was diagnosed with hepatic infantile hemangioendothelioma in November 2001
at three months of age. After failed attempts to treat her condition with
steroids and chemotherapy, she received a liver transplant in January 2002. After the transplant, we learned she also had pediatric kaposiform
angiosarcoma--a rare and deadly cancer.This is her story.
We started a support mailing list on Yahoo for families of children suffering from hepatic infantile hemangioendothelioma and related issues such as liver hemangiomas, pediatric angiosarcoma, and liver transplant. There's also a Facebook group.
Saffy's treatment diary is now on separate pages--it got much too long for one page. It also includes several pictures and links to other pages--including Saffy's Liver Transplant Photo Album (not for the squeamish).
The diary covers a lot of details. For those who don't have time for the full story, this is the big picture. (Last updated mid-April 2002)
Saffy was born prematurely in August 2001. She spent her first six weeks in the hospital while she got stronger. In November, she was diagnosed with hepatic infantile hemangioendothelioma. The vascular tumors had enlarged her liver to such a degree that they were pressing against her stomach and other organs causing problems with feeding and such. After a few short hospital stays and failed treatment with steroids, Saffy was hospitalized at the end of the year. The tumors continued to grow; and, on New Year's Eve, the pressure on her lungs caused her to go into respiratory distress. She was intubated (put on a ventilator) in an emergency situation and moved to the Pediatric Intensive Care Unit (PICU).
Saffy spent over three months in the PICU. She started chemotherapy in early January 2002, but the tumors grew unabated. Heart failure and hypothyroidism--both resulting from the strain of the tumors--complicated her condition. In mid-January, she was transferred to Mount Sinai in New York City and placed on the liver transplant waiting list as Status 1 (less than 7 days to live). She received a new liver on January 21 in an operation that took 11 hours.
The good news up to this point was that hemangioendothelioma is a benign tumor; and, once it was removed, it would be gone forever. However, the pathological analysis of Saffy's excised liver revealed that the tumors also included pediatric kaposiform angiosarcoma. Angiosarcoma is a very aggressive cancer that typically is only seen in older people who have been exposed to carcinogens like arsenic for a couple decades. It is exceedingly rare in children, which has precluded the development of an effective treatment. The medical texts only record about 30 cases in children and, of those, only three have survived.
The hope is that all the remnants of the cancer were removed with Saffy's old liver. Unfortunately, she probably can't receive chemotherapy as a preemptive measure, because her new liver is very fragile, and she is also receiving immunosuppressants to avoid rejection. We've located reports of four children and 14 adults with hepatic angiosarcoma treated with liver transplantation; but, unfortunately, none survived--the tumor usually recurred in the new liver. Of course, if it weren't for Saffy's new liver, she wouldn't be alive today.
After her transplant, Saffy slowly recovered. Her heart improved, but she had problems getting off the ventilator. It took five extubation attempts over a couple months, before she was able to breath on her own. Saffy was discharged from Mount Sinai in mid-April. She is currently at home with supplemental oxygen and a feeding tube and has a home care nurse for 12 hours per day. She visits the transplant clinic twice weekly and has numerous appointments with other specialists. She will need to undergo CT scans every 2-3 months to watch for any return of the angiosarcoma; but, until then, we can only wait and hope it's gone.
The Bibliography page lists the medical texts we have obtained while researching Saffy's liver condition.
Entire Contents Copyright © 1996 - 2014 Alexander E. Usticke. All rights reserved.