Back to School with a 504 plan

CCMC Dr. Jonathan Martin and Olivia

This past month has been pretty uneventful in regards to emergencies and excess trips to CCMC. Livvy had an MRI scheduled for a Saturday morning without sedation, but...ahem, that didn't work out. Radiology squeezed us in before her appointments with the neurosurgeon and rheumatoligist.  For the sedated MRI she had propofol and versed (which is awesome as she doesn't even remember where she fell asleep).

We went for the results on Thursday. The MRI did not show anymore degeneration and she has grown about 1 1/2" since this all began! Dr. Martin, the neurosurgeon who did her open biopsy, said she could wear the brace now between 4-8 hours a day, rather than all day, all the time (except for sleep). THIS means she can return to school 1/2 time,

I met with the guidance counselor at her school the next day and brought the Dr's request for the 504 plan. She'll be able to go to school for about 4 hours a day, wearing the brace. She won't attend the 'specials' like gym (which she is excluded from and hates anyway), art and music. She gets to stay on the 3rd floor, where her classes are located. For lunch she gets to bring a few friends to hang out with. She's so excited to go back to school and see all of her friends.


The plan for the brace: Wear it anytime she'll be walking 30 or more minutes and have it on at least 4 hours a day. At home, she can take it off and just relax!

Her pain is under control for the most part. She does complain at the end of the day and goes to lay down with her feet up. She'll also be starting physical therapy next week and I'm trying to convince her its going to be fun.

Finally a diagnosis! C.R.M.O.

Olivia had her 3rd biopsy last week, the results showed no histiocytes, nor any signs of infection, bacteria, fungus etc. Our oncologist and the team he consulted with suspected the lytic lesions were caused by Chronic Recurrent Multifocal Osteomylitis, a very rare disorder. He referred us to a CCMC rheumatologist, Dr. Zemel. After meeting with us, he consulted with Dr. Polly Ferguson, from Iowa, who specializes researching CRMO. When the oncologist mentioned CRMO, I knew just what he was talking about as I came across it on BoneTumor.org. It mentioned pustules on the hands and feet, which she has had in the past. Our pediatrician and I believed it was eczema...treated it with topical hydrocortisone...but who would have known it would lead to or be connected to this?

Today we went back to CCMC for another (2nd) pamidronate infusion. Dr. Parikh and gave us an update and said he was sure that is C.R.M.O. Compared to the 'differentials' we were faced with, this appears to be the best diagnosis we could have hoped for. It's an autoinflammatory disorder that affects 1 in a million, mostly girls around the age of 10. Dr. Zemel came in to speak with us as well.

Here's a list of things we feared it could have been: bacterial osteomyelitis, Ewing sarcoma, leukemia, lymphoma, neuroblastoma metastasis, eosinophilic granuloma, Langerhans cell histiocytosis or non Langerhans cell histiocytosis. 

The treatment for Chronic Recurrent Multifocal Osteomylitis is (for her) Pamidronate infusions (which we already started), non-steroidal anti-inflammatorys, or steroids. Dr. Zemel said she may not even need any more infusions because after the last one, the MRI showed a bit of improvement. She could take an oral biphosphonate. He wrote her a script for a strong NSAI and we're waiting for the insurance to approve the new prescription. They tylenol w/codiene knocks her out and makes her a little off balance etc.


The rheumatologist wants a 'bone scan' which differs from a full skeletal survey. It was explained to be like an 'MRI' but any altered, affected bone will 'glow' in the images. In the meantime she's a little tired/weak from the infusions but showing no signs of hypocalcemia, like last time.

Read more about Chronic Recurrent Multifocal Osteomylitis at Bonetumor.org

Another Biopsy

After meeting with our Oncologist last week, he informed us that all the Histiocytic disorder specialists and pathologists wanted a culture done to rule out the possibility that the damage to Livvy's spine was done by infection. We met with the Infectious Disease specialist at CCMC who wasn't convinced that this was an infection because had it been, it would have 'colonized' in one place, then spread to surrounding vertebre. Olivia's lytic lesions are spread out, some in the upper vertebrea, then down at her sacrum. The Histiocytosis experts, including Dr. Shiela Weitzman at the Hospital for Sick Kids in Toronto, Dr. Arceci at John Hopkins, Dr. Ken McClain in Texas, pathologist Dr. Jaffe from Pittsburgh and many others were all consulted about her lytic lesions and all agreed another biopsy was in order.

