Hello? New here, and frightened

[clouddancer22]

Thank you. I will ask. You guys are so wonderful, I am so glad I found you!!

Here is the thing - I have no idea what the symptoms were supposed to be. When she was 3 months old, I took her to her pcp for an EAR INFECTION. Next thing I knew, they were putting her in an ICU unit and calling for a Medi-Vac helicopter. I told the doctor, wow, this must one heck of an ear infection. That is when they said that it was for her heart, previously undiagnosed. We almost lost her that night, her heart was so enlarged, and the digoxin nearly killed her.

Two weeks plus, that is how long it was before we came back home from that "doctors visit." Many hours from home, no money, no clothes - we obviously weren't prepared for that. We slept in her room, and the waiting room until the Ronald McDonald house stepped in. And one failed valvioplasty and an open heart surgery later. I think I aged 10 years during that trip, lol.

That is my fear now. That a simple overnight stay for a routine valvioplasty is going to turn into another nightmare like that. We know the valve leaks. I am told they are now concerned because the aorta is starting to enlarge to accomodate the gradient. We have been told that this procedure will likely necessitate further intervention, a replacement of some type. But when? Do they know right away if it is leaking too much, or will a subsequent checkup reveal that sometime in the future????

And how on earth do people afford to go other places for surgery??? She first went to Childrens Hospital in LA, now Valley Childrens Hospital.

Sorry for all this rambling. I havent gotten much sleep lately, staying up with a sick 4 year old - fever and asthma attacks.

 

[clouddancer22]

meds

meds

And OMG, I never took into consideration possible meds - anti rejection, anti coagulant - oh she is awful about remembering to put on her retainer, let alone take any meds on a regular basis.

She has Turners Syndrome, and we gave her daily growth hormone shots for a couple of years; she was able to do her own shots the next seven years, but skipped alot I found out later.

And the excercises for the foot surgeries. I could barely get her to do them unless I watched her like a hawk.

Are meds going to be a necessity for sure???

Sorry to keep bugging you all. (((hugs)))

 

[Mary]

There shouldn't be any need for anti rejection meds. If she had the Ross procedure, or a tissue valve, chances are she won't need anticoagulants.

My suggestion is to use a private message (look at a person's screen name, highlight it and it will show you their public file, then click on send a PM) and ask some of the mom's on the site how they handled traveling and expenses for surgeries. These ladies are a wealth of information and they will help you figure some of these issues out. Sometimes a private message elicits a response faster than posting to a thread.
You might try JeanneImp or Lynlw since they seem to check the forum more often than others. EmmaCornish is in England, but she has many contacts in the United States too.

I can't imagine how you are dealing with all the issues you are, but there is help and support available here. Please keep asking questions. You need to feel you have a good grasp on the information you are receiving.
Our prayers continue to be with you and your daughter.
Mary

 

[PJmomrunner]

Hi Clouddancer:

I have not heard of anyone on this website having to take anti-rejection drugs. It is my understanding that when a replacement valve is tissue (porcine, bovine or human) it is stripped of most of the tissue until only a sort of scaffolding (made of elastin maybe???) remains and then it is impregnated with collagen. The patient's own collagen then grows into the new tissue and the two become one.

Now if they use a mechanical valve it's a different story. Still, there are no rejection drugs, but the patient has to take warfarin (Coumadin) for the rest of their lives because the hemodynamics of the mech valve are not quite as good as the tissue valve and blood tends to pool up a bit behind the mechanical flaps and such. The warfarin keeps that blood from forming clots, which of course can cause big problems.

You said something about your daughter's aorta enlarging because of the gradient.... The gradient is probably a contributing factor, BUT bicuspid aortic valves are known to OFTEN come with spongey (myxomatous) aortic tissue and studies have confirmed that, even after the valve is replaced, the aorta will continue to expand, become aneurysmal, and sometimes dissect. For this reason, at least the dilated portion of the ascending aorta is usually replaced at the same time the valve is. I know little about Turner's Syndrome, but I'm quite sure it is also linked with aortic aneurysms and dissections. Do you have a TS specialist your daughter sees? That might be a good way to get a surgical referral as well.

