Spokelet and Cycle for Life

This is a great site and a great cause. It was sent to me through a friend of Kris’ and mine. It’s jewelry for your bike but a portion of the money goes to the children’s cancer foundation – Cycle for Life.

The founder and his 6 year old son started Spokelet (which you can read about on their site). His son passed away last year of Neuroblastoma cancer after battling it for 2 ½ years.

You can check out the site here: SPOKELET

Cycle for Life

Paraneoplastic Cerebellar Syndrome

Paraneoplastic cerebellar syndrome (PCD) is a rare disorder caused by the secondary effects of cancer and is believed to be immune mediated. High titers in the patient's serum and cerebrospinal fluid (CSF) of autoantibodies directed against both neurons and tumor have been detected in some forms of this syndrome. These autoantibodies are considered the result of an immunologic response to tumor and may cross-react with cells of the nervous system, causing neuronal damage. Specific forms of this syndrome often are associated with specific antineuronal antibodies and tumors. The onset of neurologic symptoms and detection of these antibodies often precede diagnosis of the tumor; therefore, detection of these antibodies greatly assists the diagnosis of this syndrome and prompts investigations for the underlying tumor. Most paraneoplastic syndromes are rare, affecting less than 1% of persons with cancer.

Causes and symptoms

Most or all paraneoplastic syndromes are activated by the body's immune system. In response to a tumor, the immune system produces an antigen that is normally expressed exclusively in the nervous system. The tumor antigen is identical to the normal antigen, but for unknown reasons the immune system identifies it as foreign and mounts an immune response.
In general (although not always), PS develops in an acute or subacute fashion, over days or weeks. The nervous system disability is usually severe.

Treatment team

Due to the many manifestations of paraneoplastic syndromes, PS should be evaluated clinically by a coordinated team of doctors, including medical oncologists, surgeons, radiation oncologists, endocrinologists, hematologists, neurologists, and dermatologists.

Yes - Haven has an entire team! They're awesome!

Treatment

Because PS are considered to be immune-mediated disorders, two treatment approaches have been used: removal of the source of the antigen by treatment of the underlying tumor, and suppression of the immune response. Physicians often also prescribe a combination of either plasma exchange or intravenous immune globulin and immunosuppressive agents such as corticosteroids, cyclophosphamide, or tacrolimus. For most paraneoplastic syndromes, immunotherapy is not effective. Disorders involving the central nervous system, such as encephalomyelitis associated with cancer or paraneoplastic cerebellar degeneration, usually respond poorly to treatment, although they may stabilize when the underlying tumor is treated.
Disorders such as CD are usually associated with neuronal damage, and because they evolve subacutely and treatment is often delayed, neurons die, making recovery much more difficult.

Special concerns

It is important that caregivers for those with paraneo-plastic syndromes receive adequate support. The disorder typically emerges suddenly and without warning. The neurological manifestations of PS are complex and often require 24-hour patient care. Many caregivers will require quick access to information on caring for a disabled person. This includes information on social security benefits, insurance coverage, handicapped license plates, evaluations for physical therapy; handheld showerheads, and home healthcare and visiting nurses; and social workers and other support services.

Opsoclonus Myoclonus

As we dive in deeper and deeper in to Haven's world we are learning more and more about her conditions. I wanted to send all of you the best info I can. We are now working on the possibility that Haven could have Opsoclonus Myoclonus. Her condition is so indepth and out of the realm of just cancer (which is bad enough) that her doctors are researching constantly to find a way to treat her. One of their worries is that if they can't find a treatment the immune system could actually start attacking her other brain functions - as it has attack her sleeping, eating, bathroom, and weight functions. Now come the medical terms as I can't describe it that well:

This is a rare neurological disorder of unknown causes which appears to be the result of an autoimmune process involving the nervous system. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year. It affects 2 to 3% of children with neuroblastoma.

