Friday, December 4, 2009

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

Monday, November 2, 2009

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.





Thursday, October 29, 2009

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!

Tuesday, October 27, 2009

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.

Wednesday, September 30, 2009

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

Friday, September 11, 2009

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.

Friday, September 4, 2009

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!

Tuesday, August 18, 2009

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.

Wednesday, August 5, 2009

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

Thursday, July 9, 2009

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.

Monday, June 22, 2009

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.

Thursday, June 11, 2009

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.

Wednesday, May 20, 2009

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.

Sunday, May 17, 2009

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.

Friday, May 1, 2009

More good results

We went to Children's on Tuesday for another biopsy and routine tests. The biopsy, echo, and EKG were all good, with a zero for the rejection score. She had a period of tachycardia in the cath lab that was quickly controlled but sounded scary. They thought it was just the heart reacting to be poked. All of the tests for donor-specific antibodies in her blood came back negative, which is wonderful news. This means no more treatments are indicated right now with immunoglobulin that leave her feeling sick for days.

Some combination of the travel, the fasting for the biopsy, the sedation, and the associated turmoil of a hospital visit made her very fatigued this week.

We moved out of Webster on Tuesday with the help of our friend Kathie, who met us there and helped us clean and make beds. Now there is a good friend for you! We also were able to visit with Cindy & Fred on Monday night - they brought us some good Chinese food.

The next clinic visit is scheduled for three weeks. In the meantime she is very busy, and a little tired.

Sunday, April 26, 2009

The Blog Effect

We have been mostly home now for a month. I keep running into people locally who ask, “How is it going?” And when I start to say, “Well we are doing better now, but we had a difficult winter…...,” inevitably they tell me, well, I know all about it, I read the blog. Either that, or they saw the article in the Queensbury Citizen, and they read the blog.

This blog has been read (diligently) by more people than I could have imagined. The feedback has been wonderful. It helped people who really cared keep connected throughout the whole ordeal. We enjoyed all of the comments, for those of you who posted. And for those of you who didn’t, I want you to know that many people have told me they were unable to comment. I don’t know why that is, but you are not alone.

We knew there were a lot of people out there pulling for Emily, and concerned about her progress. The extent of that is continuing to dawn on me. The outpouring of support really has meant a lot to us.

Twelve Weeks - Busy is good

We've had three weeks without any medical intervention (except for one blood draw at the local hospital). It has allowed Emily to get into a regular schedule. It seems the busier she is the better she feels. She ends each day exhausted, but has been pretty happy. She is just about caught up in Math, and close behind in Spanish and Chemistry. English and History will wait for the summer.

Here's a typical day:

7:30 pre-meds, and some food
8:00 meds
8:15-9:15 Cardiac rehab at the hospital
11:00-2:30 School
3:30 - 5:30 Tutoring (two courses/day)
6:00 - 7:15 Coaching swimming
7:30 - pre med and eating
8:00 meds
8:00 - bed Chill-lax

On other days she is doing horseback riding or dance.

It is great to have our busy girl back.


Saturday, April 11, 2009

Week 10: Back in the saddle again...

A hard week ended well. We got back from Boston late Tuesday after the heart biopsy and IV IG treatment. Emily seemed tired and queasy on Wednesday, but Thursday was full-blown nausea, with cancellation of tutors and not going to school. She began to perk up a little on Friday, and woke up feeling pretty good today (Saturday, 10 weeks post-transplant). She went to the barn to try her hand at sorting the cattle--a game where the kids have to pick out cattle from a herd and move them into a pen one at a time. It was fun, and she did it until she got tired. The rest of the afternoon she's been playing a multi-player computer game with her brother--Jeff's home for Easter.

Wednesday, April 8, 2009

She's a Zero...

...and that's a good thing. Biopsies are ranked for rejection from 0-3, with 3 being acute rejection and zero being none. Emily's biopsies the last two times were 1's. The doctors were not concerned, saying that it is normal to have some white cells clustering around the myocytes "cleaning up" from the trauma of transplanting, but a zero means that there is no sign of rejection. Yesterday's biopsy was zero!

Our concern the past three weeks has been that her blood showed donor specific antibodies, which could be a precursor to rejection. They aggressively treated the donor specific antibodies with IV Immunoglobulin (IG) and oral prednisone. the IV IG made her very sick last time, so were were worried about a repeat. They gave her a different brand of IV IG and we really pumped up the tylenol.

She got through the biopsy and IV IG ok and we made the long drive home last night. She slept through the night, except for when we woke her up to take tylenol. She felt ok this morning and went to cardiac rehab at the hospital this morning. She came back from that tired and then nauseated. It wasn't as bad as last time but she opted for not going to school this afternoon. We hope we don't have to keep doing the IV IG but the doctors really like its effects.

Saturday, April 4, 2009

9 Weeks: "My life is getting a little more life-like"

This is the first week we have not had to go back to Boston and avoiding the medical management is a major step toward normalcy. Emily went to one class a day at school (Advanced Chemistry), and was tutored in the rest of her classes. The work load has been a lot, and although she has felt a little overwhelmed, she has worked hard and knows that at least a couple of her courses will get done in June and the rest will get caught up by September.

She has been going to cardiac rehab at the Glens Falls hospital, where she is the only teenager they've ever seen there. She's quite a hit with the seniors, and they gasp when they see how flexible she is. The exercise does tire her out though, which points out the need for this activity. We don't know how much is due to the de-conditioning over the past year, the surgery, or the underlying mitochondrial condition that still effects all her muscles but her heart.

The nausea that has so tortured her for the past month is partially abated, and she has been eating better. We look with dread to going back to Boston on Tuesday. The biopsy would be bad enough, but after that she is slated to have another IV Immunoglobulin treatment. That is the treatment that made her very sick three weeks ago. They will try a new formulation and hope that it won't be as bad. We also hope that her blood will not show any more antibodies to the donor heart so that she won't have to go through this again.

