The Facts, my Lovlies

Neuroblastoma is a form of cancer, found primarily in children, that attacks the developing nerve cells. In Neuroblastoma, cancerous tumors form from cells called neural crest cells. These cells are similar to those found in the developing nervous systems of an embryo or a fetus.

The human body has two glands known as adrenal glands, one above each kidney. These glands are triangular in shape and are responsible for secreting hormones that control the body’s involuntary responses such as blood pressure and heart rate. Neuroblastoma can start in the bundle of nerves in the adrenals, as well as in the nerve groups in other parts of the body responsible for the body’s involuntary responses.

There are two types of tumors that can be found in these nerve tissues. The first is GanglioNeuroblastoma, which consists of the cancerous nerve tissue mentioned above. These are the tumors of Neuroblastoma. The second is a tumor that resembles Neuroblastoma, but consists of mature nerve cells. These tumors are referred to as Ganglioneuromas and are not cancer.

Neuroblastoma

Stages:

The accepted method for determining the severity of a case of Neuroblastoma is known as the International Neuroblastoma Staging System, or the INSS. The disease can be ranked on the INSS scale of 1 to 4, with 1 being the earliest stage of the disease and 4 being the most advanced. The following is a description of the symptoms and progression of the disease as seen in each stage:

When doctors look for the location of lymph nodes on different sides of the body, they are referring to the midline, which is shown by the thick black line running down the middle of the body in the diagram below. If the disease is found on both sides (right and left) of the black line, it is said to be on “both sides” of the body.

 

  • Stage 1: There is only one tumor. This tumor exists only in the area where the disease originated and can be completely removed by surgery. All lymph nodes are found to be free of the disease.
  • Stage 2A: The tumor is not easily removed, but the disease is confined to one side of the body. (Either the tan or white side, as shown in the diagram) All lymph nodes still remain free from the disease.
  • Stage 2B: The tumor has only affected one side of the body, but it may or may not be removed easily. In Stage 2B the lymph nodes on the opposite side of the body (the “contralateral” side) are not found to show signs of the disease, but those lymph nodes on the side of the body where the disease originated (the “ipsilateral” side) have been affected.

 

 

  • Stage 3: There are three ways in which a patient can demonstrate signs of Stage 3 Neuroblastoma.
    • The tumor has spread so that it can be found on both sides of the body.
    • The tumor remains on one side of the body, but lymph nodes on the other side of the body have been affected.
    • The tumor is found on both sides of the body and the lymph nodes on both sides have been affected.
  • Stage 4: This is the most advanced stage for patients over the age of 1. Here, the disease has spread to include affected lymph nodes, bone, bone marrow, and other organs.
  • Stage 4S: Stage 4S is a classification for a “special” case of Neuroblastoma. This stage only applies to infants who demonstrate a tumor on one side of the body, as seen in Stages 1 and 2, but also have affected liver, skin, and/or bone marrow.

 

Who gets it?

 

  • Neuroblastoma is almost always found in children.
  • Nearly 90% of the cases of Neuroblastoma are diagnosed before the age of 6.
  • Of all childhood cancers, Neuroblastoma accounts for approximately 7% of the cases diagnosed.
  • The Neuroblastoma Children’s Cancer Society reports that 500 to 1,000 children are diagnosed each year in the United States.
  • Slightly more cases of Neuroblastoma are seen in boys than in girls.

 

Symptoms:

The symptoms of Neuroblastoma depend on the age of the child and the origin of the disease. The majority of the symptoms are a result of the effect of the tumors on the body as opposed to the disease itself.

The general symptoms associated with Neuroblastoma include:

 

  • Diarrhea
  • Fever
  • Anemia (weakness or tiredness due to a low number of red blood cells)
  • Hypertension (high blood pressure)
  • Loss of appetite

 

These symptoms occur often in children and can resemble a cold or flu. If your child is exhibiting any of these symptoms, it does not mean that he or she has Neuroblastoma. It is best to see your doctor anytime your child is feeling ill. Only a doctor can determine if a child has Neuroblastoma.

Some symptoms depend on the location of the tumor. When a tumor exists inside the body, it can put pressure on different nerves and organs, leading to a variety of symptoms.

 

  • A tumor in the chest area can cause a child to have trouble breathing and can make swallowing difficult.
  • A tumor in the stomach area can cause a child’s belly to swell and can cause constipation or urinary problems.
  • Tumors along the spinal cord can cause a child to develop a limp or to have difficulty walking.
  • Children with Neuroblastoma can also exhibit jerky motions in their muscles (known as “myoclonus“) and rotational movements of the eyes (called “opsoclonus“).

 

How is it detected?

