Jill's Journal

Sep 17 marked 5 years since my son had his bicuspid aortic valve and aneurysm repaired. He is now 24. He still has some leakage but things are stable! The black shirt pic is a few weeks before surgery. The rubber ducky shirt is this week from work. Difficult journeys often lead to beautiful destinations.

4 years ago today my then 19 year old son, Sebastian, had his bicuspid aortic
valve repaired. Things are still stable with his repair and aneurysm and we are still thankful for every day of good health.

We are coming up on the one year anniversary of my son’s BAV valve repair surgery. (For those not familiar with Sebastian he was 19 at the time of surgery. We had no idea what a bicuspid valve was before he was diagnosed and he was already in congestive heart failure at that time.) He has been doing pretty well. His heart remodeled nicely (his surgeon’s words) and because he still has some regurgitation (at his 3 month echo his cardiologist says mild to moderate, the surgeon says very mild. His ejection fraction was also at the lower end of normal.) I am nervous for the one year follow up echo. I hope the repair lasts because I don’t want to see him have to go through that again. With the anniversary (Sep 17) coming up I’ve been thinking about it a lot and we had a very tough couple of days at the hospital. I know another surgery is a very real possibility, I just hope that we can get several years in first. The whole surgery came at us so fast I never really had time to process the whole thing. I had hoped with his age that he would rebound to full health. While he does well we’ve had a hard time finding a job for him. He has a fifty pound weight restriction for life due to the aneurysm and the majority of the jobs in our area are physical factory work. He did find one job that wasn’t so physical but they flipped his hours around a lot and required quite a bit of overtime. Schedule changes and his Metoprolol don’t get along well. He just wants to be independent so he gets discouraged and it’s hard for me not to be able to fix it for him. We’ll get through it because, like the rest of you, he is one tough cookie whose will to live outweighed his fear.

BAV folks, Have any of you been tested for Marfan or other connective tissue disorders? Our GP, cardiologist, nor surgeon mentioned anything to us about these disorders and I’m wondering if this is something I should pursue with them. I’m not looking for new health issues for Sebastian but I don’t want to be blindsided again like we were with the aneurysm and severe regurgitation.

Hello all! It's amazing that since Sebastian's surgery on September 17th I have lost count of how many weeks it's been since his BAV and aneurysm repair. His follow up echo 12 weeks post surgery showed a "very mild" regurgitation though his surgeon assured us that was completely normal and nothing to be concerned about. (His cardiologist rated the echo quite differently and I had a week of absolute panic but I have faith in our surgeon and his opinion.) Any heart surgery is a second chance and being able to keep his valve was the right option for him at this stage of his life. Bastian's left ventricle has returned to normal size and his aorta is stable. We are just waiting on the surgeon to review his CT scan one more time to ensure his sternum is stable. He has occasional popping without pain and they want to be sure we aren't dealing with a non union. Overall, Bash is doing very, very well. He passed his GED and is ready to find a job once we get the all clear from the surgeon. He's still living at home and we haven't seen the kid smile this much in years. He is quite clear that he "is never doing that surgery again" and I hope that can be the case for him or it can at least be put off for many years while the advancements continue. It's amazing that 6 months ago I had never heard of a bicuspid aortic valve. Looking back I can now see the things we missed. My kids have always spent a lot of time with my parents and have always regularly taken their blood pressure there. Sebastian would get the irregular heartbeat symbol quite often since he was about 8 or 9 but we didn't think much of it. He went to the doctor all the time and no one ever said anything. I just thought the machine was geared for adults and he got that reading because he was a kid. It also turns out his lack of energy wasn't depression and his insomnia wasn't insomnia. Hindsight right? I don't know that it would have made it any better to have known sooner. It would have made it different, but not necessarily better. This has been an interesting journey for sure and thank you to everyone for all of your kind words and support. He's always going to be a cardiac patient and I'm always going to worry but I know where to find the folks that will talk me off of the ledge!

Sebastian’s surgery was on Sep 17 and he continues to do well. I’m going to call his surgeon on Wednesday to ask but has anyone else still had sternal clicking at the 3 month mark? He had a mini. He doesn’t have any pain with it. He’s not done any cardiac rehab and he hasn’t over exerted himself during recovery. I’m fact, I think he’s been overly cautious. He had a CT with contrast about a month ago and they said everything looked great. Would a CT show problems with his sternum?

I am greatly saddened today to learn of the untimely passing of the nurse practitioner that first heard Sebastian’s murmur and irregular heartbeat and got us on the path to a better and longer life for Sebastian. She was only 34 and I truly feel I owe her for Sebastian’s life. I don’t know what happened but she will be greatly missed.

