One Year Anniversary

A year ago today Deb was in Emily's room in the hospital when she got a call at 7 AM that a heart was available. She made a series of calls to alert us of the impending miracle.

I was at the Devon Nicole house with a load of laundry in the washing machine. I ran out to the laundry to take the wet clothes out of the washer and locked myself out of the room wearing shorts and a t-shirt. I woke up the security guard, got my room unlocked, got dressed, packed all our wet and dry clothes, asked the security guard to get a shuttle to pick me up, and got to the hospital.

Deb ran downstairs to get a cup of coffee at Au Bon Pain and melted down when there was a line until strangers saw the wild stressed look in her eyes andlet her in front of them.

Jeff got a friend to drive him to Rochester (7 AM on a Saturday for a college kid is a good friend), where our friends Tom and Jerry picked him up in Tom's plane and flew him to Boston within two hours.

Aunt Susie was on the jetway with her daughter Sammi to visit us when the call came. She abandoned her daughter at O'Hare (she was 15) and husband Jon came to pick up Sam while Susie finished her trip to Boston.

Lisa Salberg from the HCMA got a call from Emily and almost drove off the NJ Turnpike when Emily said she was on her way to surgery.

Each of you who is close to us has a story of that day, and the following days while Emily's life was changed from certain death to a total rebirth.

A year later the memories are vivid, but the feeling of constant anxiety we had are gone. The head of the transplant unit, Dr. Betsy Blume, told us: "we will fix her." She was right.

Emily had three friends over last night for dinner and girl-talk. They looked at prom dresses, had cake, and stayed up much of the night. She got up and coached a swim meet this morning. Her attendance this year has been perfect except for the days she has had biopsies.

Our thoughts keep turning to the donor and the donor's family, who are facing a year since they lost a loved one. I hope knowing that you saved several lives by your generosity at a time of horrible stress provides some solace today, and every day since your tragic loss, and for all the days ahead of us. Life is precious, fragile, and worth cherishing.

It's been an amazing year. Thank you everybody for your help and support. I can't say it was easy, but it was a lot easier than the preceding year. Love from all of us.

Bob, Deb, Jeff, and of course, Emily.

Too much reality

After our upbeat posts on the Make-A-Wish trip, we got hit with too much reality. Emily's biopsy the week after returning was a 2 on the 0-4 scale. It was presumed to be a consequence of lowering dosages of rejection meds over the summer to allow her immunity to come back. The doctors were not concerned and responded to this "mild rejection" by dialing up the meds and scheduling another biopsy. The other problem with the biopsy was that they had a tough time getting the catheter in, and after poking her numerous times they gave up on the jugular vein and entered the heart through the left subclavian vein and a 180 degree turn. It was a long, uncomfortable procedure and she left the hospital looking like she'd been in a cage fight.

Needless to say we were anxious about yesterday's biopsy. Fortunately, it went as well as these things can be expected to go. The catheter went right in and the biopsy proved to be a zero. We are waiting for one more blood test to come back but everything looked normal with the heart and the rejection seems tamed.

Emily is also really working hard to catch up with school, having missed quite a bit between the biopsy days and the Make-A-Wish trip. We're all kind of tired from the stress but greatly relieved to be back on track. The higher med doses have brought back the nausea that she had all last spring, but we are hopeful that she will slowly get used to that.

We realize that this heart transplant is not something you do and get past it, but something that requires steady monitoring and that it will have, as the doctors say, occasional "hiccups." I've never had a hiccup though that can be as scary.

Happy Holiday season to all our friends and family.
Bob

Back to Reality

Back to reality. Our trip to Seattle ended with a day at the Experiential Music Project and Science Fiction Musuem (Jimi Hendrix rocks on!), the Science Museum, the Planetarium, "Where the Wild Things Are" in IMAX, and a fancy dinner atop the Space Needle. I was reminded how much 60's Rock I listened too, how everything was "Groovy," and how much Science Fiction I read. The highlight of the Science Museum was the butterfly exhibit, and having a couple of shows where we could sit felt great after days of relentless walking on tours.

