Maya and Chris made the Atlantic Online

Maya has been suffering since last May with bouts of C. difficile (mucus diarrhea) both on and off antibiotics.  We supplement her with chewable probiotic tablets and sprinkle the contents of Garden of Life  Primal Defense Ultra Ultimate Probiotic Formula on her morning oatmeal, and the results are good, but sometimes the C. diff. happens anyway.  


Researching treatments on the internet, I have found that the protocol the CDC recommends is barbaric and has very low success rates (40%, only measured up to one month).  They are heavy regimens of antibiotics that cause other horrible side effects and cause a vicious circle. 


After remembering reading an article mentioned by my friend Waldo on his www.waldo.jaquith.org blog, I researched the topic of fecal transplant to move healthy gut flora to the gut of a sick person.  After testing my poop in a lab and finding it Grade A, we have made the choice that should Maya get sick enough, we will use my stool (processed as recommended) to infuse via enema into Maya.  The issue is that we must do it ourselves, as doctors are barred from performing this inexpensive treatment in this country.


Here is the article:  http://www.theatlantic.com/health/archive/2012/01/the-poop-outlaws-at-home-fecal-transplants-to-cure-disease/251793/

Maya gets tested for PCD at UNC Chapel Hill

Maya's self portrait doodled at UNC Children's Hospital

Maya and I flew down to UNC in Chapel Hill, NC thanks to the generosity of Angel Flights, who set us up with tickets from Hartford to Raleigh-Durham Airport in North Carolina.  At the hospital the first test Maya had was called a nasal exhaled nitric oxide test.  Using this test it was determined that Maya did not qualify for the five-year longitudinal study of Primary Ciliary Dyskinesia.  Most people with PCD has low exhaled nitric oxide, Maya did not.  She still went through all of the other tests required of the study of testing methods for PCD, but needless to say I was quite relieved to find out she most likely does not have this genetic condition.  We will receive word if one of the other tests points towards PCD, but they can take up to six weeks.

So why does she still get sick so easily?  No good answers, but I am at war with mold in our house by dousing the back of each a/c unit with bleach water every other day and eliminating any standing water in sinks, etc.  Just a hunch that mold may have a role.  She has just come off of Clindomycin and we are hoping no illness occurs anytime soon!

-Chris

Kids Say the Darndest Things...

On Sunday, while Victoria was putting Maya's swim clothes on at the local beach, Maya said, "Look, my bum is getting bigger, just like Daddy!"  Victoria, Brianna and I all had a good belly laugh together, and the father nearby had a good chuckle as well.

Maya gets to be part of a research study

The data we received from Maya's visit to Immunology in Boston was negative.  On everything.  She's clean immunologically, and no Chronic Granulomatous Disease (CGD) either.  The last suggestion the Immunologist had for us was to get her tested for PCD, or Primary Ciliary Dyskinesia.   

It turns out that it is not so easy to get tested for this rare (1:15,000 to 1:22,500) disease, and in fact there is no currently accepted method of testing that yields a conclusive result.  Rather there are a battery of tests that are being researched to yield a diagnosis, and also a host of possible genetic links that are being looked at as possible inheritable markers for the disease.

What is PCD?  Well the cilia are the little hair-like structures that are supposed to beat in a rythmic pattern of from 20-60 times per second or so and in concert with each other (think of "the wave" performed at a sport event in the stands).  These cilia line the walls of the sinuses and the respiratory tract, and are also found in the fallopian tubes (women) and sperm (men), inside the kidneys and even a few in the brain stem that have the task of regulating the cerebrospinal fluid pressure in the brain, as well as filtering germs out.  PCD is considered a mucosal clearance disorder, because the cilia are responsible for moving mucous, and the foreign substances in it, out of the sinuses/lungs.  If one has a disorder in which the cilia either do not beat at all, beat in an uncoordinated rhythm, or are in any way structurally deficient, then mucous and germs build up in the nooks and crannies of the body that the immune system cannot effectively target.  This makes the victims of PCD germ factories that are very susceptible to respiratory viruses as well as other diseases that are normally not a problem for unaffected individuals.  It is considered a less serious, but similar in progression and treatment to Cystic Fibrosis (which Maya was extensively tested for and does not have).

So we will be heading in a few weeks down to a respected research facility in a University town in a state that extends from the Smoky Mountains to the barrier islands commonly known as the Outer Banks.  Maya will be involved in the research of actually finding a combination of testing methods that yield an accurate result, and if positive may be elected to take part in a separate lifetime study of PCD patients and treatments.  There will be lots of genetic study involved to track down responsible genetic markers, so family members - be prepared to shed some blood for science please!

Maya is happy otherwise and she and Brianna just returned from a wonderful 5-day trip to visit Grandpa Gorski's house in Indiana.  They had a great time playing with Heather and Jared, tending to the chickens and playing with the dogs and cats as well.  They also spent lots of time outdoors enjoying the large yard with lots to explore.

Latest medical mystery...

Maya's reward for going through bloodwork in Boston

In November we took Maya back up to the Children's Hospital of Boston, where she visited with two specialists to evaluate Maya's continuing sinus infections/ear infections/bronchialitis. The first specialist was with hematology, and he ordered a test for Chronic Granulomatus Disease (CGD), which fit with her medical history. We also met with an Immunology/Allergy specialist who ordered an array of tests which included her protective titer responses to her childhood immunizations.

Maya screamed bloody murder when the lab technicians (one of whom looked like Moby [the musician, not the whale]) held her arm still and inserted the needle for the blood draw. Forewarned that Maya was very hard to draw blood from, and prone to "blown" veins, "Moby" did an awesome job of getting her on the first stick and she actually calmed down and was able to say thank you to the lab techs when I prompted her to before leaving the lab.

One of the issues we spoke with the Immunological specialist about was her strange response to Levaquin and Bactrim, very strong antibiotics that work well for her. She get strange red bumps dispersed in clusters on her body, like a mild case of chicken pox. She had them on her while we were there, but they were disappearing. The Dr. said we should try Bactrim again, which we did after her bumps disappeared the next week and while she had a runny nose again and sickly symptoms. Sure enough the bumps returned in 3 days. The Dr. (via a phone call) changed her to another antibiotic in the ceph family at a "prophylactic dose," at the same time he asked for her to get re-immunized for her infant Prevnar and HiB vaccines, as enough of her protective titers from those vaccines were low enough to not provide her good protection against those illnesses. He wanted her to return for testing one month after receiving the vaccines for retesting. We were also informed that her test for Chronic Granulomatous Disease (CGD) was negative, and her red/white blood counts were normal.

So she got the immunizations, yet continued to be ill with sinus drainage and occasional low-grade fevers, even a case of pink-eye. Of course she became mostly clear just before visiting Boston last week for a consultation and re-testing. I should mention that during the days prior to the re-check I also treated her with some of the "cold-killer tea," albeit at a weaker concentration than usual, liberally sweetened with agave nectar, and mixed 40/60 with milk. I also used a NeilMed Nasal Irrigation device on her during the time between the visits, with a lot of nasty mucous coming out with each use. She actually thanked me after the irrigations, saying she felt better each time. So the net result was that she was still a bit "gunky" sounding at the re-check visit, but her nose was clear.

