Piper had a history of 3 months of right leg pain and achiness with slight knee swelling that went away with cold therapy. Piper had visited her pediatrician for the complaint of pain three times over the past 3 months, and her mom was instructed to give her ibuprofen at night for a diagnosis of growing pains. Piper injured her leg in a soccer game that initiated increased right knee swelling and pain that awakened her at 3:00 am. Piper’s mom noted increased swelling of the right thigh and immediately took her to a small tertiary hospital close to their home. An x-ray of her right femur and right knee was obtained and revealed an aggressive heterogeneous sclerotic and lucent lesion with moth-like appearance, measuring 10 cm within the right distal femoral diaphysis and metaphysis. The emergency physician contacted the children’s hospital Physician Consult Transfer Center (PCTC) that is available 24 hours a day for consulting services, and was connected with the oncologist on call. The oncologist on call instructed the physician to discharge Piper if her pain was well controlled and instructed them to not allow her to ambulate until she is evaluated by an orthopedic surgeon, and they would be contacted in the morning with a follow-up plan to see an orthopedic surgeon. The oncologist on call contacted the ONN via email with patient information and x-ray reports. The ONN obtained patient information through Piper’s electronic health record (EHR), obtained the images of the femur and knee x-rays, and reviewed them with the orthopedic oncology surgeon and pediatric sarcoma oncologist. An MRI of the right femur and a CT of the chest are ordered, and an appointment with the orthopedic oncology surgeon is scheduled for the next morning following NCCN guidelines for staging of the osteosarcoma patient .
The sarcoma ONN contacts the family, introduces herself, explains her role as their partner in their upcoming journey and that she will meet them in the radiology department prior to Piper’s tests, and gives the family her contact information to call with questions or concerns. She explains the MRI and chest CT and their implications and gives them directions to the radiology department and the orthopedic oncology surgeon’s office. The ONN does an initial screen for transportation needs and notes the family does not have any at this time. Part of the intake process for the ONN is getting to know the family up front and making a connection to help guide them through the most difficult time in their lives when their child is diagnosed with cancer. During their conversations, Piper’s mom is tearful and states they are all very scared, and that they have two other children to whom they don’t even know where to begin to explain to them about Piper’s condition. The ONN reviewed with mom that the hospital has a psychosocial team that will help them tell the siblings and Piper about her cancer in a developmentally appropriate way. Mom reports she is worried they have no insurance, she was recently laid off from her job, and she and dad have recently separated. Piper, mom, and her siblings have recently moved to a small apartment about 2 hours from the hospital, and dad has recently moved in with a new girlfriend. Childhood cancer is a family disease. It significantly affects the lives of the parents, their siblings, and the community . The ONN lets mom know that we have a team of financial counselors and a social worker that will help with obtaining healthcare coverage for Piper. The ONN acts as a new resource for the patient and family. During their conversation on the phone, she listens, debriefs, offers support, and provides information until their first appointment with the orthopedic oncology surgeon and the pediatric oncologist .
The next morning, the pediatric ONN meets Piper and her parents in the radiology waiting room. Piper is tearful and holding on to her favorite blanket. The ONN greets Piper with a hug and sits on the floor next to her chair and asks her about her favorite blanket. Piper states that her grandmother had made her the blanket when she was a baby, but her grandma was gone now because she had died with cancer in her lungs. The ONN helps Piper and her parents go back to have her chest CT completed, where mom and dad were visible during the testing and could calm and talk Piper through the procedure. When Piper arrived to MRI, she was greeted by a child life specialist who asked Piper to color at a small table with other children while she waited. Piper handed over her blanket to her mom and sat with the child life specialist.
The child life specialist then went back to MRI with Piper and helped her pick out a favorite movie she could watch while her MRI was completed, explaining everything to Piper as they prepared her for the MRI. The child life specialist stayed in the MRI environment with Piper to talk with her and keep her calm. Piper’s mom and dad were also able to talk with Piper throughout the MRI to help keep her still during the exam. Child life staff members are certified professionals who are prepared at the bachelor’s and master’s level in child life, child development, and other related fields. The child life specialists are a part of the healthcare team, and they contribute to minimizing fears and stresses experienced by children, adolescents, and families as related to healthcare experiences. They support the patient’s emotional, social, and cognitive growth in the context of his or her family, culture, or developmental age. They provide age-appropriate and safe play environments, recreational events, and activities that help to enhance a patient’s understanding of medical procedures and diagnosis using age-appropriate techniques .
