Saturday, November 29, 2008
There are good days...
...and today feels like one so far. Aiden is STILL in the best of spirits! He has ran all over this ICU and insists on pushing the buttons to open the automatic doors himself. All the nurses play chase and hide and seek with him and today we got permission to take him off the ICU to the pediatric playroom upstairs a couple times a day! YAY!
On the medical front, we still have not received any results back from Iowa. I think I told y'all in the last post that there are only 3-4 places in the world that can test to see which competent is faulty in the atypical HUS disease process....Iowa is the only one in the U.S. and is where Aiden's docs sent his blood. We are hoping to hear something next week. We skipped pharesis on Thursday and picked it back up yesterday and will have it again today. So far we cannot tell that it is working and that was really putting us in a bad mood. His kidney function labs are up a little bit today and he's having to have blood transfusions about every other day. These are things that the plasma exchange is supposed to help fix....(the plasma exchange takes out Aiden's plasma and replaces it with donated plasma that should have normal plasma proteins, not the faulty ones that Aiden's body makes) Dr. Quan (Aiden's pedi. nephrologist) is out for the weekend so one of his associates who works at Cooks Childrens in Fort Worth just came to check on Aiden. He has a patient with Atypical HUS and was able to give us some insight on what this could look like long term. He said that sometimes it takes a while to begin to see changes with the pharesis..but if/when we are able to get all of the faulty proteins replaced Aiden may go into a type of remission. His pattient only has plasma infusions (not even exchange! just infusions!) every couple of weeks and does fine! Again, that all depends on the type of competent that is faulty in Aiden because there are better and worse scenarios depending on that, but just to know that is a possibility lifts our spirits in the midst of talk about kidney and liver transplants and life long weekly therapy. Don't get me wrong, we'll do whatever it takes but this gives me something to hope for.
This is all starting to feel like a run-on sentence...I'm sorry...just spewing thoughts onto the screen...
I've had a few questions about HUS and wanted to clarify. Last year they thought Aiden had HUS that was diarrhea associated...that's usually caused by ecoli or some other type of bacteria...if you remember Aiden tested negative for EVERYTHING...this type of HUS is NOT associated with diarrhea or ecoli...it's genetic, in the DNA. When the immune system is stimulated it just sets things in motion and his body kind of overreacts and beats up the bad stuff AS well AS the good stuff (blood cells, kidneys, etc) Hope that helps...I know it is confusing. If you google "atypical HUS" it gives you some good info, but it's VERY hard to muddle through all of the terminology.
Please keep praying for my baby boy. I appreciate you all.
Love,
Christy, David and Aiden
On the medical front, we still have not received any results back from Iowa. I think I told y'all in the last post that there are only 3-4 places in the world that can test to see which competent is faulty in the atypical HUS disease process....Iowa is the only one in the U.S. and is where Aiden's docs sent his blood. We are hoping to hear something next week. We skipped pharesis on Thursday and picked it back up yesterday and will have it again today. So far we cannot tell that it is working and that was really putting us in a bad mood. His kidney function labs are up a little bit today and he's having to have blood transfusions about every other day. These are things that the plasma exchange is supposed to help fix....(the plasma exchange takes out Aiden's plasma and replaces it with donated plasma that should have normal plasma proteins, not the faulty ones that Aiden's body makes) Dr. Quan (Aiden's pedi. nephrologist) is out for the weekend so one of his associates who works at Cooks Childrens in Fort Worth just came to check on Aiden. He has a patient with Atypical HUS and was able to give us some insight on what this could look like long term. He said that sometimes it takes a while to begin to see changes with the pharesis..but if/when we are able to get all of the faulty proteins replaced Aiden may go into a type of remission. His pattient only has plasma infusions (not even exchange! just infusions!) every couple of weeks and does fine! Again, that all depends on the type of competent that is faulty in Aiden because there are better and worse scenarios depending on that, but just to know that is a possibility lifts our spirits in the midst of talk about kidney and liver transplants and life long weekly therapy. Don't get me wrong, we'll do whatever it takes but this gives me something to hope for.
This is all starting to feel like a run-on sentence...I'm sorry...just spewing thoughts onto the screen...
