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One parent wishes that she could have consulted a crystal ball at the beginning of her daughter’s illness to have “some kind of idea of what we were in for. There are so many stages of letting go of the idea of what your little kid is in for in life, what they are going to do and how it is going to be.”
Another worries about whether she has done enough for her child. “I always feel that I should be doing something else or wonder whether I am doing enough to find out whether this treatment is the right way to go. The main thing I don’t want to feel years from now is that I ‘shoulda coulda woulda’ done something else and she would have been better.”
Katherine’s pediatrician then referred her to Boston Children’s Hospital, where pediatric rheumatologist Dr. Susan Kim diagnosed the toddler with spondyloarthritis. Since that day eight years ago, Katherine’s family makes the trip to Boston from their home in South Berwick, Maine, many times each year to see Dr. Kim, a doctor who “listens, supports and treats.”
In addition to monthly tocilizumab infusions in Boston, Katherine’s therapy includes sulfasalazine, prednisone, celecoxib, omeprazole, gabapentin and tramadol, as needed. For most of this school year, she has also been taking amoxicillin to “keep the infections at bay,” her mother says. Her daughter’s pain has never been completely controlled, although “right now she is the best she has ever been, and we are all crossing our fingers. She would tell you that she is in pretty bad pain every day all over.”
Denise, of New York, first noticed her daughter’s symptoms one morning about two years ago. The 2 year old had been a bit slow moving for a few days, but one Friday morning Emma had a prominent limp. By Saturday morning at 8:30, Denise and her husband were at the pediatrician’s office with Emma. That visit led to a month of visits to pediatric orthopedists who initially diagnosed transient synovitis in Emma’s knee, something that would go away on its own. “But it didn’t, and I started to get a bit more concerned, and so we went to a top orthopedist in Manhattan,” she says.
One look at Emma prompted that orthopedist to refer Emma to a rheumatologist, who made the diagnosis of juvenile idiopathic arthritis. “It was a frustration for me that we might have gotten this diagnosis a couple of weeks earlier since we were on top of it from the first day. But some people say it can take even longer to get a diagnosis.”
Pediatric rheumatologist Dr. Beth Susan Gottlieb, at North Shore-LIJ Medical Center, N.Y., is now treating Emma with methotrexate. Emma is doing well at age 4 and has few side effects from the therapy.
Jessica Krauss’s 11-year-old son, Waylon, also began troubling symptoms at age 2, when he had swelling in his left knee and couldn’t put any weight on the leg. Waylon was admitted to the hospital for two weeks and underwent numerous tests while doctors tried to determine what his illness could be. He was sent home with intravenous antibiotics for three weeks, but eventually developed a high fever and a rash, which prompted treatment with two other antibiotics. “It was dreadful knowing that something is going on with your child and you don’t know what it is,” Ms. Krauss says.
After five weeks and a bone biopsy to rule out leukemia, the diagnosis of systemic juvenile idiopathic arthritis (SJIA) was made, and Waylon was sent home with a prescription for steroids and naproxen. By age 3, Waylon was much better and the family thought the disease was gone, but by the following year his disease had flared and he couldn’t move. “We thought the disease was gone, so I didn’t realize what was causing his pain.” When she had to start giving Waylon a daily injection, she thought, “How am I going to give my baby a shot every day when I have never given anybody a shot.”
Waylon developed macrophage activation syndrome and needed a blood transfusion shortly after the injections began, but the injections kept him out of significant pain and were continued for about four years. “At that point, that’s what I cared about. I didn’t want him to hurt because he had hurt for so long while we were trying to get his medication worked out.” His rheumatologist, Dr. Terry Moore, at Saint Louis University Hospital, prescribed methotrexate, which had horrible side effects for Waylon. When tocilizumab was approved for children, Waylon starting receiving infusions every two weeks but currently needs them only once every two months. He is now in medical remission, runs track and plays on the basketball team.
Finding a New Normal
According to Ms. Wilder, the disease “has affected absolutely everything for our family. You try to find your new sense of normal, but it is constantly shifting because this is not a stagnant disease. We think we have found our baseline, and this is our new normal, but then, no, it has shifted again.” Walking to school, sitting through a movie, going to the beach are challenging, if not impossible, for Katherine and, therefore, for the family. “We constantly think, ‘OK, how is this going to work? How can we change things to make it more comfortable.’” Katherine is unable to attend school a full day, so it has been difficult for her mother to find a job that would give her the flexibility she needs for a schedule that requires taking her child out of school early or going to Boston for monthly appointments.
“There is so much that she misses out on. When she was in the process of getting diagnosed, she regressed enormously, stopped talking, stopped feeding herself, wouldn’t play with anybody. To watch that process—and not be able to help—was most difficult.”
But Katherine’s illness has also strengthened the family, Ms. Wilder says. Her two older brothers share a relationship with their sister that is incredibly supportive, she says. “They will give her the business and drive her nuts, but they couldn’t have a closer, more awesome relationship between the three of them. The boys have such an amazing understanding and awareness of life, of other kids. They have this peek behind the curtain of what people are going through that most kids and even most adults don’t get until some later point in their lives. It has made them so compassionate, so empathetic.”
