may also occur—with or without a headache—including chills, fever, flushing, dizziness, muscle aches, joint pain, fatigue, nausea and/or vomiting, and rash. If these symptoms are significant, your prescriber may decide to try a different brand of IG. Each brand is slightly different in composition, and some people do better with one brand over another. Your nurse will consult with the pharmacist and/or your prescriber to decide how to best manage your symptoms.

For subcutaneous infusions (SCIG), pain, redness, and swelling at the needle site may also occur. Be sure to let your nurse know if you notice this and if it gets worse during the infusion. One possible solution may be to change or reposition the infusion needle.

If you use SCIG, you may notice lumps under your skin at the infusion site. This is expected and happens because the fluid you are infusing takes some time to be absorbed into your body. If these areas become uncomfortable, you can apply a warm cloth or compress to the area. If they last for more than a day or are painful, call your nurse or pharmacist.

More Serious Adverse Reactions

While headache is a common reaction with IG infusions, it usually goes away during or soon after the infusion, especially if treated with pain medication and hydration.

If your headache continues for days, is especially severe, or you have a stiff neck, drowsiness, high fever, sensitivity to light, eye pain, or severe nausea or vomiting, this is a cause for alarm. These may be signs of a rare reaction called aseptic meningitis (swelling of the brain not caused by infection). You should call your prescriber and the pharmacy and get medical help right away. 

Another rare but serious situation is an allergic reaction (anaphylaxis). This usually happens during the infusion while the nurse is with you. If you get your infusions at home, your shipment from the pharmacy will also include medications the nurse can use to treat this reaction. 

You may notice symptoms of an allergic reaction after the infusion is completed and the nurse has left. Such symptoms may include rash, itching or swelling (especially of the face, tongue, or throat), severe dizziness, or trouble breathing. If these occur, call 911 and get medical attention as fast as possible.

IG may also raise your blood pressure. Your nurse will check this frequently during your infusion. If you have had high blood pressure in the past or are currently being treated for it, please make sure that the prescriber, nurse, and pharmacist are aware. If you check your own pressure, be sure to notify the prescriber if it increases in the days following your infusion.

Other Possible Concerns

IG is made from human blood plasma, so it can rarely cause blood clots. This is more likely in older adults or those who previously have had blood clots, heart problems, or blood circulation problems. Blood clots are also more likely if you have been on bedrest for a long time, are using birth control pills or hormone replacement therapy, or if you have a central intravenous (IV) catheter in place.

Call your prescriber and pharmacist immediately if you have chest pain, trouble breathing, chest tightness, or rapid heartbeats. Other signs of a blood clot can be numbness or weakness, swelling and warmth, or discoloration in an arm or leg.

You should also let your prescriber and pharmacist know right away if you notice that your gums or nose start bleeding, you bruise more easily, it takes a long time to stop a cut from bleeding, you have a fainting spell, you feel like your heart is racing or skipping beats, or you feel unusually tired. These are signs of other cardiovascular problems that may need to be treated.

Sometimes IG can harm your kidneys, especially if you have kidney disease or use certain medicines. Signs of kidney problems include swelling, especially in the legs and hands, rapid weight gain, and little or no urination. Call your prescriber and pharmacy right away if you notice any of these symptoms.

Less of a Concern

Because IG is made with donated human plasma, some people worry about getting a disease from it. The risk of getting an infection from your treatment, however, is almost nonexistent. Modern methods used to purify IG destroy bacteria, hepatitis viruses, and other infectious germs. No one can predict every new infection that may develop, though. This is one of the reasons the pharmacy keeps careful records of the specific IG product you receive.

It’s also important that you keep track of when you get your infusions, the brand, product number, lot number, and any reactions you have around the time of the infusion. This will help you determine which products worked best and can help the pharmacist figure out if you are having side effects related to a specific brand.

Please Ask Questions

Never be afraid to ask questions about any symptom or reaction you have during or after your treatment. Your infusion nurse, pharmacist, and prescriber can all help you understand any reactions you may experience and how to treat them.

Please remember: Every patient is unique in the way they respond to any treatment. Do not hesitate to let your nurse or pharmacist know if you start to notice uncomfortable side effects. Lastly, if you ever experience unusual symptoms that cause you concern, be sure to contact your prescriber immediately.   

