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.

dd

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