The visit last week to Boston was not very enlightening. The specialist repeated the previous tests, and added AH-50 and C3 and C4 levels, while telling me that he suspected her immune system was normal and that we should return to working with her Ear, Nose and Throat (ENT) doctor to check her out for anatomical issues. I explained to him that we had now come full circle, as it was the ENT specialist who recommended the Infectious Disease specialist in Hartford, who then recommended the Immunologist in Boston. He explained that from an immunological perspective she seemed within range of normal, and that he expected her follow-up blood work would confirm than her immunity was ok. He strongly recommended that we have our ENT perform a Ciliary Biopsy to check if her cilia (the little hairs in various parts of the body, especially sinuses and lungs that pulse in waves to move foreign matter and mucous out of the body) were properly working. He also upped her Ceph dose to the strongest possible.

We had the labwork performed by the same technician, whom I now call Moby, and he again did a spectacular first try draw from Maya. Smooth as Porcelain.

Upon my return from Boston, I emailed the ENT and brought him up to speed on Maya's continuing infections and what had occurred in Boston, along with the request for the Ciliary Biopsy. The Immunology specialist explained that a ciliary biopsy was a simple test with a brush a bit up the nose that scrapes off some cilia that can be examined under a microscope. Maya's ENT emailed back that the nasal scrape biopsy has been proven to be clinically useless, and let me know that to even have a 50% shot of a proper biopsy result Maya would need to go under general anesthesia and a sample would need to be taken via a bronchoscopy, not the nose.

Just today I was contacted back by the Immunologist, who had not yet received my email exchanges with the ENT, nor had he been able to win the first round of phone-tag with the ENT. The Immunologist explained that all of Maya's tests, with the exception of the AH-50 (which would take 2-3 weeks longer) came back normal, with excellent protective titers for all of the Prevnar-related pnuemococcal strains and the HiB. He said that from his perspective, her immune system responses were normal, with perhaps a bit of allergic response that doesn't show up in allergy testing. I agreed with that, because Brianna and I get the same allergic symptoms ("hay fever") to mold, ragweed and a few other things several times a year, with the difference being that when it progresses to an infection, Brianna and I respond positively to our first antibiotic, while Maya responds only to incredibly strong antibiotics, which happen to also give her bad diaper Candida rash and, in the case of Bactrim and Levaquin, body bumps.

The Immunologist said he had no explanation for her lack of response, other than suggesting some sort of ENT issue, such as the cilia. He said I should now work with the ENT with regular checkups every six months. When I asked if she should be rechecked annually for the same protective titers she needed a booster for, he said that we should, and that annual boosters might be helpful if the tests indicated lowered titers again.

So now we are left wondering why she is basically clear right now and for the past week (probably the Ceph, as she has finally developed her nasty diaper rash), and what to do to keep her clear in the future. It is very frustrating to have to "wait and see" with no real answers to very serious recurrent infections that often keep her home and unable to participate in daycare or even visiting people or playing outside. While I don't want Maya to have to undergo a surgical procedure, nor do we have the easy funds to pay for it (at least the portion that the insurance doesn't cover in our "donut-hole" period), I really feel that the seriousness of what might be Primary Ciliary Diskinesia (PCD) needs to be discovered or ruled out, so that we can have a plan for her treatment and know what to expect for the future. On the flip side, she may just have back luck and be a sickly kid who will grow out of it sometime in the next few years. Nobody can answer that with any certainty. There are many symptoms of PCD that Maya exhibits, and there are even possible genetic links with diseases associated with PCD on the maternal side. So for now the mystery continues and life goes on.

And of course, please donate to and pray for the Haitians and those helping them!

-Chris

Fellow CCAM -type III baby with spontaneous resolution!

I am so pleased that a family in Australia that we have been corresponding with has given birth to a beautiful son, full-term and with no CCAM! They contacted us for support during the pregnancy when they discovered their future son had a Type-III CCAM (same as Maya), and discovered us by this blog. We followed with them anxiously as their future baby's CCAM grew to a CVR of 2.0 at one point, which is getting dangerously large. We prayed for his health, along with many others I am sure, and by the time of birth his CCAM had resolved/spontaneously disappeared! This is especially good news as there have been very few CCAM resections in Australia, and there are no facilities that can treat an imminently imperiled fetus in the womb.

So congratulations to Sean, Emma and Jack!

Now any prayers for Australia can turn back to focusing on rain to quench their terrible drought!

Daddy likes ladies...

The quote of the day from the increasingly eloquent and observant Ms. Maya is as follows:

Maya (while sitting on Mommy's lap at the dinner table): "Daddy, you like ladies."

Chris, Victoria, Uncle Ryan: (snickers)

Maya: "Mommy's a lady."

Daddy: "Well then that's a good thing that Daddy likes ladies!"

Cats take medical center stage recently!

This past week has actually taken more time and resources (money) to address the medical issues of our cats than it has for Maya! That takes some doing.

First we had an appointment to have our oldest male cat, Hunter, taken in to follow up on some recurrent mouth ulcers he has had. Well he did a Houdini that day and managed to avoid being found for the appointment. However we had some concern with Acadia (Cadi) being too skinny thanks to the objective eye of Victoria's mom, who remarked that she was too skinny. As we are with her every day, the change was too gradual to notice. So anyway, Ryan (Victoria's brother) took Cadi to Hunter's appointment and the vet weighed her, finding her to be only six pounds! She was ten pounds at her previous check-up and had very little deviation in weight for the previous 11 years of her life, so this was a huge deal!


The vet ran the usual battery of tests and called us with the result; Cadi has diabetes. After the initial shock, which was lessened by the memory of giving my sister's cat diabetes shots when she was away several years ago because he had the same condition, I researched the issue and found that the insulin available nowadays is cheaper and better for cats, so more relief there. Then there was the issue of expense. When I met with the vet for a tutorial on how to administer the shots and monitor her I was presently surprised to find that the visit and several months worth of supplies was less than $100. The only really expensive item is the special minimum 50% protein diet she needs to be on, which costs a good 50% more than other premium cat foods. Since we free-feed all of our cats from an always-full food bowl of dry food, we need to buy this same food multiplied by five, as segregated eating is not feasible.


So then Hunter's rescheduled appointment arrives today. He still has mouth sores and the vet is concerned that they have not resolved. Although he was vaccinated against Feline Leukemia, he was retested for that as well as for kitty AIDS and a complete battery of blood and urine tests. Everything came up normal except for some slightly elevated BUN (Blood Urea Nitrogen), which was followed up with a urine test that did not confirm any renal issues. Most likely the slightly high reading was due to eating the high protein food that we have to get for Acadia. Since all of those tests did nothing to explain his mouth ulcers and the vet was stumped, she sedated him and took two samples (biopsies) of the ulcers from his tongue and is sending them out for analysis. It may take until Monday to get results from the biopsies. The other good news from the tests is that Hunter, although a litter-mate of Acadia, has no signs of diabetes or any other disease.

