Wednesday afternoon update

Chris here - Brianna and Victoria have gone down to eat lunch.  Maya is napping after a busy morning playing with Brianna, and then having her first fluid administered via her new Nasogastric tube.  It is a tube down her throat and directly into her stomach.  They apparently installed it early this morning before Brianna and I arrived at 9 AM.  She does not appear to be in much discomfort from it.  It is amazing what she is tolerating now!  She had 240cc of water injected over a 1 hour period into her stomach, and I had to walk around with her until it finished, then she fell asleep. 

Victoria and I were instructed by a representative from the company that produces the feeding bags and pumps on what to do with the home model, and how often new supplies would be delivered.  I asked the coordinating RN whether we would receive home assistance from a nurse, and was assured we would.  I also asked about school, knowing that they would not be allowed to work with her feeding tube (which is actually just for liquid, food is fine for her to eat normally).  I was told that most likely a nurse could go to her school when it was liquid time and administer it.  This is turning into a larger life-changing event than we had anticipated.  I hope her school will allow her to return, as she needs the interaction and normalcy of her friends.

Speaking of normalcy, the place in which Brianna and I stayed last night was one in which many distant families live for months at a time while a child is being cared for at the Children's Hospital of Boston.  Brianna and I saw 2 children with one of the rarest diseases in the world, Progeria, aka Hutchinson-Gilford Progeria syndrome.  According to wikipedia, only 48 children in the world currently have this condition, and we saw two and talked briefly with a South American family whose daughter was there with it.  I had prepped Brianna beforehand and she was polite and said 'Hello' to the girl that was in the common area when we went there to get some water.  I don't know if she was shy, unable to speak clearly or did not know english, but she sort of looked down and away when Brianna spoke to her, and the family tried to prompt her to speak, but I didn't hear anything more than perhaps a whisper.  It was so incredibly heart-rending to see the way this child looked.  She could have been 2 years old or 12, but she was less than 2 feet in stature, sitting in a high chair and had a wrist that looked like the wrist of a 100-year old, very bony, curled and thin.  I think that is perhaps the saddest disease in the world, where kids get all of the symptoms of old age while still babies.  It was so emotional for me that I cried in front of Brianna in our room and had a hard time explaining why.  We are so fortunate in that our family has repairable, minor issues compared to many others we have been crossing paths with.  A recurring lesson in this life is to show compassion wherever possible and express gratitude as strongly and as often as possible.

-Chris

Tuesday PM Update

It is 8:00pm on Tuesday night.  It has been an exhausting day.  Maya only had one hour long nap in the stroller today.  She just refused to give in.  She fell asleep an hour ago.  Luckily, the child life specialist was available to watch her, after she fell asleep while I went and got our clothes out of the dryer.  They have a patient/family laundry room, that has 2 washers and dryers and they were full, so I wanted to be sure I didn't tie anyone else up.  The biggest necessity in the wash today was Maya's favorite froggy blanket, that our friend June actually gave to Brianna for her birthday.  Maya just loves sleeping on it.  Yes on it, not under nor with it.  

So today, we had a consult with the speech pathologist, who determined that they needed to do a barium swallow study on Maya to determine exactly what consistency her beverages/ food needed to be in order for her not to aspirate them into her lungs.  (in lay man's terms, without them going down the wrong pipes)  

For the barium study, they had Maya sit in a carseat looking type seat, that was surrounding by xray equipment, so it gave the feeling of being in an enclosure with an open top, bottom and front.  Then, they brought out some different foods that had been mixed with the powdered barium.  (The purpose of the barium is to be able to watch the food/drinks on the xray while she swallows them to see which pipe it goes down)  They started with milk, that had been mixed to honey thickness using baby rice cereal.  She aspirated that.  Then they gave her some chocolate pudding, which she thoroughly enjoyed, and didn't want to stop eating.  She did not aspirate that.  Then it was baby banana food, which she aspirated.  Then they gave her some cream of wheat with granola, which she did not aspirate.  So in the end they told me that she could not have any foods that were thinner than pudding consistency.  I think they should have followed that statement with... until we determine a plan of action, which is what they meant but didn't state.  My reaction to that was that it was unacceptable to just feed my child foods that were thicker than pudding!  So they clarified that the "team" would meet to determine the plan of action.

In the meantime, Maya and I went back up to her room, then we went and put a load of clothes into the laundry, the soap dispenser ate my quarter and produced no soap, but some nice mother, who I overheard talking on the cell phone that her child's transplant was probably not successful, loaned me some soap.  Then Maya and I went back up to the room and ordered her food voucher for dinner.  This was something I only just learned about on day 6 in the hospital.  Apparently, instead of ordering food from the menu to be delivered to your child's room for them, you can instead order a food voucher, which is a $7.50 voucher that can be used in the cafeteria.  Had we known earlier, it would have saved us some hassle.  Anyway, so we took our voucher and went back to the laundry, after I got so excited to see that CVS had just got the new trash magazines in, and they were kind enough to give us more quarters.  We were fortunate, to arrive and find one of the two dryers unoccupied.  After laundry was switched, we headed to the cafe with the voucher.  Maya ate great, she longingly looked at my glass of ice water, and I felt somewhat guilty drinking it in front of her.  She pointed once, and I had to tell her no.  We were just finishing dinner, when I got a call on my cell phone from the floor nurse asking where we were and that the Surgeon was waiting to talk with me.  (only 1/2 hr ago, the same RN told me he was in a lengthy emergency surgery and wouldn't be free until tomorrow, which was why I had left.)  Maya got to finish the last bits of bananas and we briskly walked back to the floor.

The Surgeon and the RN practitioner for the floor shared with me that he had spoken with three ENTs, and the speech team, and that they feel that Maya has paresis, vs. paralysis of her left vocal cord.  They said paresis is temporary, where paralysis is not.  I asked how they determined that, and he shared it was b/c her vocal cord moved slightly vs. not at all.   He then shared that what needed to occur, was that Maya needed to have a feeding tube placed in her nose, so that we could give her proper hydration at home.  Yes, she is going to be leaving with a feeding tube that we will need to work.  The Surgeon said that he believed that the vocal cord will improve in a matter of weeks, although the Dr. yesterday said could be up to one year.  I will have to ferret out why the big disparity tomorrow.  They will show Chris and I how to work the feeding tube tomorrow, as well as install it.  The RN practitioner wanted to install it tonight, but I refused.  Maya was exhausted, as was I, and I was not in the mood to be shoving tubes down her nose at 8/9pm at night after a long day.  The Surgeon agreed.  The plan for tonight is to hook her up to some IV fluids via her IV in her neck, which is still good.  They are currently waiting for her to fall asleep (10pm now).

