Info really needed on mechanical valves in peds...

[bridget]

(Posted part of this in a reply to another memeber, sorry too tired to type it all over again)

Hi there. Im new here and just trying to find my way around. My daughter was born w/ a disease that causes tumors in the organs (non cancerous that we know of) and had to have a tumor removed from her LV when she was 4 days old, it was blocking 95% of the LV. While I was pregnant w/ her we got some pretty slim odds that she'd even make it thru the pregnancy, and no doctor would touch her. We finally found one who gave us a 5% chance that if she lived and was able to go to the OR she'd be ok. That surgery damaged her heart pretty bad, created a VSD, MVP and sever damage to the AV. WE THOUGHT things were going good until last year when she went into CHF.. Ah... life took yet another turn. We were given the option of doing a ROSS risky in anyone, let alone a 3 year old. But when they went into her heart they found the PV was too damaged to sustain the function, so our plan B was to do a replacement of the whole valve from the root up.. They used a tissue valve that was supposed to be "perfect" for her. They didnt want to take the chance w/ mechanical valves because she has brain tumors that cause seizures and they didnt want her on blood thinners.. Ok, so we didnt get all the info that we shouldve, couldve etc..We only knew our daughter was dying and we had to do something. So we listened to the "great advice" and proceeded w/ the surgery. Only AFTER the surgery was I able to do more research and thought OMG what have we done. Ive found that tissue valves for anyone younger really isnt a good thing. Hind site thinking back I remembered thru the fog that we were in the surgeon telling me the valve would last MAYBE 1-5 years because a younger person does so much more then a person who's lived their life. 9 mths post op we found that the "perfect valve" is showing signs of stenosis. We just went back to the doctors 3 weeks ago and they are now saying "We mightve messed up" HUH??? Even though the mechanical valves are risky for my daughter they are now thinking that really is the only way to go. So, Im on a quest for lots and lots of knowledge. Please dont belittle me for making what I THOUGHT was the best choice for my daughter. I know things can be heated sometimes when expressing opinions. I need to know what the risk are for a person/child who has never ending seizures to have mechanical valves put in (right this min we are looking to replace the AV again, but will also have to replace the MV at some point). Also the big fear of blood thinners is enough to scare the daylights out of me, but if its our only option then we need to prepare for it right? But what do the thinners do to seizure meds? There are so many things I feel I need to know, I dont even know what to start asking. I only know that this time I will not be handing over my child to yet more of the unknown and I will not make another choice for her unless I know as much as I can about it. I feel like we were blindsided last time, dangle life and death in front of a parent and see which one they choose for their child. One of the worst things to live w/ is feeling you've taken a really bad situation and made it worse all because you love your child so much and dont want to loose them. What do they do for a tissue valve that is narrowing? Not alot of info coming from the doctors at this point, their most recent comment was they were taking it to the cardio board to determine the next action. Can they do something to save the valve so it doesnt have to be replaced so soon? We are a year and 7 days post op.

If you'd like to learn more about my daughter or visit her website www.caringbridge.org/pa/arianna

Thanks for your time
Bridget

 

[Phyllis]

Bridget, my heart goes out to you as it does to all "heart moms". Have read your caringbridge site and you have so much on your plate. I have no wise words for you, but there are lots of heart moms on this forum who have been there and I am sure they will chime in soon. Our prayers and best wishes are with you for a successful resolution and I hope you find some of the answers you are seeking here.

 

[Nancy]

What a terrible situation. It just makes me so sad that a little one has to suffer so.

Sometimes in people with difficult medical problems there are no good answers, just the answers that will be life-saving at the time. If you have no choice at that point, you have to do what is right at that particular moment. A mechanical valve in a young person would have to be replaced too as the child grows and their heart pumping needs increases.

Doctors have difficulties with these decisions as well. As my husband's medical problems became more and more difficult, many doctors told us that there were no right answers, and it was uncharted territory, and all they could do was just inch along hoping they didn't do harm.

You may be seeing some of that too.

This is an excellent place and you will get a lot of support here. Bless you all.

