Rituximab Update - High Dose Chemo Immediate

Haven spent half of today at the hospital having her chemo levels taken - she was at a toxic level earlier this week and today was in good shape. They met with her Oncologist for about 40 minutes and went over any questions we had. I had a few sleepless nights this week and sent a few emails to the doctors.

As I was driving home this evening her doctor called (he actually called 3 times today but I didn't answer). Remember April 1st! ...... well things change fast around here

During the 15 minute conversation her doctor informed me that he is very concerned and extremely anxious about Haven. He has reviewed all of her tests she has undergone the past couple of months and all the blood work. As noted by him (as best I can remember):

I have been working closely with Haven for 2 months and reviewing everything from the past 1 1/2 years of treatment. Haven is regressing too fast. There are new areas of concern and some of the recent damage can not be repaired. We have lost too much time and ROHHAD is progressing. We need to move to the next step. Cyclosporin is too slow for ROHHAD. I need to stop everything now before it gets worse. I need to see results and I need to see them now. Haven will begin Rituximab next Friday at 9am. We will discuss next week when you come in for her Cyclosporin levels.

Haven will begin heavy Rituximab starting March 6 and will continue on Cyclosporin at the same time. She will be on for a longer period of time now. This is one month sooner than originally scheduled. We will start her back on the Zofran - as this will help to keep her from being too nauseous.

Haven will also have an MRI on the Ganglioneuroblastoma tumor next Thursday at 9am.


As this is now our last weekend before this immediate increase in chemo, we will be taking the weekend to relax and hang out. We have crafts to make and lots of thank you cards to send out. Haven will be sick on this chemo - we know how the lower dose of it made her last year.

Thank you everyone for always sending cards and gifts to Haven. We truly appreciate everything. Haven has been working on small gifts to send to her new friends out there.

ALAINA DIODORA -- Another Scam

So I just received this email --- which is awesome how much people are trying to steal from other people using a deadly disease affecting millions of people!

I also looked up the lady's supposed name and found another BLOG that received the same email! Since I've already had money stolen from me this year by some creeps I want to make sure to share all of these crazy things! I will continue to wait for the Holy Sprite! (you'll see below)

DEAR FRIEND,

GREETING IN THE NAME OF OUR LORD JESUS CHRIST. I AM MRS.ALAINA DIODORA,A WIDOW TO LATE MIKA DIODORA. I AM 50 YEARS OLD,I AM A NEW CHRISTAIN CONVERT, SUFFERING FROM LONG TIME CANCER OF THE BREAST,FROM ALL INDICATION MY CONDITIONS IS REALLY DETERIORATING AND IT IS QUITE OBVIOUS THAT I WON'T LIVE MORE THAN SIX MONTHS,ACCORDING TO MY DOCTORS,THIS IS BECAUSE THE CANCER STAGE HAS GOTTEN TO A VERY BAD STAGE. MY LATE HUSBAND WAS KILLED DURING THE COTE'D IVORE CIVIL WAR, AND DURING THE PERIOD OF OUR MARRIAGE WE COULD'NT PRODUCE ANY CHILD.

MY LATE HUSBAND WAS VERY WEALTHY AND AFTER HIS DEATH, I INHERITED ALL HIS BUSINESS AND WEALTH.THE DOCTORS HAS ADVISED ME THAT I MAY NOT LIVE FOR MORE THAN SIX MONTHS;SO I NOW DECIDED TO DEVIDE THE PART OF THIS WEALTH, TO CONTRIBUTE TO THE DEVELOPMENT OF THE CHRIST CHURCHS AROUND THE GLOBE. I SELECTED YOU AFTER VISITING THE INTERNET AND I PRAYED OVER IT.I AM WILLING TO DONATE THE SUM OF $5,000.000.00 U.S DOLLARS,TO THE LESS PRIVILEGED.

PLEASE I WANT YOU TO NOTE THAT THIS FUND IS LYING IN AN INTERNATIONAL BANK.I HONESTLY PRAY THAT THIS MONEY,WHEN TRANSFERRED WILL BE USED FOR THE SAID PURPOSE; BECAUSE I HAVE COME TO FIND OUT THAT WEALTH ACQUISITION WITHOUT CHRIST IS VANITY.

MAY THE GRACE OF OUR LORD JESUS THE LOVE OF GOD AND THE FELLOWSHIP OF THE HOLY SPRITE BE WITH YOU AND YOUR FAMILY.

I AWAIT YOUR URGENT REPLY.
YOURS IN CHRIST.
MRS.ALAINA DIODORA.