Sebastian saw the cardiologist on Monday for a post surgery checkup. This should have been a 2 week visit but his cardiologist was out last week so we were delayed. His incision is still healing nicely. One of his four medications was removed (magnesium) and we were instructed to ask the surgeon about two of the other three. He has a little follow up bloodwork next week and an echo was scheduled for December. The cardiologist said see him in a year. I spoke to the surgeon’s office and he can discontinue the mitigare when his 30 day supply is gone but he should continue the beta blocker and baby aspirin for a bit longer. That brings him down to only 2 pills per day. They’d like to schedule a follow up cat scan. I really hope to see a reduction in the size of his left ventricle. While we were in the cardiologist’s office I received the call to schedule my biopsy for the following morning. I am happy to report that I received the results today and my abnormal suspicious mammogram turned out to be benign calcification. I am elated that I’m not looking for a breast cancer web page to join. Some of the insurance EOB’s have started to arrive. We are fortunate that Sebastian is covered on a policy by me and also by his dad. Both policies are through United Healthcare and they still argue over who is the primary provider. His dad’s policy isn’t covering the days in the hospital before the surgery deeming it “medically unnecessary”. I will be appealing that decision as Bash had acute systolic congestive heart failure upon admission. Insurance companies think they rule the world but I’m up for some hoop jumping.

Now that the boy is on the mend it was time to get caught up on my own healthcare. I am proud to announce that Sebastian and I only share a birthday and not bicuspid aortic valves! That was the good part. The downside was I have mild aortic stenosis and two other slightly leaky valves that we are going to monitor so a welcome to the waiting room for me. I wasn’t expecting that report but who is? I also wasn’t expecting an abnormal suspicious mammogram result that now requires biopsy but it is what it is. My optimism is a little tired this year but I’m doing my best to not worry until there’s a confirmation that there is something to worry about. This year has been a little trying so perhaps a large kiss 2018 good bye New Year’s Eve party will be in order!! I think I feel a tingle on my forehead. 💩 ☘️ Just keep swimming. Just keep swimming.

Sebastian continues to do well even though his boredom level is through the roof. Better to be grumpy and healthy than grumpy with a leaky heart and aortic aneurysm right!!!??? There’s been a lot of ups and downs through this journey with the kid and I just saw Marie post that she has the sniffles and she can’t get sick. It’s probably not a good idea for mom the caretaker to get sick either! One week before Sebastian’s consultation I came down with what I call Klingonitis on my forehead. At first I thought it was spider bites but then I feared shingles. I thought oh no, I can’t have something contagious while Bastian is recovering because I can’t get kicked out of the hospital 12 hours from home. My 18 year old daughter loves her brother but couldn’t be the only one allowed to be with him. I was prepared to cut bangs and invest in some hats to hide my mutation! I hotfooted it over to the doc to find out that in times of great stress you can get cold sores on your forehead! Yup, herpes on your forehead!!! Rob, what kind of emoji can we use for that???!!! My forehead is almost totally healed. There are just a few small indentations remaining. I now refer to that as my stress level indicator and I hope I never see those sores again! Since there’s a limitation to how much I can post with a photo I’ll add it separately. Please feel free to laugh away!!!

Today marks one week post surgery. We have been home since Saturday afternoon and things are going well. Sebastian’s pain has been minimal and he says it’s his lower back that is bothering him. He’s only needed Tylenol since his drainage tubes were removed and he hasn’t taken any since yesterday. He’s had some trouble sleeping due to night sweats and some pretty strange dreams but last night was a better night. He’s getting around well and his appetite is returning. I’ve caught him looking around in the fridge several times. The biggest challenge for him right now, other than the microwave being too high for him to reach, is boredom and his loss of independence. He’s used to being on the move and I know he feels stuck here. We did make a short trip today to a local nursery for me to look at mums, and also force him to walk a little, and grabbed some lunch just to get him out of the house. I was thinking today “wow, it’s already been a week!” And he said glumly at dinner “it’s only been a week”. Sigh. It’s all in the perspective isn’t it? I return to work Wednesday but I’ll keep up the “field trips” to help him keep moving mentally in the right direction. I’m pretty dang thankful that his boredom is currently my biggest concern!

Sebastian has had a much better day. He ate a little and has walked around a few times. He looks much sturdier when walking to the bathroom. His oxygen level has been great today so no supplemental oxygen. The wires were removed this morning and the drain tubes came out this afternoon. The only remaining connection is his arm IV port. (Not sure if that is the right terminology but he’s not constantly attached to anything.). His incision bandage was removed today and the incision looks great. He had a mini so it’s about 4 inches and went right through his tattoo. The tattoo survived and is still perfectly aligned. I think he as pretty happy about that. (19 year old boy priorities!) He is very very tired and is currently napping. He normally sleeps on his side so he is finding this adjustment a nuisance but is getting around it. To his credit, he has not once said anything about regretting his decision and we’ve even seen a few smiles today.