Our doctors have only been able to find 10 cases in the world and one in the USA (Chicago)

Tumors in children who develop OMA tend to be more mature, showing favorable histology and absence of n-myc oncogene amplification than similar tumors in children without symptoms of OMA (Cooper et al., 2003). Involvement of local lymph nodes is common, but these children rarely have distant metastases and their prognosis, in terms of direct morbidity and mortality effects from the tumor, is excellent (Gesundheit et al., 2004). The three-year survival rate for children with non-metastatic neuroblastoma.

Haven had a more mature and favorable histology from her Neuroblastoma tumor - as to lead them to this possiblity.

One study came to the conclusion that: Patients with OMA and neuroblastoma have excellent survival but a high risk of neurologic sequelae. Favourable disease stage correlates with a higher risk for development of neurologic sequelae. The role of anti-neuronal antibodies in late sequelae of OMS needs further clarification.

Treatment

There is no known definitive cure for OMS. However, several drugs have proven to be effective in its treatment.

Some of medication used to treat the symptoms are:

ACTH has shown improvements in symptoms but can result in an incomplete recovery with residual deficits.

Corticosteroids (such as prednisone or methylprednisolone) used at high dosages (500 mg - 2 g per day intravenously for a course of 3 to 5 days) can accelerate regression of symptoms. Subsequent very gradual tapering with pills generally follows. Most patients require high doses for months to years before tapering.

Intravenous Immunoglobulins (IVIg) are often used with varying results.

Several other immunosuppressive drugs, such as cyclophosphamide and azathioprine, may be helpful in some cases.

Chemotherapy for neuroblastoma may be effective, although data is contradictory and unconvincing at this point in time.

Rituximab has been used with encouraging results.

Other medications are used to treat symptoms without influencing the nature of the disease (symptomatic treatment):

Trazodone can be useful against irritability and sleep problems

Additional treatment options include plasmapheresis ("washing the blood", showing similarities to dialysis) for severe, steroid-unresponsive relapses.

Haven's doctors are giving her one more week to hopefully start fixing itself and then to decide how to treat but we have not noticed too many changes and it's been about a month.

Update on Haven

We finally saw the ophthalmologist on Friday. To their surprise Haven has 20/20 in both eyes. They expected the left eye to be bad. They even tested her twice. So since vision is great. We are doing an eye patch for 2 hours a day. She is doing well with that.

A few things we have noticed over the past 3 weeks since surgery:

Haven now goes to sleep between 9 and 9:30pm
She doesn't yell when she talks
Her eating has calmed somewhat but still talks of food all the time
Personality has had the biggest change - she has been so loving and sweet and her voice is a sweeter tone.

Next appointment is an MRI on March 3rd and Oncology on March 4th

A little funny

Here are some cute funny pictures of Haven with Grandma Virginia. Grandma bought some clothes for Tovey. Tovey is her brown dog. He goes everywhere with her. All other bears have now been dressed as well. Grandma bought diapers, wipes, bottles, plates, clothing for all her animals. She loves them.

Update from Friday the 15th

Well as usual we know these appointments take 90% of your day. We spent about 6 hours at JH waiting and talking and waiting and talking some more with Oncology. Her doctors went through all of her test results and went over a few other items with us. Leaving us happy, confused, and then concerned.

First off - her tumor was about 90% benign...great news. She was not found to have any cancer of the bones and everything was looking fine on the MRI's she had taken. With the entire tumor being removed she has been placed in a Stage 1 and everything is favorable. The only unfavorable element is her age. We received somewhat of a schedule for the next year and what should be expected from the cancer side of things.

Haven will have an MRI of brain and entire body every 3 months. First one being March 3rd.
Then every month she will have blood drawn for testing. This is all for the first year. After that it will either go to quarterly or every 6 months depending on condition. We will not need to do chemo for the cancer at this time.

YEAH...great news. But with great news comes bad news.

