The process of caring for a patient with long-term polymyositis: an individual case study

INTRODUCTION

Idiopathic inflammatory myopathies are a rare group of autoimmune diseases. Depending on the pathological features and clinical symptoms, idiopathic inflammatory myopathies can be divided into several types, including dermatomyositis, polymyositis, necrotizing autoimmune myositis, and inclusion body myositis. The most common forms include polymyositis and dermatomyositis. The disease is characterized by symmetrical progressive weakness of proximal muscles. Polymyositis affects the functioning of many organs and systems, including the cardiovascular, respiratory, and digestive systems. The main clinical symptoms include progressive muscle weakness and pain [1]. Polymyositis is more common in the female population [2].
Idiopathic inflammatory myopathies are diagnosed in 2-25 per 100,000 people. The incidence varies depending on age, gender, and etiological factors. However, diagnostic data are still missing from some areas of South America, Africa, and Asia. Although the mortality rate due to myopathy is decreasing as a result of early diagnosis and treatment, the disease contributes to the development of many diseases, including malignant tumors, cardiovascular diseases, lung diseases, and infections [3].
There are different definitions of polymyositis. One of them is based on the results of immunohistochemical and histopathological examinations of muscle biopsies, and another one is based on clinical symptoms and histopathological results [4].
Polymyositis occurs in 1 in 100,000 people. Patients experience difficulties in performing daily activities, which worsen over time. Patients may find it difficult to perform activities such as climbing stairs, standing up, or raising their arms above their heads. Non-muscular symptoms are also observed in patients, ranging from systemic symptoms (e.g. malaise, fever, weight loss, joint pain, Raynaud’s phenomenon) to joint contractures, dysphagia, respiratory disorders, gastrointestinal and cardiac symptoms, and subcutaneous calcifications [5].
Polymyositis accounts for 2-8% of all cases of idiopathic inflammatory myopathies. The juvenile form is rare and most often manifests itself with weakness of proximal and distal muscles, gait instability with falls, muscle pain and tenderness, as well as high levels of creatine kinase during adolescence [6].
Early diagnosis is crucial because polymyositis is associated with a negative prognosis and reduces the patient’s quality of life. The main diagnostic methods include serological tests, muscle biopsy, electromyography and imaging tests [7].
Treatment of polymyositis includes pharmacotherapy and physiotherapy. Glucocorticosteroids and immunosuppressive drugs are mainly used. Treatment is usually started with high doses of medication, which should then be slowly reduced depending on the patient’s clinical response. In some patients, intravenous infusions of immunoglobulins are used. If the above treatments are ineffective, combination therapy is recommended. In addition to pharmacological treatment, physiotherapy has been shown to be effective in treating neuromuscular disorders in patients. Strength training and aerobic exercises help optimize the functions of the muscles and the cardio-respiratory system. They also have a preventive effect against progressive atrophy and deterioration of fitness [8].
The clinical picture of polymyositis often overlaps with other neuromuscular diseases, which often complicates the diagnostic process. Nursing staff actively participate in patient care; therefore, they should have knowledge about the disease, including symptoms, diagnosis, and treatment process [9].
A nurse is a member of the therapeutic team that performs many important roles and functions, and also performs a number of tasks that influence the patient’s clinical condition and well-being. Actions taken by nursing staff translate into the patient’s quality of life. In order to provide proper care, a nurse must have extensive knowledge, qualifications, and interpersonal skills [10].
Currently, there are no reports in the literature on nursing care for a patient with polymyositis. Due to the importance of the topic in the professional practice of nursing staff, the aim of this article is to present the process of nursing care for a patient with polymyositis, including presentation of nursing problems and interventions undertaken.