The next day we divided forces. I went to the Boeing Museum of Flight and the non-aviation losers went to the Zoo. For my aviation friends (fellow cult members), this was a really good museum. I walked through a Concorde, through Air Force One, under an SR71, and past perfect examples of warbirds from WWI and WWII. I caught up with my Southwest Airlines pilot friends Ed and Mike at the Museum, and the five of us had dinner at Pike Market. After dinner we participated in the Pike Market Ghost tour, which was mostly cold and lame. We walked around town a little Sunday morning while waiting for our limo to the airport. The trip home was uneventful but late, and our limo dropped us off at our cold and dark home at 12:30 last night. We are getting used to this limo thing--but reality is so uncaring.

We picked a great week to be gone. About 15% of the high school was out with flu last week, and now it's over two weeks since Emily's H1N1 vaccine, so she is probably immune. We had a woman coughing and snorting right behind Emily last night on the plane. Emily put on a mask and breathed shallowly.

When Emily first proposed this trip, and we found out we were going to Seattle at the end of October, I was skeptical. After all, she just caught up with school from last year, during which she missed 88 days of school. It would be unpaid leave for Deb and I, and it was to investigate a computer game that will probably go the way of Myst in a few years?

Once again, Emily's judgment was right on the mark. I really understand now how Guild Wars provided a much-needed escape for her last year, when normal activities kept getting taken away from her. I also saw a creative, fast-thinking, team-playing side of her that I don't get to see from the outside. I am soooo thankful that her mitochondrial disease "only" destroyed her heart and didn't touch her wonderful brain.

The response of the people at ArenaNet was unexpected. They seemed to get a lot out of seeing that their incredibly hard efforts at producing the best possible fantasy role-playing game could so touch an otherwise normal kid. Emily was worried going in that they wouldn't like her because she wasn't the best gamer. In fact her level of play is their perfect audience and she stole their hearts (sorry for the pun). Their smiles were as satisfying to see as Emily's. In addition to enjoying the reactions of Emily and the ArenaNet team, it was way cool to see how all the pieces of a complex project come together. Those people should take over NASA--we'd have somebody on Mars in two years ("Shipping, it's an Important Feature"), it would be on budget, it would work, and it would capture the hearts and minds of the world.

It was a wonderful trip, a great way to celebrate 9 months post-transplant, and to mark the end of heart disease and the beginning of Emily's long future. Many thanks to the wonderful folks at ArenaNet who really extended themselves for Emily and to Make A Wish who truly made a wish come true.

The ArenaNet Wish

THURSDAY! What a day! It was the best day ever. It was absolutely perfect for Emily, and the folks at ArenaNet and Make-A-Wish made it a terrific day. Ok, I'll try to settle down and describe what happened.

The address the limo driver had was a strip mall next to a Quiznos, so we started a little worried. Renee, the Make-A-Wish coordinator, called ArenaNet to get the right address. Their public address is a mailbox in the strip mall, but they directed us to their real office. No signs outside or anything, since they cannot open their doors to the 3 million active players. We were greeted and conducted around the office. Our Non-Disclosure Agreement prevents us from telling details, but we can tell you that we found out how they design a game, and how the specialists all work together.

The tour was very detailed, and they completely opened up the office to us. She sat with artists, animators, programmers, designers, writers, QA team, etc., and they showed her what they do. Then we went to lunch with the section heads in the conference room (they have 200 people working there). She told them the story of how Guild Wars helped her deal with her miserable reality of heart failure and living in the hospital by being able to retreat to a fantasy world. They asked her questions about what she liked and didn't, and she was very frank, and they took her criticisms along with her praise. She had a series of questions for them, which they answered with candor, even when the answer was, "I have no idea why we did that."

After lunch, they let her play Guild Wars 2, with a bunch of people in the office. It was a blast to play with the developers; at one point she said, "I wish there were some sticks on this beach." One of the developers rewrote the game to put sticks there. There were lots of laughs, and the team really enjoyed watching her play and seeing what she focused on.

