2/27/09 - We Love ya Lyss - Video

Lights, camera, action...

You are cordially invited to the world premier of

Get Better Soon Lyssa

a special documentary featuring Alyssa and numerous special guests, shot in her preHUS days.

Filmed, directed and produced by Steph Hasenauer, who has been nominated for Oscars in all three categories.

The red carpet has been rolled out, the search lights are scanning the sky, the paparazzi are in place and we've saved a special seat for each of you. So pop some popcorn, get a comfortable seat and sit back and enjoy.

2/26/09 - Two Good Days

We hate to jinx her, but Alyssa has had two good days in a row relative to blood pressure...the best two since she fell ill in January. This can be attributed to getting her down to her target dry weight and an increase in her BP meds. Whatever the reason, it is so nice to not have to worry about how we'll get her numbers down.

Friday, Alyssa is scheduled for a plasmapheresis treatment, then an introductory meeting with the Peritoneal Team at the Dialysis Center. http://en.wikipedia.org/wiki/Peritoneal_dialysis. This form of dialysis will allow Alyssa to live a more flexible life style. Within the next few weeks, she will have a catheter surgically implanted into her abdomen. As she sleeps each night, fluid will be placed within her peritoneal cavity through this catheter. The solution, which performs the dialysis function, is then drained from her abdomen before she awakes. This will eliminate the need for her to go through daytime dialysis, which is currently set at three times per week. It will also allow her to travel as this process can be performed most anywhere. While we haven't yet given up hope that her normal kidney function will return, each passing day makes it less likely, so we are now looking into long term alternatives such as peritoneal dialysis and kidney transplants.

Thanks to Aunt Sandy for performing "nurse" duties tonight so that Dave and Debbie could go to dinner at the home of their good friends, Jim & Sue LeBeau. It was great to talk to people that didn't have a stethoscope hung around their neck!

They have begun the genetic analysis at the University of Iowa, but we have yet to receive any results.
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2/24/09 - Monday Monday, Can't Trust That Day

Monday Monday, sometimes it just turns out that way - The Mamas and the Papas were right on target for Alyssa's Monday. She met with her hematologist in the morning, followed by a plasmapheresis treatment; things were looking good. But then her old nemesis kicked in again - blood pressure issues!

By noon her pressures had reached uncomfortable levels, so off we went to the Dialysis Center to have some fluid removed. Alyssa's dry weight http://www.davita.com/dialysis/treatment/a/1930 target, which is 60 kilos, had mistakenly been changed to 64 kilos on her dialysis chart. As a result of this error, she was carrying six extra kilos of fluids that should have been removed during her last few dialysis treatments. Extra fluid = high BP. They removed two kilos Monday, getting her down to 66 and plan on removing four more kilos during her regular Tuesday treatment. On top of this, her nephrologist, increased the doses on a couple of her prescriptions.

With all of these changes you'd figure we'd be on easy street Monday night. Wrong - her BP continued to rise into the evening leaving Alyssa with a feeling of "what do I have to do to get my BP under control?" To Alyssa, high BP equates to a fear of seizures. Something that none of want to see her go through again.

We're happy to report that by the 2:00 AM dose, her BP started to fall and has remained down this morning. She is now in her second hour of Tuesday's treatment and her BP is 149/87, which is very good for her. However, we're not going to start celebrating just yet as we have been down this path before.

So, what started as a two hour treatment on Monday morning, ended up being nine hour session that ended at 5:30 PM Monday night...sometimes it just turns out that way.
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2/22/009 - Just Waiting for Monday

The last couple of days have not been the best relative to BP, water retention & a high pulse. Whether you're in the hospital or at home, weekends are tough when your sick. It's difficult to connect with your doctor(s) so you end up with the doctor on call, who typically isn't familiar with your case; so for the most part you find yourself just hanging on for Monday morning to connect with your physician.

