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.