Introduction

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. It is a motor neuron disease (MND). This disease is characterized by gradually progressive dysfunction resulting from pathological changes affecting the neurons of the cerebral cortex, brainstem, and spinal cord. ALS is characterized by muscle weakness, respiratory failure and paralysis. The incidence of amyotrophic lateral sclerosis, often identified with motor neuron disease, is estimated at 3 cases per 100,000 people worldwide [1, 2]. The incidence of ALS, a motor neuron disease, is estimated at approximately 3 cases per 100,000 people worldwide [1, 2]. Some of the lowest incidence rates in Europe have been reported in Poland and Norway, at 2.0 and 2.1 cases per 100,000 people per year, respectively. South Korea also has a low incidence rate, estimated at 1.2 cases per 100,000 people per year. The average age of onset of ALS is 51–66 years. The incidence rate has increased in recent years due to better detection of the disease. Statistically, men are more likely to develop the disease than women (1.5 : 1). In the sporadic form, the average age of onset is 60 years [3]. In Europe, onset usually occurs later in life. Familial forms affect approximately 10% of patients. People with familial ALS typically develop symptoms at an earlier age than patients with sporadic amyotrophic lateral sclerosis. This disease develops more often in men (2 : 1). Risk factors include age, gender, genetic predisposition and long-term exposure to air pollution [4, 5].

Amyotrophic lateral sclerosis can progress rapidly, eventually preventing patients from moving independently. Muscle paresis of the limbs and trunk occurs. Difficulties in communication with the environment are observed due to speech disorders such as dysarthria. Oral feeding becomes difficult due to swallowing disorders. Due to muscle weakness (including weakness of respiratory muscles), respiratory failure and pulmonary complications are the most common cause of death in ALS [1]. Due to the rarity of ALS and its nonspecific symptoms, diagnosis may be delayed [6, 7]. Receiving a diagnosis of ALS can be deeply distressing for patients and their loved ones. As the disease is incurable, symptomatic treatment and supportive care are central to management. Effective symptom management is a main aim of care for patients with ALS, and may help improve their quality of life [4].

Material and methods

A case study method was employed, enabling a thorough description of the patient’s health situation resulting from the disease, its consequences, and the ongoing treatment. It facilitated the identification of the patient’s health problems and the development of an individualized nursing care plan. At the study design stage, standardized tools were selected to enable a holistic assessment of the patient. The applied scales and questionnaire were helpful in analysing the physical and mental health status of a female patient with amyotrophic lateral sclerosis. To collect information about the patient, an author-developed questionnaire was used, containing questions about her needs, along with standardized tools, such as the ADL (Katz), ALSFRS-R, Norton, Lovett, Ashworth and NRS 2002 scales. The International Classification for Nursing Practice (ICNP) was used to concisely and clearly present nursing diagnoses using standardized nursing language. The study was conducted in the patient’s home environment (during four meetings) after obtaining her informed consent. This work was prepared in accordance with the principles of Good Scientific Practice, the Act of 10 May 2018 on the Protection of Personal Data, and the principles of the Declaration of Helsinki.

The aim of the study was to present comprehensive care and treatment activities and selected nursing problems in a patient with amyotrophic lateral sclerosis and to construct a nursing process based on the International Classification for Nursing Practice (ICNP).

Case study

A 52-year-old patient with quadriparesis and dysarthria was admitted as scheduled to the Neurological Rehabilitation Department for rehabilitation. The patient reported no pain. Vital parameters upon admission to the ward were as follows: BP: 140/80 mmHg; HR: 70 bpm; body temperature: 36.8°C; breathing: 14/min, normal, odourless, soundless, even; height: 164 cm, weight: 55 kg, BMI: 20.45 kg/m² – optimal.

The patient was conscious and communicated verbally and logically, although her speech was impaired due to dysarthria. Her utterances were sometimes unclear, unintelligible, and incomprehensible to others. Speech fluency was impaired. Speech disorders are accompanied by swallowing disorders. Auto- and allopsychic orientation were intact. The patient received 14 points on the ADL scale. The woman sometimes used her phone to communicate. The patient’s general condition was good on the day of admission. The patient was crying and had a low mood. The patient reported swallowing problems and a 4 kg weight loss in 2 months. She had difficulty swallowing liquids and occasionally her saliva. Her husband reported that she experienced periodic choking. The patient experienced shortness of breath and reported minimal drooling. There was asymmetric drooping of the left corner of her mouth. The patient had muscle atrophy, mainly of the interosseous muscles of both hands, the thenar muscles, and the thenar muscles. She moved with the help of a walker and had a paraparetic gait. In addition to amyotrophic lateral sclerosis, the woman had Hashimoto’s disease (currently in the euthyroid stage), hypoplasia of the right vertebral artery, depressive disorders, and arterial hypertension (pharmacologically stabilized).

