After Lacey Martin’s leukemia didn’t respond to initial rounds of chemotherapy and after she spent 10 weeks hospitalized for a stem cell transplant, the 11-year-old New Hampshire girl went home March 2 with an external line for medications that her mother would have to flush and clean twice a day. Lacey’s immune system and infection-fighting ability were so weakened from her treatment that she was under isolation precautions for six months after she left the hospital. Any bloodstream infection contracted through the line, which exited her chest, would be serious and potentially life-threatening.
It is terrifying knowing your child is so susceptible to bacteria and infection and you’re doing it in the house, with kids running around and the dog. I needed to know how to do it right, and I needed to know I could do it.
Caring for a child’s central line at home is, to say the least, a daunting responsibility.
“It’s extremely scary,” says Crystal Martin, Lacey’s mother. “It is terrifying knowing your child is so susceptible to bacteria and infection and you’re doing it in the house, with kids running around and the dog. I needed to know how to do it right, and I needed to know I could do it.”
This is the frontier of efforts to improve the quality of care. With more and more complex tasks moving from the hospital ward to the home, improving inpatient safety and quality of care is only the first step. After collaborating with other pediatric oncology programs to successfully reduce inpatient central-line-associated bloodstream infections (CLABSIs), Dana-Farber/Boston Children’s Cancer and Blood Disorders Center has now launched an initiative to reduce outpatient CLABSIs.
The Martins are among the first families to participate.
On September 24, 2015, in a sixth floor room at Boston Children’s Hospital, 5-year-old Logan Lesselroth pressed the button that started the transfer of his newly harvested blood stem cells to his 3-year-old sister, Gianna.
“This,” Gianna told him, “is a gift from your body.”
The path to that moment and the stem cell transplant’s potential to cure Gianna of her relapsed leukemia was anything but straightforward. Logan has a genetic condition called medium-chain acyl-CoA dehydrogenase deficiency (MCADD), which makes it difficult for his body to convert sugar to energy. Would his metabolic disorder be passed to Gianna? Would the disorder make it too risky for Logan to have his stem cells harvested?
Diagnosed with acute lymphoblastic leukemia at the age of 4½ months, Gianna achieved a remission that lasted two years. In May 2015, the leukemia was back. With that, Mike and Marissa Lesselroth sought options for their daughter in their home state of Florida and beyond. “We talked to her doctors in Florida, and they agreed that coming to Dana-Farber/Boston Children’s was the best choice for Gianna because they offered a lot of treatment options for relapsed leukemia,” Mike says. …
Having a child diagnosed with a life-threatening illness is heart-wrenching for all parents, but when the diagnosis itself is uncertain, parents can face excruciatingly difficult decisions. This is what Katie and Josh Stevens of Idaho confronted after their son Riley was diagnosed in October 2012 with the blood disorder aplastic anemia, in which the body’s bone marrow produces too few oxygen-carrying red blood cells, too few infection-fighting white blood cells and too few clot-promoting platelets.
He was an 11-year-old skier and runner who was tired, bruised and unable to shake a cold. When his blood was drawn, he became pale, and his lips turned blue.
So Riley began a course of medications to suppress his immune system, but he had only a minimal response to the therapy. He needed a stem cell transplant.
Further testing at Seattle Children’s revealed Riley had shortened telomeres, or ends of chromosones, which stymied cells’ ability to regenerate. This is characteristic of another rare bone marrow failure syndrome called dyskeratosis congenita (DC), but Riley did not manifest other telltale signs of DC such as white spots in the mouth, lacy rash around the neck and brittle nails. Katie and Josh would need to decide between two very different stem cell transplant options. …
Allison seems an unlikely candidate to teach medicine to Mark Kieran. She’s an 8-year-old New Hampshire second grader who loves basketball, hip hop, acrobatic dancing and jewelry. He’s a pediatric neuro-oncologist with a PhD in molecular biology, not to mention decades of clinical and research experience. But teach Kieran, Allison does.
In December 2012, Allison was diagnosed with metastatic anaplastic astrocytoma brain tumors — two on her brain stem, two on her spine, and three at the top of her head. She had surgery and chemotherapy — and for two months her tumors responded to therapy. Then treatment stopped working.
Genomic testing revealed Allison’s tumors had a genetic mutation — a so-called BRAF mutation — seen in some cases of the skin cancer melanoma, which mainly affects adults.
Kieran, clinical director of the Brain Tumor Center at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, suggested enrolling Allison on a clinical trial of dabrafenib, a drug targeting the BRAF mutation in melanoma patients.
She would be the first pediatric brain tumor patient in the world to join the trial.