I had a nice surprise as I was beginning to write this blog entry, Chris and Brianna showed up.  Chris had gone back home today to get Brianna, and to take her to her Taekwondo class, as she needed some normalcy.  Brianna and Maya were very happy to see each other.  Maya even gave Brianna a hug.  Brianna did a Jedi Mind Trick on me, and got a vanilla pudding and graham crackers from the patient food center.  Maya and I rode the elevator down with Chris and  Brianna, and Maya started to cry when they left.

So I am exhausted, and still need to do some research before tomorrow.  Thank you all again for your thoughts and prayers. Love Victoria

Monday PM update

It is 5pm Monday night, and we had thought earlier in the day that we would be well on our way home at this point, but such is not the case.  We just had our ENT (Ear Nose Throat) consultation.  This came about b/c I was talking with the RN from our cardiologist's office about how well Maya was recovering.  She asked if I had any questions and I said sort of matter of factly, that Maya has been choking everyt

ime she drinks, and is that normal.  She said no it wasn't.  Then she asked us to have one of her nurses watch her drink, next time she takes a drink.  So then we followed Maya around like hawks, waiting and trying to get her to take a drink (For any of you who spend a lot of time with Maya, this is fairly amusing b/c Maya is ALWAYS thristly, and never refuses a drink), well she was not cooperating and a few hours late

r, she finally took a drink and choked.  They then had the surgeon who had performed the heart surgery talk to us.  He explained that they good news was that she was making some improvements in her sound generation, which 

leads him to believe this damage is temporary.  He then went on to say that the damage could have been caused one of two ways: 1. the breathing tube caused irritation 2. there is a nerve that surrounds the area around the PDA (area that he sutured shut during surgery), and that the stretching may have irritated the nerve. 

 

Fast forward to 4pm, and our ENT consultation.   They came in with a black instrument case, that resembled a trumpet case.  That was the "scope".  It is an instrument that has a camera and light on it, imagine a pirate's telescope, but on the end of the telescope is a piece of licorice lace, dangling down, with a light and camera built in

to the end of the licorice lace.  So Chris departed the room, since he had this scope procedure attempted on him, and didn't go through with it.  So I stayed and held Maya in the chair, while they navigated the licorice lace part of the scope down in her left nostril and down into her throat.  Maya cried, but really not that bad.  The end result of their scope was that Maya's left vocal cord is partially paralyzed.  This is what is causing her to choke on liquids, as it is seeping past the cords.   This is also what is causing the hoarse voi

ce, since the cord on the left cannot vibrate as it is suppose to.  What next?  The ENT told us that he thinks, since this is isolated to the left side, that it was probably caused during the surgery, by somehow stretching the nerve around the PDA.  He said that it is a common risk with thoracic surgery (not a risk with open-heart surgery, and had we known, maybe we would have gone for open heart, could of should of would of).  He then proceeded to say that there is no way to know if it is permanent until after a year, which means that this can last up to a year, this temporary damage.  He said the good news is that if it is permanent, they can do surgery that will move the left vocal cord into a place where she won't choke anymore.  He also said that after a few months, if she is not improving, they can inject some type of disolvable collagen that will move the left vocal cord over to improve th

e hoarseness and choking.  In the meantime, they want us to be evaluated by speech and pathology, and they want her to do a swallow study.  We are not sure whether this will be today or tomorrow.  In addition, speech will work with us to identify a new diet, as there will be some foods/beverages she cannot have.  They also said that she cannot drink thin liquids and there is a thickener that we can get to add to her beverages.  Lovely.

 

Well enough about the ENT stuff.  We also had 

our Pulmonology consult this morning, and we all agreed to not do the chest CAT scan until the 25th of August.  They assured us that there is nothing that can harm her between now and then, and that if they did the scan now, they would not be able to see what they need to due to post operative swelling in the chest cavity.  They couldn't really give us a reason for the blebs, but said that once they have a better picture of the lungs, they will be able to then determine what could be causing them and what next to do.  They did say that blebs are caused because of the lack of elasticity in the lung tissue, but that th

ey need to look at the lungs in their entirety to get the best story on what is going on.  The good news is that we were able to schedule her cardiac follow up for the same date, so that we don't have to make multiple trips here.  Although now that I am thinking about this, ENT wants a follow up in a month, and that is going to send us back her probably a week or two after that.  So end result is that we have not seen the last of Boston.  The good news is despite these weird other issues, Maya is recovering great.  She is running the halls, throughly enjoying the play room and the plethera of toys that is in the play room.  We did loose her roomate today, Natasha got to go home this afternoon, which they were very excited about.

 

Natasha peeks out from behind her curtains

That is all for now... Victoria

Some videos from Maya's heart surgery

Click on these if you want a timeline in video snapshots of her heart surgery and recovery at Boston Children's Hospital

Mommy walking in to see Maya in Cardiac ICU after surgery

That's all for tonight.  Maya was silly and happy tonight.  After Mommy, the grandmas and Brianna went to the hotel, she and I took a nap and then went down to the lobby and got food from Au Bon Pain, and Maya ate a whole fruit cup and 1/2 a yogurt.  She then played peek-a-boo spontaneously and laughed her butt off.  It was a good 1/2 hour of fun one on one play with her in a great mood.

Sunday after surgery

Maya is doing very well today.  She has continued visiting the play room and has shown very little in the way of pain.  She has not been eating much, but better than yesterday.  She was able to get blueberry pancakes this morning and seemed to enjoy them, she ate about 1/2 of a pancake.  We have met wonderful people from all over the world to talk to.  We did not realize that Boston Children's Hospital is the World premier hospital for children's cardiac procedures.

Bit of a hassle today though.  The doctor in charge of coordinating procedures for the cardiac team was trying to persuade us to wait a month before getting a CT scan on Maya's lungs.  I argued very strongly for her to have the scan done tomorrow, as the Pulmonology  doctor already agreed to.  It seems that coordination across disciplines is fair at best everywhere we have been.  The coordinator will talk to all involved and give us their decision later or tomorrow.  They'll be a fight on their hands if they don't agree to the CT scan before we leave!

Very soon big sister Brianna will arrive with both Grandma Mimi (Victoria's mom) and Grandma Dayle (my mom).  The timing kind of stinks as Maya is getting tired for a nap and both lunch and visitors will be arriving soon.  Oh well. More later...

-Chris

Saturday afternoon update

Maya is doing great.  She was given a cocktail of meds that made her seem really happy drunk, after which they removed her chest drainage tube.  She now has 2 IV sites left, one on left neck, one on left ankle, and that's it, except for monitor stickers/leads.  She has been playing and has even left the bed and gone for a walk to the play room.  Also, she was moved out of the ICU to the regular cardiac hall and her new room phone # is 617-355-3622.  We are not in the room now as Brianna's similarly-aged friend Natasha is her new roomie, and she has had a tough time sleeping and with pain, so we are all out of the room giving them quiet time.  Therefore no photos either, as the camera card reader is in the room, and I am not.

