A Day for Special Thanks

Every year when our family gathers for Thanksgiving dinner, we have numerous things to be thankful for – each other, our friends, the wonderful quality of life that we share, and even sometimes, a Yankee or Red Sox World Championship (yes, we are a house divided when it comes to baseball.) But this year will be very, very special, but if you are a regular reader of this blog you already know that – Alyssa has a new kidney, and a much improved quality of life that has come with it.

Thanksgiving dinner at the Deffs will be great example of the “Modern American Family.” In addition to our four kids, joining us will be Deb’s sister Sandy and husband Dan, Dave’s first wife, Bonnie, Sandy’s son Michael & kids Louis & Leana and our very special hero – Michael’s wife and Alyssa’s kidney donor, Jennifer. Many of the TV networks do shows this this time celebrating the heroes among us. Sitting at our table this year will be someone who redefines what a hero is and what it means to give of oneself to help another. It’s called “giving the gift of life,” and to look at Alyssa now and her renewed zest for life, it’s easy to understand how those words are used to describe organ donation. Upon hearing of Alyssa’s approval for a transplant, Jennifer was the first person to step forward and volunteer one of hers, or as we call it LBJ (Little Bit of Jen.) As with most heroes, Jen does not want to talk about her “gift” saying that it was God’s way for her to help someone in need. We know how very lucky we are to have her as part of our family and look forward to sharing a day of special thanks with her.

I would be remiss if I didn’t also offer a word of thanks to the incredible medical teams that have tirelessly worked with Alyssa since January 2009. I know that I’ll get myself in trouble by listing names, so please forgive me if I leave you out. Dr. Kouides has been our team leader, not only with day-to-day care, but also with doing the research and legwork to find & connect us with the A-HUS team at the University of Iowa and our miracle drug Soliris. Dr. Kouides & his PA, Michelle Faulkner are the foundation upon which team Alyssa is built! Alyssa’s GP, Dr. Jane Salamone and her nurse, Barb Tartaglia, have been the glue that holds Team Alyssa together; and our newest members from the transplant team at Strong, Dr. Carlos Maroquinn, Dr. Mabel Bodell, Jennifer Timm and Judy Gierlach. Thank you to all of you and those that have worked with you to help Alyssa.

We are also thankful for the expertise and support of all the many medical professionals who helped us get to this point in time. In the past year and a half, Alyssa has been a patient at Unity, Rochester General and Strong Memorial and there were countless people in all of those hospitals who deserve thanks, many of whose names we don’t even know. We are also very thankful to the Peritoneal Dialysis team at Rochester General and the Red Cross nurses who handled Alyssa’s apheresis and the neurology, nephrology, respiratory and hematology depts. of all the hospitals, and the transplant floor of Strong……thanks a million to all!!!! And of course, thanks to the University of Iowa for their expertise in the genetic research and use of Soliris (and thank you Alexion). We are thankful for our A-HUS family (namely—the Foundation for Children with A-HUS) and all of the research and helpful support they have provided. Alyssa is especially thankful for her new friend Jill from Arkansas and the special bond that they share.

And, a special thanks to all of you that have been with us throughout this tough period. We so appreciate all you cards, gifts, letters of support and the many dinners sent our way (if you are missing a bowl or casserole dish, please check with us = ) .) We have tried to keep up with thank you notes, but please forgive us if you haven’t gotten yours yet. Your support has played a very key role in keeping Alyssa and us on the right track and we are so thankful to have you in our lives!

May you and yours have a very Happy Thanksgiving!

dd

This Could Become Habit Forming

Another day at the clinic and another day full of good news. This is the type of habit that we would love to get into! Lyss is doing so well that her clinic visits will now be once a week rather than twice. She also received another treatment of Soliris.

Lab results:

White Blood Cell.....................10.0
Red Blood Cell.........................3.8
HCT..........................................30
Platelets................................446
Creatinine.............................. .71
GFR.........................................59
Tacro.................................... 16.1
Phosphorous........................ 1.5

Tacro level ( immunosuppressant) is running high which resulted in her daily dose of this med. being cut in half. Phosphorous is still low, but steadily improving through diet changes. The irony in all this is that Alyssa has had to change her diet 180 degrees from what she ate before the transplant. The foods that she avoided are now high on the priority list. A plus for her is that food tastes good again and she actually enjoys eating most of the recommended foods.

