Refresher Course

Now, you too can be on your way to becoming an oncologist! (Or, at least sound like one.) Impress people at the dentist's office, Jazzercise, and fondue parties with your new vocabulary!

Here's a handy reference guide for those of you who want to know what's going on, but don't want to go back and start from the beginning.



The Treatment Plan we received from Duke:

"Your child has been diagnosed with Wilms tumor. Wilms tumor is a type of cancer that occurs in the cells of the kidney. Your child's disease is considered higher risk because there are tumors in both kidneys (bilateral Wilms tumor).

"The most common way to treat bilateral Wilms tumor is to give two common anti-cancer drugs (chemotherapy with Vincristine and Dactinomycin) to shrink the tumors and then to do surgery to remove the tumor tissue. In some cases, an entire kidney and part of the other kidney are removed. Sometimes radiation therapy (treatment with high energy X-rays) is also done.

"There is some recent evidence that giving one extra drug (total of 3 drugs) may help get rid of the tumor cells while saving the largest amount of normal kidney tissue. This would not be considered 'standard' therapy."


The drugs:

1. Vincristine Likely side effects include hair loss, reversible neuropathy, and constipation.
2. Dactinomycin Likely side effects include nausea, vomiting, hair loss, and a drop in white blood cells, red blood cells, and platelets.
3. Doxorubicin Likely side effects include nausea, vomiting, hair loss, a drop in white blood cells, red blood cells, and platelets, and damage to the heart muscle.
   

(I will spare you the "less likely" and "rare but serious" side effects. Trust me on this one.)


The port:

Eve's port is a long, soft tube (central venous catheter) that was inserted into a large vein that leads into the heart. It is completely implanted under the skin. It is used to take blood, give drugs and IV fluids, and transfuse blood. We put Emla cream over the bump in Eve's chest where the port is to numb the skin before the port is accessed. The port is a blessing and a half, and then some.


Bone Marrow Suppression:

The bone marrow is where white blood cells, red blood cells, and platelets are made before they are released into the bloodstream. Chemotherapy works by blocking cell growth which includes both cancerous and normal cells. This causes low blood counts.

1. White blood cells (WBC) fight infection.
2. Red blood cells (RBC) carry oxygen to the body's tissue.
3. Platelets (Plt.) prevent bleeding.

Eve must have an Absolute Neutrophil Count, or ANC, of 750 or greater to get Dactinomycin and Doxorubicin. The ANC is an estimate of her body's ability to fight infection. If it is under 500, she is considered neutropenic, which means she is at a higher risk for infection. The ANC is calculated using a sub-type of white blood cells called neutrophils, which are most important in fighting dangerous bacterial infections, and bands, which are immature neutrophils.

For anyone who likes to geek out to this type of thing, the equation is:

WBC x (%Neutrophils +%Bands) = ANC

Although Matt likes stuff like this, I am happy the doctors at Duke always do the math for me on chemo days. On the days I have to take Eve into her pediatrician's office for those in-between chemo blood checks, I call Matt on my way home and rattle off some numbers and let him compute to his heart's content. He and Texas Instruments go waaaaay back...


Our home pharmacy:

1. Septra, twice daily, three days a week, to prevent pneumocystis pneumonia.
2. Miralax, daily, to prevent constipation.
3. Lisinopril, daily, to regulate blood pressure.
4. Zofran, thrice daily, for 48 hours following Doxorubicin and Dactinomycin administration to prevent nausea.
5. Reglan, to prevent nausea if Zofran doesn't work.
6. Benadryl, to be given 30 minutes before Reglan.
7. Emla, to numb port site 30 minutes before access.
   
8. Flintstones Vitamins, because it can't hurt, right?

Scans and tests:

1. Complete Blood Count (CBC) gives us the number of WBC, RBC, and Plt. This test is done before chemo and a few days after chemo.
   
2. Differential indicates the percentage of each sub-type of WBC. This is done with the CBC.
   
3. Ultrasound (U/S) uses sound waves to detect changes or abnormalities. Eve has had two of these; the first time showed the presence of tumors and the second time showed how much they were shrinking from the chemotherapy.
4. X-ray is electromagnetic radiation. Eve had an x-ray done after her port surgery to make sure it was placed correctly.
   
