Courage for Kennedy – Journal

December 23, 2005

Category: Treatment – Melenie – 12:53 am

Okay, it amazes me how things can change so quickly. We just never seem to know what is going on until it is in our faces.

Her liver failure now has a name, Veno-Occlusive Disease (VOD), and to add to the confusion, she also is suffering from Disemminated Intravascular Coagulopathy (DIC). What does this mean? Well, let’s start with the easier one to describe: DIC occurs when your body becomes confused, either by infection or in Kennedy’s case medication, and devours/stops making blood clotting neccesities (platelets, plasma, vitamin K, etc.) and this is caused by the VOD. Kennedy has no ability to produce her own clotting factors and her liver is using up all the product we are putting in. So what is VOD? According to the information we received and what we can understand from the doctors, it is when there is damage to the blood vessels (which can be caused by chemotherapy) and specifically in the case of VOD, when the damage occurs “to the cells that line the walls of small veins in the liver” and blood clots form “causing swelling of the walls and vein blockage.” Essentially, the veins became damaged, causing her body to send clotting agents to the liver and keeping them from going anywhere else, her liver becomes full of blood clots and no longer processes the chemicals in the body, such as enzymes and ammonia, and backing up the liver. Her blood is no longer able to clear itself of toxins and fluid because it can not access the liver, and this blood and fluids are backing up all over her body causing swelling. Untreated, VOD eventually causes major organ failure and death. So, how are we treating this? Very carefully. Because of Kennedy’s inability to clot blood through out her body (everything is being sent to the liver that we give her and nothing new is being made) we can not just “thin her blood with Heparin” and move on. This overloading of clots has caused full liver failure and thinning them is only part of the solution. Right now, we are flooding her body with blood products to compensate (4 units of platelets, 3 units of plasma, and 2 units of packed red cells in the last 24 hours) and using meds to clear the fluid and ammonia from her system. However, they only do so much. Kennedy’s organs are under tremendous stress and pressure from the build up of fluid so it was necessary to drain off as much fluid as we could to make room and hopefully force the fluid in her chest cavity (lungs and the area between her lungs and ribcage) to move down. This afternoon they drained off 1 liter of fluid and blood from her abdomen with a catheter. Of course, because of the condition of her liver, it will return and need to be drained again everyday or every other day as needed. The good news is Kennedy is not on a respirator at this time although her chances are greater than 50% that she will eventually need to be. She is getting respiratory therapy every two hours and oxygen and we want her to breathe on her own as long as she can without causing too much stress on her heart and lungs. They are literally treating her for the moment and making decisions about her care hourly. Kennedy is considered in critical but stable condition. How can we recover her liver? Kennedy will take a very experimental drug called Defibrotide which is currently manufactured in Italy. This is her best and maybe only chance at recovery. Because it is not currently approved for use in the US, it makes it a little more difficult to come by and now it is a matter of contacting the right people and jumping through the right hoops. Defibrotide will attack and break up only the clots in her liver causing the back up to be stopped and will hopefully allow her liver to begin processing blood products and recover. That is the plan and they have had very good results when it has been used. So the drugs we are giving her now for the VOD are only trying to do what her liver is unable to as we wait for the new drug that can hopefully give her liver the opportunity to function again. She is also beginning to turn “yellow” as billirubin builds up in her system. The conclusion is that she is getting supportive care (treating the symptoms) and soon they will hopefully be curing the problem. They continue to remind us that VOD can be fatal and that while many kids do well-Kennedy is Kennedy.

Some things we did find out. They only see VOD at Emanuel about one case every two years and Dr. Olson can’t remember the last time she saw it occur in a patient that did not have a bone marrow transplant. In her research today, she only found one patient who had DIC and liver problems, but it did not develop in to VOD. This makes our girl one in a million for sure. COG (Children’s Oncology Group), the organization that oversees Kennedy’s and many other oncology research protocols, and it’s doctors all over the US and Canada are reviewing her case and giving input in to how they think it could be treated or prevented. I reminded Dr. Olson that Kennedy has never done anything by the book since she was diagnosed and why should this be any different? We still don’t know how this will affect her chemo-she is working with COG and specifically the head of Kennedy’s particular research (AALL0232-arm DH) to make those decisions because there are drugs she will no longer be able to use. Scary for both her current condition and for treating her leukemia (please remember that Kennedy is high risk for relapse and was a slow responder to chemo).

They are also testing Kennedy for a genetic disorder that causes muscle weakness-after all, let’s just add fuel to the fire, huh?

On top of this wonderful crap sandwich-Kennedy still does NOT have medical coverage for her cancer treatment. She really is our million dollar baby!!! And worth every penny, I might add! Oh, and did I mention it was Christmas??? Merry, merry Christmas!

How long will we be here? Very good question-no one can really tell us for sure. Best case scenario, Kennedy can be transferred to the “peds floor” Sunday (although waiting on the new drug makes this very unlikely) and we will spend a few weeks there. Happy Christmas in the Penthouse suite at Hotel Emanuel. They also reminded us that she will get worse before she gets better. Well now, that is encouraging. You mean it can get worse????

How are we holding up? Again, a very thoughtful question. We get through each moment and each moment only.

How can you help? Please send food-really! We have had our fill of hospital food over the last 7 months and would gladly eat your leftovers. No, not the ones the dog won’t eat-you know those which you can’t be sure when you had them or what they are but if you think about it, and truly want to bless us, please send any extras you have. It costs a fortune to eat here and it’s only good the first few times through the menu (it does repeat remember)-after all, people rarely eat here more than a few days. I mean the grill specials are the same every week we’ve been here (7 months, remember????). Home cooked is best since take out has also been exhausted (pizza and burgers). I’m not picky! Not as bad as Keith and the kids at least. So, prayers and food. Lots of hope and faith also, and food. I actually dream about tomato soup and cheese sandwiches.


And keep the encouraging messages in the Guestbook and food coming. We actually have our own fridge right now in the PICU.

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