Growing up, we always heard there was a family history of "heart issues" and left it at that. I believed diet and exercise would be enough to keep myself healthy. In 2022 my cousin Leah suffered an aortic dissection. She is an amazing person and we are thankful that doctors were able to bring her through that emergency situation. Aneurysms took my cousin (25), aunt (36), grandmother (49), and great-grandfather (43) all at young ages. We are devastated that Leah had to go through this, but one positive result came from it. She went through genetic testing (something that should have been recommended by one of our doctors long ago with this family history). The result was that the gene for Loeys-Dietz syndrome was discovered and she passed that information on to the rest of us so we could get tested as well. I’m sad to say that a few weeks ago Leah passed away from a brain aneurysm. Much like me, she had been told she had areas in her brain she needed to watch, but “nothing concerning yet.” This is the difficulty with LDS. Without knowing measurements of other family members that have passed away, it is impossible to know exactly when an artery might give. Everything must be done with caution so that preemptive measures can be taken. Because of the information we were given, myself and all the kids were tested. Everyone one of us tested positive. For all 7 kids to be positive is a statistical improbability (0.78% chance) as doctors keep pointing out. The average life expectancy for people with LDS is 37 years. Getting this information from Leah could potentially add decades to the lives of myself and all 7 kids. Loeys-Dietz is a connective tissue disorder that affects all parts of the body. For years I have been asking my doctors if there was anything that tied all of my different issues together and no one could say yes. This does. Everything from allergies, asthma, autoimmune issues, arthritis, chest pains and headaches can possibly be connected to this. The biggest fear in LDS is the life-threatening possibility of aneurysms in the aorta or arteries in the brain. Because of this, all of us will have to do MRI testing every 6-12 months for life depending on the results. Every one of them showed aortic dimensions on the higher side of normal, with three of them getting into that concerning range. All of them will need to take blood pressure medicine for life to hopefully slow down the growth of the arteries and delay any potential aneurysms (kicking the can down the road as the doctor put it). This is life-changing. They will always have to be conscious of the activities they do (no contact sports, no excessive exercise, no roller coasters, and even being mindful when it comes to roughhousing to name a few). The first immediate concern was with Cailyn's pregnancy and delivery. A lot of monitoring and testing and anxiety went into that and prayers were answered that both Cailyn and Lance came out of that healthy and well. In fact, there was no other way that her delivery could have gone better. As for me, the reality is that had I not known about this, there is a good chance I would have a dissection in the next few years. My tests have shown that my aorta is enlarged beyond what they are comfortable with for LDS patients. On March 19th I will be having a surgery to replace my aorta and a portion of the carotid arteries leading into my neck. This is to avoid waiting around for the very likely scenario of having a dissection and emergency surgery. It is an open heart surgery and I will miss the rest of the school year at work with 12 weeks of recovery time expected. In addition to the aorta, my MRIs have found some concerning results in the arteries in my neck and brain. I am in the process of getting an appointment with a neurologist to determine what treatment, if any, will be needed to treat this issue. One of the biggest issues we face is the relative lack of knowledge in the medical field about this disorder. We have had multiple ER visits where we had to educate the doctors about this. Many doctors I have seen do not know what this is or what to do about it. We have a good cardiologist at Children's Hospital who has been helpful in getting us letters we can take with us places to help them follow protocols with all of us. Mostly I wanted to update those I have not had the chance to speak with yet. This is definitely a stressful thing, but as we gain knowledge and get plans in place for dealing with it all, we can begin to breathe a little. The biggest prayer we have right now is that this surgery will be successful and without complications. Following the surgery, we pray that we will have the right doctors and can make the best plans for further treatment and monitoring. Naturally, we face a great deal of stress and anxiety on a daily basis and as each kid gets older and truly understands what this means and what impact it is going to have on them, our prayer is that they can all handle it well. A few people have asked for resources/info on this so here are some links. Or if you are bored or curious this tells you all the areas that can be affected by this A few people have asked for resources/info on this so here are some links. Or if you are bored or curious this tells you all the areas that can be affected by this: https://static1.squarespace.com/.../LDSF_HeadToToe_2012-1... And Loeys-Dietz Foundation has a lot of information: https://www.loeysdietz.org/en/resources