-Chris

Friday- Afternoon update

Maya has had a better afternoon.  She has been having some rest, they are keeping her on pain meds enough so she is more comfortable.  She was able to eat some chocolate ice cream and drink some milk too.  We are still in the ICU, and no word on if they are going to move her or not.  I am glad we are here.  They did however get her the bigger crib, which is nice.

The other new wrinkle, is that the surgeon who did the heart operation saw what he called "blebs" on her left lung when he was fixing the heart.  He said that they were only on about 5% of her lung, and dispersed equally over it.  Not that we understand what that means exactly.  A bleb, as it was explained to us is like a blister filled air sac that has thinner membranes than the rest of the lung tissue.  Chris had a consult with the Pulmonary Dr. on duty, and he agreed that further investigation is warranted and will try to arrange for a Monday AM CT scan.  We would like to leave either knowing what we need to do about these "blebs" and their total coverage area on both lungs, or that they are just a slight aberration that are not harmful in any way and can safely be monitored every few years.  There is surprisingly little information on these "blebs" in children on the internet, as most of the info is referring to adults with emphysema or some kind of lung cancer or disease, or that they have no known cause.

Friday am Update

It is 11:15am Friday morning-

Last night, Maya apparently had more "episodes". What I mean by
episode is that she would wake up, be ok for about 5 minutes, then get
a look of terror in her eyes, then she would cry, but you can't hear
it b/c she is still with larangitis from the breathing tube. Then her
heart rate goes up to 190+ (ok is around 130 or below)and her O2 sats
go down to 60 or lower (ok is 100-90). She then gets another dose of
morphine, the doses back off to sleep. According to Chris, who was in
the room with her last night, she had these every hour. I on the
other hand slept beautifully in the bed in the family sleep suite. I
went to bed around 9:30-10pm, and woke up at 7:30am, which I
desperately needed. I am feeling much better this morning. The
family sleep suite is located on the 6th floor of another building in
the hospital. There are I think 25 sleep rooms, which consist of a
twin bed, night stand, and phone. The sleep suite also has showers
and towels. As I mentioned before, those beds are on a lottery basis
for parents with children in the ICU only.

This morning update- Maya had a few more episodes, but within the last
hour, she has been awake and not crying, which is great, although I am
not sure if it is the sedative they just gave her so that they could
take her arterial line (IV in the wrist that monitors blood pressure),
and one IV in the foot. They also were able to give her a sponge
bath, change her diaper, and put on some PJs (amazingly, they were not
the typical clown pattern, but a comfy material with tigers on it.)
We got to speak with the cardiologist and ICU Dr. just now and they
are not yet ready to take her chest tube out, as they originally had
planned to do b/c she still has quite a bit of stuff coming out of
it. They were also giving her percussion treatments (yes, just what
it sounds like, they were beating on her back and chest), to get the
gunk in her lungs loose and moving. Apparently if she sits still too
much, she collects fluid and gunk (like my technical terms?), which is
obviously bad. They say that she is ready to go to the regular floor,
but that they have no open beds to move her too. We are obviously not
upset about that, because she gets more 1-1 from the RN.

New update- I actually started this blog around 9:30am, but keep
getting interupted. We ordered Maya some food (scrambled eggs, wheat
toast and fruit cup). The nurse moved Maya from the bed and into
Chris's lap. She tried a bite of eggs, but cried through it and
didn't want anything else to eat. Her and daddy are now sleeping in
the chair together and getting some needed rest.

I will finish now and send it before it becomes lunchtime! Thanks
again for all who have sent comments and their thoughts- Victoria

Chris Gorski
seekerhiker@hotmail.com

Friday AM update

It is 11:15am Friday morning-

Last night, Maya apparently had more "episodes".  What I mean by episode is that she would wake up, be ok for about 5 minutes, then get a look of terror in her eyes, then she would cry, but you can't hear it b/c she is still with larangitis from the breathing tube.  Then her heart rate goes up to 190+ (ok is around 130 or below)and her O2 sats go down to 60 or lower (ok is 100-90).  She then gets another dose of morphine, the doses back off to sleep.  According to Chris, who was in the room with her last night, she had these every hour.  I on the other hand slept beautifully in the bed in the family sleep suite.  I went to bed around 9:30-10pm, and woke up at 7:30am, which I desperately needed.  I am feeling much better this morning.  The family sleep suite is located on the 6th floor of another building in the hospital.  There are I think 25 sleep rooms, which consist of a twin bed, night stand, and phone.  The sleep suite also has showers and towels.  As I mentioned before, those beds are on a lottery basis for parents with children in the ICU only.

This morning update- Maya had a few more episodes, but within the last hour, she has been awake and not crying, which is great, although I am not sure if it is the sedative they just gave her so that they could take her arterial line (IV in the wrist that monitors blood pressure), and one IV in the foot.  They also were able to give her a sponge bath, change her diaper, and put on some PJs (amazingly, they were not the typical clown pattern, but a comfy material with tigers on it.)  We got to speak with the cardiologist and ICU Dr. just now and they are not yet ready to take her chest tube out, as they originally had planned to do b/c she still has quite a bit of stuff coming out of it.  They were also giving her percussion treatments (yes, just what it sounds like, they were beating on her back and chest), to get the gunk in her lungs loose and moving.  Apparently if she sits still too much, she collects fluid and gunk (like my technical terms?), which is obviously bad.  They say that she is ready to go to the regular floor, but that they have no open beds to move her too.  We are obviously not upset about that, because she gets more 1-1 from the RN.

New update- I actually started this blog around 9:30am, but keep getting interupted.  We ordered Maya some food (scrambled eggs, wheat toast and fruit cup).  The nurse moved Maya from the bed and into Chris's lap.  She tried a bite of eggs, but cried through it and didn't want anything else to eat.  Her and daddy are now sleeping in the chair together and getting some needed rest.

I will finish now and send it before it becomes lunchtime!  Thanks again for all who have sent comments and their thoughts- Victoria

We are with Maya in the Cardiac Intensive Care Unit

It is 3pm and we have been with Maya now since 1pm. She is doing
great, although when we came into the room, she was just coming to,
and was really upset and had a frog voice from the breathing tube just
being removed. Once we gave her the binky, she calmed down and fell
back asleep. Her RN (they have 1-1 while they are in the ICU) Sara,
is with us until 7pm tonight. She is very nice. Maya has 4 IV sites,
two in her feet, one in her neck and an arterial line in her wrist
(this is how they are monitoring her blood pressure). She also has a
chest tube, draining blood from the chest cavity, on the left side
below the surgical incision area. She also has some oxygen blowing on
her to keep her oxygen saturation (O2 sats) up, as with anesthesia, it
can make them sleepy, and not taking in enough air. See attached
picture of Maya in her crib/bed in the ICU (the RN said she ordered a
bigger crib, but we haven't seen it yet)

Surgery successful, she's getting patched up now

Maya's surgeon came to give us the word that she came through wonderfully.  The operation was quick and nothing new was found of concern regarding the heart.  The PDA was closed with a clip and the place the anomalous vein was cut from was both sutured and clipped.  The clips are titanium and will be integrated with the body, and as I have no titanium objects, I did not know whether they are influenced by magnetism or not.  Turns out they are not.  This is important as she will be able to get MRI's in the future with no concerns for the clips ripping out.

