Introduction
The scenario is dedicated to A, a 15-year-old-boy who has been diagnosed with Ewing Sarcoma on his left leg and is scheduled for an amputation. He expressed his worries about the treatment and his near future. Within the case given, it is crucial to prepare A for the treatment and demonstrate significant nursing management. Initially, it seems reasonable to define the essentials of Ewing Sarcoma.
Ewing’s sarcomas have a monomorphic histological structure, represented by densely spaced small cells with a rounded nucleus, blurred outlines of the cytoplasm, and indistinct nucleoli. In most cases, fibrous layers are visible, dividing the tumor into strips and irregularly shaped lobules. Mitoses are rare; foci of hemorrhage and necrosis are common (Ludwig et al., 2021). According to the results of light microscopy, Ewing’s sarcomas are referred to as the so-called group of “small round cell tumors”, which morphologically puts it on a par with alveolar rhabdomyosarcoma, low-grade neuroblastoma, some lymphomas, etc.
The impossibility of a purely morphologically confident differential diagnosis dictates the need for an integrated approach to pathomorphological examination in the case of suspected Ewing’s sarcoma. To determine the histogenetic affiliation of Ewing Sarcoma, an integrated approach is used, including light microscopy, immunohistochemical and cytogenetic studies, and tissue culture. A unique feature of Ewing Sarcoma is a special chromosomal anomaly – a reciprocal translocation of the 11th and 22nd chromosomes, capturing the q24 and q12 bands of both chromosomes, t (11; 22) (q24; q12), with damage to the EWS and FLY1 genes (Sbaraglia et al., 2020).
The below discussion will focus on the opportunities for treatment of Ewing Sarcoma, as well as exploring a nursing management plan for A to deal with the circumstances. It will be important to provide a developed plan for the post-amputation period and determine a nurse’s role within it. A thorough explanation of particular actions for A will be critical for him to attain mental strength, confidence, and readiness for the future.
Treatment
Ultrasound, angiography, MRI, and radioisotope scanning allow not only to determine the true intramedullary and soft tissue spread of the tumor process, as well as the presence of metastatic foci but also provide an opportunity to dynamically monitor the effectiveness of treatment. Previous studies indicate the high information content of PET both at the stage of primary diagnosis and the subsequent assessment of the pathomorphological effect of PCT (Gargallo et al., 2020). In this case, the lack of positive dynamics may be the basis for changing the treatment regimen or preoperative radiation therapy. Prior to any treatment, morphological confirmation of Ewing Sarcoma should be obtained by trephine biopsy, followed by histological and/or immunohistochemical, molecular genetic, and/or cytological studies. If necessary, the morphological conclusion is confirmed by the results of an open biopsy.
An important step in the treatment of Ewing’s Sarcoma is a local effect, including radiation and/or surgery. The frequency of local recurrences varies from 10% with surgical control of the primary tumor to 26% with inoperable forms and depends on the volume of the tumor, its operability, type, and intensity of exposure (Sole et al., 2021). According to Italiano et al. (2020), Ewing Sarcoma is characterized by high radio sensitivity. As a rule, radiation therapy is an integral component of the combined treatment of inoperable formations or non-radically removed tumors, reducing the risk of recurrence. The effectiveness of radiation exposure directly depends on the accuracy of determining the area of tumor lesions of the bone and soft tissues. The main method for assessing the irradiation field is MRI (Morales et al., 2021). There are reports in the literature of improved local tumor control after hyper-fractional irradiation. This mode is used in patients with inoperable tumors, for example, with lesions of the pelvic bones, as well as after non-radical operations.
Radiation therapy in children has its own characteristics, ignoring which reduces the effectiveness of exposure and causes severe radiation damage. This is, first of all, the continued growth of tissues and the presence of growth zones in the bones, as well as differences in the radio sensitivity of healthy tissues and tumors and the rapid growth of neoplasms (Gartrell & Rodriguez-Galindo, 2021). In the case of A, this aspect cannot be neglected and must be taken into account when developing a specific treatment plan.
Intensive induction therapy followed by consolidation and adequate radiation therapy, despite high toxicity, remains the only way to overcome adverse prognostic factors. Among them, one can single out massive widespread pelvic tumors and the presence of metastases at the time of diagnosis (Knott & Cidre-Aranaz, 2021). The toxicity of induction chemotherapy can be overcome by competent modern accompanying, transfusion, and antibiotic therapy. At the same time, it is necessary to ensure close and round-the-clock monitoring of patients in a hospital, especially in the phase of aplasia of hematopoiesis.
