Teen Topics – The Top 10 Things to Remember

It often seems that the “taboo topics” in life aren’t really all that relevant until your teen years. As the seasons change, new milestones come and go.  This can be an especially trying time in the life of an adolescent with congenital heart disease, especially as they are undergoing the major transition of leaving the nest and going off to college, joining the workforce, or just moving far from home.  Dr. Aaron Kay, Director of the Adult Congenital Heart Disease Program at Indiana University Health, has the following Top Ten list to help ease the transition and cap off, for now, PCHA’s Teen Topic Series. This post was originally published in November 2017.

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TOP 10 THINGS TO REMEMBER

  1. Congenital heart disease requires lifelong follow-up
  2. Where operations were completed
  3. How to find a copy of all operative reports as available
  4. The current list of medications, especially blood thinners and rhythm drugs
  5. If they have a pacemaker or not, and a wallet card
  6. Whether they need antibiotics before dentist, tattoos, piercings and similar procedures
  7. What restrictions their heart disease places on activities they can do, and how that can affect jobs, etc
  8. Need to maintain health insurance whenever possible
  9. How to find a new doctor that specializes in their disease if they have to move
  10. Who to call if they have any questions or concerns

 

 

Congenital heart disease requires lifelong follow-up

Many patients with CHD feel well at this point in their life, and in many cases, without significant symptoms, they may not see the need to see a doctor.  There is a common misconception that CHD can be “cured” (which is possible in a very small minority of lesions).  Some conditions require follow-up more frequently than others.  The “transition” period is an especially risky time for teens to become “lost to follow-up” as they may not necessarily know how to make their own appointments, and may not know how to find a new doctor.

Please discuss with your CHD provider who they should be seeing as they become adults, and if your child has not seen a CHD provider in the last 12 months, now would be a great time for a quick visit to make sure your teenager has a better understanding of their specific problem and how frequently they need follow-up.

 

Where operations were completed / How to find a copy of all operative reports as available

Ideally, your family would already have a copy of all the operative notes from all surgeries, catheter procedures, pacemaker implantations, and electrical studies of the heart.  A great deal of time and unnecessary testing can be saved if you have a copy of this information. Knowing the exact history of what was done, who it was done by, where, and when can really help a new CHD physician rapidly identify the best strategies for follow-up for your child.

 

For example, it is very helpful to know what kind of artificial valve, as well as what size of valve, was implanted. It can help a CHD physician understand how long to expect the valve to last without replacement, as well as the likelihood of being able to replace it in the cath lab, versus being able to follow by just echocardiography or cardiac MRI imaging.

 

 


The current list of medications, especially blood thinners and rhythm drugs

If your child is on Coumadin to prevent a blood clot, this is incredibly important information.Likewise, rhythm control drugs are important to know about. Many of these drugs will have interactions with other new drugs that are commonly prescribed, for example, antibiotics.  In some cases it may be important to consider getting a medical bracelet for your loved one in case of emergency.

 

 

If they have a pacemaker or not, and a wallet card

A wallet card identifying the manufacturer of a pacemaker or defibrillator is very important, especially in case of emergency.  You don’t want to think about bad things happening to your loved one, but if they suffer an ICD shock and are temporarily unconscious and unable to speak for themselves, emergency personnel with access to this information will be much better able to care for your child.

Additionally, as patients move, they often will need to have one cardiologist for the “plumbing” and another for the “electrical work.”  It can save a lot of frustration to know ahead of time that a patient requires appointments with both types of cardiologists, and at most centers we can make arrangements for your loved one to see both types of cardiologists at a routine office appointment on the same day

 

 

Whether they need antibiotics before dentist, tattoos, piercings and similar procedures

Although endocarditis is rare, the consequences can be very severe. Guidelines changed about 10 years ago, but overall whether or not a patient needs endocarditis prophylaxis is a matter of clinical judgment. I advise you to ask your physician or other provider about this.

 

 

 

 

What restrictions, if any, their heart disease places on what type of activities they can do, and how that can affect jobs, etc

 

If your child is not allowed to lift heavy weights due to their condition, they should be steered toward a job that does not require heavy physical activity.  For the most part, pediatric cardiologists already do an excellent job with this type of counseling.

 

 

 

Need to maintain health insurance whenever possible

Fortunately, at least for the time being, CHD patients can remain on their parents’ medical insurance until they are 26 years of age.  This policy gives ample time for most patients to have a plan to have insurance for themselves, although there are exceptions.

Without insurance, many preventable things can’t be appropriately prevented.  Virtually no one can afford open-heart surgery, catheter procedures, or other major heart procedures out-of-pocket.  If your family does not have private insurance, please ask your care team for resources to learn how to find alternative insurance through Medicaid or other similar State programs.

 

How to find a new doctor that specializes in their disease if they have to move

A great resource on the web is the Adult Congenital Heart Association clinic directory, which can be found here.  This directory can be searched by each state and virtually every large city currently has a program listed on the website.  Contact information to help you make an appointment is available in this directory.

You should also discuss with your CHD care team which doctor they would recommend.  For some very complicated types of congenital heart disease, it may be important to determine where to move based on the availability of appropriate ACHD resources.  Of course, in many cases, patients may still continue to “go home” during summer and college breaks and continue to see their original cardiologists.

I would recommend that if a young adult with CHD lives more than a few hours away from their hometown that they should have at least a one-time “checking in” appointment at a local hospital or clinic, so that they can have their chart on file in the electronic medical record of the nearby hospital, mainly in case of emergency. Also have a plan in place for where to be transferred in case of a serious medical emergency.

 

Who to call if they have any questions or concerns

This may be the most important issue.  The default option in case of emergency is the emergency department; however in many cases, a timely call to the right person can save an unnecessary trip as well as a lot of frustration. You should discuss with your current CHD care team who to call in the future, and when.  Many routine and unexpected questions are going to occur throughout your child’s life, so knowing who to consider a “medical home” is quite valuable.

