Working as a family nurse case manager for active duty military families is fulfilling and rewarding. Military families often juggle healthcare issues when the service member is away on a deployment or mission. Emphasis of military family case management is on meeting the healthcare needs of the family to ensure the well-being and readiness of the active duty service member. The healthcare concerns may be as simple as making a medical appointment to having a complex diagnosis that needs professional case management. I find my role most satisfying when dealing with pediatric situations that become a learning experience. This article describes a military child and family’s journey that fostered my growth and knowledge as a family nurse case manager.
Ella was born with a rare congenital malformation known as proximal femoral focal deficiency (PFFD). Proximal femoral focal deficiency (PFFD) is an extremely rare congenital anomaly with an incidence of 1.1-2.0 in 100,000 live births (Uduma et al., 2020). The condition is characterized by failure of normal development of the proximal femur and hip joint (Westbury & Davids, 2018). The cause of PFFD is unknown, but some studies have reported a possible disturbance during prenatal development, which may have come from an outside blunt trauma, infection, or exposure to toxins or chemicals. PFFD is considered non-hereditary and usually diagnosed prenatally with radiographic images or at birth with the appearance of classic PFFD signs. The classic appearance of the affected side is a shortened, sizeable thigh. The lower limb is generally fixed, abducted and externally rotated. The consequence of having a lower limb deficiency is an impaired childhood growth, abnormal gait, cosmetic implications and psychosocial behavioral changes (Uduma et al., 2020).
PFFD is a challenging orthopedic condition that requires the collaboration of pediatric orthopedic surgeons, nurses, prosthetic experts, and physical therapists. The aim of treatment is to achieve symmetrical leg length and to optimize the ability to ambulate. The severity of the leg discrepancy and presence or absence of a functional foot and ankle helps determine treatment options. Surgical reconstruction, orthotics or prosthetics, and limb lengthening with hip stabilization are various treatment alternatives for a child with PFFD. Ella’s parents consulted with eight orthopedic specialists to discuss the appropriate treatment option that would provide Ella with the best long-term functional outcome. Ella was born with an undeveloped hip and significantly shortened femur, which is deemed a more severe form of PFFD. A surgical reconstruction, rotationplasty, was recommended by each of the orthopedic specialists as a treatment option. After lengthy considerations, Ella’s parents decided their daughter would have the rotationplasty.
A rotationplasty is a functional surgical procedure involving the resection of the affected bone while rotating the limb 180 degrees and reattaching it to form a knee joint. The outward presentation is a short leg with the foot on backwards. The foot and ankle assume the function of a knee joint, allowing the attachment of a purposeful and customized below-the-knee prosthetic.
Ideally, a rotationplasty is performed on children between the ages of two and four. The timing of early intervention allows ample time for bone growth throughout childhood. Ella’s parents were convinced that the procedure would greatly improve Ella’s quality of life plus the ability to maximize her performance and participation in functional activities.
When I first met Ella, I quickly noticed her infectious smile and charming giggle. She has a strong affinity for Disney princesses and cheerful melodies. It was easy to overlook the supportive leg brace and slight limp as she continually strived to keep up with her older brother and gave the impression that she had no physical limitations. Ella’s parents confirmed that she is a smart, joyful and energetic child who loves to play, sing, and dance. Ella demonstrated many behavioral skills appropriate for a 2-year-old. Two year olds are able to follow simple instructions, use simple phrases, show increasing independence, and play simple make-believe games.
Naming her affected leg “Leggy” clearly demonstrated her active imagination. She showed a willingness to learn, the ability to think and express, and interest in exploration. Ella appeared content, easily satisfied and happy when surrounded by her family and all things that sparkled her joy.
Ella and her family were opened to case management services 10 months before the surgery. An assessment of the parents’ concerns surrounding the rotationplasty helped guide the development of an appropriate case management care plan. Keeping the short-term goal of a successful surgery in mind, Ella’s parents were proactive in determining potential challenges surrounding the surgical procedure. They expressed concerns with insurance coverage, transportation and lodging, supplemental costs for equipment, childcare for brother Jack and accommodations for Ella. The uncertainty of outcome, the invasiveness of the rotationplasty and the stress of hospitalization were identified stressors for them. According to DeMaso & Snell (2013), parental distress can interfere with parents’ ability to respond to the emotional needs of their child, with their ability to help their child generate effective coping strategies, and both immediate and long-term outcomes. Ella’s parents understood that providing Ella with age appropriate care and delivery of information along with quality family interactions were strongly recommended for the long-term journey. Ella’s coping and adjustment would be impacted by her behavioral stage of development. Ella may show signs of regression or act like an infant with increased stress or anxiety. It is also common at her age to fear strangers, feel a loss of control, be afraid of medical supplies or equipment attached to her, and only think of herself. Incorporating play with every interaction would be well received by Ella, who is only concerned with her own thoughts. Essential interventions to help Ella cope with the hospitalization, surgery and recovery process include being honest, providing opportunities for choice and control and encouraging autonomy.
An essential element was psychological readiness for Ella and her family. As a military family, they are expected to adapt to new situations and be resilient to constant change that aligns with the duties of the service member. The possibility of Ella’s father being called to deployment or military assignment added to the worries of a pending surgery. Children and families often experience an increase in stress with a parental deployment (Pexton, Farrants & Yule, 2017). A plan was implemented to engage them with available military family resources, particularly FOCUS (Families Overcoming Under Stress), which is aimed at helping families build on current strengths, enhance communication and strategically problem solve. The family’s strong social support system played a key role in surgical preparedness. Organizing a timeline of events, on a calendar, assisted Ella’s parents to identify opportunities for assistance from family, neighbors, and close friends for childcare, meal assistance and light household tasks. Allowing Ella to stay connected with her teachers and peers offered a distraction and lessened any fears or anxiety about the surgery.
