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DOI: 10.1016/j.sedeng.2019.10.003
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Available online 29 April 2020
Nursing care plan in febrile infection-related epilepsy syndrome
Plan de cuidados de enfermería en el síndrome epiléptico relacionado con infección febril: a propósito de un caso
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María Palanca Cámaraa,
Corresponding author
mpalancacamara@gmail.com

Corresponding author.
, Rosa Güell Barób
a Unidad de Epilepsia Refractaria, Servicio de Neurología, Hospital Universitari i Politècnic La Fe, Valencia, Spain
b Unidad de Cirugía Vascular y Angiología, Hospital Universitari Joan XXIII, Tarragona, Spain
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Table 1. Nurse assessment of the FIRES patient in the acute phase according to the NANDA, NOC and NIC taxonomies.
Abstract
Introduction

Febrile infection-related epilepsy syndrome (FIRES) is a serious epileptic encephalopathy of unknown aetiology, which leads to refractory epileptic status and neuropsychological deterioration. It appears mainly in children and has 3 phases: initial, acute and chronic. In the chronic phase, educational intervention is important to reduce and address exacerbations. Its prevalence is low (1/100,000 children in Europe) which makes it a disease of interest to present as a clinical case with a nursing care plan.

Case

This is a 16-year-old male patient who debuted at age 7 and has intellectual impairment and abehavioural disorder. Approximately every 21 days he has exacerbations requiring hospitalisation, a situation that we address in this case.

Care plans

The main needs we find are breathing (risk of aspiration), food (risk of electrolyte imbalance), elimination (urinary and faecal incontinence), mobility (deterioration of physical mobility), communication (deterioration of verbal communication) and safety (tiredness of the caregiver's role, risk of falls, risk of infection, risk of deterioration of skin integrity).

Conclusions

In the acute phase, the need for safety is the most affected, requiring the necessary prevention measures. On the other hand, hospitalisation is a good time to work with the family, optimising the resources provided (home service, ketogenic diet, among others). A multidisciplinary approach is essential for planning and carrying out care.

Keywords:
Epileptic syndrome
Encephalopathy
Children
Nursing evaluation
Resumen
Introducción

El síndrome epiléptico relacionado con infección febril (FIRES) es una encefalopatía epiléptica grave de etiología desconocida, que deriva a estatus epiléptico refractario y a un deterioro neuropsicológico. Aparece en niños principalmente y presenta 3 fases: inicial, aguda y crónica. En la fase crónica es importante la intervención educativa para reducir y afrontar las agudizaciones. Su prevalencia es baja (1/100.000 niños en Europa) lo que lo convierte en una enfermedad de interés para presentar en un caso clínico con su plan de cuidados de enfermería.

Caso

Se trata de un paciente varón de 16 años que cuya enfermedad se inicia a los 7 años, y presenta un retraso mental y un trastorno del comportamiento. Cada 21 días aproximadamente presenta agudizaciones requiriendo hospitalización, situación que abordamos en este caso.

Plan de cuidados

Nos encontramos principalmente alteradas la necesidad de respiración (riesgo de aspiración), alimentación (riesgo de desequilibrio electrolítico), eliminación (incontinencia urinaria y fecal), movilidad (deterioro de la movilidad física, riesgo de deterioro de la integridad cutánea), comunicación (deterioro de la comunicación verbal) y seguridad (cansancio del rol del cuidador, riesgo de caídas, riesgo de infección).

Conclusiones

En la fase aguda, la necesidad de seguridad es la más afectada, requiriendo las medidas de prevención necesarias. Por otro lado, la hospitalización es un buen momento para el trabajo conjunto con la familia, optimizando los recursos proporcionados (servicio a domicilio, dieta cetogénica, entre otros). El abordaje multidisciplinar es indispensable para la planificación y realización de los cuidados.

Palabras clave:
Síndrome epiléptico
Encefalopatía
Niños
Valoración de enfermería
Full Text
Introduction

Fever-Related Epileptic Syndrome (FIRES) is a severe epileptic encephalopathy that leads to refractory epileptic status and neuropsychological impairment.1 We understand epileptic encephalopathy as a disorder in which epileptic activity by itself can contribute to severe cognitive and behavioural disturbances beyond what might be expected from the underlying disease.2 Furthermore, refractory epileptic status is defined as seizures that persist after at least 2 doses of the appropriate parenteral treatment.3

FIRES appears in previously healthy children preceded by a febrile infection. Its origin is unknown, and no infectious, metabolic, immunological or genetic cause has been clearly identified.4 The age of onset is from 3 to 15 years, although it can also appear in adults.1 The prevalence in Europe is estimated at 1/100,000 in children and adolescents, and the annual incidence is 1/1,000,000.5

The condition presents in 3 phases: initial, acute and chronic. In the initial phase a simple febrile infection appears, followed a few days later by an acute phase with highly recurrent or cluster-like seizures that rapidly evolve into refractory epilepsy.6 The electroencephalogram (EEG) shows temporary and frontal focal discharges, with diffuse slowing and frequent bilateral diffusion.7 Finally, the chronic phase is characterised by refractory epilepsy with neuropsychological impairment.6

With regard to the approach for the patient in status epilepticus, it is important to manage the airway and ensure adequate ventilation and circulation as in any emergency situation. Decreased level of consciousness, as well as the treatment used, can compromise respiratory function, therefore vital signs should be monitored, the patient should be placed in a lateral decubitus position to prevent aspiration and ensure peripheral venous access. The patient should also be protected from possible injury secondary to uncontrolled movement.8

In the chronic phase, repeated seizures can damage the cognitive abilities of the brain, especially the functions of the frontal and temporal lobes.4 This results in learning problems, behavioural disorders, memory problems, sensory changes and reduced mobility.4 In other words, the affected person may have special needs, with communication difficulties, as well as motor impairment and inability to self-care.