They found some areas (5%) of thin lining on the left lung which the Surgeon felt were nothing to be concerned about, but that we will have the Pulmonologist check out anyway and rule out anything to be concerned about.

Updates will follow later as more things to show and tell occur.

-Chris

Maya has gone in for surgery

We arrived at the hospital at 6:30am to the pre-op admitting area.  They checked uAs in and we waited with the other families that were also waiting for surgery.  We saw some of the families that we had met yesterday.  One of the families in particular has been amazing to watch.  They are from Maine, and are here with their 14yo son Josh.  I am not sure all of Josh's issues, b/c he is in a wheelchair and has some other disabilities as well.  Josh is quite funny, and keeps everyone laughing.  He loved Maya yesterday, and remembered her name this morning.  He introduced us to his nana, aunt, father, father's girlfriend, and others he had met that morning.  What amazed us about this family was the closeness that they have.  In addition to Josh there are two other children, a girl who will be a freshman in college this fall and another boy, who is probably in high school.  The mother, step-father, two siblings and Josh were at the hospital with us all day yesterday, a very long day.  Not once did they get annoyed with each other.  They were all joking around with each other, and showing genuine love and concern for Josh, something that you don't always see with teenagers.  As I mentioned before, Josh had some issues.  His mother shared that he was born here at Children's and was born with only 1/2 a heart.  He didn't have an aorta.  She said that he had multiple surgeries when he was born, all of which caused him to have a stroke each time.  The last surgery he had was when he was 4yo and it left him in a coma.  So here they are 10yrs later and Josh with his blue lips (cyanosis) was undergoing surgery again, this time to place a shunt in one of the major arteries.  I can only imagine their fear, considering the outcome of the last surgery. 

As I mentioned before, we were all corralled together in the pre-op waiting area, and the three cardiac surgeries that were scheduled for 7:30am this morning (Josh, Maya and Natasha, who I will share more about later).  Then the nurse came and called all three families.  We were all escorted up to the 3rd floor which is the pre-op holding area and surgery waiting area.  The pre-op holding area only allows 2 people to be bedside with the patient, so Josh had to say goodbye to his entourage.  Most touching was watching his brother say goodbye to him, with tears in his eyes, which caused his mother to cry.  Josh of course tried to lighten the mood for all of us with humor.  We were then escorted to the pre-op beds.  Maya, Natasha and Josh were curtains apart.  At this point, a nurse came in and asked us to get Maya into the scratchy hospital gown(I think there must be one company that makes these gowns for kids, because they are the same design in all the hospitals we have been to.  It is the clowns and circus animal print in red, navy and yellow.  We experienced the same ones at Philly and Hartford too.  Maybe they get a good discount on the ugly fabric in China or somewhere.  Anyway, so we got her into the gown while Maya was watching a show on the tv about funny pets on the Animal Planet channel.  We were there for about 15 minutes watching the show when the anesthesiologist came in with the pink medicine (this is the stuff that knocks her out).  She spit some of it out, but we got most in.  Then the anesthesiologist shared with us that there were some changes today from yesterday.  She said that the breathing tube will most likely come out before she goes to the ICU.  This is due to the fact that they want to give her a caudal (shot in the buttock region that works like an epidural, but goes up vs. down), the purpose of which is to eliminate the pain in the chest cavity area, which is going to be very painful.  This will cut the pain down for 12+hrs, and eliminate the need for her to be so sedated right after the surgery.  We were a bit nervous because there is always the "slight risk of nerve damage, that is very rare".  UGH I hate even hearing these things.  Of course they are required to tell you this b/c they have to warn you of all the risks in advance, so you can sign away.  So after this long discussion, another anesthesiologist had now joined us, as did the surgical nurse.  It was like they were hawks waiting for the kill.  They were waiting, it was just for the "pink medicine" to kick in so they could take her back for surgery.  Then the surgical fellow came in to talk with us.  He was able to answer my question about the location of the chest tube.  I wanted to know if it would be in the same location as the cut for the surgery.  It is not.  She will have a small incision on the left side where the chest tube will be inserted, and she will have a horizontal incision going from the breast area to midway on her side ( I am guessing it will be about 3 inches long).  For some reason, I was thinking it would be vertical.  Poor Maya.  Now she will add these scars to her left side in addition to the 4 small scars on her right side from the CCAM surgery.  Bikini model is not in her destiny. :-0
So we had about 10 minutes of the hawks waiting.  Then they came with the heated blanket and we gave Maya big hugs and kisses, and they took her away for surgery...
We then went for breakfast in the cafe, and now we are back up in the waiting area.  The nurse just gave me an update that they have made the incision at 9:35am.
More to come... Victoria

Pre-op procedures complete, ready to go 1st in line tomorrow

Last night we had a late departure time for Boston. Between Victoria finishing up work projects, me setting up an AOL Instant Messenger account and program for Brianna so we can use web cams to chat with her while we are up here, and the emotional farewell to Grandma and Brianna, we didn't move out until 10:00 PM, and didn't get into the hotel until past 12:30. We then had to awaken at 5 AM for 7:30 AM start to a day of pre-op procedures and consultations. So needless to say we are all exhausted.

Before I describe Maya's day, let me say that Brianna was such a compassionate and loving sister yesterday to Maya. She insisted Maya accompany her to Taekwondo, and I know it was because she wanted the extra time to see her sister before she left for the hospital. Brianna is aware of generally what is going to happen and how long Maya will be gone for. On the ride home from her Taekwondo lesson, Brianna said that she didn't want Maya to go. "I want to get the operation for Maya," she offered, then demaned. How loving is that?!

So on to today. After check-in, we were taken to a central control room where all of the pre-op procedures and all of the patients were written on a white board, and as procedures were finished, the nurses crossed off that procedure. There were about 12 children all getting the same pre-op day procedures done. We ended up talking with many of the children and parents and spent the day crossing paths with them as we waited in various areas. The procedures and consultations/legal sign-offs were pretty quick, but the waiting was very long, especially to talk to the surgeon, which didn't happen as he was too busy operating. We did meet with the cardiologist, anesthesiologist, and the surgical fellow from the team. The one bit of information that we learned from the surgical fellow was that her procedure is actually very painful, much more so than open-heart (cut open the chest via the sternum) procedure, although it is safer, as she will not be put on a heart-lung bypass machine. So they will keep her out cold the whole day, so deeply that a breathing tube will be doing the breathing for her.