5. CT Scan is an x-ray test with a special machine that rotates around the patient, giving cross-section pictures of the body. For Eve, this provides better imaging of the kidneys and tumors than an MRI. She is sedated for these scans so she will be still.
   
6. Magnetic Resonance Imaging (MRI) uses magnetic waves to see inside the body. For Eve, this gives better images of the blood vessels inside of the kidneys which is important for the surgeon. She is sedated for these scans so she will be still.
   
7. Echocardiogram (Echo) is an ultrasound of the heart. Eve has had two echos- once before the start of chemo and another at week 7 to make sure her heart is okay from the Doxorubicin.
   
8. Electrocardiogram (EKG) studies electric signals of the heart. Eve has had one EKG prior to the start of chemo.

(Did you make it this far?)


After the surgery:

We wait for the pathology report to come back from the tumors. The doctors need to see how the tumors responded to chemotherapy in order to decide what the therapy post-surgery will be. There will definitely be more chemo and perhaps some radiation.




I hope you got all that. There will be a test in the morning, folks.

The C Word

First things first--- Eve had a wonderful weekend. Absolutely nothing to complain about. She even let me give her medicine today! We look at her and think, this child has cancer???

If Matt and I weren't aware of what the word oncology meant, we wouldn't have known Eve had cancer until two days after we were admitted to the hospital. No one spoke the C word. No one. There was lots of talk of chemotherapy, tumors, the Children's Oncology Group...but it wasn't until that Sunday evening that one of the doctors used the word "cancer." Not that we were in denial of what was going on, but hearing the word for the first time kind of knocks the wind out of you.

Now two weeks into this, we can make tacky jokes around our house. "Give her some chocolate milk...she has CANCER!" "Cancer trumps swine flu!" "She has cancer; don't make her sit in time out. Daniel will get over her kicking him in the head."

Natalie and Daniel are now officially card-carrying members of SuperSibs! It's a cool program for siblings of cancer patients. They receive things in the mail throughout the year. Their first packages came with coloring books, tattoos, bracelets (think Lance Armstrong), and membership cards with their names on them. The best thing in the packages were the "How I'm Feeling Today" magnets. The kids can put a special magnet frame over the face that best describes how they are feeling. Natalie likes the face with the tongue sticking out, so she decides she is also feeling "overwhelmed." Daniel says he doesn't feel anything, which I take to mean he is numb. Hopefully it is comfortable.

Thursday, October 29, 2009

I can't believe just five days ago I had never even heard the word "Wilms" and now I am on my way to an honorary doctorate in the subject.

I misspoke yesterday when I said Eve had an EKG...she had an echocardiogram. This morning she got an EKG, which proved her heart is working very well.

Dermatologists came in to check on a curious rash that turned out to be something called lichen striatus. It should go away on its own in a year or so. There goes my theory about this strange, linear rash being related to anything that is going on right now. (I guess not everything HAS to be related to cancer.)

Matt and I both had our blood drawn so the genetics team can compare our blood to Eve's (see first update). There was a miscommunication about my birth year and Matt got some funny looks from his phlebotomist as she thought I was born in 1991. I guess cradle robbers freak out people in the pediatric wing.

We spoke with a clinical social worker who put into words some of the feelings we have been having. It's hard to hear, but helpful at the same time. I am not a cryer, but this week has been emotionally exhausting. I don't think I have felt this drained before.

The nurse from supportive care came up to educate us about all the nitty-gritty stuff. We talked about medicines. We talked about fevers. We talked about who to call at 3 a.m. There was a ton of information. She sent Matt to the pharmacy to pick up the seven prescriptions we will need here at home. The bag he came back with looked like it contained a Bojangles tailgate special inside. There are three anti-nausea medications, one baby laxative, one blood pressure medication, one antibiotic (to prevent pneumonia), and a numbing cream to put on her port before it is accessed for chemotherapy.

Eve got her first round of chemo this evening. She did great...stayed still for the most part. One of the medications (Doxorubicin) is red in color, so 30 minutes after the chemo, her diaper was red. Thankfully, we were told to expect this, or else I would have frantically been hitting the nurse call button. We are supposed to wear gloves when we change her from now on since the drugs are secreted in the urine.