The ONN meets the family at their appointment with the orthopedic oncology surgeon and the pediatric oncologist. The MRI is reviewed with the family, and the orthopedic oncologist discusses possibilities of cancer, including the diagnosis of osteosarcoma versus Ewing sarcoma due to the nature of the aggressive lesion seen on the MRI. A PET scan to complete the staging workup following NCCN guidelines is scheduled for the next morning prior to a biopsy of the tumor [12, 14]. The family requested to go home for the night and return instead of being admitted, so they can call extended family. The orthopedic oncology team arranges for the biopsy, and the ONN contacts the family with instructions and directions. The ONN contacts the research team with the possible new diagnosis of Ewing sarcoma or osteosarcoma for possible clinical trial options for Piper. The biopsy of a possible malignant bone sarcoma is the final most important planning procedure prior to initiation of treatment . Piper’s biopsy confirms her diagnosis of high-grade osteosarcoma. The ONN meets with the family in the surgery waiting consultation room with the orthopedic surgeon and the pediatric oncologist to relay the diagnosis of cancer to Piper’s mom and dad.
The oncologist reviews with Piper’s parents that her chest CT was negative for lung disease, and her PET CT scan only showed activity in her right leg; therefore Piper’s disease is considered localized. The pediatric oncologist explains osteosarcoma to Piper’s parents and discusses with them that pediatric bone tumors are rare but are the most common neoplasm in children with the most common of these bone tumors being osteosarcoma, with approximately 400 patients identified each year.
Bone cancers represent 5% of all cancers in children 5–9 years old, 11% in children 10–14 years old, and 8% in children 15–19 years old, and that osteosarcoma most commonly occurs in the long bones of the lower limbs . Piper’s mom tells the oncologist that her mother and sister have both died of cancer. Her mom died of lung cancer last year and her sister of breast cancer when she was 30. Mom expresses concern that it may be hereditary. The oncologist explains that in rare cases, osteosarcoma may be associated with predisposition syndromes such as Li-Fraumeni syndrome (p53 tumor suppressor germline mutation), Rothmund-Thomson syndrome (RECQL4 gene mutation), radiation exposure, hereditary retinoblastoma (mutation in the RB1 gene), osteochondroma, fibrous dysplasia, chronic osteomyelitis, Werner syndrome (loss of function mutation in WRN gene resulting in premature aging), and Bloom syndrome [14, 15]. The oncologist explains to Piper’s parents that a genetic workup by a geneticist will be completed to include a cancer-focused patient and family history.
The pediatric oncologist goes on to explain the standard therapy to treat Piper’s osteosarcoma combines chemotherapy and surgery that consists of a limb-sparing surgery or amputation. She goes on to explain the role of chemotherapy in stopping the cancer cells that may be proliferating systemically in Piper’s body and the need to stop these cancer cells from continuing to divide out of control and spread to other parts of her body. The chemotherapy includes up-front intensive treatments including cisplatin, doxorubicin, and methotrexate for 10 weeks. This will hopefully provide preoperative shrinkage and consolidation of the primary tumor, and facilitates the resection of the tumor and limb-sparing procedure . After recovery from surgery (usually 2 weeks), chemotherapy with cisplatin, doxorubicin, and methotrexate is continued for another 18 weeks and that treatment should begin within the next few days. She explains that clinical studies have shown that patients need to have both tumor resection and chemotherapy to have good outcomes . Piper will need a few more tests prior to starting her chemotherapy and will have a port placed to receive her chemotherapy.
After the oncologist and surgeon leave, the ONN sits with Piper’s mom and dad. Piper’s mom is distraught and states she feels “numb.” The physical and emotional care information that the family receives at the initial appointments can become very overwhelming. It’s the role of the ONN to assess absorption of information given at this initial appointment and continually update and reteach families about their child’s disease and treatment options . The ONN discusses the chemotherapy Piper will receive and the plan for Piper to get a port placed. The ONN has brought two teaching notebooks and reviews with mom and dad what a port looks like and how it’s accessed .
She explains the side effects of doxorubicin and the possible short-term or long-term effects of cardiomyopathy, and that Piper will need a baseline EKG and echocardiogram prior to her first dose, and then she will be monitored throughout her treatment regimen for any changes in her heart function . The ONN also explains the chemotherapy drug cisplatin, and that it can cause changes in Piper’s hearing and that she will need a baseline audiogram prior to receiving her first dose, and will have subsequent retesting throughout therapy to evaluate for hearing loss . The ONN lets Piper’s parents know that she will arrange for her audiogram, EKG and echocardiogram, and admission for a port placement and initiation of chemotherapy.
As the pediatric ONN goes back to the surgery recovery area with mom and dad, Piper is awake and crying. She asks her mom and dad immediately upon approaching if she is going to die. Developmentally, she understands the word cancer as death because it is associated with the loss of her grandma and aunt. The ONN and Piper’s parents explain to her that her cancer is different and there are medications she can receive to help kill the cancer. School-age children are capable of conceptual thinking in combination with concrete images. Through memory, they can navigate information about self, the problem, and an end goal. Before the age of formal operations, children at Piper’s age are not capable of abstract thought . Because most of the information about cancer and its treatment are abstract and foreign to children, the pediatric ONN must give explanations that are related to their world of experience and in simple concrete terms . Discussing the chemotherapy as the medicine to make the cancer go away is concrete and to the point for Piper.
