By: Pretty Momma
Imagine yourself as the parent of a child. Your hopes and dreams take shape as you see them grow and conquer their fears. You feel success from their successes and when they fall your heart aches to console them. Imagine yourself the parent of a young teenager. You see the frustration in them on a daily basis, trying desperately to cope with expectations, stress, and pressure in a rapidly changing environment, while they themselves are racing to grow into their own shoes. Imagine yourself as a proud, attentive mother of an athletic teenage son nearing the prime of his life. One morning you find that your son has slept in. You call and knock at his door to no response. You open the door, and panic, finding the son you have raised and nurtured, cared for, and disciplined lying lifeless and cold; a result of Sudden Arrhythmia Death Syndrome. Heart conditions such as Long QT Syndrome need to be advocated more for patient education to raise awareness in preventing such sudden deaths.
Long QT syndrome is a condition that affects the heart's rhythm that can cause fast and chaotic heartbeats. The heart can only beat erratically for so long before it causes sudden death. Prolonged QT, “LQTS” can be an inherited dormant error in a person’s genetic code or can be acquired due to certain medications and medical conditions. Inherited LQTS has been associated with 17 genes so far, with hundreds of mutations. Although genetic test can identify those with this disease, it does not pick up 20% of people who do have the disease. A known parent with LQTS has a 50% chance of passing the gene down to their child. LQTS may also be acquired. Acquired LQTS can be caused by certain medications of medical conditions. There are more than 75 medications that are known to lengthen the QT interval causing drug induced Long QT Syndrome (Mayo).
Often LQTS is discovered by accident by being picked up on an ECG. An ECG measures the electrical impulse as they travel through the heart. Long QT syndrome results from abnormalities in the heart’s electrical charging system (Mayo). Imagine each heart muscle has tiny holes called ion
channels. These holes open and close to allow important substances (sodium, calcium and potassium) to flow in and out of the heart muscle cells. In LQTS the holes in the cells that allow these important substances either do not properly open and close, or there is not enough holes to allow for the proper amount of substances or more through the cell to create the heart’s electrical activity (NHLBI).
A person with LQTS may not experience any symptoms and may be diagnosed posthumously. When diagnosis does occur it is usually after a person has some type of event such as: unexplained fainting, drowning or near-drowning (due to fainting while swimming) or unexplained cardiac arrest (NHLBI). This could cause a patient to seek diagnosis; however the first symptom could be the only symptom as LQTS can cause sudden death.
Treatments include education for awareness, lifestyle changes and medications. Lifestyle changes include avoiding medications that could cause prolonged QT intervals, staying well hydrated during illness, reducing loud or startling noises (including alarm clocks), staying away from situations that make you angry or excited, and avoiding prolonged strenuous activity (especially swimming). The most common medication class used for treatment is beta blockers. These drugs slow the heart rate and work by blunting the way the heart reacts to adrenaline, which can cause the heart to beat faster in times of fear, stress and/or exertion (Mayo). LQTS cannot be cured but these treatments can help to prevent these dangerous arrhythmias.
Long QT syndrome is an under-diagnosed disorder. The prevalence of LQTS is difficult to estimate. It may be expected to occur in 1 in 10,000 individuals. LQTS is more prevalent in female patients and usually presents in childhood, adolescence and early adulthood. Sudden death occurs more in boys than girls. LQTS is thought to cause about 4,000 deaths in the United States each year (Sovari).
To reduce the cases of sudden death by LQTS, first degree family members should be offered genetic testing, clinical evaluation, and treatment with the ultimate goal to prevent sudden death (Statton); this will identify those who have the genetic code for LQTS so they may take preventative measures to reduce their chance of this deadly arrhythmia. Raising awareness of this disease is paramount for individuals to gain the knowledge they need in order to watch for the tell-tale signs and symptoms in themselves and their family members. Once this disease is well known we can start to identify those who carry the gene, and we can create personalized medicine for each individual. This would help reduce the number of children suddenly dying at the hands of this disease.
References
Mayo Clinic Staff. (2015, October 27). Long QT syndrome. Retrieved February 10, 2016, from <http://www.mayoclinic.org/diseases-conditions/long-qt-syndrome/basics/definition/con-20025388>
NHLBI. (2011, September 21). Long QT Syndrome. Retrieved February 10, 2016, from
<https://www.nhlbi.nih.gov/health/health-topics/topics/qt>
Sovari, A. A., MD. (2015, December 31). Long QT Syndrome. Retrieved February 10, 2016, from <http://emedicine.medscape.com/article/157826-overview>
Statton, EL, IM Weston, K. Cederquist, J. Jonasson, BA Jonasson, S. Mörner, A. Norberg, P. Krantz, and A. Wisten. "Genetic Screening in Sudden Cardiac Death in the Young Can save Future Lives." Int J Legal Med (2015): 59-66. Jan. 2016. Web. 10 Feb. 2016. <http://www.ncbi.nlm.nih.gov/pubmed/26228265>
Imagine yourself as the parent of a child. Your hopes and dreams take shape as you see them grow and conquer their fears. You feel success from their successes and when they fall your heart aches to console them. Imagine yourself the parent of a young teenager. You see the frustration in them on a daily basis, trying desperately to cope with expectations, stress, and pressure in a rapidly changing environment, while they themselves are racing to grow into their own shoes. Imagine yourself as a proud, attentive mother of an athletic teenage son nearing the prime of his life. One morning you find that your son has slept in. You call and knock at his door to no response. You open the door, and panic, finding the son you have raised and nurtured, cared for, and disciplined lying lifeless and cold; a result of Sudden Arrhythmia Death Syndrome. Heart conditions such as Long QT Syndrome need to be advocated more for patient education to raise awareness in preventing such sudden deaths.