 

[Lynlw]

And how on earth do people afford to go other places for surgery??? She first went to Childrens Hospital in LA, now Valley Childrens Hospital.

I'm not really sure exactly hat you are talking about, if it is transportation and you have to fly there are plenty ofgroups that help, if you are talking about a place to stay while your daughter is having the surgery. There usually is some where for the parents to sleep, either in the room or a sleeping lounge, also many of the children's hospitals have a ronald mcdonald house , or something simular, for the families to stay at. that usually is not expensive and I believe they will work w/ what you can afford.
Some hospitals are set up much more family friendly than others, when Justin had his surgeries at dupont, there are 2 beds in each room for parents as well as free parking and meals, but most hospitals aren't like that. As for where to get the money, there aren't many organizations that will help, actually after Justin had surgery one year and then BE the next we ended up having to declare bankruptcy (
Fist I would decide where to have the surgery, then go from there, once you know where you are going, the social workers are pretty good at letting you know what kind of accomidations there are, Lyn

 

[PapaHappyStar]

This article:

The Bicuspid Aortic Valve
Braverman AC, Güven H, Beardslee MA, Makan M, Kates AM, Moon MR
Current Problems in Cardiology- 2005 9 (Vol. 30, Issue 9)

has a section on the Turner Syndrome, it causes BAV and seems to be a chromosomal abnormality which occurs due to a fault in early cell division, so is not heritable.

I am sorry about implying you would need anti-rejection medication for a tissue valve, what I meant by rejection was slow calcification ( and loss of pliability ) in the valve leaflets. The body still treats the valves as foreign material -- I dont know the details of what they do to prevent tissue rejection though.

Regards,
Burair

 

[clouddancer22]

Papa, Turner's notoriously causes aortic stenosis, not a bicuspid valve. I think 90% of Turner's have no heart problems. But the combo was not so good. My dad has a bicuspid aortic valve, and I am thinking I do as well.

I can't post much now, my keyboard is getting wet. Too many tears. Sorry.

 

[Mary]

Clouddancer,
Go to Chat; I'm there and will try to answer some questions.

 

[Ross]

I'm curious, how is it that someone that has had this many surgeries is on NO MEDICATIONS as your profile says?

 

[PapaHappyStar]

Papa, Turner's notoriously causes aortic stenosis, not a bicuspid valve. I think 90% of Turner's have no heart problems. But the combo was not so good. My dad has a bicuspid aortic valve, and I am thinking I do as well.

I can't post much now, my keyboard is getting wet. Too many tears. Sorry.

Thats good info, perhaps I am missing the point here. The point is to try to understand how best to help you with getting your daughter the best possible treatment, and you some support. Congenital heart disease is a difficult thing to come to terms with and you and your family have definitely had more than your fair share.

Please know that when you start posting here you become part of a diverse group of people who each understand at least some of what you are going through and some may understand most.

All the best,
Burair

 

[tobagotwo]

Some thoughts about the Ross Procedure...

In my opinion, a successful Ross Procedure is the best AVR outcome, barring a repair. However, two themes tend to run through those Ross Procedures that don't do as well over time. One is aortic root deformation, which torques the valve, causing it to close unevenly over time. The other is myxomatous (weakened) tissue that is either undetected or still developing in the pulmonary valve that is transplanted to the aortic position.

I believe there are ways to surgically stabilize the aortic root during the procedure, which can resolve that issue before it starts. Especially if the patient is a BAV, this option should be discussed with the surgeon.

The surgeon needs to be highly experienced and skilled, and as capable of determining the status of the pulmonary valve as that expertise will allow. It will be his determination on the spot as to whether the pulmonary valve will be transplantable, or whether they'll have to fall back to the backup plan. So far, there isn't an external method of determining the tissue characteristics of the pulmonary valve tissue, as far as I am aware.

There are also problems that can show up with the pulmonary replacement, but they don't compromise the aortic position, frequently stabilize on their own, and are a less demanding surgery if they do need to be replaced.