When Piper returns for port placement and admission for chemotherapy, the ONN contacts the oncology child life specialist and asks her to go to the preop area to see Piper and show her what a port looks like with “chemo duck.” Chemo duck is a stuffed animal that has a port along with a dressing and tubing that can be flushed. Chemo duck has a book that talks about why he needs his port and how it gets accessed for his chemotherapy. Upon arrival to the preop area, Piper’s mom states that she told Piper her hair would fall out and she was concerned about what that would look like. The oncology child life specialist quickly ran back to her office and returned with Ella, a friend of Barbie who is fighting cancer, is bald, and has a few different wigs from which to choose. Piper is excited to open the box and immediately talks about which color wig Ella will wear. Play enhances the educational opportunities for children of all ages .
After arrival to the floor, the pediatric ONN meets with Piper and her parents, answering questions or concerns prior to her chemotherapy initiation. The ONN strives to initiate family-centered care regardless of Piper’s parents being divorced. She gives them both teaching notebooks and materials, and ensures that they both receive the information. The foundation of family-centered care is the understanding that the family is the true expert in the care of their child and the primary source of strength and support .
The pediatric ONN ensures consults are sent through the “new patient bundle,” which includes a fertility consult (to be completed prior to chemotherapy initiation), social work, psychology, palliative care team, recreation therapy specialist, child life specialist, art therapy, music therapy, massage therapy, pastoral care, genetics, and the school liaison.
The social worker evaluates for financial barriers and helps Piper’s parents apply for healthcare assistance and community resources. She helps mom and dad problem solve childcare as a team due to their recent divorce. Security was called in the surgery holding area when dad’s new girlfriend showed up. Guidelines for visitation were discussed and documented for dad’s girlfriend. The pediatric ONN and social worker collaborated with mom and dad to discuss boundaries of the girlfriend’s visitation to limit additional stress to Piper and the rest of the family.
Piper and her family met with the pediatric psychologist to assess for emotional functioning individually and as a family unit. Piper expressed concerns about the fighting and not wanting her parents to fight more since she was sick. Piper felt it was her fault that her parents were fighting. Piper practiced biofeedback to help her learn to calm herself when she felt anxious or upset about her parents or her cancer. The psychologist also met with Piper’s parents and her siblings to help in their adjustment of their new journey into the world of pediatric cancer. Piper’s siblings are both afraid that Piper is going to die. The ONN engages them in helping to hold Piper’s hand during access or counting during the cleaning of her hub prior to medications . By engaging them in Piper’s care, they find a way to cope and help their sister.
Piper’s mom is also worried about Piper not being able to attend school. The ONN assures mom that a consult was initiated to the STAR program. The STAR program has a schoolteacher on the oncology floor to help patients stay on top of their schoolwork. The school liaison meets with mom and Piper and explains that she will communicate all information to Piper’s school and work on getting her a tutor. The ONN encourages mom to contact the school to find out if her teacher will allow Piper to Skype into the classroom weekly to stay connected to her peers. The school liaison will also arrange to visit Piper’s school and explain to the children in Piper’s classroom that Piper has cancer, and she will leave a bear and backpack on Piper’s chair in the classroom while she is absent and going through treatment.
On the second day of Piper’s admission, the ONN found Piper in the playroom, playing games with other pediatric cancer patients and the recreation therapist.
Piper is engaged and laughing. She reports she was feeling a “little yucky” this morning, but she didn’t want to miss play group. Piper has experienced art therapy where she said she got to “hang out” with Mary, the art therapist. During her art therapy session with the art therapist, Piper not only created an art project, but she ended up expressing her fears and concerns about being made fun of when she loses her hair . Piper also enjoys music therapy when she gets to learn to play the drums or guitar. She verbalized it makes her laugh when her dad covers his ears, and that making loud music is fun. Music and art therapy are effective in promoting socialization by increasing interaction, verbalization, independence, and cooperation. It can enhance the patient’s relationship with hospital staff in developing a sense of trust and decrease isolation during the hospital stay [22, 23]. To help control Piper’s nausea and vomiting, massage therapy has been consulted. Massage therapy helps control chemotherapy-induced nausea and vomiting . It also helps patients relax. The ONN also gives Piper’s mom and dad a tour of the parent resource center where they can go to relax, exercise, get a haircut, and even get a free massage twice a week.
The sarcoma team collaborates with the palliative care physician and a physical therapist who meets the osteosarcoma patients at initial diagnosis to start preparing them for their upcoming surgeries, while the palliative care physician works with the oncology team to manage Piper’s side effects [25, 26]. These members of the team will stay with the patient from diagnosis through their surgical intervention and after therapy to help them regain their precancer state.