Last night, the Kidney Foundation held its annual Kidney Walk Picnic, an event to honor and thank the year's leading fundraisers. Although Lyss was not number one again, she was among the leaders and was the highest in Internet solicitations. In recognition, she received suite tickets to the Buffalo Bills/Steelers game. Kyle and I are big Steeler fans, so we're both proud and thankful.
dd

Monday 11/15

After a less than stellar weekend, today's scheduled trip to URMC brought plenty of good news and a plan to work on a couple areas of concern. We love our medical team, but it never ceases to amaze us how far off schedule they always are. Visits are regularly between seven and eight hours...but I digress, back to Alyssa.

The weekend was a little rocky. She had to deal with a rather nasty headache and a case of the "shakes." Her hands were shaking so much that she was unable to even hold a pen, resulting in Mom taking over the writing of her thank you notes - well at that's Alyssa's story and she's sticking to it. Blood pressure and pulse were also high (160/115 & 120.) Much of this may be due to a high level of Tacrolimus, which is a drug that she takes twice a day to help prevent rejection of the new kidney. All of these are common side effects Tacro leading her Dr. to half the daily prescribed amount for the next couple of days. It's all part of the delicate balancing game that is part of dealing with a transplant recipient's life. Alyssa has really been very fortunate to-date, with a minimum of drug changes needed.

The rest of her labs were some of her best so far. Phosphorous was up to 1.4, White Blood Cell count back to normal and negative results on all of the virus panels that were run. And how about that Creatinine - .67, which is just outstanding! Staples came out today and Dad learned how to do the daily flushing of her catheter. She even got the go ahead to drive very short distances.

It's the kind of day that we hope for every time that we go to URMC. How can you not be happy when you see the Doctors continually smiling and shaking their heads in amazement. Alyssa is beating the odds, but that shouldn't surprise anyone.
dd

Lab Results

For the medically inclined, here are the latest lab results:

White Blood Cell.....................10.9
Red Blood Cell.........................3.4
HCT..........................................30
Platelets................................409
Creatinine.............................. .72
GFR.........................................59

I'm leaving for NYC this morning for a "Boy's Weekend" with my 3 sons and the Dave Matthews Band. I'll update only if there are any developments with Lyss.

Have a great weekend!
dd

A Little Clarity

Seven hours today versus 14 Monday wasn't the only improvement - Lab results were also better, including a drop in the White Blood Cell (WBC)count to 10.9. Most everything else fell in the acceptable range with the exception of Phosphorous which results we are still waiting for. They drew a couple of virus panels due to the high WBC count on Monday, but those take a few days to get back. Blood pressure and pulse are still trending high so adjustments will be made to BP meds.

So where are we - good results, bad results. Good days, bad days...what does it all mean? Are we where we should be? Is this what the Dr's expected to see? Well, today brought us a little clarity. We had one of those rare, special, and often terrifying moments, when the doctor looks up from the eight inch thick file, puts down the stethoscope and just talks to you. Sensing anxiety in Lyssa's voice and demeanor, Dr Bodell said "Alyssa, you are doing incredibly well, better than we could have expected." Shortly after, her surgeon, Dr Maroquinn came in and just couldn't stop smiling while telling us that "he is thrilled with her progress." As both said, when you do something with no precedent to compare it to, you just don't know how it will all turn out. Both feel that she is doing better than expected.

We're only two+ weeks into this, with a great deal still ahead for Lyss, but for one day let's celebrate how far she has come!
dd

The Ups & Downs

Unless you have lived it through it, it's very hard to understand the emotional highs and lows that accompany almost every day for someone living through aHUS; and in Alyssa's case, the additional issues that come with a transplant associated with aHUS.

Take today for example...it started normally, Lyss felt good when she got up and had a great time hosting Jennifer, Michael & Leana this morning. Then just like that, things changed this afternoon. First her labs results came back with some issues regarding White Blood Cell count (14.0,) Red Blood Cell count (3.6,) and a variety of other hematology variances. The imunosuppressants may be raising havoc here, but to be safe, they will do a "virus panel"
tomorrow to see if there are any underlying issues at play. When Lyss asked her coordinator if she should be worried, she got a "not yet" for a response. Gulp!

Then, while watching TV, Lyss felt a little out of sorts, which always sends us running for the blood pressure cuff - 158/110 with a pulse of 120. Not good numbers, so back on the phone to the coordinator who increased her blood pressure meds immediately and for the near future. It's been an hour now, and her pulse has started to come down.