All of this cat news is not to be taken that Maya has not been the subject of any medical concerns. On the contrary, we had an appointment with the Infectious Disease doctor at CCMC, who was unable to give us any answer for Maya's repeated sinus infections that only respond to Levaquin and was only able to point us to Yale or Boston for an Immunologist (which we actually thought he was going into the appointment, go figure) and give us standing prescriptions for Azithromycin and Levaquin and tell us to use them as needed when new infections arose, but only until symptoms were contained, not for a full period as the doctrine is drilled into our heads regarding antibiotics.

So after both an internet request and a follow-up voice mail request we were contacted by the Hematology department at Boston Children's Hospital and were told that we needed to get medical referrals from our Pediatrician for both their Hematology and their Immunology/Allergy departments, as her possible issue(s) span both disciplines. We were also told to gather every lab, record, study and note from every doctor who had ever looked at her and send them to Boston prior to any appointment that will be set up. Good thing we have most of that work already done and just need to update some scanned PDF files of recent notes and labs to get the CD's up to date and send them up soon.


Now for the day-to-day news. Brianna has had a great summer at Brodie Camp and is becoming a very confident swimmer. She is very darkly tanned despite our daily application of 50 SPF sunblock prior to her 5 and 1/2 hour camp day, but is very fit looking and feeling due to her daily activity level. I dread the switch back to school when her activity level will be only a fraction of what camp provided! She also has made new friends and cemented current friendships and should have no social problems starting first grade in a few weeks.

And one other thing - she has lost her fourth baby tooth! They all were from the bottom center four and the two center ones are growing in and are about 1/4 in already. The one on the right is somewhat behind and twisted from where it should be, so if it does not improve quite a bit she may be headed for braces to correct it in the future.

Maya's sophistication in speaking is continually amazing. She says sentences very precisely and with the complexity of someone much older. That is unless she's overtired or wants something. If you're lucky at these times she'll warn you, "I in a mood." Most of those times she'll just scream, cry or whine or maybe call say to you, "You bad *insert boy. man, girl or lady here*." This is of course funny but we have to withhold a smirk and tell her that is not nice to say.

Maya looking like a toddler Ted Nugent - dig the necklace!

She also enjoys puzzles, picking raspberries, playing sophisticated imaginative games with her dolls and toys and taunting her sister, which is actually a mutually enjoyed sport, much to the frustration of their parents.

Victoria has been working a lot lately but will be getting a little break to have a Peace Corps Ecuador group informal reunion in San Francisco at the end of the month.

I have been continuing job searching, which has been about as fruitful as looking for a good time with the opposite sex in Provincetown during Gay Pride week. (It's just a joke, I'm a nutty left wing liberal whose on your side people, so no protest e-mails please ;) ) I've even opened up my search to much lower-paying professions that I would enjoy, such as motorcycle/scooter sales and/or repair, to no avail. It's tough out there people!

Bye for now,
-Chris

Language complexity, swimming and health stuff...

Maya was laying in her bed with me next to her tonight and she was talking to me about all sorts of things. She noticed my bracelet and she said "Mommy who give you ring?" I told her it was a bracelet and asked her who she thought might have given it to me. She said "Grandpa Jack?" "Auntie Cole?" and I told her no, Richard gave it to mommy. She then proceeded to tell me "Mommy next time talk to Richard get Maya bracelet". Then she said, "sissy big big girl lose teeth." I told her yes, and that the tooth fairy took the teeth and brought sissy $10. At that her eyes got big and she said "sissy big big girl buy bracelet money." Then her eyes got big again and she said, "Ask Richard money buy bracelet Maya big big girl." The complexity of her language is just growing in leaps and bounds!

The other feat for Maya today was that we were in the hot tub tonight and she swam under water without taking in water in her lungs. After that, she keep dunking her head under and up and laughing.

As for Brianna...she has been amazing us these past few weeks with her ever-evolving swimming skills. First it was 2 weeks ago that she first learned to doggie-paddle without the life jacket. Then this past weekend she went underwater for the first time. She quickly progressed to swimming under water with and without goggles. Then yesterday at camp, she jumped off the dock (with a life jacket), but that is huge for Brianna. And tonight, she did the back-float in the hot tub without me holding her. It is amazing to watch her and you can see the gleam in her eyes when she realizes that she can actually do it. Brianna had another big event today at Taekwondo. She earned a trophy because she has 3 medals now and 9 stars for her uniform. She will be taking the test to become a blue belt soon.

-Victoria

Chris here with a Maya health update- Maya is just getting over a sinus/ear infection that she has had for nearly two months. She is taking Levaquin, which is an antibiotic of last resort after being on Augmentin ES, Ceftin, Zithromax and then Clindomycin, none of which helped her in the least. This is one of the same old problems that we hoped would be addressed by the PAPVR surgery, but it looks as if it is truly an immune system problem, complicated by allergies to, among other seasonal things, mold. The allergies, which are mitigated by Zyrtec, but not enough for the worst of high allergen level periods, proceed to post-nasal drip and sinus infection. The same thing happens to Brianna and I at the same time, but antibiotics work on the first try for us both. Maya unfortunately either has worse bacteria that take hold, or some compromise in her immune system that allows the same bacteria that are treatable in Brianna and I to stay alive in her body. We also tried nasal cortisone sprays to reduce inflammation in her sinuses, but they lead to bad nosebleeds in her, as does dry air, so they cannot be used.

Last week she had a sinus cat scan that confirmed sinusitis swelling that was pretty bad. When I discussed the possibility of a fungal infection the Ear, Nose and Throat doctor said that he would have seen the characteristic "spotting" pattern of fungal infections on the cat scan, which he did not. So now we need to pursue the immunological cause of her recurring infections, which the Levaquin is actually working very well on, but it is a focused antibiotic so that may give us a clue as to what particular Immunoglobulin (talking out of my butt here now, not enough research yet) is low in her system and why. Ironically we had a through panel of immunological tests pursuing the same answers last year (when her infections actually went away after Zithromax combined with my cold-killer garlic tea, which she absolutely refuses now), and will now need to delve even further down this road.

Luckily she is very healthy and strong otherwise, and her infections seem confined to the sinuses.

Brianna loses her first tooth, uh, teeth - May 30 2009

Brianna loses her first tooth, uh, teeth - May 30 2009 - 2
Originally uploaded by SeekerHiker

Brianna had a fiber wafer this AM and got a surprise - the tooth we were waiting to have fall out any day finally did so. Its neighbor was just as wiggly and bleeding, so Brianna let me give it a gentle tug out as well - a real two-fer! She was a bit melancholy at loosing her teeth as seen in the video, but she cheered up just fine and we had a good day taking a motorcycle ride to Campbell Falls and playing in the water.

Maya's first exposure to death...