Many different tests can be done to determine if your child has Neuroblastoma and how far the disease has progressed. Most of these tests are relatively painless and easy for your child.

 

  • One of the best ways to test for Neuroblastoma is through a urine test. Neuroblastomas give off a substance called VMA (vanillyl mandellic acid). If VMA is found in your child’s urine, it is probable that there are Neuroblastomas present in the body. The urine test is almost 100% effective as a Neuroblastoma indicator.
  • Another type of test, known as MIBG (meta-iodo-benzyl guanidine), can also detect Neuroblastoma in the body. In this test, a substance known as MIBG is injected into the body. Neuroblastoma cells take up MIBG. After the injection, the MIBG levels are measured. If a significant amount of the substance has been taken up and the patient’s MIBG level is low, it is likely that Neuroblastoma cells are present.
  • CT (computed tomography) scans and MRI (magnetic resonance imaging) tests can be done to detect tumors resulting from Neuroblastoma. Both of these tests require that your child sit or lay still while pictures are taken by a machine. These machines allow doctors to see inside your child’s body on a very detailed level.

 

Is it curable?

The prognosis for Neuroblastoma depends greatly on the child’s age and the stage of the disease. The farther along the disease has progressed, the more difficult it can be to cure. Children under the age of 1 year tend to demonstrate a greater survival rate.

Unfortunately, diseases in very young children can be difficult to diagnose early on because the child cannot always verbalize a particular complaint.

Every child is different, and there is no universal generalization that can be made as to the future of a patient with Neuroblastoma. Your doctor will best be able to gauge the stage and prognosis of your child’s disease. The following is intended to provide an overview of some statistics from the National Cancer Institute regarding past cases of Neuroblastoma.

 

  • Children of any age with Stage 1 Neuroblastoma have a cure rate greater than 90%.
  • Long-term survival in Stage 2 patients over the age of 1 year can range from 75% to 90%, depending on the age of the child and the success of the treatments administered.
  • Patients with Stage 2B or 3 Neuroblastoma over the age of 1 year have a survival rate of approximately 50% to 70%. Those younger than 1 year of age have a cure rate of around 80%.
  • Patients with Stage 4 or 4S Neuroblastoma under the age of 1year tend to have a cure rate of 50% to 80%. Those patients over the age of 1 year have a cure rate of 10% to 40%.

The Lack of Warning Signs

A few of you have asked what the warning signs of Ronan’s cancer were. I’m thinking there are some newer readers out there, who have started reading this blog, without going to the beginning of Ro’s story. Or maybe I never explained it well. I never re read what I write and I can only imagine the jumbled mess of words that I probably display. When I write, I don’t think. I just feel and my fingers just kind of take off, without thought, letting my feelings do all the work. Thanks for sticking with me, throughout this journey of love and pain. I don’t know if I tell you all often enough, but the love that you have for my baby, for my family, for me, for my crazy writing; means the world to me. So thank you.

I am going to recap for those of you who are wondering what the signs were. For those of you who will now fear everyday that your child could be next…. because sadly I’ve learned this can happen to anyone. I will forever be pissed at the lack of knowledge I had for Childhood Cancer before all of this. Every parent should be informed and aware once they have children. If I had been aware, would we have caught this sooner? I’ll never know, because unfortunately, nobody can give me a straight answer. They don’t even know what is the cause of this awful disease. And there are too many people in the world who don’t care enough to fight and find out, because it is so rare. Or because it is too painful. Bullshit. Even if only 50 kids a year were diagnosed; which is not the case….. that is way too many to still be dealing with the nobody knows in 2011. Around 800 kids are diagnosed a year. 800 kids who survive by chance, not because there is a cure. It’s pure luck. My Ronan deserved to be lucky and I’ll never understand why he wasn’t. I would give anything to have him be the poster child for this disease, because he survived it. Not because he is dead and now I have to take his name and my pain and turn it into something positive. I have to take the death of my child, my baby, my soul mate and give everything I have to this so maybe, someday, people will start paying attention to kids who have cancer. Just maybe, I can help be a voice to save another Ronan out there; so another mom won’t have to find out what it’s like to sit in her dead child’s bedroom and sob while holding a bag full of his ashes. That is the only physical form I have left of Ronan now. His body, his eyes, his fingernails, his sweet little lips, the nape of his neck where I used to get my “sugar,” from. They are in a bag that looks like sand. All because of Childhood Cancer. All because Doctors, Science, Medicine, and Awareness has failed in 2011. Nobody wants to pay attention to Childhood Cancer. I guess it feels better for people to focus on things like Education because seeing a child with cancer, is too hard to handle. Ignorance is bliss. Best saying ever.