These may seem like a couple of minor issues, the problem for Lyss is that almost every day is filled with bumps in the road like these. She has a significantly compromised immune system now due to the imunosuppressants and the Soliris, so each irregularity has to be watched carefully to ensure that it isn't a precursor to some thing major. Our hope is that the further we get from the transplant date, the fewer the issues. She also will learn to address some of the issues on her own as she becomes more familiar with them.

To say that we are proud of how she handles these situations would be a gross understatement! She calmly makes the calls to the appropriate medical source and takes whatever action is needed. She has very successfully worked to bring an order and calmness to each day. Not dwelling on the negative and trying to look for the positives in her situation. Sounds simple/easy right? Try living every day walking on the edge of cliff, never quite sure when something will send you over the edge; or, working with doctors that are the some of the best at what they do, but can't really tell us what our expectations should be due to the uniqueness of this disease. And do Mom & Dad handle it? Probably not as well. Speaking for myself, every time that I'm not with them in the hospital or clinic, and a get a call from Deb, my heart sinks into my stomach and stays there until I'm reassured that all is well. Again, a feeling that you would have to live through to understand. It sucks - for everyone involved. But we will fight through every little blip, praying and hoping that better days are on the horizon for Lyss.
dd

Nice Day at Home

No procedures on Tuesday, but that didn't keep Alyssa at home. She went over to Rochester General Hospital for a social visit with Dr Kouides and his hematological team that were instrumental in getting her to where she is now. While falling just short of a ticker-tape parade, hugs and smiles were everywhere! We are so thankful for all that this special group of people have done for Lyss. A bond has been formed that will last a lifetime.

Today will hopefully bring another day of rest before returning to the hospital tomorrow for a Soliris treatment and clinic visit. Let's hope that this one is under Monday's 14 hour day!
dd

Monday Night - 7:00pm

Changing of the Guard

Deb and I have switched places and now I'm with Alyssa at URMC as she continues the infusion process. It typically takes six hours, but we're not sure what kind of rate they have her on, so it could be as late as 10:00pm till she is finished. Unfortunately, she can't trade places with anyone, for a much needed break. This will be at minimum a 12 hour day and could go as high as 14. But we're looking at these as "steps forward" toward her goal of regaining her life.

As most of you know, I started writing these posts when Alyssa fell ill in 2009. It was a way for us to keep everyone informed as well as provide me with an outlet for emotions, frustrations and quite honestly, fears. With a blog, you never quite know who your audience is, but hope that you are providing information that's interesting as well as informative. Our source site, Blogspot has now added a feature that allows us to track geographically where our readers are located. Over 1,000 are scattered throughout the US, but a large number of followers are from Japan, Canada, Germany, Russia, Ecuador (cousin Lisa,) Singapore, Spain UK, etc. Our assumption is that they are searching Soliris and hitting on our site. Alyssa's and two other similar a-HUS/Soliris transplants (Brandi in Iowa & Jose in Spain) are being watched very closely by both the medical community and families dealing with this awful disease from around the world.

A toast - May the information that we bring you continue to trend positive and the list of successful transplant names grow longer!
dd

Monday at 2:30pm

Good and Bad News

It's going to be a long day at the hospital for Alyssa. She had labs drawn at 8:30 am, followed by her twice weekly meeting with the transplant team. With the exception of her Phosphorus (down to .7 from .9) her results were very good. However, the Phosphorous is so that low that it will need to be corrected through a four hour transfusion. This is probably what caused Lyss to feel so bad on Sunday. Right now she is Radiology getting a catheter placed in her chest after which she will need a couple hours to recover and eat (fasting since midnight,) then right back to the Cancer Center for the transfusion. Not the ideal way to spend the day after feeling so lousy yesterday.

Due to all of the blood draws and transfusions she has received over the past 21 months, her poor veins are almost impossible to locate, hence the need for a catheter. We won't know what type they have chosen until she is in recovery. It will need to provide access for both blood draws and transfusions and most importantly, be able to stay in place for several months. More on this later.

Lyssa Facts
87.7 million to 1 - the odds of getting a-HUS in the United States - but she still hasn't won the lottery

dd

Wrong on Both Counts

Today wasn't better than yesterday and I'm not done with the dreaded toolbox...so much for yesterday's predictions. It's hoped that both are just temporary setbacks.