I've had a few questions about HUS and wanted to clarify. Last year they thought Aiden had HUS that was diarrhea associated...that's usually caused by ecoli or some other type of bacteria...if you remember Aiden tested negative for EVERYTHING...this type of HUS is NOT associated with diarrhea or ecoli...it's genetic, in the DNA. When the immune system is stimulated it just sets things in motion and his body kind of overreacts and beats up the bad stuff AS well AS the good stuff (blood cells, kidneys, etc) Hope that helps...I know it is confusing. If you google "atypical HUS" it gives you some good info, but it's VERY hard to muddle through all of the terminology.
Please keep praying for my baby boy. I appreciate you all.
Love,
Christy, David and Aiden
Wednesday, November 26, 2008
My sweet Aiden
First off, I want to thank everyone for their thoughts, prayers and words of encouragment. Although we are unable to respond to everyone or update everyday you will never know how much it means for us to know that everyone is pulling and praying for our little toot. Please don't stop! We need these prayers now more than ever and I know they are what are helping to keep David and I sane. Some of this may be repeat info. for you but I wanted to give a clear picture of what has happened the last week and what is going on now.
Last Monday (Nov. 17th) we brought Aiden to Dallas for his routine 6 month checkup with his nephrologist. He looked great and they drew routine lab work and told us they would see us in 6 months for a recheck. On Wednesday we got word back that his lab results (his kidney function labs BUN, Creatinine) were abnormal. We took Aiden to the local ER that night to have them redrawn, hoping it was a fluke. When they came back abnormal again we were transported to Dallas to Medical City Children's Hospital pediatrics so that he could have further testing by his pedi. nephrologist. During all of this Aiden was in GREAT spirits..happy, playing you would never know that anything was going on kidney-wise. His urine output continued to be normal. He did have the croupy-cough that refuses (even now) to go away but other than that he looked GREAT! They watched his labs Thursday and Friday and saw the BUN/Creatinine rising so decided to do surgery to put in a peritoneal dialysis catheter (like Aiden had last year) and do a kidney biopsy and place a central line all at the same time. After the surgery we were moved to the PICU because Aiden was having some arrythmias and they wanted to watch him over night. At that point his potassium levels were getting higher and they went ahead and started dialysis that night too. He did well on the dialysis for a little over 24 hours and they took him off to see how he'd do without it....
The biopsy showed that Aiden has chronic kidney injury and a rare form of HUS called atypical. It is so rare that there is not an excepted course of treatment, it is trial and error. Doctors have just been making headway in figuring out what this type of HUS does and how to treat it in the past 10 years. It is genetic and basically his immune system goes overboard when stimulated (illness vaccines) and attacks his kidney and blood cells as well as bacteria, etc. There are several different types of atypical HUS and some differences as far as treatment depending on the type. There are only a few places in the world that even know how to test for types of this and it is not an absolute given that they will be able to narrow it down at all. They test for this in Iowa, France and Italy. Aiden's blood work has been sent to Iowa and we are waiting for results but I have no idea when to expect them.
In the meantime they are performing plasma exchange (plasma apharesis) on Aiden daily. The proteins that are abnormal in Aiden and responsible for the attacks on the kidneys and blood cells basically "live" in the plasma. They take Aiden's plasma out and replace it with healthy FFP's (fresh frozen plasma). The idea is that eventually we will rid his body of the bad proteins and replace them with good ones therefore stopping the attacks on his blood cells and kidneys. They are uncertain how long it will take to get him to a normal level or how often we will have to do apharesis to keep his levels normal. It will be an ongoing thing, it's not a one time fix, but hopefully it will prevent him from having to be on chronic dialysis.
At some point in the future (a year? maybe longer?) Aiden will most probably have to have a kidney transplant. The damage to his kidneys is to great from this ongoing HUS. The doctors say that even though his labs were normal after the episode last year that there has always been a "smoldering" case of HUS in his body. After the transplant he will still have to do plasma apharesis (or whatever other kinds of treatments we find that may help) to keep his body from attacking his new kidney.
We are restarting dialysis tonight because he is a little fluid overloaded and they are putting him on apharesis in just a little bit. It takes about 3 hours ( the apharesis) so Ativan is our friend (for him, not us haha).