As parents, we just don't know when we have reached a critical stage that requires immediate attention, or just a set-back that can wait until the next "work day." Everything out of the ordinary is scary, especially when you're dealing with the issues that we are with Alyssa. We feel like we have been hanging off a ledge for several days now. Alyssa's BP has been much higher than it was a week ago, which is most likely due to fluid retention. Yesterday, Alyssa was 12 lbs over what they call her ideal "dry weight." All of this is due to fluid retention. They removed four pounds through her dialysis on Saturday and plan to get five to six more off on Tuesday. But, in the meantime, the excess fluids drive her BP to levels that leave us very concerned. So Monday just can't come soon enough. We'll be meeting with Dr Kouides, Alyssa's hematologist, during her plasmapheresis treatment in the morning. He has been one of our guiding lights during this ordeal, who along with his incredible knowledge, brings a sense of calmness that we have all valued over the past month.

We'll update you after our "reunion" with Dr K.
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2/20/09 - Just the Facts

A brief update with nothing but the facts as they are tonight:

Blood pressure is still all over the place. One hour up, the next down, but mainly up. Extremely frustrating and definitely taking a toll on all of us. We often feel that we are put in a position of having to decide on med changes - a position that none of is comfortable with being in. We have gone from "Intensive Care" to "Parent Care."

Kidneys are still not functioning, but Alyssa has produced some "pee." Nothing to get excited about at this time, but it is a change in the right direction. Whether or not Alyssa's kidneys will again perform their bodily functions is yet to be determined. If functionality is not regained within two months of the initial failure, they are determined to be in permanent renal failure status...Alyssa is now at five weeks

The plan is to get three plasmapheresis treatments next week, followed by two the following week.

Dialysis treatments will remain at three per week indefinitely.

Alyssa's three primary physicians return from vacation next week and we plan to meet with all of them.

No word yet on results from the genetic testing being performed at the University of Iowa.

She remains on 12 different medications that are taken between 6:00 AM and midnight.

Her new renal diet is a killer - no pizza or mac & cheese - Alyssa favorites.

Despite all of the above, Alyssa is trying to remain optimistic, although she's had a few difficult moments the last couple of days trying to deal with enormity of it all. She hasn't had any easy days lately and it's beginning to take a toll on her.
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2/18/09 - A Bottle of Red, A Bottle of White

Today was plasmapheresis day. The process went well and Alyssa's blood pressure readings stayed within an acceptable range. Over the past month you have read here about this amazing pheresis machine and how much it has impacted both Alyssa's and her father, Dave's lives. Many of you have asked what you can do help Alyssa. With that in mind, we'll give you a little background on plasmapheresis then offer a suggestion.

Plasmapheresis is a blood purification procedure used to treat several autoimmune and other blood related diseases. The plasmapheresis process, which was invented in 1971, uses centrifugal force to separate blood into its components: red blood cells, white blood cells, plasma and platelets. Prior to the invention of this process, HUS resulted in a 100% fatality rate.

When Alyssa receives her plasmapheresis treatments, nurses from the Red Cross administer the procedure. There are four nurses she typically sees--Holly, Heidi, Mary Ellen and Bobbie. They are our saints and second "moms" to Alyssa. They have always been very supportive and informative and we so appreciate their expertise. They bring far more to the table than their knowledge...they bring a passion and caring for the patient that is second to none!

The high cost of a pheresis machine makes it cost prohibitive for the local hospitals to purchase their own machines; so we owe a great deal of grattitude to the Red Cross and their incredible staff for filling this void. Countless lives have been saved as a result of this process and the care and expertise with which our "magnificent four" administer it!

Each time the procedure is done (probably 30-35 so far) the hospital or i.d. tags are read and matched to the bag of plasma (usually 12 bags) individually. They repeat her name, i.d. number and blood type and match it to the blood bag for each of the twelve bags. Alyssa has of course become quite familiar with all of this and her "A-positive" blood type. Today for the first time she heard "A-negative" read out during the matching process and of course questioned it since she is positive. Holly explained that an A-positive person can receive A-negative blood but not the other way around. We are hoping Alyssa hasn't already depleted the A-positive blood supply!!!

