Friday, December 3, 2010

Odds & Ends

One of the challenges of writing a blog dealing with health issues is providing the readers with pertinent, interesting and timely information. That's easy to accomplish during crises and before and just after surgeries/procedures when news & changes come fast and furious. Fortunately for Lyssa, the news has been mostly positive over the past month. We could never have dreamed that she would come this far this fast. However, each day brings a set of ups and downs that are inherent with a transplant patient; especially one dealing with a-HUS. As we have shared many times, Alyssa is only the second a-HUS patient in the USA and fourth in the world to undergo a kidney transplant with the drug Soliris. A procedure that had only a 10% chance of success prior to the development of Soliris. So the bottom line is that no one really knows what to expect, which means that every little change in her readings or even how she feels, bring heart stopping moments to us all. I wrote to a friend the other day and described our daily routine as that of walking along the edge of a cliff - we love the view, but always worry that we could stumble at any time. Telling you about each of these crises would drive both you and us nuts, so we try to keep these updates at the 50,000 foot level, but will always provide you with any significant changes.

Lab updates - Results from 12/2 were good, with only a few tweaks needed to her pill regimen.

Prednisone has really been taking a toll on her. She has swollen/puffy ankles, legs and especially in her face. The plan is to gradually lower her doses over the next few months.

Beginning next week, Lyss's Soliris treatments will be moved back to Rochester General Hospital under Dr. Kouides.

Lyss and family had a great time at the Steelers/Bills football game last weekend as guests of Greg Connors. Not only was he a great host, but the game turned out to be one of the best played in Buffalo in a long time! Thanks to Greg and The National Kidney Foundation of Upstate NY.


Thursday, November 25, 2010

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!


Thursday, November 18, 2010

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
Creatinine.............................. .71
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.

Monday, November 15, 2010

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.

Friday, November 12, 2010

Lab Results

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

White Blood Cell.....................10.9
Red Blood Cell.........................3.4
Creatinine.............................. .72

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!

Thursday, November 11, 2010

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!

Wednesday, November 10, 2010

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 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.

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!

Monday, November 8, 2010

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!

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


Sunday, November 7, 2010

Wrong on Both Counts

Today wasn't better than yesterday and I'm not done with the dreaded 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.


Saturday, November 6, 2010

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 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!

Wednesday, November 3, 2010

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.

Tuesday, November 2, 2010

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.

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!

Monday, November 1, 2010

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! :)

Sunday, October 31, 2010

Day 6, Sunday at 1:30pm

She's Home

It's great what a couple plasma transfusions and 24 more hours of recovery have done for Alyssa. Even last night, we would have bet against her leaving the hospital today, but here she sits, playing with her two dogs, Quincy & Lili. The crit came back up to 30 and the swelling in her leg has dramatically decreased. The doctors felt that the emotional uplift that she'll get from being at home will help speed along the healing process. She needs to return tomorrow to the clinic as an out-patient for tests and monitoring, but that should only be for about four hours, then back home.

It's amazing to see how far she has come in just five days. She is feeling so much better and the kidney is going strong. Much to her relief, peeing is like riding a bike, even though she hadn't done it for year, she just slid onto the seat and away she went!

Jennifer is still pretty sore, but doing well at home. Last night they held a birthday party for her daughter Leana, who's best gift was having mom home again.

Again, thanks for all your support. We can't tell you how much it's meant to all of us. We are going to try and get back to a "normal" life over the next few days. I'm taking Ryan back to Oswego this afternoon and Deb & Mark will be back to work and Kyle back to Athena tomorrow and Lyss...she'll be enjoying everyday, so grateful for the incredible gift that Jennifer has given her and bound and determined to return to as a normal of life as she can.

Labs: (for you Heidi)
Red Blood Cell...........3.2
Creat....................... .90
Potassium.............. 3.6
Phos........................... 1.4


Saturday, October 30, 2010

Day 5 - Saturday at 12:30pm

A Few Setbacks

As is to be expected, Alyssa has had a few new challenges to face over the last 15 hours, which will require that she continue to stay in the hospital for at least one more day. Last night, they noticed swelling in her right thigh area, which was accompanied by pain when Lyss walked. With blood clots at the top of the "Watch List," she was taken down to ultrasound to determine if there was indeed an issue here. Thankfully there wasn't, but some additional lab work did raise some other red flags. Her crit had fallen to 20 from a normal of 31, which will require a two unit blood transfusion today and her potassium had also fallen to an unacceptable level also requiring a transfusion. She is very uncomfortable, with pain and nausea issues, so to put it mildly, it hasn't been a very good day so far. She is fine with staying another day as she knows that they can best care for these issues right here in the hospital.