For Denise, one of the hardest parts of her daughter’s illness has been the uncertainty that she is doing everything possible for her child. “I want to know why certain doctors pick certain medications,” she says. She reads online and talks to other parents whose children may have the same diagnosis as Emma but are being treated with different therapies. “Maybe there is no answer, but we wish there were more data or information regarding comparative outcomes for parents to read and understand.”
The uncertainty and fears that come in the middle of the night, along with a parent’s reluctance to “bother the doctor,” are common themes for these parents. Ms. Krauss hesitated when her son turned 3 and his disease was flaring to call the doctor, not realizing that she could have an “open line of communication” with his rheumatologist, that she should call him if there was new pain. Now she knows that she can call Dr. Moore at any time and he will always call back.
Ms. Krauss knows she is lucky with the insurance plan that she has, given the high cost of the infusions that Waylon needs, but she also knows that other parents are not so lucky. “Doctors have to realize the cost of these drugs. I hope there are options out there to help pay for these infusions, because if that’s what your child needs, you are going to do it whether you can afford groceries or not.”
Looking back at the early days of their children’s illnesses, these parents most remember pain, confusion and frustration, and say they could really have benefited from a crystal ball to prepare them for what was yet to come. They continue to search for information, and each has joined some type of network with people who understand their worries and questions, including the Arthritis Foundation; Pediatric Rheumatology Care and Outcomes Network or PR-COIN, a network of rheumatologists, nurses, therapists and parents; InspiRAtional Moms, initiated by Genentech to help raise awareness of the challenges of living with or care for a child with juvenile idiopathic arthritis; and social media outlets.
According to Ms. Wilder, parents need more written information, particularly at the beginning of their child’s diagnosis. “Just to have something in your hand, tangible, that can give you an idea of what it’s going to be like, that you can turn to when it’s 3:00 in the morning and you are flipping out.”
Doctors need to understand that parents are willing to try anything if they think it will work for their child. “Doctors believe that drugs are supposed to fix this stuff, but I wish they could keep their minds open to alternative treatments, anything that is going to help these kids,” Ms. Krauss says. “I wish they would be more open to explore those solutions to see if there may be a solution that would help.”
“There is a huge interest in alternative therapies,” Denise says, “as there is a lot of information out there suggesting that diet could affect this. In an ideal world, these alternative treatment options would be part of a rheumatology center so that parents wouldn’t have to struggle to find information and reputable practitioners to complement their child’s current treatment regime,” she says.
Doctor-Patient-Parent Relationship
These parents and their children have a close relationship with their child’s pediatric rheumatologist, a relationship that gives them “great support,” according to Ms. Krauss.
Ms. Wilder says her daughter “is super excited to see Dr. Kim every month, like a long lost best friend. She can be feeling horrible in the car, and then when we get there, she is all bubbly and excited.” Her daughter told her recently that she wishes everyone could have a Dr. Kim.
“For those of us who have been on this side of it for a while, we really appreciate the people who have chosen this field and who do this work and are the ones that will call us after they go home at night just to check and make sure that our kid is OK,” Ms. Wilder says.
“I know that this is their job and this is what they do, but I am guessing that 99% of them could have had a better-paying job, could have had a less stressful job, but they chose to do this work. We all really appreciate it and look for ways to be able to say that. They are very unique and special people who chose to put themselves in a very overworked, very underserved, very underappreciated job, but they are going to get in there and do it well,” she says.
Kathy L. Holliman, MEd, is a medical writer based in Beverly, Mass.
Sharing the Decision Making with Parents
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Parents dealing with their child’s juvenile arthritis can be confused about therapeutic options and why a certain therapy has been recommended instead of another. Ronald Laxer, MD, academic physician and physician administrator at The Hospital for Sick Children and the University of Toronto, says that even if the rheumatologist gives information about those options, parents can walk away confused. “The information may be said too quickly, it may not be clearly explained, only one parent attends the clinic and has trouble transmitting the information to the other, or the parent forgets to ask certain questions when they come in with their child.”
Use of a shared decision-making tool can help foster better understanding and communication and is an “important process to go through with the family,” he says.
PR-COIN (Pediatric Rheumatology Care & Outcomes Improvement Network), a learning network established in 2011 to foster collaboration among pediatrics centers and improve the outcome for patients and families with JIA, has developed the Arthritis Medication Choice Cards that can be downloaded and used in any office setting. The cards are designed to focus the conversation, with the family members and the patient deciding which topic to discuss first, second, third and so on.
Each card is a different topic related to medication choices, including how often different medications are given, potential side effects, how quickly medications have an effect, the cost and stopping the medications.
Research published in Pediatric Rheumatology in 2013 led to development of the decision-making cards for PR-COIN.1 The researchers found that clinicians use a variety of methods to inform families about therapy options and tailor the information according to what they perceive to be the family’s information needs, level of comprehension or mood. Because clinicians are concerned about overwhelming parents with too much information, many parents of children being treated for JIA “are left with persistent questions, information needs, long-lasting concerns and worry about treatment adverse effects.”
“When you sit down and talk about treatments using the shared decision-making tool, all the information is discussed, and together you make a decision. The families feel more engaged. When I use the decision-making cards, I find that the family is much more comfortable at the end of the conversation,” Dr. Laxer says.
Reference
- Lipstein EA, Brinkman WB, Sage J, et al. Understanding treatment decision making in juvenile idiopathic arthritis: A qualitative assessment. Pediatr Rheumatol Online J. 2013 Sep 30;11(1):34.
