Can you tell me a little bit about your family’s SADS journey?

In utero, our doctors thought that my daughter Madison had heart block, and I was sent to a specialist.

They confirmed that it was not a heart block – she just had a low heart rate. When Madison was delivered, we decided to go with a pediatrician cardiology specialist. He did an EKG and an echo. She had some periods of apnea. So he sent her home on a monitor, so if her heart stopped beating or she stopped breathing for any length of time, the alarm would go off. Before we could even leave the hospital, my husband and my parents had to be trained in CPR, just in case she stopped breathing.

At the age of six, Madison was playing soccer on a hot and humid day. It was always in the back of my mind that something might be wrong with her heart. And as a nurse, you just always think about this kind of thing. She came over to me and said, my chest hurts. So I held her and felt her heart, and I could feel a dysrhythmia. That was on a Wednesday. On Friday, we had a movie night, and Madison curled up in my lap. I had my hands over her chest, and her heart was fine. And then all of a sudden, I felt another dysrhythmia. So I called the pediatrician on Monday, and he said, get her to the office as soon as possible. He did an EKG, and then sent her over to the hospital for a chest X ray and an echocardiogram. And he said, Mandi, I think Madison has Long QT Syndrome. We need to get her to a electrophysiologist.

The EP said her numbers weren’t that high, and that she didn’t have LQTS, and didn’t need any sports restrictions. We followed up with her pediatrician every year for physicals. He did an EKG on her again, and he said, Mandi, I’m just going to tell you if this was my daughter, I would have her on medication. So we set up another appointment with her EP, and it was almost like a divine intervention, because the doctor that we were supposed to see had an emergency, so we went in and we saw his partner. And it was a whole different scenario. He did the EKG and a stress test, and decided she did have LQTS. He gave us a script for genetic testing, Propranolol, and an AED. He told us she couldn’t play sports. At that time – in 2008 – we reached out to the SADS Foundation about genetic testing, and they said insurance didn’t cover it.

What challenges did you face after diagnosis?

I’m a mom on a mission, and it was important for me, as a parent, that children be able to get tested under insurance. So I went in front of Florida legislation to ask why genetic testing wasn’t approved. Now, Blue Cross Blue Shield – who we had at the time – will pay for genetic testing. Then my mission became AEDs. I went in front of legislation and tried to get that approved as well. While that wasn’t as successful as the approval for genetic testing, I did get an AED in the schools Madison attended.

Madison had a cardiac arrest while playing in the front yard at the age of 10. That’s when she got an ICD. But for two years, everywhere Madison went, we carried an AED. It had to be within three minutes of her classroom. I would make sure that the teachers knew how to operate it, what it sounds like, where to put the pads. Every year, I would offer to train her teachers. We opted for Madison not to participate in PE. A few years later, we changed physicians to one recommended by the SADS Foundation. Since she hadn’t had an event, she could participate in non-contact sports. Madison ended up trying out for cheerleading, and she did volleyball at the church.

What advice would you give to other parents who are sending their children to school for the first time?

In the summer, I would write a letter to the principal letting them know that Madison would be coming to them. And I outlined what she would need. Be an advocate for your child – because out of every student population, there are several with medical conditions, and you want there to be a plan in place.

I’d offer to teach up to 10 people CPR for free. The more people that were trained in CPR, the more people that were able to save Madison’s life. I’d tell them about other considerations – that if she became lightheaded or experienced chest pain or shortness of breath with any kind of exercise, that she should be allowed to sit down and be observed by the school nurse. I mentioned that the school should have at least one AED that should be no more than three minutes away from any part of the school and grounds. And then I put that Madison had an ICD and was on beta blockers, and the medications are quite protective, but do not prevent 100% of episodes. I put her activity restrictions  – that contact sports are prohibited, but Madison was allowed to try out for sports such as cheerleading, tennis, volleyball, and golf. I put that she needed to avoid medications that overly stimulate the heart or prolong the QT interval.

How has the SADS Foundation helped you and your family throughout your journey?

They’ve been very supportive of us on our journey. They’ve been very informative, and they have educated us in ways that are paramount to taking care of a child with an arrhythmia condition. I think their new webinars are just phenomenal. I feel indebted to SADS, and I continue to support their mission.

Will was 6 years old when he first experienced a fainting episode. He was walking up the hill with his dad & sister one day from school when he fainted. We couldn’t tell if the kids were making it up & playing a joke on dad while I was at work. The next morning he fainted again. I work for a private practice as a Nurse. Will’s general practitioner (GP) wasn’t taking chances, so I collected him for a cardiograph. She sent it away for formal interpretation.