There is good news to share, the catheter is out and her kidney continues to pump out those fluids!

Friday, October 29, 2010

Day 4 - Friday at 8:00 pm,,``

Laps Around Nurses Station

Nicole & Marie Playing Hairdresser

Very Good Day!

All has gone well today. The new kidney has continued to put out copious amounts of fluid, the lab reports were good, and she is no longer receiving IV fluids. Laps around the nurse's station are becoming easier as she regains her strength and it's T-Minus 3.5 hours until the dreaded catheter is removed. As of midnight she will no longer be attached to any machines! Incredible how far she has come in only four days.

We're still hoping for a Saturday morning discharge, which at this point is dependent on good morning labs and her ability to prove to the physical therapists that she is mobile enough. While it will be really nice to have her home, we are smart enough to realize that if she's not ready that she should stay a little longer. On the other side, everyone knows the worst place to be when you're sick is in a hospital due all the nasty "bugs" that run around here.

Debbie was under the weather today and had to stay home, so it's been a difficult day for her not being able to be with Lyss. She is feeling better tonight and plans to be part of the evacuation team if Lyss leaves tomorrow.

Following are some of today's key labs:

BUN.............. 12
GFR.............. 59
Pottasium.... 3.5
Creatinine.... .89
Glucose........ 110

Talk to you tomorrow

Day 4 - Friday at 11:30 am

Good morning - all still going well. Last night was relatively quiet with Lyss getting some much needed rest. Urine output has remained at a high level, allowing the doctors to begin weening her off all the extra fluids that they are pumping into her. It also means that she can rid herself of the catheter at midnight tonight. Talk about your "midnight madness," the catheter has been Alyssa's biggest issue following the surgery, so this is really good news! She has begun the countdown!

Still don't have the lab results from today, but will post as soon as available. We're off now for a walk around the halls. If we can build up her strength, a Saturday discharge may be in her future.


Thursday, October 28, 2010

Transplant Day 3 - 1:30pm

It may not be gourmet quality, but Alyssa just had her first solid food, post transplant. Spaghetti was the main entry with a side of rice. Another milestone reached.

Transplant - Day 3 11:15am

Jen has loaded up the flowers and checked out of the Inn. She's now on her way to her daughter's birthday at school.

Thank You Jen - we love you!

The Deffs

Transplant - Day 3 10:30am

What a Difference 24 Hours Makes!
Lyss is doing so much better, both physically and emotionally following a pretty good night's rest. This morning she took her first lap around the nurses station, and had very little discomfort in doing so. Our goal is for three more round trips today. She is still on a clear fluids diet, but hopes that this will change soon. Her biggest complaint is the catheter, which will probably be staying with her for a couple more days.

She will receive her third dose of Soliris this afternoon at 1:00 pm. Later today I'll write a post that details what the Soliris does and its important role in Lyssa's future.

One lab result: Creatinine...............1.1 - absolutely phenomenal - even the doctors are blown away.

Finally, but most importantly, Lyss just looked at me, and with the biggest smile said "I'm so glad I did this - I feel so great!" "It's been a long time since I felt like this."

Please pass the tissues!


Wednesday, October 27, 2010

Transplant - Day 2 - 7:00 pm

Bad Start - Good Finish

What started out as a bad day is ending on a much higher note. Nothing seemed to be going right for Alyssa following a sleepless night. Her catheter was not working properly; her meds weren't arriving on schedule; her i.v. line went bad; she needed to have a PIC line surgically implanted in her arm to better regulate her drugs; her blood pressure was elevated; she had to have an ultrasound requiring pressure right on her new incision site; she was out of her room for several hours for tests, and, well, it was time for an emotional breakdown and a good old fashion cry. She has been through so much over the last 20 months and it kind of all came to an emotional head today. Things started improving around 5:00pm and have continued upward since.

