Thursday, May 14, 2009

What's Going On, By Kevin

Okay, I know some of you have either been told this by us or have read it on your own searching yourselves on Google, but we are told some people still don't really know what is happing with our baby girl that is due August 23rd. Because first two kids were C-Sections, this one will be as well and is usually 7-10days before the due date. We know we have to have baby up in St. Pete so baby can immediatly be brought to All Children's which is accessible through a walking tunnel that connects to the hospital Ashley will give birth. Now, I will go with our baby to the CARDIAC ICU, which I'm told is an ICU on steriods. But, Ashley will have to wait until she is cleared to get out of bed and after a C-Section is at least 12hrs, if not longer.
Now, below is something I found that best explains the KNOWNS. What is not told is what comes along with these procedures (breathing/eating issues, recovery time stays in hospital, limited to no visits except by parents)as well as the unknowns (breathing/feeding tubes at HOME as well as hospital, longer recovery times at hospital, constant sickness that leads to more stays at hospital, how much (if at all) can we hold our baby, "lockdown" during RSV season which is from Fall to Spring, total cost of all this, and many others.
We know not two cases of such rare CHD (Congenial Heart Disease) are the same, but to make matters worse, pretty much evertyhing Ashley has seen/read, is freaking her out. Babies are constantly ending back at the hospital and all seem to eventually get feeding tubes as well as oxygen tubes. We will no matter what be spending a lot of time in St. Pete and with two boys starting a new school this year for Kindergarden and K-4 along with the fact we both have to work (Ashley gets great benifits (health care) and is pretty much main reason she works and now we need it more than ever), it is going to be very difficult to juggle eveything and try not to disrupt the boys life too much. We want them to still eat very healthy, get to bed (preferably their own) ontime (8PM), and not to be worring/scared. We will also still have a house (hopfully)to clean, yard to mow, bills to pay, grocery shop, get kids to/from school, kids "homework"/activities, and everything else that must still go on in life. I'm falling a sleep, so that's it for now but please read below for at least the KNOWN's coming up on our lives! I've highlited some areas in Red to get main points visible.

What is a single ventricle cardiac anomaly?

The term "single ventricle anomaly" is purposely non-specific. It is used to describe a group of cardiac defects that may differ quite dramatically from each other but share the common feature that only one of the two ventricles is of adequate functional size.
Because of this feature, the ultimate plan for reconstruction is actually quite similar for most of these anomalies. All will generally undergo staged reconstructive procedures ultimately resulting in a "Fontan circulation."
Some of the anomalies described as single ventricle defects include:
Tricuspid atresia
Hypoplastic left heart syndrome
Double inlet left ventricle
Many of the heterotaxy defects
Some variations of double outlet right ventricle (This is what we are told we have KPL)

Diagnosing and evaluating single ventricle anomalies
In most cases an echocardiogram will be able to define the details of the cardiac anatomy for the initial diagnosis in the newborn period. Echocardiography will also be important in following the anatomy and function of the various components of the cardiovascular system, such as valve or ventricular function, as the child grows.
Cardiac catheterization is only occasionally necessary in a newborn with a single ventricle anomaly, if there are details of the anatomy that cannot be determined by echocardiogram. However, patients with single ventricle anomalies will have a cardiac catheterization prior to the second surgery (Glenn shunt) and again before the third surgery (Fontan procedure). These cardiac catheterizations are done to look at the anatomy, particularly of the pulmonary arteries, and to obtain pressure measurements in the heart. These pressure measurements are important in determining if a patient with a single ventricle anomaly is a good candidate for surgery.
Catheter interventions such as dilation or stenting of pulmonary arteries or coil occlusion of abnormal collateral (extra) vessels may be performed at the time of these cardiac catheterizations.

Fontan Circulation
In the normal heart each ventricle does a separate job. The right ventricle pumps blood to the lungs, the left ventricle pumps blood to the body.
In a single ventricle heart, there is only one ventricle large enough to do the normal job of pumping blood. Thus, we need to configure the circulation to maximize the efficiency of this single ventricle.
This ultimately requires committing the single ventricle to doing the harder work of the heart, pumping blood to the body. The job of getting blood to the lungs must be done without a pump.
Whenever there is only one ventricle large enough to do a normal job of pumping blood, we need to configure the circulation to maximize the efficiency of this single ventricle without overworking it.