Yesterday we (Brianna, Maya, Chris) were driving down Rt. 8 to a party for Brianna's friend Emma. We saw a brightly colored bird behaving strangely on the breakdown lane. I stopped the car and backed up to a bird we later discovered was a juvenile Scarlet Tanager. I had never seen one before and both Victoria and I later agreed it was the most beautiful non-captive bird we had ever seen.

I had to direct traffic from the right lane into the left as when I first approached the bird it made a lame attempt at flying and ended up in the middle of the right lane. I herded it back to the breakdown lane and on my first attempt, caught it with a two handed cupping motion. It didn't struggle much, but seemed fairly lively except for a downy black feather stuck in its beak and black gunky stuff along both sides of its beak.

We stopped at a BK drive-thru for some lunch and waited in the bowling alley parking lot for the party to begin. While there I was able to coax the bird to drink some water from a bottle cap and I used a napkin and water to clean its beak. Maya and I got out of the car and caught a carpenter ant to feed to the bird, and after a few times worth of flicking it out of its mouth it finally ate it. We then left the car windows open, and as it was an overcast day in the low 70's we were unconcerned about heat for the bird. We put it in a BK bag on its side with the cap of water in there and the bag lightly folded over and the bird was fine when we left the party.

Driving home, the bird fluttered about in the bag and all seemed well until we got home and went to show the bird to Victoria, who was heading up a tag sale at our house. It had the strange curl of its toes that we knew was bad from when we tried to nurse Latka, our baby Blue Jay from two years back. It also would not stand and flapped limply. Within minutes it gave a strange arch to its back, as if taking one last great leap into flight and died in my hand.

Upon a brief examination and using deductive reasoning it seemed to have probably suffered a hit into a windshield, a glancing blow that gave it moderate internal injuries and some slight external bleeding. He probably picked at the external site and got his beak bloody and a feather stuck to his beak at that point, then regained enough strength to hop around dazed. After we picked him up he continued to slowly bleed internally until his death about three hours later.

Brianna said we should bury him and I agreed. Maya wanted to help. I dug a small hole and placed his little body into it and we explained to Maya that he was dead and his body did not work anymore. We told her his spirit was free now like an angel to go fly with other bird friends in heaven. She seemed cool with this and said bye bye Tiki (oh yes, she named him while we were at the bowling alley birthday party), then she used a child's hand shovel to gently put the first bit of dirt on him. Brianna and I finished with the dirt and then Maya produced a beautiful handkerchief she pilfered from the tag sale and she refused help as she carefully covered the grave with the handkerchief and placed stones on it to hold it in place. She then scampered off to play without a care.

Later on that evening, Brianna took a bowl of water and a spoon and said she was going out to do something for Tiki. I saw her sprinkling the water over the lawn with the spoon and I asked her if it was an offering for Tiki. She said yes, and that she hoped his friends would come visit. Kids can be so deep.

This morning Maya went to the back door and said to Victoria "Bird sleeping dirt." Victoria explained that yes, his body was sleeping forever.

Maya is getting too darned smart and funny, so quickly!

It seems like right in front of our very eyes, every day, Maya is becoming more and more of a little individual who is articulate, independent and funny. This past Sunday Brianna decided she was too mature to go see a live "Blue's Clues" show with Grandma Dayle, Auntie Nicole and her cousin Savannah. Maya was more than content to take her place! According to Grandma, she was enthralled by the show. This means her attention span is now long enough that she can go without a screaming fit to leave for at least an hour and a half - woo hoo!

The day prior, Brianna spent the afternoon with her friend Ali. As it was a gorgeously warm day, Victoria and I decided to try a motorcycle ride with Maya. Maya was wary at first about putting on the confining harness and helmet to allow her to be safely attached to me, but it turns out she was just cranky tired. After we got underway Maya promptly fell asleep and I led Victoria to the Dewey-Granby Oak I recently was amazed to see on a shortcut that my TomTom led me through in Granby. We parked the bikes, awakened Maya and she spent a good half hour playing on the tree and checking out the property there. We then rode to a nearby roadside food stand, had some sodas and snacks and then rode Rt. 20 around the Barkhamsted Reservoir to the part of the Compensating Reservoir nearest our house, where Maya got "nakie" and waded in the chilly water before we headed around the corner for home.
The photos and short videos of our Dewey-Granby Oak tree visit are on our Flickr page here.

Maya's language skills blossoming!

First of all, we officially announce that there will be no party for Maya/Brianna family/friends (the one that was previously just postponed due to chicken pox). We have decided that there is just too much going on. Sorry for the confusion, but Brianna already had three parties and Maya had a small one and she's good with that.

Now that that news is announced, let me tell you Maya is talking much more now. Still not near peers, but making progress. In the above video she uses her improved pronunciation to play a game in which she makes you close your eyes, then she goes and brings her wipes box and says, "Open Eyes!" You then must act very surprised. Great fun the first dozen times in a row...

Brianna is now a full Green belt in Tae Kwon Do. Her friend Grace has started taking classes as well and Brianna is so excited to have one of her friends in class again. Her friend Isa used to be in her class but "graduated" to a more advanced class a while back.

Brianna is also doing excellent in Kindergarten. She is reading at between levels 3 and 4 and writing sentences phonetically in a journal.

Below are some recent pictures of the kids:

Below is a picture of my Great-Aunt Edna taken by my mother recently with one of her great grandchildren. I love the spry energy she is giving in this picture. Definitely one of my new favorite photos.

Tiny Family B-day for Maya - Uncle Ryan now has the Chicken Pox!

We had a nice little party with Grandpa Jack and Grandma Dayle at our house for Maya's actual birthday last night. The main family/friends B-day is still on hold pending complete freedom from the Chicken Pox virus at our house.

Despite the unlikeliness of anybody getting Chicken Pox nowadays with the immunizations that kids have been getting since 1996, it seems that Victoria's brother, Ryan, who has been living with us, has developed a mild case of Chicken Pox as well, thus confirming that Maya did indeed have it. Researching the incubation period, it seems to be around two weeks. This interestingly puts the start of Maya's symptoms about two weeks after our return from Walt Disney World. Isn't airplane travel fun? A new surprise every time!

Ryan did have the pox as a young 'un, and thus his current case is mild with just some minor itching. We are waiting for his case to "crust over" or turn dark enough red and with no indication of new marks or itchiness before rescheduling the family/friends party for Brianna and Maya. What an extended birthday season this is turning out to be!

Plenty of photos posted up at http://flickr.com/photos/gorskipics

Birthday party cancelled due to...Chicken Pox???

This past week we've been seeing little bumps on Maya that looked like various sorts of bug bites. We were unsure of their origin (Victoria and Brianna insisted on labeling them "spider bites" although there are no visible spiders in our house, no one else had bites, and spiders biting humans is for the most part a defense mechanism, not a way to feed. Well after Brianna's Little Princess party on Saturday, we found more bumps and one that was round and looked like a pustule that you would see with poison ivy. With winter in our area and all, poison ivy was kinda unlikely, so we looked on the 'net for possible reasons and narrowed it down to a mild case of Chicken Pox (last week a low-grade fever, current runny nose, no other symptoms).

