Imagine yourself learning that your child has a life-limiting disability. The emotional toll of such a diagnosis could be devastating, even to the strongest person. Emma Wallace and her family are just one of the many receiving care through VNH’s Pediatric Palliative Care (PPC) program. Alison Wallace (AW) and VNH’s Pediatric RN Lily Burke (LB) talk about the PPC and caring for Emma.
VNH: What is Emma’s diagnosis?
LB: Emma has a neurological condition called Aicardi Syndrome in which she lacks the “bridge” (called the corpus callosum) that allows nerve impulses to travel from one side of her brain to the other. This affects both her motor and cognitive abilities, in addition to causing seizures. She cannot sit up or stand on her own because she has poor tone throughout her muscles. Her communication is limited and she shares her feelings using eye contact and facial expressions.
(Visit aicardisyndromefoundation.org for more about Aicardi Syndrome)
VNH: When did you learn about her condition?
AW: When I was five months’ pregnant, the doctor saw on the ultrasound that she had enlarged ventricles. Then they saw that the corpus callosum had not developed. We had a normal birth, but at 4½ months, Emma had her first seizure. There are three symptoms that doctors look for when they diagnose Aicardi Syndrome: no corpus callosum, Epilepsy (recurrent seizures), and eye abnormalities. The eye abnormalities were observed when she was 3 years old, and that’s when the actual diagnosis was made.
VNH: What is a typical day like for Emma?
AW: A typical day begins when we get Emma up in the morning. We disconnect her food pump (because she eats overnight) and then give her her anti-seizure meds. Then we put on the machine that shakes her – it is a vest with hoses that’s connected to a machine that shakes her to get the stuff moving and help her breathe. It’s more convenient than having to tap her back and front. She also has a nebulizer mask to help her breathe.
LB: She has what’s called a Chest PT vest which she uses two times each day for 20 minutes. The vest has tubes which use air pressure to vibrate the vest and the patient to help loosen secretions so the body can manage them more effectively (or they can be suctioned out). The vest works with her other breathing treatments to help prevent pneumonia and/or respiratory infections.
AW: She has a history of getting pneumonia. I’d say we’ve been to the hospital every six months because she comes down with pneumonia. Other than that, we get to doing every day stuff. Emma has just entered second grade. She is 7 years old.
LB: Emma is in a great public school in Vermont. She is in a mainstream classroom even though she has medical needs. But she goes to PE and art class, etc. She has a great aide at school who helps with all her needs. It was hard to find the right person, but the aide she works with is great. Emma participates in class activities when she can, but the other kids interact with her even if she is not doing what they’re doing. So it’s good for the other kids in class, too.
VNH: What kind of care does Emma receive from VNH?
AW: Lily comes once a week (sometimes once every two weeks) to listen to Emma breathe. It’s been very helpful to have Lily come. There have been times when it has been easier to have Lily come to us rather than going to the doctor. She is very caring. We love having her.
LB: I listen to her lungs to try to tell if Emma is possibly getting sick before she actually does. This is one of the goals of her treatment – we want to try to make her to be less susceptible to pneumonia. The other goal of our visits is to give her parents peace of mind.
AW: Andrea comes once a week, too. Andrea works on projects with Emma and her older sister, Elizabeth. Sometimes they will go for a walk, but they also do creative projects. Andrea was helping the girls make clay beads. They also made cards for Valentine’s Day. Both girls look forward to Andrea’s visits. She helps Emma participate in the way she can. It’s nice for the girls to have something to do together.
LB: Andrea Herrington is an expressive therapist who uses creative projects to help the girls (Emma and Elizabeth) to play together. It is important for the girls to learn to interact. Although Emma is non-verbal, she does go through all of her emotions and feelings.
One time, Emma had made a choice about something using her eyes, but Elizabeth wanted to do something else. Andrea said that Emma made a choice and they needed to do that. Elizabeth was frustrated that they couldn’t do what she wanted. It was a very normal interaction between sisters, and that’s really good.
VNH: What is the best part of the day for you and Emma?
AW: Best part of our day is when I am holding her, especially if she is vocal that day. I love having our one-on-one time. I feel lucky to be her mom. She wouldn’t be who she is if her condition were different.
VNH: Worst part of the day?
AW: I’d say that’s not being able to just get up and go and do things. There’s more involved with a child with disabilities to just to do regular things. You know, just to go out, you have to pack up her equipment and all her stuff just to go anywhere.
LB: It’s so clear how much the family loves Emma. She is able to go swimming and play with the family’s four dogs. There are so many everyday things going on and that’s what we love to see.