Case Presentation

Maria, a four-year-old Latina female with familial cardiomyopathy and severe biventricular dysfunction, was admitted to the hospital for worsening heart failure symptoms and placed on the heart transplant wait list. Maria’s parents brought her to the United States for this lifesaving procedure as transplantation is not available in her country of origin. During her intense and prolonged five-month hospitalization, she experienced many typical medical complications that are known to arise in these critically ill patients, including three cardiac arrests and subsequent requirement of a Berlin heart placement (an external temporary ventricular assist device that takes over the normal heart function by pumping blood directly to the pulmonary artery and into the lungs) that helped to keep Maria alive while she waited for a donor heart to become available. Subsequent medical complications included a subdural hematoma secondary to anticoagulation treatment necessary while being on the Berlin heart and ultimately receiving a heart transplant after a grueling six-month waiting period. Initial consultation to the transplant psychologist was for assessment and management of significant anxiety and to provide emotional/behavioral support to the patient and family during expected prolonged admission.

Treatment Description

Patient

Maria’s mood and behavior was remarkably deviated from her baseline functioning outside of the hospital setting. She initially presented as timid, not making eye contact or speaking to providers, with significantly decreased interest in previously enjoyed activities, not eating, and scared/anxious of the hospital setting (i.e., hypervigilant to IV/pump alarms, distrustful of medical staff, difficulty cooperating with vitals/ medical procedures, picking at lips/fingers to the point of bleeding, crying/ screaming when separated from parents, and low frustration tolerance). There were also language and cultural barriers that interfered with her adjustment.

Many behavioral interventions were extremely useful to Maria throughout her hospitalization, despite the complexity of her medical condition and treatment. As often helpful for preschool-aged children, Maria immediately coped more effectively with a daily schedule/structure of events to help her get back into a routine, which helped provide her with more predictability and decreased hypervigilance of staff coming into her room. Implementing medical play, including more novel play strategies, such as medical water play and medical collages, also helped Maria build rapport with staff and aided in desensitizing her to medical equipment, giving her increased empowerment and control over her medical experience, and providing an outlet for her worries and fears in a developmentally appropriate manner. Using finger puppets, gloves, and stress balls as well as squeeze/pull toys aided in diminishing skin picking behaviors.

As Maria required further cooperation with medical interventions, a token economy system was implemented to reward Maria’s desired behaviors, including engaging in daily self-care activities, allowing pump checks, dressing changes, and ambulating post Berlin heart placement. Distracting strategies also helped Maria get used to this bulky external device, including having many “treasure hunts” on the medical floor to aid in ambulating and maneuvering pumps and hoses post Berlin heart placement. Including systematic desensitization strategies to assist Maria in adjusting to the big, heavy, and noisy Berlin heart machine was useful, as was personifying it by painting on a face and nicknaming it “Ike.” Alternatively, as Maria’s physical status and her functioning improved, a reverse time out technique was implemented in her hospital room to provide a consequence and decrease the parental/staff attention she received for undesirable behaviors (i.e., tantruming/hitting).

To help Maria more appropriately express her frustrations a feeling faces game was used during which she could visually pick out what emotion(s) she was feeling at the moment. Additionally, Maria engaged in relaxation training with staff/parental modeling to help her cope with medical procedures, including receiving developmentally appropriate information shortly prior to procedures, watching on her doll what the procedure would entail, and developing a coping plan (e.g., breathing away the pain, holding mother’s hand, watching favorite DVD for distraction, etc.).

Other interventions implemented throughout her stay included helping with separation anxiety. This was accomplished by successive approximations to goal, including having parent in hospital room, but slightly further away from patient/clinician each time, and eventually out of the room with increasing duration and frequency. A timer was used to count down to parental return and Maria was distracted with therapeutic games during parental absence with reward provided upon parental return. Use of shaping strategies were also utilized pre- and post-transplant to teach pill swallow training as Maria was to return to her native country after her heart transplant where immunosuppressant medication is not available in a liquid format.

Family

As this family was geographically separated due to seeking life-saving medical care for Maria that was not available in their country of origin, family interventions were also indicated. Modern technological advances, including Skype, were useful in keeping family members connected to one another and being able to attend team meetings in a virtual format. Voice banking was also useful for father to pre-record Maria’s favorite songs and read her bedtime stories which helped soothe her throughout her hospital stay.

A corner stone of psychology and working with patients/families includes a heavy dose of rapport building, without which many interventions and mental health advice would not come to fruition. Psychoeducation may also be overlooked, but it is a powerful tool, especially for parents of medically ill children. This can provide a foundation for parents to discuss with a pediatric psychologist child-rearing practices and how to foster healthy development, adjustment, and mental wellbeing in the uncommon setting of raising their child in a hospital for a prolonged period of time. For Maria, discussions took place regarding typical behavioral regression seen during stressful experiences and hospitalizations, the need for developmentally appropriate self-soothing comfort items throughout her hospital stay, how to wean her from her pacifier as her fifth birthday approached, and ongoing toilet training.

Discussions with Maria’s parents included how to divulge information to Maria and her siblings about her medical condition, as well as the need for the Berlin heart and heart transplant. Maria’s parents told her that her own heart was not working well and that these “pumpies” were going to help her heart work until she received a new one. Parents are typically advised to give simple and clear developmentally appropriate explanations, follow their child’s lead, and provide honest answers to their child’s questions.

Further parental interventions also included discussions and support surrounding their own trauma history after losing a child at 5-months-old with the same diagnosis as Maria and feeling guilt/remorse for not seeking medical care outside of their country, witnessing Maria arrest and be resuscitated a number of times, as well as processing their fears given the many medical complications while awaiting transplant.

Medical Team/Environmental

Given Maria’s many transitions in a short period of time it was recommended that she have a consistent nursing team to foster her attachment to medical providers. Nurses were matched based on their cultural background, language, and calm demeanor. Staff were also encouraged to have positive playful interactions with Maria outside of their routine cares which progressed to daily “dance parties” with staff members as well as letting her troop of stuffed animals earn honorary M.D.s and help check her Berlin heart pump heads. With the help of Child Life, Maria’s hospital room was also transformed and decorated similarly to her room in her native country, with a play mat on the floor, height appropriate table/chairs, and many family pictures.

Commentary

Solid organ transplant cases are frequently multifaceted, as by the time a child requires a transplant most every other treatment option has been exhausted and transplantation is necessary to prolong life. Despite the medical and psychological complexity of working with solid organ transplant patients, cases like Maria’s are extremely rewarding because every bit of psychological training comes into play as one works with the actual patient, their family members, and the medical team. It is gratifying to know that in such challenging and sometimes seemingly insurmountable high-stress situations, pediatric psychologists can make a huge impact in the psychological adjustment and wellbeing of these patients and families.

About the Author

By Melisa Oliva, PsyD
Boston Children's Hospital
Pediatric Transplant Center & Department of Psychiatry