Amyotrophic lateral sclerosis was diagnosed in 2023. The first symptoms of the disease were numbness in the fingers, trembling hands, and inappropriate laughter or crying. She subsequently developed speech difficulties and considerable difficulty pronouncing words. As a result, the patient underwent detailed medical investigations. She was hospitalized in the Neurology Department in May 2023, where amyotrophic lateral sclerosis was diagnosed. The patient does not currently work, but was previously professionally active. She lives with her husband in a small town. The woman denies substance use. Her husband smoked. The patient reported an allergy to steroids used during previous treatment, which caused skin eruptions.

Physical examination on the day of entry into care:

• the patient had a mild deficit in self-care but tried to remain independent in performing daily activities. Assessment of functioning on the ALSFRS-R scale: 26 points;

• skin and mucous membranes: clean, dry skin, skin lesions (allergies), currently no risk of pressure ulcers – 16 points on the Norton scale;

• oral cavity: natural teeth in good condition, moderate salivation;

• respiratory system: shortness of breath when walking, bathing, and dressing;

• circulatory system: blood pressure 140/80 mmHg, pulse 70 bpm – regular, easily palpable; warm skin, no swelling;

• digestive system: decreased appetite, decreased thirst, oral nutrition, constipation occurs; NRS 2002 scale < 3;

• urinary system: spontaneous urination, with occasional urinary incontinence;

• senses: vision – impaired, the patient wore glasses; hearing – normal; sleep – normal; impaired speech, communication problems;

• musculoskeletal system: the patient moved using a walker or with the assistance of another person, paraparetic gait; weakened muscle strength – 2 points on the Lovett scale; there was increased muscle tension in the upper limbs – 2 points on the Ashworth scale;

• nervous system: equal pupils; periodic postural syncope, dizziness; hand tremors which prevented her from holding small objects and making precise movements; full consciousness – 15 points on the GCS scale;

• mental condition: labile mood, with difficulties in maintaining affect, and a tendency to react with uncontrollable crying. Moderate depression – 25 points on the Beck Depression Inventory;

• medications taken: Riluzole PMCS 50 mg, Symfaxin ER 75 mg, Clatra 20 mg, Hydroxyzinum 10 mg.

The process of caring for a patient with amyotrophic lateral sclerosis based on ICNP

The nursing process is a systematic and scientific method of nursing that aims to improve its standards and effectiveness, recognize the patient’s individual care needs, help meet them through planned activities, and evaluate the results obtained. In order to select the appropriate care strategies, patient observation and extensive knowledge of clinical processes are necessary. The traditional nursing process consists of five stages. These are: data collection, nursing diagnosis, planning, implementation, and evaluation [8].

The International Classification for Nursing Practice (ICNP) is the international standard for nursing terminology and is a helpful tool in developing and documenting nursing care. The main purpose of introducing the classification of nursing practice is to standardize the terminology used by nurses, mutual understanding between ethnically and culturally different groups, and ensuring high quality nursing care [9].

The International Classification for Nursing Practice allows more efficient supervision of nursing work and increased opportunities to disseminate the achieved results [10, 11]. This work uses ICNP terminology using the ICNP Browser [12].

Diagnosis 1. Difficulties with communication.

Diagnosis according to ICNP. Impaired verbal communication [10025104].

Aim 1. Reducing difficulties with communication.

Nursing interventions:

• Providing appropriate conditions for conversation (maintaining eye contact, ensuring a quiet environment);

• Demonstrating patience and understanding when communicating with the patient;

• Using non-verbal communication;

• Suggesting other forms of communication (writing on a paper, using phone);

• Teaching the patient’s family effective communication strategies;

• Allowing the patient sufficient time to communicate without rushing them;

• Facilitating contact with a speech therapist.

Nursing interventions according to ICNP:

• Making it easier to communicate needs [10038196];

• Environmental safety management [10042507];

• Patient teaching [10033126];

• Family teaching [10033086];

• Referral to speech therapy [10044577].

Implementation 1. Presenting the patient with the possibility of communication with writing on a piece of paper, telephone, gestures and ensuring silence contributed to improving communication with the patient.

Evaluation 1. Communication with the patient is more efficient.

Result according to ICNP. Able to communicate [10025025].

Diagnosis 2. Difficulty moving due to the progression of the disease.

Diagnosis in ICNP. Impaired ability to transfer [10001005].

Aim 2. Reducing difficulties with mobility.

Nursing interventions:

• Falls risk assessment;

• Providing the patient with the most necessary items close to her;

• Encouraging the patient to exercise;

• Encouraging the use of assistive devices;

• Providing a safe environment;

• Encouraging the patient to exercise under the supervision of a physiotherapist.

