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.