 

[Lynlw]

hi Bridget good seeing you. Honestly, you know my thoughts are alot like Nancy's. I know you've been talking about getting other opinions, and was wonderring if you've been able to get that started. I saw your message In Justin's GB. and Have been trying to find time to ge back to you. IF I'm thinking right, wasn't the thoughts to go with tissue, also related to Arrianna, needed proceddured /sugeries in her brain, and the benefits/risks involved w/ those on coum? as Nancy said, sometimes there just aren't the really no "good " answers, escpecially in kids and adults w/ complex issues. Alot of the discussions here are "easier' for just (and i am NOT saying Just a valve in anyway meaning it is not tough and/or serious) a valve or valve/conduit and not in hearts that have been rebuilt or chances will have had and will continue to need multiple surgeries no matter which valve you choose. (for example Justin just had his 5th, OHS and 5 major centers said the choice of dacron conduit/tissue valve is the bet option for him, even tho that won't last forever) and with coumadin or with out coum, also all the other body part functions play a role int rhe "best' choice, benifit/risk, for example a person with a lot of GI Bleeds might do better w/ the tissue and having to have it replaced , then dealing with the additional risk of GI bleeds w/ coum. where someone who is young without other "issues" mechanical /coum could "win" the benifit/risk gamble.
How did everything go in Ohio w/ her brain things? I checked when we were home last week, but have fallen behind since the 2nd surgery, Lyn

 

[Susan BAV]

...One of the worst things to live w/ is feeling you've taken a really bad situation and made it worse all because you love your child so much and dont want to loose them...

I hear you, but Bridget, you made the best decision you could at the time with the information you had available. And who can say how things might have gone if the docs had used a different valve? You very well may have made the best decision under those circumstances; there's no way of really knowing and hindsight is not always 20/20.

I wanted to welcome you to the site although I don't have any specific knowledge about mechanical valves in peds. When I was 17 and had my first heart surgery, I only recall my doctors talking to me about tissue valves, which they said [then, about 28 years ago] that I would need to replace every 10 years or so at that age. As it turned out, I didn't need a valve replacement until more recently.

Take care and post again.

 

[geebee]

Bridget,

I want to welcome you and say I am glad you found your way here but very sad for the reason. I will add your daughter to my prayers.

You are now armed with more information and the ability to make some choices. Try not to look back because the one thing you wanted to do was accomplished - your daughter is alive. That very fact will allow you to move forward in hope and be more active in the decision making.

You should try to PM our member Gijanet. Her Katie has had 5 surgeries and now has a St. Jude mechanical valve. I am sure she would be happy to pass along the wisdom she has gathered over the years. Also, Emma has a little girl with an ON-X valve. Both children are extremely active and doing well.

Please feel free to ask questions and/or just come on for support. We will help you in any way you need.

 

[Emma]

Hi,
I'm so sorry this is a quick one but I'm just on my way out - I promise I will come back and write more tomorrow to try and ease your mind a bit.

My daughter has had her mechanical valve for over 6 years now (on-x) and just to respond to one point made in a couple of posts...

Chloe had a 25mm on-x valve put in when she was just 18 months old and the hope is that, as that is a adult sized valve she won't need it replacing again.
Of course, you can never tell, but thats what the surgeons and cardiologists think at the moment. All depends on what size valve the surgeon can get in - not all mechanicals need replacing again, even when done as very small children (and Chloe was a VERY small 18 month old at time of surgery with an enlarged heart)

I really will come back to write more about our experiences later.
Hope this helps a teeny bit in the meantime

Love Emma
xxx

 

[WayneGM]

I don't have any knowledge about mechanical valves in peds but I wanted to welcome you to the VR community. Sorry your daughter has to go through this but you've come to the right place for support and information. Best wishes to both of you.

 

[ALCapshaw2]

When it comes to Mechanical Valves, the Standard St. Jude Valves (NOT the latest version) are the GOLD STANDARD for DURABILITY with a 30 year track record to back them up.

