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!

I come to you at the turning of the tide...

Well yesterday/last night was pretty terrible for our whole family. I came home a little early with a fever and strong stomach cramps occurring. I entered a house full of puking females, with one also (over)filling a diaper with liquid hourly. Poor Brianna puked every half-hour all day, but she was so mature about it and always grabbed a bowl. I have no idea how many times Victoria tossed, but she was none too happy yesterday or when I left today. It was a strange night of listless people trying to sleep while moving from room to room, looking for a place with less noise, and one or the other children would stir and want story-reading or other attention every few hours. As I was the least of the sickies, Victoria tagged me to take full care/entertainment for Maya, the second least of the sickies. She would need nebulizer treatments every four hours, a diaper change every two and Pedialyte every hour and a half or so. I dearly wanted good sleep, but my stomach cramping wouldn't allow for it, so I made the best of bonding with Maya as we read books, watched TV (including Homeword Bound, one of my mushy tearjerker favorites that reminds me of my childhood companion, Chuggy), changed her clothes and diapers several times and tried sleeping in the same room. That worked except her binky would pop out every half-hour and she'd cry until I got up and popped it back in. I finally took her into the uncomfortable guest bed with me and she sweat quite a bit but did not wake up much. This allowed me a good 3-4 hours of straight sleep and I awoke this AM feeling very good. I bathed Maya and myself and went into work after dropping Maya off at school. Brianna was still a bit listless and her and the still sick Victoria stayed home together. I was going to take pictures of the girls laying there looking like a grisly scene of death from the Steven King book/movie "The Stand," but when you're sick, the idea of looking for the camera and then uploading the pictures is just too much of an effort.

Sick, sick, and sick of being sick!

Our family has been dealing with illness since the beginning of December, with Maya being particularly affected the whole time. We have each had sinus infections, bronchitis (bronchialitis in Maya) and just Brianna and Maya had ear infections as well.

There was a short window of time at the end of December in which Maya was not too sick (just a runny nose, no fever) and was able to undergo the procedure of getting ear tubes placed and a adenoidectomy. The idea was to reduce her frequency of both sinus and ear infections. Brianna had great results with no ear infections during the time she had tubes.

Well the reality is, this time there is some nasty virus going around, or maybe two or more viruses. Victoria has spent the last few weeks couging up phlegm with bouts of minor gastrointestinal distress as well, even after a run of antibiotics and prednisone. Brianna had a sinus infection and an ear infection that seems to have cleared up with antibiotics. I had a bit of a sinus infection occur early in December and again in January, but they both cleared up with antibiotics. Now I have something deep in the lungs causing tightness, nasty phlegm tinged with blood, painful coughs, occassional sore throat and some brain fog. I will be seeing the doc again tomorrow, but as for Maya...

She continues to get worse despite her operation, treatment with Ceftin, Omnicef, Prednisone and Augmentin ES (currently). She had a week of 101-102 degree fever despite Tylenol last week (finally over) and now she has a nasty rattle when breathing, which the dr. says is in her sinuses, not in the lungs, and a chest x-ray ruled out pneumonia but discovered inflammation of the brancioles, or bronciolitis, causing difficulty breathing for her. We are giving her nebulizer treatments of Albuterol every 4 hours and Atrovent every 8 hours. She has been vomiting since last week and now has severe diarrhea and is very tired out. Victoria has missed many days of work and my mother has helped out watching Maya as much as she can. At Victoria's lastest visit to Maya's dr., she was told this nasty virus is around and the symptoms may last for 3 weeks. This seems to be a new virus of 3 week duration on top of the one in December. When will it end? Poor little Maya tries hard to keep smiling, but she is crying more than ever. Grandma, Victoria and I have all been exploded upon by her various fluids several times in the last week, and Victoria and I take turns getting up nights to give her nebulizer treatments or help her when she vomits. We are giving her small amounts of Pedialyte frequently per the dr., and she is very hungry and thirsty, but giving her any volume gives her more vomiting and the idea is that small amounts will pass more easily. This is very frustrating to see Maya suffer for longer than I have ever seen in a child. Brianna always responded pretty well to antibiotics in the upper range of what is given, and shook off viruses pretty quickly, but Maya is not. It could just be a particularly nasty virus as the dr. tells us she is seeing, and I have been noticing a long-lasting cough pattern in my co-workers the past 3 weeks, so a few strong viruses may be kicking around. It just plain sucks. And you never know when a bacterial infection may creep back in on top of the virus, so do you ask for more antibiotics or what? If you do, you may be wasting money and building up the immunity of the germs, but if you don't a bacterial infection could then lead to severe pneumonia damn quickly, Jim Hensen style.