 

 

 

 

Dr. Kay is the Director of the Adult Congenital Heart Disease Program at Indiana University/Indiana University Health.  He is trained in both Adult Cardiovascular Medicine and Pediatric Cardiology.  He is originally from Huntington, Indiana and has degrees in Spanish and Engineering from Purdue University.  He completed training in Internal Medicine and Pediatrics at The University of Cincinnati/Cincinnati Children’s Hospital, and completed a dual fellowship in Adult Cardiology and Pediatric Cardiology at Nationwide Children’s Hospital and The Ohio State University.

 

CHD and the Taboo Q&A: Your Questions Answered

 

Today we revisit a couple of Q&A videos that were originally posted in October of 2017. The first video is an interview with Dr. Madsen, on alcohol and drugs. And the second video is an interview with Dr. Gurvitz on college, tattoos, and contraception. 

Dr. Nicolas Madsen – Activity, Drugs & Alcohol, Related Conditions, Staying in Care

Nicolas L. Madsen, MD, MPH joined the Heart Institute at Cincinnati Children’s Hospital Medical Center in July 2012. He is the Vice Chair of PCHA’s Medical Advisory Board.

 

 

 

 

Dr. Michelle Gurvitz –  College, Tattoos, Contraception, and Transition.

Dr. Gurvitz is an assistant professor of pediatrics at Harvard Medical School and a staff cardiologist with the Boston Adult Congenital Heart program at Children’s Hospital Boston and Brigham and Women’s Hospital.

 

 

 

 

 

Thank you, Dr. Madsen, and Dr. Gurvitz, for your willingness to share your knowledge and experience!

 

School Intervention Series: Making A Difference

In the last week in our series on how CHD affects us at school and work, we hear from Kyle Herma, a School Intervention Specialist at Children’s Hospital of Wisconsin. Although this blog was originally posted in the summer of 2017, these tips, tricks, and recommendations for navigating a school’s system as a person who is affected by CHD, is still applicable today. 

 

Twenty nine years ago my sister was born with Hypoplastic Left Heart Syndrome (HLHS), a congenital heart defect that left a chamber of her heart severely underdeveloped at birth. This was at a time when medical technology and surgical repairs options were very limited for a complex baby like my sister. While she ended up losing her battle with HLHS, her short time on Earth ultimately began a battle much greater – the fight to eradicate congenital heart defects completely and in the process, improve medical outcomes and quality of life for those currently affected. Today, the Herma Heart Center (HHC) at Children’s Hospital of Wisconsin is known for having the best published survival rates of HLHS in the world. However, a top recognition like this is not achieved without constant work towards excellence and innovation in all areas of care.

 

Herma Heart Center

How does this relate to PCHA’s “Back-to-School” theme this month? It does on so many levels! Two years ago I was a kindergarten teacher, loving every minute of every day guiding 4 and 5-year-old kids as they discovered their world. I worked in the inner city of Milwaukee at a low-income Charter School – all of my students considered “at-risk” due to a variety of different statistics. Every day I sought to plan lessons that not only were rich in academic content and student engagement, but also focused on building a classroom culture of strong future leaders and community advocates. While I had always been involved with the Herma Heart Center on various levels because of my family’s deep connection, I was a teacher. I loved being a teacher.

In 2015, I got a call. The Cardiac Neurodevelopmental Follow-Up Program, one of the HHC’s leading whole-child focused programs, was expanding and looking to hire a School Intervention Specialist after they noticed a very high need for multifaceted school intervention in students with complex health needs – specifically in the area of pediatric cardiac neurodevelopment. The job requirements outlined a liaison-type service, with the goal of working to ensure clear and consistent communication between the medical staff, the family, and the child’s school at all times. I began researching far and wide. I wanted to learn everything I could about how CHD affects a child’s neurodevelopmental functioning and what type of supports schools have in place to modify for or accommodate these children. All of my searches came up empty! There was nothing. While significant literary research supported that children with complex health needs and chronic illness are at a greater risk of reduced student engagement, higher disruptive behavior, lower academic achievement, an increased exposure to bullying (among many other well-documented negative educational outcomes), structured programs for school re-entry and intervention are rare. In this moment I knew that I needed to step in to fill a role much larger than a classroom teacher.

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The School Intervention Program officially “went live” in February 2015. I began with a very focused population of heart transplant patients that pinpointed those re-integrating into school post-transplant, but also offered intervention services to all of our heart transplant patients no matter how many years post-transplant they were. The response was huge. My patient population quickly grew to all heart transplant patients (including those wait-listed for transplant) and several patients with advanced heart failure who were anticipating a future transplant. My pilot study served 55 cardiac patients, ages 3 (preschool) to 24 (college). The schools’ concerns that were addressed included: attendance and absence support, special education support, attention and behavior plans, and documentation/medical record communication – just to name a few of the big categories. Of those 55 patients, 57% have exited the program with their school concern fully resolved, 36% still receive ongoing school intervention but are making great progress towards their school goals, and 7% transitioned to different medical centers where school intervention could no longer be followed. I’ve witnessed one of my high school student graduate with his class just 5 months post-transplant, I’ve heard from a school nurse that she could not believe a student is finally getting to live a “normal” life without any medical interventions needed during the school day, and I’ve helped a mom send her 7 year old to school for the first time because she finally felt confident the school could handle his needs.

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You may be thinking, “That’s great, but my child did not have a transplant. This doesn’t help us.” I am hear to tell you that it does! By doing a formal pilot study on a small population of patients and proving there is an urgent need for formalized school support, I am establishing both attractive outcomes data and the sustainability of this type of position. I frequently share these outcomes with colleagues, speak about my work at a director and leadership level, and even present at international conferences just so people can see how this seemingly non-medical work is directly related to improved medical outcomes. And guess what? People ARE listening.

 

Herma_Kyle

I am excited to be sharing with you over the next couple weeks to help bridge the very different worlds of medicine and education to ensure that children with complex health needs, specifically CHD, are not falling though the gaps. Here’s to a great school year!

 

Kyle Herma is the School Intervention Specialist serving the Herma Heart Center at Children’s Hospital of Wisconsin. Kyle has been at Children’s since February 2015 conducting a formal pilot study on school intervention and the impact it has on a child’s overall medical outcome and quality of life. Prior to this position, Kyle was a teacher at Milwaukee College Prep’s 38th street campus. In both roles, Kyle has shown her dedication to serving children who are placed at-risk for school failure and ultimate mission to achieve equal access to quality education for all.