"But wait," you say, "Don't they immunize kids for Chicken Pox nowadays?" Sure they do. But a small percentage of those who get immunized can still get Chicken Pox, and most of those are a mild case. Always read the fine print!

So Victoria spent a good part of the night calling people to cancel the family and friends Brianna/Maya party planned for Sunday, and then calling the people who came to Saturday's party to warn them of their Chicken Pox exposure. Sorry world! I have no idea where she got it from, but if anyone is likely to get a rare medical condition, it is Maya. She is as happy as can be anyway, and seems to be unbothered by the pox.

Right now I am blogging from a surgical center as Victoria has a minor procedure performed. I've got my healing thoughts going for a minor non-event...

-Chris

Our girls are growing up fast...

It is now January 13 and I am writing as I lie here next to Brianna as she sleeps sick with some bug. She threw up at least seven times since waking up this morning and this is the first day she has missed school so far. For all that she seems to be taking it rather well. She is getting so mature! She can read basic books now with very little assistance. She is into Hannah Montana, which is purely via peer influence as she has never seen Miley Cyrus in any program or movie. She also is developing nurturing skills since getting kittens for Yule/Christmas.

The kittens were rescues from New York City in the municipal pound where they kill fairly quickly. The rescue organization in Stamford had the little brothers neutered and up to date on all immunizations. They were twelve week old tuxedo coat mixed breeds with upper respiratory infections and ringworm fungus patches when we got them. The organization was great in supplying us with medicine for free however, and they are in almost tip-top shape now. Brianna named "hers" Boston, short for the Red Sox, and Maya named hers "Baby." Baby is slightly more outgoing than Boston at the moment, and Brianna enjoys cradling him and toting him around like a child. He loves the attention and she loves the fact that he allows her to lug him around. They both have Siamese in them and purr, talk and play a lot. They are also natural fetch and retrieve cats, playing fetch with no training and bringing toys right back to the thrower.

On to our other little love muffin, Maya. She is healthy at the moment with the exception of some out of range labs that we are investigating. Her stomach "G-tube" was accidentally yanked out by me one night in late December when I grabbed her by the front of her pajamas in her crib to pull her up to change her. I felt and pop and she cried for about 20 seconds. Of course I rushed her into our bedroom, opened the pajamas and found the mushroom end of the tube now outside of her body. Victoria called the GI doc on duty and he said not to worry, it might leak a bit for a few days but should close right up. It did not leak and did close right up and we were able to avoid the cost and trauma of sedating her again to have it removed by her GI doc, which was scheduled for January 16. She was so relieved to have it out and still proudly lifts up her shirt, points to the tube wound and says, "Gone - Happy!"

She has had a few bouts with sinus infections since the last post but is clear right now. She also has had several episodes of nosebleeds, similar to a pattern that Brianna had and still occasionally does. My solution is to give her some Nasonex nightly during the dry air months and keep a humidifier on in their room - works 100%.

In other news I have been unemployed since December 19 and we have gone into tight budget mode. The kids are down to two days a week at daycare, the minimum to keep a slot open there. I take care of them the rest of the week all day except for Brianna in the afternoons with Kindergarten. I hope this is very temporary as they deserve the peer interaction and the professional structure and projects at the daycare facility, but work in my computer field is hard to come by and devalued to 1/3 the wages I could get a few years back. At least I am not alone as many others are in far worse shape in this new "near-Great Depression."

We also have Victoria's brother Ryan staying with us in Maya's former room. The girls now share Brianna's room and they enjoy Uncle Ryan's attention. Ryan will be attending courses at Northwest Community College this semester and is doing that unenviable twenty-something process of figuring out what to do with his life. Unfortunately for him and his generation, the opportunities are poor right now. Hopefully by the time he lands a degree the prospects will be much brighter!

Brianna, Maya, Victoria and I along with Nicole, Eiko, Savannah and Jake are being taken to Disneyworld at the end of the month for 4 days by Grandpa Jack! Brianna is so excited! We looked up which rides she can ride at the Magic Kingdom and Epcot and she is tall enough for all of them - the requirement is 40" and she is 46" - woo hoo! She even can ride Space Mountain and she is mega excited to try roller coasters! We hope for good weather and no illness in anyone for the duration of the trip.

The next post should either be about Disney or about the results of Maya's latest rounds of tests to figure out her abnormal lab findings.

Here's to hope and peace starting January 20!

-Chris

Maya's second and Brianna's sixth Christmas coming fast!

The picture above is about two months old now - it was taken by the photographer who works with the day care center. Maya's face is still fairly rash red from the NG tube and tape that had been removed a month or so earlier.

So here it is almost Christmas. Brianna is getting so mature - Hanna Montana has captured her attention and is the latest fad for her. Maya seems to gravitate to Arial from "The Little Mermaid" as her own personal idol.

Maya has been fairly healthy, but we have discovered that her blood work indicates she is hypothyroid which she will have further work to determine if it is consistent and perhaps what the cause is. She also just developed a triple case of conjunctivitis, sinus infection and ear infection which the doc gave her amoxicillan for. That's the weak stuff but maybe it will work this time, we hope.

We are also trying to remove the "binky" steadily from Maya, and boy is she going through some miserable withdrawals. She chews on cup lids, straws, even her fingers and can cry pathetically for long periods without her binky. We let her have it to sleep and now when she's so uncomfortable from being sick.

So our house is being affected like the rest of America - I have lost my contract job and will soon be on unemployment. Looking for work is an exercise in masochism - all of my skills are valued at 1/3 what I am accustomed to making at them. I need to rework myself into something useful that doesn't require expensive schooling. Any ideas? Perhaps welding so that I can build bridges when the "Infrastructure" funds come through under Obama? I applied for the Census Bureau (coming up in 2010 - some jobs available now), aced the test so that I am qualified for the Supervisory level, but learned that pay tops out at $20/hr. I just finished making $32 for way less responsibility. That sucks. Jobs for PC techs without many levels of certifications and programming skills are at $10-16 per hour. Bad, bad. I really hope my case goes well with the State and I get my job back. That would be a very bright spot in a very bleak near-mid future.

Also many friends and family members have lost jobs, and the list grows daily. A good friend now has worked 2-3 jobs at a time for months, 75+ hours per week, and is still going to file for bankruptcy and her family is going through hell. A co-worker (former now) is about to file for bankruptcy. A good friend's new girlfriend has filed and has left CT to move in with him across the country to try to start fresh. It is hard to feel optimistic in these circumstances, but we must try and remember to have gratitude for what we DO still have and compassion for those who have less. Charity is more important now than ever, and we need to get used to a reworking of capitalism and what the American dream means after the investment economy has failed us.

Enough with my political diatribe. Take care this holiday season, treasure your friends and family and learn to appreciate the little things. And drop guilt - if you can't afford gifts, flat out declare it to the world (we have) and take control of your finances - giving to real charity when feasible. Please pray, meditate upon, project positive vibes toward, focus your will upon (whatever works) peace and happiness and a renewed hope and vision for humanity.

