I have to admit, for a long time, Derek and I didn't consider ourselves parents (or parents-to be) of a special needs child--mostly because Jordan's needs aren't exactly visible or noticeable. However, these past nine or so months have completely changed our views on the definition of special needs. We've realized that yes, Jet does have special needs, but because they are "hidden" (unless his shirt is off for you to see his "zipper"), they aren't obvious.
Last week, I had a chance to participate on a panel of moms of special needs children. As with most panels, there are many questions to be answered within time constraints, plus many of the questions asked didn't necessarily pertain to our situation. That being said, I wanted to take some time to answer some of the questions (or questions that we've personally been asked), because again, not many people would consider a congenital heart defect or congenital heart disease (CHD) a special need. I hope these questions help you understand at least a little bit of what CHD is all about. Get ready for a crash course on a very small subset of pediatric cardiology.
- What exactly is CHD? How is it diagnosed? What type does Jet have?
CHD stands for Congenital Heart Defects, or Congenital Heart Disease. It's a fancy way of saying your heart is not normal at birth, and needs to either be corrected or have some sort of palliative care plan in place. It's the most common birth defect (approx. 1 in 100) and about 20-25% of those are considered critical (source: CDC) with over 40 varieties of CHD. Some of these defects go undiagnosed for years because they are asymptomatic until middle-late adulthood, some of these can be detected in utero as early as 22 weeks gestation, and yet others are noticeably present at birth. In the U.S., there are, I believe, only 13 or 14 states that require pulse oximetry for diagnostic purposes at birth for critical congenital heart defects (our state being one of them). Any and all kids with a diagnosed CHD will require lifelong care, specialists, tests, and possible surgeries and/or interventions to maintain the functionality of their heart. There is no cure for CHD.
Many heart defects can occur on their own or in a group (Tetralogy of Fallot) and some occur as part of a syndrome (i.e. Down's Syndrome, DiGeorge Syndrome (q22), Williams Syndrome, and Heterotaxy Syndrome). Jordan happens to have Heterotaxy Syndrome. Heterotaxy can come in many forms; and very rarely are two cases alike. Jordan's case is classified as "Heterotaxy/situs inversus, left atrial isomerism, with functional polysplenia." That doesn't include the list of heart defects he was born with (as each child with heterotaxy is different), but it does narrow down specific issues he could face throughout his life. In layman's terms, his official diagnosis means his heart and abdominal organs are mirrored, with his internal organs having a "tilt", and multiple spleens (anywhere from two to six) functioning as one. It also means he will have lifelong followups with cardiologists, pulmonologists, immunologists, and really, just a lot of ologists. If you aren't sure what exactly that means, here's a fun diagram from the Heterotaxy Network that can help explain what your internal organs look like (situs solitus), and what Jet's look like.
Next week, Jordan will be undergoing open heart surgery to further correct a mitral valve leak, which is contributing to his pulmonary hypertension (PAH), which snowballs into lung issues. Ironically and technically, Jordan also is diagnosed with asthma--completely and totally unrelated to his heart condition. This means that when he gets a respiratory virus or infection, not only does the asthma have an effect on his breathing but so does his PAH, which makes his heart work harder as well as his lungs unable to maintain his body's oxygen levels. This causes his oxygen saturations (sats) to drop to unhealthy levels. When your sats drop, your brain doesn't get the oxygen supply it needs, and this can have a long term and permanent physical effect on your body if not corrected. By correcting this now, we hope to alleviate some of the breathing difficulties Jordan has and prevent irreversible and irreparable damage. They'll also be repairing something called "cor triatriatum" which is definitely not spelled like it's pronounced. Basically, they'll be clearing out some tissue that's divided his two atria into three; another irony, since he was born with just one.
In addition, due to his left atrial isomerism, he is at a higher risk for complete heart block and due to results from his most recent Holter monitor plus his unusual tiredness, Jordan's doctors have decided to go ahead and put in a pacemaker now. Because of his age, his pacemaker will be considered "temporary" because the leads will be sewn to the outside of his heart and the actual pacemaker will be placed under his sternum/in his abdominal wall. This mainly because younger children, let's be honest, aren't known for their balance and agility and having it placed there will minimize some risk associated with him falling on it and disrupting the leads (yet we still need to be careful). Once he is older, he will have a permanent pacemaker put in the "normal" place (upper chest muscle) and the leads hopefully put inside of his heart, and then he will only need the batteries changed periodically. Unless medical advancements are made (and they very well could be), Jordan will have this for the rest of his life.
- What are ongoing supports you have in place for your child?