Deb and I have been trying to get back to work. We think that our stopping work in January may be responsible for the downturn in the global economy, so we clearly need to fix that.

Monday, March 30, 2009

8 weeks

Emily made her first trip back to school to go to lunch and Chemistry on Thursday, just short of 8 weeks post-transplant. She really enjoyed seeing everyone and being a celebrity for a few minutes. She will be going to school most days for one class (80 minutes) for now. We have to work that around her tutoring at home and cardiac rehab at the hospital. The transplant team said she needs to be careful of exposure at school and elsewhere, but she does not need to wear a mask to school. She is trying not to walk through the halls when all the kids are passing classes, and she is very careful about what she touches. From being completely isolated, it is a little unnerving to have all this exposure to people, but we can't keep her in a bubble.

We don't have to go to Boston this week at all. Yipee. But next week the school/tutoring will have to work around a trip back to the hospital on Tuesday.

She is very happy to get some of her normal life back. She has been feeling pretty well most days, with some bouts of nausea.

Wednesday, March 25, 2009

Back to School

After a good check up on Tuesday, Emily was given the green light to start going back to school part time. She will begin next week going to Chemistry class. She has started with her home tutors this week for all her courses. She will continue with them and try to catch up while getting to go back to school a little as well on a limited basis.

Emily also started at Cardiac Rehab today. The program is directed at mainly adults who are recovering from heart attack or coronary artery disease. She is quite an anomaly with her young transplanted heart. She works out on the machines while her heart rate and blood pressure are monitored. The goal is to learn how her new heart and her body responds to exercise, and try to start work on some conditioning and strength.

She does not feel well every day, and has pretty low energy. The doctors are still trying meds to make her feel better, reduce the nausea and start gaining some weight.

It is a little scary to go out into the germy world, but Em is really looking forward to getting back to school and seeing her friends. The transplant team feels her immune system is strong enough to risk some routine exposures.

Saturday, March 21, 2009

The Cranes Fly




Emily (with help from Miranda) strung up all of the paper cranes in her room. They look fantastic.

Thanks again Gators, and good luck at States!

Tuesday, March 17, 2009

Back and forth

We returned to Webster Monday night. Jeff drove out with us and he drove up to pick up Kendra at Holy Cross. She came down and had a very fun dinner with us. The take-out food we ended up with (our first few choices were closed) was pretty horrible, but it was great to see Kendra. Sorry about the food Kendra. We will do better next time.

Clinic was ok today. The transplant team is still working with Emily to try to sort out the meds and get her feeling better more consistently. We are hoping to stay home all week in Queensbury and return to clinic in a week.

Here at home Emily has started to string all the 1,000 paper cranes made by the Gators to hang in her room. We will post a photo when she gets the whole thing done. It is gong to be quite something...

Saturday, March 14, 2009

Still here, not feeling too well

Emily didn't seem to react too well to the IVIG treatment. She was sick during the night. We called the cardiologist on call at 4:30 am. We got some tylenol in her, and later she was able to take her morning meds. She has a terrible headache and feels sick. She has slept most of the day. As long as she doesn't continue to run a fever and can keep fluids and meds down, we probably can stay the course here. It is hard to see her feeling so bad. Since all of the tests this week showed good heart function there is not concern this is due to graft rejection. It is most likely just a reaction to the IVIG, but it is still pretty crummy.

Friday, March 13, 2009

Home again

We went back and stayed in Webster last night and went in this morning to the hospital. The IV Immune Globulin treatment went fine. The worst part was the prophylactic treatment with benedryl which made Emily really sleepy. Well, that and getting stuck for another IV.

We were able to meet with the transplant team and ask some questions. We won't know for a while whether this treatment will need to be repeated or not. The rest of her evaluation including the echocardiogram today still looked good.

Jeff is on spring break and caught up with us in Webster last night, and came in with us for the treatment today. It was nice to have him around. Jeff went to meet up with friends and bring Chris home.

It is great to be back home again, but we are really tired. And, of course, it will be brief, as we need to be back in clinic on Tuesday. I think we should look into a frequent flyer pass for the Mass Pike.

Thursday, March 12, 2009

A Setback

We were way too happy yesterday when we thought we'd be home for four days. Dr. Singh, one of the transplant doctors, called to explain that they got test results back today with some unexpected bad news. Her blood is positive for a donor specific antibody. This was not expected because prior to transplant the screening indicated that she was negative for any Human Lymphocyte Antibody (HLA), which means that she should not have reacted to the donor's tissue. However, this does happen sometime and if untreated it may lead to "humoral rejection," a blood mediated rejection. This is a different rejection mechanism than tissue rejection, but the outcome can be as serious. They have told us to go back to the hospital for an out-patient treatment of IV Immune Globulin (IG). This may be a one-time treatment or it could be a recurring treatment, depending on whether or not the antibody continues to show up in her blood.

We are leaving this afternoon for an overnight in Webster, and then back to the hospital for the day tomorrow. We expect to get back tomorrow night, but obviously we have not control over schedule.

Wednesday, March 11, 2009

HOME!


Fedex came through with a few weeks of CellCept and we jammed the Prius full of stuff and got on the turnpike westbound about 1:30. When we got home she just wandered around and said, "I'm home, in my own house. I know where everything is." She's sitting in her bean bag chair stringing up the cranes. Even though we have to go back Monday a few days here seems luscious! It seems like it has dawned us anew--Emily had a heart transplant a few weeks ago, and we're home from it.

Tuesday, March 10, 2009

Going HOME!!

Well, we are going home to at least visit for a few days and sleep in our own beds!

Emily had a biopsy today. Those results and the rest of her other tests were good. So we were released to go out of the area and go home to Queensbury.

We need to wait for a FedEx delivery of meds, which should be some time tomorrow, and after that we will travel home. We can spend 4 or 5 days there before we have to be back for clinic on Tuesday.

Jeff is on spring break starting Friday, so we will catch up with him, too.