Back to School Q&A Panel

This week, we are continuing to learn about how CHD affects people in their work, or at school. This is an interview that was conducted back in August 2017, but is still relevant to our topic today. 

 

Congratulations on the start of a new school year! Please introduce yourself. What grade will you or your child be entering?

Frances: My name is Frances and I volunteer as the blog coordinator for PCHA. My husband and I live in California and have a confidant and outgoing 3-year old daughter who was born with severe mitral valve prolapse and a VSD. She had a very successful open heart surgery at 8 months old. She’ll be starting a couple mornings of preschool this year.

Margaret: Hi! My name is Margaret and I’m a heart mom to an awesome 8-year old heart hero named Kieran who will be starting 2nd grade. We’ve been through Birth to 3, as well as the IEP and 504 Plan process. I am also a parent adviser to our local hospital’s School Intervention Program.

Jack: Hello, my name is Jack Radandt and I was born April 15th, 2001, with Hypoplastic Left Heart Syndrome (HLHS). I had three open heart surgeries by the age of three, Norwood, Glenn, and Fontan. I lived a pretty normal life after my Fontan surgery. I was able to attend school, and even keep up with the other kids my age. At age eleven I experienced heart failure. I went to Children’s Hospital of Wisconsin in Milwaukee and was placed on the transplant list in October of 2012. I then needed to be on a device to bridge me to transplant. In December of 2012, I had surgery for a device called Heartware Ventricular Assist Device or (HVAD). I was the first single ventricle child in the United States to have the device and second in the world. I was also the first single ventricle child in the world to go home on this device. I had the HVAD for five months, until I received a heart transplant on May 20th, 2013.

What are you or is your child looking forward to most this school year?

Jack: I am looking forward to all the speaking events that I was able to get this school year.

Margaret: Kieran says, “gym!”

Frances: Making new friends. She has the ability to make a friend wherever we go, even when we run errands!

Do you notify your or your child’s school or teacher about your or your child’s heart condition? If so, how do you go about doing so?

Margaret: We definitely do. I’ve learned that teachers and school staff really appreciate being informed. Not every CHD student will need an IEP or a 504 plan, but we have both. Each teacher gets documentation about his HLHS, health, and classroom needs. Each year, I’ve met with school staff before school starts to make sure we’re all on the same page and to answer any questions. He has an excellent team at school that is communicative and proactive. We don’t just think about the regular classroom teacher — it is important to have a plan in place so that the school nurse, office staff, lunch and recess supervisors, gym teacher, substitute teachers, and any other school professional who might work with Kieran be informed of his health plan.

Jack: I do notify my school’s faculty, staff, and students of my condition, and I am very open about my scar and surgeries.

Frances: When filling out her general medical information for the preschool, we noted her cardiologist in addition to her pediatrician. She also has a medical device identification card for her annuloplasty ring in case any emergency arises affecting her heart. While our daughter has zero restrictions and no known issues otherwise, we included a copy of this card for the preschool and let the director know about it as a precaution.

Do you or your child have any limitations or require medication during school? If so, how do you handle this?

Margaret: Kieran has HLHS, and his cardiologist has requested that he stay indoors when it is below freezing. On these days, he gets to pick a classmate to stay inside and play board games with. He also has an adaptive PhyEd teacher working with him in gym class. He has a water bottle with him all day to prevent dehydration. For fire drills, he has instructions in his health plan to be allowed to wear a coat outside if it’s cold out. There are certain things we take on a case-by-case basis, such as field trips and walking trips.

Frances: Our daughter doesn’t have any limitations or medications, though the future is uncertain. Her heart will need to be monitored more closely during puberty as her device may be affected during this exponential growth period.

Jack: I don’t have to deal with this because I take my medication right before I go to school and right before I go to bed.

What reaction do you get from the staff if you notify them? Do you feel this affects how the teacher and/or staff interacts with you or your child?

Frances: They thanked me for sharing the information. They haven’t mentioned it otherwise, and from what I’ve seen do not give her any special treatment.

Margaret: We’re fortunate to have a neighborhood school that fosters a very positive learning environment for everyone, and is innovative about classroom adaptations. When we’ve notified them, they’ve responded very positively with a can-do attitude. Last year at our back-to-school meeting, not only did Kieran’s new teacher attended, but the office staff, principal, school nurse, and almost everyone involved with his IEP. Shedding light on the HLHS and the secondary challenges we’ve faced helps them understand how to meet Kieran’s needs better, as well as helps them understand why he (and we as parents) sometimes act the way we do.

Jack: Some of the faculty and staff that find out about my condition feel very uncomfortable about the whole situation.

How open are you or is your child about CHD with peers at school? How does this affect your or your child’s relationships?

Jack: I am very open about my condition. This is my life and I’m not ashamed of it at all.

Frances: Since she’s still very young, our daughter doesn’t make a point to either hide or reveal it. While I want her to be proud of her scar, I also want to allow her to talk about CHD on her own terms whether that’s mentioning it to close friends or being a vocal advocate. She is a naturally confidant and extroverted individual, so it doesn’t seem to bother her when someone points it out or asks. For now we focus on making sure she knows her scar is something good and how to respond in situations. I don’t make any consideration with clothing when it comes to her scar, choosing her outfits based on the weather and her own personal preferences.

Margaret: Kieran is very sensitive about his heart condition. We don’t actively keep it a secret, but we don’t actively volunteer information about it to his peers, either. He feels very strongly that he is a “normal” child and wants to be seen that way. Everyone is different, and I know many heart parents who believe it’s important for their child to be CHD advocates, but I feel it’s important for now for him to feel comfortable at school and have it be a “safe space” for him to feel normal. He sometimes does participate in CHD activities with me outside of school, but he doesn’t understand why they’re important. He sees his “heart friends” as regular friends. Many heart kids don’t truly know the gravity of some of their heart defects until they are much older. A cause that affects him much more, and has for years, is hunger and his desire to see everyone in the world have enough food to eat. I think that’s wonderful. We should all be able to focus on areas of need that spark our sense of fairness.