On a more positive note, despite illnesses Maya began standing up on her own on December 31, and she now walks while holding onto a chair or walker-type toys. She also crawls forward very fast and climbs over small obstacles. She still only says "Moma" definatively, but she understands many words and loves to play peek-a-boo just like Brianna used to with napkins and such. I have heard her say DaDa or PaPa on many occassions, but have been unable to get her to repeat it or to have it linked to greeting or asking for me. Ironically she has learned to imitate the "tch, tch" sound one uses to call a pet over and uses it to call to Hunter (one of our 3 cats who begs at dinner). She also waves to everyone, it is very cute. She doesn't understand when some adults, animals or children don't wave back, but when people do respond, she is all smiles.

Maya also has learned to eat just about anything. She loves firm tofu, as long as it is flavored or in miso soup. Her love of miso soup is the same thing Brianna had as one of her favorite foods as soon as she started eating, but Brianna refuses the seaweed and scallions in the soup, while Maya eats them right up. She also digs citrus, while Brianna has always refused any citrus. She was getting into canned beans, but now she just plays with those mostly. She also tries to cry for sweeter foods when meals are presented, to the point of getting angry and yanking away the spoon or wiping the food off of her tray. She picks up and eats most food herself, and is getting less patient with spoon feeding. She often yanks the spoon and shoves it into her mouth, usually upside down or in a way that leaves the depression of the spoon still level with food, which she then flings to the floor. It won't be long before she figures out how to use the spoon herself.

She seems to be very interested with how things work. She gets a hard look of concentration when studying the controls for the TV (she frequently changes channels with the remote), and she also learns how to open things, get around obstacles and find anything to put into her mouth. She seems to have great concentration on figuring out how mechanical things work, and little interest in dolls and such. When I let her play Baby Giggle on my MacBook, she is always trying to put the screen to the closed position, to look for the objects that she thinks must be hiding behind the screen, because she can't feel them by touching the screen - very funny! Quite a difference from Brianna. I think Maya will end up being my mechanical helper with repairing cars and motorcycles. She also seems to have less interest in affection than Brianna had/has. She is not a cuddly girl and does not like giving or receiving kisses. That is OK, some kids are just that way. She does respond to us with smiles and likes to be tickled or thrown around, so no autistic tendencies or anything, just not much of a hugger/kisser and very independant.

Here she is in a typical meal setting:



Brianna has been doing very well with how she treats her sister and protects her from danger (trying to touch electrical appliances, etc.). She can be so sensitive and caring at times and exactly the opposite at other times. Brianna also is doing great in Taekwondo and has a White Belt with many stripes. She listens to her master amazingly well and her self-discipline while in class is in sharp contrast to most situations outside of class. All-in-all she is a great kid, and loves school, learning to read, playing educational games on her computer, drawing and writing letters. She is very verbally advanced and her sense of humor is well-developed. She can finally trick me and knows when I am tricking her most of the time. She and I are very similar on an emotional level, which allows me to empathize with her emotional states and understand them, but that does not mean I can diffuse her quickly when she has a tantrum. It takes time and emotional distancing. Victoria and I tag off on helping her ease into bedtime, which can go well or be an hours-long struggle. Maybe that TV nanny would be interested in filming an episode or two with us...

Long time, no updates!

Daddy - What took you so long?

It's been a long time since we've done an update. We have learned that having two children is exponentially more time/energy consuming than having one child. With one you can take him/her nearly everywhere with you with ease, and if one parent needs to do something solo, no problem. The other parent or a family member can handle the child fairly easily.

Now with two children, especially with one being an infant, everything needs to be carefully planned and prepared for, and going anywhere takes at least 45 minutes just to prepare/pack for, and can be much longer if the infant needs feeding first.

Along with Victoria being back at work full-time and resuming heavy travel, I have had a heavy workload and my current job does not allow for "non-work related" Internet use. The result being that there has been no free time and energy left to blog. But I have a window of time now that will allow me to fill-in the past few months.

Maya is doing great. She is amazingly big for her age - way off the charts. She is currently 22 pounds and 27"+ long. She wears size 4 diapers now and her clothes that fit comfortably are the 12 month ones.

