Saturday, February 28, 2009
Thanks for your patience!
(See, I told you there would be no anger)
Today's update is # 51. A great deal has happened since that first posting on January 14th...we have gone through the lowest lows and have felt some great highs watching Alyssa fight back against strong odds. We haven't shared everything with you, but have always tried to keep you informed as to the major details surrounding this horrible disease, HUS, and its impact on Alyssa and to a smaller degree, our family.
Tonight's update will have a little different tone to it. In her 1969 book, Elisabeth Kubler-Ross introduced her Kubler-Ross model relative to grieving. Although we usually associate the concept of grieving with death, it can also be applied to other major/catastrophic changes that impact our lives. As a reminder the five steps are:
Personally, I didn’t have time for Denial as everything was happening so fast. I also skipped Anger and went directly to Bargaining, after being told that Alyssa might not live through this. I’d have made any deal, and still would, to ensure a good outcome for Alyssa. Depression, well, that's popped in and out since the start; and Acceptance – sorry, none of us are ready for that!
So Anger is where I’m at now. Angry that this horrible disease, which thankfully strikes so few individuals, somehow found my daughter. Angry that her life will never be the same again. Angry that she rarely feels at peace anymore, worrying about what lies ahead. Angry that she needs to spend parts of six days a week attached to machines. Angry that she may need to be on some form of dialysis for the rest of her life. Angry that at 21, she should be out enjoying life to its fullest, not stuck at home too weak to move. Angry that when she finally does get out, she barely has the energy to walk into a store and back to the car. Angry that many of her so called “friends” have slowly disappeared (but also very thankful for those that have provided her with much needed support.) Angry that important tests were not performed early on that might have changed her outcome. Angry that I can’t find the right thing to say when I find Alyssa and/or her Mom crying. Angry that we don’t all wake up to find this nightmare over with.
I’m angry, but I’m also very thankful for all the incredible support that Alyssa has received from family, friends, strangers, and especially from her team of physicians and nurses that have worked tirelessly to help her.
I promise that # 52 will be void of any overt anger. Anger is a wasted emotion, but one that we all need to use at times so that we can move on. Thanks for allowing me this opportunity to vent.
Friday, February 27, 2009
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.
Thursday, February 26, 2009
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.
Tuesday, February 24, 2009
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.
Sunday, February 22, 2009
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.
Friday, February 20, 2009
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.
Wednesday, February 18, 2009
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!
Tuesday, February 17, 2009
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.
Sunday, February 15, 2009
A brief word on the discharge process - We're not quite sure how seniors or those living alone can get through all of this without help. Alyssa is taking 10+ medicines, so she may not be the best example, but between going through the discharge instructions, filling the prescriptions and setting up a daily schedule, this became a very time consuming and confusing project. We ended up creating a spreadsheet in Excel which helps us better track all the elements of Alyssa's care. But it is a very fluid process, especially when you are trying to control blood pressure. The timing and dose of the meds are constantly changing - if her bp drops too low we back off on her next dose; too high, we move up the timing, which then changes the whole timetable. It's a challenge, but one that we embrace enthusiastically!
Alyssa started off her day by going to Greece Baptist Church's morning service. She was recently baptized there along with her brothers, Ryan & Kyle. For varied reasons, the three of them did not complete the classes at age 14 which is the typical timing. Pastors Steve, Bob and Sarah have visited with Alyssa in the hospital and many members of the congregation have followed her progress through this blog. Sarah was surprised and delighted to see Alyssa walk into the service (late of course;) recognized her presence and gave thanks for her incredible progress. It was a special moment for all of us!
Yesterday we promised a word on the diagnosis process. Based on all data received to-date, Alyssa has been tentatively diagnosed with Hemolytic uremic syndrome (HUS) a disease that occurs primarily during infancy and early childhood. It is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Alyssa's is called Familial, Atypical HUS, which affects less than 10% of all HUS patients. It is largely due to mutations in the complement proteins factor H, membrane co-factor protein and factor I, leading to uncontrolled complement system activation...basically, she has a defect in her complement gene. As mentioned before, this disease normally strikes kids under the age of 10; rarely in the teens and almost never beyond 20. To say that Alyssa is in rarefied air would be a gross understatement. Some reports state there are as few as 300 - 600 cases within the USA. And almost all of these involving children under 12. Alyssa did find a 28 year old patient living in Arizona and has begun communicating with her.
The way her hematologist, Dr Kouides describes it, Alyssa had to fall victim to the perfect storm - just the right mix of circumstances to "tip her gene" and cause her to fall ill. The fact that she made it to age 21 before becoming symptomatic can be looked as a positive sign for her relative to relapses. It may be another 20 -30 years before she experiences a potential relapse or maybe never.
We are a generation that now runs to the Internet to uncover information on every topic of interest. The information you will find relative to HUS can be very frightening. With so few impacted by this disease, information reported isn't necessarily the most current and at times inaccurate. It is a nasty, nasty disease, but Alyssa has defied the odds and beaten it so far and we expect nothing less than more of the same from her in the future.
There is no real way to predict what the future will hold for Alyssa. The genetic testing that she is currently undergoing may shed more light on this topic along with the exhaustive research that Dr Kouides and his colleagues from throughout the country have put into her case. It may or may not confirm the diagnosis. For now, we will take it one day at a time and celebrate each step forward. Alyssa wouldn't have it any other way!
Saturday, February 14, 2009
On Monday she will begin her out-patient treatments on a six day per week basis. Sunday is her off day.
We'll give you an update tomorrow as to a more specific diagnosis and where we go from here.