Monday morning I walked in to work my shift and the doctor called me in to tell me this was serious. The results came back as Brugada Syndrome and the doctor explained how dangerous this could be for Will. The drive back to tell my family and pick up Will to take him to emergency was spent fighting back tears. We waited all day for the cardiograph to be reported on by a specialist at another hospital. Nope. The doctor told us it was alright to go home since the specialist said it was okay. My gut feeling wasn’t good. At work I told Will’s GP I wasn’t feeling confident and she agreed. She referred us to a specialist at Westmead Children’s Hospital. We were all sent for cardiographs.

I had been a Cardiac Nurse for many years; unfortunately I knew what I was looking for on the cardiographs that were handed to me to walk back to the specialist. I felt sick. He promptly informed us William had Brugada Syndrome and so did I! He then referred us to the appropriate specialist to deal with this. Whilst waiting Will developed a fever. We had been told this could cause a fatal heart rhythm in Brugada patients.We were transferred to Westmead hospital via ambulance. During a 2-3 week stay, Will had a myriad of tests & consults. We both have loop recorders now monitoring us remotely. I began informing family about the rate of passing it on, which was 50%. My nephew, now diagnosed, is 14. We don’t know if our other 2 children have this.

Our biggest struggle was educating others, as nobody knew what this was! Not even the doctors and nurses we came across. We had to convince Will’s school to fundraise for a defibrillator because there was no other assistance. We had to approach the education department because the school struggled to understand just how important a defibrillator was in saving our son’s life. Not friends nor family. We then raised $2,500 to buy our own defibrillator so we could at least feel safe.

Each year we have a new teacher to explain what Brugada Syndrome is, and they typically look at me horrified. The play dates & sleepovers he misses because the parents are reluctant. We are understanding and every day is a challenge, but we try to stay positive never knowing what our future holds. We stand on the sidelines hoping all will be ok so he can play soccer in the plight to help him feel normal. The loop recorder makes him feel “different;” he struggles & will see a psychologist next week. We know we are luckier than many with this condition.

Please share in the hope for a cure! We love our Will & we hope for a future with all our wonderful children.

Name: Richard Lamphier
Location: Atlanta, GA  
Occupation: Program Manager Project S.A.V.E of Children’s Healthcare of Atlanta, an affiliate of Project ADAM

Why were you drawn to nursing as a career? 
I had a good friend who passed away. While he was in the hospital, he had 2 male nurses who encouraged me to be a nurse in his honor.

How long have you’ve been in nursing?
35 years. I joined Project S.A.V.E 6 years ago. Before that I worked in hospital cardiac ICU and cath lab.

What first got you interested in cardiac issues and prevention of sudden cardiac death?   
I had a team mate in high school who had a SCA. My coach did CPR on him and I will never forget the desperate look in his face when he was doing CPR. I want to try to make sure no one else has to go through that feeling of helplessness.

What are some of your recent projects and accomplishments?   
We recently passed the Sudden Cardiac Arrest Prevention Act in Georgia – Jeremy Nelson and Nick Blakely Sudden Cardiac Arrest Prevention Act (SB 60).  I’m a member of the Georgia High School Association’s (GHSA) Sports Medicine Advisory Committee. GHSA mandated that all high school coaches in Georgia are required to have CPR/AED training at least every 2 years. We have between 15,000-18,000 coaches in Georgia. The law went into effect July 1, 2019. I’ve been busy training coaches.

Can you tell us about a time when you were personally involved in a life saved?
Outside a hospital- one time in 35 years. It occurred at a health & wellness expo where Project S.A.V.E. had a table. Someone came to table and said “We need your help!” There was a toddler with complex medical condition and had no pulse, and had stopped breathing. I started CPR, with another nurse for 1- 2 minutes (with what seemed like hours!) and then I felt the child’s heart beat return under my hands.

Is there anything you are looking forward to? 
I’m looking forward to helping host the 2019 International SADS Foundation Conference in Atlanta. I’m also looking forward to the impact mandating CPR/AED training for coaches will have.

Is there anything you would like to say to fellow nurses about attending the SADS Conference in Atlanta? 
Some of these conditions are so rare in our pediatric population that it’s a great opportunity to learn about them. It’s important to recognize a SADS condition. Also a great opportunity to interact with patients and families to better understand what can be done in your schools and community. In addition to the healthcare provider program, the Family Conference will have continuing education hours for nurses.

What activities do you enjoy in your free time? 
Atlanta has some great things to do. I’m kind of a foodie. Atlanta has some great restaurants. I enjoy going to local sporting events, Atlanta Botanical Garden , and exploring all of what Georgia has to offer. We have the North Georgia Mountains with over 1,000 mapped waterfalls and the beaches of Southeast Georgia.  

