Going to the Emergency Room: Tips for People with Neuromuscular Diseases

The biggest problem at an ER may not be the one you go in with, but the one you encounter there

When a medical emergency strikes — and the patient is a person with a neuromuscular disease — it’s not just getting to the emergency room quickly that’s critical. It’s also critical to ensure the ER staff understands the patient’s special needs caused by muscle disease.

“Most ER doctors will NOT understand these diseases,” states Gregory Carter, medical director of the MDA Regional Neuromuscular Center at Providence St. Peter Medical Center in Olympia, Wash., and co-director of the MDA/ALS Center at the University of Washington Medical Center in Seattle.

“Because of that, a respiratory patient can end up with an unwanted tracheostomy when an ER doctor pushes for aggressive care,” Carter says, noting that this has happened to several of his patients with Duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA) and ALS (amyotrophic lateral sclerosis).

Respiratory problems are the top reason that people with neuromuscular diseases go to the emergency room, says Carter. Other common problems are dehydration and constipation/abdominal pain.

But the biggest danger at the ER may not be the problem you go in with, but the problem you get there. Besides the aforementioned unwanted tracheostomy (insertion of a breathing tube through the front of the throat), individuals in respiratory distress may be given supplemental oxygen, even though their problem is not caused by lack of oxygen but by weak respiratory muscles. Too much unnecessary oxygen can cause a potentially life-threatening suppression of breathing.

In addition, certain muscle diseases (including some muscular dystrophies, central core disease and multi-minicore disease) can cause a dangerous reaction to anesthesia called malignant hyperthermia.

Some neuromuscular disease symptoms can lead to serious misunderstandings in the ER, warns Michele Dressman, a pediatric nurse in the emergency room at John Hopkins Hospital, Baltimore, and longtime head of an MDA summer camp medical team.

For instance, she’s found that people with Friedreich’s ataxia often have slurred speech, leading ER providers to suspect drug or alcohol abuse. Or, a person whose neuromuscular disease causes floppy extremities and a lack of reflexes may be sent for a full spinal work-up looking for injuries. A young adult with advanced DMD who is complaining of abdominal pain may be treated for stomach problems but actually may be experiencing a cardiac event.

The best strategy for going to the emergency room is to be prepared before the emergency strikes. To that end, here are some tips from medical experts, and adults and parents of children with neuromuscular diseases.

Provide updated and complete medical information

One ER physician who understands neuromuscular disease and why it sends many to the ER is Anne Rutkowski, a full-time ER physician in Harbor City, Calif., and the parent of a daughter with congenital muscular dystrophy.

In order to communicate effectively with health providers in an emergency, Rutkowski recommends MedicAlert, the gold standard for getting personal health information into the hands of paramedics and ER staff.

The distinctive MedicAlert bracelets or necklaces spell out diagnosis and allergies, and provide a phone number for accessing more detailed information like medication lists and personal physicians.

But, warns Greg Adams, director of educational services for MedicAlert, keeping MedicAlert records current is vital to getting the best results in an emergency. When ER doctors call the service, they get both the information and the date when it was last verified. Adams says providers look for information that is less than 1 year old and prefer information that’s been confirmed in the last 90 days. This means updating MedicAlert records every time you get a new prescription, stop a medication, or get a new diagnosis or physician.

Buddy up: Take an advocate to the ER

Even when ER patients can talk for themselves, it helps to have someone else there — a spouse, parent or friend — to remind medical staff that the patient has muscular dystrophy, not multiple sclerosis; no, she can’t get up on the bed by herself; no, he can’t lie flat, he has contractures; and so on. And a buddy can push the nurse call button if the patient can’t.

Having a companion ensures that not only does the ER staff know about any special concerns, but so does the lab or X-ray staff. For Mary Sammons of Enterprise, Ore., who has limb-girdle muscular dystrophy, the misunderstanding began when the X-ray staff put her in a chair with wheels, but apparently no brakes. “The chair slipped out from under me when I tried to transfer and I was clinging on for dear life to the X-ray machine while three others where trying to get me back in the wheelchair,” she recalls ruefully.