Happy Valentine's Day to all!
Friday, February 13, 2009
However, it did help us to make a decision though...when she leaves the hospital, (yes, you read that right,) she will need to continue dialysis and plasma pheresis treatments on a three times per week, each, basis. Two per day is just too many, so Alyssa has decided to go six times per week for shorter periods of time, versus seven-hour sessions, three times per week. So here is her future out-patient schedule. Plasma Pheresis - M-W-F at 9:00 am & Dialysis - Tu-Th-Sa at 6:00 am. Alyssa is not a morning person, so getting to Irondequoit by 6:00 am may be our biggest, future challenge. The fact that she can't drive for a while due to her seizures, will put mom & dad right square in the driver's seat...creating our second biggest future challenge. Why 6:00 am? Time slots for dialysis are difficult to come by. The other option was 9:00 pm.
Blood Pressure had a couple hiccups today, for the most part has been very good. Blood labs were also excellent. She is scheduled for a dialysis treatment on Saturday morning, then nothing more until Monday morning.
So just when will the heroine of our story be going home? You'll have to keep blogging with us to find out, but don't stay away too long....
Thursday, February 12, 2009
She has made amazing strides in the last 48 hours. She's up walking and out exploring this mass of hallways and rooms that has been her home for the last two weeks. She commented that it was nice to see the walls and not just the ceilings that she saw as she was wheeled from room to room. Tonight we had dinner in the Twig Cafe with her Aunt Donna and Uncle Brian. As we all sat there talking, it suddenly hit me that there at the table was a smiling, happy Lyssa, sitting in a spot that her mother and I had shed so many tears over the past two weeks. It was a moment that we thought might never come, much less this fast. It felt great and it's something that I will treasure forever.
Blood pressure levels have been great. So much so that they are now beginning to lower a couple and eliminate one.
Her blood labs are so promising that they will now begin tapering her off of the plasma pheresis treatments. Three each for the next three weeks, then two per week, then hopefully none.
On Monday, three vials of Alyssa's blood will be sent to the University of Iowa for genetic testing. They are specifically looking for a genetic defect in what is called the complement gene. This defect can cause severe cases of HUS. Having this information is crucial to determining future treatments and helping in preventing relapses. Preliminary results are expected within 3-5 days. This type of testing is very expensive and we are very thankful to Rochester General Hospital for underwriting the costs. Just another example of how they go above and beyond at RGH!
Like spring training, anything can happen in life and has with Alyssa. She is beginning anew, with a new team of players behind her, optimistic about the future and ready to accept the many changes that will challenge her in the coming months.
Wednesday, February 11, 2009
Blood Pressure - So far there have been NO spikes or abnormally high readings. She has stayed within the desired ranges throughout the day, even during her dialysis treatment, which was a first. We don't want to jinx this, but we're cautiously optimistic that we're getting closer to finding the correct doses that will keep her BP in check.
Walking - she's up and around, beginning the process of getting her strength back. She's also excited that she will no longer need heparin shoots in her belly. She certainly has some very colorful bruises, often referred to as a "Heparin Belly."
Her blood levels remained strong today, so she is beginning a slow withdrawal from the prednisone. This will take a few weeks to accomplish as they are very careful not to withdraw it too fast.
Results: While we still don't have a definitive cause/name for what has made Alyssa so sick, we are starting to eliminate more and more possibilities:
High Blood Pressure - which came first, the high BP or the kidney failure? On the day that Alyssa first entered Unity Hospital, her blood pressure was normal at our GP's office. However, later that evening when she was admitted to the hospital, she was already suffering from acute renal failure and high BP. This leads the doctors to believe that the high BP is a result of the renal failure, not vice-versa.
Lupus and ADAMSTS13 tests came back negative, but may have been tainted by numerous transfusions. Other tests for both are pending.
Several other smaller tests were all negative.
As all of her neurological tests have been negative lately, Neurology signed off on Alyssa today, asking only that she come back for a follow-up MRI in three months.
All-in-all, a very good day!
Tuesday, February 10, 2009
We're running out of ways to discuss the same issue, so we'll lead with Wikopedia's definition. BP isn't Alyssa's only issue, but it is the main one now. This morning her readings were 149/96, not in the normal range, but not real bad either. Then as the morning wore on (and the pending arrival of the dialysis machine grew closer) up went the pressures...160/105; 160/120. Yet another adjustment was made and down again came the numbers, where happily they have stayed for the balance of the day. We'd love to have just one day of consistent, normal numbers.
Alyssa is adjusting to life on a regular floor. She misses the 1/1 attention that you receive in the ICU, but is very happy to have improved enough to warrant not needing that level of care. Unfortunately there is a very loud patient next door. Sleep will be very difficult, but no complaints.
Tonight we're proud to bring you a very special guest writer, the person behind this blog, our miracle girl, Alyssa:
I just wanted to come on and thank everyone from the bottom of my heart for everything you have done for me and my family, and for following this blog. This has been one of the toughest things my family and I have gone through, and we are so thankful to have such great friends that we know we can count on. I believe that being in all your prayers has really helped me get through this; so please keep on praying and so will I and I will get through this. You all mean so much to me, and this definitely opened my eyes to the people around me and how loving and careing my friends and family are. Also thank you to everyone who has come up to visit me, it is wonderful to have visitors to keep me company since it can get a little boring up here, and I miss everyone so much! So keep on coming please!! Well in finishing I love you all so much, and keep on reading, it means so much! Thank you all for everything, I will never be able to thank you enough.