They pulled us aside and asked if she could stay a couple of extra hours. They convened a group of their eight senior designers and Emily, and they asked Emily to help them design an "Event" for the game. If you want to see it, buy Guild Wars 2 when it comes out, but I can tell you that Emily came up with an idea they really liked. It was fascinating to see her basic premise turn into a detailed scenario, complete with dialogue, scenery, and props. They really treated her as a design professional, and her humor and creativity made the day. It was a truly amazing session. Afterward they moved to a work station, where some of the characters were actually programmed.

The designers all exchanged e-mail addresses, and Kristen said she and her husband play Guild Wars 1 and they'd like to show her some tricks if she joins their group. She and one of the writers, laughed like old friends. Mike, the Founder and President, thanked her for coming, saying how she really brightened up their day. Them thanking us seemed silly, since they gave Emily the best day ever. Thank you ArenaNet! And Make-A-Wish!

Make-A-Wish Seattle

We got up at 3:30 AM this morning to await a big stretch limo that took us to Albany, where we were met by Linda of Make-A-Wish. She saw us off to Southwest Airlines, where we changed planes in Chicago and arrived in Seattle by 11 AM local. Renee, from Make-A-Wish here met us and started us on Emily's dream trip. The highlight will be Thursday, when we will spend the day with the game developers of Guild Wars, the game world that Emily lived in while she was sick last year and recovering this Spring. The rest of the trip will be doing fun tourist stuff.

We had a good lunch at the Baguette Box, walked to the waterfront, and went to the Aquarium. We really liked the harbor seals, fur seals, sea otters, and the highlight was the Giant Pacific Octopus. We got to handle sea urchins, anenomes, and starfish. Afterward Emily and Deb went to a fancy day spa for a manicure. More on this trip later.

Update: Wednesday. We had a big, touristy day. We had a bagel breakfast, then went to the Bodies Exhibit. We got quite an anatomy lesson, and we were relieved that Emily's old heart was not on display. Very interesting, but a little creepy.

From there we went to lunch at a Wrap place on the waterfront, and went on to Argosy Tours for a tour of the harbor. Great views of the skyline, the huge commercial port operation, and Mt. Ranier poked through the distant mist for a while. Digging down into our energy reserves, we went on to the "Underground Tour" of Seattle, where we explored the pipes, rat tracks, and abandoned basements of the old part of the City, which burned in the 1880's and was subsequently rebuilt higher, leaving all the old first floor buildings underground. We got a ribald history lesson, learning that the largest source of tax revenues was a tax on the "seamstresses," who worked nights in the clubs.

We conquered the bus system to get back to the Hyatt, and met our friend Rachel Xu for dinner at a fancy Asian fusion restaurant. A long and enjoyable day, but we are tired from all the walking. Tomorrow is the Big Day at Arena Net. The limo picks us up at 9:45.

Back to School

Emily is back to school full time. We realized that she had not gone to a full week of school since probably last November.

Full days are long, and she still runs out of energy by the end of the day, but she is doing really well, working hard, and keeping up. She is in AP/Honors everything, and has a very challenging schedule.

She has gone back to coaching with the Gators swim team this fall and enjoys working with the kids. She is an editor/contributor to the school paper, and is starting up on the Quiz Bowl team again.

Last week brought a trip back to Boston. The photo is from Children's Hospital Transplant Picnic at Kimball Farms in Westford, MA. Unfortunately I was unable to get any pictures of her soaking the doctors/friends from CHB with her bumper boat, although she was quite good at it. It was a beautiful fall day and lots of fun. I was amazed at the number of energetic young kids running around wearing stickers that said, "Liver Recipient" or "Heart Recipient." These transplant kids are miracles.

We recalled that last year at this time we had gone to CHB and met Dr. Blume for the first time. We expected to be sent away with, "She is not sick enough to transplant." But alas.

Everything checked out well at clinic. It is good to have the medical condition be almost a footnote.