Long QT syndrome is a condition that affects the heart's rhythm that can cause fast and chaotic heartbeats. The heart can only beat erratically for so long before it causes sudden death. Prolonged QT, “LQTS” can be an inherited dormant error in a person’s genetic code or can be acquired due to certain medications and medical conditions. Inherited LQTS has been associated with 17 genes so far, with hundreds of mutations. Although genetic test can identify those with this disease, it does not pick up 20% of people who do have the disease. A known parent with LQTS has a 50% chance of passing the gene down to their child. LQTS may also be acquired. Acquired LQTS can be caused by certain medications of medical conditions. There are more than 75 medications that are known to lengthen the QT interval causing drug induced Long QT Syndrome (Mayo).
Often LQTS is discovered by accident by being picked up on an ECG. An ECG measures the electrical impulse as they travel through the heart. Long QT syndrome results from abnormalities in the heart’s electrical charging system (Mayo). Imagine each heart muscle has tiny holes called ion
channels. These holes open and close to allow important substances (sodium, calcium and potassium) to flow in and out of the heart muscle cells. In LQTS the holes in the cells that allow these important substances either do not properly open and close, or there is not enough holes to allow for the proper amount of substances or more through the cell to create the heart’s electrical activity (NHLBI).
A person with LQTS may not experience any symptoms and may be diagnosed posthumously. When diagnosis does occur it is usually after a person has some type of event such as: unexplained fainting, drowning or near-drowning (due to fainting while swimming) or unexplained cardiac arrest (NHLBI). This could cause a patient to seek diagnosis; however the first symptom could be the only symptom as LQTS can cause sudden death.
Treatments include education for awareness, lifestyle changes and medications. Lifestyle changes include avoiding medications that could cause prolonged QT intervals, staying well hydrated during illness, reducing loud or startling noises (including alarm clocks), staying away from situations that make you angry or excited, and avoiding prolonged strenuous activity (especially swimming). The most common medication class used for treatment is beta blockers. These drugs slow the heart rate and work by blunting the way the heart reacts to adrenaline, which can cause the heart to beat faster in times of fear, stress and/or exertion (Mayo). LQTS cannot be cured but these treatments can help to prevent these dangerous arrhythmias.
Long QT syndrome is an under-diagnosed disorder. The prevalence of LQTS is difficult to estimate. It may be expected to occur in 1 in 10,000 individuals. LQTS is more prevalent in female patients and usually presents in childhood, adolescence and early adulthood. Sudden death occurs more in boys than girls. LQTS is thought to cause about 4,000 deaths in the United States each year (Sovari).
To reduce the cases of sudden death by LQTS, first degree family members should be offered genetic testing, clinical evaluation, and treatment with the ultimate goal to prevent sudden death (Statton); this will identify those who have the genetic code for LQTS so they may take preventative measures to reduce their chance of this deadly arrhythmia. Raising awareness of this disease is paramount for individuals to gain the knowledge they need in order to watch for the tell-tale signs and symptoms in themselves and their family members. Once this disease is well known we can start to identify those who carry the gene, and we can create personalized medicine for each individual. This would help reduce the number of children suddenly dying at the hands of this disease.
References
Mayo Clinic Staff. (2015, October 27). Long QT syndrome. Retrieved February 10, 2016, from <http://www.mayoclinic.org/diseases-conditions/long-qt-syndrome/basics/definition/con-20025388>
NHLBI. (2011, September 21). Long QT Syndrome. Retrieved February 10, 2016, from
<https://www.nhlbi.nih.gov/health/health-topics/topics/qt>
Sovari, A. A., MD. (2015, December 31). Long QT Syndrome. Retrieved February 10, 2016, from <http://emedicine.medscape.com/article/157826-overview>
Statton, EL, IM Weston, K. Cederquist, J. Jonasson, BA Jonasson, S. Mörner, A. Norberg, P. Krantz, and A. Wisten. "Genetic Screening in Sudden Cardiac Death in the Young Can save Future Lives." Int J Legal Med (2015): 59-66. Jan. 2016. Web. 10 Feb. 2016. <http://www.ncbi.nlm.nih.gov/pubmed/26228265>
Pretty Momma -
ReplyDeleteThank you for writing an article about this subject. I also agree that LQTS should be advocated more to raise awareness. Several years ago, a good friend of mine discovered he has Long QT Syndrome after finding himself being airlifted from our college campus to the nearest metropolitan hospital because he suffered from acute complications during a workout. Ultimately, he survived his heart attack, had a pacemaker implanted, no longer trains for triathalons, and learned that his LQTS was inherited and that his younger sister also has it after having all his siblings undergo testing.
