Living with long QT syndrome
Our world came crashing down in March 2006. My niece had an odd episode with her heart and, unsure which side of the family it came from, we all got tested for long QT syndrome. Long QT syndrome (LQTS) is a rare inherited heart condition that causes irregular heartbeats. This can lead to palpitations, fainting, and sudden death due to ventricular fibrillation. After many months of anxiety waiting for our results to come back we got a call to come in to the doctor’s office. My heart sank hearing that my two daughters (ages two and almost four) and I tested positive for long QT. My first feeling was sadness and then that quickly turned to disbelief. Looking back we determined that there had been four sudden “heart attacks” in our extended family and now 17 of us have tested positive for LQTS.
During my first two pregnancies I had experienced tachycardia; at one point my heart rate reached 211 beats per minute and I was hospitalized. I had heart ablation done, still not knowing I had long QT. It wasn’t until three years later that we were diagnosed. Both of my girls, Mallory and Dalena, tested positive but my son was negative. We were all put on beta blocker medications, which slow down the heart and help to prevent irregular heartbeats. The girls began to see Dr Sanatani regularly, whenever he came to our area with the outpatient clinics. At the time, we lived in a remote community outside Terrace, BC with no access to an ambulance. Thus, if we needed one it was quite scary.
We relocated to Vancouver a few years later and if I thought being so far away from an ambulance if we needed one was scary, I soon found out how wrong I was. Living in the city and dropping my kids off at school was even scarier. I always had the fear of something happening and that I wouldn’t be close enough.
Sure enough, when Dalena was in grade one, I got the frightening phone call from the school. All I could hear was “Is this Carmen? We have Dalena here in the office and we are waiting for the ambulance to arrive.” It was a good thing I was very close by to her school. I had been on my way to Children’s Hospital for an appointment with my son. I could not drive fast enough and I just wanted everyone out of my way. I parked my van, ran into the school as the ambulance was still driving down the street. I could see my baby girl sitting with the principal, she was clammy, very pale and could still not talk. The paramedics came in and did an ECG right away and loaded her in the ambulance. Along the way they did another ECG and that one was still abnormal. Dalena had been sitting in class and all of sudden she had chest pain and couldn’t talk. She tried to put her hand up to tell her teacher, but nothing would come out. Her teacher could tell something was wrong and immediately got the principal. To this day I am so grateful that my daughter was in good hands. The school handled things very well.
At the hospital we were seen by a doctor that I wasn’t familiar with. I felt scared and very numb and it felt like all I could hear was a snowy TV. I recall them talking to me about a pacemaker or going another route and implanting a Reveal device. It was decided that a Reveal device would be implanted and it would be a simple procedure. They told me it would be simple, but in the back of my mind I was terrified. A Reveal is a device about the size and shape of a rectangular cigarette lighter. It monitors your heart’s rate and rhythm and sends the results to the Heart Centre at BC Children’s.
The plan was that Delana would keep the device in for 3 years and if she had more episodes we would look at other options. She did have another episode of some sort, but we’re not sure what exactly it was as the battery was dead in the remote control to activate her Reveal device. I learned very quickly to change the battery every month (more often than required) just to be on the safe side. When Dalena has a new teacher or caregiver I have to teach them how to use her device in case of any episodes. This means holding the remote over her device and pressing the button until the green arrow comes on. Many people are scared of having to use it and I don’t blame them! Having to carry a purse around with something so critical (the remote) at the age of 9 isn’t normal, but is our “new normal.” Give a 9-year-old girl the responsibility of carrying a purse everywhere and it will eventually get left in the mall washroom, which can be very frightening.
Managing the medications for all four of us (my son has other health issues) can be very challenging. I was always worried that I would make an error in giving medication to Dalena. I accidentally did give her Mallory’s medication one night. I love the advantage of having our medication “blister packed” now. When having to administer medication between the four of us, it makes life a tiny bit more simple.
The girls are now 11 and 9 years old and both are doing well. I’m glad to say that my eldest daughter, Mallory, hasn’t had any episodes but I worry constantly as she is very athletic. She has had the most difficulties with the beta blockers (weight gain and severe headaches) so she has had to change her meds a few times. We think we have found the right one for now. I was initially very nervous to allow them to play soccer but after the okay from cardiology they have been enjoying a fairly active life; Mallory is playing basketball as well.
Seven years ago I was in denial—I didn’t think that this diagnosis would have a drastic impact on our lives. Today I’m thankful for the beta blockers and for my close proximity to our cardiologists. Dalena has cardiology check-ups every six months and Mallory and I go annually.
I am always making the people in our lives aware of the symptoms: chest pain, shortness of breath, dizziness, fainting and sudden cardiac arrest; however, I really try to focus on being positive! I am so happy to have connected with the CHN, thank you for allowing me to share a tiny piece of our life.