LIVING PROOF Jake Scott was stricken during surgery, but early recognition of MH's symptoms saved his life.

My son Jake had just turned 19 and was scheduled to undergo a septorhinoplasty revision to fully repair a deviated septum. The first surgery went perfectly, so I arrived at the surgery center where I used to work as a surgical nurse thinking the second procedure would be routine. My son was the anxious one. He worried about waking up during surgery or even dying on the table. I reassured him. Nothing will happen. I promise.

But something did happen. About 45 minutes into surgery, Jake's CO₂ level began to increase. His heart rate also started to rise and he became tachycardic. The CRNA also noticed that his core body temperature began to rise and paged the supervising anesthesiologist. Together they called the hotline of the Malignant Hyperthermia Association of the United States (800-644-9737) and the expert who answered advised them to immediately begin the MH emergency response protocol. It wasn't a drill. It was the real deal and my son is alive today because the surgical team reacted calmly and with purpose. They recognized the warning signs of MH and initiated the response protocol, including administration of dantrolene, without delay.

This article isn't about how to react to MH emergencies. You've probably run through your facility's response protocol countless times. It's about taking those drills seriously. It's about walking into every surgery expecting the worse, so you're always prepared and alert for signs and symptoms of MH, and ready to enact the response protocols that are hopefully second nature.

Is this really happening?
The surgical liaison called my name an hour after the procedure began. I remember thinking that they called me much sooner than before, that the procedure must have gone smoother than anticipated. I waited in a consult room, and one of the surgical nurses who I had worked with for many years entered and took my hand. What the heck is going on?

"Kim, Jake had what we think was an MH reaction," she said. "He's fine, he's stable and it's all under control." I was in disbelief. I knew what MH was and how rare it is. Wait. What? Oh my god, oh my god. That's all I could manage to say.

The nurse was very reassuring. She told me that Jake's temperature was back down, that his heart rate was slowing again. The surgeon came in to let me know Jake was doing well, but that they'd be transferring him to the intensive care unit at the local hospital.

I wanted to see him before he was transferred, so they led me back to the recovery room. It was very difficult to see my son still unconscious and intubated with EMTs, physicians and nurses milling around his bed. They told me I could let him know I was there, even though he was still sedated with a propofol drip. It was frightening. Even today, the emotions of that moment flood back.

Two anesthesiologists rode in the ambulance to the hospital with Jake. One of the center's nurses insisted on driving me over. Two of the nurses who cared for Jake at the ICU were former nursing students of mine. I knew he was in good hands, but I still spent the night by his bed, reacting to every blip of the monitors and probably being too attentive to his care like only a mother with a nursing degree can. The ICU staff extubated Jake the next morning. He progressed slowly, but recovered fully. He survived.

Warning signs
Patients with gene mutations associated with MH susceptibility and members of their immediate families are at increased risk, but might not be aware of the dangers. Just because high-risk individuals underwent previous surgeries without incident doesn't mean MH won't develop during subsequent procedures.

It occurs in about 1 in 100,000 adult surgeries, according to MHAUS, although the exact incidence is unknown.

Genetic testing showed I carry the gene for susceptibility, but I've undergone 2 surgeries without incident. Jake also had a previous surgery, and was unaware he was at increased risk. MH is that unpredictable. You don't necessarily know when MH will strike. Never let your guard down — always be prepared to act quickly, even when MH isn't anticipated.

Know the triggering agents: inhaled general anesthetics desflurane, enflurane, ether, halothane, isoflurane, methoxyflurane, sevoflurane, and the muscle relaxant succinylcholine. Episodes can also occur in the hour following emergence from general anesthesia, so remain alert for warning signs in recovery.

Patients experiencing an MH reaction will usually have a steadily increasing expired carbon dioxide level, tachycardia, muscle rigidity, and severe metabolic and respiratory acidosis. Don't look for a rapid rise in core body temperature. That telltale sign often occurs after the other early warning signs. Reacting to it might be too late.

Be clear and comforting when communicating with the loved ones of a patient who's stricken with MH. The nurse who talked to me was kind and therapeutic. She knew me, but I'd like to think she would have been that way with anyone in the same situation. Even though I knew more about MH and had a clinical background, the efforts the staff, surgeon and anesthesiologist made to keep me informed and comforted made a big difference in how I reacted and processed what was happening to Jake.

GENETIC TESTING
Determining MH Susceptibility

DRILL TEAM Testing can identify at-risk individuals, but your team still needs to be prepared to act when MH isn't suspected.

Part of the difficulty in reacting to my son Jake's malignant hyperthermia event was where to turn next. Patients who are concerned about their own susceptibility or want confirmation that an episode they experienced was in fact MH are often told to undergo muscle biopsy testing.

That's easier said than done. I'm a nurse, health-literate and know where to find resources on the Internet, but I didn't know where to begin to find out why Jake had an episode and if any other members of my family were in similar danger. The experts on the hotline of the Malignant Hyperthermia Association of the United States recommended muscle biopsy testing and told me where I could go to get it done.

The University of Minnesota is 1 of 5 facilities across the United States and Canada that offers the service. I scheduled an appointment with an expert there, but the clinic is 3 hours from where I live, and I had concerns about the risks and cost of muscle biopsy testing, so I also contacted a genetic testing center at a health system near my hometown. After a counseling session, staff drew Jake's blood and sent it off for testing to see if one of the known genes associated with MH susceptibility could be identified. Jake was positive for a mutation of the RYR1 gene, the most common mutation associated with MH. I was tested and also found to have the RYR1 gene mutation.

My advice: Suggest that patients who are concerned about MH first contact a local genetic testing center. It's an accessible and affordable place to start in their quest to determine their MH susceptibility.

— Kim Scott, RN, MSN

Be ready
I was a surgical nurse for more than 15 years and involved in more than 20,000 surgeries, but never witnessed an MH episode. Sure, like your staff, I rehearsed the response protocols during drills. But did I treat them seriously enough, like my son was the one stricken on the table? You never know when MH is going to strike. It doesn't happen often, but when it does, early recognition and reacting quickly can mean the difference between life and death for the son or husband or daughter or mother of someone in the waiting room. Practice like it's the real deal, because someday it will be.