So, my friends. The signs. The Warning Signs. There really wasn’t any. Fun huh. I had taken the 3 boys to my parent’s in Washington State, just as I had every summer since the twins were born. We spent almost all of July there and returned to Phoenix at the beginning of August. We had the BEST time. We spent most of our time, doing simple things, like playing outside, fishing, enjoying time with my beautiful 70-year-old, youngest ever, stepfather; who is a big kid himself. He ADORES my boys and the time they have with him every summer, has always been one of my favorite things to watch in life. Ronan was eating normally, sleeping normally, running around like his crazy self. It was a few days before we were heading back to Washington when I took the boys and made them put on button up shirts, as I wanted to get a picture of them for our annual Christmas card. I spent an hour or so, snapping pictures of them, which has always been another one of my favorite things to do in life. As I was finishing up, going through the pics, I noticed Ronan’s left eye. It looked a little off in the picture that I had taken. Here it is:::

I assumed it was just some dust, allergies, or a bug bite. We had spent a ton of time outside and any of those things could have easily been the culprit. We returned to Phoenix on a Saturday, a few days after this picture was taken. Ronan’s eye still looked not quite right. Woody noticed right away. We made a doctor’s appointment for Monday morning just to check it out. The thought that it could have been Cancer, never even crossed our minds. Our lovely doctor, thought Ronan just had a cyst on the inside of his eyelid and refered us to a Children’s Opthamologist I took him that next day and ended up walking out in the middle of the appointment with her because of her rudeness and lack of concern/compassion that she had for my child and a mother who was scared shitless. I was panicking while trying to stay calm. I threw Ronan in the car, with his eyes still dilated and found another Child Opthamologist to see Ronan that next day. They had originally told me that they couldn’t see him until September. I told them that was not acceptable, that something was going on, and he needed to be seen that next day. They listened and we saw Dr. Cassidy the next day. He knew that something was wrong right away and sent us to PCH for a CT scan. Even at this point, I didn’t think Ronan had Cancer. I mean, really? That doesn’t happen to kids in this world. Especially not my Ro. I was about to learn in a very hard way; that it in fact does. More often than people realize.

So, that is it peeps. The warning signs, which really were none. But guess what??? Doctors can test for this kind of cancer with a simple urine test. YUP. It’s as easy as that. But once again, the bullshit answer to that is not enough kids are diagnosed. And I’m sure insurance doesn’t cover it. I’ll always wonder if Ronan was born with this. What if we would have caught it earlier… would he still be here? Most kids that are diagnosed with Neuroblastoma, are a Stage IV, because it is a silent killer due to the lack of symptoms. The lovely lack of symptoms, but you test for it by peeing in a cup. I am not an expert on why this isn’t done. I’m sure there are 100 different reasons. I am just a mom. Another mom, who has lost a child to cancer. But I was never given a choice as to whether I wanted my child tested for this or not. I can guarantee you, if I had been educated, and given a choice, all 3 of my kids would have been screened for this disease. Just like when they test for Down’s Syndrome when you are pregnant with a child. I was always given a choice for that and I always chose yes. I would have chosen yes, if Childhood Cancer would have been put on the plate. Would that have changed the outcome of all of this? I don’t know. But I should have been given the choice as a parent, to make that decision myself. The Medical world has a responsibility to make people aware. They are not doing it, so it’s parents like me who have to do it, until things change in a very drastic way. To them, Ronan is a statistic and nothing more. They just killed one of the most beautiful things to have ever touched this planet. And now, I have to figure out what to do with all this because I will never come close to feeling peace again in my life; unless something is done.

G’nite my lovilies. G’nite my Ro. I may be too tired to write to you tonight. I love you to the moon and back and hope you are safe.

P.S. You all know what a pink ribbon means. Did you know a gold ribbon is for Childhood Cancer? I didn’t, before all of this. Did you know that September is Childhood Cancer Awareness Month? I didn’t. I am embarrassed, ashamed, and failed as a parent because of my ignorance is bliss life that I indulged in for so many years.

P.P.S. FUCK YOU, CANCER!!!!!!!!!!!!!!!!!!

xoxo

Thank you.

 

 

 

 

Ro baby. Thank you for visiting me in my dreams last night. It was so unexpected as I was beginning to think it was never going to happen. You were at home, with us, where you belong. You were bald, but running about just as you always did. You were happy. I watched you and started to cry out to your daddy that I didn’t understand how you were so sick, and you were going to die, because you looked and acted so healthy. I knew your fate in my dream. I knew that you were going to be taken away from us, in the end.

I’m sorry. I miss you so very much. Thank you for my gift of being able to see you in my dreams. I love you and hope you are safe.

xoxo