The "Fontan circulation" refers to this configuration where the single ventricle pumps blood returning from the lungs to the body, and the blood returning from the body travels to the lungs via direct blood vessel connections without a pumping chamber. In any individual child there may be different procedures needed to achieve this goal.

Requirements for a child to be a good risk candidate for a Fontan circulation
For a heart with a Fontan reconstruction to work well, there are a few crucial features that must be maintained. These key factors must be kept in mind when planning the medical or surgical management of children with single ventricle defects from the first day of life onward.
The single ventricle must not be overworked for a long period of time, in terms of either having to pump too much blood or pump at too high a pressure.
The pulmonary arteries must grow well without stenosis (narrowing) and must remain low resistance (or be very relaxed). If the pulmonary arteries are narrow or if the resistance in these vessels is high, blood will not be able to flow into them without a pump, so the Fontan will not be successful. (We are told they see beginning stages of Stenosis and expect near to full stenosis at birth, so we are told to expect surgery the first week of birth, see below. KPL)

Lastly, leaky or tight valves may adversely affect the function of the ventricle or the flow of blood to the lungs.
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Operative stages for achieving a Fontan circulation
The type of operation needed in the newborn period is quite varied depending on the specific type of single ventricle cardiac defect. In some babies there is not enough blood flow into the lungs, resulting in cyanosis. In these babies, a tube graft is placed from an artery (usually the left subclavian or left innominate artery) to the pulmonary artery. This is called a systemic to pulmonary artery shunt or Blalock-Taussig (BT) shunt. (This is the first surgery we are told to expect the first week of life because of the Stenosis, KPL)
In other babies, the flow of blood into the lungs may be excessive, placing an extra burden on the ventricle and exposing the pulmonary arteries to dangerously high pressure. In these babies, a procedure will be performed to restrict blood flow to the lungs. This is done by placing a piece of material or a "band" around the pulmonary artery.
Other newborns have more complex heart disease and require more complex operations, such as the Norwood procedure for patients with hypoplastic left heart syndrome.
Rarely, a baby with a single ventricle anomaly will have "just right" flow into the lungs so that an equal amount of blood flows to the body and the lungs. These babies do not require intervention in the newborn period.
Whatever is needed in the newborn period, the aim is typically to balance the blood flow between the lungs and the body, achieving stable oxygen levels and adequate heart function.
The second stage for most children with single ventricle anomolies is undertaken around three to six months of age. The operation is called a "bi-directional Glenn" or sometimes a "hemi-Fontan."
During the Glenn operation the large vessel that drains blood from the head and upper body back to the heart (the superior vena cava) is taken off the heart and sewn directly to the pulmonary artery. If a prior BT shunt was present, it is removed. If a pulmonary artery was previously placed, it may be removed but can also be left in place in some situations. (This is our second surgery we are to expect, KPL)
The Glenn operation has two major advantages in most children. First, because the connection is a direct one between two blood vessels, rather than made of artificial matter, it has the ability to grow with the child.
Second, it removes some of the work of pumping blood to the lungs from the single ventricle so that the ventricle will no longer have to pump all of the blood to the lungs in addition to all of the blood to the body, which places it at risk for early heart failure. In most cases this stage is tolerated the best of all the stages with a survival rate of 95 percent or better.
After the Glenn operation most children will have oxygen saturation levels of 75 percent to 85 percent.
The third and final stage in the reconstruction of a single ventricle heart defect is the Fontan completion operation. This operation is usually performed at 2 or 3 years of age, based on the child's size and clinical status. (The third and final KNOWN surgery, KPL)
During the Fontan operation, blood returning to the heart from the lower half of the body (via the inferior vena cava) is connected directly the blood from the pulmonary arteries. Up until now this blood has bypassed the lungs resulting in oxygen levels lower than normal.
After a Fontan operation, oxygen levels will be nearly normal (90s). The two most common methods of performing the Fontan completion today are the "lateral tunnel" and the "extra-cardiac" techniques.
In the lateral tunnel method, a tunnel-like patch is placed inside the atrium so that blood returning from the inferior vena cava is directed through this tunnel. A connection is then made between the end of the tunnel / top of the right atrium and the underside of the pulmonary artery.
In the extra-cardiac method, the inferior vena cava is taken off of the heart and a synthetic tube, usually Gore-texTM, is sewn directly to the top of the inferior vena cava and to the underside of the pulmonary artery, routing the blood flow outside of the heart.
In either method, a hole or "fenestration" is often made between the Fontan circuit and the right atrium so that if pressures become very high in the Fontan circuit, there is a "pop-off" into the heart. Patients with fenestrations may have a more stable post-operative course with smaller and less prolonged plerual effusions (a common complication after Fontan surgery). Many fenestrations close spontaneously many months after surgery, but can also be closed during a cardiac catheterization procedure if deemed necessary.
Currently, when patients have been well prepared for Fontan completion, the success rates are 90 percent and higher.