Lyss has had a blue, funky day, just feeling blah! It may be the residual effects of doing too much over the past couple of days. She's had plenty of visitors and probably too little rest, so today we quarantined her to the couch with total control of the remote. Apprehension over tomorrow's catheter placement is also weighing heavy on her mind. Even though she has been through more procedures than she can remember, the thought of being cut open again so soon is not high on her to do list. Hopefully this will be the last one for awhile! She also has labs drawn and meets with her doctors, so it will be another long day in the hospital. But at days end, she'll be back home with us, which is always a good day.

Questions? Use our comment area to ask any questions you may have on the transplant or a-HUS in general.

dd

Saturday Night

One of my favorite baseball writers, Buster Olney, always ends his articles with the line - And today will be better than yesterday. I can't think of better way to describe Alyssa's progress...everyday she feel better, stronger and more optimistic about what lies ahead. Her labs continue to amaze her doctors, but its her family and friends that notice the biggest difference. The smile, spunk and zest for life that disappeared in January 09, are on the way back. Our old Lyss is slowly, but steadily reappearing.

Yesterday she ventured out of the house, albeit in a wheel chair, to find some clothes to fit her new trim figure (-20 lbs) and to pick up a few items at Bed, Bath & Beyond. Her donor, Jennifer and her family joined us for dinner last night, which was the first time that they have reunited since Jen left the hospital. It was fun to listen to the two of them share their "war stories" relative to their surgeries and follow-up care. Jen reports that it really feels like there is an empty spot where her kidney used to be, but that she is recovering nicely. It was a very special night!

Tonight a bunch of Alyssa's friends came over for pizza, wings and game playing.

I apologize for not posting since Wednesday. To be honest, I needed a break, but that wasn't the only reason - In typical Deffenbaugh style, we appear to have bit off more than we can chew. We have been trying to get our house ready to list and sell (a process that we started before we knew the transplant date) and have unearthed numerous projects that we are certain will make our house the one that everyone wants to buy. However, I'm done watching the HGTV channel, making non-stop trips to Lowes and pretending that I know which tool to use...well at least until tomorrow when the Realtor comes by for a tour and evaluation. By the way...do you ever wonder why we fix up our homes so that someone else can move in and enjoy them?

Back tomorrow with more on the medical side including a new Question & Answer section.

Set those clocks back!
dd

Best Day Yet!

It was a very good day for Alyssa, in fact, the best in a very long time. It started with a typical "Alyssa move." She called Strong and told them that she'd enough procedures in the last week and didn't want to go through with the scheduled surgery to place her home catheter in her neck. Luckily, they agreed that she could wait until next week--she did have some persuasive arguments that her body needed to heal and she had not really had any good days since the surgery, with blood transfusions and stomach issues, etc., in addition to recovering from the surgery itself. So, she had a break and was able to just go to the hospital and get her blood checked. She will still go tomorrow for the Soliris treatment, but they will do it through an i.v. instead of a catheter. That is how the first infusion was done prior to surgery. Even though she is a difficult "stick," the phlebotomist managed to get her blood without her even feeling it. She wants her to transfer to the infusion area for tomorrow!

Alyssa managed to go for a walk outside today and walked back up to 6-1600 to say hello to her favorite nurses, especially Mrs. Olsen who so patiently answered a million questions for Alyssa both before and after surgery! She also ran into Staples for a new folder for all her new medications (dad's wasn't pretty enough), stopped at mom's office and now has three friends over helping her wash and blow dry her hair. A very full day on day 8 post surgery! Walking through the hospital today, with her not knowing the route, seemed really weird, but very thrilling. This has hung over her head for so long and everyone has worked so hard to get her to this point, it just doesn't seem real yet. We dismantled her PD machine and all of the paraphernalia that goes with it. We still have about 40 boxes of fluid in our basement that we will donate to free up lots of space!! Even with all of these physical changes, and Alyssa's 12 visits to relieve her bladder a day, it is hard to believe we have made it to this point. It is really an awesome display of humanity (in the form of Jen's complete selflessness and unbelievable generosity), medical advances (transplant and Soliris) and perseverance (Alyssa).

It is always a little awkward to brag about our children publicly, but we wouldn't be telling the whole story if we didn't acknowledge our daughter's bravery in going through all she has faced since January 2009 to date. She rarely complains and has very seldom shed any tears. I'm embarrassed to say that she sailed through this with much more grace and fortitude than I could ever have mustered. She is truly amazing and has learned so much about what is important in life, as well as the practical lessons this nightmare has taught her. She has now become her own advocate, learned the PD process, (which she took to like a fish to water even though it is too complex for the majority of dialysis patients to use), learned to give herself shots every 2 weeks and to keep track of a multitude of medications for high blood pressure and kidney disease, as well as doctor appointments, and even managed to be the number one fundraiser for the Kidney Foundation in 2009, and one of the top fundraisers this year. She even spoke in front of our church earlier this year and explained about a-HUS and her kidney disease. She hopes to become an advocate for peritoneal dialysis and help others get through difficult times and believes that she can use this experience to benefit others, and turn a negative to a positive.

