Cyclosporin Chemo Treatment

I wanted to take a moment - since my brain can't stop tonight - and explain Haven's chemo treatment. If you look up Cyclosporin on the internet you will find the following definition:

Ciclosporin (pronounced /ˌsaɪkləˈspɔrən/), cyclosporine (USAN) or cyclosporin (former BAN), is an immunosuppressant drug widely used in post-allogeneic organ transplant to reduce the activity of the patient's immune system and so the risk of organ rejection. It has been studied in transplants of skin, heart, kidney, liver, lung, pancreas, bone marrow and small intestine. Initially isolated from a Norwegian soil sample.

So - we know Haven has not had an organ transplant - why in the world would we agree to use this?

I just want to say this chemo is NOT for Haven's Ganglioneuroblastoma Cancer. It is for ROHHAD Syndrome. But there is not treatment or cure for ROHHAD, right? Right! So here is a bit of history.

In Oct. 2007 - Haven had a 45 minute seizure (after 2 years of looking for a diagnosis). She went through rigorous testing of Cushing's Syndrome and a CT Scan for Pheochromocytoma. After 2 months of testing she was officially diagnosed with Neuroblastoma Cancer on Dec. 28, 2007.

February 2008, Haven had a 14 hour surgery to remove the tumor which was located in her abdomen against her spine. The Aorta and Vena Cava Arteries were wrapping around the tumor - as it had been there from birth but never located. Mainly because the doctors wouldn't listen and only did MRI's of the brain. But that's another story.

We waited about 3 weeks after surgery and then started Chemo for the cancer. Or so we thought!!!! For 3 weeks after surgery Haven became "normal". It was crazy the difference and then all of the sudden everything started up again!

End of March 2008, our Oncologists sat down with us for about a 6 hour meeting. I remember the conversation. They told us about this "disease" that they read about in Chicago. That is was possible that Haven had it but they didn't know. They told us about how this boy was undergoing treatment and research but the doctors didn't know what was happening until the day he stopped breathing. But we were told they didn't believe that would happen to Haven but they were going to try to keep it from happening.

CANCER - seriously, this is all we heard. After 6 hours all we knew is our daughter has cancer - who cares what else the doctors were talking about. A bunch of jibberish to us and doctor babbling. What we didn't understand was that the chemo was for ROHHAD.

RITUXIMAB - chemo used for B cell non-Hodgkins lymphoma and other autoimmune disorders. Haven took 6 doses of this chemo in about a month. Instantly, we noticed an amazing difference. Haven lost weight, she started sleeping all night, she stopped sleep walking, she was out of diapers, she was rejecting food instead of begging for it. She was happy. This chemo did make her sick usually 2 days after receiving it.

CYTOXIN - chemo which was used for the Neuroblastoma Cancer. We used this for about 6 months. No changes on this but nothing went bad. We gained weight extremely slow and she did have to go back in to diapers when we stopped the Rituximab. But we were comfortable with chemo at this point and it was "normal" to us.

PREDNISONE - steroid used for 11 months of Haven's treatment. We had to lower her sodium intake to under 100mg a day and an extreme diet. The doctors expected her to gain about 30 pounds on this but we managed to keep her at only gaining 10 pounds the entire year.

Fast forward to November 2008 - meeting with Endocrinologist. Haven is officially diagnosed with ROHHAD and we are told she could stop breathing anytime between age 5 and age 7. What? What is ROHHAD? I don't remember ever talking about ROHHAD...who said that? Well a very long meeting with Oncology and I am reminded of the conversation in March when they told us! Again - we only heard the word cancer...after that nothing else mattered.

So we tapered off the steroids and cut all chemo out for 3 months.

December 31, 2008 - Haven is approved by the medical board to start Cyclosporin.

Now here we are and why are we taking this chemo. She doesn't seem to be better and everything seems to be spiraling downhill quicker than I can type and I type pretty fast!

Our doctors believe they know what is causing ROHHAD to happen so we are testing it. So to speak. They believe the immune system is attacking the hypothalamus causing it to malfunction. Now this could only be with Haven so this is NOT a diagnosis for anyone else or a reason for anyone to run to their doctors. ROHHAD is different, but similar, for all of the children diagnosed.

Why would Haven's immune system attack her hypothalamus? Haven's Ganglioneuroblastoma Tumor was a slow growing tumor. It is believed that it took on the characteristics of her hypothalamus --- causing the immune system to get confused between the "real" hypo and the "fake" hypo. It was unsure of which area to attack and it went for the brain instead of the actual cancer.

So in their thoughts - and no one knows what causes ROHHAD and 95% of doctors probably haven't heard of ROHHAD - Haven's tumor is removed but her hypothalamus is still there. So to her immune system it thinks the "bad" tumor is still present and it is still fighting for her.

With Cyclosporin we have shut down Haven's immune system by probably about 25% just to test out the theory. IF Haven shows any signs of getting better: losing weight, sleeping better, potty control, hunger control.....they know it's working. IF Haven doesn't show anything....everything stays the same....they know it's working. IF Haven gets worse: gains weight, sleeping is worse, pottying is worse....they know it's not working.

April 1st, 2009 - this is a big day. This is the day her doctors will collect all of her data over the 3 months of testing and they will take it to the medical board to review. IF she is showing signs that immune suppressant is working they will get approval to shut her immune system down 100%. Essentially stopping it from attacking the hypothalamus and letting the hypothalamus take control back of it's functions. Then after a certain unknown amount of time they will start the immune system back up.

IF she shows signs of getting worse they know it is not the immune system and they know it is not working. At that point we will have to discuss if there is anything left to try or if we just monitor her for the inevitable.


I just wanted to make sure everyone understands - at this point we can't even worry about the cancer or if it can/will come back. Right now our main focus is on the ROHHAD. Once we figure that out we can go back to the cancer. So, I will ask everyone to pray, wish, hope (whatever you do) that immune suppresant will work and we will figure out what causes ROHHAD and what can be done to treat it.