Parent’s Perspective: Beyond Palliative Care

Published on in CHOP Family News

PACT’s Support Helped Us Cope with Rare, Life-limiting Condition

Jones Family PACT The Jones family, clockwise from top left, Claude, Monica, Jaydon, Isaiah and Cailyn Between my three children, my family sees eight different specialists at The Children’s Hospital of Philadelphia. Each one is important for treating specific conditions for a specific child. But another group, the Pediatric Advanced Care Team (PACT), holds a special place in my heart.

Most families at CHOP are never in the position to meet the amazing providers from PACT. PACT is called when a child has a life-limiting condition or develops a disease that cannot be cured. Team members provide palliative care and help families handle these difficult, stressful and heart-breaking situations.

I was introduced to PACT when my son Isaiah was 6 months old. After many miscarriages, Isaiah and his twin, Jaydon, were born Dec. 14, 2005. Jaydon was healthy; Isaiah was born with an extremely rare condition, idiopathic infantile arterial calcification (IIAC). He had calcium deposits throughout his body, including his arteries. There is no cure and, at that time, there was no treatment. Isaiah wasn’t expected to live 24 hours, but managed to beat the odds. After three months in the Newborn/Infant Intensive Care Unit, he came home with hospice care. It was touch and go.

It seemed like we were back at CHOP in the Emergency Department every other weekend. It was in the ED where Carolyn Long, MSW, a social worker in the PACT team, approached me. She told me: “I’ve read Isaiah’s chart; I know what’s going on, and we can help you if you let us.”

I admit I was standoffish. With my husband, Claude, on deployment orders with the Army National Guard, I prided myself in holding down the fort. I thought I was doing a great job.

Carolyn helped me understand it didn’t make me weak to admit I needed help. Support from PACT has changed our lives. Members of PACT helped me create the best quality of life for Isaiah and to accomplish my goal of keeping him out of the Hospital.

It was PACT that convinced insurers that Isaiah needed 16 hours of home care nursing a day. He uses a wheelchair, oxygen, blood pressure machine, suction, pulse ox and a neck brace, needs daily breathing and chest treatments, receives nutrition through a feeding tube, and is nonverbal and legally blind. Since age 3, he has gone to St. John of God School for Special Children where he has speech, physical and occupational therapies and special therapies for the blind. His sight has improved to where he’s now near-sighted. He can laugh and make noises, and he chimes in at appropriate times. The school is using touch screens to help him with communication so he can tell us what he’s trying to say. He now is on 11 medications.

Medically, nephrologist Kevin Meyers, MBBCh, is the man in the cape. He has come up with a concoction of medicines that have managed Isaiah’s IIAC.

There are 168 people in the world with reported cases of IIAC; 22 of them are still alive. One is Isaiah; another is my daughter, Cailyn, who was born in February 2009.

Cailyn, now in kindergarten, has milder IIAC. It caused a stroke and seizures, which, thankfully, were controlled by medication. She is followed by the Stroke Program, Nephrology, Cardiology, Allergy and Rehabilitation Medicine. When doctors wanted Cailyn to stay on an experimental medication for IIAC, I refused because of the side effects. Members of PACT were awesome, supporting me and reminding me that I have the right to make my own decisions about what’s best for my children.

Chris Feudtner, MD, PhD, nurse coordinator Gwenn Laragione, BSN, RN, CCM, CHPPN, nurse practitioner Gina Santucci, MSN, APRN-BC, child life specialist Kelly Gross, BS, CCLS, CTRS, and Carolyn have all been there for us over the years. We started with palliative care and, as Isaiah stabilized, moved into the full breadth of what PACT offers: coordinating care and communication among all those specialists, connecting us with social services, helping Jaydon cope with having ill siblings, and supporting us emotionally.

Now I’m using my experiences at CHOP to help other families. I was hired as a family consultant in February, and I’m working on projects to incorporate the family perspective into patient safety, complex care and well-sibling issues.

— Monica Jones