Watching her interact with her friends and be her chatty and energetic self is such an indescribable high ----it gives us hope that our Alyssa is back to herself again. She spent so many months having no energy and being nauseated and unable to go out and enjoy life at what should have been a very precious and exciting time in life. Thankfully she has gained from the experience. As someone said to me, this is the stuff of what life has to offer at its fullest--the best and the worst. I am optimistic that the worst is behind us and thankful that the best has brought us to this point!! Thanks for sharing these highs and lows with us and supporting us through it all.
Deb

Rough Ride Today

Alyssa has had a rough time today, spending most of her time riding the "Porcelain Bus." Unfortunately, this seems to be a common side-effect for almost all of her meds. As a result, the doctors want her to back off on a couple of them; and, to avoid dehydration, she will have to increase her intake of fluids. We all know what that does to her, so it doesn't look like she will be getting of the bus any time soon.

Jan and Michelle, from the Kidney Foundation, stopped by this afternoon for a visit and presented Lyss with a special "transplant necklace." She was very happy to see both of them.

Sadly, due to today's issues, tonight's visiting hours have been cancelled. We hope to reopen for business tomorrow.

The Deff boys are off to Buffalo tonight for Dave Matthews. Alyssa will be there in spirit. We're hoping that she can make the Madison Square Garden concert later this month.
dd

A Day of Rest

Nothing like a day at home! No tests or procedures today, so Alyssa gets to spend a day resting and catching up on missed TV shows recorded on her DVR. Yesterday's labs were very good - with Crit leading the way, staying strong at 30. She does have some phosphorous issues, but they are regulating that through pills. There was some concern that the surgeon took out the pic line. This was put in to provide easy & quick access for Alyssa's Soliris infusions. Due to all she has been through, she has incredibly small veins, which requires the IV Team be called in to place the line each time. So to ease the process, she will have a tunnel catheter placed in her chest tomorrow afternoon. This will be the third catheter she has received in a little over a week. This one should be good for several months.

Blood pressure today has been running high again - 156/114. It may take a little while to get the proper meds and doses to bring this under control.

Lyss had several visitors last night and mom & dad joined in by cracking open a new bottle of wine to celebrate - I can't remember a drink ever tasting better!

Baseball fans - 109 days to Pitchers & Catchers report to Spring Training!
dd

A Message from Alyssa

Hello everyone; I figured it was time for me to write something on the blog so here it goes. It's Alyssa by the way! :) This morning was a rough morning to say the least, had to wake up at 7am to go to the clinic for a blood draw and to meet with doctors, which was hard because I was so tired from being up all night, PEEING! :) No complaints here. It may sound weird to say, but I LOVE TO PEE! haha. Anyway the doctors said everything looks good as of now, which was wonderful news, even though all I wanted to do was go home and sleep! So I finally got home around 12:30 and spent almost an hour trying to figure out these meds. It's unreal. There are at least 30 individual pills that I take at 9:00am and again at 9:00pm and a whole bunch more in between. I don't know how people do this without help considering it took me, my mom and my dad to figure it all out! But I got them down, without pleasure. They are not pleasant pills. Anyway I'm getting tired so I just wanted to come on here and say Hi and thank you to everyone for all the prayers and thoughts sent my way. I really do believe there is a higher power and he helped me through this because everyone was asking him to. I am so grateful to have had this opportunity and I have so much trouble putting it into words how to thank Jen but she is an AMAZING person, who was willing to go through hell just to make me feel better. She is so wonderful and I wish I could show her my thanks, but there just aren't words. SO Jen (& Mike, Luis & little Leana, I know this has been hard on you too) I LOVE YOU, and you truly are my hero and a gift from god. I hope you feel better soon, and we will forever have a bond and tie that most people will never know. We actually call the kidney LBJ (Little Bit of Jen). And let me tell you all, this is one good working kidney, it pee's all day and night and my levels are almost TOO good. Love you Jen, you're the best!! Anyway, it's time for me to lay down, this is exhausting. Friends on the TV, and sleep sleep sleep!! Hope to see you all soon! :)
-Alyssa