Health Information

Wellness is Within Your Control

Lisa was in her mid-twenties when she suddenly came down with asthma. She had never had allergies or wheezing as a child—the time when this sort of disease usually strikes people. But now she was desperately sick, struggling for breath on a daily basis, often needing to go to the emergency room for intravenous medication that would open her lungs and allow her to breathe again.

Her doctors told her this condition was chronic, that she would have to take medication for the rest of her life, and she’d have to be careful about all the things that could trigger an asthma attack. There may even be things that she would never be able to do again. Dancing, for example.

Like many people who are diagnosed with a chronic medical condition, Lisa was devastated. She became depressed, gained weight, and developed other health problems, all of which left her feeling out of control and helpless. 

One day, however, she learned about a class at the children’s hospital for people newly diagnosed with asthma. She decided to go. Even though it was aimed at kids and their parents, that class changed her life.

She learned what was happening to her body when she was having an asthma attack and what she could do about it. She learned about changes she could make to her home to reduce things like dust and mold and feathers that triggered wheezing. Most importantly, she came away from that session feeling empowered. She wasn’t as helpless as she thought.

That class set Lisa on a path toward wellness. She studied everything she could find about self-care and holistic options for treating chronic conditions. She changed her diet, started exercising more, saw a therapist, started meditating, and spent time in nature. Lisa was determined to prove those doctors wrong.

It took several years and a combination of mainstream medicine and wellness practices, but it happened. Lisa gradually decreased the amount of medication she was taking, and one day she realized that it had been years since she’d had an asthma attack. She could even forget about that inhaler that she always carried with her like a lucky rabbit’s foot “just in case.”

While not everyone who lives with a chronic condition can completely leave it behind in this way, most health problems can be improved by actively taking charge of the things in your life you do have control over. Here are some important areas to address:

Take care of your body

This includes getting enough sleep, eating a healthy diet, and getting enough exercise. In fact, paying attention to these practices puts you way ahead of the curve for all healthy living habits. All three help you think more clearly, lower your stress, improve your mood, and feel less fatigued. They can also help you control your weight, which can reduce your risk from other health conditions, such as diabetes and heart disease. And they all have a positive impact on your immune system and can reduce inflammation.

And while you’re doing good things for your body, don’t forget the things you shouldn’t do that are toxic to your health. Smoking, for example. Quitting is the single most important thing you can do to get your health on sound footing. Taking it easy when it comes to using alcohol and caffeine is also a good idea.

Mind your mental, emotional, and spiritual health

You are more than your physical body. Paying attention to how you are feeling, your mental space, and your spiritual wellbeing can have important influences on your physical health. You may be surprised to see how effective it can be when you take steps to control excessive stress, toxic thoughts, and harmful emotions like anger and anxiety.

There are so many ways to do this: See a therapist or talk to a friend. Start a meditation, mindfulness, or yoga practice. Keep a journal. Pray. Cultivate gratitude and find joy in all that is around you. Take a walk in the woods. Be creative. Cuddle with your furry friends. Make time on a regular basis to do what makes you feel good and helps you connect with the Divine.

Don’t forget your peeps!

Research shows that a strong social support system is extremely important for staying physically and mentally healthy. Having positive interactions with the people in your life makes you more resilient to stress, decreases the impact of post-traumatic stress disorder (PTSD), reduces the impact of disease, and helps you live longer. Studies also show a positive impact on mental health, immune function, cardiac health, and more. And having the support of friends and family enhances your ability to go along with good health habits, such as eating a nutritious diet, exercising, and smoking cessation.

It’s not just being on the receiving end of support that has an impact. Helping others helps you, too. Lending a hand has been shown to lower stress, disability, and mortality in the giver as well as the receiver.

But beware: poor social support can have a negative impact on health. If interactions with the people in your network cause conflict, criticism, and excessive demands, that’s a cause for concern. You may want to distance yourself, choosing instead to interact with the positive people in your life.

Health Information

Sleep on It

Getting a good night’s sleep does more than just keep you alert during the day. Proper sleep is critical to good mental and physical health. Of course sleep helps you concentrate and think more clearly. But research shows that a good night’s sleep is also associated with other positive health outcomes.

Improved brain function is the most obvious benefit of getting enough sleep. In addition to helping you focus better and think more clearly, studies show that those who sleep well are more productive and perform better on mental tasks. Memory improves with adequate sleep, which helps you learn better, improves problem-solving, and allows you to form new insights better.