The last order of business at the hospital was her H1N1 vaccine. This poor child has been covered head to toe in band aids, but she takes them like a champ!

The nurse removed the needle from the port and we set off for home, only to find I-40 went from 4 lanes to 1. After a seemingly long journey back, Eve ran inside and put on Natalie's ballet tutu and danced around the house. We finally put her down a little while ago in our room and hope that she can make it through the night without too much nausea.

1 down, 5 to go.

Matt and I have been overwhelmed with the support we have been receiving. With flu season underway, this is a particularly hard time to be immuno-compromised. We will miss visiting with people but know that it is in Eve's best interest to be extra-vigilant and keep her healthy while she goes through this treatment.

Thank you for keeping our family in your thoughts. We are so happy to be back home tonight and can't wait to wake up in the morning to be surrounded by all of our children! (It would be nice if they slept in a bit, though.)

Tuesday, October 27, 2009

Today was another eventful day for Eve. She got an EKG to get a baseline of her heart, so we can compare what the Doxorubicin might do to it down the road. She was very cooperative. Her heart looks good, although there is a small hole in the right ventricle that allows blood to flow into the left. A cardiologist will come and speak with us tomorrow, although one of Eve's doctors said there is no need for concern because of the direction of the flow. (If the flow were reversed, there would be a murmur.) He said he isn't worried about it because it is not uncommon and we would never have known if she hadn't had the EKG.

Eve also had her surgery today to put in the port. I am truly shocked by how quickly kids recover. She was bouncing around the room shortly after she woke up! The port will remain accessed while we are in the hospital so Eve's blood can be taken for labs. She does not appear to be in any pain.

Eve was visited by opthamologists today who dilated her eyes and studied them per request of the genetics team. Her eyes are perfectly fine, although her pupils were so big that she looked like she jumped out of an anime cartoon.

We spoke with a nurse from Support Services who began to talk to us about all the expected side-effects of the drugs Eve will receive. We have quite a lot of reading to do in the book that she gave us, but I'm sure we will be experts in a few weeks. I certainly know a lot more than I did four days ago.

Lastly, we signed the consent forms for the chemotherapy. Eve will start tomorrow while her port is still accessed. Our plan is to get her into Friday's clinic. Tomorrow's chemo will be inpatient, but the remaining treatments will be outpatient at the clinic.

As it has been explained to us, she will arrive and have her blood taken to test her white and red blood cell and platelet counts. If they are high enough to continue, she will then be given the drugs, which are administered fairly quickly (two of them take 1 minute and the third will take 15). The biggest part of the visit will be waiting for the lab results, as the lab usually gets backed up. If we come during peak hours (10-10:30 am) we can expect to be at the clinic until 3 or 4 pm. Our goal is to arrive early so we can get her labs back and start chemo as soon as possible.

Eve is apparently well-known throughout the country's oncology community now, and her doctors here are fighting over who will get to treat her. She hasn't let this situation get her down and is still as sweet as ever.

We will be discharged either tomorrow or Thursday, depending on how chemotherapy goes tomorrow. Tonight is the last night the tumors have left to grow!

Monday, October 26, 2009, part 2

We spoke with the oncologists this evening and finally got some answers as to what course of action they recommend taking with Eve.

The MRI revealed some uncertainty as to what the spots on her kidneys really are. The doctors still believe that the right kidney has a large Wilms tumor in the upper portion, but the lesions in the lower part may be nephroblastomatoses that should, in theory, rapidly respond to chemotherapy. The left kidney may indeed have small Wilms tumors. We will not know for certain what these spots are until they are removed and sent to pathology.

The kidney is in a shell, which means the tumors are contained. The doctors do not want to take a biopsy because there is a chance of spilling contaminated cells into the abdomen, which would raise the stage of the cancer. They recommend we treat with an agressive therapy plan before removing the tumors.

This plan was developed by a Wilms expert in D.C. who runs a clinical trial in which the patient is given 3 (rather than the standard 2) drugs during therapy. Evidence suggests that the extra drug in combination with the standard two may help get rid of the tumor cells while saving the largest amount of normal kidney tissue. He weighed in on Eve's case and recommended we act as if the Wilms is bilateral, which is to treat aggresively. In the trial, 13 out of 14 children showed a favorable response to the treatment.