This is an interesting question. Since Jordan's need is physical, his supports tend to be less behaviorally or developmentally focused, meaning it's less about therapies and more about maintenance. Currently, we see our cardiologist every three months, and more often if needed. We also have a pulse oximeter at home to measure his sats if he looks blue (lips, nail beds) or if he is coughing, wheezing, or retracting. We have a nebulizer to administer twice daily lung treatments as well as rescue medicine if he begins wheezing. Due to his asthma (and PAH) we have an asthma plan in place from his pulmonologist. Plus, we have "normal" CHD daily medications, like aspirin, epaned, lasix, etc. Because of Jordan's previous surgery, or for reasons unknown, we do have physical therapies and feeding therapies in place as well to help him catch up to his peers with his gross motor skills.
- What do you most want people to know about your child and your family?
We are normal. We can go to parks, play outside, have playdates, go to church, childcare, and Sunday School. No special treatment is required. But we are not normal. There are times that we can't participate. There are times that when our son starts to cough, we immediately start to worry if this is serious or not. Certain times of the year, we just won't be going out. We are sticklers about certain vaccinations (whoa, that's a can of worms I won't discuss further). We have appointments and therapies in place that we need to schedule around. We deal with anxiety, PTSD, and loneliness, which is not exclusive to CHD parents but also to many parents of any special need.
- How can our family best meet your child's needs in our home during playdates or parties?
This is a great question, really, with a super simple answer. Educate yourself and use common sense. I'm not asking you to take a six week course on first aid, but simply assess the situation, and ask us if special accommodations need to be made. For example, if you're going to take Jordan somewhere where there will be a lot of physical activity, tell us in advance so we can give him a breathing treatment beforehand. If you see him take a particularly hard fall, inform us so we decide if we need to contact our doctor (because of his pacemaker). This next one should be a no-brainer regardless, but wash your hands, wash your hands, wash your hands. And my kid's hands. And your kid's hands. Really, just wash all the hands. And if anyone--anyone--in your home or that has been to your home is sick, was sick, or still has symptoms of being sick within 48 hours of Jordan or our family coming over, please tell us and understand that we may decide to forego the invitation. (Honestly, that last bit is something I feel should be practiced regardless.)
And, just to clarify, when I say sick, I mean any sort of respiratory illness. Obviously, we don't want the stomach flu either, but while a respiratory type illness can make your child uncomfortable and even warrant a visit to prompt care (and yes, "healthy" kids can be hospitalized too, I'm not denying that), to date every respiratory illness has landed Jordan in the emergency room and an overnight stay at our favorite children's hotel, I mean, hospital. I'd take a 24 hour stomach bug every week (gross, not really) compared to watching my son struggle to breathe. And please, please remember, Jordan is not even close to being a "serious" case. There are heart children much, much more at risk for more serious complications due to respiratory infections or viruses, even death. Please, consider them before you take your sick child out and about to play groups, etc.
- How can we help OUR children best understand/be sensitive to YOUR child's needs?
Another great question! We don't know what the future holds for Jordan. And obviously, his friends are also two and three years old. Clearly, none of them care right now. Someday though, they might notice things that are different. Help them understand there's a possibility that Jordan won't be able to play rough or hard. That he might always be a little bit behind physically. Help them understand that this isn't something he is doing on purpose or for attention, especially if he ends up missing school for longer periods of time. Help them understand that Jordan's scar is not weird, or funny looking, but instead be in awe of their friend's bravery and resilience. Teach them how to pray for Jordan (as some of you already are). Teach them empathy, because as Jordan gets older, there may come a time when he struggles to understand his diagnoses and why it happened to him.
- How can your friends best support you during times of struggle with your child?
I'll just list out a bunch of things off the top of my head, in no particular order. Don't forget about us when we are housebound for days or weeks or months due to illness. Text us. Call us. If possible, accompany one of us to a doctor's visit or test, because sometimes it's hard for both Derek and I to go at the same time. Check in to see if anything's changed diagnostically. Meals are welcome when we've spent a day at the hospital or in the Emergency Room, or the day of a hospital discharge. (Even if it's McDonald's.) While the doctors have a very positive outlook on Jordan's lifespan and quality of life, we have no guarantee. These are very real fears, so please, understand them by lending a listening ear--not giving advice or assuming overreaction. Know that our plans can change in an instant. (We're still getting over the fact that we traded in seeing Rogue One for a two night hospital stay at Hotel OSF.) Most importantly, pray for us. We are completely, 100% winging it. Anything and everything we've learned thus far was a crash course as things were happening.
Psalm 139:13-14 says this: "For you formed my inward parts; you knitted me together in my mother's womb. I praise you, for I am fearfully and wonderfully made. Wonderful are you works; my soul knows it very well." While we are all uniquely made by our Father, we can't help but think of Jordan when we read this verse. In fact, we can't help but apply it to any special need. As Jordan's book reads, "He formed you quite perfectly, then said 'I'm done.' I'm happy there's you, because God made just one."