Healthy sleep patterns, especially during middle age, may also help you avoid dementia as you get older. Mental health issues, such as depression and even thoughts of suicide, are associated with poor sleep quality and sleep disorders, such as sleep apnea and insomnia.

The benefits of bedtime are not limited to the brain, though. Studies show that those who sleep well perform better on physical activities as well as mental ones. Athletic performance, for example, was shown to improve with more sleep. In contrast, functional limitations, such as walking, grip strength, and ability to perform independent activities, was worse for older women who slept less well.

Good quality sleep is essential for other factors associated with chronic illness, too. For example, not getting enough sleep is one of the strongest risk factors for obesity. Adequate sleep helps regulate hormones and other factors that affect appetite and blood sugar (an important factor for those with diabetes), which helps you maintain better weight control.

Getting enough sleep also improves immune function and decreases inflammation. So sleep may be an important part of your therapy if you have an autoimmune disease like myositis, CIDP, myasthenia, and others. Better sleep may also lower your risk for developing serious conditions like heart disease and stroke.

So how do you get a good night’s sleep? Good sleep hygiene is all about putting yourself in the best position to fall asleep easily and sleep well throughout the night. Here are some tips for making that happen:

  • Stick to a consistent sleep schedule. Go to bed at the same time every night, and wake up at the same time every morning, even on weekends. Make sure this schedule allows for 7-9 hours of shuteye.
  • Unwind with a relaxing bedtime routine. Give yourself 30-60 minutes before tucking yourself in to leave behind the busyness of your day. Take a warm bath, meditate, write in your journal, read…anything that helps you de-stress and feel drowsy. Anything, that is, except activities that involve a blue light-emitting screen.
  • Make your bed a device-free zone. Phones, computers, and e-reader devices emit a blue light that reduces melatonin levels in your body, making it more difficult to fall asleep. When you use them before bedtime, they can keep your brain more alert, which also makes it harder to fall asleep. Your phone’s dings, pings, and rings and the light that pops on when notifications come through are also disturbing to your sleep. Best to turn the phone off at night.
  • Exercise every day. Thirty minutes of exercise a day not only improves your health and physical functioning, but it can improve sleep quality as well. Being active outside, where you are exposed to natural light, can also help to regulate your sleep cycle. It’s best, however, to avoid vigorous exercise within an hour or two of bedtime.  
  • Limit caffeine late in the day. Caffeine’s effects can last up to seven hours after your last cup of coffee, making it more difficult to fall asleep.
  • Make your sleep space cool, comfy, dark, and quiet. Bedroom temperatures are better when they’re lower than the rest of the house (between 60°F and 67°F). A comfortable mattress, pillows, and bed linens also go a long way in helping you fall asleep and stay asleep. And keep the room dark and quiet (use earplugs if you must) to keep you from waking up.
  • Manage your stress before you go to bed. Try not to bring your worries to bed with you. Write down what’s bothering you or make a to-do list for tomorrow so these concerns don’t keep you up at night. Meditation or other relaxation techniques can also calm your mind.
  • Save your bed for sleep and sex only. It’s best to avoid working, talking on the phone, watching TV, or other activities when you’re in bed. That way your brain is conditioned to expect to fall asleep when you climb under the covers. Even reading can keep your brain on alert, so it’s better to read in another comfy spot before slipping into bed.
  • Avoid tossing and turning. If you have trouble falling asleep, especially if you wake up in the middle of the night, don’t just lie there. If it takes more than 20 minutes to fall asleep, try getting up and doing something relaxing—drink a cup of herbal tea, read a book (on the couch, not in bed), meditate—until you feel tired.
  • Limit daytime naps. Napping during the day can make it harder to fall asleep and stay asleep during the night, especially for older people. If you must nap, don’t do it late in the day, and limit it to 30 minutes or less.
  • Prioritize sleep. While it’s tempting to work, study, socialize, or exercise late into the night, burning the midnight oil is not a good idea. It’s important to treat sleep as a priority in the same way you do eating three healthy meals every day.
  • Avoid these things: consuming alcohol in large quantities, eating a large meal late in the day, or smoking at any time. All of these activities cause sleep disruption.