The biopsy went much better today with no versed, and a new cocktail of meds. Emily recovered fairly quickly and was even able to pull it together to go to a tutoring session for two hours this afternoon after a morning biopsy.

We will now transfer the tutoring back to Queensbury next week (Queensbury teachers beware!).

We are looking forward to seeing everyone at home, but Emily is still restricted about exposure to a lot of people. She can not go to any public places yet (school, restaurants, etc). We have to be careful not to expose her to anyone who has a cold or has been exposed to a lot of people. So be patient with us while we are being fussy about who we see, and when we make you wash your hands when you come in, and won't let you touch Emily!

Yipee. We are looking forward to getting home!!

Saturday, March 7, 2009

Five-weeks

We were hoping to go to once a week in the hospital, but we ended up going Tuesday, Wednesday and Thursday. Emily was suffering from nausea all weekend and her heartbeat was elevated to about 120. When we went in on Tuesday her echocardiogram and EKG were both good, so they weren't too worried about the heart, but they gave her a Holter monitor to try to record any episodes of fast heartbeat (tachycardia). The Holter is a beltpack recorder that is hooked up to electrodes that continuously records an EKG. Naturally with the Holter she had no episodes of racing heart when they took it off on Wednesday. On Thursday we had the results. Her sleeping heartbeat went back to 90, wich is her heart's intrinsic beat, so everything seems ok with the heart.

Even with a good heart, feeling sick to your stomach can ruin the day. They gave us Zofran to control the nausea, and it works great, but it is highly restricted, very expensive, and suitable only for short terms. We are hoping the nausea comes from the Bactrim, an antibiotic that may be wiping her gut flora. We switched the Bactrim from daily to every other day in double doses. Hopefully that will let us figure out which drug is making her so uncomfortable. She is eating live culture yogurt with added culture (Culturelle).

We have clearly transitioned from the post-operative phase to the "living with the meds" phase. It's not nearly as exciting, probably not as interesting, but ultimately will determine the quality of life.

I had a big adventure this morning. I flew home to run some errands. It was a nice day and I wanted to fly mostly to charge the battery in the plane and keep my own currency up to date. I spent about half an hour in the house and 45 minutes in the accountant's office (filed a later return form). After next week, if Tuesday's biopsy is ok, we might start to transition home, spending up to five nights a week there and only two here in Webster. Besides, the ice derby fishing contest was today so there may not be as much excitement to watch if the ice fishermen pack it in and go back to drinking in their homes and bars instead of out on the lake.
Bob

Sunday, March 1, 2009

Four-week anniversary

We have made it through four weeks post-surgery. We've been at Webster, MA, entertained by the ice fishermen, movies, books, and the treadmill. We made trips to the hospital on Tuesday and Thursday. Tuesday was a heart biopsy. The procedure went ok but evidently while in the surgical suite and medicated on morphine and versed, Emily got a little weepy. No big deal, versed dis-inhibits emotions, but the anesthesiologist, who prefers unconscious patients, added more versed. When Emily came back to us she was very dopey and said that she remembered the whole procedure and it went well. Fifteen minutes later she couldn't remember the procedure. Dr. Blume came in and talked to her and said that she wouldn't remember any of this and she'd come back later. Emily gave Dr. Blume some presents she'd been saving, but fifteen minutes later she couldn't remember giving her the presents and was very mad that she was so dopey she kept forgetting stuff. That's the way most of the day went. Dr. Blume came back and told us the results of the catherization (normal, but slightly increased pressures) and what to expect over the next few months. Emily conversed with her, but later in the day forgot the conversation. When we got back to Webster Emily was very angry about losing the whole day to the drugs. The doctors and nurses all agreed to back off the versed in the future.

Thursday was a clinic day. We got there after 7 AM, went to Phlebotomy for a blood draw, took the morning meds, got an echocardiogram (good heart function), and met with Dr. Blume. She quickly realized that Emily had no recollection of the discussion on Tuesday so she went over the same information. Dr. Blume would say, "even though you will start to feel good you have to restrict your behavior..." Emily would start to reply and Dr. Blume would say, "Now you are going to say_____ because that's what you said on Tuesday and we've already had this discussion." Emily laughed and said, "you're right!" Emily met with another specialist and Deb and I took a walk, only to be called back because Emily had thrown up. She felt lousy from the meds. Her tutor showed up and somehow Emily had the strength to have almost two hours of school. Then we went back to Webster.

This weekend has been fun. Deb's sister Susan and Emily's cousin Samantha are visiting. Sammy and Emily are very close. The picture shows them enjoying Uncle Tom and Aunt Nancy's "edible arrangements" fruit cluster. The bare sticks all contained chocolate covered strawberries, which were the preferred fruit.

The coming week includes two more trips to the hospital, which also include two more long sessions with the tutor. Thanks again everybody for your support, generosity, and concern.

Sunday, February 22, 2009

Webster Lake

Nothing much to report from here, so that is good news.

We went back to CHB for clinic on Thursday, too. The weather was difficult (heavy rain and moderately heavy traffic), but the visit was uneventful. The echocardiogram still continues to look good and no one seems concerned about Emily's little cough, which has not gotten any worse. Her med levels were perfect so all of the medical aspect appears to be going well. She also gained one pound. We are working on that. Cousin Lisa helped on that front by making a great dinner for us for Weds and Thursday.

The physical therapist put Emily through a pretty hard workout on Thursday. Emily was quite discouraged because she found even the little bit of treadmill quite challenging. She feels like she should be doing what she was able to do with Coach Irion back in the fall. Everyone keeps reminding her she needs to give herself some time since she had a major illness and then major surgery only 3 weeks ago. She has been using Uncle Tom's treadmill to do some walking each day since the weather has either been cold and windy, or rainy (today). We seem to have missed most of the snow that you Queensbury folks have had to deal with. Thanks to Rick who is keeping our driveway plowed.

Friends have brought boxes of movies for us to select from which has been quite fun. Cindy and Fred Johnson-Mettler brought us a box full yesterday and it was great to see them (as well as thier movies).

