Had our follow up and Linnea's DNA test results are back.

Wednesday, Oct 15th, we headed to St Louis. Kai & Linnea both had echos and saw Dr Sharkey. Linnea’s aortic root has not grown at all, so excellent news! If Linnea’s DNA test comes back positive, then she won’t need this surgery until her aortic root gets to 4.0 cm. Kai’s heart looked fine, but Dr Sharkey didn’t think it was squeezing quite as hard as it did before the surgery, so she did an EKG as well. I believe what she told us was that the electrical signals just weren’t making all the way around his heart like it was supposed to, but that was not anything to worry about, it was still pumping hard enough. They will both go back in 6 months to be checked again. Linnea will have an MRI w/ contrast to check for other dilations/aneurysms. She agreed to do MRI’s instead of CT’s (CT’s have too much radiation and now the kids are old enough to stay still for the amount of time an MRI takes). Both kids will start Cozaar(Losartan). She agreed to write prescriptions so that I could send them to be filled w/ a generic from a Canadian pharmacy (they have generic there and we don’t here. I can get 90 pills for $75 there w/ no insurance cvg and it would cost me $180 here w/ insurance cvg). I asked Dr Sharkey how many Loeys-Dietz patients they see at this clinic and she said there are now 10, so 8 others besides my 2 kids. Not a whole lot!

After the kid’s appointments, we had lunch at the California Pizza Kitchen then we went to go see a movie. Linnea and I wanted to go see Beverly Hills Chihauhau and Kai wanted to see Eagle Eye and Bryan decided to see BHC w/ us. We were all pleased w/ our choice.

Thursday the 16th, we went to get my chest xray first. They of course didn’t have the order for it, so I went to check in at the doctor’s office and then they gave the me the order and I had to go across the hallway and get the xray, then back to the office. I met w/ Dr Moon first. He said I was doing fine, as expected. Everything sounded/looked good. He didn’t have an answer for a lot of my questions, but said those types of things should be directed to the head nurse who would pull my file and try to answer them from there. There was an out patient nurse practitioner, Rita Gardner, who was w/ Dr Moon during our meeting. I will try to summarize my conversation w/ Dr Moon.
1) My aortic root was 4.6cm.
2) Everything in the surgery went as expected.
3) The lady cutting on me was Laura Adams, who is a surgical tech or something, but he said they are the ones who always do the initial cutting on the chest.
4) My body was cooled to about 32 degrees centigrade (89.6 degree Fahrenheit).
5) One of the bypass tubes (to the heart/lung machine) goes in the atrium and the other one goes in the aorta above where the graft goes (I am not exactly sure if it is in the arch, before the arch or what, but I would assume it would be before the arch so that the arteries coming out of the arch would get the blood flow).
6) My heart was not stopped until after the bypass tubes were put in (so I would assume that is how I lost some blood)
7) My heart was stopped w/ chemicals and then when blood started flowing thru it again, it started on its own, no drugs or shocks needed. (that’s cool!)
8) There was no glue or anything on the outside of my incision, just stitches that are just below the surface.
9) The drain tubes were about a 32French which is the equivalent to .96cm, so not quite 1 cm. Seemed a lot bigger than that!
10) I have 8 wires in my breastbone holding it together. I have the xrays showing them.
11) He laughed when I asked him where the top part of my boobs went. He said he didn’t do anything w/ them and had not heard of others going somewhere. Hmmmm, ok, maybe I was just thinking they were bigger 
12) The person doing rounds every morning was Dr Denenlinger (sp?) who is the chief resident. Dr Moon says he came to see me every weekday. I don’t remember seeing him on Thursday or Friday, just Wednesday and the day I left (Monday)
13) He’s never heard of Zanaflex and so does not know of any dangerous interaction w/ Cipro and can not answer why the pharmacy would not have caught that, suggested maybe it is not a known interaction. I told him the pain doctors knew about as soon as they saw the chart and I found the information on the internet.
14) They didn’t know why the ICU staff wouldn’t call Bryan when I asked them to.
15) They told me that in the ICU, there is one nurse for each patient, so there is no reason why I should have been left alone for some long.
16) They didn’t know why anyone would give me meds that is clearly on my allergy bracelet. Even if the doctor ordered it, the nurse should not have given it to me when she checked my allergy bracelet.\
17) The central line was taken out when I left the OR because of the risk of infection. So, I asked if I had to have another surgery, could I request it be left in for administration of meds & drawing of blood and he said I could request it, but more than likely it would be taken out because the risk of infection is too great. (If that were true, then why would the Children’s Hospital leave Kai’s in until the day he left?)
18) When I asked him why the adults in Barnes were treated so differently than the children in the Children’s Hospital, he said “because adults understand more of what is going on and can handle/tolerate pain and situations better than children.” I said, “if they can wait to put all IV’s in a child until after they are asleep, then why can’t they do it w/ adults” he said, “adults can handle having IV’s put in” I explained to him that I was not able to tolerate someone digging around in my veins and if it was really necessary to have IV’s in before the OR, then why would the main anesthesiologist have told the other one to just wait until we get to the OR to put in the IV’s? At one point in time, he actually said, “Well, this is not a vacation, it is a surgery and recovery” and he said it a couple of times. I was shocked at the lack of compassion and lack of understanding of how some very small changes in procedures and treatment of patients could make some huge improvements in patient care.
19) He and Rita both encouraged me to write a letter to Maggie Turner, the head nurse and the one who asked me to fill out a feedback form. The felt like I had valid concerns that someone needed to be able to give me some answers on and make sure it doesn’t happen to someone else.
20) He did go thru the photos and videos and point some things out that we didn’t know what they were.