As far as backup plans, a tissue valve will have a shortened useful life, due to a 16-year-old's highly active body chemistry. One strong theory is that chemicals on the valve's surfaces eventually signal to the body that the tissue is damaged (or not living), and the body then attempts to coat the valve leaflets with calcium to protect and isolate them. This makes them inflexible and rough-edged, so they become difficult to open and leak from the bumpy, uneven closure seams.

However, you and your daughter should be aware that the use of a mechanical valve as a backup will require the use of Coumadin (wafarin), making it very difficult, risky, and unlikely that she would be able to bear children. As her future choices are at stake, that issue also warrants consideration.

This is an entirely unenviable position for a parent to be in, and I am truly grateful that I was the patient in my family, and not my son. You and your daughter will be in my thoughts as you go forward.

Best wishes,



Best wishes,

 

[sfconstrct]

steve in so ca.

steve in so ca.

cloud dancer, you have recieved alot of good advice, all will work out, the only thing i might be able to help you with is a couple of surgeons in l.a. check them out on the web just google their names. Both seem very impressive. The first was recommended by my cardiologist Dr. David Kawanishi, pretty sharp guy ( might want to check him out too). He recommended Dr. Taro Yokoyama private practice and operates out of St. Vincents in L.A. Interviewed him, it is obvious he does alot of valves on all types, little hard to communicate and seems to do the tried and true he takes on all cases very young to very old. He supprised me upon our visit and told my wife and I it's possible i have anyerism in my aorta so I just had a cat scan last week and haven't heard yet. If not I will just have to replace my bicuspid arotic valve (my hope) if not he said he would replace the arotic root and valve at he same time. I am 50 and in limbo of which way to go I was pretty sure I would have to go mechanical cause of my age then he said the latest bovine valves are or should last 20 years. Sorry to ramble bout me. Anyway this other doctors name has come up a couple of times as I have all kinds of people recommending the best valve guy they know of, most of my referrals have come from friends in the medical field. Anyway I am getting ready to go see him looks very impressive. Dr Vaugn Starnes big dog at USC he does alot of work with pedatric and sounds like he is cutting edge. I was given his name through a neighbor that is a c.o.o. of an Orange County hospital and the other was a cancer specialist well known at USC Dr. Starnes did his valve and said he is the man. Chech em out and good luck. Steve in the o.c.

 

[Diane]

Clouddancer,
You are right.....there is a surgeon from Stanford, Dr. Hanley, that comes to Childrens' a couple of times a month. He is an excellent surgeon.....I believe he is one of the top-rated pediatric cardiac surgeons in the country. I have Tetralogy of Fallot and he did my pulmonary valve replacement up at Stanford last year. I am an adult so I went to Stanford even though I live in Madera. However, I had a post-op visit with Dr. Hanley's assistant at Childrens'. That way I didn't have to have someone drive me back up to Stanford 10 days after discharge.

Best wishes,
Diane

 

[clouddancer22]

Hi Guys!! My friends!!!

First of all - ROSS - what do you mean?? My daughter doesn't take any meds. What meds is she supposed to be thinking about?With the Turner's Syndrome symptom of short stature she had to have daily growth hormone shorts for about 8 years. Ummmm, yeah, she takes antibiotics for dental appts. And some antibiotics and also tubes for the constant ear infections. And I guess a few pain pills, not many, for the surgery discomfort for the foot surgeries . . . am I missing something?? Her blood pressure is 118/65 - do we need meds for that?? I thought that was still in the ok range? Am I missing something, is there something I should be thinking about that I missed? Oh goodness, I am worried now.

Second of all, today was the pre-cath appt. I was really uncomfortable with having to argue with the surgeon whether my daughter had had open heart surgery or not. He insisted I was mistaken. It came down to him demanding to look at her scar. OK, after that, he took us a little more seriously.

And I am in a really angry mood right now! It was just revealed to me that her pulminory valve is leaking as well. I wondered about that, and they said that they didn't want to worry me. Last appt. they revealed that they were worried about the enlarging about the aorta. I guess they didn't want to worry me about that either. Hey! I am the mom here! I need to know, want to know this stuff. I AM NOT A MORON!!