This still feels like the perfect place to rest and recover. The most exciting thing here is watching the ice fishermen, and with apologies to all you ice fishermen, I can't imagine anything more boring. Tuesday Emily has her second biopsy, and sadly she is looking forward to it as a break from trying to catch up on reading the "Iliad" which she had been doing all week.

Wednesday, February 18, 2009

Emily is Famous

There was an article in the local paper today (the Queensbury Citizen insert to the Post star).

http://www.poststar.com/regions/queensbury/ Click on the link below the picture of the dog with Emily, and also look to the right and the top three article links are about Emily.

We went back to clinic yesterday, and everything appears to be good. The echo looked good and the blood tests were all pretty good. Dr. Singh and Jodi took out some remaining stitches.

They are still adjusting her med doses; they warn us the highest risk of rejection is the first 6 months. But so far, so good. Another visit on Thurs and another biopsy (to look for signs of rejection at the tissue level) in a week. We will continue to go 2x per week for a while, and then go down to once a week. At that point we can decide if we can go all the way home.

Going in and out of Boston yesterday was not too bad. We have to be there early, so we were up at 5, but beat the traffic, and were home for lunch. Other days will not be that fast. Then we all took naps. We have gotten good at napping.

Emily has a little cough, but they said not to worry. They will check on her on Thurs.

Sunday, February 15, 2009

1,000 Paper Cranes


Coach Kristi came to Webster Lake yesterday and brought Emily a gift from the Glens Falls YMCA Gators Swim Team. As you see in the photo, they folded 1,000 paper Cranes. With it they sent a book, signed by all the team. It also explains the significance of the cranes. It tells us that an ancient Japanese legend promises that anyone who folds a thousand origami cranes will be granted a wish by a crane, such as long life or recovery from illness or injury. The Gators folded a 1,000 cranes for Emily, with the wish that she recover quickly and enjoy a long life of prosperity and happiness.

Each one of the cranes is beautiful, altogether they are amazing. Thank you Gators for this wonderful gift and the effort you put in to show Emily your support. She misses everyone there, and hopes she will be back to help Coach Kristi to start the Long Course season in April.

We are doing well in this beautiful quiet house on Webster Lake. We have been mainly sleeping and reading. It is lovely here.

Thanks again everyone. The Gators and the GF YMCA have been an important part of our our lives for a long time, and your thoughts and support means a lot to us.

Deb, Bob & Emily

(PS -- Brother Jeff had an AWESOME meet at SUNYACs this weekend, doing best times in 400 IM, 200 fly and 200 back. He placed 3rd, 4th and 5th. The team had a spectacular meet, dominating for both the men's and women's team standings)

Friday, February 13, 2009

Safe Haven


Cousin Steve Roberts met us at the Mass Pike exit yesterday and escorted us to his parent's house on Webster Lake. We entered and we felt the tension of the hospital just lifting away. We visited for a short time and heated the dinner that Cousin Lynn had left in the refrigerator. We went to bed early, without checking vitals, slept all night without checking vitals, did not hear any beeping alarms, missed the 6 AM weighing, and got up in time for 7:30 meds. A few more nights like that and we may start to feel normal.

Kathy Gow came by with tons of food that she collected from our friends who contributed to our very own food bank. She took the picture of us with Webster Lake, frozen in the background. We intend to hang out, rest, eat, watch movies, and try to decompress from the tension of the past few weeks, months, and years.

Emily stayed up all morning, ate a big lunch of grilled cheese, apples, and ice cream (she has lost 14 pounds so all the dessert rules are suspended). She's napping now in her quiet, private, lakeside room. Thanks Tom and Nancy for the house--it's perfect.

Bob

Thursday, February 12, 2009

Go!

We're outta here!

Wednesday, February 11, 2009

Get Set

The biopsy procedure went fine today. We went from being the first case to the second, which delayed the whole day. The docs who watched said every thing looked fine with the heart and pressures in the heart. We will not have results from the biopsy for a day or two. She also had a chest x-ray and blood tests. The med levels were good this morning, so that means that we seem to have found a good dose of Prograf for now. Her neck is sore tonight from where the put in the catheter.

So they still say we will be discharged tomorrow. We hope it is true! We can't wait to get out of here. I know that this has been an incredibly fast recovery, but 3 weeks in this hospital has still seemed incredibly long.

For comic relief this afternoon, Bob took a singing greeting card (Thank you Wanda) and he and Emily made a door bell for our "ante-chamber" door with string and surgical tape. It worked for a while, much to everyone's amusement. Then the over-zealous PT staff came in and broke the string. Bummer. They better get us out of here soon, or Bob is going to re-wire the whole place.

Tuesday, February 10, 2009

Get Ready

So they are getting us ready to be discharged. We had med training today. We prepared a 3-page spreadsheet of meds and doses. We collected up some of the meds from a special pharmacy, and ordered some from CVS. We met with a metabolic specialist to review the supplements she is on.

Emily had a so-so day. She still has little appetite and her digestive system is still upset. However, it seems that we are balancing out the meds better, and are zeroing in on a dose for the cardiac meds. There were no headaches today, and her blood pressure was better, too.

Tomorrow is a biopsy, and hopefully discharge on Thursday.

I did laundry. I know you are all proud.

That was weird!

Emily is very good at describing things, and she scored some points with the nurses yesterday in describing the removal of her pacer wires. During her surgery, three wires were pressed into the outside of her heart and passed through her chest to the outside. These "pacer wires" were left so that she could be connected to a pacemaker if her heart needed some regulation. Her new heart hasn't missed a beat so it was time to remove the wires. We were a little apprehensive about removing the wires, since removing the chest tubes last week was very painful and gross.

The nurses said, "we ask all the patients what it feels like and all they say is, 'that was weird!'" So one of her nurses grabbed one of the wires, counted down from three and pulled about 8" of wire out of her chest. Emily yelped, "that was weird!" She said it didn't hurt but was just strange. They proceeded to pull the other two wires with a little squirm from Emily each time.