She is a great smiler and it is easy to get her to do a deep smile!

The opposite of her smiles is the pouty face she puts on before crying. This has become more frequent as she seems to have been teething for at least a month now on and off. Her chubby face combined with her pout makes her look like one of those 1920's babies that you see often on the funny greeting cards.

She has also begun to use her hands to manipulate things. She grabs at my mouth and makes cooing sounds which I imitate back. I put a french fry in her hand last week when I was at lunch with Brianna, and she waved it around and mushed it a bit near her eye. Brianna laughed and said she looked like a pirate waving a sword.

Brianna adores her little sister and is so good at attending to her and helping out mom and dad! She loves making Maya smile (not a tough job) as well as helping feed her, burp her, get her clothes and bathe her.

Brianna has been taking swimming lessons and is getting more confident at it. We are still waiting for her to be able to submerge her face without fear, but she kicks well and can go way out into deep water with her floating swimsuit on.

On to Maya's latest medical news. We had her 4 month exam with a cardiologist. She has 4 cardiac-related issues we are watching and will follow-up on in a year.

1. PDA - she still has this, and although it is small the cardiologist recommends we have it closed off when she is a few years old. He does not expect it to close on its own at this point, and although it may cause no visible problem, it leaves her with an increased risk of cardiac infection. He wants to wait until she is bigger to insert a catheter up to the PDA site via her leg - just like an adult getting a shunt installed. Right now it is riskier due to her small arteries.

2. PFO - this is smaller than first diagnosed. The cardiologist labels it as minor and does not anticipate any need to intervene.

3. Enlargement of right-side of her heart/asymmetrical pressure . The cardiologist beliefs this may be a residual effect of the CCAM and will monitor.

4. Slight intermittent murmur unrelated to any of the above. Not expected to be a problem, most of us have one that is undiagnosed.

Maya and Brianna are also happy to have a new cousin, Jake Ryan. Savannah is doing very well as a big sister to Jake.

Maya doing great - followup Thursday in Philly

Before you read on, check out this video link from MSNBC - this is a little boy that went through the worst case scenario that we were preparing for with Maya, and the team at Texas Children's Medical Center did their first fetal open CCAM surgery - way to go doctors! We can now add Texas Children's Hospital to the list of CCAM fetal surgery centers.

http://video.msn.com/v/us/msnbc.htm?g=83EF17D0-4EE1-4D28-9F76-46F8C135C82D&f=00&fg=copy

Maya is doing great! She was 15 lbs. 5 oz at her latest doctor's visit, and solidly (pun intended) off the charts at the 98th percentile for weight, head circumference and length. The "chart" stops at 95%. She still has bouts of pain from acid reflux even being on the max dose of Zantac for her size, and gas is another cause of discomfort for her, but it is getting to be a problem less often. She is alert more, and has started day care at the same place as Brianna. Brianna is a very protective and proud big sister. We were at a restaurant on Sunday and Maya started crying quite a bit. Victoria took her outside to console her and told me to stay at the table with Brianna. Brianna was very concerned with Maya and wanted to go with Victoria outside - I told her to stay with me and tried to catch her. She screamed in a guttural tone, "Let go of me!" (which I did immediately, having never heard that tone before) and ran out to check on Maya.

Brianna is also becoming very motherly in another way - she is concerned about my bonding time with Maya. She always asks me as soon as I come home, "Daddy, Maya's eyes are open, do you want to hold her?" This may be a reaction to Maya being with Victoria so much of the time due to breast feeding, but it still is cute seeing Brianna try to "balance out" the family.

Brianna is now able to pedal her training-wheeled bicycle very well by herself. She rode close to a mile last night on a "rails-to-trails" down the road from us. Her only issues are being able to use her brake properly (she keeps putting her feet down to stop), and consistently looking ahead where she is going. Once she gets those down pat, we will wean from the training wheels. Victoria and I have two new bikes and a trailer for the bikes, so we ride Maya behind us and get great exercise whenever possible. We are now big fans of the multi-use trails.

Brianna has also mastered driving her electric jeep and has learned to operate her LeapFrog portable video game system. She is advancing intellectually in leaps and bounds. She still has volatile emotional swings that cause fits that make her seem like the worst brat on Earth, but can bounce back quickly to be as sweet as can be. We have learned that sugar is bad, and chocolate is much worse for making her hyper. Her attention span is also short but intense, and changes constantly. Once she states she is done with an activity, getting her to finish with it is almost impossible (puzzle, game, etc.) I am trying to get her to follow through with tasks, because I see my patterns in her, and one of the things that has held me back the most in life is my lack of attention to detail and follow-thru.