She will also have a "central line" installed for the entire time she will be in the Cardiac ICU, which is an IV inserted into the jugular vein in her neck. That brought back visual images of my dad waiting for his liver transplant because he had the central line as well. I do remember that dad told me it wasn't painful, despite how disturbing and uncomfortable it looked. As for her recovery, she will be in the ICU and heavily sedated for a few days, with a nurse dedicated and in direct sight of her exclusively at all times. She then will be graduated to a general pediatric cardiac unit for about another week once she is deemed to be eating, breathing, etc. on her own. Once she is in the general unit and doing well we will bring up Brianna with Grandma Mimi for some joyful reunions. We hope if things are going well to bring Brianna to the New England Aquarium, the Children's Science Center and other tourist sites in the city.

After we left the hospital at 3:30 PM, we came back to the hotel and headed out to dinner. We found an IHOP nearby and I fed Maya exactly what I knew she would love, and as much as she wanted. When she saw the children's menu and we asked her to point to the picture of what she wanted, she got a mischevious look in her eyes and hit her palm on every item on the menu, one at a time, many times over as she laughed and we laughed hysterically. I ended up ordering her Double Blueberry Pancakes and a fruit cup. She ate quite a bit of them with various syrups, the strawberry seeming to be the best liked by her. She really loved the whipped cream as well.

After dinner we stopped at a market and I bought her milk and Fig Newtons
so that she could eat as much good (as in tasty) stuff as possible before her midnight cut-off time. It will seem like an eternity to her before she feels free and eats without pain or restrictions, so I wanted her to live it up.

So here are the pictures and movies of Maya from today-

Here is what the hospital looks like as you walk towards it from the garage

Maya getting ready for a chest x-ray

Exhausted Maya sneaks a nap

Maya tries on sunglasses to be funny at the hotel

Maya tries to fit her sunglasses on daddy

Maya plays while waiting at the hospital

Fast service! She is now scheduled for surgery next Thursday, the 31st.

Victoria was called by Boston Hospital with a date of July 31 for Maya's surgery. They want us there on the 30th. One week to prepare, man things are moving quickly! We were told to expect a 7-10 day recovery period, so I'll be loving that muddy water and Boston will be my home for nearly 2 weeks, and probably Victoria's for a good portion as well.

A plan to avoid Open-Heart Surgery provided by Boston!

Pre-anesthesia:

Natural nap while waiting her turn for the MRI machine (bumped by 2 emergency cases):

Recovery Room:

Yum - fruit cup before leaving the hospital!

I just received a call from the cardiologist in Boston that we are working with. She told me that Maya's Qp/Qs ratio is a combined total of from 1.8 to 2:1, with about 1.5 being from the anomalous vein, and the rest from the PDA. She reported that the surgeon who would be working on Maya planned on going in via a space between the left ribs and resection the vein onto a portion of the left atrium of the heart, all while it is beating! The cardiologist informed me that this method carries far less risk than being on heart-lung bypass.

So needless to say we are excited (I had to text-message the info to Victoria, who is giving testimony in a civil case today) and ready to go. It could be as soon as mid-August.

Heading in...




Ready to head home...


-Chris

Anticipating the Surgery Plan

It is now the evening before Maya gets her sedated Cardiac MRI, and Victoria and I get the Childrens Hospital of Boston evaluation and plan for treatment. We have already been told it is likely she will need open heart surgery due to the evidence that her right ventricle is enlarged. Their opinion is that any enlargement is evidence of a problem (excessive pressure) that must be corrected, as in opposition to the opinion of the cardiac staff in the Childrens Hospital of Philadelphia (albeit without Qp/Qs data that Boston noticed was missing from her report and had to request from her aborted catheter procedure at the Connecticut Children's Medical Center), who felt that her ventricular enlargement was "minimal" and "within normal range."

Victoria drove up earlier today to meet with the anesthesia staff, and I waited until after work to drive up. Victoria says that Maya was so excited and animated and gleefully squealing with the sights and sounds of the city. They were able to get some chinese food for dinner, and the restaurant was selling a large container of fresh watermelon chunks for $1.50, so Victoria bought them and Maya loved eating them. They then went for a swim in the pool and had a bath before Maya fell asleep.

Tomorrow AM will be very difficult for Maya (and for we who will be trying to distract her), as the requirement for sedation is no food past midnight tonight and only clear liquids until 9:30 AM tomorrow. The procedure will not begin until 11:30 AM minimum, and Maya loves her regular meals!

Brianna is staying with Grandma until Tuesday, and is looking forward to Maya having successful surgery and recuperation so they can each get a new kitten or perhaps a puppy if mommy's allergies permit.

More when we find out a plan and get a chance to digest the info...

Until then, here is recent video of Maya playing happily:

And the pendulum swings the other way...

Today Victoria was contacted back by a cardiologist with the Boston Children's Hospital. Victoria sent over all of the information regarding Maya in our possession to request a consultation, as well as to set up an appointment to get a sedated Pulmonary MRI to measure the Qp/Qs ratio (quantity of pulmonary blood flow to systemic blood flow expressed as a ratio). In addition, we are requesting a further procedure called a bronchoscopy be done at the same time on the advice of pulmonology in Hartford, as well as by Dr. Flake in Philadelphia, with the purpose of determining if Maya has any constriction or rigidity in her bronchi, or if her Anomalous Pulmonary Vein is causing pressure on her lung(s).

The Boston cardiologist looked at all of Maya's reports, and actually called Hartford and requested additional data that was apparently not in the reports we brought to Philadelphia nor which we have ourselves. That data shows some sort of preliminary Qp/Qs pressure data that was measured or calculated during her Hartford catheter procedure. The cardiologist says that the data points to a ratio anywhere from 1.4:1 to over 2:1, and that the flow through the Anomalous Vein is very high, just as we were first told in Hartford. She says based on what she sees, she agrees with Hartford on the need for Open-Heart Surgery. The data from the cardiac MRI will quantify the ratio more definitively. So now we are just waiting for Boston's Pulmonary staff to agree on a date/time they can work with cardiology while Maya is sedated and get both tests/procedures at once. It should be soon.