They asked her to describe it so that they could explain it to other kids. She thought for a few minutes and said, "it's like pulling dental floss through your skin instead of through your gums." The nurses liked that explanation and thanked Emily for the good description.

We're glad to see the pacer wires gone. The only tube left in her is one IV in her arm. That's quite a reduction from the maximum after surgery, which was three chest tubes, three pacer lines, a breathing tube, a stomach tube, a line in her neck, one in her groin, a right atrial tube in her heart, and three peripheral IVs (I may have missed a couple). All these holes are healing, and the stitches that close the wounds will be going soon. She is really over the worst of the surgery, but of course faces a long road adjusting to lifelong medications.

Monday, February 9, 2009

Probably Thursday

The team decided it would be best to stay inpatient through the normal course of waiting to have the first biopsy. That is scheduled for Wednesday, and it is to check the heart tissue for signs of rejection. If the biopsy looks ok, and all else seems stable, we may be discharged on Thursday. We will go to my uncle and aunt's house and come back for our clinic visits. We will hopefully be back in Queensbury in 4 -6 weeks.

It was a busy day with lots of doctor consults, EKG, echocardiogram, removal of pacing wires and even a walk/ride outside in the garden. Emily felt much better today, with better med balance, lower blood pressure, and consequently less headache and upset stomach. The goal was to eat and drink more and she did pretty well, even though she does not have much appetite.

We have kept the GI bug at bay, and our hands are raw from washing and washing. We don't let anyone extra in our room, and we try to touch nothing outside of the room. Emily's tutor came back today and she is scheduled to have an hour a day of tutoring starting tomorrow on the days she feels up to it.

Our day still starts with a 6 am vitals and weigh in. Then some food, and a blood draw at 8. So we try to go to bed early, but we have to have a nurse come in with meds and vital signs at 10.

We had fun opening cards and some packages today. Thanks for all the mail! We have more stuff here now than can fit in the Prius. Hopefully tomorrow will be another good day of healing.

Meds

The tricky part of treatment post-transplant is to set the doses of the anti-rejection meds. THis involves trial and error of dosing and measuring serum levels daily. Emily's surgical recovery has been very smooth. Getting the meds straightened out is taking some work. She is on a lot of meds that will be temporary, some she will be on for the rest of her life. It is very important that the level of the primary antirejection drugs stays at a certain level. Unfortunately, Tachro seems to make Emily's blood pressure go up and gives her headaches and nausea. So we are working at trying to keep food in her and keep her stomach settled while she is taking these drugs and yesterday her blood levels were way too high, making her feel sick.

The other challenge is that there is a nasty GI bug going through the hospital. The staff is being very careful, but by my estimate about half of the nurses were out sick last night. We are being very cautious, staying in our isolated room and not touching anything from the outside world.

The transplant team is meeting this morning. They will review her meds and discharge plan. We'll update the blog later. Rounds on Mondays are late so we won't hear until late morning.

Sunday, February 8, 2009

Air Handling

So here's another one. Bob, my beloved geek of a husband, starts roaming around the room with a strip of toilet paper, testing the breeze, shortly after we moved into this special isolation room on Thursday. He determines that the airflow in the room is negative (sucking air in from the hall), when for an immunosuppressed patient, it should be positive, with the air blowing out when you open the door to keep the germs in the hall. OK, so it seems wrong, so he asks about it and is told, "that is the way it always is." Not a good answer to give Bob. So, long story, but this gets elevated to the level of a hospital policy issue. And, the idiots, believe it or not, on Friday, came and reversed the air flow WHILE WE WERE IN THE ROOM. So the dust in the ducts from 3 years of sick, isolated patients was blown in on us. Another scene for SCRUBS. We have not yet come down with any detectable diseases.

Dr. Blume, who now I think of as Wonder Woman, breezes in here yesterday morning with a box of Dunkin Donuts. The woman is a treasure beyond measure. I only wish someday I could get her to sit still long enough to have a cup of coffee an chat. So we asked her if the air handling issue got up to her, and she lauighed and said, "Are you kidding, it was almost the only thing I did all day yesterday." And she said what the take home lesson is, is that when she is pushing us to leave this place we should listen. She is more worried about Emily getting sick here in this loony bin, than sending her out of here with us.

So at first I had a panic attack (mild) when it was suggested we should be discharged soon (Thursday or Friday or Wednesday or Monday, depending on who you talk to) . Now I understand why Dr. Blume wants us out of here. All we are doing now is adjusting meds, and we can do that out patient. There is a GI bug going around the hospital. The staff are back on precautions with Emily, and wear gloves and gowns in the room (like our photos from the ICU). Bob and I are wearing gloves outside the room.

The problem is that Emily is still adjusting to the meds. Her stomach did not take to the meds this morning and her blood pressure is up and a headache is back. These are normal hiccups in trying to adjust meds post-transplant, but very unpleasant none-the-less. At least the air is blowing in the right direction now. It also helps block out the hall noise.

Saturday, February 7, 2009

One Week

One week ago, at 7 am, our nurse came in and told me that our doctor, Dr. Singh, was on the phone for me at the front desk. It never crossed my mind what the call might be, although I should have known. We were so prepared to be waiting for weeks or months, it did not even cross my mind that this was "THE" call. But, in fact, his calm voice said, "We have a heart for Emily."

All the nurses knew and were standing in a large semi-circle behind the desk, watching me. I told him we were ready, and he told us what we needed to do. He said he'd be here in an hour and a half, and that I could call Bob, but wait to wake up Emily. I have to confess that I shook like a leaf, and the nurses asked if I was ok. I told them I needed coffee.