Will a child who has had a Fontan operation be able to function as well as a child with a normal, two-ventricle heart?
After a successful Fontan surgery, the reconstructed single ventricle heart has achieved its maximal efficiency in terms of ventricular work and near normal oxygen levels, but its capacity for work will usually not match that of a normal heart when examined using sophisticated testing.
The limitations children experience due to their heart defect, though, can vary greatly. At one end of the spectrum there are children with Fontan circulations who have participated in competitive sports such as swimming and gymnastics. Most children are on a blood thinner called coumadin after their Fontan to prevent clots from forming in the Fontan circuit. When a child is on a blood thinner, you must take extra careful to avoid falls or head trauma as they are at increased risk for internal bleeding.
Other children may have significant limitation in their capacity for exercise.
Most children fall somewhere in between the extremes.
How long a heart with a single ventricle reconstruction can function is not known. It is now just over 30 years since the first successful Fontan operation was performed and many improvements in surgical technique and medical management have occurred over this time period.
Late complications including irregular rhythms and heart failure may be occur. Some speculate that most single ventricle hearts will not function efficiently beyond 30 to 40 years, but improvements in surgical technique and medical care may increase this age significantly. In some cases, if the ventricular function deteriorates significantly, heart transplantation may be considered.
Because of the possibility of late complications, continued regular follow-up with a cardiologist for the life of a patient with a single ventricle anomaly is essential.

Wednesday, May 6, 2009

In Case Anyone Is Curious

I did take Todd to the dentist Monday. A few weeks after whatever accident he had to damage the tooth...don't worry I was reprimanded by the dentist. In my defense, Todd did not complain about the tooth hurting until this past weekend and there was no outer trauma to his mouth to give me a head's up.

His tooth is actually fine...besides being a nasty gray color. He was very good for the dentist. My kids are freaks. They LOVE going to the dentist. Anyway. He got an x-ray of his tooth and the nerve is still alive and there is no fracture to the tooth. The dentist did say we (and Todd) need to be careful with the tooth so as not to do anymore damage to it. The dentist said given time the tooth will heal and could even go back to being white. The things you learn. I would much prefer his tooth to go back to white because those suckers do not get loose and fall out until around 7 years of age. 3 more years of all his pictures with a gray front tooth? I hope not.

Of course being the boy he is I'm sure he will do something to retraumatize that poor tooth and it will come out sooner rather than later.

Tuesday, May 5, 2009

I Like My Normal

I recently read a post from another blogger that perfectly describes how I am feeling right now. I wish I could express with my words the same eloquence but I lack the story telling skills so vital to this type of writing. In all honesty I had already discussed this with my counselor and to an extent in a previous blog post. I now know the phrase that sums up my experience...I am not normal.

I am not normal because I am not having a normal pregnancy ending with a normal baby.

The reason I am so hesitant to be around people and talk about the baby is because everyone assumes that all is well and good. And really, there is no reason why anyone would assume anything different. I look like any other normal pregnant person. However, when faced with questions/comments such as "are you getting excited for the is a girl right?"...the answer lays stuck somewhere between my logical brain and my constricted throat. I end up stuttering or mumbling same lame answer. The end result is always the same, I am unfriendly. And I get to do this every day.

But, do I lie? I feel such the hypocrite.

Or do I tell the truth to a stranger, casual acquaintance or a friend who does not already know what is going on?

Do I shock the hell out of them and, I am not at all excited about the delivery of this baby and here is why. Right now I can protect the baby to an extent. Once delivered there is not one damn thing I can do. Nothing. Pure powerlessness. I can only sit back and watch while my tiny infant is poked, prodded, stuck, tested, hooked up to machines bigger than her tiny form, has her chest cut open, her heart operated on, go through more at one week old than most people will ever experience in a lifetime. She will not understand what is happening and I can do nothing but watch. I will not be able to hold her or nurse her, the two basic ways to reassure her in the way babies understand with touch and smell. She will only know sterility, discomfort, pain. For at least 5 weeks. And this is just stage one.