When it comes to technical improvements in fluid flow and lower propensity for Clot Formation, the On-X Valves offer great promise. They have been around for 10 years (55,000 implanted) so it's not like they were just invented yesterday. See www.onxvalves.com or contact Catheran Burnett, Manager of Education and Promotion, at [email protected]

'AL Capshaw'

(I have a Standard St. Jude Aortic Valve and have requested an On-X Mitral Valve when the time comes for that valve to be replaced)

 

[susieq14]

Bridget,

I have no words of wisdom for you as this is way out of my league but I am sending you and your dear daughter lots of prayers and positive thoughts. I am so sorry for all she has had to deal with. I hope that you find some help here on the Board and from the doctors.

Godspeed!
Susie

P.S. Please don't beat yourself up for the decisions that you have made in the past. You did all that you could in a difficult situation. You now need to focus your energy on Now and the Future not on the past. Take Care!

 

[Karlynn]

Bridget, just wanted to say that I'm glad you found us. We're here to be your cyber-shoulder. I'll put your daughter in my prayers.

 

[briansmom]

Bridget,
My heart really goes out to you. I understand the "did I do the right thing" thinking. They are testing my son next week to determine if he needs to go on the heart transplant list. I've done a fair amount of questioning my decisions. On the other hand, I agree with Susan, I made the best decision with all the facts available at the time. Hindsight is 20-20, the rest is just out best guesstimate. My best friend actually has a son with the same disease as your daughter. He just turned 18. He is an amazing young man and we love him a lot. He tests out at about a 5-6 year old level in terms of schooling, but I don't know where to classify him socially. He is one of the happiest fun kids that I know. He will never forget a name, nor does he ever forget anything stupid that anyone has done. He knows the words to every song he has ever heard and loves to sing karaoke. His parents were told that he would not live long, but so far so good. He has only 1 small tumor on his heart and it has not caused any noticeable problems.
As for the seizures, I do not know a lot about them. Is the concern that the seizures cause a certain amount of brain trauma themselves or is it the physical thrashing about that is the concern? Please look into the On-X valve. Our docs are pretty happy with an INR of 1.5-2.5 with this valve vs 2.0-3.0 for other mechanical valves so maybe that could minimize the risk. All in all, this situation just completely sucks!! You have no "perfect" options.
You are in my thoughts and prayers. Let us know if there is anything else that we can do.

 

[bridget]

Thank you..

Thank you..

Ive been trying to read more on this site in between taking care of my 2 little terrors (ha, Arianna has a little sister who is 19mths old and she of course is 4, and they are mentally around the terrible 2's! God HELP ME!!!) But just from the few days that Ive been able to read so far has helped me more then what Ive learned on my own in the past few years!

Im not sure what to answer first so here goes..

I really do try not to look back at the choices we made last year, but its hard not to. Knowing that sooner then later she will have to be put thru hell again really isnt easing my mind. Arianna also suffers from PTSD because she woke up during the surgery. They THOUGHT they gave her enough amnesia meds along w/ the narcs in order for her not to feel anything, but because of her other organ involvements they had to error on the side of caution. She told her cardio she hated that room, she could see someones breath, and really hated the music (and even sang some of it).. It wasnt until her surgeon came in that we understood. She looked at him and said "I remember your eyes.. Why did you play that music?" He thought she was joking until she started mimicking opera? music. The color drained out of his face and he said "Oh my God.. Baby girl Im so sorry!" Ha, sorry just doesnt cut it. On top of it her trough levels for her seizure meds were out of wack, guessing it was because for the first time in 4 years her heart was actually working like it was supposed to and they had to do levels 2x a day. That wouldve been good had she NOT ripped out all her lines and told everyone she was done (you'll have to read about that part on her site.. HORRIBLE!)