Brianna will be coming with us to Philadelphia Thursday and is very excited to do so.

More testing...

Maya is still at the Connecticut Children's Medical Center (CCMC) in Hartford undergoing observation and tests. Victoria has spent 2 nights there and I have spent one keeping a 24/7 vigil with Maya. Deb (Victoria's Mom) has been a great help by caring for Brianna at home. Her first 2 nights there were bad, with some uncontrollable crying spells with back arching and consequent low oxygenation and/or turning blue. Also a few "blue spells" for no reason with good oxygenation. A main culprit is believed to be GERD or acid reflux. They started her on a minimal dose of Zantac with little/no effect which our primary pediatrician (Dr. Brown) prompty increased to the max dose. This appears to be working darn well, as she has had no crying spells of note since then.

To rule out other issues, Avon Pediatrics doctors Brown and Perks have ordered consultations by a cardiologist and a pulmonologist, as well as a sleep study and a barium swallow study. All visits/tests have been done with the exception of the barium study, to be done at 3 PM today. We hope Brianna will swallow the barium, as we are strongly opposed to the alternative of snaking a feeding tube down her nose to force it in.

The cardiologist sees no indications of issues arising from her PDA or PFO, and thinks they will close on their own, and wants us to set a follow-up appointment for 3 months from now. The pulmonologist thinks the GERD is responsible for closing off her airway intermittently, and has ordered a lengthening of the originally prescribed barium study by 20 minutes (why? I don't know). The sleep study results are unknown at this point as well. We are sure that when the results are in Avon Pediatrics will call us ASAP, as they have been doing all along.

GERD is not unexpected in her, as Brianna had it pretty bad for a few years and was also on max-dose Zantac. It eventually resolved on its own.

So hopefully Maya will be home later tonight or tomorrow at the latest.

-Chris

Unexpected possible turn for the worse

We made it safely home Sunday night and enjoyed some time with Maya seeming near-normal on Monday. Today (Tuesday, May 1) however, Maya has some perceived difficulty breathing and some skin-tone changes on one side of her body, as well as feeding changes. On the advice of Dr. Flake (the surgeon from Philadelphia), Maya is going into the CT Children's Medical Center to get a chest X-ray and to be put on an oxygen saturation monitor. Victoria has brought her there and I will be joining her after work. We hope there is nothing serious going on, but if so, we will aggressively seek the best treatment for her. I will update when I know more.

-Chris

Going Home Today!!!

This is Chris - I just got the word from Dr. Flake that Maya looks great and, pending the results of another x-ray, she can go home today - yipee!

I fed her 3 times last night, and she is eating great and is very alert when feeding and after for a while.

Maya gets drain removed - doing great!

Her chest drain tube was removed tonight, and there was immediate relief for Maya. We took her right out of the crib and Victoria held and fed her plenty and was even able to burp her with no visible discomfort to Maya. She even smiled at me when I made funny faces at her and gave her raspberry kisses.

-Chris

A Good Feeding - Progress!

This is Chris - Victoria is having a much-needed rest on a fold-out chair bed next to Maya's crib.

Maya just woke up enough to gulp down 4 oz. of milk, with much less discomfort than last night. She is back to sleeping now. Her drain is starting to leak fluid around the outside of it, and not draining on the inside, so it possibly is clogged. Air is not accumulating near her lungs in X-rays however, so the docs are not concerned. The plan is to do one more X-ray this evening, and if all looks good, remove her tube. This will leave an open wound that they will cover with gauze. This wound should close up within a day.

We had an observant resident dr. notice her abdominal distension (which she has always had). They did an abdoninal x-ray and discovered a weak area of muscle wall near her belly button. They are not overly concerned, but this could lead to a hernia and will also need to be followed. It is amazing how the other doctors, who were so focused on her CCAM, missed other things going on. I love seeing a resident doctor come by, because they are so observant and pick up on things that more experienced doctors overlook. An astute resident in Hartford, present when Victoria was induced, noticed an intermittant murmur in Maya's heart, but that info was never followed up on by other doctors. That was most likely the "PDA" that they discovered on this visit.