What I failed to factor into this whole equation is that the pain meds don’t really touch his pain. It’s been a long night of him trying to get comfortable and sleep and of course once he slept for longer than 3 minutes they were in to draw blood. I feel pretty helpless and exhausted. He is frustrated, exhausted, and angry. They want him to breathe deeper and keep his heart rate down but when he’s in this constant state of pain it’s pretty much impossible. Yet another professional here said this is standard for his age group. Ugh. I did talk the physician’s assistance into one more dose of dilaudid so he is currently dozing. She is supposed to be in anytime to pull the wires and then the tubes a little later this morning. I sure hope this brings him relief.

I’m not going to lie. This is tough. We were able to see him about an hour after surgery and he was still on the breathing tube. We stayed with him a while but as he started to stir we left for dinner. I am here for him, however, I do have a limit for how much I can handle and couldn’t be around to see the breathing tube removed. We came back after dinner and he was off the tube but it was a pretty miserable site. He was nauseas and wretching. I again had to leave the room and had a short sobby moment in the lounge. They gave him some meds and in about 15 minutes he was settled back down. We got him some socks and his own blanket and pillow and left him to the care of his nurse. I felt absolutely horrible leaving him but we were not allowed to stay in the ICU and honestly I was wiped out. There is a program in Philadelphia that provides a room for out of town people and families having procedures done in and around the city. My daughter and I were placed with a local physician and his wife a couple of miles away. Joan and Larry are angels. They gave us one of the guest rooms in their home and Joan even brought us milk and cookies. It was nice to have a non hospital shower and a real bed. We both slept really well until Bastian texted me that everything hurts and it hurts to breathe. I told him we were in the way and have been here since. He didn’t have a great night. The breathing tube was put back in and he was aware of it for what he described as a few hours before they removed it. He said with it forcing air he felt like he couldn’t breathe at all. They’ve had him in a chair all morning and when we arrived he said his pain level was an 8. His throat hurts from throwing up and the breathing tube and coughing makes him cringe and hide his face. His chest X-ray this morning was good and they have said the neckline, catheter, and groin line can be removed. The drain tube has to stay a little longer but they are going to disconnect it from the machine and just use bulb suction. They gave him torodol about an hour ago and he is now reporting his pain level at 6 and a half. I can tell it’s helps because he’s been more engaged with me and he’s also coughing more. He was avoiding it before. He’s been drinking water and has eaten a few crackers. They want to give him oxy but it tends to upset the stomach. They also want to get him back in bed to remove the neck and groin lines. He’s using his little breathing torture contraption because he has zero interest in having the breathing tube tonight and has told the nurse he is never coming back here! I know he doesn’t feel like it’s getting better but I have seen the improvement in the 2 hours I’ve been here. (Just as a side note 2 different surgeons and the first ICU nurse said that the pain for these young guys is so much worse compared to the older folks. They didn’t really give an explanation but his experience isn’t necessarily representative of what yours will be.)

About an hour ago I received the update from the OR that the valve and aneurysm had been repaired and that Sebastian was off the bypass machine. They were then closing and he’d be moved over to ICU. Overall, after the incision was made, the surgery took about 5 and a half hours. The surgeon met with us a little while ago and said it went well and that there is no longer ANY leakage! My daughter and I have not seen him yet, and honestly I’m a little afraid I’m going to get emotional, but we have turned a HUGE corner today!!

He’s with the anesthesiaologist now and heading to OR. They said they’d call me around 830 with an update. He was in better spirits when they started some medication in the IV and he said this would be like time travel for him. We all got a few hours of sleep last night and tried to keep the mood light. They got him up around 5 to start with the wipes, nose goop, vitals, etc. They let us sit with him in preop and we could tell when they started the meds in the IV. He said it was cold and that his head felt wide and then started chatting more. He had been pretty stoic up to that point.