AND...the kidney is still pumping out large amounts of urine and her lab reports are very good. For the medically inclined, here are a few key numbers:

GFR...........................................3................................................. 18
Creatinine...............................17.2.............................................. 3.2

These are amazing changes and show that we are headed in the right direction. She's sitting up at the edge of the bed for a short time and having her first post-op food - broth and jello. Most importantly, her smile is back and she's talking up a storm. All-in-all she's about where the doctors had hoped she'd be. They have a plan and it's going as drawn-up.

On the other side of the unit, Jennifer is recovering nicely. Although still very sore and a little groggy, she is up and around and on solid foods. The plan is to have her discharged tomorrow at 9:00 am so that she can go to her daughter's birthday party at school. She'll be spending the next few weeks at home to help get her strength back.


Tuesday, October 26, 2010

Transplant Time - # 7 10/26 - 5:30 pm

It's Working!
Back in the Room

Alyssa has arrived back in her room. She actually feels pretty good, certainly better than poor Jen does right now. This is pretty common as the transplant recipient is so used to feeling lousy, that just by adding a kidney and the resulting benefits that come with it will make a huge difference in how they feel. She's still drifting in and out but when awake, she is so excited and very expressive about what has happened today. Thrilled to have the surgery behind and ready for the next step. What she (and mom & dad) need, is a good nights rest. We are all going on 40 hours without any real sleep.

Tomorrow brings a new day filled with hope and optimism.

Let's Rock!

Transplant Time - # 6 10/26 - 3:15 pm

She's out and doing great! Alyssa made 150 CCs of urine in the first 30 minutes and has been going strong ever since. She is now in recovery and expected to remain there for another two hours. Dr Marroquin, Alyssa's lead surgeon, said that he was very happy with how the surgery went. He said that Alyssa has very small veins and arteries, so she will need to be on extra blood thinners over the next few days, but that all else went very well. Poor Dr Marroquin... he quickly found out what a DeffenHug is. We thank him and his his team for their skilled hands!

I'm normally not at a loss for words, but am at that point now. This has been such an incredibly emotional day for our family. While we know that this is just the first mile in what will be a marathon for Alyssa, we can now say that the "race is on." Thank you to all of you for your words of encouragement and especially for your prayers. They mean more to us than we can say. We ask that you keep them coming in the days and weeks ahead.

aHUS is still enemy #1 to us. With our wonderful team of doctors, the drug Soliris and your support, we will win this battle!

Next update when she arrives back in her room tonight.

Transplant Time - # 5 10/26 - 3:00 pm

Jen is out of recovery and back up on our floor. Our hero is in a great deal of discomfort, but says that she absolutely made the right decision and, like the rest of us, is looking forward to watching Lyssa regain that quality of life that left last year.

We haven't told you much about Jen, and that's only because that's the way that she wants it. She is an intensely private, religious and loving person, who just doesn't understand why such a big fuss is being made about what she is doing for Alyssa. She felt that it was God's calling for her to undertake this incredible act of kindness; and that it's only natural to want to help a loved one.

We have always known that Jen was a very special person. She came into our nephews life several years ago and immediately showed through example, the value of church and family and gave him a sense of pride and accomplishment that had been missing in his life...You might say that she also brought Michael a better quality of life. As he always says, "I'd be lost without her in my life." Together they are raising their son Louis and their daughter Leona. She has always handled with grace and poise all the obstacles that life can throw at you.

We all want to say that we made a difference with our time spent here on earth. Jennifer, while still in 20's, and by just being herself, has already touched and improved more lives than most people ever will.

Thank you Jen for helping us take the most important of steps in helping Alyssa get her life back. May others use your incredible act of kindness as a guiding light for all of us to find a way to help others.

The Deffs will never be able to thank you adequately for what you have done for us. We can tell you how much we love you and that we will try to "pay forward" what you have done for us.

Transplant Time - # 5 10/26 - 1:00 pm

Just heard from a member of Alyssa's surgical team - still about two hours to go, but everything is going as planned. The best news is that the surgical team has done what they call the "Pee Dance" which is what they do after the transplanted kidney makes it first urine. Another great milestone that we've passed! So get out your favorite dancing shoes, pick a great tune and join us in a rousing version of the "Pee Dance." - Gentlemen, please remember to lift and lower the seat!

Transplant Time - # 4 10/26 - 11:00 am

Jennifer has finished her surgery and came through with flying colors. Her surgeon, Dr Berry reports that she gave Alyssa a beautiful pink kidney, which is now being placed into Alyssa. Jen will be in recovery for another couple hours and Alyssa's surgery will continue for two to four more hours. So far so good! If this kidney squirts as much fluid as our tear ducts have it will be a successful procedure.