With love and best wishes for those who still have secure jobs, and better wishes for those that don't that something better will appear for you,

-Chris

The Cute Little Tyrant

Maya has been growing into a toddler more every day. She now has about a 30-50 word vocabulary and loves to say "Hi" and "Bye-Bye" to people. She can go from being extremely affectionate and caring to a little comedian to a sneaky little bully in a short period. She has been affectionately written up at day care as perhaps training to be a boxer. The worst target of her random hits is her sister. I think Maya gets a kick out of Brianna complaining to us that "Maya hit me again" or "Maya hit me in the face." Maya then is subject to a time out in a corner, which she goes and takes willingly. We started out counting to twenty, but now we count to fifty and she canot have her binky during the time out. She then is required to "say sorry" to the person hit and give a hug to that person. She also does this willingly and affectionately. It is very cute how she "says sorry." It is not verbal, but she made up her own sign, which is a sort of shrug to her right shoulder, with equal up movement on the shoulder and down movement from the head. I have it on video and will get it up here eventually.

Medically she has been stable, with the exception of her stomach peg tube. The site needs 2x daily treatment with a corticosteroid cream to prevent the development of a granulomatous wound. Even with the cream the site still looks red and pusses a bit. She can speak and drink well now, but she still has the tube in because we have been told that it would be dangerous to remove it before mid-January, as there is risk of stomach injury if the stomach has not fully adhered to the abdominal wall yet. So we still watch the tube closely and it still is an interference with her freedom of movement and ability to lie on her stomach and develop that skill (prone activity).

So that's that with Maya. Brianna is doing very well in Kindergarten. We got her first report card and had our first parent/teacher conference and she is performing at or above expectation in all areas. She is noted as being kind to other students. We like.

Victoria is as I type undergoing laparoscopic exploratory surgery to look for endometriosis or other possible explainations for recurrent pains in her abdomen that doctors have not been able to determine the source of by MRI (or CT Scan, I'm not sure which she had), ultrasound or x-ray. It would be nice to find out and if possible resolve her discomfort today. Hopefully we'll have some indication of a cause...

OK, the doctor just called me. They did find some minor areas of endometriosis which he removed. They also discover dialated veins (like vericose) around her uterus, which are apparently somewhat common and can cause generalized abdominal or pelvic pain after prolonged standing or at the end of the day, but are no big deal. Lying down relieves the dialation so in the AM people with that condition usually are discomfort free. He has some ideas for treatment for those which we get to discuss when Victoria wakes up and is able to converse - about an hour or so from now. So all in all perhaps some pain relief will occur for her now - yah!

Happy Thanksgiving everyone!
-Chris

Excellent News Confirmed!

Maya just had a swallow study done and she can swallow just fine! We just need to await clearance from her GI doctor for an all-out drinking fest!

We assume that after that the PEG stomach tube can be removed ASAP.

Maya joined cousin Maverick to see the Wiggles today!

Maya got a special treat today thanks to Aunt Michelle. Michelle (Victoria's cousin) had purchased four tickets for the Sunday, November 2 Wiggles show in Albany at the Times-Union Center. Her older son Phoenix, who is six years old, decided that the Wiggles were too immature for him, so he said he did not want to go, but his younger brother Maverick was still gung-ho. Michelle then invited Victoria and one of our kids to go in Phoenix and Dana's (Michele's husband) place. Victoria was not feeling up to it in the morning, and Brianna was sharing the same sentiment as Phoenix, so I went with Maya. Maya loved the excitement of seeing such big buildings in Albany. For a moment it reminded her of one of the other cities she's been to for medical procedures and she said, "Noooo" when I went to take her out of the van. I reassured her this was no doctor visit, and when she saw that her cousin Maverick was there too, she calmed quite a bit. Maverick is just a bit older than Maya and he is almost fanatical about the Wiggles. Maya had no real experience with the Wiggles, and I doubt she's ever seen them on TV, but she enjoyed her first show experience very much and did a bit of dancing and also moving up to the short wall separating the floor seats from the upper levels and getting into the "mosh pit" of kids dancing up there.



So after meeting Michelle and Maverick at the Colonie Center Mall near Albany and eating at Friendly's there, we piled into Michelle's van and made our way to the Times-Union Center and very cheap ($5) parking only 2 blocks away.

From the parking lot we got a great view of "The Egg," a performance center made famous in They Might Be Giants' "The Egg" from their album "Venue Songs."



We were there a bit before the show and found our seats were only 3 rows up and to the right of the stage. The view was still very good. They put Wiggles stickers on each seat for everyone, nice touch. I went to the concessions and bought 2 fruit cups for the kids. Of course when I presented them I said, "Fruit salad, yummy yummy" (insider Wiggles humor).

The first thing I noticed when the Wiggles entered in their "Big Red Car" was that Greg, the "Yellow Wiggle" had been replaced. Apparently he has quit the Wiggles due to a medical condition.



Another thing I learned is that the Wiggles, for all of their worldliness, seem to have as much geography knowledge about the USA that most USA kids have about Australia. Anthony, the blue Wiggle, as part of his banter with the other Wiggles, said that he just learned that Albany is the capital of New York.

The show was fun for Maya and Maverick. Maya especially loved the song "Twinkle, Twinkle Little Star," as she sings and does the motions for that at preschool. Maya, towards the end, was getting very tired and needed a nap, so was a bit grouchy. Maverick was just plain silly, doing slides on the floor as well as dancing.

It was a good time. Thanks to the Heleba family http://www.helebafamily.blogspot.com/ for the invitation and the tickets!

Halloween 2008

Grandma Dayle, Aunt Nicole, Uncle Eiko, Savannah and Jake came to New Hartford to go trick-or-treating with Brianna and Maya. Brianna was a bride with Maya as her flower girl (very creative Mommy), and Savannah was a fairy and Jake was a monkey. After visiting two of our neighbors we rendezvoused with the rest at the New Hartford Firehouse, where they have open festivities for the kids each year.

After that we trick-or-treated up Cottage Street. The kids were very polite and Maya was even trying to say "thank you" to people.

More pictures and video can be seen at http://flickr.com/photos/gorskipics/sets/72157608627987433/

Some excellent news!

Maya just returned from a visit in which her vocal chords were visually checked by a scope. The specialist at CCMC declared them to be back to normal! Anyone who has heard her screeches, screams or developmental babbling in the past few weeks could have told you that was likely to be the case. As for whether or not she can return to swallowing liquids, that apparently is something that cannot be determined conclusively by a scope. There is an element of "learning" there that she may or may not still know. She will need to go for a swallow study as soon as they can fit her in for one to determine if her body can properly swallow liquids without choking or silently "aspirating" them, i.e. allowing them into the lungs. Once she is given the A-OK for liquids, the PEG tube will be sceduled for removal, another operation in which she is sedated.

Regarding her other parts, her lungs, sinuses and ears have all been free of infection in the past 3 weeks or so as well. Yes, it is true, I have had her on an even stronger version of the garlic/ginger tea during this period due to her having pneumonia in the lead-up time to her PEG tub placement/bronchoscopy at the beginning of the month.