Deb

Labor Day

After sharing Labor Day with the same friends at Center Lake in Beckett, MA for 22 years, we weren't going to break tradition this year. We stayed overnight at Cindy and Fred's lovely camp and enjoyed the best weekend of weather all summer. We went swimming and sat around a lot, making the kids deliver beer via kayak to the adults on the raft. The kids went kayaking and canoeing, and Fred took them all for tube rides. The tubing was a good test of Dr. Emani's surgical skills. She caught some big air and came crashing down. Nothing came loose so he must have done a good job.

This picture shows her new high fashion haircut, but of course she just came out of the water here. We'll have to find one when she's dry and styled.

The adults moved to shore and continued their inane conversations. At one point we insisted that anyone who sit with us could not say anything that sounded smart or provoked any thoughtfulness. A few hours of that can purge the mind and move one a few steps toward true enlightenment--at least nobody was driving.

The kids played board games and after dark sardines--hide and seek where once somebody finds a kid they hide with the original kid who's "it" until they are all hiding together and trying not to giggle. Emily was the oldest in the group, which is pretty interesting because I remember when she was the youngest. All the formerly oldest kids are off to school or adult life.

The next day we did some more swimming and sitting around. Emily was the one who had to show the other kids how to free a bluegill sunfish from a fish hook, as shown below.

She started school yesterday and has now had all her classes. A little stress yesterday with her AP Chemistry teacher (second year of chemistry), who had no large print materials for her and seemed resistant to helping. Her heart transplant did nothing to fix her other medical problems, and the low vision makes it impossible for her to learn from regular books and materials. Although the law requires the teachers to provide her with the tools she needs, we have found a very wide range in enthusiasm for compliance. With the help of her guidance counselor and other support people we elected not to fight an unsympathetic teacher all year and she moved to Physics today, where the teacher is hard but fair. Her other teachers seem great, and we are looking forward to a year that goes a bit smoother than last year.

Looking back from today, 9/11/09, I am first reminded of 9/11/01, when I was in Manhattan and saw the iconic Twin Towers crumble into rubble from a too-close vantage point in Midtown. I feel for the families of those innocent victims.

As I look back on our own last year I remember sending Jeff off to college as a freshman, worrying if he will succeed, and if he will have a good time, like I did in college. We were reeling from the news from Dr. Maron that we should make an appointment to see the team at Children's about a heart transplant, "just in case." Fortunately Jeff did great without parental input, because our focus left him for the year. We were deep in denial. Emily seemed tired a lot but otherwise ok. We made an appointment for October and began reading about transplants on line. Very scary stuff--much of it old, made us view a transplant as a desperate act that might prolong death. However, some of the new stuff made it sound like a transforming procedure that let somebody lead a normal life and could reset their life's clock. We had a lot to learn.

She's a Junior!

After a heart transplant only 7 months ago, and missing 88 days of school, today Emily is finally caught up on all the work for 10th grade. I remember last fall listening to Dr. Blume with some doubt when she said most of their transplant kids manage to stay at grade level and graduate with their class. Well, Emily had some awesome tutors, and a lot of support from her school, but mainly she worked really hard and did all the work to catch up. She has a little pre-reading for AP courses for the fall to finish next week (as do some of her classmates I imagine), but she should be able to start Junior year right on track with everyone else.

Emily had a great time in Chicago. Thanks again to Jamie & Rob, Rick and Bonnie and Susie & Jon for a great trip.

On the medical front, she has had to have blood tests about weekly and several changes in her meds to try to get her white count up a little. Although her immune system needs to be continuously suppressed to avoid rejection, it is a delicate balance with the meds to keep everything at the right level and not leave her too prone to infection (especially as she gets ready to head back to that germ-fest of a school).

We are going to Beckett, MA this weekend to catch up with some Colgate friends. Hopefully Kath will get a good photo of Em's new haircut, and we'll show you later. All of us out east here are supposed to have a beautiful holiday weekend. Enjoy.

Good luck with back to school everybody! Wash Your Hands!

Vacation!

Emily is off to Chicagoland for 6 days. Her cousins Rob & Jamie gave Em a terrific gift of a flight on a charter jet! Emily left from our local airport in her private jet this morning, and flew to DuPage County where she was met by her Jennings cousins. Emily will spend a few days there, and then go up and visit Jamie & Rob and the kids for a few more days and then jet home.