What is your or your child’s favorite subject or activity?

Frances: She loves the arts – dancing, painting and music.

Jack: My favorite subject in school is biology.

Margaret: Definitely gym. He also likes math and music.

What, if any, concerns do you or your child have in regards to CHD for the school year?

Margaret: My biggest concern is that somewhere, at some point, there might be a breakdown in coordination at school. I worry most when there is a substitute teacher in the classroom, because I’m not informed of it, and I have no idea if they’ve read his 504 plan and understand it. Luckily, he comes into contact with many staff throughout the school day, and I think they all do a good job keeping an eye on him and all the students. Sometimes, because he wants to be seen as “normal” in front of his peers, he’s not as assertive as he should be. I also worry about rough play during recess. This has been a problem at times throughout the past school years, where he will be tackled or otherwise roughed up during normal play, which has resulted in some bruising because he is on blood thinners.

Frances: You would have no idea our daughter has CHD besides her scar, so my only concern is her peers setting her apart in a negative way because of it. Her preschool focuses very much on emotional competence and socialization in a play based setting, and what we liked most about it when touring was how respectful every child was to each other regardless of their differences.

Jack: I am in a very small school so I really have no concerns besides being ill.

If you or your child has a high sensitivity to illness due to CHD, how do you or your child combat this at school?

Frances: While she doesn’t have a high sensitivity, her pediatrician still errs on the side of caution by making sure she gets the first flu vaccination that comes in for the season which we are thankful for. We also chose a preschool with a smaller class size and a strong emphasis on cleanliness.

Jack: I am sick a lot so missing school is always a big concern.

Margaret: The school nurse, or sometimes the classroom teacher, is really great about informing us about illness at school. They will email us personally if a lot of kids are out sick, or with certain communicable illnesses requiring all parents at school to be notified, the school nurse sends home flyers. We are most concerned about things like strep and seasonal flu. If it’s an outbreak the classroom, we would most likely keep him home until it had passed. We use hand sanitizer, get a flu shot, and try to get enough sleep and eat healthy. We emphasize to Kieran the importance of good hygiene. His school has a great custodian and they are good about keeping the classroom and school wiped down.

What is your biggest hope for yourself or your child this school year?

Jack: To remain on honor role and avoid illnesses.

Margaret: That he will make more friends and feel more included socially. Not only is he an only child, but like many CHD kids, especially those with critical heart defects, he is a bit behind for his age socially. Add to that the fact that he easily tires during playground games and has to take breaks during physical activities, he sometimes feels frustrated that he can’t keep up with other kids, especially most of the boys. Of course, I also hope he has a great learning experience this year and finds areas of learning he really loves.

Frances: I hope she will be able to make new friendships and start a solid foundation of a love for learning!

What area(s) is your child most successful at school?

Frances: Since day one, my husband and I have never experienced separation issues with her. She’s very adaptable, confidant and according to her preschool teacher, not at all afraid to ask questions.

Margaret: He a wonderful singer, and is also very creative when it comes to visual art. Last year, he did after school Spanish and book club, which was good for him. He is successful at reading, although he pretends to think it’s “boring.”

Jack: Science class over any other classes.

Thank you all for joining us this past month thoughout the Back to School Series, and best wishes for the new school year! 

Over the Counter Medications

As patients with pre-existing conditions, who may be on a list of prescription medications, it’s important to know which over-the-counter (OTC) medications are safe for CHD patients  Recently at an Adult Congenital Heart Disease Conference, Justina Damiani, Inpatient Cardiology Pharmacist at Lurie Children’s, shared her recommendations for OTC meds, for those everyday illnesses or aches and pains.
*Please be sure to also consult your own doctor and pharmacist before starting or ending any medication.

General OTC Medication Tips

Always look at the active ingredient.
  –  Some brand name products may contain medications that are harmful.
  – Also, watch out for multi-ingredient preparations. Many cough/cold medications contain pseudoephedrine or phenylephrine which, are not recommended.
  – Avoid medications with a “D” at the end of the name.
Always check the appropriate dose
Always check the maximum daily dosage

 

 

OTC Cough and Cold Products

Pseudoephedrine:

Similar to phenylephrine, pseudoephedrine is also commonly used to treat nasal congestion. These products are stored behind the counter at your local pharmacy and require an ID for purchase. If pseudoephedrine is in a combination product it is usually identified as the “D,” for example Mucinex D ® or Claritin D®. The reason the we recommend to avoid use is that pseudoephedrine commonly causes increases in blood pressure, heart palpitations, increases in heart rate and potentially cardiac arrhythmias.

Phenylephrine:

Phenylephrine is commonly used to treat nasal congestion during the common cold. This can be purchased as a single ingredient product or in combination with other medications. If it is a combination product there is usually a “PE” on the label. While this is very effective in treating nasal congestion, it also can cause unsafe increases in blood pressure. Although rare, it can also worsen heart failure or induce cardiac arrhythmias.

 

Ibuprofen (Motrin)/Naproxen (Naprosyn):

Ibuprofen can be safe to use in certain people. Definitely talk to your provider if you are interested in using this for yourself, or your patient. Ibuprofen is often used for pain control and to treat fevers. This drug can be very helpful for both of those indications. The reason it can be considered unsafe is that it can hurt the kidneys. This is more of a concern in patients who already have underlying kidney issues.  This medication is commonly used in patients with cardiac disease unless you have baseline kidney issues or you were told to avoid it by your doctor.

 

 

Heart Safe Cold/Flu Products

 

Coricidin HBP (High Blood Pressure) is recommended for anyone greater than 12 years old.

 

 

 

 

 

The below chart lists the Coricidin HBP products available for  various Cold and Flu symptoms, as well as products suitable for day or nighttime.

 

 

Often, heart patients experience symptoms outside of cold and flu, such as gastrointestinal irritation. Below are some tips on OTC medications for these issues.

 

OTC GI Medications

• Abdominal bloating/gas
– Simethicone
• Constipation
– Miralax
– Senna
– Docusate
– Bisacodyl
• Nausea
– Calcium carbonate (Tums)
– Do not use Pepto-Bismol or any product with bismuth subsalicylate

 

Herbal Products

Another option to give careful consideration is the use of Herbal products. While the category name may sound safe, the product may not always be so.