So on our first week anniversary we are grateful for:

1) the donor and the donor's family
2) Lisa Salberg and the HCMA - when we typed HCM into Google the first day of diagnosis we found all the info we needed, and better yet we found Lisa who got us in to Tufts the next week to see Dr. Maron.
3) Dr. Marty Maron - who saw us the first time as an added last case on a Friday night. he correctly diagnosed a diffiucult case, followed Emily closely and correctly determined when she needed to come to a tranplant center.
4) Dr. Blume and the CHB transplant team.
5) Great nurses and care here
6) health insurance
7) Aunt Susie and Jeff being here for those first days
8) Coffee
9) a private room
10) all of you who are sending your love and support

deb

Blog stats

We have been impressed with how effective this blog has been at communication. We are living in a cell-phone free zone. We can walk out to the elevators and stand and talk in a public place, but that is difficult. The blog has really helped us to stay in touch and we enjoy the comments. Jeff compiled the following statistics last Thursday night:

5,315 pageviews (not counting refreshes when nothing changed)
2,700 visits
685 visitors (supposedly unique, but more matched to unique computers than unique visitors)
Visitors from 33 states and 7 foreign countries
75% of visitors came to the site by typing in the address
and apparently the site was found with yahoo search a handful of times also.

Friday, February 6, 2009

Transplant Surgical Team



The transplant surgery was done here by Dr. John Mayer, and Dr. Ram Emani. Here is Em two days post transplant with Dr. Mayer (photo by Aunt Susie) and on the right with Dr. Emani today (Day 6, photo by me). Dr. Emani said today, again, that they are very pleased how well she is doing. She had a kind of sleepy day. She is trying to make herself eat and walk to keep improving, but we think the pain meds are catching up and and making her sleepy. She has an awful lot of meds on board, and is being weaned off some that are given in hight doses early on. All of these have side effects, but sleepy seems better than headaches. She had a visit with friends today and she did manage to smile with Dr. Emani.

Little Stuff

It is amazing that after a major major event like a heart transplant, it is the little stuff that gets you.

Em's new heart has not missed a beat. Her chest incision is not terribly painful and looks great (thanks Dr. Emani).

But what hurts is the IV lines, the headaches, the nausea from all the meds. I guess these are not small things in the sense that pain is pain. The other thing is that most of these side effects are related to the immuno-suppression and anti-rejection drugs. That part of the treatment is at least as difficult as the surgery, and actually lasts a life time.

OTHER THOUGHTS (from a noisy room at 5 am)
I realized yesterday as we settled into yet another new room, this feels a bit like camping in the midst of high technology. Each day we have an adventure, and many nights we have to set up camp in a new spot. We have to find food, worry about where we will sleep, ration clean clothes, and have a tiny little space to live in. We hoard plastic bags and scraps of paper. That all seem silly when you look out the window and we are in downtown Boston.

Sometimes it feels like this is a strange horror movie where there are patients and medical staff running around doing thier jobs. But in the background, visible with only special 3-d glasses, are zombie-like parents. You see them in the elevators carrying food, in AuBonPain in pajamas and slippers, leaning agains walls using cell phones. They are invisible to the visitors and hospital staff....ok, maybe not a good movie plot.....

Thursday, February 5, 2009

Out of the ICU

You know your viewpoint has narrowed when checking into a cardiac care unit feels like the pressure is lifted. They told us this morning they were going to discharge her right away, and it happened at 4 PM, which is record fast hospital time.

Yesterday ended up with everybody exhausted by four failed attempts at a PICC line, an IV that failed overnight, a reaction to one of the IV meds that caused her arm to look burned, and the other arm still sore from the last failed IV that infiltrated fluid and swelled her arm. The intrusive ENT people determined that a vocal cord was damaged (duh, she sounds like she's been smoking for ten years) but that it would probably heal by itself. She sounds better today and we have vetoed their idea to do "swallow studies." Another bit of hospital speak causes alarms to go off for us; if they say, "don't worry, it's not a big deal," don't believe it. They mean it's not a big deal for them!

Emily woke up in some pain. She realized that a component of the pain yesterday came from her reacting to the pain and having her blood pressure spike to 150/90, which exacerbated the headache. Last week's heart never got her blood pressure above 80/50, so the new heart is putting out more blood than she's ever felt. She consciously calmed herself and managed he pain while waiting for the meds to kick in. Then, in another feat of self-control, her stomach started to turn at a liquid medicine she had to take. She said that she was not going to puke, and she ate a few dry crackers and relaxed and the wave of nausea passed. Then she got up for a walking tour of the floor to keep her circulation and lungs working.

We packed up in the afternoon and waited for our new room to be readied. While Deb and I were out, they told her the room was ready. She got out of bed and walked out of the ICU, but the echocardiogram lady just arrived to do an echo. So two nurses, a lady pushing a cart with all our belongings, and the echo lady and her big equipment followed Emily through the twisting hallways of the 8th floor. Imagine how surprised Deb and I were when we got back to the room and it was empty! We've settled into a private room now and Emily is asleep.

I marvel at this wonderful gift of a new heart, especially when we see the echoes. It is working perfectly, but it must feel a bit worse than Emily does. It went through some type of horrible ordeal in the donor, then spent a few hours in a bucket of icewater, and then it was lovingly placed in a safe new home. Even now, it has no nervous connections to Emily, and it beats away at its default pace of about 95 beats per minute, now knowing how much it is appreciated by us all. Every year millions of people die of heart failure, and only a few thousand get another chance with a heart transplant. Among those, the matches are often not as good. We are sooooo lucky.

Wednesday, February 4, 2009

UpS AND dOWNS

We started out with an annoying IV line that made Em's arm swollen and really hurt this morning. There were lots of people coming in to see Em and telling her how well she looks. There is some concern about her throat, since she is still hoarse. They may have an ENT come look later.

She was told she could go for a walk all the way to 8 East to visit her old roommate. So we marched off with the PT people, almost running to catch up. (Miranda, if you are reading, there are some great floors here for sliding, if we didn't have to wear skid-proof socks). Everyone on 8 East was delighted to see her.

That activity made her tired and she went to rest. When she woke up, she had a very bad headache. At this stage, the new heart is beating hard, and there a lot of meds that have complicated effects. We worked through trying to deal with the headache. It had been decided earlier to try to put in a new PICC line. Unfortunately that did not work (with several tries), and she ended up with a new IV instead. Now she fell asleep after so many proceedures.

