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Prepare Yourself

5 Second Summary

Although not exhaustive, these steps will help better prepare you or a family member for an unexpected ER visit.

When a medical emergency occurs — and the patient is a person with a neuromuscular disease — it’s not just getting to the emergency department (ED) quickly that’s critical. It’s also critical to ensure the ED staff understands the patient’s particular needs.

“Many emergency department physicians have a limited background in neuromuscular disease,” states Lisa Wolfe, M.D., associate professor of medicine in the Division of Pulmonary Critical Care and Sleep Medicine at the MDA Care Center at Northwestern University in Chicago. Unfortunately, many people don’t discover this potential problem until they’ve arrived for emergency care.

What’s an Emergency?

Respiratory problems are the top reason that people with neuromuscular diseases go to the ED. Dr. Wolfe explains that pneumonia can occur on its own, or aspiration of food particles can cause smoldering infection or acute airway blockages needing emergency care.

Seated white woman with red hair in a high messy bun and shades on top of head with purple printed shirt

Frances Kiperman

Other common reasons people with neuromuscular disease visit the ED include:

  • Flu-related complications
  • Trauma from falls or accidents
  • Cardiac issues, such
  • as associated
  • cardiomyopathies
  • Gastrointestinal issues, such as constipation
  • Orthopedic issues, including muscle or joint pain and hip subluxation

Expect the Unexpected

In the event of an emergency, “most people who have neuromuscular conditions go to a community ED first,” says James Naprawa, M.D., a pediatric emergency medicine physician at UCSF Benioff Children’s Hospital in Oakland, Calif. Because most ED staff have little contact with neuromuscular conditions, they might overlook signs of distress or misinterpret the problem.

“The kids I see are typically adolescents coming in with breathing difficulties,” Dr. Naprawa says. “They tend not to look very distressed because of the weakness in their muscles. They may not be coughing. They can appear surprisingly well.”

In 2018, Dr. Naprawa co-authored a paper that appeared in Lancet Neurology on new care considerations for Duchenne muscular dystrophy (DMD), which led MDA to co-publish the new Duchenne Guide for Families (find it at mda.org under Care & Services in MDA’s Resource List for Families). Dr. Naprawa found that working on the paper opened his eyes to the nuances of caring for neuromuscular disease patients in crisis. Now, he is quick to recognize muscle weakness, and he is aware that these patients may be taking steroids to prolong their muscle strength, can have abnormal heart rates and may experience problems that are rare in other patients.

Frances Kiperman, a Florida resident who lives with myasthenia gravis (MG), has dealt with a lot of misunderstandings in the ED over the years. One involved a physician who thought that her legs looked OK, despite the fact that she could not stand independently. The doctor pushed her wheelchair up to a gurney and told her to get on. As she leaned on the gurney, still using her chair for support, he abruptly pulled the chair back, thinking he was moving it out of her way. “I managed to punch him on the jaw as I fell to the floor,” she says.

But not all surprises in the ED are bad. Years ago, when my oldest daughter, who had spinal muscular atrophy (SMA), was home from the hospital after having a tracheostomy, the surgical trach became blocked. At the ED, the physician took the time to teach me how to change the trach. Under his guidance, I removed the surgical trach and replaced it with a removable, cleanable one. This lesson helped me handle subsequent blockages at home and probably saved us many more trips to the ED.

Advocate and Educate

Any time you visit the ED, it is critical that you or a loved one has your medical information on hand. You can do this by having a printout or cards (some people laminate them) that you keep in your purse or backpack, a photo of that information on your phone and a medical alert pendant or bracelet with your diagnosis and emergency contact. Your caregivers should all know how to access your medical information in case you cannot speak for yourself.

Having a friend or advocate with you in the ED is also valuable for the practical help they can give you for small tasks like reaching the call button and holding drinking cups. You and your advocates need to be aware that their presence with you in the ED is a privilege, not a right. If a buddy, spouse or parent causes a commotion or is verbally abusive to staff, they will be sent away.

Advocating for yourself is an important skill to develop as well. Oregon native Toria Tozer, who lives with SMA, recounts a time she landed in her local ED with a gallbladder infection that led to sepsis.