Of course we know and pray that God could change all of this and make our baby boy 100% healthy. But,we are prepared to fight and do whatever it takes to keep him at his healthiest and happiest no matter what. We ask for your prayers to help Aiden and us stay strong. He is beautiful and so much fun. The doctors and staff are all in love with him, of course. I have truly never received a greater joy or blessing that my little toot. He is worth any sacrifice. I only wish I could take his place.
We will update as often as we can. Thanks for the love and support.
David, Christy & Aiden
Last Monday (Nov. 17th) we brought Aiden to Dallas for his routine 6 month checkup with his nephrologist. He looked great and they drew routine lab work and told us they would see us in 6 months for a recheck. On Wednesday we got word back that his lab results (his kidney function labs BUN, Creatinine) were abnormal. We took Aiden to the local ER that night to have them redrawn, hoping it was a fluke. When they came back abnormal again we were transported to Dallas to Medical City Children's Hospital pediatrics so that he could have further testing by his pedi. nephrologist. During all of this Aiden was in GREAT spirits..happy, playing you would never know that anything was going on kidney-wise. His urine output continued to be normal. He did have the croupy-cough that refuses (even now) to go away but other than that he looked GREAT! They watched his labs Thursday and Friday and saw the BUN/Creatinine rising so decided to do surgery to put in a peritoneal dialysis catheter (like Aiden had last year) and do a kidney biopsy and place a central line all at the same time. After the surgery we were moved to the PICU because Aiden was having some arrythmias and they wanted to watch him over night. At that point his potassium levels were getting higher and they went ahead and started dialysis that night too. He did well on the dialysis for a little over 24 hours and they took him off to see how he'd do without it....
The biopsy showed that Aiden has chronic kidney injury and a rare form of HUS called atypical. It is so rare that there is not an excepted course of treatment, it is trial and error. Doctors have just been making headway in figuring out what this type of HUS does and how to treat it in the past 10 years. It is genetic and basically his immune system goes overboard when stimulated (illness vaccines) and attacks his kidney and blood cells as well as bacteria, etc. There are several different types of atypical HUS and some differences as far as treatment depending on the type. There are only a few places in the world that even know how to test for types of this and it is not an absolute given that they will be able to narrow it down at all. They test for this in Iowa, France and Italy. Aiden's blood work has been sent to Iowa and we are waiting for results but I have no idea when to expect them.
In the meantime they are performing plasma exchange (plasma apharesis) on Aiden daily. The proteins that are abnormal in Aiden and responsible for the attacks on the kidneys and blood cells basically "live" in the plasma. They take Aiden's plasma out and replace it with healthy FFP's (fresh frozen plasma). The idea is that eventually we will rid his body of the bad proteins and replace them with good ones therefore stopping the attacks on his blood cells and kidneys. They are uncertain how long it will take to get him to a normal level or how often we will have to do apharesis to keep his levels normal. It will be an ongoing thing, it's not a one time fix, but hopefully it will prevent him from having to be on chronic dialysis.
At some point in the future (a year? maybe longer?) Aiden will most probably have to have a kidney transplant. The damage to his kidneys is to great from this ongoing HUS. The doctors say that even though his labs were normal after the episode last year that there has always been a "smoldering" case of HUS in his body. After the transplant he will still have to do plasma apharesis (or whatever other kinds of treatments we find that may help) to keep his body from attacking his new kidney.
We are restarting dialysis tonight because he is a little fluid overloaded and they are putting him on apharesis in just a little bit. It takes about 3 hours ( the apharesis) so Ativan is our friend (for him, not us haha).
Of course we know and pray that God could change all of this and make our baby boy 100% healthy. But,we are prepared to fight and do whatever it takes to keep him at his healthiest and happiest no matter what. We ask for your prayers to help Aiden and us stay strong. He is beautiful and so much fun. The doctors and staff are all in love with him, of course. I have truly never received a greater joy or blessing that my little toot. He is worth any sacrifice. I only wish I could take his place.
We will update as often as we can. Thanks for the love and support.
David, Christy & Aiden
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