K, so like Lyn had mentioned *BTW how is Arianna's buddy doing? She wanted to come see him in the hospital, but because she was stuck in OH it wasnt possible.. Will update you on the visit, but short and sweet the brain surgery didnt happen * we've been looking for other opinions because our cardio whom we've had since I was 16 weeks pregnant w/ Arianna decided out of the blue to take a position in GA, leaving us high and dry and full of questions a few months after her OHS. Ive been searching for other cardio's to take his place and frankly Im not getting that cozy comfy feeling that we once had.. It seems like the more doctors we talk to the smaller the odds get. I realize Arianna wont be around forever, but if I can give her a good quality of life vs outliving me then Ill take it. Our latest obstacle was from the cardio we had to see in Cincinnati(thats where all her other treatments are done for Tuberous Sclerosis).He was defently NOT for her having the OHS last year, with the thought of "wait and see" approach. Which in my mind was like saying "Why dont you just give up now?" Once again he started w/ the mumbo jumbo about how she's a complicated case and we should wait to see how her heart progress's.Well we already tried that an almost lost her..He didnt think we shouldve used a tissue valve to begin w/, especially since she is so young, but on the other hand had no other options to give us.

Ive been reading alot about the Cleveland Heart Clinic and also talked to one of the cardios' I work w/ ( I work in a hospital of all things.. isnt that comical!) he deals only w/ adults but even recommended it, but like me didnt know if they take peds. Anyone know? My thought is why not give them a shot? We're already going to OH every 3 months anyway. Ive signed up w/ their website as well to get any and all info I could. But if anyone has any info let me know.

Ahh a heart transplant... I know thats not a end all be all cure, it could pose alot more problems, but in a way it would be a blessing. Only its not in the cards for Arianna. Ive got a pretty strong opinion on this one, but last year it was put to the people who are responsible for saying yeah or nah if they will list people and they said NO because with her diease you never know what organ will be effected and there are too many people out there that they KNOW will actually benifit from it, unlike a question mark who they cant say for sure tomorrow her kidneys wont fail due to the tumors, or she wont come up w/ LAM *another sucky disease that effects the lungs, runs high in females that have TS* yada yada yada.. Her cardio said something a long time ago about "Hopefully by the time we really need to replace the whole heart the plastic heart will be perfected and she will get one". Hmm anyone want to speed the process along any? Ive never heard of it before, but hey Im game.

Ive still got alot to learn about the different mechanical valves, INR and all that,the responses went over my head since they've never been an option for us, but I see that I have to learn more about them now. A few of the reason's they've always said NO to them was because of her seizures, you never know how they will present. For a while she only had complex partials, which in itself is bad, but then they switched to drop seizures *where out of the blue she would just drop to the ground* and then to the dreaded grandma seizures *convulsions* and now we're back to the partials. Arianna is and isnt a surgical canidate to have some of her tumors removed *has over 36, stopped counting and asking after that* there is a big nasty covering 2 areas of her brain that they thought was the cause of her seizures, which was wonderful to hear, take it out and they will be gone right? Nope, not the case. Eventually it will have to come out because it just keeps getting bigger and bigger, but its not where the seizures are coming from. In that aspect they'd rather her not have blood thinners because that could make an already dangerous surgery worse, but if it comes down to her having to have them then what choice is there? Dont you know Ive found at least 100 kids that have TS and dont you know NONE of them have had any of these medical issues/problems? Some of them have had OHS to remove the tumors, but none of them have had the bad fallout that Arianna's had w/ hers. Thats pretty frustrating.

In case you havent noticed Arianna is kind of like Pandora's box, open one part and you get more puzzles.. When a doctor looks at me and tells me " I dont know" I trust that he really doesnt know because she's such a complicated case, but at the same time its hard to hear as a human being your always looking for the answers. Ive had people be hateful and say "How can you put an innocent thru so much knowing she's going to die anyway?" My response is she's got so much life, even if her organs dont, she's got dreams already, there are defently things she wants to do in life *her latest one is to be a nurse but not to stick people she wants to hold their hands when they have an ouchy * she's got sheer determination and a strong will to live. When she says she's done, thats when we stop. She tells us alot "Its not my time to go to God's playground.. not yet". As hard as that is to hear coming from a 4 year old, I trust that she will be able to tell me when she's had enough. She told us last year she didnt want to do the surgery and we almost canceled it. It wasnt until a week before the surgery that she changed her mind after spending the day at an amusement park that she said "This was the best day of my life, Im ready for my surgery now."