Falling asleep and staying asleep can be a problem for some, especially when you’re taking certain medications, such as prednisone. If you’ve tried these tips but sleep continues to be a problem, discuss this with your doctor. He or she can help you figure out whether an underlying medical condition is causing your sleep issue and work with you to solve it.

Health Information Patient communities

COVID Vaccine and Our Patient Communities

Across the country we are starting to see the light at the end of the tunnel as the rollout of COVID-19 vaccines has begun. For many of our patients with primary immunodeficiency diseases or autoimmune conditions, however, getting vaccinated is not a straightforward decision. They have lots of questions and must weigh carefully a number of considerations.

Immunologist and rheumatologist Dr. Terry Harville has some concerns, too. “There are questions in my mind that haven’t been fully answered,” he says about the new vaccines that have recently been granted emergency use authorization by the Food and Drug Administration (FDA). “And I’m worried that there will be unintended consequences.”

The fact that we have only two months’ worth of safety data from the clinical trials testing these new vaccines from Pfizer/BioNTech and Moderna leaves many medical professionals worried about longer-term effects. Others are unsure about how the vaccines will react in patients with rare autoimmune and primary immunodeficiency diseases, because testing did not include people who live with these conditions.

Despite these concerns, Dr. Harville—along with most other healthcare practitioners—stresses that most people should still get the vaccine. Because when hundreds of thousands of people get COVID and one or two percent of them die, the statistics overwhelmingly support COVID-19 vaccination.

Dr. Harville, who is a professor of medicine at the University of Arkansas for Medical Sciences and an expert in primary immunodeficiency disorders, offers some guidelines for those who are trying to make a decision about the vaccine and might be at greater risk for complications from it.

If you have a primary immunodeficiency (PI) disease, you probably already know that you need to be careful about taking immunizations, especially if they are made with live virus. The COVID-19 vaccines that are currently being administered, however, are not made with live virus. They are created from a man-made part of the virus’s genetic code called mRNA. This means they are safer for those who are unable to take live virus vaccines.

With a few exceptions, Dr. Harville says, “It is the general consensus that we don’t see any reason to not vaccinate those with primary immune deficiency diseases.”

One exception is interferonopathies, IDs associated with dysfunction in components of the immune system called interferons. Another exception is CTLA4 deficiency. These are conditions in which patients tend to develop autoimmune disorders. Dr. Harville recommends that individuals with these types of ID should probably not get the currently available COVID vaccines. While there is no data to support this at this time, there is concern among immunologists that vaccination in this group of patients may trigger an overly aggressive immune response that would be harmful. As more data becomes available, better recommendations will be forthcoming.

If you use immune globulin therapy, there appears to be no reason you shouldn’t get the vaccine. In fact, even if you get the disease, you may actually be better off than others.

“What’s interesting is,” Dr. Harville says, “if you look at SARS-CoV-2 infection in patients with antibody deficiencies—whether that’s X-linked agammaglobulinemia (XLA) or common variable immunodeficiency (CVID)—when these patients are receiving appropriate immunoglobulin (IG) replacement therapy, they tend to have milder disease.”

Intravenous immunoglobulin (IVIG) has even been used, in some cases, to treat COVID-19 infection. While clinical trials to prove effectiveness are still ongoing, some physicians believe IVIG does decrease the risk of severe complications. IG manufactured from the plasma of people who have recovered from COVID-19 infection is also being tested as a treatment.

If you have had a severe reaction to immunizations in the past, this may be a reason to opt out of getting the COVID vaccine. This includes those who have a primary immunodeficiency disorder and developed the infection after receiving a vaccine. It also includes autoimmune disease patients who have experienced a severe flare of their symptoms after a vaccination.

In the 1976, there was a rise in cases of the autoimmune condition known as Guillain-Barré syndrome (GBS) that was thought to be triggered by the swine flu vaccine given that year. This was an inactivated virus vaccine, not the type of mRNA vaccine we now have with COVID. Nevertheless, if you’ve had GBS in the past, be sure to let your healthcare provider know this, as it may be a consideration for whether or not you should get the COVID vaccine.