“I had been in the ED three times prior,” she says. “They finally decided I needed my gallbladder out, and I requested a 45-minute transfer to an academic hospital in Portland. The doctor argued with me, saying I’d be dead by the time I got there. I told him last time the hospital tried to do surgery, I aspirated due to my small airways from SMA, and if I didn’t transfer, they’d probably kill me anyway.”

In the end, Tozer was transferred to the hospital she requested, and she learned the power of self-advocacy. “I was proud of my ability to handle myself in what could have become an even more dangerous situation,” she says.

Be Prepared

If at all possible, bring your own medical equipment to the ED, especially bipaps or other respiratory support items. Some hospitals may not have access to equipment such as a cough assist machine, and, Dr. Naprawa points out, patients and caregivers feel more comfortable with the equipment they’re accustomed to.

White couple seated, with bald man in checkered shirt with arm around woman with black hair in white shirt

Toria Tozer with her husband, David

Dr. Wolfe cautions that some hospitals may not allow unfamiliar equipment to be used. In that case, she advises asking if the hospital can provide equipment that is similar to the patient’s and a respiratory therapist trained on that equipment.

Along with a list of current medications that should be included with your medical information, try to bring along your actual medications, especially if some of them are specifically for your neuromuscular condition. The hospital pharmacy may not have what you need on hand when you need it.

Before you put this magazine down, write down the important information listed in the sidebar — don’t put off documenting your physical needs.

Having that information when you arrive at the ED’s sliding doors will help the staff inside get you the right care. It could be the thing that saves your own or a loved one’s life.

Right Place, Wrong Care

Emergency department (ED) doctors and nurses who are unfamiliar with neuromuscular diseases might miss signs of distress or misinterpret them. Here are issues to watch for in the ED:

Appearance: A patient whose muscles are floppy can be subjected to a full spinal workup looking for injuries, especially if they aren’t able to speak for themselves and aren’t wearing medical alert jewelry.

Communication: Friedreich’s ataxia (FA) can cause slurring of speech, and spinal muscular atrophy (SMA) or amyotrophic lateral sclerosis (ALS) can make speech difficult to understand, leading doctors to unnecessarily test for drug or alcohol abuse.

Respiratory distress: Emergency medical staff’s immediate reaction may be to give oxygen, but if the problem is weak respiratory muscles, the extra oxygen could dangerously suppress breathing. In some cases, an ED physician may want to do a tracheostomy when all that is needed is to provide noninvasive supportive care while the underlying respiratory infection or obstruction is resolved.

Anesthesia: People with certain inherited neuromuscular diseases (Becker muscular dystrophy, Duchenne muscular dystrophy, myasthenia gravis, SMA and central core disease, among others) are at increased risk of a dangerous reaction to anesthesia called malignant hyperthermia. Talk with your healthcare providers about whether you have a specific risk.

Do It Now!

Going to the emergency department (ED) means you or your loved one has a medical situation that can’t wait. Here are three ways to prepare in advance to get ED staff up to speed on your needs as quickly as possible:

  1. Keep your important medical information in one place. This should include information on your condition, a list of your medications and a list of your healthcare providers with contact information, including on-call numbers and how to contact key doctors in an emergency. The International Ventilator Users Network has a thorough healthcare form that covers everything from medications and vent settings to typical vital score readings and positioning instructions. Find this resource at ventusers.org/vume/PatientInfo.pdf.
  2. Practice with your doctor. At your next appointment with your care team, ask what kinds of situations might come up in the ED and what information an ED physician would need when treating you. Find out where your doctors have hospital privileges so you can go there if you have a choice.
  3. Have an advance directive. An advance directive, or healthcare directive, lets your medical providers and caregivers know the amount of medical intervention you are willing to accept. Ask your MDA Care Center team about setting up an advance directive.

Once you have documented this information, give copies to your medical providers, family and caregivers. Also tuck a copy in your backpack or purse and keep images of the forms on your phone.

And don’t forget to review the information at least every three months, or whenever your medications or treatments change.

Donna Albrecht is a health writer who lives in Northern California with her husband and border collie.

Next Steps and Useful Resources

Disclaimer: No content on this site should ever be used as a substitute for direct medical advice from your doctor or other qualified clinician.