Myotonic Dystrophy (DM) is one of the over 40 muscular dystrophies. As the name muscular implies, muscles are affected. Since the heart is a muscle it would be easy to assume that the muscles in the heart are what is affected. Not usually so with DM. In both DM1 and DM2 it is the electricity of the heart that is usually impacted.

It is important for those with DM to see a cardiologist who is experienced in the treatment of DM. My family has annual cardiology appointments with Dr. Daniel Cox at the University of Utah, who treats people with congenital heart problems including DM. We keep these annual appointments for everyone, including Andrea, even though it has seemed that she didn’t need to see the cardiologist since there were no obvious effects. In fact, Andrea almost fell through the cracks this year. When I realized that Kent had his annual appointment, I remembered that Andrea usually goes with him and that she didn’t have an appointment. When I called to make her appointment, they coincidentally had just had a cancellation for the next Wednesday so we took the appointment.

At the beginning of these appointments an EKG is always done. When Andrea’s EKG was done, Rachel who was performing the EKG, left with the results. Almost immediately Dr. Cox showed up in Andrea’s room. Interesting, he must be ahead of schedule. He asked Andrea how she was feeling and she sarcastically said “Terrific.” He asked her if that was true and she told him that she was doing fine. So then Dr. Cox proceeds to ask her if she feels faint, light headed, or exhausted. Andrea tells him that she is always exhausted but does not feel faint or light-headed . He asks her if she can feel her heart racing and Andrea says that she cannot feel that.

Now we find out why Dr. Cox was so quick to come in to Andrea’s room. He tells Andrea that her heart is in Atypical Atrial Flutter. And what that means is that the bottom of her heart is beating at the normal 80 beats per minute but the top is beating at 300 beats per minute. Wow, crazy. And Andrea wasn’t even noticing anything abnormal. Dr. Cox says that they need to get her out of the Atrial Flutter but that involves either shocking her heart, you know, like you see on television. Or an ablation where they go into her heart and cauterize the spot that is creating the Flutter. The ablation is desired because it might be a permanent fix. First she will have to see a cardiac Electrophysiologist who can then schedule the procedure.  Dr. Cox will alert Dr. Steinberg, the electrophysiologist, that he needs to get her in to his office ASAP. He explains to Andrea that because her heart is doing this that it will also be likely that she will need a pacemaker/defibrillator placed at the same time as the ablation procedure. Supposedly, if she already had the defibrillator, it would have been able to take her out of the flutter. Andrea has known that she was eventually going to need a pacemaker because once a family member receives a pacemaker then the rest of the family, or first-degree relatives, are likely candidates. It is even valid to place a pacemaker in the rest of the family merely because their first-degree relative has one.

In the meantime, until Andrea has the ablation, because her heart is beating erratically it can cause blood clots. Andrea will need to go on Eliquis, a blood thinner, until the flutter is stopped and they determine that her heart is beating correctly.

As they visit, Dr. Cox tells Andrea that the Atypical Atrial Flutter started sometime between the appointment a year ago and now.  And they try to figure out possible effects that Andrea may have noticed, trying to determine when this Flutter started. I erroneously assumed that it was coincidentally happening at the time of the appointment and that it was a momentary event.

Andrea and her partner Mark play Frisbee golf at least once a week. Andrea has been proud of that because there is a lot of walking. Andrea can handle the Frisbee Golf walking because it is broken up by lots of rests while she watches the others throw their Frisbees. This activity gives her exercise and gets her out of the house. Just under a year ago Andrea had to stop going with Mark because she felt exhausted and faint but didn’t understand why (there was the symptom that Dr. Cox asked her about when he first came in the room). They decided that must have been when this flutter started. She has been at risk ever since this flutter started and she didn’t even know it.

When Andrea went home that night she had a hard time sleeping for several nights because she was afraid of not waking up. But, since she was now on Eliquis she is probably safer now than she has been for the last year.

In my recently released book on Amazon Surviving Myotonic Dystrophy: A Mother’s Struggle to Care for Her Family with a Rare Disease I have additional information about the effects on the heart. On page 79 it says:

“Heart Problems: The electrical system of the heart, which is responsible for controlling the speed of the heartbeat, can be affected even when there are no other symptoms. In some people this can cause dizzy spells and blackouts, but a problem with the heart rhythm can be there even without symptoms and may need to be treated to stop it from getting worse.”

One of the causes of death in DM is heart failure, sudden death. Without modern technology all of my family would be at risk of sudden death. I know of families with DM that have a not too far distant ancestor that had sudden death at a young age. Andrea will be the last of our children to have a pacemaker placed. It feels frustrating to my children that they need pacemakers but I gently remind them that without them they might not survive.

Warren was the first in our family to get a pacemaker. He got his in 2016. Then each year after that Michael then Chad got theirs.

Andrea’s appointment with Dr. Steinberg, the electrophysiologist, is the day after her birthday. Happy Birthday Andrea.