So the Ross procedure is out. Obviously since the pulminory valve is already leaking, it would not be suitable on the aortic side, with even more pressure.

I guess the possibilities of tommorow are: One, they will do this dye thing during the cath, and see that the valve is too bad. Well, that is good, we can scedule what we want when we want. Second, they will do the cath, and it will indeed buy her more time. Third, they will do the cath, and the valve will explode, necesitating an immediate tissue valve transplant, they only do pig valves. I get no choices in my daughter's health.

Her valve has been cut, and ballooned so many times. They say that after the age of 18, it has been found that the valve has so calcified that it crushes like egg shells. She is 16 1/2 now. Not 18, but not a baby either. I don't know. I just don't know.

Thank you all that went on chat with me. I do appreciate that. I am sorry for wigging out so much, I just felt so much in the dark. Today, I feel I have more knowledge. I actually am more comfortable today than I was last week. The info is more dire, but at least I know it all, and feel more in control of my beautiful daughter's future, as well I should being the mom.

And thank you all for sharing so much knowledge with me. I was armed with that today when we saw the surgeon. He takes this case so much more seriously now.

I would pray that they decide not to do anything tomorrow. A transplant is inevitable, but I would like to plan it. I am not happy with the info I was given, armed with you guys' info. They only have one tissue valve choice, a pig valve. I would like someone open to choices, and sharing info.

Thank you so much. Her cath is scheduled for tomorrow at 10:30 am. Please pray for her, her name is Mikaela. And she is awesome!

Hugs, Ursula and family

 

[geebee]

I hope things go well for your daughter.

I am confused by a couple of comments. How could it be that the only valve offered by a hospital in California, in 2006, is a pig valve. That just doesn't make sense to me. Also, you said a transplant is inevitable. What kind of transplant?

Also, how many repairs has she had? You mentioned that her valve has been cut and ballooned many times? I did not think multiple repairs were possible but maybe I am mistaken. I thought valves were repaired only once and, if the repair didn't hold, the next surgery was replacement.

Maybe there is just confusion because you are so upset. I think it's important for you to continue on with accurate information and clarity so you can fight for your daughter's well being.

Thanks and best wishes to you and your family.

 

[Lynlw]

I personally would not use a surgeon, that I have to argue with about wether or not my child had heart surgery, didn't he read her records? altho it was nice a surgeon met w/ you before the cath, we never talked to any until we were discussing surgery, Lyn

 

[BDMc]

Facts Really Do Help

Facts Really Do Help

Dear Ursula, I'm so sorry you and Mikaela are having such a tough time! I will keep you all in my prayers. I'm glad to hear you are feeling better. The facts do help one feel a little more in control, or that at least you know what's going on. I have two assignments for you to help deal with your anxiety: I... Focus on the facts, and keep the docs and nurses focused on them too. II... Try think of as many ways as you can to express your love for your daughter to her without saying it. I hope this helps. Lastly know we are here for you. Brian

 

[RCB]

"Second of all, today was the pre-cath appt. I was really uncomfortable with having to argue with the surgeon whether my daughter had had open heart surgery or not. He insisted I was mistaken. It came down to him demanding to look at her scar. OK, after that, he took us a little more seriously."

No should have to "argue" with a doctor about something that should be a matter of simple medical history! I have never heard of a doctor demanding to see anyone scar as proof of OHS- 1. a scar proves nothing. 2. any doctor
who asked for such proof should be reported to the medical board in your state. 3. If it were me and I were treated like that, I would take my daughter
and run from his office. Why would you let your daughter be treated by such an incompetent jerk!?

 

[clouddancer22]

I don't know everyone, that is why I am asking! I am lost and confused and scared! Thank you all for your help in guiding us in this situation.

 

[Ross]

Hi Guys!! My friends!!!

First of all - ROSS - what do you mean?? My daughter doesn't take any meds.

Surgery:She had open heart surgery for a bicuspid valve, stenotic, aortic. 5 valvioplasites.
Heart History:Severe aortic stenosis
Medications:None

By the way, how was your trip to Greece back in March?