So how has Maya been otherwise? She is walking like a champ now, and is on a temporary respite from her nearly constant sinus/ear/respiratory infections. It may just be a coincidence but after exhausting every antibiotic given to her, I decided to go old school herbal on her, old wives' tale style. I made a decoction of 1 big pot of water into which I put:
-Many old onion skins
-Many old garlic skins and cloves gone soft
-dried spearmint
-dried raspberry leaves
-dried dandelion root
-Dried Stinging Nettle
-a good amount of local honey

I simmered that 2.5 gallon pot down to 1 3/4 gallons or so, then put it into containers and refrigerated it. We give Maya this tea in a sippy cup as often as she'll take it, and we add 1/2 packet of Splenda, as the honey was not enough to mask all of the nasty tastes. She drinks it quite well, and has been symptom free for nearly a week, at the height of allergy season - put that in your pipe and smoke it, nay saying doctors and your supporters! In all fairness we have added liquid Claritin to the liquid Zyrtec we were giving her before, as we also have done with Brianna, although of course picky Brianna won't touch the tea. Brianna is still having asthma attacks. Score a big endorsement for the common immune-system bolstering herbs, I say!

So Maya still doesn't say much, but she has said "Anna" for Brianna, "dog" or "dog-dog" for dogs, "da-da" both for me and for any motorcycle she sees, as well as for any item she does not know a name for. It could be like a "What's that?" for her. Ma, or "Ma-ma" is the term she uses both for Victoria and any food item she desires (Freud, what would you say here?), and of course she is great at pointing and grunting for anything she wants or wants you to turn your attention towards.

She also climbs now, almost anything your average 3 year-old would. She is a dare-devil and will climb small chairs and rock them until she tips them over. She emerges from the wreckage unfazed and tries another daring act. She loves riding in the bicycle trailer, although she now pushes against Brianna to assert her independence and desire for more space. She will take out her helmet and put it on, then hand me or Victoria our helmet(s) when she is outside and wants a ride. When inside she will get me my shoes to indicate her desire to go for a ride.

She also smooches now - big open-mouthed kisses. I have a great photo of her smooching Austin at Tyler's birthday party, but I cannot find my card reader, so cannot get the pics onto the computer today, sorry. I hope these 2-week old videos will suffice for now.

Always get a second opinion!

Today in Philadelphia the world went from upside down to rightside up for us. After a team of cardiologists spent more than an hour performing an exhaustive echocardiagram on little sedated Maya, Dr. R gave us his findings and opinion - no ASD (hole in heart), PFO gone, PDA very small and of no significance in regard to blood oxygenation and the PAPVR, being only one of 4 or 5 veins returning pulmonary blood, is not significant enough to cause current respiratory illness or future problems, and that no enlargement of her right ventricle was noticed. So basically he told us there is no need for surgery and he expects that, other than the PDA closure that will still be needed via a catheter, she should have annual echocardiagrams to watch the heart, but that she should have a normal life without pulmonary hypertension, as the pressure differential should be too small to lead to pulmonary hypertension. He said when we get her catheterized again to close the PDA, have them measure the pressure differential. We plan on having this done in Boston as a sort of 3rd expert look at the situation and because we have heard terrible news twice now in Hartford that turned out to be, well, at the least, overly dire.

So thank you all for your good juju, prayers, dedicatory yoga sessions, etc. They seem to have taken a sad song, and made it better. Maya was so excited in Philly seeing the tall buildings, the buses, the crowds of people...she was literally squealing with joy much of the time. After the good news, so was I on the inside. And Brianna was well-behaved and a very concerned sister who loves Maya very much.

And we thought the CCAM was serious...

Today Maya underwent a catheter scope procedure in which the intention was twofold:

1. Investigate her enlarged right ventricle in her heart, and if not serious enough to require intervention,

2. Perform a coil closure of her PDA

If step one uncovered a condition that would require open heart surgery, they would terminate the entire procedure without closing off the PDA and refer us to a pediatric cardiac surgeon.

Well that is exactly what happened. After about two hours of a procedure expected to last three hours, the Dr. who performs catheter procedures informed us that he did not close the PDA and proceeded to explain, with the aid of some images he printed out of Maya's innards on x-ray, that she had a rare condition called Partial Anomalous Pulmonary Venous Return of the Upper Left Lobe (PAPVR). The link provided explains the basics, at the bottom of this entry are more detailed links and quotes. From how I understand the basics, knowing very little about the complex workings of the cardiovascular system, it is a vein that grew from her left lungs' upper lobe and attached to the right atrium, creating a loop in which oxygenated blood is returned to the lungs rather than circulating throughout her body. This creates additional work for the heart, resulting in enlargement of the Right Ventricle. This enlargement creates additional pressures which result in "strengthening" of the arteries serving the heart, which really means narrowing my muscle tissue rather than the hardening of the arteries caused by cholesterol and Coronary Artery Disease seen in adults, but the consequences are the same - Pulmonary Hypertension which just gets worse. The timeline could be permanent damage by age 80 or by age 6, nobody can predict, so the correct thing to do is the operate on the heart and move the vein to the correct area of the heart (I'm still to unsure of where it will go and how they do it) via open heart surgery.

While undergoing any open-heart procedure, the surgeon will look for and repair any other repairable defects, and it is likely that Maya also has an ASD (Atrial Septal Defect) or basically a hole in the chambers of her heart as well which they saw on ultrasound wile she was sedated today. They are not sure if they actually saw one or not, because ultrasound at the extreme angle they needed to use showed a hole, but it could have been an anomaly due to the angle of view. Doppler ultrasound did not show blood flowing across the possible ASD, but the cardiologist told us this could be because a flow from the other chamber offset the pressure differential so that it looked balanced at that time) no flow across the possible "hole." In any case, an ASD, whether seen thus far or not, is likely in a PAPVR patient and will be easily and quickly sewn shut or patched during open-heart surgery.

The Surgical team of CCMC (Connecticut Children's Medical Center) will be discussing Maya's case tomorrow and we should hear back from their surgeon by later this week. We will then go back to CHOP (Children's Hospial of Philadelphia) for a second opinion. We are leaning towards CHOP as where we would like this operation performed due to their understanding of Maya's former CCAM diagnosis and removal, and the fact that they were so diligent as to first discover her heart issues while she was in for her CCAM removal surgery. They have volumes of data on her and are meticulous in their research and follow-up, and that is very important to us. Also they perform more than 1,000 pediatric open heart operations per year, so their experience is nearly unmatched.

How rare is this specific condition? I give you the following quote from: http://www.ihop-net.org/UniPub/iHOP/pm/6741000.html?pmid=2225404

"Partial anomalous pulmonary venous return.

Van Meter C, LeBlanc JG, Culpepper WS, Ochsner JL
Department of Surgery, Ochsner Clinic, New Orleans, LA 70121.

Anomalous pulmonary venous return from the left lung is an extremely rare condition that is reported sporadically and in general in case reports. From 1964 through 1988, we identified 13 patients with this condition, all of whom underwent surgical correction. This represents the single largest reported institutional experience with this anomaly. The patients ranged in age from 15 months to 40 years. Seven were asymptomatic, and six had symptoms ranging from recurrent pulmonary infection to moderate congestive heart failure. Six had anomalous venous return from the entire left lung, and seven had anomalous return from the upper lobe only. Eight of the patients had associated cardiovascular anomalies. Four of the patients underwent surgical correction via a sternotomy approach with cardiopulmonary bypass to allow correction of coexisting intracardiac anomalies. The remaining patients underwent surgical repair through a left thoracotomy. The technique included high ligation and division of a persistent left superior vena cava with anastomosis to the left atrium at the site of partial excision of the atrial appendage. There were no deaths and only one complication in our series."