PS: I also received one from a "guy" named MUHAMADAD ALLAUDDIN and another one named DENIS BAYI --- Total amount of money they are willing to send to me is over 20 million dollars! One has liver cancer and the other is about to be shot by his dad's enemies in a war torn country that is no longer safe to live in!

I'M GONNA BE RICH!!!!!!

Comparison photos - ROHHAD and Neuroblastoma signs

So - randomly I go through photos and remember when everything was normal in our lives. Here are some photos of Haven right before everything went crazy. These are from 2006 - I have uploaded a bunch of photos to her flickr site. You can click HERE to see them.

Haven working on bikes with her dad in the garage. This was in May 2006. She was 2 years and 4 months old.

June 2006 - Uncle Kevin's graduation.

Haven with big sister Kendall and Grandma Sing

Haven with Grandma Virginia.

June 2006 - Having fun with Kendall

July 2006 - the very next month. Gained about 25 pounds in one month.

With dad - July 2006

Haven and Kendall - December 2006 - after many multiple doctor appointments. Diagnosis - mom overfeeds child reduce calories to 1,000 per day.

December 2006

Haven's 3rd birthday - January 26 2007. Begins having major stomach pains putting us in the hospital and doctors thinking mom is insane. Doctors start treating me like I have Munchausen Syndrome. Begins to have blood in the potty and doctors diagnose that she is pushing to hard give her prunes.

3rd birthday party. Wearing size 6 at this time.

Gained approximately 35 total pounds (up and down) in 6 months.

Doctor update

I finally talked with Haven's doctor today about most of her testing. Here is what I got in the not-so-medical version. It was a lot to take in so hopefully I can remember most of it.

Cyclosporin levels : Haven was at a high toxic level today. We have been told to not give her the chemo tonight and to do a partial dose until she comes in for her next visit.

Bone Density Scan: she has a pretty significant bone loss in her hips and some slight loss in her spine. She could easily fracture her hips if she were to fall or something. Since she doesn't feel pain unless it's extreme we may not know if she does fracture it. So we're going to be watching her. Endocrine will be contacting us to start her on medication to help build up her bones.

Sleep Study: They are rated 0 - 4. Haven is a level 2 right now. Level 0 is perfect sleeping and level 4 is an emergency. She has quite a bit of snoring - but the gas exchange was not affected. So for now they will attribute it to weight instead of Hypoventilation but she must be watched. We will need to meet with Pulmonology to review and do all follow-ups. Oncology was not clear on a lot of the report. He said there was quite a bit in there this time. He did say her brain wakes up about every 15 minutes or so but her body does not. Her brain is malfunctioning between sleep stages and she never hit REM sleep.

Ultrasound: Arteries and veins are clear and there are no clots in the legs.

Still waiting on a few more things and preparing for surgery. I have been collecting as much data as I can from other moms to help our doctors.

Haven will have an ABG - Arterial Blood Gas count during surgery. I asked why other children can have their fingers pricked to be checked but Haven has to go to the artery in the wrist. Explanation: ABG is the only test that is the most accurate for blood gas results. Someone who may be stable or on a ventilator may only need to check by finger. But by finger will never get accurate readings. They need to get the most accurate reading from Haven for a starting ground.

Oncology warned me that she will be in a lot of pain from the ABG. He said (quote) "It hurts like the dickens". So she mostly likely won't want to move her arm and definitely won't want to move her wrist around for a while. But with lowered pain sensors I'm hoping she is not too bad.

Anyway, I am tired and the phone call today gave me an instant migraine. American Idol is not on so Haven is off to bed and we will have our "party" night tomorrow.

Love to all.....anything else I get I will pass it along! :)

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.

Homemade Pizza - Haven Style

Haven decided last week that she really wanted pizza. Well, Haven can't have pizza - there is just way too much sodium in pizza so she has not had any. Then she watched the cooking channel! Dad and Haven decided they would make pizza at home and skip the salt! I believe they spent 5 hours on Sunday making pizza dough and pizza.

Haven learned about yeast and how it grows and how to set it. Our crust is wheat flour, yeast, and a small pinch of brown sugar.

Adding the sauce. This is not normal tomato sauce you buy. They made the sauce themselves. Kris squished tomatoes for the juice, cut up onions, garlic cloves, sage, basil, oregano and who knows what else. Then they cooked the sauce to blend the spices and added it to our dough.

Haven is added our fat free mexican shredded cheese. This is a mix of 4 cheeses that are fat free...and really with all the spices you can't taste a difference. The only difference is texture and for her health we'll deal with texture!