The treatment would consist of two consecutive 3-week cycles of chemotherapy. Eve will receive Vincristine (weeks 1-6), Dactinomycin (weeks 1 & 4), and Doxorubicin (weeks 1 & 4). There are certainly side effects, some very serious, but the doctors feel that the risks are worth taking. One of the more serious side effects is damage to both the heart and liver. These will be monitored throughout therapy.

After the chemotherapy is complete, Eve will have an MRI to evaluate any changes. If the chemotherapy has the desired effect, she will go on to have surgery to remove the affected parts of the kidneys. After the surgery, the tumors will be sent to pathology to determine what exactly they are, and then the next form of therapy (chemotherapy and/or radiation) will be decided upon then.

As of tonight, the doctors are going to try to get her into the O.R. tomorrow to have the port put in. We hope to have her first treatment this Friday.

Thank you all again for your support and well wishes. We are extremely blessed to have so many wonderful people in our lives. Even though we haven't been able to respond to messages, we have read each one and your thoughtfulness is truly appreciated.

Monday, October 26, 2009

We had a change of plans...no surgery today. Since there are lesions on Eve's left kidney which are considered to be pre-disposed to cancer, the oncologists want to be sure that they have explored all options before removing the right kidney. There is most likely a large Wilms tumor in the upper part of her right kidney, but the lower part is questionable as to what it is, whether it be Wilms or nephroblastomatosis (not yet cancerous lesions that may or may not turn into Wilms).

Eve was sedated and given an MRI a few hours ago. The oncologists here at Duke have been assessing her case with oncologists all over the country, and everyone is weighing in before they proceed with anything. From what we understand, there are two basic options that they are debating- either remove the kidney and then give chemotherapy, or start with chemotherapy to try to shrink any questionable lesions, hoping to salvage part of the right kidney instead of removing the whole thing. If the left kidney were clear of lesions, they would simply go forward with removing the right kidney, but knowing that one day she might have to have part of the left removed, everyone wants to be sure they can't save any of the right.

A geneticist came in a few minutes ago to check out Eve and see if there were any obvious signs of some syndromes that some people with Wilms have. While she didn't see anything in her examination, Matt, Eve, and I will be getting genetic testing done on Thursday to find out if there is something abnormal with her genetic make-up. It takes several weeks before getting results, so we won't find out anything before then. Of course, this very well could be just an isolated case of Wilms unrelated to any syndrome, but they want to be sure. One of the doctors theorized that the cells in Eve's kidneys were abnormal in utero.

Again, we appreciate all your support and will update more when we find out the results of the MRI.

Saturday, October 24, 2009

Some of you might have already heard we are in the hospital with Eve. This week at her two-year-old checkup, her pediatrician found a lump in her abdomen and sent us to get an ultrasound. We got the U/S yesterday morning and there appeared to be tumors on her kidneys. We were sent to the Duke Pediatric Oncology unit yesterday afternoon and today Eve got a CT scan which confirmed she has a Wilms tumor on her right kidney, as well as some questionable cells in her left.

As of now, the plan is to remove the right kidney on Monday, as most of the kidney has turned into tumor. We will most likely just monitor her left kidney with regular CT scans, as there does not appear to be a tumor there, but we will need to wait for the head Radiologist to look at her scan in the morning to make sure. She will also be getting a port put in her chest when she is under anesthesia on Monday so when she starts chemotherapy, they won't need to put in an IV each time.

Her blood pressure is very high, so she is on lisinoprol to bring it down. Apparently this is related to the tumor on her kidney and should be resolved once it is removed.

Right now, she is comfortable since she can finally eat. Eve is pretty tired and a little cranky from all the poking and prodding and starving (she went for a day and a half with no food or drink), and lack of sleep (she didn't get a nap yesterday and stayed up until 3 am!...didn't sleep in, either). She isn't attached to an IV pole right now since she is eating and drinking, so that is also making things more comfortable for her.

I just wanted to fill everyone in on what is going on with us. We are grateful for all of the support our friends have given us and ask that you keep Eve in your prayers. I will update more when we have more information. I refuse to google any of the terms we have heard. I prefer to get all of the information from our wonderful doctors!