So did you catch the number as of 1990? That's right, in bold there - 13, with the youngest being 15 months of age. Maya is not quite 14 months, so another record may be smashed by our daughter who exists to push the envelope of what is "typical."

I think what took the docs this long to figure out the severity of her heart problems is the fact that she is not, "failing to thrive" as is expected with compromised heart/lung function, meaning that most kids with these issues are born tiny, eat little and grow at the bottom of the curve for their age. Maya is well off of the high end of the charts for her growth, and actually was referred by our pediatrician to an endocrinologist for her size and precocious maturation rate, so go figure. If she didn't have these heart/lung issues, she might well be Babyzilla and bring on the end of western civilization by rampaging through cities, Godzilla-style, always looking for that perfect binky or bottle of milk in 55-gallon bottles. So big-baby = discount parental complaints of breathing issues and minimize significance of abnormalities until evidence is unmistakable,

*Side note to parents - if you know something is wrong, and the doctors are not finding anything or investigating all avenues, keep pushing! Your gut feelings are valid and will be vindicated. Or possibly you are delusional and need psychiatric intervention, no matter - keep pushing and eventually help will come.

So for now I leave you with the following stuff I found:

1. Bad site design with some dead links, but excellent information on the heart on its diseases in easy to understand terms:

http://www.geocities.com/HotSprings/1652/

Info from Charlotte Children's Hospital:

"Partial Anomalous Pulmonary Venous Return (PAPVR) - Summary

The four pulmonary veins return oxygen-rich blood from the lungs to the left atrium. From there, blood flows through the left ventricle to the aorta, where it is pumped to the rest of the body.
In Partial Anomalous Pulmonary Venous Return (PAPVR), one or two of the pulmonary veins return blood to the right atrium instead of the left atrium. This causes oxygen-rich blood to flow back to the lungs instead of on to the rest of the body.
Many adults with PAPVR also have an atrial septal defect (ASD).
Concerns and Symptoms
Because some oxygen-rich blood is continually flowing between the lungs and the right atrium, the right chambers of the heart may become dilated. Over time, this may cause an abnormal heart rhythm. In addition, too much blood flow to the lungs may increase the pressure in the lungs blood vessels, leading to a condition called pulmonary hypertension.
If only one of the pulmonary veins is affected by the disorder, you may not have any symptoms. If two of the veins are affected, you may experience shortness of breath during heavy exercise.
Treatment
The operation to repair PAPVR is performed under general anesthesia.
First, the surgeon makes a vertical incision in the front of the chest, opens the breastbone, and exposes the heart. Blood from the heart is redirected to a bypass machine. The bypass machine does the job of the heart and lungs during the operation.
The surgeon then opens the heart. If you have an ASD, the surgeon will create a patch that closes the defect and also redirects blood from the anomalous pulmonary vein to the left atrium.
If you do not have an ASD, the surgeon simply redirects blood from the anomalous pulmonary vein to the left atrium.
After the defects are repaired, the surgeon shuts down the heart-lung bypass machine and the heart starts beating again. The surgeon then closes the breastbone and chest incision, and applies bandages to the incision site.
Recovery
After surgery, you will be moved to the Intensive Care Unit (ICU). There they will monitor your heart function and other vital signs.
Most patients remain in ICU for at least one night after surgery. Once you are stable enough to leave the ICU, you will be moved to a regular hospital room.
You will remain in the hospital for several days after surgery. The exact length of stay depends on the type of surgery and how well you are recovering.
Before being discharged, the staff will explain any medications to take. These may include antibiotics to prevent infection while the heart heals, prior to dental work or any other procedure. They will instruct you on any activity limitations or required follow-up care.

Background

Partial anomalous pulmonary venous connection (PAPVC) with intact atrial septum is a rare congenital cardiac defect. As the name suggests, in PAPVC, a few of the pulmonary veins return to the right atrium instead of the left atrium. Thus, some of the pulmonary venous flow enters the systemic venous circulation.
Embryologically, PAPVC is similar to total anomalous pulmonary venous connection (TAPVC); however, these conditions differ in that all pulmonary venous vessels connect to the right side of the heart in TAPVC.
Anatomically, PAPVC can involve a wide variety of connections, and PAPVC from the right lung is twice as common as PAPVC from the left lung. The most common form of PAPVC is one in which a right upper pulmonary vein connects to the right atrium or the superior vena cava (SVC). This form is almost always associated with a sinus venosus type of atrial septal defect (ASD).
The right pulmonary veins can also drain into the inferior vena cava. The left pulmonary veins can drain into the innominate vein, the coronary sinus, and, rarely, the cavae, right atrium, or left subclavian vein.
Pathophysiology

A number of factors determine the ratio of pulmonary blood flow (Qp) to systemic flow (Qs). The shunt magnitude, expressed as the Qp/Qs ratio, and other factors determine development of symptoms and complications.
The most important factor is the number of pulmonary veins that drain into the systemic circulation. The more veins that anomalously drain, the more blood returns to the right side of the heart. Some authors have suggested that this defect becomes clinically significant when 50% or more of the pulmonary veins anomalously return.
In addition, the source of the returning blood plays a role in determining the clinical effect of the defect. In an individual who is upright, blood flow to the lungs is primarily directed to the lower and middle lobes. Therefore, more blood returns to the systemic venous circulation in individuals in whom the anomalous connection drains into either the right middle and lower lobes or the left lower lobe of the lung.
An associated cardiac defect, such as an ASD, may either exacerbate or alleviate disease progression.
An associated noncardiac condition (eg, scimitar syndrome) may influence disease development. Scimitar syndrome, or pulmonary venolobar syndrome, is characterized by abnormal right-sided pulmonary venous drainage in the inferior vena cava and malformation and/or hypoplasia of the right lung, with abnormal arterial supply of the right lung.
Over many years, excessive pulmonary venous return to the right side of the heart causes right atrial and ventricular dilation. This has a number of consequences, including risk of arrhythmia development, right-sided heart failure, and, rarely, development of pulmonary hypertension.
Frequency

United States

Most data regarding prevalence of this condition have been garnered from autopsy series that estimate an incidence of 0.4-0.7%. However, autopsy series may overestimate the clinical significance of this condition, as many of these cases were asymptomatic; thus, the true incidence of patients who present antemortem with this condition is lower. Clinical diagnosis of isolated PAPVC is quite rare. PAPVC occurs in approximately 10% of patients with a proven ASD.
Mortality/Morbidity

Few data are available regarding mortality due to this lesion because mortality credited to the defect occurs only in adults and the diagnosis has historically been made at autopsy. Major morbidity, including arrhythmias, right-sided cardiac failure, and, rarely, pulmonary vascular disease, also primarily occurs in adults.
Race

No data exist regarding racial predilection.
Sex

No data are available regarding sexual predilection.
Age

PAPVC is a congenital defect. Clinical evidence of this congenital defect may not be apparent until the patient reaches middle age.


