On Dec. 28 Andrea, our 41 year old daughter, had her ablation done to treat the atrial flutter in her heart, which was diagnosed this last Sep 27 at her annual cardiology appointment. From my understanding, atrial flutter is similar to atrial fibrillation except that the flutter is more centrally located and therefore easier to find and treat. The ablation was successful and she was released from the hospital the same night because the team did such a good job of paying attention to the anesthesia risks for those with DM. A week before her procedure, due to Myotonic Dystrophy (DM) and anesthesia concerns we scheduled a pre-op appointment at the clinic where the procedure would be performed. We schedule these pre-op appointments anytime a family member has an upcoming procedure that might involve anesthesia. We always hand the team a copy of the form that we download at this link. https://www.myotonic.org/sites/default/files/pages/files/MDF_PracticalSuggestionsDM1_Anesthesia2_17_21.pdf This is a lengthy form. There is also a summarized condensed form available to give the medical team at https://www.myotonic.org/sites/default/files/pages/files/MDF_PracticalConsiderationsAnesthesiaQG_1_21.pdf Andrea’s anesthesiologist was very fascinated with the Myotonic Anesthesia guidelines that I gave him. He was impressed by the list of credible doctors that put together the paper. When we met with him he told us that anesthesia is different now than it used to be. Succinylcholine, one of the higher risk anesthetics is hardly ever used anymore, according to him. But, that being said, I would never trust a medical team to know how to treat a DM patient’s anesthesia needs without visiting with them and providing them with the myotonic guidelines. Andrea’s anesthesiologist handled everything with Andrea very well. After her procedure he even used a drug to reverse the anesthesia. Andrea’s breathing recovered almost immediately and she was very coherent. They monitored her for many hours. Her breathing and cognition remained constant and she was released to go home late that night. It is my understanding the malignant hyperthermia can be a problem with many Muscular Dystrophies. Issues with malignant hyperthermia is not necessarily more common with DM than the regular population. But, there can be many other complications of anesthesia for those with DM. Even someone who has a very minor case of DM is still at risk for these complications. Educating your anesthesia doctor and surgical team is very important. For instance, my husband Kent had an appendectomy in 2012. It was at a local hospital, not the University of Utah (which is our regional MDA hospital). I took in all of the anesthesia guidelines and gave them to those doing the surgery. They seemed to listen well and paid attention. They used propofol, which can supposedly be one of the safer anesthetics to use. I am not sure, but I assume that they even used a low dose of propofol. The surgery was at 3 a.m., in the middle of the night. Everything went well and they brought Kent to a patient room after recovery. They watched him closely and took good care of him. By 5 p.m., 14 hours later, Kent’s oxygen rates were running a little low. They had him take a couple of deep breaths and his oxygen sats (saturation) went up to normal and they told us that he could go home. I was concerned that his oxygen levels weren’t staying high enough and mentioned it to them. They reassured me and told me that he was fine since he was able to bring his rates up when he took deep breaths. I wasn’t confident in my own opinion so I let them send him home. The next time that we met with our neurologist, Jacinda Sampson, I told her about the appendectomy surgery and about the lower oxygen sats when he was released. She was quite concerned that they had released Kent with his decreased oxygen sats. She told me that the next time something like this happens that I need to have the medical team contact her. She said that she would tell them that if they want to release him that is fine but that she will provide him with a bed at her hospital immediately after leaving their hospital. She was pretty sure that would cause them to change their mind and keep him longer. Jacinda Sampson is no longer our doctor and she is now at Stanford. But, her words gave me a lot more confidence in how to handle things. If I were ever in that situation again, I wouldn’t hesitate to be more assertive or to reach out to our local MDA doctors. Or, I would reach out to the Myotonic Foundation to ask them to help me find the resources needed to advocate for my family member. After Andrea’s procedure they didn’t send her home with any prescription pain medications. She didn’t need any because a pacemaker/defibrillator wasn’t placed so there were no incisions. Only a small cut at her groin where the doctor threaded all of the leads into her heart to do the ablation. They told her to treat it with ice if needed, and she could take Tylenol for the pain. Andrea is on blood thinners due to the flutter so she can’t take ibuprofen. I do want to mention here the risks of narcotics for those with DM. Narcotics can be as dangerous as anesthesia. We even have a dear friend that passed away from narcotics given after being released from the hospital after surgery. The team did everything correctly regarding anesthesia and DM but they sent her home on narcotics. She passed away in the night due to the suppression of her breathing from the narcotics. My husband had to use narcotics a long time ago to treat the pain from his multiple myeloma. Kent had eight collapsed vertebra and was in a lot of pain. The medication options weren’t working well enough for him. We knew that narcotics were very risky for him. His medical team told us that a standard dose of dilaudid was 2 mg. They prescribed liquid dilaudid for Kent. They had him start with about one-fifth of the 2 mg. dosage. Because of his hypersensitivity to medication that was enough to treat his pain without suppressing his breathing. They started him on the narcotic when he was still in the hospital and they supervised him to make sure that he didn’t have any complications. Surgery, anesthetics, and pain medications are all very risky to our DM population. But, sometimes they are needed so there needs to be a lot of information provided to the treating doctors and a lot of caution needs to be used.