Note to self: do not go for a walk outside of the hospital at 330am because you will not get back in. I’m sure I could have gotten in through the emergency department but this place is huge. I can only imagine what they would have thought of me walking in there in my pajamas and squinting from the lights. It was a quite nice morning nonetheless. I slept in the car for 3 hours or so and was able to get back in. The consent form was signed this morning and it’s a go for 645am Eastern tomorrow. Sebastian is being very quiet today so far so hopefully the Xanax and Xbox combination will get him through today. I’m waiting to hear where my daughter and I will stay while he is in ICU. There are families in the area that provide a room for $5 or $10. I’m not opposed to staying in the lounge in our floor or in the car. (I have a Ford Flex and with the back seats folded down I have a piece of memory foam. I wasn’t sure how Bash would travel after the surgery so I wanted to have a laying option). We are hanging in there and ready to be in the other side of this!

Sebastian was admitted yesterday afternoon for Monday surgery. I asked about being admitted over the weekend but they said we would have to say he was experiencing symptoms and come through the ER. Sebastian chose to avoid the ER. I would again used the word “resigned” to surgery. He was the most agreeable I’ve seen him during the admission process and meeting the surgeon but he did have a bit of a freak out moment later on. He was sitting in his bed with his shoes on so I was ready for him to bolt. The nurse noticed he was agitated so my daughter and I left the room. As we were standing at the nurses station an alarm went off and they all started to scramble to our room. The nurse waved them off but apparently when your heart rate goes above 135 the staff responds in force. The nurse practitioner talked to him and convinced him to stay and Bash agreed to a Xanax. Today was a little rocky starting because the pulmonary doc told Bash he has the lung capacity of a 43 year old. I wasn’t able to ask if his lung function will improve later. My daughter and I went to a local Target and bought an Xbox so Bash has something to occupy his time. Unlocking the HDMI port on the tv in his room was an almost all day event but I finally got it. It involved a CVS run for a Universal remote and several hours research to find the right hack to get into the tv programming codes!! Our surgeon, Dr. Plestis, seems like a genuinely nice man and the staff here has been very welcoming. The rooms are private and we are able to stay with him. My daughter is working on her college classes, I’m getting ready to read a book and Bastian is setting up the Xbox. I know I’ll still be nervous during surgery but I think I’ve made my peace with this whole thing. The bypass machine used to really scare me and seem unnatural but my thinking has shifted more to isn’t it amazing that they can do that and they do this every day.

Consult was today and we have hit a small delay. Sebastian has picked up a bit of a cold and chest congestion so they have him on an antibiotic. Looks like the earliest now will be next week.

Sebastian’s surgery is scheduled for September 13th. He has chosen to have his valve and aneurysm repaired via mini sternotomy. This morning he had the corotid ultrasound and pulmonary function test. We are still waiting on the insurance approval for the angiogram. The plan is to do it and the bloodwork once we are in Pennsylvania. My kids and I will be leaving Sunday morning and are splitting our drive into 2 days. The consult is Tuesday and then surgery Thursday if all goes as planned.

Sebastian has opted to go with the BAV repair. We’ll be scheduling the consult and surgery with Dr. Plestis tomorrow. He and Dr. Balkhy at the University of Chicago have been working on his case together. When Sebastian was about 10 we went to a local small amusement park. I don’t remember what ride he had been on but he didn’t look happy when the ride ended. It wasn’t an unhappiness because the ride was over. It was clearly that he didn’t enjoy it. I asked what he thought and he confirmed my suspicions and then got right back in line! I said I thought you didn’t like it and he replied that he didn’t but he didn’t want to be scared so he was doing it again. I don’t think he’s going to willingly jump back into line for another heart surgery until he has to but he’s demonstrated bravery before and I’m glad he’s been able to find it again. We aren’t at the finish line yet but it’s gwtting closer!

It’s been a kooky day. I had a fantastic discussion with Dr. Cuiffo this morning. I contacted him Friday for what is now really a 5th opinion and he sent me his cell phone number to chat. He is quite a lovely person and he said he is too Italian not to talk directly to people. I learned a lot from him though I’m not the decision maker here. The first two surgeons I contacted had both recommended, and only recommended, repair given his age. Repair sounds better than replacement to someone new to all of this but wow, once you look further into the details it is a very technical operation. Dr. Cuiffo was very honest with me and concisely laid out the three options available to Sebastian. That was the good part of the day. When I presented Bastian the options he responded with emotion and didn’t even completely listen before he left. This is so hard for him. I have to somehow get through to him that we cannot change that he has this valve but accept it and move forward. Of the three options there isn’t one that’s perfect. They all have their good and bad. His depression speaks for him and he reacts before he thinks. I know he is ready to just be a 19 year old kid again. I’m ready for that too but we aren’t there yet. I’ve got to get him to nail down what is most important to him and he’s going to have to compromise on something. He first wanted what would give him the best chance for no further surgeries. Then it was no sternotomy. Now it’s no blood thinner. I’m trying to hit a moving target and I’m just feeling a little defeated. Anyone else had repair recommended to them? Thoughts about a teenager on Coumadin?