• What supplements or herbal products should I avoid?
– List may change based on daily prescribed medications
– Ask your physician/advanced nurse practitioner/pharmacist before
starting any herbal products or supplements

 

Medication Optimization Tips

Get to know your pharmacist!
– Is there a local pharmacy that has “down” time or a counseling room?
– Do not be afraid to ask them questions
Use the same pharmacy to fill all your medications
– Occasionally a specialty pharmacy will need to be used
Request refills for prescriptions about 7 days in advance
Remember the new year
– Insurances often change
– Co-pays renew in January
Can you get a 3 month supply near the end of December?
– Prior authorizations may need to be renewed around this time!
Medication storage
– Keep away from direct heat or humidity
• Store OUTSIDE of bathroom
• Away from direct sunlight
– Avoid extreme cold temperatures
– Store in an easy to remember location
– Store medications away from children – locked up?
• Remembering to take your medications
– Take medications same time everyday
– Use pill boxes
– Use an APP on your phone
Medisafe – Pill & Med Reminder
CareZone – App

 

Additional Notes: Tips in case of Medication Changes by Other Providers: 

Keep an updated medication list with you at all times
Ask your pharmacist for counseling on all new medications
Ask your pharmacist to verify or double check your medications if
they look different when you pick them up
Call physician/advanced nurse practitioner for any changes in
cardiac medications by outside providers

 

Final Thoughts

Try to use single-ingredient over-the-counter products
– Ask about proper dose, including maximum dose per day allowed
Ask your pharmacist/physician/nurse practitioner about any new
herbal products
Use medication reminders for taking and reordering
Know when to call physician/nurse practitioners
Get to know your pharmacist!

 

Recommendations courtesy of : Justina Damiani, PharmD, BCPS

Justina Damiani is a clinical pharmacist at Lurie Children’s Hospital and primarily works in the cardiac intensive care unit. She received her PharmD from Purdue University. She completed two years of residency, the first at the University of Georgia followed by a pediatric pharmacy residency the University of Michigan. She enjoys running, yoga, and spending time in warm weather.

 

 

 

 

Father’s Day – A Heart Dad How-To

For Heart Dads new to CHD,  feeling a little lost, or those not sure where they fit in the CHD world, Aaron Carpenter shares what he found, after 8 years on the CHD roller coaster, worked best for him and his family. His tips just might help you too!

I am the dad of two kids, one with complex CHD and one with minor CHD combined with a rare airway disease.  The kids like to say our family is 2 in 100, since every 1 in 100 babies are born with CHD.

Dads generally have two goals when it comes to their family – happiness and health. Heart dads are certainly in that group, but the process of obtaining and maintaining that second goal can be much more complicated.  CHD is your own, often unfair and definitely unwanted, version of normal.  You live by the calendar, whether it is doctor appointments, medication dosing, or interventions and surgeries. I like to say heart dads are regular dads with a lot more to remember.

The voices of heart dads are sort of quiet in the CHD community. If you’re anything like me, you’re an introvert who pays attention to data and isn’t so great with support groups or big picture ideas. I want to be just as involved and just as knowledgeable as the next heart parent, but how do I do that in the world of Facebook groups and blogs? It only took my wife and I eight years on the CHD rollercoaster to come up with a plan, a split of duties so to speak. We are both involved in ways that work to our strengths and preferences. Our sons see both of us doing work in the CHD sphere and we always come together as a family for local CHD activities.

Here are a few of the things I have found helpful along my heart dad journey, helping my kids to be happy and healthy, and somehow finding myself happy and healthy too:

 

Teach a new skill/game/trick

Teaching your kids new things is fun and exciting for them, but it also helps promote a strong bond and connection with you, which is far more important than the rules of any game. I still remember when each of my boys could dribble a basketball, hold scissors the correct way, and especially when they learned to swim. And we are working on shuffling a deck of cards now, along with reading Harry Potter.

 

Compare scars 

It’s important for your child to know that their scars don’t define who they are or who they will become, but it can also be something they have pride in.  Show off any scars you may have so they can see how their own scars might change over time.  When I was 12, I crashed my dirt bike and ended up with an emergency splenectomy and a scar the full length of my abdomen. This provided a great connection with my son after his first surgery and throughout his preschool-aged years.

 

Promote connections

Promote a connection to any other family members who have heart disease, even if it is long-distance. Find other heart kids/teens/young adults so they have role models for different stages of their lives.  Even though we know our kids are 1 in 100, at times they can surely feel like they are the only ones going through this. My father-in-law had open-heart surgery shortly after my youngest son’s Glenn surgery. It was very touching when he removed his shirt so that he could compare his scar to my son’s over video chat. Even though their grandparents live across the country, they are bonded through their hearts with Pops and through gardening with Mimi.

 

Manage medications

Know your child’s medications and dosing schedules inside out, backwards, and on little to no sleep.  Anytime there are changes to the medication schedule, be it sickness or higher doses as they grow, I build an Excel spreadsheet with the new dosing schedule.  It gets printed and taped to the kitchen counter so that I can mark off doses when given. My spreadsheet was super handy during the weeks following my son’s Fontan surgery when meds are given around the clock. Sure there are apps for this, but doing the spreadsheet was my way of being involved and connected.

 

Attend medical appointments

Attend every appointment that you can, no matter how routine it is or how quickly it goes. Each interaction with your child’s clinicians is an opportunity to advance your knowledge on how to best care for you child. I think we all worry about our CHD kids growing up and taking over their own care. Start young and show them what staying in care looks like, which means us dads need to go to our own doctors too!

 

Use your strengths

I am an analytical computer nerd with a passion for physics so I built the medication dosing spreadsheets and dove into the world of cardiology, until I learned everything that I could about my kids’ specific anatomy.  Everyone is talented or passionate about something.  The real trick is figuring out how to channel that energy into something that helps your family or the broader CHD community.