The results of the bronchoscopy/endoscopy (needed to place the stomach port properly) have been generally good, which is a mixed relief for us as we are still exploring why she has too many lung/sinus infections. Bost procedures showed no evidence of GERD (acid reflux), which many doctors have looked to blame and has never been proven. The endoscopy showed nothing out of the norm in the esophagus or stomach, and the bronchoscopy showed slight (minimal to mild) floppiness of some of the larger bronchial passages, but not enoungh to be of any signifigance. Also, Maya is now free of atalectisis which she had present since her PAPVR repair heart surgery in Boston on July 31. Her lungs looked very good and the sputum in there was very thin. Samples of it cultured came back free of bacteria, but with the presence of some 'macroscopic' (visible to the naked eye) particles of unknown signifigance. We are still piecing together some information that may lead to an answer that these particles may be a piece of, stay tuned.

On the negative side, Maya's PEG site has been infected since it was placed, leading to pain for her and worry for us. The wound was just categorized as "granulation tissue" and we are now treating it with steroid cream, as antibiotics do nothing for granulomas, as they are part of your own body overreacting and attacking some of its own cells. Don't look if squimish...

She has also been physically more active. At times she is a riot, at other times a little bully. She pushes Brianna a lot, sometimes just to see her reation, other times because Brianna is intruding on her personal space or competing for attention (like fighting over sitting on my lap). And Brianna then loves to complain about it to us. Sibling rivalry, what a joy. On the good side, there are times when they play excellently togather and are silly and make each other laugh like crazy. That is awesome to hear after all that Maya has been through.

Well our heartfelt thanks and love go out to everyone who has been praying, meditating, directing healing energy, thinking positive thoughts, etc. for Maya. They definately have had strong effects. Don't stop just yet, as there are a few other issues that we have not divulged yet on this blog because we do not have anything other than a pattern of information without a confirmed diagnosis, but she still has some health issue or issue(s) left to resolve.

And to Abby - you are our inspiration. We all love you and pray for the best for you. Your suffering is unfair and your strength and maturity with what is happening in your body is amazing. There are no answers why that can make any sense now, but we pray for your suffering be less and your enjoyment of life to be more. You are so strong and smart and Brianna talks about you all of the time. Our hearts go out to you and your family and just know that there are many of us that would take your place if we could.

More later...
-Chris

A lot has been happening

My apologies for the length of time since the last post. So much has been happening, mostly with Maya medically, that there has not been much time or energy left to blog.

Maya has been having several problems with her NG tube. Her skin is so sensitive to all of the adhesives used, as well as the NG tube material itself, that she gets an oozing red sore under the tape w/in a few days. Changing the tube to the other nostril weekly to alleviate the rash on one side temporarily solved the problem, but has stopped being effective as the interval now required to change the tube is to short to allow enough healing of the other side's rash. Also the procedure to remove/replace the tube is traumatizing to Maya. In addition, she has been developing aspiration (at least in part) based pneumonia due to aspiration of either foods, stomach acid or saliva, despite treatment with acid-blocking medication. The NG tube is a foreign body that does not allow for full closure of the spinchter at the entrance of the esophagus into the stomach, so it allows a path for acid to reflux back up the throat, possibly then over and down into the lungs.

To reduce the NG tube problems, we are having a P.E.G. tube/port placed into her stomach on Friday which will allow food/liquids access as well as samples of stomach acids via a port in her side. She will still be able to eat solids, but liquids will be through this port, just like the NG tube. At the same time she will have a bronchoscopy to examine her lungs and trachea to look for structural abnormalities and to test her mucous to see if they can find a cause for her continued infections.

In addition we have noticed, as well as her pediatricians, that she has significant bowing of the tibia, more pronounced near her ankles and this is happening at the same time her growth has slowed or stopped (since spring). Tests for Vitamin D deficiency have paradoxically led to a finding of an extremely high 1,25 Dihydroxy Vitamin D (Active form of Vitamin D), which can be caused by, in order of liklihood: Sarcoidosis, Tuberculosis, Hyperparatyroidism, hereditary syndromes and various inflammatory diseases. So far we have a negative skin test for TB, but that is common in children - a follow series of three day's of stomach acid samples will be upcoming via her port. She also has negative tests for hyperparathyroidism. Sarcoidosis tests will be performed soon, as well as genetic testing.

I have to run now, time to take Brianna to Taekwondo. Her are a few pictures of Maya's NG tube and rash:

-Chris

Good news and bad news today with Lung/Sinus CT Scans

Today Victoria and I woke up at 3:30 AM and hit the road by 4 AM with a sleepy Maya to have two CT studies performed under general anesthesia. One was an examination of her sinus passageways to look for blockages or other reasons why she is so prone to sinus infections. The other, the chest CT study was to look for something Victoria and I were dreading and haven't talked to many people about...blebs. Our concern was that they would be large, widespread and possibly cancerous requiring major surgery, etc. to try to correct.

We had Maya under sedation right on time at 7:30 AM, and her studies were over in about 45 minutes. We then were able to walk over to the Ear, Nose and Throat clinic and consult with her assigned Dr. on short notice to interpret her sinus study. Maya was found to have normal sinuses with some "mucousal swelling" in her front sinus passageways, which was to be expected with a current sinus infection and/or with an NG tube adding to irritation. So basically it was good news that nothing was awry with her sinuses, but it was disappointing not to have a physiological and simple reason for her recurrent infections.

So then we had a long window of time until our 4:30 PM appointment with our assigned pulmonary doctor, which we filled by taking Maya to a TGI Friday's about 8 blocks away. Maya had not eaten since the night before, and was so hungry she ate a 1/2 rack of ribs and all of her fries. She then played a bit in the public square out front watching the large flock of pigeons and occassionally walking towards one. When a pigeon would take flight it would startle her, the first time she even fell on her butt as a result. It was very funny and I have video I will eventually post of it, but no time now.

When the time came to consult with the pulmonologist, Victoria and I had butterflies in our stomaches expecting ominous news. What actually happened is that the doctor and a visiting pulmonary doctor from Barcelona, Spain took some time to go over Maya's full history. While this was happening we both felt the doctor was unprepared and had not reviewed the CT scan, which was true, but the doctor redeemed himself by getting up to speed while briefing the visiting doctor as he pulled up the CT images. His initial opinion was that no blebs were visible, which the Spanish doctor agreed with, so he refocused the consultation on what he felt was relevant - a current pneumonia-like state in which Maya has areas of mucous caused by aspirated fluids, and a resultant or leftover state of collapsed lung tissue from her surgery. He also noted what was most likely a hiatal hernia that he was surprised was not discovered in earlier swallow studies. In either case, he said she needs chest "percussion" to be delivered by a qualified pulmonary doctor and we agreed to have him contact our pulmonologist in Hartford. Apparently we can be trained to do this ourselves. He also said the cause of her current infection was aspiration of fluids (saliva, water, etc.) and that Gastro-Esophageal Reflux Disease (GERD or acid reflux) was the largest contributor to this issue. He said the NG tube exacerbates the GERD, as does the hitherto undetected hiatal hernia, and he reviewed our doseage of Rantinidine (Zantac) and found it to be appropriate, but increasable if need be. He said the #1 priority needs to be controlling stomach acids and any fluids from entering the lungs, and the NG tube makes that nearly impossible. He introduced the idea of a G-port tube directly into her stomach, which I was not ready to entertain yet as it felt to invasive, but after consulting nurse friends sounds like a better idea to allow her esophagus, vocal chords and lungs to heal. Of couse that may mean no intake of any food or liquid via eating, simply all food pumped into her stomach, which is another major step downhill for us, which we hope will be reversable once her vocal chords heal, but with no guarantee. This also will likely further delay her ability to return to school, which was tentatively set for next week. That suck bad, as she needs social interaction badly.

