Let’s explore the treatment of Long QT Syndrome in terms of the medical care given to someone with the disease. Today we’ll look at medications. Another day we’ll address the other two forms of treatment, medical devices/surgery and lifestyle changes. Often a combination of treatments is employed, with each form bringing value and its own bag of problems.
There are two goals to consider when deciding upon treatments. The first objective is to prevent the long QT heart from ever beating out of control. You really want to be successful in meeting this goal. If the heart does go out of control, then the next objective is to prevent sudden death. You really, really want to achieve this one.
The attached list of medications are used as a preventive measure. I have Long QT Type 1, so mexiletine is foreign to me since it treats type 3. It would make sense for me to keep my potassium levels up, since type 1 is a deficiency in the heart’s potassium channels. Fish oil seems to be one of those foods that is good for everything. Let’s talk beta blockers.
For my family, taking beta blockers has been akin to taking a daily vitamin. We are very blessed not to suffer from side effects. Others find them extremely debilitating, particularly the fatigue aspect. You generally feel fine with Long QT Syndrome. You may have never had a cardiac event, and are being given medication because your condition was discovered on an EKG. You were living a pretty normal life. The medication knocks you out, and you now feel anything but normal.
There are several different beta blockers, but some are better than others. I started my Long QT life on once a day atenolol. If I remember correctly, my babies were started on three times a day propanolol, since it came in liquid form. As they grew older, they were switched to atenolol, because that was what I took. This all changed for my children in 2004. It took another eight years for me to change.
Our children and I took a little summer trip to visit my brother and his wife. They had a pool in their back yard, and the kids were having a great time playing in it. I was watching my son calmly float on his back, with his arms wrapped around a large beach ball resting on his chest. I saw him release the ball, and sink to the bottom of the pool. There was no call for help, or slashing at the water. I knew exactly what had happened, as I recalled my own personal experiences. He was having a long QT cardiac related event.
We got him, and everyone else, out of the pool. My sister-in-law called 911. My son was making moaning noises, and seemed to be in a seizure, but he wasn’t breathing. Somehow it looked different than what I expected. He wasn’t laying motionless. After all the CPR classes I had attended, I was unsure of whether to begin resuscitation measures. He started breathing on his own. His heart had recovered. That hesitation in my reaction still gnaws at me.
There was nothing more the paramedics could do after they arrived. He was breathing, and crying. I took him to the ER for them to monitor the situation, and to contact his electrophysiologist for further instructions. One of my older sons, the humorous one, volunteered to go with me to help the afflicted son be distracted from his trauma. I soon began to regret that decision. His idea of helping was describing all the ER equipment in the room as torture tools and evil robots. He did eventually promise to buy his brother a cool t-shirt, which calmed things a bit.
After we returned home we reviewed the situation with our doctor, and noted the following:
- He was taking the beta blocker atenolol, which is not very long lasting in your system.
- He had missed a couple of daily doses during our little vacation.
- He had been ill earlier in the week, and not eating very well.
- He was swimming, which is the most common activity for a Long QT Type 1 event.
Our kids were immediately switched to the beta blocker nadolol, because it remains effective in your body longer. I have attended several SADS Foundation conferences since that event, and they began stressing the use of a different beta blocker than atenolol.
After the second conference where they delivered that message, I personally switched over to nadolol. My doctor was not concerned because of my age, and lack of any cardiac events since I had begun taking beta blockers. He left it up to me. My hesitation stemmed from reading about others suffering from the beta blocker side effect of fatigue. I had no problems with the switch, and felt more confident in the effectiveness of the nadolol.
Next we worked on doing a better job of taking our medication. Here are a few tips:
- Use a weekly pill box. It is easy to see if your medication has been taken that day. When there were six of us taking pills, it made it easier to fill the pill boxes on one day, rather than opening six pill bottles daily. If you have young children then you need to safeguard the pill boxes as they are not childproof.
- Use your smart phone. Set an alarm, download an app, or use a text message service to remind you to take your pills.
- Make taking your pill a set part of your routine, just as you would brushing your teeth. Associate it with another task, such as a daily drink of orange juice.
- Place your medication in a place to visibly remind you to take it.
Your beta blocker’s job is to keep your heart beating normally. It only works if you take it.