Addressing financial concerns and reviewing Ella’s insurance coverage was another critical task. Ella was scheduled to have her surgery out of state, which required foresight in arranging transportation and lodging, childcare, and the transition of care. Ella’s parents had decided on the orthopedic specialist that was the best match for Ella without fully understanding the insurance costs and fees. An explanation of the cost savings, utilizing network providers and services, provided a better understanding of their out-of-pocket responsibility. Ella’s parents acknowledged available financial resources offered to military families, like NMCRS (Navy Marine Corps Relief Society), which provides free and confidential financial counseling and interest-free loans or grants to service members having trouble meeting urgent financial needs. Advocacy for the family with the insurance company and benevolent organizations alleviated some financial worry and strain.
The drive to the hospital was a four-hour car ride. Ella’s grandparents flew into town to assist with caring for Ella’s brother. Securing accommodations was uncomplicated as the medical institution was affiliated with a lodging facility conveniently located nearby, that offered services to patients and families receiving care at the hospital. With advanced planning, Ella’s parents were able to reserve a room for their extended pre and postoperative stay. Considering Ella’s long separation from home, Ella’s parents remembered to transport a few Ella’s prized possessions that would offer her a comforting distraction from the surgery. Considering the ride back home post-discharge, Ella’s parents received assistance with obtaining a specialized car seat and customized wheelchair. The opportunity to plan ahead financially, strategically, and psychosocially, allowed Ella’s parents to effectively focus on Ella’s rotationplasty and surgical recovery.
Upon Ella’s return to the local area, case management services continued to ensure that initial challenges were overcome and complications were addressed. As a military dependent child, it was mandatory to enroll Ella in EFMP (Exceptional Family Member Program). EFMP is a military program directed at offering comprehensive and coordinated community support and resources to military families with special needs. Additional case management interventions for an effective transition of care include:
- facilitation of physical therapy and occupational therapy services
- psychosocial support, through formal and informal providers, for the child and family
- educating teachers, classmates, and caregivers
- utilization of appropriate, supportive local and military family resources.
The professional case manager plays a vital role in improving care transitions by advocating for the individual, facilitating collaboration among care providers and ensuring that communication is shared among all stakeholders and across care settings (Campagna, Nelson & Krsnak, 2019).
Ella’s parents yearn for their daughter to have improvements in her quality of life, optimal functional performance, and maximal participation in an active lifestyle. They recognize that Ella may continue to face ups and downs with PFFD as she grows and develops. The reliance on a multidisciplinary team to offer guidance in the therapeutic management of her congenital femoral deficiency will continue throughout her medical journey. Professional case managers help Ella achieve the best quality outcomes since case managers are strong advocates and supporters of patient needs. The knowledge that I gained about the case management process from Ella’s unique and fascinating condition is useful feedback for delivering quality care to future pediatric patients of active duty service members. Ella’s positive disposition and strong family foundation proved their significance in her ability to remain resilient through the hospitalization and surgical process.
EPILOGUE
Ella became a big sister during the recovery process of her rotationplasty. She and her brother, Jack, welcomed baby brother Max. Ella’s father was able to avoid a deployment during the whole surgical process and is facing retirement this year from the United States Navy. Ella was finally able to put on a pair of rain boots with her modified “Leggy.” She uses the bathroom without assistance. Recently, Ella celebrated the one year anniversary of the rotationplasty. Next school year, Ella will be starting in a new school as the family is re-locating. Ella’s ability to cope with PFFD and the new adjustments in life will continue to evolve as she matures and develops. As a military child, Ella naturally acquired a sense of resiliency and adaptability, which are strong attributes that she can build upon to overcome potential physical and psychosocial challenges related to her PFFD.
REFERENCES
Uduma, F. U., Dim, W. M., & Njeze, N. R. (2020). Proximal femoral focal deficiency — a rare congenital entity: two case reports and a review of the literature. Journal of Medical Case Reports, 14(27). https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-020-2350-y
Westbury, D. E. & Davids, J. R. (2018). Proximal Focal Femoral Deficiency (PFFD): Management Options and Controversies. HIP International. 19(6). https://doi.org/10.1177/112070000901906s05
Doger, e., Kopuk, S. Y., Cakiroglu, Y., Cakir, O. & Yucesoy, G. (2013). Postnatal appearance of the thigh. Short externally rotated thigh was shown in postnatal appearance of fetus [Photograph]. Retrieved from https://www.hindawi.com/journals/criog/2013/637904/#case
Wick, J. M. & Alexander, K. M. (2006). An illustration of the portion of the femur excised in a patient with proximal femoral focal deficiency [Illustration]. Retrieved from https://pubmed.ncbi.nlm.nih.gov/16927586
Demaso, D. R. & Snell, C. (2013). Promoting copingin children facing pediatric surgery. Seminars in Pediatric Surgery 22(3) 134-138. https://doi.org/10.1053/j.sempedsurg.2013.04.004
Pexton, S., Farrants, J., & Yule, W. (2018). The impact of fathers’ military deployment on child adjustment. The support needs of primary school children and their families separated during active military service: A pilot study. Clinical Child Psychology and Psychiatry, 23(1), 110–124. https://doi.org/10.1177/1359104517724494
Campagna, Vivian, MSN, RN-BC, Nelson, Sheila, MSN, RN, Krsnak, Jean & MBA, RN. (2019). Improving Care Transitions to Drive Patient Outcomes: The Triple Aim Meets the Four Pillars. Professional Case Management, 24, 297-305. https://doi.org/10.1097/NCM.0000000000000387