Both Dave and Alyssa have been able to be treated and keep their conditions under control thanks to plasmapherisis and the Red Cross. When most of us think of the Red Cross, thoughts of disaster and fire relief come to mind and of course...blood donations. We all know how important blood donations are when we think of accident and trauma victims and everyday surgeries as possibly requiring blood transfusions; but never realized how many diseases are helped by them as well. It is unlikely anyone would want any "Deffenblood" anymore, but we are really thankful for anyone who does donate the gift of life through their blood.

So here's where you can help. There is always a need for blood donations, especially with the drain that the "Deffs" have put on the local supply. What you may not realize is that there are different types of donations. Whole blood donations are the most common, with the primary components of whole blood being red blood cells, platelets, plasma, and cryoprecipitate.
Apheresis is the process of removing a specific component of the blood, such as platelets or plasma, and returning the remaining components (red blood cells and plasma or platelets respectively) to the donor. This process allows more of one particular part of the blood to be collected than could be separated from a unit of whole blood. For example, the amount of platelets collected in one apheresis donation is five to eight times more than in a regular blood donation. Platelet apheresis donations also allow donors to give a lot more often if desired. Platelet donors only need to wait 3 days before they are eligible to donate again, versus 56 days for whole blood donors. Fresh, frozen plasma is what Alyssa receives three times per week. Dave receives intravenous immunoglobulin (IVIG) which contains the pooled IgG immunoglobulins (antibodies) extracted from the plasma of over one thousand blood donors.

Whole blood is red in color, plasma is yellowish and IVIG is clear. So whether you want to donate a "Bottle of Red or a Bottle of White," be assured that your donation will make a difference in someones life...it already has in the Deffenbaugh's!
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2/17/09 - Dialysis Issues Strike Again

After two rather uneventful days, today has broken pattern and not been the easiest for Alyssa. Dialysis days are never easy, but we were hoping that her recent good blood pressure readings meant that this might change. I guess that were still not at that point yet.

For whatever reasons, Alyssa awoke this morning with nausea and a general feeling of malaise. Her BP was up slightly, but not too high for concern. This would be her first dialysis session at the Center on East Ridge Road, so she had to be there an hour early for paperwork followed by her normal three hour session. As the treatment went on, her BP continued to rise, with a final reading of 166/119...levels that we hadn't seen for several days and had hoped were behind us. What is so difficult for us is not knowing if the BP rise due to the treatment itself or are her meds being drawn out by the dialysis machine during the treatment. There doesn't appear to be any agreement within the medical profession as to which medicines are impacted by dialysis. It's very frustrating not having this information available, especially when you think of the millions of individuals that need dialysis, this should be basic info!

It's now 3:45 PM and she has been sleeping upstairs for about an hour now - her BP is back down to 126/88 and we're all taking a deep breath again. Alyssa can't help but worry about seizures every time that her BP rises and/or when she feels "shaky." She is on such a roller coaster ride.

As noted Sunday, Alyssa has found a fellow HUS patient in Arizona. They are communicating regularly through AIM and are both excited to have found someone that understands what they are going through. They both fell ill in January. Her new friend is 28, and married with a three year old daughter. . Her mother, who is a nurse, has moved in with her to help out. We ask that you keep her in your thoughts and prayers also.

It's rather quiet around the house now as the "brothers" are all off skiing at Holiday Valley for a few days. This has been an annual trek for the Deffenbaugh's, so we're very happy that Mark could continue the tradition by taking his younger brothers down to Ellicotville. We all plan to go next year!

Just to get an idea of how life has changed for Alyssa - tomorrow morning when you get up, note the time and think of what it would be like to be in a chair attached to a machine for the next three hours. That's just part of her daily regiment; but we have yet to hear her complain about it. Mom & Dad want to know why, but not Alyssa. She has made us both very proud.
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