As her doctors (hours now) kept explaining and we kept feeling more and more confused. If Haven only had the tumor and the cancer we'd be looking great and pretty much in the clear. But Haven does not only have this. She has Hypothalamic Dysfunction and Paraneoplastic Syndrome. What does this mean? After 6 hours I can give a brief definition to the best of my understanding.

When Haven's tumor developed around age 2 her immune system kicked in to try to kill the tumor. However, not being a normal tumor, her tumor kinda morphed itself into a Hypothalamus. Meaning - it took on the same characteristics and hormones of her Hypothalamus. In turn this confused the immune system and it was unsure of what to attack. In the confusion, the immune system began attacking her actual Hypothalamus of the brain causing functions that most of us do not think about to stop working or change and did not go after the actual tumor. Notable changes - personality, bodily functions, vision, appetite control. Her tumor was actually giving her the hormones and not the brain. So as it stands right now her immune system is still attacking the Hypo.

Our doctors at JH have not had a case of this ever, and have made it clear to us that there are less than 1% of children in the world - meaning approximately 5 - 1o kids in the entire world that have had this. Not thousands but only 5 to 10. Haven now being one of them. Over the past few months our doctors have been working hard to find anyone that has had a child come in with similar symptoms as Haven....and recently they found a doctor with a similar case. He's in Chicago - had a child with the same disease - and found a way to treat it. Only this case was further along than Haven due to not knowing what it was. Our doctors are working on a way to make sure Haven does not get worse.

Another item they are working on is part of PNS - called - Opsoclonus-Myoclonus Syndrome. I have added a link to this on the side. I have not had time yet to fully research it - but her doctors are working on this as a possible item.

Treatment discussion: We're going to give Haven approximately one month to see if her brain can counteract the immune system and fix itself sortof. We want to see what changes are made to her by just removing the tumor. Already in 2 weeks we have seen major changes in her moods, emotions, and other areas. We're taking notes of anything we may notice - as we have noticed things that most people wouldn't. If her brain is unable to start fixing itself we have a few options that the doctors are looking in to with the doctor from Chicago.

1. Chemo - they would do chemo on this part of the brain to try to correct it.
2. Immune suppresant - pretty much shut down her immune system until everything in the brain starts to fight back.

Well right now #1 doesn't sound half as bad as #2 to us. But we are currently in a standstill. We could tell her doctors don't know what to do and are pretty much in the waiting game with us. We're very excited we are not starting chemo right now - yet I'm sad I went ahead and cut her hair off. I really wanted to wait until age 5.

We have another full day meeting with the doctors on March 4th - after her MRI - and will be able to give more updates as to Miss Haven.

Love to all

Hubby needs a break

So last Thursday - V Day - I felt as though my hubby needed a break from the crazy week of being with Haven on his own 80% of each day and wanted to send him away to relax. His mom sent me a text that she and Simon would be heading to Michigan for Grandma's 86th birthday! HAPPY BIRTHDAY GRANDMA! My brain went crazy - he hasn't gone away for an extended amount of time since December....he needs to go! He needs the break and I needed one on one time with my baby girl. So I sent him off to Michigan for the weekend and in usual fashion. I have this insanity thing that happens to me everytime he takes a vacation or I make him go....I change something. Now usually it's just my hair or I go shopping a lot....this time....I painted the house!

My bathroom was this aztec pink color (I picked but soon hated) and so I changed that to a tan brown. ABSOLUTELY LOVE IT.

My office was pure white...but then I started falling in love with this art I found on the internet and bought some of it. I have had it for about a month and have wanted to paint my room purple. WHY? Temporary insanity. It has turned in to a bigger project than anticipated but thought I'd share some pictures.

Haven also has been begging for HOT PINK in her room - currently her walls are light pink. So I bought her some hot pink to decorate her room. Those are soon to come. Enjoy!

My baby angel

Video from tonight

From Haven

Happy Valentine's Day