CLINICAL

Section 3 of 9
Authors and Editors
Introduction
Clinical
Differentials
Workup
Treatment
Follow-up
Miscellaneous
References


History

Children with partial anomalous pulmonary venous connection (PAPVC) usually remain asymptomatic and are referred based on an incidentally noted cardiac murmur. Symptoms may occur in older patients and may be secondary to right-sided volume overload or pulmonary vascular obstructive disease.
Determining the natural history of this condition was difficult before the era of direct cardiac imaging (ie, echocardiography, cardiac catheterization), as the diagnosis was made only postmortem.
The development of complications from PAPVC clearly depends on how many pulmonary veins abnormally return to the right heart. A single anomalous vein is usually not hemodynamically significant and, hence, does not produce any symptoms.
About 10% of patients with an ASD also have PAPVC and may have symptoms of right-sided overload.
Dyspnea may occur in adults but is rare in children. A child may experience exercise intolerance as a symptom in cases in which more than 50% of pulmonary veins anomalously drain.
Palpitations may reflect cardiac arrhythmias, which are almost always supraventricular in origin, but this is rare in childhood.
Hemoptysis is a rare symptom that reflects either chest infection or the development of pulmonary vascular disease.
Chest pain may be evidence of right heart ischemia but does not occur in childhood. More commonly, chest pain may be a manifestation of recurrent bronchitis.
Associated defects (either cardiac or extracardiac) can produce symptoms.
Peripheral edema can occur in adults with cardiac failure.

Physical

Physical examination findings are usually more revealing than the history, but positive findings depend on the volume of abnormal pulmonary venous return to the right heart. If only a single vein is anomalous, the physical examination findings may be normal. In a patient with a larger volume of abnormal veins, physical examination findings are similar to those of an uncomplicated ASD. These findings include the following:
Left parasternal lift reflects right ventricular dilation. Impulse in the second left intercostal space reflects pulmonary artery dilation.
A soft systolic ejection murmur is heard over the pulmonary area, reflecting turbulence in the pulmonary trunk due to increased right ventricular ejection volume. The second heart sound is always widely split but may have normal respiratory variation.
In healthy individuals, inspiration increases systemic venous return to the right heart, causing a delay in the pulmonic closure component of the second sound. This phenomenon also occurs in patients with PAPVC who have an intact atrial septum. However, in patients with PAPVC and ASD, volume flow into the right heart is always increased, and respiration further augments that flow. Therefore, splitting of S2 proceeds from wide during expiration to wider during inspiration. This does not occur in patients with a significant ASD, in whom second heart spitting is wide and fixed. In the presence of an ASD, variations in systemic venous return during respiration are counterbalanced by reciprocal changes in flow through the ASD, maintaining total right ventricular flow more or less constant. A mid-diastolic murmur due to increased transtricuspid right ventricular filling may be heard over the tricuspid valve area at the lower left sternal border.
Cyanosis does not occur, even in older patients in whom pulmonary hypertension develops, as a right-to-left shunt cannot develop in the absence of an atrial septal communication.
Right-sided heart failure signs in adults include hepatomegaly, jugular venous distension, ascites, and peripheral edema.
Pulmonary vascular disease may occur in older adults, although this is rare. Clinical signs of pulmonary hypertension include a right ventricular parasternal lift, absence of systolic murmur, narrowly split S2 with a loud pulmonic component, and, occasionally, an early, high-frequency murmur of pulmonic regurgitation. Cyanosis does not occur in the presence of an intact atrial septum.

Causes

No causes of this condition are known. No evidence has implicated common teratogens (eg, drugs, infections) in the genesis of PAPVC. No evidence for a genetic predisposition has been reported."


http://www.charlottechildrenhospital.net/body.cfm?id=404&oTopID=404

Do these pictures look like a child failing to thrive to you?

Maya is growing up quickly!

Maya is turning one this Sunday! And my what changes she has gone through in the past year, and even the past month! She is on the verge of walking unassisted, and her spoken vocabulary consists of Moma, Dada, some attempt at a "B" sound both for "ball" and "bubbles" and a tongue-clicking sound to call her cats. Non-verbally, she uses signs to say no (shakes her head, actually), sign to indicate "all done" for meals and also to indicate "more" for food. She accassionally gives open-mouthed "kisses," primarily to a doll, but sometimes to Victoria and once to me.

She still loves baths and water in general. She understands quite a bit, and when I tell her it is bathtime she crawls over to the bathroom and stands trying to climb into the bathtub. She loves music, and often bops her head, headbanger-style to the music, or claps along. She does clap very well, and also has fun "giving fives!"

On the negative side, she can be a bit of a brute, and has the strength to back it up. She can fling food/objects quite hard during dinnertime and will shove your hand or any object away when she is frustrated. She is becoming a very "antsy" child who hates to sit still or wait for anything, especially food. On the other hand she still sleeps well, which is a real blessing.

Maya also is quite capable physically. She watches how a cap is applied to a container and will try it again and again until she is satisfied she knows how to do it. She yanks things off of shelves, but will also spend time putting objects into shelves/bokes/containers. She can turn electronic devices on and off, and is becoming almost as good as Victoria and I at working the TV remote. Oh yes, and Maya is making her first attempts at scribbling with crayons - she uses her left hand for the most part and needs to learn to hold the crayon vertically instead of horizontally for more success, but she is trying!

She and Brianna still adore each other, and Brianna is quite protective. We think Brianna is quite an active handful, but Maya is becoming much worse. She needs lots of physical motion to be happy and quickly bores if sitting still. In the bath she is becoming a splasher. She loves to grab at mouths and noses, but is quite gentle with animals and other babies, such as her cousin Jake, below:

Maya and Brianna also went to visit Grandpa Davis and Donna in Florida last week. Maya developed another sinus infection down there, but they both had a blast in the warm weather, playing with the dog, "Pooh Bear" (Grandpa's yellow labrador retriever) and visiting the beach. Maya had one small fright when a larger wave washed over her while playing at the water's edge, but she recovered quickly.

The sinus infection persists, however and she is visiting the doctor today. Her breathing has been better though, and her checkup with a cardiologist revealed a PFO that appears closed and her PDA to still be present and large enough to warrant closing. She is scheduled to have a catheter put up her leg, Dick Cheney-style, to close the PDA on April 21.

So that's it for now. More pictures and news as the kids feed it to me!