Ana’s Story Revisited

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Ana Today (at 13) – She got her hair highlighted the day after we found out she’d need to start chemotherapy again.

The story of Ana’s original diagnosis and the beginning of the long roller coaster ride of treatment is posted here. She was 11 years old in August of 2012. She turned thirteen this past May.

For those that haven’t been following the blog, I’m writing this page to summarize Ana’s journey and where we’re at now.

Intense stomach pain on her lower right side landed Ana in the ER on 8/25/12 following what had been a summer of worry – she’d been losing weight, lacked energy, was very pale. We learned that day that she had an enormous tumor on her liver which filled her abdomen. It was about 22 cm at the time (I believe), possibly a bit bigger. She was transported from our local hospital to Westchester Medical Center where, after about 15 days, she was finally diagnosed with Inflammatory Myofibroblastic Tumor (IMT). This is a tumor that is generally considered benign – removing it surgically is considered a “cure.” Ana spent weeks in the hospital and continued to lose weight. We were told she needed a liver transplant, but with the support of her oncologist, we opted to try and shrink the tumor and save her liver.

She had to be given IV nutrition, and was so depressed that we thought she’d lost the will to live. We didn’t know if she’d leave the hospital. Starting her on steroids prior to chemotherapy helped her regain her appetite and also seemed to stop the tumor’s growth. She was discharged after her first course of chemotherapy in early October in much better spirits, and her course of chemotherapy lasted about six weeks. During this time she lost the majority of her hair, but the tumor growth seemed inhibited. However, A CT scan at Sloan Kettering in November of that year revealed the chemo wasn’t doing its job, and the surgeon there said he couldn’t save her liver (we’d consulted with three other surgeons at that time, all of them said she needed a transplant).

Ana had her liver transplant in February 2013, and the tumor (which had grown to about 26 cm and weighed about 10 pounds) was removed along with it. We’ll be forever grateful to Ana’s donor. She had one episode of rejection (on her 12th birthday), which required hospitalization and heavy doses of anti-rejection medication for the next few months – this may have set the stage for the cancer’s imminent return since her immune system was greatly compromised.

She appeared cancer free for six months, but a scan in September of 2013 showed two potential lesions in her liver. These were dismissed as benign glands by her original oncologist.

Then, an MRI scan in January of 2014 demonstrated that these nodules had gotten bigger and also what looked like nodules in her lungs. She had a CT scan of her chest and abdomen in January 2014 which presented us with the worst possible news – she now had tumors in her abdomen and lungs.

Ana had the abdominal lesions removed by her transplant surgeon, Dr. Kato, this past February 2014, but we still had the lung tumors to worry about. The pathology revealed these tumors were the same disease as her original tumor – meaning, this was still IMT and it was assumed that the lung tumors were the same disease (a complication of transplant patients which is a type of lymphoma called PTLD was ruled out at that time.)

It is very rare for IMT to recur, but when it does it usually appears in the same area where it originally presented. It almost NEVER spreads, as Ana’s has done. The doctors were baffled. We switched her to her current oncologist, Dr. Yamashiro, at Columbia Presbyterian at that time (she was already being followed by a hepatologist at Colulmbia since her transplant).

We then met with Dr. Yamashiro to discuss Ana’s treatment options. He started her on Celebrex (an anti-inflammatory) and Dr. Martinez (the hepatologist) reduced her anti-rejection meds – a lot. The theory was that the high dose of anti-rejection medication may have suppressed her immune system too much, allowing the cancer to recur. in March 2014 we got a brief reprieve when it appeared that her largest lung tumor got a little smaller. However, Ana’s liver enzymes spiked in April and she was put on steroids and increased anti-rejection meds at that time.

A CT scan in May 2014 (two weeks before her 13th birthday), showed the lung tumors were growing – and there were more of them. We were introduced to Dr. Middlesworth, a pediatric oncology surgeon who wanted to do two extensive lung surgeries to try and remove as many tumors as possible. I was desperately afraid to put Ana through these surgeries, and it seemed she was given another reprieve when Dr. Yamashiro finally got the results of the full genomic sequencing of the tumors removed in February. He had discovered a novel gene fusion and he suggested she try a drug called Jakafi to target the mutated gene. So, the lung surgery was put on hold while we obtained the drug.

In the meantime, we went to Atlantis in June of 2014 – the trip was funded by Make A Wish.

Ana had yet another CT scan in July 2014 after we got back from the Atlantis trip (in good spirits, hopeful about the Jakafi, and relieved to have avoided the lung surgery thus far). But this hopefulness was short lived. We learned there was a new tumor in her pelvis, and it was very large for having appeared in about a month. The lung tumors took a temporary back seat to the pelvic tumor, which surprised Dr. Yamshiro and was very worrisome since it was so close to her bladder. She had it removed in September 2014 and did very well. She started 8th grade about five days later.

She’d been on the Jakafi since about July, and a PET scan in September and another in October gave us yet another reprieve. The lung tumors were stable on both scans. Dr. Yamashiro would keep her on Jakafi and it was now a watch and wait scenario. We kicked around the idea of “what ifs” at that time – if the lung tumors started growing, then there certain things he could try, but chemotherapy was at the top of his list. I pushed this to the back of my mind and we pushed on, relaxing into a normal schedule for the next few months.

This post from Ana’s December 6, CT scan is where Ana’s story starts for some of you, but as you can see from everything I’ve written above – it’s just the next phase of a very long, grueling journey. The last scan showed tumor progression in her lungs. Finally, horribly, the Jakafi had stopped working. We learned the very next day that they also saw two nodules in her liver. Ana was adamant that she wouldn’t get chemotherapy again, and I didn’t want to put her through it knowing that it didn’t work the first time. But that left us with few options, and the prospect of Ana getting very sick very quickly.

She’s since changed her mind, and a subsequent conversation with Dr. Yamashiro also made me more hopeful that chemotherapy may be able to stabilize Ana’s cancer, or even put her back into remission. But to buckle up and start the ride again – well, it’s overwhelmingly exhausting.

On the day of her scan, we started talking with Ana about some things she might want to experience before she gets very sick, and that’s how her wish list was born. At first talking about it was painful and emotional – I’d made the mistake of calling it a “bucket list” – but when I switched the language to wish list, Ana opened up more. It includes experiences she’d like to have as well as some material things she’s always wanted. I posted it here. We have a Paypal account in Ana’s name where people have been generously donating to contribute to this list, though some have also preferred to contact me directly to see how they can help grant one of her wishes.

She will start chemotherapy next month and I will continue to update the blog throughout this next leg of Ana’s journey.

 

 

 

 

3 Comments So Far

  1. Thanks for sharing this story. It is heartening and helpful to actually feel the love woven throughout. It is inspiring as well to read of the courage and bravery in the face of major health challenges! I look forward to reading the wish list and contributing to fulfill her dreams sending hugs and healing energy
    Ann

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