 

[gijanet]

Bridget (((hugs)))

Bridget (((hugs)))

Welcome to our family, but I am so sorry you have to be here. I just got through skimming through Arianna's carepage, and, wow! I thought my Katie had run me through the meatgrinder. Your daughter is adorable and sounds so precious; she sounds a lot like Katie in so many ways.

Katie is six and has heterotaxy syndrome with polysplenia and situs ambiguous (also known as left atrial isomerism) and has numerous complex heart defects to top it all off. She has two bilobed (left) lungs, malrotation of the gut, midline liver, two spleens that we know of, bilateral superior vena cavae, dextrocardia, an interrupted inferior vena cava, double outlet right ventricle, an unbalanced complete atrioventricular septal defect, L-transposition of the great vessels (also known as CCTGV), pulmonary stenosis, and a severely leaking common AV valve. She has had five paliative heart surgeries which have quasi-repaired her little heart making it work for her. She does have a mechanical common AV valve (her mitral and tricuspid are actually merged as one valve) and has been on coumadin for almost two years now eek: ACK! I can't believe July 12th will be our two year anniversary!) Life on coumadin has not been nearly as bad as I thought it would be. Yes, we have had our head hits that aged me a few more years, but turned out to be perfectly okay. We have also had a few INR ups and downs (mainly with stomach bugs and antibiotic usage), but it has not been the nightmare I thought it would be. Luckily, I found these guys here before our valve replacement and they squared me away beforehand. It didn't totally appease my anxieties, but definitely helped, and later when I had questions, I knew right where to come.

Bridget, please do NOT beat yourself up over decisions you have made on Arianna's behalf. I AM speaking from experience. I constantly beat myself up over decisions I have made on Katie's behalf. Did I do the right thing? What if we had done this? What if I had gotten a sixth opinion from Dr. Hanley? or ????? I suffer from insomnia (yeah, could you guess posting at this hour? ) and play headgames driving myself insane over these types of questions. I know, though, in my heart that we can only make the decisions we make based on what we know at any given time. It is obvious that you have sought out information and we have to trust the specialists some time to help us make those decisions. The problem with our children is that they are so unique. Let's face it. There just isn't the medical history for our kids' doctors to draw from. Had Katie been born twenty years ago, she most likely wouldn't be here. While that pains me to even type that, I, have to accept that. With advanced medical technology and improved surgical advances, our kids are living and breaking new medical ground. Our surgeon is world renowned, but has only performed three valve replacements on fontan kids, and Katie was his first simultaneous fontan and valve replacement. Comforting that was not! I've learned not to pay any attention to statistics. A couple ofweeks ago when I couldn't sleep, I was researching left atrial isomerism yet again - ya never know - there might be new info. One study from Canada stated that the mortality rate for left atrial isomerism with single ventricle function was 50% from birth to age one, and only 27% surviving at twenty years. But things are improving everyday and advances are being made everyday and I have to focus on that. Please do that, too. Where I was going with all this is to say that I don't think your doctors steered you wrong out of negligence. I think that they just don't have enough case histories like Arianna to read and draw upon. They are breaking new ground.

Pleae forgive me as we just got back into town a couple of hours ago and my mind is foggy. I read Arianna's page, but wasn't sure if she had had all of her surgeries at Rainbow Babies or not. Is there another medical center that specializes in Tuberous Sclerosis that you could get a second opinion from? I'm not saying to ditch RB, but because TS varies in severity and accompanying anomalies, it is possible that another center might have had a case similar to Arianna's. (Is there possibly a TS forum anywhere where you could contact other parents to see if one of their children has similar anomalies and problems?) Also, I would contact Al Lodwick. He is our coumadin guru. While he may not have a ped patient who is on seizure meds and coumadin, I would bet that he has an adult patient who is on seizure meds and coumadin. Please contact him with a list of Arianna's meds and see if he knows of any possible interaction problems with coumadin. Go to the anticoag forum and there will be a link to Al's site. He is a wonderful, compassionate man and will help in any way he can. WE have corresponded on numerous occasions.