CASE PRESENTATION

The patient was hospitalized for the first time due to muscle weakness at the age of 9. Initially, the weakening of muscle strength affected both shoulder girdles. After a year, she also began to feel weakness in the pelvic girdle muscles. Congenital myopathy was initially suspected in the patient. Therefore, physical and respiratory rehabilitation was used. At the age of 10, after the progression of clinical symptoms, polymyositis was diagnosed and pharmacological treatment was initiated – prednisone at a dose of 1 mg/kg/day – after which transient stabilization of the disease was observed.
At the age of 14, the patient began to experience limited mobility of the left eyeball. Over time, the patient developed upper limb paresis and then quadriparesis, accompanied by the preservation of physiological reflexes. Due to the progression of the disease, pulses of methylprednisolone at a dose of 1000 mg/day and azathioprine at a dose of 100 mg/day were administered.
Intensification of immunosuppressive treatment did not affect the stabilization of the disease. A deepening of the flaccid quadriparesis was observed, and contracture in the jaw and knee joints was observed. Moreover, gait disorders were increasing, from initial difficulties in movement, through the need to use walking aids, to complete immobilization of the patient at the age of 20. The therapeutic process included physical rehabilitation, verticalization, and respiratory rehabilitation. The patient obtained 2 points on the ADL (Katz Index of Independence in Activities of Daily Living) scale to assess the patient’s independence, which means severe motor impairment.
A 24-year-old patient was admitted to the Neurological Clinic due to long-standing myositis. The woman was diagnosed with progressive flaccid paresis of the upper and lower limbs. On admission, her general condition was found to be good. Neurologically, severe global flaccid quadriparesis with relative sparing of the distal muscles of the upper and lower limbs, absent knee reflexes, weakness of the axial muscles (poor head control), and contractures in the jaw and knee joints were found. The spirometric examination revealed signs of respiratory restriction with normal blood gases, without saturation disorders and clinical signs of hypoxia of the central nervous system. There were no significant abnormalities in the circulatory system.
During her stay at the clinic, the patient underwent ECG, EMG, echocardiography, spirometry, and a dermatological consultation. The EMG examination revealed electrical silence in the right biceps brachii and quadriceps muscles. When trying to move, there was a very pronounced sense of gradation. In both muscles, numerous necrotic areas and severe damage of a primarily muscular nature were found. Spirometry revealed forced vital capacity (FVC) restriction of 23%, with no signs of obstruction. During a dermatological consultation, acne was diagnosed.
The patient was recommended constant physical rehabilitation, verticalization using a verticalizer to stabilize the spine, regular respiratory physiotherapy, intensive treatment of respiratory infections, and dermatological treatment.
At the age of 29, the patient was diagnosed with breathing disorders occurring mainly at night, requiring the use of non-invasive oxygen therapy. The optimal ventilation parameters were then determined: ACMF VT 400 ml, F 17/min, PEEP 2 cm H2O, I/E: 1/1.5, Vt west. 1.4 Vt, frequency west. 1/14.
During the last hospitalization in 2009, chronic respiratory failure was diagnosed. The Medical Research Council (MRC) shortness of breath severity scale was used for assessment. The patient received a score of 4, which means shortness of breath prevents the patient from leaving the house and occurs while performing basic daily activities. The risk of pressure ulcers was assessed based on the Doreen Norton clinical scale. The patient scored 8 points, which means a high risk of developing pressure ulcers.
The patient was 160 cm tall, weighed 40 kg, and had a BMI of 15.62, which means third degree malnutrition. Esophageal dyskinesia was diagnosed. Swallowing and keeping fluids in the mouth was significantly difficult. The patient received 15 points on the MNS (Mini Nutritional Scale). For this reason, enteral nutrition (liquid diet) was administered through an established gastrostomy. Moreover, contact with the patient was difficult due to slurred speech and impaired verbal communication.
The MRC scale was used to assess muscle strength. The patient scored 0 points on the MRC scale, which means no muscle tension. The patient often reported muscle pain associated with chronic inflammation, which she usually assessed as 5-6 on the Visual Analogue Scale (VAS).
In the interview, the patient stated that she lived with her parents, was single, had a secondary education with a high school leaving exam, and had no children. She described the living conditions, care and support from her parents as good. The patient noted a significant limitation in social interactions resulting from her deteriorating health condition and immobilization.
The Beck Depression Scale was used to assess the patient’s mental state. The patient scored 24 points, which means moderate depression. The patient reported chronic depressed mood and anxiety related to the disease, ailments, significant limitation of independence and negative prospects for the future related to the continuous progression of the disease. The treatment included monitoring vital signs, electrocardiographic monitoring, pulse oximetry, gastrostomy (bronchofibroscope), oxygen therapy, and vein catheterization. Therapeutic treatment included primarily respiratory rehabilitation, non-invasive mechanical ventilation at home (NIV), hygiene and care treatments, anti-decubitus prevention, gastrostomy care, and patient and family education.
Since 2009, the patient has been receiving continuous home care. The patient is cared for by her mother, an anesthesiologist, a nurse, and a caregiver. The patient was placed on a ventilator 10 years ago. She is fed with PEG. The patient hardly speaks and only moves one finger. This allows her to communicate using a laptop. For more details, refer to the Table 1.