The neurosurgeon would not do another open biopsy, I agree. I wouldn't want to put her through that again. The other doctors would have preferred another open biopsy. My choices were: Let the 'disease' progress or hope that it goes away on its own then deal with her back later, OR, get another biopsy to pinpoint what the heck is going on and start treatment before more damage is done. I chose to do another biopsy, a needle biopsy.

Thanks to Christine from Childlife!

Olivia had the needle biopsy yesterday. This time they went in for the lytic lesion on the sacrum (tailbone area). The took core bone samples and the doctor who preformed the guided CatScan biopsy said the bone was more dense this time and sclerotic. Hopefully they can either prove or disprove that this is Histiocytosis or an infection.

She was very groggy, cranky and irritable after the anesthesia and versed. Since her procedure wasn't done till about 3pm and she hadn't eaten since the night before, she was pretty much 'starving' and demanded pizza from Vinny's. She ate, took tylenol w/codiene when the pain kicked in and went to sleep for the night. She was supposed to meet with her tutor yesterday (2nd session) but we had to cancel and I'm canceling todays session to let her rest.

Huge thanks to Christine from ChildLife for spending ALL DAY with us yesterday. She is a great comfort to Livvy, made her laugh and provided some fun games to help keep her mind off everything. I can't stress enough how helpful Childlife has been.   

First Infusion and Jonas Brothers Charity Softball

"Road Dogs" Jonas Brothers Charity Softball

Olivia went for her first pamidronate infusion on Thursday, August 12th. Pamidronate (Aredia) is a 4 hour biphosphonate IV treatment that is given in the hospital. Since there wasn't much room in the Connecticut Children's Medical Center Hemo/Onc clinic for Livvy to lay comfortably, with me, and her big sister during treatment, they sent us up to the 8th floor and gave us a private room for the day, although there is an infusion center on that floor as well. (That's the same place we spent two weeks in July). They brought her lunch (she loves their chicken tenders) and we tried to play games. She even went on her laptop for a little way to play a game and complain about the treatment on Facebook lol.

Jonas Brother's Road Dogs Tour Bus

Brighter Hope Campaign & The Jonas Brothers
The Children's Hospital has a Brighter Hope Campaign, with plans to expand the hemo/onc clinic to include private infusion rooms and 8000 feet of space (much needed). The day after her treatment, we went to New Britain to see THE JONAS BROTHERS play a charity softball game to help raise funds for the new center.

 While we were getting the infusion, the nurse offered us tickets to see the Jonas Brothers play softball at the Rock Cats Stadium in New Britain. We got 6 tickets and took her most supportive friend along with us. Livvy was tired and had a little temp when she woke up. She took her Tylenol w/codiene and we picked up her friend and headed out. At first she was in good spirits. By the time the game started she was pretty worn out and hot. After we got the chance to see the Jonas Brothers in person, Livvy felt a little nauseous so we decided to leave before the game actually started. When we reached our driveway she was really upset and said, pretty much that everything hurt. Took off the brace, got her in bed and she slept most of the day.

Olivia, her awesome & supportive friend, and little sister

Last night was pretty bad. Everything hurts, from her toes, fingers, knees to her head. This morning, same thing and a temp of 101.1. Apparently this is 'normal' for up to 48 hours following the first treatment. She woke up, had tylenol and went back to sleep saying she didn't even want to get out of bed. She's sleeping so I decided to update the blog and post the pictures from yesterday.

"Road Dogs" Joe Jonas stretching

About Pamidronate Infusion: 
"Cancer cells that spread to the bone can secrete substances that can cause cells found in the bone called osteoclasts to dissolve or "eat away" a portion of the bone.  These tumors or lesions weaken the bone and can lead to complications.  Some of the complications resulting from this bone breakdown are bone pain, fractures and less commonly, hypercalcemia (increased levels of calcium in the blood).
Aredia is a bisphosphonate.  Bisphosphonate medications are used to slow down the osteoclast's effects on the bone.  In doing this it can be useful in slowing down or preventing the complications (bone pain, fractures, or high calcium levels) of the bone breakdown."

Progress (sort of)

check out the decorated "Minerva" brace

Olivia followed up with hemo/onc. last Thursday, the plan was to get a a brain MRI, x-rays of her jaw, a urine sample and some additional lab work to once again 'rule out' all the 'bad stuff' and any autoimmune disorders. The jaw x-ray was done because she complained of sensitivity when a nurse took her temp by ear.