 

Have a strong partnership

This is probably one of the most important things heart dads can do.  The CHD journey is no joke, and having a solid relationship with your spouse, co-parent or significant other is crucial. Divorce rates among parents with medically-needy children are super high for many reasons, not the least is the incredible amount of stress. Whoever said that building a house together was a true test of a marriage, never had kids with CHD. Talk to your partner, listen to your partner, go to counseling separately or together if needed. You are a team.

 

Find other CHD dads

I have met a few other heart dads along this journey, from fatherhood veterans to fatherhood freshmen. There is a common bond between us because of CHD and no other dad has ever understood the gravity of seeing my son’s pink fingers and toes for the first time after Fontan surgery. Sometimes we don’t even talk about CHD or our kids but being together with those who truly get it is validating.

 

Take care of yourself

Let’s face it, the CHD journey is a marathon that never really ends.  If you don’t stop and decompress once in a while you are going to burn out.  Find a hobby. Exercise. See your doctor every year for a physical and actually tell them how you are doing (I am still working at this). Please, take it from my years of doing so, don’t compartmentalize everything until you explode.

 

Get involved with the CHD community

There are a lot of options once you are ready to take this step. Does your state have a PCHA chapter? Do other CHD organizations have a presence in your community? Do you have a skill you can lend to the broader CHD community? Will you send emails or make phone calls to your legislators, asking them to support key CHD measures? Can you visit Washington DC in February for lobby day? Do you want to just go bowling with other heart dads? Do it!

 

Heart dad is a designation that I doubt any of us asked for, but it is a badge we proudly wear. It says that we are fighting the CHD battle right alongside our kids, doing whatever it takes to keep them happy and healthy. Be proud of the work you are doing in your family. Tell people you are a heart dad, advanced dadding required.

 

Aaron Carpenter is the proud Heart Dad of two, a Software Engineer at University of Washington (Go Dawgs!), an alum of North Carolina State University (Go Wolfpack!), and master of the post-op medication spreadsheet. He routinely empties his vacation time bank at children’s hospital visits and enjoys running, hiking, and grilling up a good burger.

Aaron welcomes messages from Heart Dads and science enthusiasts everywhere at amcarp8@gmail.com.

New Diagnosis – Pulse Oximetry

This week we will hear two perspectives on the benefits of pulse ox and the effort to make this non-invasive test a standard screening of newborns. Pulse Ox screening has made it possible to detect CHD in many newborns that would have otherwise gone home undiagnosed. This week’s contributions were provided by Dr. Gerard Martin, a pediatric cardiologist, and Ms. Lisa Wandler, a pediatric nurse, from the  Children’s National Heart Institute and  Dr. Matt Oster, MD, MPH , a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta.

 

 

Dr. Gerard Martin, a pediatric cardiologist, and Ms. Lisa Wandler, a pediatric nurse, from the  Children’s National Heart Institute discuss with us how pulse oximetry screening works , as well as its benefits and limitations. 

Screening infants for Critical Congenital Heart Disease (CCHD) using pulse oximetry is recommended in the United States, but this was not always the case!  As of this summer, all 50 states and the District of Columbia will be screening for CCHD.  In many countries, this important life-saving screen is not yet standard for every newborn.  

How CCHD pulse oximetry screening works:

CCHD screening is simple, painless and takes only a few minutes to perform.  It typically takes place around 24 hours after birth either in the newborn nursery or in the mother’s room.  A sticker with a special light probe is placed on the baby’s right hand and either foot.  The measurement that the light probe takes helps the medical team determine whether the baby may have CCHD and require further assessment and testing.

How it has changed standard screening for newborns:

Prior to the implementation of CCHD screening, as many as 50% of infants with CCHD were being discharged from the hospital without anyone knowing of their heart problem.  Without CCHD screening using pulse oximetry, routine newborn screening could help identify hearing and other rare but serious conditions in babies just after birth but not heart defects.  

Benefits/importance of screening:

An undetected heart problem in a baby can lead to severe health problems for the baby and even death.  This newborn screen has helped to decrease the number of babies lost to undiagnosed heart defects and saved many lives.  The CDC continues to investigate the impact CCHD screening has had in the U.S., but an early estimate is that the number of deaths due to CCHD has gone down by 33% in states requiring this important newborn screen.

Limitations of screening using pulse oximetry:

Screening improves the detection of CCHD, however, not all types of CCHD are able to be detected using this screening method.  It remains important to follow the instructions of a baby’s pediatrician and other doctors as newborn assessment and pre-natal ultrasound remain important other ways CCHD can be identified.

Early symptoms of CCHD can include rapid breathing, difficulty feeding and bluish skin.  If your baby has these symptoms, tell the baby’s doctor.

 

The Critical Congenital Heart Disease Screening Program at Children’s National Heart Institute is composed of Dr. Gerard Martin, a pediatric cardiologist and Ms. Lisa Wandler, a pediatric nurse.  The team at Children’s National has worked on implementation, education and CCHD screening advocacy for over ten years and has provided guidance at the local, state and international levels to those interested in CCHD screening using pulse oximetry.  The team can be contacted at pulseox@cnmc.org

Gerard Martin headshot, cardiologist, children’s national heart institute

 

 

 

Dr. Matt Oster, MD, MPH  discusses the progress brought by pulse ox and the distance yet to go. 

 

In my medical career, I’ve been able to see what was once just a promise or an idea be transformed into reality – the ability to screen well-appearing newborns for critical congenital heart disease. While a baby may appear completely well by anyone who sees her, lurking beneath the surface could be subtle hypoxemia – an abnormally low concentration of oxygen in the blood.- secondary to a congenital heart defect. The application of pulse oximetry to detect such hypoxemia has thus allowed clinicians to detect many previously undiagnosed cases sooner, a change
that has led to decreased infant mortality from critical congenital heart disease.

This dramatic change in the care of newborns did not come easily. It took scientists studying the issue to determine whether this could work. It took policy makers the vision to implement this change in their regions. And, most importantly, it took the tireless advocacy of parents and others to call for, and when needed essentially demand, such change.

The application of pulse oximetry to screen for critical congenital heart disease is a true public health success story. And, it has even seen spillover effects in that many children with hypoxemia due to causes other than heart disease are being detected and treated. However, there is still much work to be done. We need to figure out the best way to implement this screening in special settings such as the neonatal intensive care units, home births, or areas of high altitude. We need to improve the quality of the program so that it is implemented consistently and correctly for all newborn. We need to help public health agencies monitor and track the success of this program.