I enjoyed your introduction. It was dramatic and captivating – it was a successful hook to engage readers. I also like your explanation of the mechanism behind LQTS. However, the only thing that I would suggest adding is a few more sentences at the end of that paragraph (third paragraph) that explains the specific consequences of disrupted electrical conductivity. Also, since this article is directed towards the general public, I would recommend defining several technical terms, such as arrhythmia and posthumously. Once again, this was a well-written article and the only lingering question I have is a much broader question than the assignment entailed – what are some of the complications and consequences LQTS poses with other diseases and conditions? Are those with LQTS at a higher risk for developing other diseases or are they less susceptible to developing other disease (such as those with sickle cell anemia being less susceptible to malaria)?
Sincerely,
CoconutDreams
CoconutDreams,
DeleteThank you for your comment. I’m happy to hear that your friend was able to be treated in such a scary event.
As for your questions. I didn’t find any information regarding LQTS causing issues for other conditions mostly due to the fact that as long as the heart is not in an arrhythmia it is functioning.
On a side note I did read an article regarding a link between autoimmune diseases (lupus, RA, connective tissue diseases) and LQTS. The link seems to be the autoimmune disease causing LQTS (not the other way around). The idea being behind antibodies in patients with some of these autoimmune diseases clocking specific cardiac channels which causes LQTS. (I looked for a link between LQTS and Type 1 diabetes because my husband has both, and was unable to find any.
Here is the link to the article in case you want additional information.
http://www.news-medical.net/news/20150707/Researchers-identify-link-between-autoimmune-diseases-medications-and-Long-QT-syndrome.aspx
Thanks
-PrettyMamma
P.S. I have printed this comment like 3-4 times but it seems to keep dissappearing. So I hope you get this.
BigFudge
ReplyDeleteSo I just want to start off by saying that I love cardiology, especially when it comes to arrhythmias. I myself contemplated writing about arrhythmias, but I felt I didn't possess the writing skills to convey my points. So I am very impressed with how you wrote this, and felt you did just the right amount of detail. You chose your words carefully and it worked great.
In terms of what you could work on, I wish you would’ve included a picture of a sample ECG. It’s pretty minor; all I had to do was do a google image search, but it might’ve helped make clear to me why it doesn’t cause bradycardia. I assumed there’d be more time in between beats, as opposed to merging P and T waves.
I am curious how you found out about this, as well as how commonly it leads to VTach. (as opposed to going from tachycardia straight to VFib)
BigFudge,
DeleteThank you for your comment. You are right, a picture of the EKG would have helped explain things.
I found out about LQTS because my husband is a genetic carrier (he’s not treated because of his type 1 diabetes and luckily has always been asymptomatic, he was tested because his younger brother was worked up for fainting spells as a child) and since we are expecting our first child and they have a 50% chance of inheriting the genetic mutation I want to be informed.
As for your question, I actually couldn’t find a number or percent of how common LQTS leads to VTach and VFib. All the articles I read just went to explain these being the complications of LQTS.
Thanks
-PrettyMamma
Zombieland
ReplyDeleteAwesome paper that is well-written and follows easily. I really like your introduction paragraph. It really grabs the reader and makes me want to finish the article. One thing that might improve it: I wanted to know more about what the "tell-tale" signs and symptoms are that parents could look for. Just curious, would screening every infant at birth with a harmless ECG help in very early diagnosis?
Zombieland,
DeleteThank you for your comment. Yes a routine ECG could be done to pick up the long qt interval in an otherwise possible seemingly healthy child. Also a blood genetic test could be done but would be more costly.
-PrettyMamma
I found your blog post very very interesting. I am a high school athletic coach and knowing sadly how often kids are collapsing everyday from undetected heart conditions I know how important it is that these conditions are caught and treated. I had no idea about long QT syndrome. I have been in the medical field for some time, but had never heard of this. The one area I think you could have included more about was who is at the biggest risk for this syndrome. The one question I have is are athletes more prone to experience this than others? Thanks again for a great post!
ReplyDeleteSeahawks gal,
DeleteThank you for your comment. To answer your question athletes are not really more prone to getting LQTS but the exercise and sports can set off an arrhythmia. Which is why you often seen child in news stories playing sports when they pass out.
Thanks
-PrettyMamma