During the COVID vaccine clinical trials, a very small number of participants who received the Pfizer vaccine developed a severe, life-threatening allergic reaction. Since then, others have also had what is called an anaphylactic response soon after getting vaccinated. If you have ever experienced shortness of breath, tightening in the throat, swollen lips or face, or other severe allergic symptoms, you should probably not get this vaccine in particular. (Other vaccines using whole virus are currently in development and will likely be safer.) Anyone who does get a COVID vaccine should be observed for at least 15 minutes and be sure the healthcare personnel administering it have emergency equipment needed to respond to anaphylaxis.

Regardless of your disease, everyone can reduce their own risk. Dr. Harville stresses that, even if you get vaccinated, it’s still important to wear a mask, wash your hands, and maintain physical distance from others.

“Ultimately whether you take or do not take the vaccine is an individual patient decision,” Dr. Harville says. “This should be made after weighing all the risks and benefits and based on a discussion with your physician.”

We highly recommend that you learn more about the risks and benefits of COVID-19 vaccination as it relates to your particular situation. The Centers for Disease Control and Prevention (CDC) offers these guidelines for COVID-19 vaccination for those with underlying medical conditions. In addition, a number of disease organizations offer educational programming about the impact of the virus and the vaccine on specific disease conditions.

Please also see disease-specific statements about COVID-19 vaccines from these patient organizations:

Guillain-Barré syndrome – GBS|CIDP Foundation

Immune Deficiency Foundation

Myasthenia Gravis Foundation of America

The Myositis Association

Myositis Support and Understanding

Platelet Disorder Support Association

Health Information IG Therapy Patient communities

Choices: IVIG versus SCIG

Many patients with autoimmune disorders and primary immune deficiency diseases depend on regular infusions of immune globulin (IG) to keep them healthy. For most of the nearly four decades since immune globulin therapies have been available, patients have had only one viable option for how this treatment was given. It was only available as an intravenous (IV) infusion.

Since 2006, however, when the first IG product was approved for subcutaneous (SC) administration, patients have had a choice about how they received their treatments. Both products are considered equivalent in terms of efficacy, but there are lots of other factors that may make one preferable over the other. Providers usually have their own sense about how IG should be administered, but we asked IG users for their thoughts on the pros and cons of each option.

Convenience is the biggest factor in which route patients prefer. Ironically, both IV and SC users think their choice is most convenient.

Rebecca, for example, has been getting IVIG for 12 years after being diagnosed with common variable immunodeficiency (CVID). She speaks for many when she says, “I like that I only sacrifice one day every three weeks for treatment.”

The convenience of once-a-month infusions with IVIG comes at the expense of independence, though. IVIG poses higher risks, because it goes directly into the vein rather than under the skin. So it must be given under a nurse’s supervision, whether that is in the hospital, an infusion center, or at home. This means it also has to take place on a schedule that may not always be convenient.

Those who use SCIG usually take their infusions once a week rather than once every three to four weeks or so. Still they prefer the control they have over when they infuse, because they do it themselves. As Brandina, who has myasthenia gravis, says, “I love that I can administer it myself. The treatment days are flexible, and I can take the medication with me, so I don’t have to plan my vacation around treatments.”

Infusing once a week is also inconvenient for some SCIG users, but for most this is a minor drawback. As Jen, who has specific antibody deficiency, says, “I absolutely love SCIG. There are so many more pros that I could list and only this one con.”

Getting infusions at home, whether it is IV or SC, is also a convenience. This has become especially important since the COVID-19 pandemic has made it less desirable to go to a healthcare clinic. Brynne, whose six-year-old daughter uses IVIG for juvenile dermatomyositis (JDM), was grateful when her overnight hospital infusions were changed to in-home infusions because of coronavirus restrictions.

Making the most of infusion time is something IVIG users have worked into their lives. Sitting in an infusion center or even hanging out at home with a nurse for six to eight hours or more can be a huge inconvenience, but it doesn’t have to be wasted time. Dana, who has dermatomyositis, likes IVIG, because it forces her to take time for herself and relax. And Robin, who has CVID, uses the time to crochet.

Mary, whose husband has myasthenia gravis (MG), prefers to get his IVIG at the hospital infusion center for other self-care reasons. “He loves the heated, vibrating recliner,” she says. “And they provide snacks and lunch.”

Adverse effects can be more of a problem with IVIG. In fact, this is often the reason patients switch to SCIG, which has far fewer reactions. Symptoms can range from fatigue, fever, flushing, chills, and ‘‘flu-like’’ symptoms to more life-threatening reactions like anaphylaxis (severe allergic reaction) and blood clots.