Six Months!

We returned to Children's for Emily's 6 month check up and biopsy this week. Her biopsy was again a 0, meaning no rejection. We were very happy and relieved to have her getting past this milestone with flying colors.

We can't say the biopsy day went smoothly, but they got it done, and the results were good. As we were told, even though you are scheduled to be first case (7 am) and turn your life upside down to get there on time, it does not prevent sick babies being born and emergencies over the weekend. Or to have one of the cath labs break down. Our 7 am test became a 12 pm test, with the fun extra 5 hours of fasting. Ugh. At about 4 hours into waiting, when it seemed calm and they were threatening to get ready to take her in, she smiled at us and said, "If this was an episode of House, I'd have a seizure right now."

But she did well, it was good to see some of our transplant team friends. Better yet, we got out of there and were able to have a fun dinner in Boston with Cindy, Fred & Amelia.

She still struggles with low energy and nausea at times, but overall is doing well.

The summer is speeding by. Emily is working very hard with her friend and tutor, Mr. Hummel, to finish AP World History and 10H English. It will come down to the wire to be a junior when September 10th rolls around.

Emily has a trip to Chicago planned for August 19th to see family right after the AP World test. She feels a little like her summer was stolen away by having to catch up for all the school she missed.

We also had a fun time in Boston doing her first college visit to BU. The conclusion was that BU is a great place, and she definitely loves the idea of college! So we've gone from heart failure through transplant to planning for college in less than a year.

Hope you are all having a great summer.

Enjoy!
Deb

Return to Webster

Emily had a clinic visit on Tuesday. To make it less of a chore, she brought along her cousin Samantha who is visiting for the month with her Mom and brother. We drove out Monday night and stayed with Tom and Nancy Roberts, who moved back to reclaim their house from us in May. They were gracious hosts--they prepared dinner and took us for a tour of the lake on their pontoon boat. It was nice to go back there on a warm evening after all the days of cold wind blowing over the ice-covered lake last winter. It was great to be there with Emily feeling good and the threat of imminent rejection diminishing.


The clinic visit went well; Emily checked out perfectly. She is being weaned off the steriods and her other anti-rejection drugs are stable for now. We go back in a month for a biopsy, and if that goes well it's a biopsy every three months. She has been swimming, hiking, horseback riding and generally back to a full schedule. She still can fatigue rapidly if she overdoes it--her muscle weakness from the underlying mitochondrial disease has not been fixed--but she is having fun again. When Sam leaves next week she will be forlorn, but for now all is good.

HCMA meeting

A few weekends ago we flew to Morristown, NJ to attend the annual meeting of the Hypertrophic Cardiomyopathy Association. This is a patient advocacy group that hosts a meeting each year where patients can mix with the leading experts in the field who study HCM and other health care professionals who attend to get continuing education credits. The academic content is top notch, but the most interesting part is the relationships forged between the HCM patients. Information is exchanged, along with war stories of dealing with hospitals, insurance, employers, classmates, family, and the rest of humanity. The picture shows us with Emily's all-time favorite doctor, Marty Maron and his assistant Murdena Mills. Way back at the beginning of this blog is a picture of Marty and Murdena visiting Emily in the ICU three days post-surgery, when her cheeks were puffy with steroids, her hair was greasy with unmentionables, and her eyes glassy from narcotics.

What a difference a few months makes! Her biggest trauma this time was that the hotel threw her out of the bar for being underage. True to form, the President of the HCMA, Lisa Salberg, intervened on Emily's behalf. Lisa has forcedCongress to pass legislation to make genetic discrimination illegal, schools to install AED's (Automatic External Defibrillators), and insurance companies to support travel to Centers of Excellence that she forced hospitals to set up, so a rule in a hotel was no match for Lisa. Her greatest victory of course was to get Marty Maron to take Emily on as a patient just two years ago and see her on a Friday night after hours so that Emily got the care that saved her life. Consequently Emily got to hang out with the doctors and patients until they threw us all out of the bar because of the arrival of a bellicose wedding party.