Patients with a good response to induction therapy, despite the primary prevalence, tumor dissemination, and age, can expect a 50% five-year survival, regardless of the type of consolidation performed (Kondo, 2019). The choice of the type of consolidation depends, first of all, on the readiness of the medical institution and the experience of performing autologous transplantations among the staff, the presence of somatic and infectious pathology in the patient, and other risk factors.
Currently, the results of the treatment of Ewing Sarcoma remain unsatisfactory. To improve them, research has begun in a new direction based on the biological characteristics of tumor cells. It is known that tumor markers reflect its main properties, including proliferative capacity, neoangiogenesis, propensity for invasion, metastasis, and apoptosis, as well as dependence on exogenous and endogenous regulators.
Nursing Management
The problem of rehabilitation of persons who have undergone limb amputation is one of the most difficult. Restoration of functions lost as a result of limb amputation has not only medical and social but also economic significance. The main problems faced by persons who have undergone amputation are as follows. First, it is limb prosthetics with the maximum possible restoration of physical activity. Second, it is the need to correct phantom pain syndrome and mental disorders. Third, it is the compensation for the main disease that led to amputation. Fourth, it is social adaptation and rehabilitation (Parnell & Urton, 2021). The theoretical foundations of generally accepted methods of rehabilitation of persons with limb amputations have been studied at a sufficient level. These include, first of all, various forms of therapeutic physical culture, physiotherapy, and massage. However, in the practical solution of the issue, there is a significant lag behind the theory. All patients who underwent amputation, in addition to physical mutilation, develop various mental disorders, and only some of them seek specialized help. Psychological support is usually provided by nurses and does not have a specialized nature.
When the lower limbs are amputated, the statics of the body is significantly disturbed, and the center of gravity moves towards the preserved limb. The consequence of this is the tilt of the pelvis, which leads to curvature of the spine and increased load on the preserved limb (Parnell & Urton, 2021). Atrophy of the muscles of the stump is also observed. After the amputation of the lower limb, physical rehabilitation is used in three periods: early postoperative (from the day of the operation to the removal of sutures), the period of preparation for prosthetics (from the day of suture removal until receiving a permanent prosthesis); while mastering the prosthesis. Therapeutic gymnastics is started on the first day after the operation (Ülger et al., 2018). The classes include breathing exercises, exercises for the healthy limb, isometric exercises for the preserved segments of the amputated limb and truncated muscles, and trunk movements from days 2-3. On the 5th-6th day, phantom-impulse gymnastics is used, which is very important for the prevention of muscle atrophy of the stump and is performed during the life of the amputee.
For A, in the early postoperative period, physical therapy should be used against the background of drug therapy (breathing exercises, exercises for a healthy limb) to reduce inflammation at the amputation site and reduce swelling. Early activation of the patient will begin on the 2nd-3rd day: passive, active-passive movements, sitting, independent exercise, and isometric tension of the preserved segments of the amputated limb will be applied. Physiotherapy procedures will include back massage for 3-4 days; 5–6 – phantom-impulse gymnastics for the prevention of atrophy of the muscles of the stump (Webster et al., 2019). Before the final formation of the stump, a temporary prosthesis will be used for 1-2 months for adaptation to daily physical activity.
Physical therapy classes will continue with a small group and independent methods. Massage of the stump and preserved limb will be offered. The massage of the stump will be performed with the utmost care, as incorrect execution of the techniques can lead to increased sensitivity of the stump. In the case of diabetes or obliterating atherosclerosis, the treatment of the underlying disease will remain extremely important.
After the stitches are removed, they begin preparing the stump for prosthetics. The lesson is aimed at ensuring that the stump can be supportive and have the maximum range of motion, its muscles must have the ability to contract, and the strength of the contractions must be as large as possible. For this, mobility is restored in the preserved joints of the amputated limb. When reducing pain and increasing mobility in the preserved joints, the classes include exercises for the muscles of the stump, which contribute to the formation of the correct shape of the stump: active exercises first with the support of the stump, and then independently and with the support of the instructor’s hands, exercises to reproduce the amplitude of movements.
To prevent curvature of the spine, general strengthening, corrective exercises, and exercises from increasing the strength and endurance of the muscles of the upper shoulder girdle are used. Standing and walking exercises are started on crutches 2 to 4 weeks after surgery, while balance exercises are used (Escamilla-Nunez et al, 2020). In the future, for faster rehabilitation of the patient, a temporary prosthesis can be used until the final formation of the stump. After that, they proceed to use a permanent prosthesis of a different design.