I cried thru most of the time I was w/ Dr Moon and Rita. I was just so upset. I was really hoping that by talking to them and airing my concerns, I would get the answers I needed and could find some closure in all of this, but it was obvious that that was not going to be the case. Needless to say, when Dr Braverman came in I was still upset and had a hard time talking to him in the beginning. But, unlike Dr Moon, Dr Braverman looked me directly in the eyes and said he was really sorry that I had such a bad experience w/ the surgery. He really seemed genuinely concerned, empathetic, and patient, not in any hurry to leave the room. One thing Dr Braverman said to me was that most people who go in for open heart surgery are very sick, don’t feel good when they go in, so they actually feel better when they get out, but I felt fine when I went in for this preventative surgery, so it was natural that I would feel much worse when I got out of the hospital.

1) But he reassured me that I was doing fine. Everything sounded good.
2) He just wants my blood pressure down further. He would like it to be around 110/70 and it is currently 110-130/80’s. So, he has me taking another 50mg of Cozaar at night as well as in the morning. So, 50 mg Cozaar am & pm and 25 mg of Toporol XL am & pm. If that doesn’t do the trick, then in a week or so, he might have to add a diruectic or increase the Toporol XL.
3) He is certain that as my heart continues to heal, my heart rate will drop some more to a more “normal” level. My maximum exercising heart rate for now should not exceed 120. That won’t take much.
4) He is going to do MRI’s yearly from head to pelvis to check for aneurysms and will see me once a year, but wants to see me again in January, then I am not sure if he wants to see me in April when the kids come down, or not until July which would be 6 months from January.
5) He agreed to fill out the paperwork for the Handicap Plaqard for 3 months so I don’t have to walk as far to get into work for a while.
6) He said I need to start exercising more to help get my body back to where it was prior to the surgery, increase my stamina. Since I can’t do much more walking than what I am doing now (because of my ankle), then I need to get back on my stationary recumbant bike, but I will have to monitor my heart rate.
7) He said they are finding more and more people who have a mutation for a connective tissue syndrome, yet the mutated genes are not expressing themselves (the person appears to be like a carrier instead of being affected, but they are still autosomal dominant genes and they just don’t know why the expression is so different even with the same family). I didn’t ask him how many adults w/ Loeys-Dietz are seen at Barnes/WashU, but it sounds as if more are being diagnosed all the time.
8) Since Loeys-Dietz is not a regulated communicable disease, there is no official “database”. (I think I am going to do some more investigating. I think there needs to be one out there for those who want to be counted)

So, although I came away from the appointments w/ the news that I am doing fine and there is nothing to worry about (just need to get my blood pressure back under control), I didn’t feel the relief that I thought I would.