Okay, enough of my ramblings tonight. Bridget, I feel your pain and will help in any way I can. Will check in soon when I have mybrain back (hoping anyway). But please talk to Al. Let's get this interaction thing nipped in the bud before we go further. Many hugs. Janet

 

[Gnusgal]

Bridget,

First of all, welcome to VR. I have found it to be a source of comfort, support, and knowledge beyond compare. I hope you do as well.

As for your daughter's situation, I wish I had some answers. But just as the doctors have no idea, I'm even more in the dark. But I do understand about making decisions that MIGHT have made things worse. When I was 21 we had a doc ask why I hadn't been "repaired" (ASD, VSD, PS), so we jumped on it and I had my first OHS. Since then I've had more surgeries in the past 10 years (12) than I did my entire childhood (4). Is this because of the surgery, or because of my getting older? We'll never know. I know my mother sometimes wishes we had not made the decision to have the surgery. But immediately after I had it I had so much more energy that I'd ever experienced and I finally knew what "good" felt like. Even if it only lasted a few years. At least I got to experience it, right? We can't begrudge the doctor for his advice, since he did consult other docs and recommended the option he thought was best given the information we had at the time. Is it possible that a different doctor would have gone with something else? Sure. But it wouldn't have done any good, since the prospect of being "fixed" sounded pretty darn good to us.

Okay, sorry for rambling, but I hope you realize that you didn't make the WRONG choice. You made the right choice for the moment. And if you hadn't made the choice your daughter wouldn't be here to drive you crazy. Now you're facing another choice and I know you will do everything in your power to make sure it is the right one. But remember, you can only get the information that is out there RIGHT NOW. Who knows what will be discovered tomorrow.

As for the "plastic heart" idea, my mom's been rooting for the pig hearts for years now. We know eventually a transplant is in my future, but we sure do hope it will wait until technology can make transplants more accessable and with less meds to go with it. I agree, though, that a transplant can be a blessing in some ways. After all, I look forward to the day that I can have a "normal" heart and feel what "good" feels like again (not that I think I'm feeling particularly bad, mind you). However, I know that "good" doesn't come without other prices to pay, so I'm willing to wait until technology catches up.

Good luck to you on your quest for information. You came to the right place, that's for sure. I double Janet's recommendation to contact Al about the coumadin/seisure meds. He's a world of information at our fingertips.

 

[Lynlw]

Bridget I'm no sure If I said this before, but from what I know the kids that were going to clevelend speciffically went for Dr Mee, he is the one that was the ped specialist that was one of the best in the world. Since he left, most of the kids with complex CHDs that I KNOW, which granted is a small %, either went elsewhere for the 2nd stage of ther repairs or decided to go elsewhere IF they didn't have any surgeries yet. I'm not sure who they got to replace him that is the ped expert and what his specialty is, (Dr.Mee was THE world specialist on the double switch). BUT It wouldn't hurt to have her records sent there and pick their brains, I also would probably contact Spray at CHOP (I get more amazed at his talent and the thought and effort he puts into things BEFORE surgery and has a well thought out plan A,B and C) Boston, In boston Maybe you could even ask about the clinic trials they are doing for valve replavement in the cath lab (Justin wasn't a candidate, because he needed conduit)or see if there is something else they could offer, they have lots of research going on, I actually contacted them again when Justin needed his last surgery, because when we were there 3 years ago, we talked alot about the tissue enginered valves out of the kids own heart tissue, but it is still too early for the clinical trial for that, BUt they are really good about giving opinions from records and cds of caths, echo's what ever you have) and of course I'd contact Katie's doc Bove in U of M, there are some really good surgeons in Cal, but these places all have great programs , and are big enough they have seen alot AND also have mojor center beside hearts they can consult with AND ar e relatively close.

Janet, Arriana had her surgeries at duPont, Dr Norwood was her first surgeon that offerred to operate when noone else would. (that is how I know Bridget)
I think Pizzari, did the valve replacement, but my mind is sleep deprived, Lyn