Our Nurse today's name was Nick and he was a lot of fun. He worked really hard to help Em with the headache. Tonight is the last night with some of th IV meds, so it is possible that she could go back to 8 East tomorrow. We'll see.

Thanks to the Borgos and the Gatorades for sending a great picture for Em's room. She is always telling everyone about her swimmers. She loved the photo.

Scenes for "Scrubs" TV Show

The purpose of this blog is to chronicle Emily's journey through heart transplant. This includes some stories of the activities around her that influence the experience. I'll include just one of the stories today that if it appeared on the TV show "Scrubs" would seem unbelievable. Now I believe that all those silly hospital antics on TV are based on real experiences.

Emily checked into Bed 24 in the ICU on 8 South after her surgery. Children's Hospital built two new ICU units down the hall in the middle of the ICU, just past Bed 12. Rather than number these new units 26 and 27 and have them in the middle of the floor, they renumbered all the beds above 12. Emily's Bed 24 became Bed 26. They came by and changed the number outside her door on Monday, and then the fun began. The nurses quickly adjusted to the re-numbering. The front desk ladies were sending visitors all over the place but that sorted out too. New numbers went up in minutes, and the operation proceeded like clockwork. The IT guy came in to reboot the computer that monitors vital signs so that it would display the correct bed number on the monitoring station.

After re-booting the computer it went into "Demo mode" and did not display Emily's vitals but insteady showed a hypothetical patient. Deb, Emily, Aunt Susie, and the nurse all understood that the monitor was not displaying real data, but the people monitoring out in the hall thought that her pulse, respiration, blood pressure, atrial line pressure, and other telemetry had crashed. About ten people came rushing in with a cart, EKG, and miscellaneous equipment. They looked at her numbers and started pasting wires on all over. They said her pulse had crashed and they didn't know why. Deb was looking at the strong pulse in her neck and said "she's fine, the monitor is on Demo." The emergency team continued to prepare for her demise while Emily said, "what's going on?" Deb assumed they were putting on backup leads but the team thought she was in distress. Finally Deb spoke up firmly over the crowd and said "the monitor is on Demo; it's not displaying a real patient." Finally the crash cart lady said, "oh" as the light dawned and she relaxed. They were gone as quickly as they came.

The nurse chased away somebody from pharmacy who delivered the wrong meds and our vigilence against the gremlins of the bed numbering continues unabated.

Emily got up and walked around the entire floor this morning, all the way to 8 East. She feels better except that her left hand and arm have swollen up. They pulled an IV to give that hand a rest. It is very sore.

Tuesday, February 3, 2009

Good end to the day


The day started with the hard job of getting the chest tubes out (see below). After a nap, Emily got up and went for her first walk. She was assisted by the physical therapy team and they made it down the hall and back. She worked really hard and accomplished much more than what was expected.

A truly watershed moment came for us when the did an echocardiogram this morning. We have seen many of her echos in the past (there's one at the bottom of this blog) but this one looked different--no thickened septum, no big "nose" protruding into the left ventricle, and most amazingly, when they turned on the Doppler to visualize the blood flow it positively whoooshed through without any regurgitation. We are finally convinced she has a new heart! Emily said about fifty times today, "I had a heart transplant three days ago!"

After sending Jeff back home last night, we had to say goodbye to Aunt Susie this afternoon. Their help has been great and we miss them already.

She had some chicken soup and a little pudding but her digestive system is not back in full swing yet. They took her PCA morphine "patient controlled analgesia" away this afternoon, and the pain got a little ahead of her. She got a shot of morphine that brought it under control, but it makes us realize how much she is coping.

After dinner we had a celebrity visit from Murdena Mills and Dr. Marty Maron from Tufts Medical Center. We were referred to Tufts after the original diagnosis of HCM and Dr. Maron directed her care up until the time he determined that a transplant would be sooner rather than later. That proved to be a difficult and timely decision, as her old heart began to give out very quickly after the referral to Children's. He and Murdena braved the snow and rush hour traffic to come across town to visit, and Emily and her parents were thrilled by the visit. They told her how her courage has been an inspiration to them and to many other people. Thanks, Dr. Marty!

The day is ending with more meds being added to suppress her immune system, another walk, and a much deserved rest. Although she is exhausted, you can see by the picture that her cheeks and lips are rosier than they've been for a long time as her new heart pumps more blood around than she has ever been used to before.

Day 3

She had a pretty good night and a very hard early morning. She slept pretty well, and at about 5, they started to get her ready to take out the tubes draining her chest. This was done with versid, so I am hoping she won't remember it all, but it was very painful. She is sleeping again. Having the tube out will ultimately make her much more comfortable, but she was hurting when she went back to sleep at 7 am. She was very brave, but it is hard to see it hurt so much.

They will continue to add and taper off a variety of meds working on anti-rejection and immunosupression. THey are weaning her off oxygen and the morphine. Without the tubes she will be able to get up an walk more.

Interestingly the heart is still settling in. Without nerve connections, apparently the feedback is very differnet and it adopts its own rythm. The doctors say her heart rate of a 100 is normal for a new heart, and it will settle down a little as it gets used to its new home.

Monday, February 2, 2009

Photo Op in ICU


Here is Emily and her support group (Aunt Susie was taking a well-deserved nap).

Sitting Up




















Emily got up out of bed for the first time. It was exhausting, and pretty hard because of all the tubing she was hooked up to.

Planning on Walking

Some doctors just came in to tell Emily that they'll be taking a few tubes out of her chest, and that they will have her try walking later today. She's had a little bit to drink and tried some jello but that seemed like a lot. She is doing deep breathing exercises to clear her lungs. This makes her cough and hurts, but it is good for her.

The new heart is beating very strongly; she has noticeably more color in her lips and her pulses are strong. They are starting to ramp up the anti-rejection medications.