But the biggest change we need to make is figure out a way to increase the sensitivity of the screening. Yes, the program has detected thousands of babies that may have previously gone undetected. But there are still many newborns with critical congenital heart disease that are being missed, even newborns with hypoplastic left heart syndrome. This is typically due to the fact that hypoxemia may not yet be present. We need improved diagnosed methods beyond pulse oximetry to help detect these children. This is not an easy task, but scientists are indeed working on it. And when it’s ready, we’ll need the help of advocates and policymakers to make it a reality.

 

Dr. Matt Oster, MD, MPH is a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta, and he holds Emory appointments of Associate Professor of Pediatrics in the School of Medicine and Associate Professor of Epidemiology in the School of Public Health. He earned his MD at the University of Pennsylvania School of Medicine and his MPH in epidemiology at Emory University Rollins School of Public Health. After completing residency training in pediatrics at the University of California-San Francisco, he did fellowship training in pediatric cardiology at Emory University. When not seeing patients, he serves as director of the Children’s Cardiac Outcomes Research Program at Sibley Heart Center. His research interests include newborn screening for congenital heart disease, the epidemiology of congenital heart disease, and long-term outcomes for patients with congenital heart disease.

 

 

 

New Diagnosis – Fetal Echo

More often now, than a generation ago, babies born with CHD are being diagnosed prenatally. This week, Dr. Sheetal Patel, from Lurie Children’s Hospital of Chicago and Assistant Professor of Pediatrics, Northwestern University Feinberg School of Medicine, explains the role prenatal echocardiography plays in diagnosing CHD and the benefit of that early diagnosis.   

 

 

Congenital Heart defects (CHDs) are among the most common birth defects, affecting 1 out of every 110 babies born. Each year nearly 40,000 babies are born in the United States with CHD, ranging from simple lesions that may not need any interventions to complex CHDs that can be fatal if appropriate treatment is not provided soon after birth. Research shows that prenatal diagnosis and early detection of these complex CHDs is associated with improved surgical outcomes. With improvement in diagnostic technology with Fetal echocardiogram today, about 70% of complex CHDs are detected prenatally. Goals for detection are targeted at 100%, and we are aiming to reach there with improved awareness and better screening techniques.

A fetal echocardiogram is an ultrasound test performed during pregnancy to evaluate the heart of the unborn child and can be performed as early as 18 weeks gestation. Diagnostic accuracy for detecting complex CHD with a fetal echocardiogram is as high as 95%. It is a non-invasive procedure performed with an ultrasound probe placed over mother’s belly. Generally, the pain or discomfort that results from the probe pressure on the mother is minimal. It involves detailed evaluation of baby’s cardiac structures including cardiac chambers, valves and major blood vessels. It also evaluates fetal heart rate and rhythm. This test can detect CHDs such as missing heart chambers (such as hypoplastic left heart syndrome, hypoplastic right heart syndrome, and many other variations), abnormal great arteries (such as transposition of the great arteries, truncus arteriosus, interrupted aortic arch, etc), abnormal cardiac valves (such as atrioventricular septal defect, pulmonary valve atresia), or large hole between cardiac chambers (such as a large ventricular septal defect). There are limitations of fetal echocardiography that it may not detect minor cardiac valve abnormalities, small holes between cardiac chambers or coarctation of aorta that develops after birth.

Early detection of CHD before baby’s birth has many advantages.

Prenatal diagnosis of CHD allows for necessary preparation to provide highly specialized care that the baby will require soon after the birth and prevents the hemodynamic compromise that can result if this CHD was undetected. This preparation involves coordinated care by multiple teams with expertise in pediatric cardiology, neonatology, pediatric cardiac intensive care and pediatric cardiovascular surgery. In addition, social worker, child life specialists, and palliative care teams may be available to help parents cope with the diagnosis and treatment. An important aspect of early detection is to provide expectant parents the opportunity to have detailed counselling.  This counselling helps parents to better understand their unborn child’s heart condition and interventions that might be needed.  This aids parents in their research to choose a center of excellence for their baby’s care. The goal of this prenatal counselling is for parents to be armed with knowledge, process the information over time, and maximize the family’s preparedness for the journey and transition to a birth of their new baby. Research shows that those mothers who knew about their baby’s heart condition prior to the birth were less anxious once the baby was born as compared to mothers who found out about the defect after baby was born.

There are some standard indications for fetal echocardiography during pregnancy.

Not every expectant mother needs to have a fetal echocardiogram. However, if the risk of having CHD in the unborn child is expected to be higher than general populations, a fetal echocardiogram is indicated. These risk factors include having a prior child with congenital heart defects, maternal diabetes, maternal infections during pregnancy known to affect baby’s heart, etc. Mother should discuss with her obstetrician if a fetal echocardiogram is indicated based on the family history and her own medical history. If indicated, a fetal echocardiogram should be arranged to be performed between 20 to 24 weeks gestation which is an ideal time for accurate diagnosis of CHD. Other indications for fetal echocardiogram include abnormal findings on obstetrical screening test such as increased nuchal thickness, abnormal cardiac images during the level II anatomy scan, chromosomal abnormalities (such as trisomy 21, trisomy 18, trisomy 13, Turner syndrome, etc) detected during the prenatal genetic testing, or other organ malformations noted during the anatomy scan. These abnormal screening tests indicate higher risk of CHD in the fetus and therefore, a fetal echocardiogram is indicated.

What happens after a fetal echocardiogram detects CHD in fetus?

A pediatric cardiologist performing the fetal echocardiogram will discuss the findings of CHD in details with the expectant parents. Tailoring the counselling to the parent’s needs over time is critical as parents can be very overwhelmed during the initial hearing of a diagnosis of CHD. Counselling should include discussion about implications of this CHD on baby during the pregnancy, what support and care this baby would need soon after the birth, what interventions, procedures and surgeries would be necessary during neonatal period, and what other procedures or surgeries would be needed later in life and what is expected overall prognosis with this CHD. The goal of this counselling process is to provide information to parents that would help them with their decision making to choose their options. The options are described in details that include preparing them for this journey to have child with CHD, palliative care or other family planning options.