Besides having a buddy, continue to be a self-advocate, advises Annie Kennedy, MDA senior vice president for advocacy. “You know what’s normal for you. Many ER docs don’t know neuromuscular diseases well and may minimize symptoms that are actually quite significant. If something isn’t right and your gut tells you so, advocate until someone pays attention to you.”

And remember: Although providers usually are happy to allow an advocate to stay with an ER patient, this is a courtesy. If the buddy, spouse or parent becomes emotionally overwhelmed or verbally abusive, they probably will be shown the door back to the waiting room.

Advance directives speak for you

There is a document you must prepare if you want to be sure your wishes are followed in a life-or-death situation. Advance directives are available online, but be sure to get one that’s recognized in your state. They contain several parts:

• a medical power of attorney, designating the person (and possible backups) who can make medical decisions if you’re unable to speak for yourself;
• specifications about how much (if any) life support you want, the level of pain management you want; and whether you want to donate some or all of your body; and
• the name and contact information of the physician you want to handle your medical care.

Many MDA families use the Five Wishes Living Will. The document (which costs about $5) can be customized and translated into 26 languages and meets the legal requirements in 42 states. Not only is it useful in emergencies, but it can help families start important-but-difficult discussions about care in times of serious illness.

It’s not all bad

Although ER providers aren’t always current on neuromuscular conditions, you might be surprised by how much they can teach their patients, or their parents and buddies.

For example, one night shortly after my daughter with SMA received a tracheostomy, her appliance started to block and I was having trouble clearing it. Since it was about time to replace the surgical trach with a removable/cleanable one, the ER doctor took the time to teach me what needed to be done and supervise me as I changed her appliance.

Definitely scary — but extremely helpful.

Sometimes an ER temporary fix can actually lead to a better situation. For example, Anne Rutkowski recalls an ER patient with ALS who came in with a problem with his feeding tube.

The hospital didn’t have the exact type of tube he’d been using, so she used a Foley catheter as a temporary fix. He liked it so much that when she called him to tell him she had the tube he needed, he told her he liked the Foley better and wasn’t interested in trading it in!

Emergency Room Prep

• Get the facts from your doctor. During a regular visit, have an “emergency” conversation with your MDA clinic physician and specialists (pulmonary, cardiac, etc.). Ask what symptoms should be taken seriously, and what kinds of special circumstances might come up in the emergency room. Find out where your regular doctors have hospital privileges, so if you have a choice of emergency rooms, you can go there. Ask if you can call them while en route to the ER. If you’re at a hospital where your regular medical team doesn’t have privileges, the ER staff may be willing to reach out to them anyway.
• Have an advance directive. The “Five Wishes Living Will” document can be a good starting point. Call (888) 594-7437 for more information.
• Put important information all in one place. Prepare a folder that can be quickly grabbed in an emergency, containing all necessary insurance information; medical diagnosis; doctors’ names and contact info; current prescriptions and supplements; and any recent test results.
• Have remote access to your medical information. To contact the MedicAlert service described in the main article, call (888) 633-4298. Small, portable memory-stick-type devices can be loaded with personal medical info and carried in a purse or on a keychain. Check out MedicTag (medictag.com) and MedIDCard (877-916-3343). Each costs less than $50.
• Bring a friend or relative. They can provide comfort and distraction, advocate for you with medical staff, and take notes on all procedures, medications and tests — helpful if you have questions later or want to challenge your bill.
• Prepare for a long wait. A book or personal music player can make the time pass faster. Parents, remember to bring your child’s favorite lovey or blankie; it can significantly reduce their stress. Bring a little cash, so parents or buddies can have a cup of coffee or sandwich. (But don’t give any food or liquids to the patient without permission from the ER doctor.) And don’t forget a cell phone and charger!
• Speak up. Whether you’re in the waiting room or inside an ER examining room, tell the ER staff about ANY change in your condition. They aren’t mind readers and might not realize your status has changed if no one tells them.