Palliative care helps prepare the patient for their upcoming limb-sparing surgery or amputation by prescribing gabapentin 2–3 weeks prior to their surgery and then escalates doses during the acute phase . Piper’s mom had also requested a referral to integrative medicine to review other therapies they could use. The pediatric institution does not have an integrative medicine practice, and when the ONN contacted the collaborating adult hospital, they would not take Piper as a patient due to her age. The ONN contacted the palliative care physician and nutritionist to see mom jointly to discuss aspects of nutrition and other modalities of care. The physical therapist works with the patient and family, regaining strength and as much functionality as possible prior to surgery. The same physical therapist sees them the day after their surgery to start working on rehabilitation with a goal to achieve their highest functional status to permit them to return to their role in society and hence enjoy dignity and improved quality of life .
Piper and her family have a lot of questions about the limb-sparing surgery, including limb salvage, rotationplasty, and amputation. With the consent of Piper and her family, the ONN arranges for patients and their parents who were treated for osteosarcoma and had a limb salvage, rotationplasty, or amputation to meet Piper and her family. After meeting these three families, Piper decided she wanted to have the rotationplasty surgical procedure. This procedure involves resection of the lesion in accordance with good cancer surgery guidelines, and then a rotationplasty of the Van Nes type is performed. The ankle then functions as a knee joint, and the final result is a below-knee amputation rather than a high above-knee amputation with functional results . After Piper decides on the rotationplasty surgery, the ONN arranges for her to meet another 10-year-old osteosarcoma survivor who has the rotationplasty and is very active and plays basketball and softball.
During Piper’s first hospital stay, her bedside nurse contacted the ONN to let her know that Piper was refusing to take her oral medications. Mom also reported she had never really had to take pills prior to her diagnosis, so she was not surprised. The ONN contacts the child life specialist and requests they work on pill swallowing with Piper during her admission. The child life specialist has a “Mario” game of pill swallowing where the candy pills are of varying sizes, and at each level, Piper gets a prize. Prior to discharge Piper was at level 3 of the 5 levels, and was very excited for her next return to the hospital to work on getting to level 5 of the pill swallowing game.
The ONN met with both mom and dad and completed discharge education including signs and symptoms of anemia, neutropenia, and thrombocytopenia; who and when to call with questions, concerns, or fevers greater than 100.5 around the clock; mouth care regimen; tips for controlling nausea or vomiting; and treatment for constipation. The ONN also reviewed the treatment calendar and upcoming schema. The ONN picked up the prescriptions from the pharmacy and had the parents write both the generic and brand name on the medication bottles; then she reviewed each medication’s dosage, purpose, schedule, and side effects. She had both parents repeat back the medication list. Lastly, the ONN had both parents practice taking Piper’s temperature with a Tempa•Dot. Parents of newly diagnosed pediatric cancer patients require specialized education in order to care for their child with a newly diagnosed cancer . The Children’s Oncology Group Family Handbook for Children with Cancer is utilized as the teaching guide for newly diagnosed pediatric cancer patients . It is essential that families understand the basics of their child’s disease, what to expect from their chemotherapy and biotherapy, how to recognize adverse side effects, what is considered an emergency, and how to seek help .
Upon discharge, the ONN takes Piper and her family to the outpatient Hematology/Oncology Clinic and the Outpatient Hematology/Oncology Infusion Center for a tour. The ONN reviews what to expect during her next clinic visit and chemotherapy admission. Piper becomes anxious and begins to cry when discussing having her port accessed. The ONN takes Piper to the treatment room and allows her to place lidocaine cream on both her “chemo duck” and Ella, Barbie’s friend with cancer. Prior to Piper’s first return to the clinic, the ONN contacts the child life specialist and Piper’s psychologist and lets them know when Piper is coming, so they can be present to help support and distract Piper during her first port access. The ONN communicates patient information through all team members to provide seamless care for the patient and family. This enhanced communication among healthcare providers builds relationships within the team and directly enhances the patient experience .
At Piper’s first clinic visit to have a physical exam and labs drawn, the ONN reviewed Piper’s lab results with mom and dad, including how to calculate the absolute neutrophil count (ANC). She discussed the possibility of needing blood or platelet transfusions between her chemotherapy admissions. Piper’s mom verbalized to the ONN that she is concerned about the distance they will be driving, but she does not want to have Piper treated anywhere else. The ONN discusses the possibility of mom taking Piper to an outside cancer clinic in their area for transfusions only. The ONN collaborates with a local outpatient clinic and arranges for orders for transfusion to be sent to the clinic. When Piper required blood or platelet transfusions, she was able to stay closer to home and not travel 2 hours to the pediatric institution. The social worker also provided transportation resources, including gas cards and mileage reimbursement to the family.