It appears that robotic nor mini thoracotomy are minimally invasive options for my son. I suppose the mini sternotomy is still better than a full and hopefully the boy will come around to the idea. He is going to be very angry and was only accepting of the other surgery options. I have not broken the news to him yet. (It seems I always get my thoughts together here first so thank you for that.) Dr. Plestis explained the surgery to me over the phone today and said we need to do something sooner rather than later. He is very concerned about the enlargement of his left ventricle and said he estimates in 6 months time his heart may not be in good enough condition for the surgery. He also has concern about that ventricle returning to absolute normal function with surgery right away. I spoke with Jane at northwestern again today and since a mini sternotomy is also available there I am going to hopefully sway Bastian into a consultation there since it’s so much closer to home. Wish me luck. I’m going to need it.

The good news is the CT scan went well on Monday. There were no weird reactions to the dye and Sebastian was actually done and back in the lobby before his actual appointment was scheduled. An office that runs ahead of schedule. Huh, weird. The bad news is “prominent aortic root” on the test result report. I’ve not put on my “mom the heart valve researcher” hat yet today but I assume the sentence “the ascending aorta measures up to 3.8 cm in traverse direction” is code for aneurism. We talked a little about this before the CT scan and I told him it’s common with the bicuspid condition. I know it’s also fixable, however, I must admit I am disappointed to have to add something else to his surgery. I’m not so sure he has accepted the surgery. I feel like it’s more appropriate to say he’s resigned himself to it. I haven’t heard if the office called him today to tell him. He’s currently not home and I looked up the result online. I have a feeling this is going to depress him further. I think this would be easier to accept for him if he was having some sort of symptoms. Aside from being tired there’s been nothing odd. That’s wonderful but it’s made it extra hard for him to grasp the what could happens of this condition. The CT scan results will arrive at the surgeon’s office by the end of the week and we’ll go from there. It still sounds like we will be heading to Pennsylvania.

A little pre CT scan bloodwork today for the kid. Sebastian was in good spirits and I was pretty happy I brought us to the right location. Our cardiologist is in Lafayette, Indiana and the IU Arnett hospital is HUGE and spread all over the city. We have now visited 3 locations during this diagnosis journey. A frazzled mom, an angry 19 year old, an hour drive and an hour time change have had mixed results! This crazy train all began with a visit to our family doctor in April for blood in his urine. The NP performed some in office tests and noted a murmur. Based on his other symptoms she feared endocarditis and sent him for an EKG that did not come back with results to write home about. Next came the echo which landed us with an emergency visit to the cardiologist the next day. The cardiologist thought his friend the infectious diseases doc was lonely so he sent us his way for some jabs with needles. Fortunately there was not an infection so he decided he didn’t want to see us anymore. 😄 Reluctantly, Sebastian agreed to the TEE even though he told doc in the first visit there was no way he would do it. The TEE did not go well. The sedation did not work well enough and Bastian was fully aware of what was going on. After the anesthesia things progressed as they should have. That was a long waiting room wait! The nurse did update me a couple of times but our hour and a half procedure took about 5 hours. That day the cardiologist who performed the procedure explained the bicuspid aortic valve to us and rated the leak moderate and said we’d just have to watch it. We were both relieved and Bastian was talking about jobs he was going to apply for now “that he knew he wasn’t going to drop dead”. Our victory was short lived when the office called back the next day to say that his colleagues had reviewed the results and upgraded the leak to severe and recommended surgery. Sebastian was very angry. I’ve never been a 19 year old boy, but I have been 19, and worrying about heart surgery has no place in a teenager’s brain. There’s enough worrying about finding your place in the world. I can certainly understand his anger. He’s starting to come around and talked a little about surgeons on our drive home today. He now believes he can have the surgery without it being traditional open heart. I am a bit befuddled that our cardiologist told us that there were no minimally invasive options available because I asked...more than once! I’ve never been a take the first opinion gal so the research began and I started consulting surgeons. I’ll be calling Northwestern tomorrow because a surgery closer to home would be ideal but we’ll travel if we must. His 18 year old sister (my daughter) and I now have a plan to buy us all matching pajamas for his recovery. It’s silly but it will be our show of solidarity. (Sis has been checked for the condition and does not currently have any sign of a murmur. I DO have a murmur and have an appointment in early September. My EKG and Echo 15 years ago were normal but I’m now in my dreaded 40’s) So next up, CT scan on Monday...and pajama shopping... and a 19 year old boy rolling his eyes because his mom and sister are shopping for matching pajamas! ❤️