On the bright side, we have qualified for Birth-3 programs and will be getting speech and possibly occupational/physical therapy to help Maya to get to age appropriate milestones in areas that she is suffering in.

So we have to wait a week before we have Boston Pulmonology's full report and can breathe easy regarding the bleb issue, which we will then have reviewed by CHOP in Philadelphia. In the meantime we get to look forward to consulting with and coordinating between: ENT Doctors in Boston and one in Hartford, Pulmonologists in Boston, Hartford and Philadelphia, Gastroenterologists in Boston and Hartford, Speech and physical therapists at home, a visiting nurse and our primary care physicians, ensuring they all recieve the proper and timely reports from each other while we work to find someone to take the role of an impartial case manager to coordinate and facilitate grop information exchange and consensus.

So that's where we are - tomorrow (or ASAP thereafter) we need to visit CCMC in Hartford to get Maya "modified (seated) percussion treatment" to loosen mucous and to learn to deliver it ourselves, as well as discussing the G-tube option benefits given here comprimised pulmonary state. And of couse figuring out day care for Maya that will accomidate her medical needs.

I am learning to adjust quickly and just be greatful that Maya is happy and affectionate and funny. We love both our kids and they are great gifts here to teach us so much about responsibility, altruism, sacrifice, gratitude and humility. I hope that all of Maya's conditions are temporary and will heal in the next year, but if they don't, we'll research long and hard into the best options to get her the best quaity of life given her medical limitations.

Thanks to all of you that have sent encouraging comments!

-Chris

Brianna's First week in Kindergarten

Brianna started Kindergarten at New Hartford Elementary School on Wednesday. There was a picnic at Giant Steps (her daycare) before the afternoon Kindergarten students left on the bus. Mommy, Daddy, Grandma Mimi and Maya all got to eat lunch with Brianna and her friends before they left. They did a great job getting on the bus, and Mrs. Scully, Brianna's teacher, told me she had a great first day.

Brianna said she loved her first day in Kindergarten. She knows how to bring things home by having a folder with one pocket that says "Home" and one packet that says "School" for things that need to be sent home from school for mommy or daddy to look at.

Brianna said she loved her second day even more than the first. She had music class that day with Mrs. Flower. In music class they did singing. On Friday they has a substitute teacher who was nice, and they saw the classroom caterpillars turn into chrysalises. She said when they left the classroom one was hanging like a "J," and when they came back it was in a chrysalis. They also had art class that day with Mrs. Rood. The project was drawing a picture of herself with crayons.

Brianna also likes the milk she gets every day at snack time. She used to dislike nap time at Giant Steps, but now that she has no nap time, she is very tired after coming home and has gone to bed earlier. Kindergarten is hard work from playing so much!

Brianna wants to say that she has three new friends in Kindergarten - Sadie, Marissa and Jordan.

Brianna's Open House for Kindergarten

Brianna was so excited to meet her teacher and principal on her open house for Kindergarten. She is in Mrs. Scully's class at New Hartford Elementary School. She had no trouble fitting in and even listening through a whole story. There are photos and short videos here: http://flickr.com/photos/gorskipics/sets/72157606972186387/

And here are the longer videos: http://video.google.com/videosearch?q=Kindergarten+%22Chris+Gorski%22&__q=Kindergarten&btnG=Google+Search&lr=lang_en&dur=&emb=on&so=0&num=10#

Sorry for the quick entry, but gotta run to first send-off at pre-school...

-Chris

NG (liquids tube) out, then back in...

On last Wednesday August 20, Maya went with Victoria back up to Boston for a follow-up barium swallowing study as well as an examination from the Ear, Nose and Throat doctor. The hope was to see significant progress with her vocal chord mobility and her ability to swallow without aspirating liquids into her lungs.

At first things seemed hopeful, as the GI team who removed her tape and saw the horrible red inflamed and flaking skin underneath wanted to provide an alternative way to get liquids to Maya. Then did a swallow study and determined that Maya could eat yogurt, applesauce and strawberries without aspiration of liquid into her lungs, but no blueberries, as these were seen to aspirate silently (no choking) into her lungs. How blueberries, which are near impossible to get anything thinner than a goo out of when squished, could possibly be worse than strawberries that squirt lots of juice with every bite, is beyond me. I wonder if the test were performed multiple times, with different foods administered in different orders, in the results would be the same.

Later that day, the ENT doctor scoped her vocal chords and concluded basically no change. Not a very hopeful sign. And also not fitting with the swallow study results which supposedly indicated some improvement. They don't want to see us again for another 4-6 weeks, so no quick improvement is occurring.

In any case, they kept the tube out and told us she should be able to get enough liquids by eating applesauce, yougurt and strawberries. It hasn't been working. She chokes often on the yogurt and applesauce and her previous heavy output of urine had nearly disappeared, and a strong hacking cough has developed, as well as a gain of 2 lbs from the 20th until yesterday. Not good - Victoria and I decided she should be back on the NG tube for proper hydration.

So today I took her to the Pediatrician's office, where the doctor heard her cough but said her chest sounds clear. Nevertheless, they sent me to get a chest x-ray done to rule out a pocket of fluid the stethoscope might not have picked up. At the same time she had no explanation for the deep cough and agreed that she should be back on an NG tube for fluid.

So then I tried to track down an alternate material for an NG tube beside her polyurethane ones that irritate her skin. I was told the only alternative is a difficult to locate "argyle" type, which the Hartford CCMC Gastroenterology office did not have, nor did they recommend due to its stiffness and discomfort factor. So I went to that office and had them reinsert a NG tube, which I did the brave daddy thing for and left the room while the nurses handled my feebly screaming daughter who also threw up during the procedure. Hellish, but the thought of her spending eight to twelve months thirsty and gaining weight like crazy was unacceptable.

Now that the tube is back in and I administered a bolus of liquid she has been happier and eating better. I just the tube is the lesser of two evils and we will experiment with different tapes to minimize her skin irritation.

To all who have been sending kind comments, we appreciate it, and keep them coming. Responses may be slow in coming however, as our household has far more going on than just the Maya medical drama, so free time to email is very low right now.