So experience is best for operations, but for diagnoses, I recommend letting a medical resident examine a patient. They have all the conditions fresh in their minds and are open to new info.

I will be pulling Maya duty tonight while Victoria stays with Ronald. She may update www.flickr.com/photos/gorskipics from my laptop back in the RMH room. To contact her try her cell or e-mail Victoria.Gorski@gmail.com. Also videos are posted at http://video.google.com/videosearch?q=Chris+Gorski&num=10&so=1&start=0

-Chris

Pictures of Maya post-op

Brianna look - Maya has a stegosaurus blood pressure cuff - how lucky!

Here are pictures of Maya's war wounds.

To contact Victoria please use her new E-mail address: Victoria.Gorski@gmail.com

Or for Chris the good ol' Seekerhiker@hotmail.com

She got to suck down 1.5 ounces of milk tonight like she hadn't had a drink for days - she gulped like crazy for about 1 minute, then her pain was too great so we laid her her back down (we held her up to bottle-feed) and she was given some morphine. No opening of the mouth after that.

2nd update on Maya's surgery- 9pm Friday

Victoria here... it is almost 9pm, they still haven't let Maya have milk yet. The politics of the hospital and the CYA. The NICU RNs won't do anything unless it is written in a work order from the Dr. It is interesting to watch the pecking order, lots of different RNs and MDs and which ones can actual make decisions vs. others and the conflicting information they say. Like one said a few hours after surgery she could eat, now we have to wait until after 8pm and when she is alert and wanting food.

The results of the xray showed fluid in the chest cavity, according to the NICU RN, that is normal and they are not yet concerned. They are watching for respiratory distress, which she is not displaying. The next xray will be in the morning.

I am staying at the hospital again tonight. They have a sleep room for me again and I intend to go to bed earlier tonight. Chris is staying at the Ronald McDonald House again tonight. Tomorrow night, Chris is going to have hospital duty and I will stay at the RMH.

Chris and I did manage to get out and grab a bite to eat outside the hospital tonight. We ate at a whole in the wall Indian restaurant. There were a lot of security guards on the UPENN campus tonight. We think there is a big sporting event or something going on. They were searching one man's suitcase outside one of the other hospitals... strange. But it was nice to be out of the hospital and spending some time focusing on something else for a while.

Next update tomorrow morning...

Maya came out of surgery doing great!

This is Victoria. Maya was finished with surgery at 3:45pm. The surgeon met with us and told us the surgery went very well. He was able to complete it laproscopically vs. opening her up. He told us that the tumor was actually on her middle right lobe vs. her lower right lobe as they had originally thought. He said the tumor was large and hanging down blocking the view of the good lower lobe. So Maya's upper lobe and lower lobe will grow and fill in the space where the middle lobe would have been. They said there was no issue/complications from the cardiac issues (see previous blog on PDA/PFO issues).

Next steps... Maya currently has a chest tube in which has suction on to drain any fluid and/or air in her chest cavity. Later tonight they will do an xray of the chest with the tube in to see if there is any fluid or air in the chest cavity. Tomorrow if all is going well, they will remove the chest tube and do another xray to ensure there is no air and/or fluid in her chest cavity. She is currently on IV fluids and may be able to have milk tonight. They are monitoring her and if she is fussing, they will give her a bolus dosage of morphine for pain. Right now she is not fussing and is probably still on the pain drugs from surgery. They are also giving her humidified oxygen via a tube on her nose to help with the dryness in her throat from where the breathing tube was. We do not yet have an estimated discharge date yet. We will know more after the chest tube is out. We do know that they want her on antibiotics until Sunday as a precaution for the PDA/PFO as well as the chest tube.

If we get news from the xray before too late tonight, we will do another blog update today. Otherwise our next update will be tomorrow. That is unless Chris feels inspired to add on to the above ;-)

Thank you to all for your healing and prayers... they worked!
Love you all
Victoria and Chris

Maya has gone in for surgery- 11:30am

Just a quick update to share that Maya has just been wheeled in for surgery. She had a bit of a rough night last night according to the nurse, she cried a lot. (I went and slept in a sleep room from 1am-7am) They stopped letting me feed her at midnight.

We expect her to be out of surgery anytime between 1:30pm- 4:30pm. Once we get word, we will update the blog. They have PC access in the NICU for families, so since she will be recovering in the NICU, we will be near enough the PC to give an update.

Please send healing and prayers. Thanks.
Victoria and Chris