On Friday, the 17th, Rita Gardner called me. She said she was really concerned about me since I had been so upset during my appt w/ Dr Moon and she wanted me to know that she was really sorry that I had such a bad experience in the hospital and that I should not only write a letter to Maggie Turner, I should write a letter to patient services too, it needed to go higher than the head nurse. She thinks that my questions and concerns are very valid and need to be addressed. She said that she understood my need for closure and wanted to help in any way she could. She said she too was kind of surprised at the “not on vacation” statement by Dr Moon, but said she thought he was just trying to convey that surgeries are never fun. She asked me about my appt w/ Dr Braverman and how I felt after talking w/ him. I explained that I felt like he was genuinely concerned and empathetic and didn’t seem to be faking it at all, then she asked me if I thought Dr Moon was faking it. I told her I thought he tried hard to be genuinely concerned, but didn’t really convince me that he was. We were on the phone for 37 minutes. I was just really impressed w/ her compassion and interest in helping find some answers and closure. I knew she was genuinely concerned. It’s too bad the hospital doesn’t have more people like her employed there. She is truly concerned about improving patient care and satisfaction and is willing to go the extra mile to do what she has to to achieve it. I felt so much better after talking to her.

Not too long before her call, I received a call from Mary at Dr Pegram’s office. Linnea’s DNA test came back positive. It didn’t upset me too much, because I knew it was going to come back positive. At least now I know that when her aortic root nears the 4.0 cm mark, then we need to start thinking about surgery for her. Hopefully, we will have a while. Kai’s test results have not come back yet, I suspect because the lab is waiting to hear from BCBS since it is Kai’s primary insurance. Well, we know it too will come back positive.

Friday night, I went out w/ Stacey, Phillip, Shauna and some of Phillip’s friends. It was so nice to be out w/ my peeps! It made me almost feel normal again. It was great! I can’t wait to get back to work, but I have to get my blood pressure controlled and increase my stamina a bit before that happens. Rita faxed over my release form stating that I could start working from home for 4 hours a day for 2 weeks starting November 3rd (thru the 14th), then go back to working at work full time November 17th. That will be 10 weeks, so my breastbone should be pretty healed by then. I am excited about going back to work, I am just paranoid about going back to my pre-surgery rate which is getting from point A to point B in a good time. I notice that it doesn’t take long for my chest to start hurting if I get to walking too fast. I don’t want to overdo it and then start hurting and freak out the people around me. But, I want to jump back in, hit the ground running, and come out swinging!

Oh, last weekend was when Chris (Sloan’s husband) ran the Ford Ironman World Championship Tri-athelon in Kona, Hawaii. I tracked him all day long on the computer. The winner finished just over 8 hours and Bryan and I were watching everyone cross the finish line. I texted Sloan a couple of times to see how he was holding up. She said he was really hurting by the time it came to the running portion. Well, after watching for more than 9 hours, about a minute or less before Chris crossed the finish line, my computer froze up (or it was our fabulous Comcast service) and by the time I got it back up, he had already crossed. So, I missed him crossing the finish line! I was so pissed!!!!!! I really wanted to see that. I am hoping Chris will have purchased the DVD and I hope it shows all the finishers. He finished in just over 11 hours 17 minutes I think. He didn’t make one of his goals to finish under 10 hours, but I still think it is an amazing accomplishment to finish it at all. I don’t think I could do as much as he did in my lifetime let alone in 11 hours!

This week, I go to Dr Rink, my physiatrist, to see what he can do for my neck and back. He is the one who was giving me the trigger point injections in my back and neck before the surgery. I did talk to his nurse a while back and she said he reviewed the MRI I had again and said I do not have the Chiarri malformation, but I am still not convinced since it was taken in the flexed and extended position, but I am hoping he will have some ideas on how to stop the pain in my neck and back. I think I am also going to call Dr Hurford’s office and see if he will see me when he sees Kai and Linnea on the 31st. Someone has got to figure out a way to get rid of this nearly constant pain in my neck and back. I just can not accept having this chronic pain for the rest of my life. It scares me to think that both my kids’ backs are more curved than mine and it makes me wonder if they will have worse pain than I do as they get older. I certainly hope not. It is just so hard to function w/ chronic pain.

Kai and Linnea both have their yearly check up w/ the pediatrician on the 29th. That is when we address the “early puberty” issue concerning Linnea. Keep your fingers crossed that she is not going thru that yet. They will both get their flu shots then too. Oh, that will make them happy, NOT!!! I need to get mine scheduled as well as my mammogram (I got a reminder in the mail today)

Oh, Linnea bowled today. Her average going in was 40, her first game was 44 and her second game was 77, so pretty good today. She just might get bowler of the week next week w/ those scores!

Ok, enough for now. Time to go to bed. Happy Birthday to my cousin Bret tomorrow (19th)! Ha! He’ll be 40!!!!