Adding the toppings. Now I'm not a fan of mushrooms but she really wanted some on there. So we added it and then removed them from her slices before eating. This way she had some of the flavor but not the salt. Mushrooms are very high in sodium.

Green bell peppers - Haven loves bell peppers. She loves everything spicy. She also added purple onions and more garlic cloves! I love garlic so I always ask for more garlic.

And the final touch - grapes! Who doesn't love grapes on their pizza. We didn't have pineapples (because mom can't eat them) but it doesn't stop Haven from being creative. She thought of grapes and we said why not. It actually was very good.

This was the third pizza made. We kept refining the dough and making it just a bit better each time. This one is made with roasted red peppers, onions, same dough and sauce but we added ground beef. Yes ground beef is bad but we do the best we can. This is 96% fat free ground beef - cooked and drained. Another big hit with everyone!

PS: when I use the term WE I don't include myself. This is Haven and her dad. I have been banned from the kitchen since making oatmeal this weekend. According to Haven I can't even microwave oatmeal - all the oats were hard and the fruit was still frozen. She said it would be best if I just did laundry and let dad do the cooking.

Enjoy! :)

ROHHAD and RARE DISEASE DAY

Just wanted to remind everyone - this Saturday, February 28th is Rare Disease Day.

RARE DISEASE DAY is not only in the USA. There are events planned all around the world. Check out the web site and check out your location. UK starts on the 25th!

While I am constantly researching ROHHAD and Ganglioneuroblastoma Cancer I sometimes run across new sites that I have never seen before. Mainly because they have my daughter's name in them or they list something relating to ROHHAD. About a week ago I found Little Miss Hannah's web site. Hannah is 6 months old and is fighting Gaucher’s Disease Type 2 or 3. Since her mom shared Haven's link to bring awareness to Haven's rare disease I wanted to share with you Hannah's link.

I would like to thank some other brave women who have emailed or sent me letters about their child's stuggle with ROHHAD. Although they are worried that it may push me to lose all hope to hear their stories I am overjoyed to hear from these moms.

Although some doctors want to make this disease be our fault and accuse of us causing this disease we have to be strong in knowing we did nothing as parents to make this happen. We all have the same story from our child's birth. I am reading your letters and everything is like deja vu - I'm sure all of you feel the same when I send you Haven's story.

For 2 years we were told it was our fault and we were overfeeding our child and we were doing everything wrong. I have been angry for 3 years now and even when I tell myself I'm not going to be angry anymore I am. ROHHAD SUCKS! It's horrible....I hate it. No one understands it and it seems like you are alone. None of us have slept in years due to worry about our children. We all pretend to go on like everything is normal....but when I take my daughter shopping for clothes I am reminded it is not normal.

People stare and believe it or not, people say stuff. People come up and say stuff about how our children are fat and that we should be ashamed of ourselves as parents and how we are ruining our child's life. I'm sorry but I want to go insane on these people but out of respect for my daughter I keep it as civilized as I can. I can't promise that I'm always civilized. I know all of us as parents with ROHHAD children are going through this. AND I shouldn't have to lock my daughter in her house so she won't be stared at by ADULTS! Anyway, I hear all of you and I completely understand what all of you are going through...we just have to be there for each other.

I took Haven shopping Saturday to buy some new clothes - as she has again grown out of everything we bought only a couple of months ago. She didn't want to try anything on - she didn't even really care to look at the clothes. She told me "I don't know why we come shopping - nothing fits me". This is extremely difficult for me to hear and even more for her. I don't know what to do here. She's 5 years old and wears a size 12/14 shirt.

April 1st Haven's doctors meet with the medical board again about her ROHHAD and where to go. Until then I will be working on some reports and charts for the doctors to take with them. I am collecting data from all of the mom's I talk to. I have a few that do not want their name or daughter's names used - and that is fine. They are still sending me all the info I need to make my report.

Some day there will be a treatment and cure for ROHHAD.

All our love to our wonderful friends fighting ROHHAD:

Mason Byrom



Joshua Wooten


Sophia Adamo

Sleeping

P1100645
Originally uploaded by bamboozleroo

I was taking videos of Haven sleeping last night. I missed when she was yelling that her dad ruined the entire day because he made the wrong recipe. :)

Pain Sensors and ROHHAD

With ROHHAD the sensitivity to pain is affected. Most children have limited pain receptors although we know a few that do not have this.

When Haven was about 1 year old we began to notice that she never cried and never felt any pain when she would fall down or get hurt. But we didn't think much about it. Then when everything went downhill at age 2 I paid more attention to it.