DISCUSSION

According to Christopher-Stine and co-investigators, polymyositis is a chronic disease that has a long-term negative impact on the patient’s quality of life and daily functioning. Patients experience periods when symptoms are less severe and periods of exacerbation. During exacerbations, increased pain, fatigue and weakness are often noted. During this period, a higher degree of disability and lower productivity are also observed. Periods of exacerbations may indicate disease progression and may require specialized clinical intervention and medical care, including from nursing staff. Patients often notice muscle weakness in the thighs, arms, hands and neck. Skin problems such as rash or skin ulcers may also occur. According to the authors, in patients with polymyositis the need for hospitalization and specialist treatment increases by 44%, and the risk of mortality is elevated three-fold compared to the rest of the population. Despite improved treatment effectiveness, the disease reduces patients’ quality of life and makes everyday functioning difficult [11].
The analyzed case confirms the above reports. The patient’s disease was diagnosed in childhood and progressed over the years, disturbing the functioning of subsequent organs/systems. The severity of the symptoms increased, making everyday functioning increasingly difficult and impossible. A number of physical, mental, and social care problems were noted in the patient with polymyositis. The subsequent findings included severe pain (muscle pain), weakness, skin and infectious lesions, and digestive and respiratory disorders. The disease had a negative impact not only on the patient’s physical condition, but also on her mental health and social life.
As Płaszewska-Żywko and Kózka point out, in order to ensure effective care, nursing staff should follow the principles of the nursing care process. It involves carrying out a number of activities, starting with the collection and analysis of information about the patient and his or her condition. Then, nursing problems should be identified, nursing diagnoses should be established, and action goals should be defined. The next step is to prepare and implement an action plan. The final stage is the evaluation of the actions taken, which allows one to determine whether the intended goal has been achieved. At each step, the actions taken should be documented [12].
In the case of the process of nursing care for a patient with polymyositis, the first step was the analysis of available medical documentation, a nursing interview with the patient, and a community interview with the patient’s family and medical staff. Based on the analysis of the collected information and personal observations, care problems were identified and care goals were defined, both regarding the hospitalization period and long-term goals related to home care after the patient’s discharge from the hospital. Hygiene and care activities, patient and family education, and emotional support were provided.

CONCLUSIONS

The patient with polymyositis had many ailments that reduced the quality of life in bio-psycho-social aspects. A properly planned and conducted process of nursing care for a patient with polymyositis allowed for improvement of the patient’s clinical condition and well-being. The patient’s symptoms were reduced or eliminated. Moreover, educational activities and instructions adequately prepared the patient and her relatives for self-care, including continued self-care after the end of the hospitalization period.

Disclosures

This research received no external funding.
Institutional review board statement: Not applicable.
The authors declare no conflict of interest.

References