The most frequent side effect is headache, which can last several days and be more severe than a migraine. Some, like Lola, who has Sjögren’s syndrome, even get aseptic meningitis (inflammation of the membrane covering the brain) after infusions. This causes debilitating headaches, dizziness, and other symptoms.

Scar tissue and knots of fluid under the skin from subcutaneous infusions was a drawback for those using SCIG. These knots usually disappear within a few hours, though, and any redness or swelling at the injection site usually decreases over time.

Pain from being stuck with needles is not an insignificant side effect, regardless of whether it’s IV or SC. Whether it’s having to stick oneself multiple times or whether it’s having difficult-to-access veins, nobody likes to feel like a pincushion.

This can be especially challenging for children. Nancy’s nine-year-old daughter has JDM and receives IVIG at a pediatric infusion center. She says having ultrasound to find and insert the IV needle makes a world of difference for her daughter. Being spoiled by the nurses also takes some of the sting out of the whole ordeal.

Fluctuations in therapeutic effect is another reason many people switch to SCIG. An IG dose is mostly metabolized by the body over about 22 days, whether it’s given IV or SC. With IV infusion the dose reaches its peak immediately and dissipates over the next three to four weeks. This means that some patients will feel their symptoms returning as IG levels in the blood go down.

“As I got closer to my next treatment date, I would start to feel the effects of needing my next treatment,” says Karon, who has MG. “After I received it, I could tell I had just received a boost and had more energy.”

Giving IG under the skin makes the blood levels rise more slowly. And because SCIG is given more frequently—usually weekly—IG levels in the bloodstream fluctuate far less, so patients don’t feel that fatigue and other symptoms returning.

Whatever you decide about IG therapy, Lea, who has used IVIG for 22 years to treat CVID, offers this important advice: “You have to listen to your body and watch how it reacts to everything and try things until they work for you.”

For those who would like to learn more about IVIG or SCIG, please contact the CSI Pharmacy advocacy team at [email protected].

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Get Your Flu Shot Now

For some, getting a flu shot is just part of the fall routine every year. Immunization against the virus that causes influenza prevents millions of people from getting the disease, makes the disease less severe if you do get it, and can prevent death from severe disease and its complications. According to the Centers for Disease Control and Prevention (CDC), influenza causes an estimated 12,000 to 61,000 deaths every year in this country.

This year, because of the COVID-19 pandemic, physicians and public health officials say it’s even more important that everyone over the age of six months get a flu shot. This is especially important if you have an underlying health condition that makes you more at risk for developing complications if you get the flu. While the influenza vaccine will not prevent you from getting COVID-19 (researchers are still working on a vaccine for this different virus), it will help you avoid getting seriously sick with or—heaven forbid—dying from the flu. And the fewer people who get the flu, the more it saves healthcare resources that are still urgently needed for treating COVID-19 patients.

Those who are at high risk for flu complications are also at greater risk for getting COVID-19 and having serious outcomes. If you have an autoimmune condition or immune deficiency disease, such as myositis, myasthenia gravis, pemphigus and pemphigoid, chronic inflammatory demyelinating polyneuropathy (CIDP), and primary immunodeficiency (PI), this means you. You are at a much greater risk for getting sick with the flu and at greater risk for developing complications like pneumonia if you do. If you have heart disease, cancer, or diabetes, getting the flu can also make these conditions worse.

Getting the flu vaccine, however, is not a straightforward decision for some who have these conditions. For example, some people with a history of Guillain-Barré Syndrome (GBS) may have developed this form of muscle paralysis after receiving an influenza immunization. According to the GBS|CIDP Foundation International, the association between GBS and flu vaccines is inconclusive, but they suggest these individuals avoid the vaccine in the future. If you have had GBS, especially if it developed four to six weeks after getting a flu shot, you should talk with your doctor about the risks and benefits of getting the vaccine again.

Another consideration is the aerosol form of the flu vaccine. This is a live attenuated (weakened) influenza vaccine that is given through the nose. While the injected vaccines are made with inactivated virus, the nasal spray is made with live organisms that have been weakened but are still able to activate the body’s immune response against the disease.