Mastering the prosthesis includes three stages: the first stage is learning to stand with equal support on both limbs and transferring body weight in the frontal plane. The second stage is teaching the transfer of body weight in the sagittal plane, carrying out training of the support and transfer phases of the step with the prosthetic and preserved limb. The third stage is mastering the skills of uniform step movements. In the future, it will be walking on an inclined plane, turns, on stairs, on rough terrain, and elements of sports games.
Massage is prescribed for 7-10 days after the operation, provided that the healing of the wound is favorable, to reduce muscle tone. After the wound has healed and surgical sutures have been removed, the stump is massaged to prepare it for prosthetics. Among the physiotherapeutic procedures used to relieve phantom pain are light therapy, electrical stimulation, darsonvalization, electrophoresis of iodine, mud in the part of the stump, and general baths (Parnell & Urton, 2021). After 2-3 days after the inflammatory phenomena have subsided, they proceed to thermal procedures – baths (radon, coniferous, hydrogen sulfide).
Hence, in line with care goals, nursing interventions will focus on maintaining correct posture to prevent musculoskeletal complications. Further, it is the maintenance of muscle strength and joint mobility, which is facilitated by the active implementation of necessary daily activities, exercises for the range of motion, and special exercises prescribed by a specialist doctor (Parnell & Urton, 2021). Nursing interventions will also be aimed at maintaining independence when moving, assisting with moving (moving and turning the patient in bed, moving the patient from bed to chair, to a wheelchair, to a wheelchair, supporting the patient when walking, handling a falling and fallen patient). At the same time, a necessary condition is to ensure the safety of the nurse when moving the patient (wheelchairs, wheelchairs, and beds must be fixed before the patient is moved, and their height must be the same).
Moreover, in preparation for walking, the beginning of walking using crutches or a cane will be implemented. This will take place in conjunction with a specialist doctor, and the corresponding muscles are strengthened by performing exercises, selecting an auxiliary device, teaching its safe use, support and protection from falls, and assessment of the patient’s condition (Gailey, et al., 2020). In this vein, it will be essential to provide assistance to the patient using an orthosis/prosthesis, cooperating with a prosthetist and a doctor. Within this scope, care is given in order to speed up healing after amputation, the formation of a residual stump, minimizing the formation of contractures, training in its correct use and removal, care of an orthopedic appliance, care of the skin in contact with the appliance, assistance in overcoming psychological problems associated with the loss of a limb, getting used to a prosthesis/orthosis.
Conclusion
Ewing Sarcoma, the mechanisms of initiation and development of which remain the subject of further research, is an extremely difficult disease to develop an effective strategy for treating patients. This is largely due to its resistance to standard treatments. Currently, none of the methods used to treat patients with Ewing Sarcoma has shown a qualitative superiority over others, which makes further in-depth research in this area possible and necessary. The study of the genetic characteristics of the tumor can contribute to the creation of unique drugs for targeted therapy, which will ensure high treatment efficiency and survival of patients with Ewing Sarcoma.
Standard multimodal treatment includes induction chemotherapy followed by surgery or radiotherapy, followed by multi-drug consolidation chemotherapy to control subclinical micro=metastases. The main condition for the operability of patients is radical and elasticity of tumor removal, which guarantees the absence of recurrence. When planning surgical treatment, first of all, the possibility of performing an organ-preserving operation with subsequent replacement of the removed bone and joint with a graft or prosthesis is considered. Current treatment protocols for Ewing’s sarcoma include chemotherapy both before and after surgery.
However, despite all the outlined treatment opportunities, in the case of A, the boy will be inevitably affected by the amputation of his left leg due to the low chances and the severity of the disease. Hence, it was essential to develop a particular nursing management plan to prepare and assist him in dealing with the situation. The second section contains a thorough explanation of what actions will be taken and how challenges will be overcome.
Care goals will be considered achieved if the following conditions are met. First, the patient will demonstrate improvement in physical mobility. This should include maintaining muscle strength and joint mobility, following an exercise program, and not contracting. Second, the patient will move in compliance with safety rules. It is important here to demonstrate the movement with the help of others or to perform the movement yourself. Third, the patient will walk with maximum independence. Assistive devices for walking with maximum safety will be used, and prescriptions regarding the permissible load on the limbs will be observed. Fifth, the patient will demonstrate an increase in exercise tolerance. He will not experience episodes of orthostatic hypotension, fatigue when walking, and gradually increasing the distance and speed of walking.
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