Today we went back and the Onc said the x-ray "appeared" to show another mass on her jaw and 'possibly a fracture'. We then talked (cried some too) about treatment, which will include something like Fosamax, prednisone and vinblastine.

Impatiently waiting at the clinic

She was sent down for a CT Scan, which she did like a trooper, without SEDATION (yay!) We went back and waited in the clinic for the results, while the Onc Dr. prepared a form to bring to DMV for a temp-handicapped parking pass (long walking takes a toll) and a wheel chair, in case we want to actually go somewhere FUN.


The CT Scan was CLEAR and showed no lesions- just a lonely little tooth that hasn't erupted yet! So back to the drawing board.

No definitive diagnoses other than "non langerhans histiocytosis," and still trying to rule out the uglier things. They delay is because the sample taken during biopsy were weird, two showed normal cells, the other can't be pinpointed.  Still waiting for more guidance from John Hopkins & North Shore's opinion.

We Love Becky from Childlife-she rocks!

In the meantime, the Onc suggests starting fosamax to rebuild the bone...the steroids were on hold because of the brace/ fracture. Once she's out of the brace we can start steroids, which is in about another week or so. Maybe vinblastine...he gave me lots of homework to do, printouts off all the meds/side effects, etc. We also want to be SURE we treat whatever this is the right way, without "masking" anything else. Whew. So we're home now. As a reward for not freaking out during the CT scan and all her bravery today (not one tear during blood work!) we're going out for dinner.

(By the way, this type of back brace is called a Minerva Brace.)

Hopeful: Vertebra Plana and Bone Regrowth, Back Braces

People keep asking why Olivia has to wear a back brace. The short answer is to stabilize her spine. Here's the long answer:

As you can see, whichever form of histiocytosis she has, caused lesions in the vertebra, leading to vertebra plana, or the flattening of a vertebra. In her case, this caused excruciating back pain (in several areas) and the pain even traveled around her rib cage, above the area where her stomach would be. (So much for the mild milk allergy causing pain!) During her open biopsy, the nerve that was causing all the pain was snipped. Between that and the initial round of steroids that pain is gone.

Initially I was told about two options, one would be to put Livvy in a brace and allow the bone to regrow. The bone could either fuse to the next vertebra naturally or could regrow into its original shape and fill the space. The other option would be surgery with rods, but that's off the table right now. She's in the brace for 6 weeks and is set to start steroids this week.

I did some research on Vertebra plana in children and came across a study called "Vertebra plana. Long-term folow-up in five patients," published by "The Journal of Bone and Joint Surgery" out of Massachusetts. The study followed five patients treated at the Department of Orthopedic Surgery in Rome, Italy. All of the patients had Histiocytosis and were followed for between twelve and thirty years after the initial diagnosis. They were between the ages of 4 and 12 at the time of diagnosis. Years later, none of the patients had any symptoms related to the original disease which caused vertebra plana and the bones regrowth was between 48 and 95 percent of normal. This gives me great hope.

The image below was included in the study. The boys in the study were diagnosed at the ages of 4,5,7,10 and 12. Follow up ages were 19, 25, 19, 40 and 30 respectively. The boys wore plaster jackets, back braces and one had chemotherapy.

Vertebra plana case study over several years

The link to the study on Vertebra plana is here: Vertebra plana follow up study. (It opens as a PDF file).

Here are some pictures of Olivia's spine, taken during a CT scan at the Connecticut Children's Hospital in July 2010.

We are going HOME!!

The results are in-although not "black and white". Olivia does have Histeocytosis, which can be treated on an outpatient basis! Her slides will be compared with another patient of Dr. Parikh's, with a similar, rare case of histeocytosis (non langerhans). Slides will also be sent to Dr. Robert Arceci at John Hopkins. We'll be back here sometime next week to meet with the neurosurgeon and onc/hemo docs for a treatment plan. She's SO excited to be going home!!!!!

Waiting and Passing Time

Olivia's been doing great in her back brace. Early this morning we did a lap around the floor before the neurosurgeon came to check on us. He said he'd probably have pathology news from Oncology-Hematology doctor (Dr. Parikh)  later in the day. After that, we met with physical therapy and did some more walking. We visited the activity room but due to some really hyper boys in large play-cars, we left to avoid being bumped. Luckily, Valentine the Clown stopped by and had Olivia cracking up.