The intranasal vaccine is not recommended for those younger than two, older than 50, or those who have a weakened immune system, including some patients who take immune suppressing medications. If you care for or live with someone who is immune compromised, you should also avoid the nasal spray. And if you have an underlying medical condition that puts you at risk for developing severe complications from the flu (such as chronic lung disease, heart disease, kidney disease, liver disorders, neurologic and neuromuscular disorders, blood disorders, and diabetes), it’s important to check with your physician before taking the nasal flu vaccine.

According to the Immune Deficiency Foundation, those with certain forms of immune deficiency (common variable immune deficiency [CVID], severe combined immune deficiency [SCID] or Bruton’s agammaglobulinemia) are unable to develop protective immunity following vaccination. This means their bodies don’t have the infrastructure to develop the immunity needed to keep them from getting sick, so vaccines will not do them any good. In fact, for individuals with these forms of PI, live vaccines—including the influenza nasal spray—may put them in danger of developing severe disease.

The only reason not to get a flu shot is if you have a severe, life-threatening allergy to the vaccine or any of its ingredients. This might include gelatin, certain antibiotics, or other ingredients. If you get hives when you eat eggs, studies have shown that most times you can still get the flu shot. If you have a more serious reaction to eggs or you are worried about this, you should of course talk with your doctor. Egg-free alternative vaccines are available.

The CDC has more information about seasonal influenza and how to prevent it.

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Clinical Trials for Rare Diseases

The idea of clinical trials is much in the news these days as medical researchers work hard to identify new treatments and vaccines for COVID-19. A clinical trial is a formal research study that, among other things, tests new therapies to see how well they work and if they are safe to use in humans. It’s an essential step in the Food and Drug Administration’s (FDA) process for approving new therapies.

For those who live with rare diseases, though, clinical drug trials have a more direct impact on their day-to-day lives. Many rare disease patients have few or no options when it comes to effective treatment of their symptoms. Even when medications with confirmed effectiveness are prescribed, they often are used without FDA approval (a practice known as off-label use), which sometimes makes it difficult to get insurance to cover the cost.

Getting new medications to market requires extensive testing in order to attain FDA approval. These studies need to have a sizable number of participants in order to show accurate results. That’s not a problem for common diseases like heart disease and cancer. But with so few people with diseases like myositis, myasthenia gravis, or pemphigus, it can be difficult to get enough qualified participants to show clear results.

That’s why many of the patient organizations that we work with encourage their members to consider taking part in a clinical trial if at all possible. This can be a big decision, one that you should discuss with family members and your doctor to be sure they are comfortable with your participation and the role they may need to play in the process. But it’s a great way feel like you are contributing to a cure.

Clinical trials are not limited to testing medications, but here we focus on clinical trials testing safety and effectiveness of new drugs. We’ve outlined answers to some common questions that can help you understand the general process of participation in such trials. Specific questions about a particular trial can be answered by the study coordinator of that trial.

How does a clinical trial work? Each clinical trial follows a strict set of rules outlining who can participate, what processes will be done, how participants are protected against risks, how long the trial is expected to last, and more. This protocol is designed to clearly answer specific research questions about the treatment. These protocols are outlined for all medications being tested in the US on the NIH website Here you can search for a trial based on disease, drug, location, or other criteria. Each trial also has contact information for additional information.

What is informed consent? All research participants have the right to know exactly what they are getting into. Informed consent is the process of providing you with essential information about the study before you decide to take part. Members of the research team will provide written and verbal explanation of the details of the study, including its purpose, how long it’s expected to last, tests or procedures that will be done as part of the research, and who to contact for further information. All known risks and potential benefits are also explained and included in the document. Before you decide to sign this document (which means you agree to participate) you should ask questions and be sure you understand everything included in it. It’s important to note that taking part in a clinical trial is voluntary, and you can leave the study at any time. 

Why do some patients not receive the treatment? Clinical trial protocols often involve comparing outcomes of patients who receive the new drug or treatment with those who are given a placebo (a non-active substitute). Most times neither the researcher nor the participant knows whether the drug or a placebo is being given. Participants are usually randomly assigned to the treatment or the placebo group. In this way, researchers can clearly see that the medication was responsible for the effect and not some other cause, such as participants getting better on their own.

What are “phases” of clinical trials? New medications and other treatments take place using a step-by-step process that begins in the laboratory (preclinical phases) then moves on to testing in humans. Each of these phases seeks answers to different questions.