Transplant Time - # 3 10/26 - 9:00 am

Jen & Alyssa in PreOp

Both Jen & Alyssa are in the OR. Jen has been in for about 90 minutes, with Alyssa following an hour later. The wait begins and anyone that knows me knows that I am not a patient person. We are very confident as they both are in good hands.

Transplant Time - # 2 10/26 - 6:00 am

Alyssa is just finishing her second treatment of Soliris. This dose was 1200 milligrams versus the 900 she had on Tuesday. The infusion is given just before the surgery to ensure that her protection is at the highest level during and after the surgery.

We were just informed that they will take Lyss down at 6:30 am to the OR. Jennifer, her donor is already there. Alyssa's part of the procedure will start around 8:00 am.

We're rolling - off to the ER.

Monday, October 25, 2010

Transplant Time - #1 10/25 - 9:00pm

Jen & Alyssa - What a Team!

First Soliris Treatment

The waiting is over...we are all sitting in Alyssa's private room in the University of Rochester Medical Center (URMC.) After a rather loud, “Glee” filled ride to the hospital (led by “River Deep, Mountain High”,) we have settled in and are all anxiously awaiting Alyssa's Kidney Transplant, which will take place tomorrow morning around 6:00am. It has been a long, tough road for Alyssa, but we are all hopeful that she is about to see a big change for the better beginning tomorrow! Although she is very excited about the potential of getting her life back, she is understandably very nervous about entering into the uncharted waters of this procedure.

It's been a while since we've posted, so I'll provide a brief overview as to what the plan of attack is, then provide continuous updates as we get into the transplant and related activities over the next hours, days and weeks. Each update will show a time and date. For a detailed history as to how we got to this point, please see the older postings that began in January 2009, when Alyssa was diagnosed with Atypical Homiletic Uremic Syndrome (aHUS,) which immediately resulted in total kidney failure.

Without going into too much detail, Alyssa will be only the second patient to ever undergo this procedure in the US and only the fourth in the world...the first transplant in the US was performed just two weeks ago at the University of Iowa Medical Center on a 13 year old girl who is doing just great! The transplant itself isn't what concerns us the most - it's the return of the aHUS that almost took Alyssa from us a couple of times in early 2009. The doctors tell us that the odds of the aHUS returning following a kidney transplant are 80-90%, which is why kidney transplants on patients that suffer from aHUS are rarely performed.

Due to research at the University of Iowa & the Mayo Clinic, doctors have found that an "orphan drug" named Soliris, which was developed specifically to treat Paroxysmal Nocturnal Hemogloginura (PNH,) has shown great promise in treating individuals with aHUS. However, all the treatments performed to-date have been on patients that had aHUS, but, had not lost the use of their kidneys. So far it has worked very effectively with these patients.

Soliris is only reason that Alyssa will be able to proceed with her kidney transplant. Without its ability to fight off aHUS, the doctors would never attempt this very risky procedure. Whether or not the Soliris works and how the new kidney reacts are questions that no one can answer as there is no history or precedent. But this we know, three years ago, Alyssa would probably never have survived the aHUS attack. Six months ago, they would never have attempted the transplant. Medicine keeps moving forward and we can only hope that we at the point where they can give Alyssa back her life. In 2009, her life as she knew it was taken from her. She may look great on the outside, but on the inside is a body and soul that has been ravaged by this awful disease. Her beautiful smile hides the daily battles that she facing doing even the most basic of activities. As many doctors have told us, she is battling a horrific disease, but at a time when there is hope to help her. It sounds cliché, but all of her lead doctors have told us that if this were their daughter, they would proceed as we are.

We ask that you keep Alyssa and Jennifer in your thoughts and prayers tomorrow and over the coming weeks. Your support has helped get her this far, now we need it for the next big step.

We are not naïve to what we are about to face. The two page addendum to the standard surgery consent form, listing 15 additional items specific to Alyssa’s case, is a chilling reminder of this, but we are very optimistic that this is all going to work perfectly for her. God has opened many doors for us to get to this point and we are praying that he continues her healing and that her donor, Jennifer, flies through this with ease. Thanks for your support and prayers.