Our moment came when Emily participated on a "Patient's Panel" about heart transplants. In concise, clear, and straight-forward language she explained how she felt when she was diagnosed with HCM and had to get an ICD, and then how she felt when she was told she needed a heart transplant and had to deal with the waiting and then the surgery. She held her audience of 250 spellbound, and her parents were mighty proud.

Good heart, bad parking

We took Emily in for another follow-up visit and heart catherization/biopsy. The procedure went very well, and the results were excellent. She had another 0 score for rejection and her blood tests (C4D and PRA) were both negative. The echo looked good, as did the pressures in the heart.

She is very busy finishing up the school year and preparing for final exams. She does not seem to have the stamina to do a full day of normal activities, but she has been working really hard at preparing for her exams in 3 courses. Although it is a relief to be worrying about normal things like exams, it is frustrating for her to be doing it with low energy and working around things like heart biopsies...

Our big problem with the visit to Children's this week was that someone broke into our car overnight in the parking garage. They stole our GPS (bye, Charles) and also Emily's backpack with her notebooks and calculators for school in it. Grrrr. How low is that? Stealing things from people whose kids are sick enough to be overnight at Childrens...

With the help of security and Harvard Univ police, and Boston Police, we recovered her notebooks. We called the glass guys who were able to repair the smashed window the same day, so we were still able to get home that night after the biopsy. Jeff ran out and bought new calculators, so Emily was tired but good to go back to school on Wednesday.

We are now on a monthly clinic schedule for the summer. We are really looking forward to seeing friends and family in the next couple months. Emily still has two courses to finish up over the summer, but it will be nice to have a new schedule and some more down time.

Good Report

Emily and I drove out to Boston to go to clinic yesterday. Bob had a meeting and couldn't come with us. Although this was probably the easiest hospital visit thus far, it is surprising how tiring it was. Emily and I just were wiped out today.

Everything looks great with the heart. I think the transplant team was pleased to see how well she is doing. We related Emily's progress in school and in cardiac rehab, and they were pleased with how far she has come. She can do 30 minutes of exercise, and actually, for the first time in her life, get out of breath. The issue she has been having is that after she works out she hits a wall so hard that she falls asleep in the car on the way home, and can't wake up for school at an hour later. So the transplant team said it was ok to stop going to rehab. That made Emily happy (and me) because now she will be able to go to class more reliably each morning. She needs to stay active and healthy, but using the rehab and pushing her exercise limits is not really necessary for her right now.

That is one of the things that makes working with our transplant team so wonderful. Whenever they can, they really try to consider the whole child in the management and care. This is a simple example, but there was no hesitation that giving up the last sessions of rehab was worth it to allow a little more school. We really feel like they listen to Emily and are very flexible about making her recovery about her whole recovery.

She has come so far in 3 and a half months, it is almost unbelievable. (in contrast we have a friend who is still in the hospital waiting for a heart after 3 months). Going back to the hospital is very challenging, because there is always the lingering fear that they will find something wrong and we will have to stay. I think that is part of the drain of going back that makes us so tired the day after.

We crashed in on Cousins Steve and Lisa & family for dinner, and literally ate and ran. They are among the many households we know that are adjusting to life with college kids returning for the summer. It was fun to hear more amusing stories about summer jobs and crazy schedules, and so many friends around to catch up with, and lots of food being consumed, and issues about all the "stuff" they bring home. Sounds familiar, eh? It was really great to see them all and we loved the dinner.

No News is Good News

We haven't posted because not much has happened, and that's good news. Emily is busy with school part time, tutoring, cardiac rehab three times per week, horseback riding, dance, and coaching the little swimmers. In fact she is at the annual swim team banquet tonight. I got her to stand still for a picture before she left. She looks pretty good for 15 weeks post-transplant, don't you think?

She still gets really tired and needs a nap most days after the exercise, but she's getting a pretty good 30 minute workout. She is occasionally a little nauseated, but most of the time she is eating pretty well. We are able to get her blood draws for her Tacrolimus (Prograf) levels done locally, which means we don't need to be at Children's at 8 AM when we go there, which is down to once every three weeks.