Each Fetal Cardiac Program have unique set up to provide this detailed counselling. At Lurie Children’s Hospital; these services are provided through our fetal cardiac program at The Chicago Institute for Fetal Health. Following the initial consultation, parents have a “Comprehensive Fetal Cardiac Consultation” which includes a follow up fetal echocardiogram to assess the evolution of the CHD (if any), consultation with a fetal cardiology team composed of a pediatric cardiologist, neonatologist, cardiac intensivist, cardiovascular surgeon, social work, and other specific team pertinent to the diagnosis. For example, a consultation for prenatal diagnosis of HLHS would include a specialist for “Single ventricle Program”. Parents may also choose to meet with “cardiac neurodevelopment team” and “Child life Specialist” if they are interested in learning more about these important aspects of their child’s quality of life in future.  The number of partners at the table during this meeting can seem overwhelming to some parents and modifications are made to this process to meet each family’s needs.  

Our goal as a comprehensive fetal cardiology team is to arm families with information, answer questions, form a united care team to provide cohesive CHD care pre and post-natally, and optimize the chances for the most successful outcome and quality of life throughout a lifetime.  

 

References:

Fetal Echocardiogram: https://www.luriechildrens.org/en-us/care-services/specialties-services/medical-imaging-radiology/diagnosis-services/heart-evaluation-testing/Pages/fetal-echocardiograms.aspx)

CDC: https://www.cdc.gov/ncbddd/heartdefects/data.html

 

Dr. Sheetal Patel is Associate Director of Fetal Cardiac Program at Ann & Robert H Lurie Children’s Hospital of Chicago and Assistant Professor of Pediatrics at Northwestern University Feinberg School of
Medicine. Her clinical interest lies in fetal, neonatal and pediatric cardiology. She is passionate about prenatal diagnosis of congenital heart defects. Her research interests are in evaluating outcomes in
congenital heart defects, with special focus on single ventricle heart defect and Fontan palliation.

New Diagnosis – Prenatal Conquering CHD Kits

This week, our State Chapter Coordinator, Melanie Toth, shares her experience with us as  a new mom finding out her son would be born with a congenital heart defect and how PCHA helps to make sure other families don’t have to go through what Melanie’s did alone.

 

Like many heart families, when diagnosed with their child’s heart defect, your world is flipped upside down. It was no different for our family. I remember us walking out of the room not really understanding or knowing what just happened. We had only walked into the doctors office for our 20 week ultrasound, and left finding out our child would be born with a heart defect, that I couldn’t even pronounce. While I have always had a love and hate relationship with congenital heart disease, over the past decade I have learned a lot. I can’t change the diagnosis we were given that day, but I did wish I could change the information that our family was given when we left the doctors office.

We weren’t aware that CHD was the #1 birth defect or that too many families walked in similar shoes as us. We left feeling hopeless and alone, with a paper that said, “Tetralogy of Fallot”. My husband and I went online to get more information on our son’s diagnosis and, to say the least, were completely overwhelmed. There was different information on various websites, and we just felt more hopeless.

If 10 years ago our doctors would have handed us a PCHA Prenatal Conquering CHD Kit, life would have been just a little easier. From resource cards to navigate important CHD information, to guided questions on what to ask your medical team, and most importantly, how to connect with other heart families, the prenatal kit is like a life raft for new families. Through PCHA State Chapters and working with hospitals, prenatal kits have offered the much needed information and personal connection that every heart family deserves. If 10 years ago our family was given a prenatal kit, instead of walking out with our sons defect written down, our family would not have felt so alone in our heart journey, during a difficult first year with our son’s surgeries.

I feel honored and blessed to help our PCHA State Chapters as National State Chapter Coordinator. Personally helping heart families and helping set up chapters to empower families, is the best pay it forward our family can offer.

 

Melanie’s heart journey began in June 2008, during a routine 20 week ultrasound. She and husband were devastated by the news that their unborn son Luke would be born with a congenital heart defect (Tetralogy of Fallot) and required heart surgery at a week old and again at 9 months old. Feeling very scared and alone during the roller coaster ride of a CHD journey, Melanie decided that no other heart family should feel alone. In 2010, she has started a support group for heart families in Chicago. Working nationally and locally with various CHD organizations. In 2016, she began volunteering with PCHA’s new state chapters, to offer families more than just support. She is currently the State Chapter Coordinator for PCHA National helping to develop state chapters.

 

Recap – Arrhythmias and Cardiac Devices

Complications from surgery, scar tissue left behind, and an underlying disease can bring about arrhythmias in CHD patients and lead to need for a cardiac device. Gathered here are the contributions and resources from PCHA’s Arrhythmia Blog Series. 

 

Jarvik: For Smaller Hearts

PCHA’s next series introduces the various Cardiac Devices involved in the treatment of Congenital Heart Disease and the associated conditions. In the first post, Dr. Adachi tells us about the Jarvik for small hearts, a ventricular assist device used to help pump blood through the body.

 

Terri’s Story

Often times, CHD patients face issues with the rhythm of their hearts. In some patients, this can mean additional treatment is necessary, with either medication or a cardiac device. In this week’s post, Terri Elliott, an adult CHD patient, shares her experience with an arrhythmia that led to her receiving an implantable cardioverter defibrillator. or ICD.

 

The Elephant in the Room

With each CHD, there is no certain path, no one course looks like the next, and complications vary widely. Though a patient may have a specific structural diagnosis, different arrhythmias may develop over time, however patients and families are not always made aware of this possibility immediately. That’s why the diagnosis of an arrhythmia may surprise parents and send them for a loop, just when they think they’ve seen it all.  This also makes it difficult for parents to know just how much to share with teacher and other adults in their children’s lives, just as Alison Connors shares with us this week.

 

Bradycardia Explained (Part One)

This week former Cardiac Nurse, Carol Raimondi, provides us with the first of a two-part resource on arrhythmia and the various diagnosis and treatment. Up first: Bradycardia.

 

Tachycardia Explained (Part Two)