Haven does not feel pain. She knows she should scream or say ouch or something when she should probably be hurt. But doesn't really feel pain - so I know when a doctor asks if something hurts she can't really tell.

One time Haven feel asleep while going potty (she randomly falls asleep in the middle of a sentence). All of the sudden there was a loud noise in the bathroom and some scrambling. Haven had fallen off the toilet and hit her head on the edge of the door. Leaving a large lump in the middle of her forehead with a crease down the center of it for hours from the door. Never once screamed or cried or even made a noise.

Earlier this week Haven was making fruit skewers and you guessed it, jammed a skewer in to her hand. All she says was "ouch" very calmly. But she had a skewer in her hand and it was bleeding. She was clearly not bothered by it half as much as we were. Now there is a bit of a hole in the center of her hand - we keep peroxide on it.

It is definitely clear to us that Haven could fracture a bone and never know it. So when she does say something hurts we listen.

Long Week.....

This has been the longest week in a long time! In 4 days we've had 5 doctor appointments! We're still waiting on results from everything. We need results from the sleep study, bone density scan, and cardiology. Haven's Cyclosporin levels were good yesterday.

We met with the surgeon on Wednesday this week about the tumor on her back. He said with the size of it he would have to make a fairly large incision in the back and it would make her extremely uncomfortable. He did say it felt like it wasn't connected to anything and that with this type he would have to "scoop" it out. However, in the end we chose not to have it. He said the risk of the surgery to her health far outweighed the benefits right now. Later down the line we will remove it. But Haven has a lowered immune system and in one month her immune system could be completely shut down.

I was hoping to have some updates by the weekend of anything....but these things take time.

Love to all!

Oncology ROHHAD Meeting 2-13

So I remembered this morning that I forget a lot! I believe it's because we see way too many doctors and we have way too many conversations with doctors and ourselves that we forget who we've told something to and what we've even told them. I was talking with my friend Emily this morning and realized I had already told her and then I was talking to Julie (Mason's mom) and realized I hadn't told her. AND I talk to Julie multiple times a day. I know I've called family this weekend to tell them but I don't talk to all of the family. So here is an update from Haven's meeting last Friday with Oncology.

So as most know, I'm the loud one in the family and I am constantly challenging the doctors. Which I'm sure is really annoying to them. Well, I have not been very happy with the chemo we are on and felt like Haven was getting worse and worse. I thought this was supposed to be the cure to her ROHHAD and I haven't seen anything.

On Jan. 1st Haven was 68 pounds and on Feb. 13th she was 78 pounds. Instantly I was upset and emailed her doctors and had Kris talk to them about it. I wanted her off the chemo immediately. 10 pounds in one month is crazy! I also gave Kris a list of questions I had so he could ask them for me and he was texting me while he was in there. The doctor always tells him to just incase he forgot anything. The doctor knows Kris will be in trouble if he forgets anything....I think it's a joke between the two of them!

When I got home on Friday Kris explained what happened at the visit and now I understand why Haven is on Cyclosporin and what we are doing.

APRIL 1ST - this will be a big day! Here is what I now understand:

She is on Cyclosporin until April 1st when her doctors will take all of the results, good and bad, to the medical board. At that point they will decide one of two options.

1. IF we are seeing results and noticing changes: They will change her chemotherapy
2. IF we are not seeing any results they will start to monitor her without the chemo

As always, we ask for straight to the point honest scenarios from the doctors. The chemo she would change to (which he did not give a name) would be a heavy dose and would make her sick. Guaranteed she would be sick and that she will be at a high level risk for infection. Essentially putting Haven in a "bubble" world for a while. Everything would need to be sterile and she would be wearing the mask.

IF we go this route we will most likely not allow visitors to the house as we will need to keep her away from anything she could possibly get.

Our last resort would be for nothing to work and to just monitor her. Why? Everything can go bad so fast. We know there are 30 or so kids in the world that have ROHHAD. But what we also know is that ONLY 6 children have Ganglioneuroblastoma Cancer mixed with ROHHAD. Haven has both. Of those 6 children, 2 have passed away before age 12.

So I will lay off the doctors for a while and follow their programming and appointments and keep notes of anything new or any changes. If you are planning to visit Miss Haven the best times would be between March 1st and March 22nd. After March 22nd we will be preparing for her surgery and hopefully news from the medical board.

PS: the charts were wrong on Haven. She was never up to 77 pounds. On Feb. 4th she weighed 74 pounds and today she is down to 71 pounds. We have lost about 1 pound per week.