Nursing interventions according to ICNP:

• Mobility assessment [10030527];

• Teaching the patient how to increase tolerance of physical activity [10024660];

• Patient activity management [10044971];

• Environmental safety management [10042507];

• Cooperation with a physiotherapist [10050378].

Implementation 2. Providing a safe environment, classes with a physiotherapist and a walking frame contributed to the improvement of the patient’s movement.

Evaluation 2. Reduced difficulty in moving. The patient is more willing to engage in activities.

Result according to ICNP. Able to transfer [10028322].

Diagnosis 3. Exertional dyspnoea.

Diagnosis in ICNP. Functional dyspnoea [10029414].

Aim 3. Minimizing dyspnoea.

Nursing interventions:

• Assessment of the severity of shortness of breath;

• Assessment of the rate and pattern of breathing;

• Monitoring oxygen saturation;

• Encouragement to learn breathing exercises;

• Providing support during cases of shortness of breath;

• Avoiding factors that worsen shortness of breath.

Nursing interventions according to ICNP:

• Assessing respiratory status [10036786];

• Monitoring respiratory status [10012196];

• Monitoring blood oxygen saturation using a pulse oximeter [10032047];

• Teaching about how to increase tolerance of physical activity [10024660];

• Encouraging the use of breathing techniques [10006834];

• Encouraging rest [10041415];

• Providing emotional support [10027051].

Implementation 3. Avoiding situations that aggravate shortness of breath and learning breathing exercises involving slow inhalation through the nose followed by slow exhalation through the mouth did not relieve the patient’s dyspnoea.

Evaluation 3. The patient continues to have shortness of breath when walking and performing basic activities of daily living, such as bathing and dressing.

Result according to ICNP. Functional dyspnoea [10029414].

Diagnosis 4. Risk of aspiration due to difficulty swallowing.

Diagnosis according to ICNP. Impaired swallowing [10001033].

Aim 4. Reducing the risk of aspiration.

Nursing interventions:

• Assessment of the patient’s independence in eating meals;

• Ensuring the patient is seated upright while eating;

• Adjusting the consistency and temperature of drinks and food;

• Assisting the patient in feeding;

• Family education about feeding.

Nursing interventions according to ICNP:

• Assessing the degree of independence [10002723];

• Assessing eating or drinking behaviours [10002747];

• Teaching about fluid intake [10032939];

• Teaching about food intake patterns [10032918];

• Assisting with eating and drinking [10037269];

• Providing the family with guidance for the future [10026375].

Implementation 4. Giving thickened liquids and modified-consistency meals while the patient was seated upright was intended to reduce the risk of choking.

Evaluation 4. Reduced risk of aspiration.

Result according to ICNP. Improved fluid intake [10047330] and improving food intake [10047324].

Diagnosis 5. Depressive disorders and anxiety caused by the disease.

Diagnosis according to ICNP. Depressed mood [10022402], Anxiety [10000477].

Aim 5. Reducing anxiety and stabilizing mood.

Nursing interventions:

• Assessment of the risk of developing depression using the Beck Depression Inventory;

• Assessment of attitude towards hospitalization;

• Demonstrating empathy and understanding in the relationship with the patient;

• Supporting the patient during the disease process;

• Encouraging the patient to contact support groups;

• Encouraging a conversation with a psychologist;

• Informing the patient that mood changes may be associated with neurological changes related to the disease;

• Administering medication as prescribed by the physician.

Nursing interventions according to ICNP:

• Assessment of depressed mood [10026055];

• Assessing the attitude towards the disease [10024192];

• Providing emotional support [10027051];

• Providing social support [10027046];

• Anxiety Management [10031711];

• Referral to therapy in a support group [10024558];

• Patient counselling [10031062];

• Teaching about disease [10024116];

• Medication Administration [10025444].

Implementation 5. The Beck scale indicated depressive disorders. Explaining the course of the disease to the patient, providing support and using pharmacotherapy were reported to reduce her anxiety and liability.

Evaluation 5. Anxiety decreased and well-being improved.

Result according to ICNP. Improved mood [10050027].

Diagnosis 6. Risk of falling due to repeated episodes of imbalance.

Diagnosis according to ICNP. Risk for fall [10015122].

Aim 6. Minimizing the risk of falls, ensuring a safe environment.

Nursing interventions:

• Falls risk assessment;

• Recommending appropriate assistive devices;

• Assisting the patient with mobility using assistive devices;

• Ensuring the availability of a second person to assist the patient when standing up;

• Ensuring the patient’s safety;

• Recommending home modifications to the family;

• Using non-slip mats while bathing.