What advice would you give to someone who is interested in getting involved with cardiac advocacy efforts to make their community safer? 
I think the best way to start getting involved with cardiac advocacy is to demonstrate the behavior you want to see in others. My wife and I both have AEDs in our cars, we donate AEDs to the community we live in and teach a lot of CPR/AED. We have a son-Deacon, 6- who knows the basic CPR steps: to call 9-1-1 and get an AED.

From here, I would recommend connecting with likeminded people, who are interested in advancing the awareness of cardiac issues. You could join us at www.choa.org/projectsave  for more information.

I think another great place to connect with is your state nursing association. I am currently the President of the Georgia Nurses Association.  We are always looking for speakers and causes for our members to get behind. I have a goal that all 140,000 nurses in Georgia would teach 5-10 people CPR/AED every year. That would be another ½ to 1 million people ready to respond in a cardiac emergency.

Another suggestion is to get to know your local elected officials- Mayor, city council person, school board member, State Senator and State Representative. Meet with them; let them know you are the content expert when it comes to cardiac issues, including awareness and preparedness.

As a LQT patient-parent as well as an educator-advocate of students facing chronic medical conditions in a large school district, I have learned that “peace of mind” can go a long way in learning to thrive despite medical challenges.  We often feel sidelined by our medical conditions and anything but normal. School plays a crucial role in creating much-needed normalcy for the student/family who may feel life is beyond control when facing these challenges.   

I was very fortunate that my child’s school district recommended a 504 plan to address, not only AED accommodations but also the combination of beta-blocker side effects and unmedicated ADD symptoms with which my son was struggling. However, many LQT families I have had met at SADS Conferences have indicated that they have been told by their school that their child did not need a 504 plan to implement a health care/safety plan including AED access and training.

As an educator, I know that it is a pain to go through the 504 process and much easier just to tell a parent, “No need to worry…We don’t need a 504 plan to provide those accommodations.”  I would recommend parents of LQT students with an AED prescription provide a written request for a 504 plan since an AED is something that the student needs to receive an appropriate education. You are NOT asking too much. 

504 plans are typically considered when a student has an impairment that substantially limits major life activities to help “level the playing field” for that student. Restriction or limitation from many normal school activities due to a potentially life-threatening condition should clearly fit that category. A 504 plan is a legal document (unlike many informal school safety plans) which the school must take seriously. With a 504 plan, a school is legally responsible for purchasing an AED as well as maintaining it and making it publicly accessible (including staff trained to use it during all school activities in which that student participates).

Civil rights are not optional. A child has a right to an evaluation before schools determine if he/she is eligible under Section 504. If refused, the district must provide the parent with notice of procedural rights. At the point, a parent may request a hearing by the district’s Section 504 coordinator and/or, if necessary, follow up with the Office of Civil Rights.   

It’s going to take some work.  You’re going to have to push your body to do things it doesn’t want to do but it will be well worth it to stay in the driver’s seat and not let your condition rob you of a full life!  It’s not going to be easy but you can learn to function and even thrive despite chronic medical conditions through a team approach:  Student, Parent, Medical Provider, School Administrator/Guidance Counselor, School Nurse, Teachers.  Everyone needs to be on the same page.

Pace Yourself.  Moderation is the key:  Don’t underdo it, but don’t overdo it either!  “Underdoing it” leads to withdrawal and isolation.  “Overdoing it” could make you “crash” and then just stay in bed all day.  Give yourself a break when you need one but then go back and tackle the task or goal.  Just don’t stop or you might get stuck!  Whatever the pace, keep moving forward.

Be sure to communicate pertinent medical information. Request to meet with your school team to share medical documentation and discuss most appropriate accommodations and supports available to address these needs in the school setting.  Additionally, a gradual one-to-two week (sooner is better than later) school-reintegration is often helpful in cases of previous extended absence.  Physically entering the building is often half the battle.  A successful school intervention plan includes the student showing up at school and trusting the school staff to implement it.  Since there is no “one-size-fits-all” plan, it may also take a little time to adjust and better individualize it once you have attended school for several consecutive days.  Medical release of information documents can further open up lines of communication between the medical provider and school to tweak the plan, as needed.

Stick to the plan!  Only stay home from school if you have a severe injury or a condition that could jeopardize the safety of yourself or others.  In such cases, your medical team should provide additional information to help your school team determine an alternative plan.  If you don’t push it, you’ll never get past it! 

Develop an extended school support system of appropriate personnel and friends with whom you can share basic information about your condition.  When appropriate (e.g. when you are having a particularly hard time being at school due to your typical struggles), talk about your feelings with these team members.  You don’t have to focus on your physical symptoms (that can make them worse)!

Celebrate Success and don’t look back…except to view how far you have come!