I believe in prayer and faith. I believe in God. However, right now, it all seems inadequate somehow. Too little for me to do when faced with such huge tasks.

And it sucks. And it is not fair. And I am resentful. And I am selfish. And to be truthful...I don't want to be positive right now. I hate this and I hate the way I feel.

I do not want to be a "heart" mom. I do not want to have a chronically ill child. I do not want to rack up ridiculous medical bills. I do not want to have to come home with an oxygen tank, feeding tubes and heart monitors. I do not want to be scared all the time. I do not want to miss Kyle's birthday. I do not want to miss Halloween and quite possibly Thanksgiving. I do not want to miss the Gator games. I do not want to be away from my boys when they need me. I do not want to miss out on Friday kids nights. I do not want to miss the boys laughter and silly antics. I do not want to sleep in a bed without my husband. I do not want to have my husband two hours aways when I need him to take care of the details or hug me. I do not want to adapt to a new normal.

I like my normal just the way it is now.

Friday, May 1, 2009

A Conversation With Todd may need an interpreter for this one.

This morning as I was finishing getting ready for work, Todd comes into my room carrying two pairs of shoes. One pair is his regular tennis shoes he wears to school everyday. The second pair are the new dress shoes that Pappi bought him. However, the new "dress"shoes look a whole lot like fancy tennis shoes (these particular shoes are the only ones he would agree to get after tying on like 5 other pairs of dress shoes...these got the seal of approval because well, one, they are comfy and two, they make him run fast). I generally do not let him wear these to school as the playgrounds he plays on are filthy and have a tendency to make nice, new dress shoes look crappy. My husband, to avoid the 20th fight of the morning with Todd will occasionally give in and let Todd wear the shoes to school. I guess this morning Kevin was not going to cave, you know, since he did yesterday.

In my house, when Todd and Daddy are arguing (every dang morning!) Todd's solution is to come to me and get me to settle the problem. No different this morning...hence the two pairs of shoes he was holding when he came into my room.

Todd: "look at me, I need to change into brown pants." (The child is already fully dressed minus the shoes...and we are all running late)

Me: "why...I like what you are wearing, you look so handsome."

Todd: "no I don't...this outfit looks nervous."

Me: "ummm, sorry, nervous?"

Todd: "yes mama, nervous. And these shoes (his blue and white tennis shoes) do not match!"

Me: not yet speaking to Todd, um, you are wearing blue shorts and a white polo with blue trim..."honey, how do they not match?"

Todd: "I need brown pants and shirt."

Me: Totally not getting where this is going..."sweetie, your tennis shoes match fine, please put them on."

Todd leaves me and goes straight to Kevin...holding up his brown dress shoes (I am presuming as he does like to gesture with objects...wonder where that comes from...). In a somewhat triumphant voice..."mama said I could wear my dress shoes."

Huh? I think my jaw actually dropped a little. How in the world did he get that from our conversation. I did correct that statement with a "no I did not say that."

Five minutes later....wailing from the other room...Todd: "I want mama to tie my shoes..."

Apparently I am the only person in the whole wide world who can correctly tie Todd's tennis shoes. And of course he tells whoever else is tying them that they did not do it right.

On another note...Todd rolled out of his bed a few weeks ago and banged his mouth on his stool. Did not have the first clue as he never cried or complained about falling out of the bed or his mouth hurting. Right before Easter I caught a glimpse of his mouth when he was talking. Guess what? He did not hit the outside part of his mouth...oh no, he hit his top two teeth. One worse than the other as his top left tooth is completely gray. I am getting seriously creeped out as I wonder if this child who looks so much like me is going to repeat every stupid thing I ever did. As a young child (I'm sure some will remember) I took it upon myself to soap the bathtub so I could ice skate. Being oh so wise at my tender young age I did not calculate that the tub would be so slippery I could not possible even stand with out falling. Cracked my chin and mouth of the side of the tub. Damaged my top left tooth. Turned the sucker gray. Since I then went on to hit the tooth again, have a root canal and then have my top two teeth surgically removed...I am seriously hoping Todd does not continue to follow my path.

Between Todd's grey tooth and his funky twang...I am positive I am living with a redneck.