Later occupational therapy stopped by to help Olivia re-learn some basic care skills and how to safely maneuver in everyday situations. We started a friendship bracelet, did some sand art and got part of a pillow craft project completed. The point of physical therapy and occupational therapy is to teach Livvy to get around in her brace, so we can hopefully get out of here soon. According to another Onc/Hemo doc, the goal is to get her stable enough to go home, regardless of how late the path results come. This way, we can go home and come back for outpatient treatment or be admitted for treatment, depending on the actual diagnosis. Overall, we just have to take it day by day.

Tonight we had some drama getting blood drawn for labs, her little veins just wouldn't cooperate. Tomorrow we'll head down to the lab for that, tonight we're just taking it easy. Dr. Martin, the neurosurgeon, stopped by again and said he expected pathology results or "news" for tomorrow.

Decorated Backbrace

Olivia was fitted with a back brace this week. Though we don't quite know why her spine is the way it is, we can help stabilize her with a brace. She says it looks like a refrigerator. We think she looks super cute-we decorated the brace so that it's one of a kind, Miss Olivia is a fabulous artist ;)

What Happened to Livvy?

Olivia was admitted to the Connecticut Childrens Medical Center in Hartford on July 1st. Because it's difficult to update family, friends and colleagues on "what's going on" with Livvy, I decided to keep a journal to share with you. I also hope this journal can help other parents if God forbid they find themselves desperate for answers or a diagnosis for their child.

Backstory
So how did this start?

Back in December 09, Olivia first complained of back pain. The pediatrician and I figured it was related to a slip on ice or something like that and she was given Motrin. The pain persisted for weeks and the doctor didn't think this was unusual, eventually the pain subsided, but was always worse at night. This particular pain was very low in her back, as if she fell on her tailbone.

This Summer
In early June, Olivia began complaining about back pain and a pain that stretched around her upper abdomen and ribs. I thought maybe it was gas or something, but on a Monday morning, she was in SO much pain I took her to the ER. Xrays didn't show anything abnormal so they sent us on our way, recommending Motrin and change in diet. A follow up with the pediatrician resulted in a 'mild milk allergy' that could have caused cramps, constipation and the muscle spasms in the back. Because she was in so much pain, and couldn't lay down, she missed the last week of school. I tried taking her for a second set of Xrays, but they sent us home because she refused to lay down because of the pain.

As June came to an end, Olivia's condition worsened, the pain, though it came and went, could be excruciating at times, waking her up screaming from a sound sleep. She wouldn't sleep in her bed because she said she could pull herself up easier on the couch. At one point the pediatrician recommended she see a physical therapist or chiropractor. She refused to let the chiropractor touch her-thank god. She also refused to lay down for x-rays. The pediatrician suspected her muscle spasms were exaggerated by anxiety and prescribed ativan, followed by zoloft.

CT Scan
My baby girl INSISTed she wasn't 'crazy' and was in real, unimagined pain. The dr finally set up the CT scan on Thursday, July 1st.

Spinal Lesions
The ct scan showed several lesions and the loss of an entire vertebrae, as if it totally collapsed or crumbled. The original hunch was "langerhans cell histiocytosis" eosinophilic granuloma. There are three kinds of 'histiocytosis' it could be, the first (name above) affects just bones, second, this same thing could affect other tissue in the body and the worst of this class of histiocytosis is lymphoma. Soon after, the CT Scan was followed by a needle biopsy, delayed by the 4th of July weekend, which came back "inconclusive." Next was a full Skeletal Survey (Xrays of all her bones), an MRI and and abdominal CT Scan.

An "open biopsy" was then done to take samples of the affected vertebrea lesions, and send them off for testing. The open biopsy surgery was done on July 7th. So far, the only news is that this may be a "Non Langerhans cell histiocytosis", again, waiting on pathology.

A few days later, still healing from a 5 inch incision along her spine, she was fitted with a brace to prevent further spinal collapse. In the meantime, today is Monday, July 12th, and I'm still waiting on Pathology results.

For most of this ordeal in CCMC (hospital) she was on a morphine drip, IV fluids, steroids, etc. Since they don't have a diagnosis, and her mood went from bad, to dangerously off the charts -they took her off both for the time being and the pain seems to be under control with Tylenol w/codeine and Ativan.