Nursing interventions according to ICNP:

• Assessing the risk of falls [10023520];

• Assisting with walking using an assistive device [10036520];

• Mobility Assistance [10036508];

• Environmental Safety Management [10042507];

• Teaching about home safety [10032960];

• Using (using) a risk reduction technique [10044985].

Implementation 6. The use of auxiliary equipment, a non-slip mat in the shower and the implementation of assistance from a second person during bathing and standing up helped reduce the risk of falls.

Evaluation 6. The risk of falls was reduced.

Result according to ICNP. Improved balance [10047348].

Diagnosis 7. Risk of deterioration of nutritional status due to dysphagia and reduced food intake.

Diagnosis according to ICNP. Risk for impaired nutritional status [10037224].

Aim 7. Preventing deterioration of nutritional status and maintaining adequate nutritional intake.

Nursing interventions:

• Assessing the patient’s nutritional status;

• Monitoring body weight regularly;

• Monitoring food and fluid intake;

• Assessing swallowing ability;

• Assisting the patient during meals;

• Collaborating with a dietitian to develop an appropriate diet;

• Educating the patient and family about proper nutrition.

Nursing interventions according to ICNP:

• Assessing nutritional status [10030660];

• Monitoring body weight [10032121];

• Monitoring nutritional intake [10036003];

• Assessing swallowing [10030690];

• Assisting with feeding [10035763];

• Collaborating with dietitian [10039435];

• Teaching about nutrition [10024618].

Implementation 7. The patient’s nutritional status, body weight, and food intake were regularly monitored. Assistance was provided during meals, swallowing ability was assessed, and the patient and family received education regarding adequate nutrition. A dietitian was involved in planning an appropriate diet to reduce the risk of nutritional deterioration.

Evaluation 7. The patient’s nutritional status remained stable, with no signs of deterioration or malnutrition.

Result according to ICNP. Adequate nutritional status [10025002].

Summary

Amyotrophic lateral sclerosis is a rapidly progressive, incurable neurodegenerative disease. ALS is a relatively rare disease with no clear early symptoms, which can result in delayed diagnosis. In most cases, the disease is initially manifested by weakness of the hand muscles, mainly the thenar muscles, while in later stages, bulbar and pseudobulbar symptoms such as dysarthria and dysphagia as well as breathing disorders are observed [6].

The case described here illustrates the main symptoms of ALS. The patient was admitted to the hospital in 2023 due to numbness in the fingers, trembling hands, emotional instability and problems in speaking. As a result of muscle weakness, the patient’s speech is slurred and incomprehensible. It is therefore important to identify alternative communication methods, including non-verbal communication. Medical staff should demonstrate empathy, patience, and understanding when communicating with the patient.

Another symptom experienced by the patient is dysphagia. Budrewicz et al. (2018) noted that dysarthria is a symptom that often occurs together with dysphagia. When dysarthria is identified, a thorough assessment is needed to determine whether swallowing difficulties are also present [13]. In this case, the patient receives thickened liquids and modified-consistency meals. These symptoms are consistent with the findings reported by Gorzkowska and Klimkowicz-Mrowiec (2023), who stated that these are the most common symptoms of the disease [4]. Hübner et al. (2017) also described symptoms experienced by patients with ALS, including increased muscle tension, general weakness and emotional liability [14].

ALS is a progressive disease with symptoms worsening over time. The progression of the disease is an individual matter, depending on many factors. According to Lynch (2023), rapid progression of ALS may occur in patients with other comorbidities. The author emphasized that depression reduces the quality of life and shortens patients’ lives [15]. In the described case, the patient struggles with depression, which adversely affects her mental and physical condition. Depression causes low mood, isolation from society and reduced motivation to counteract the disease. Rehabilitation is an important element in improving the quality of life and delaying the progression of the disease [16]. All actions taken by the interdisciplinary team are aimed at alleviating the symptoms of ALS and improving functioning [17]. Nursing care for the patient and family has a key role in providing comprehensive health care. Empathy and professionalism in the daily care provided by the nurse help to establish a therapeutic relationship with the patient and her family, which is extremely important when caring for person with a life-limiting disease.

Conclusions

The use of the ICNP in the care of patients with amyotrophic lateral sclerosis enabled comprehensive identification of care problems, planning of appropriate interventions and systematic evaluation of care outcomes, supporting the provision of high-quality care to the patient and their family.

In patient care, it is important to carry out tasks in accordance with the nursing process, which emphasizes the individual and holistic dimension of each person.

Amyotrophic lateral sclerosis is a rare, inexorably progressive and incurable neurodegenerative disease that causes gradual muscle weakness, leading over time to muscle atrophy and respiratory failure.

Disclosures

This research received no external funding.

Institutional review board statement: Not applicable.

The authors declare no conflict of interest.