These are the questions posed during each of the phases:

  • Phase 1 – Different doses of the drug are tested for the first time in a small group of healthy people—usually less than 100.
    • What are the side effects?
    • Is this drug safe to continue testing?
  • Phase 2 – The drug is tested in a larger group of people who have the disease—ideally a few hundred (but usually fewer in rare diseases).
    • What are the side effects in this population?
    • Does the drug work as treatment for this specific disease?
  • Phase 3 – The drug is tested in a much larger group of people who have the disease—ideally several hundred to several thousand.
    • What are the side effects?
    • How well does the drug work to treat the disease?
    • How much of the drug should people take?
    • How does the drug compare to currently available treatments?
  • Phase 4 – This phase happens after the drug is approved by the FDA and is being used by patients.
    • What are the long-term effects of the drug?
    • What is the best way to use this drug as treatment?
    • Are there other risks to using this drug?
    • Are there other benefits not previously identified?

How safe are these experiments? While all medical interventions carry some amount of risk, those who participate in clinical trials are not “guinea pigs.” The FDA requires researchers to include in their protocols certain measures to ensure the study is ethical, that the rights and welfare of participants are protected, and that the risks are reasonable when compared to potential benefits. And all research organizations require any protocol that involves human subjects to be reviewed, monitored, and approved by an independent Institutional Review Board (IRB) of scientists and ethicists.

You can learn more about clinical trials at the National Institutes of Health (NIH) website.

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How to Reengage with Less Risk

We’re now four months into a global pandemic, and lots of us are just tired of staying home. Most of the folks in our patient communities, however, have underlying medical conditions that put them at higher risk for developing severe COVID-19 infections. So while others consider reemerging into the world again as states start lifting social distancing restrictions, our patients might be reluctant to take this step.

Still, completely avoiding contact with others for an indefinite period of time is not a viable option either. Human beings need physical and social contact with other human beings, if only to stay sane. The social isolation we’ve been enduring since shutdowns in March has caused serious anxiety and depression for a significant number of people, especially those who live alone.

Fortunately, public health experts say the decision doesn’t have to be either/or. While staying home is still the lowest-risk option for avoiding infection, there are lots of ways to socialize that don’t involve crowded indoor spaces where no one is wearing a face mask (the highest risk option). Weighing the risks of contact with others against the possible benefits to your health can help make the decision-making process easier. Here are some things to think about:

Consider the risk for you and your immediate contacts. If you or someone you live with is especially vulnerable to infection, this is an important factor in weighing how much you want to risk coming in contact with someone who might give you COVID-19.

It’s also important to think about those outside your household with whom you may want to socialize and how much exposure they might have had too. You may want to consider creating a pandemic “pod,” a small cohort of friends or relatives with whom you choose to interact somewhat normally but who agree to stay socially distant outside the pod.

Keep tabs on how the virus is spreading in your community. The number of new cases, hospitalizations, and deaths varies widely in different areas of the country. Check local health department reports or find out here whether these numbers are increasing or decreasing where you live. If they are climbing or remain high, you may want to rethink whether going to a salon for a haircut is a good idea, even if restrictions have been lifted.

Think about how risky the activity is. Most people know the basics of how to stay safe from coronavirus infection. Keep these ideas in mind as you make decisions about venturing out in public. These include:

  • Wearing face covering when out in public significantly reduces transmission, both for the wearer and the ones they are with.
  • Maintaining a distance of at least six feet from others decreases the chances of contact with infected droplets.
  • Large gatherings, especially if they are indoors and especially if others are not wearing face covering, significantly increase the risk that someone will pass on the virus.
  • Encounters in the outdoors are safer than those in close indoor spaces.
  • Limiting the time you spend among others, especially if it is indoors, reduces the chances of encountering the virus.
  • Bringing your own (BYO) food and drink means a lower risk of transmitting the virus through touching a contaminated surface. Bringing your own chairs or picnic blankets also helps you keep your distance from others in an outdoors group.
  • Handwashing with soap and water or using hand sanitizer is still the most effective way to prevent transmission of coronavirus and other disease-causing organisms.

And finally, here are additional tips from NYC Health to keep yourself and your loved ones safe and healthy as pandemic restrictions start to lift. Be careful out there!