Sunday, February 1, 2009

Address

Several people asked for the address at Children's.

It is:
Emily SInger
8 East, Cardiology
Children's Hospital Boston
300 Longwood Avenue
Boston, MA 02115

Thanks everyone. Notes and cards are great. We can't have flowers.

24 hours

It has been an amazing 24 hours. She hated having the breathing tube in, and struggled with that all morning. However, about 1:30 they took out the tube, which is amazingly fast. She was really relived and much more comfortable. She really really wants something to drink, but her digestive system is still affected by anesthetic, so she needs to go very slowly. She was able to make some small jokes, and after the tube was out we got some smiles. All of her data show she is doing great; the heart is working fine, and her other systems are strong. In fact, she is a bit of a phenomenon on the ICU; no one can believe she is recovering this fast. Right after they took the breathing tube out of her, a nurse stopped in her tracks, did a double-take, noticed Emily was missing her intubation tube, and asked if Emily really already had it out after less than 24 hours, we responded yes, and the nurse said, "She's a rockstar!"

ALthough recovery is going well, she has a long way to go. Moving hurts, and she needs to keep breathing deeply to keep her lungs working well. They are working on pain management, and being unsedated so early means she is thirsty and hungry and unable to start taking anything. We hope she has a quiet night. Aunt Susie is with her right now, letting us sleep.

Thanks for everyone's support. She really is a superstar, she is working really hard and is one tough kid.


Surgery Went Well

Emily is somewhat awake this morning, and every indication is that the surgery went extremely well. The new heart is "banging" according to one of her surgeons. Her ejection fraction, the measure of how well her heart pumps blood is now 4 times as high as it was previously. She has complained about the breathing tube and said she's hungry and thirsty. We may be on the fast track to get out of the ICU. Will post later...

Saturday, January 31, 2009

New Heart is Beating

The surgeon just came in, the surgery has gone well so far, they are closing her up. It seems like the new heart is working fine, and is currently beating well without the help of any drugs. We should be able to see her in about an hour.

Heart Transplant

Emily was offered a heart this morning, so there is about a 99% chance that she will be having a heart transplant sometime later this morning or this afternoon. We'll keep this as updated as possible.

Thursday, January 29, 2009

Here for the duration

The transplant team met yesterday morning and it was decided the best plan would be for us to just stay here in the hospital until transplant. That way, Em can stay on the Milrinone, and keep feeling well and be strong for surgery. The doctors are happy with the balance of meds, and very happy with how well she feels. Since she is high on the list, the doctors are optimistic it could be soon, but there is really no way to know. Maybe only weeks to wait, not months.

She is doing pretty well. Yesterday was supposed to be her first day of school with the tutor, which will be 2 hours a day. But, alas, as it was for many of you, it was a snow day at Queensbury, and consequently a snow day here. So instead, she made a painting of a horse on one of the ceiling tiles for the activity room.

So perhaps today will start the tutoring. We are also high on the waiting list for a private room, which is unbelievably helpful in making this scene bearable. We've had three roommates the last three days, and the disruptions make rest very difficult. As a long-term patient we get higher priority for a single. They had a large number of transplants in January, and post-transplant patients all get private rooms. Some of them are going home and some rooms are opening up, according to our spies. Keep your fingers crossed.

Tuesday, January 27, 2009

Settling into a routine at Children's





We are settling into a routine. The Milrinone is making her feel much better. Her lungs are clearing up and her energy level is back to where she was several months ago. However, we are realizing that she should probabl stay on the Milrinone, and the attached pump and IV line, until the transplant. Hopefully that won't be too long.

We are starting to develop a life in the hospital, as opposed to suspending our lives while here. Emily is starting physical therapy, a tutor is coming every day for two hours, and she has "activities" in the activity room. They are bringing a dog in to play with her "pet therapy" and she has charmed all the staff. They still wake her up a few times a night (and her mother) and the PICC line continues to be a hassle (sometimes clogging).

We had some great visits: Lynn, Steve, and Courtney Roberts, Kathie Gow, and John Ogden. Kathie took some pictures that I will try to attach.

Emily qualifies for a "make a wish" and she is thinking of things to wish for her. If anybody has any novel ideas let us know. She has thought of doing a segment on HCM or heart transplants on the Today Show. John Ogden is trying to get olympic gold medalist Ryan Lochte to call. I have suggested that she ask for a new airplane for her family, but that hasn't really gone over well. So comment on the blog if you know her and think of things that might be impossible for her to do otherwise.

We're hanging in and appreciate everybody's support.

Monday, January 26, 2009

Settling into 8 East, with a few hiccups

After the PICC line adventure, we moved into a room on the regular floor. It was a double but we were assured that nobody else would be moved in. That lasted about 3 hours, when a post-surgical girl and her family moved in during the middle of the night. The room is very small with two families. This seems to be a sleep deprivation experiment.

We spent most of yesterday in the "activity room" to give the other family a little space. Emily is feeling much better and her lungs are clearing. One of the docs spent some time with us and thinks that it might be best if we just stay here for at least two weeks. There's little point in going home, having her feel bad again, not being able to go to school, and then having to come back. They think we live too far to go home with the Milrinone pump, but if we find housing nearby we might be able to leave for a time. While on IV meds the likelihood of getting a transplant is much higher.

I left last night shortly after they found out that one of the ports on the PICC line was clogged, probably with clotted blood from a morning blood draw. They were going to soak it with an enzyme, wait two hours, and then try to flush it. That means another sleepless night for Deb and Emily.

Jeff left yesterday for his 11 hour train ride back to Rochester. It was great having him here but somebody has to maintain normalcy.

I am losing my housing today at the Devon Nicole house today, so I need to figure out housing. This place is just short walk from the hospital; the only alternative this close is the Best Western at $170/night, so the logistics are challenging. Also, I told the airport I would leave on Wednesday, but heavy snow is forecast so that isn't happening. It's amazing how busy we can keep ourselves without doing anything.

Thanks to everybody for your support...Bob