There is no clear, specific and effective treatment for these patients, but it is common to use burst suppression coma induction in the epileptic status and acute phases, while in the chronic phase various treatments are used such as ketogenic diet, rituximab monoclonal antibody, stimulation of the vagus nerve, immunotherapy, surgery, steroids and plasmapheresis, obtaining different results depending on each case, and therefore the patients must be treated very individually.1,4

Repeated seizures together with cognitive impairment imply a high need for care and frequent admissions. The family and especially the primary caregiver experience a very high burden of care, as well as a strong emotional impact. Day centres and residential care can be a good option to reduce this burden and help the child's development, but they also involve a cost.

The incidence of FIRES is low, in fact, it is considered a rare disease,5 but affected patients should still receive comprehensive and quality nursing care. The nurse must have the necessary knowledge to care for these patients and their families. The case study enables us to examine the disease in greater depth and establish a standardised care plan to improve the care of future patients.

Description of the case

16-year-old male patient, right-handed, no history of interest. At 7 years old he began with epileptic status in the context of symptoms of fever of several days’ duration. The origin of the fever is unknown. He was treated with antiepileptics, corticoids and immunoglobulins and diagnosed with FIRES. At 13 years of age, he underwent resective surgery to address the probable right frontal origin which was not effective.

Since the onset, the patient has suffered cluster seizures of 3–4 days’ duration, which sometimes leads to status epilepticus and repeat every 21 days, approximately. The seizures consist of a cephalic turn, tonic posture and fall, followed by a secondary generalised seizure (sGS). He requires pharmacological treatment (valproic acid, lacosamide, lamotrigine and clobazam) and the use of protective devices. It also requires outpatient and specialist follow-up, and hospitalisation approximately every 21 days for treatment of refractory epileptic status.

The patient has moderate mental retardation and behavioural disorder and is dependent for the basic activities of daily living (ABVD), with difficulties in the following: feeding, elimination, body hygiene, mobility, dressing and communication.4

His main caregiver is a parent and he attends a special education centre 8 hours a day. He carries out leisure activities under supervision such as cycling, swimming, using video games or skating, and is able to establish interpersonal relationships

In June 2015 he was admitted to the Refractory Epilepsy Unit of La Fe in Valencia, coinciding with the periodic onset of seizures. On arrival, he presented non-convulsive epileptic status and a lacerated contused wound in the left occipital region due to a fall. He was monitored by video-EEG after the relevant informed consent was signed by his legal representative, since the data obtained may be used for educational purposes. Vital signs were also monitored.

A slowing down and disorganisation of the brain activity was observed on the EEG tracing, with generalised epileptiform activity, synchronous and asynchronous with predominance of left hemisphere, with a tendency to periodicity and rhythmicity, without the patient making clear progression or recovery of his baseline status. By administering antiepileptic drugs intravenously (valproic acid, lacosamide, levetiracetam and clonazepam), the epileptic state was reversed, recovering its pattern characteristic of epileptic encephalopathy on the EEG, moving from generalised epileptiform activity to slowed background and multifocal epileptiform activity.

During his hospital stay the patient was in a state of coma which reverted with the use of intravenous antiepileptic drugs, with difficulty in sphincter control and impossibility of swallowing. He had no infectious complications, bronchoaspiration or added skin lesions. After 7 days he was discharged, having recovered his former functional state

Nursing assessment

Due to a decreased state of consciousness and according to the Virginia Henderson model,9 the patient has the following altered needs:

  • -

    Respiration: risk for airway obstruction and bronchial aspiration.

  • -

    Nutrition and hydration: inability to swallow food of any texture.

  • -

    Elimination: presence of urinary and bowel incontinence.

  • -

    Mobility: the patient is bedridden, unable to maintain a sitting or standing posture.

  • -

    Rest and sleep: the sleep pattern is altered due to epileptic status.

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    Maintaining body temperature: inability to express thermal sensations and cover or uncover the body to maintain an adequate temperature

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    Dressing - inability to choose and wear appropriate clothing.

  • -

    Hygiene and skin: inability to maintain adequate body hygiene. The patient's immobility induces a high risk of pressure ulcers (score of 9 on the modified Norton scale10). In addition, the patient has a scalp wound in the occipital area of the skull, requiring suture and topical dressings.

  • -

    Communication: inability to communicate and express himself with either verbal or non-verbal language. Preserves reactions to pain (Glasgow index 7) and reflexes.

  • -

    Safety: there is a high risk for fall (score of 4 on the Downton scale11). The family expresses feelings of tiredness due to the patient's state of health, frequent hospital admissions, distance from home to the hospital, etc. All this affects them negatively at work and in interpersonal relationships.

  • -

    Beliefs and values: the patient lacks the awareness to control these aspects.

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    Work and self-realisation: inability to participate in recreational activities or to learn.

Diagnoses

From the altered needs, we identified the following nursing diagnoses12:

  • -

    Respiration:

  • -

    00039 – Risk for aspiration related with (r/t) cognitive impairment

  • Nutrition and hydration:

  • -

    00102 – Self-care deficit: feeding r/t cognitive impairment manifested by (m/b) inability to ingest sufficient food

  • -

    00195 – Risk for electrolyte imbalance r/t inability to take in fluids

  • -

    Elimination:

  • -

    00110 – Self-care deficit: toileting r/t cognitive impairment m/b inability to use the toilet

  • -

    00020 –Functional urinary incontinence r/t cognitive impairment m/b involuntary loss of urine

  • -

    00014 –Bowel incontinence r/t cognitive impairment m/b involuntary soiling

  • -

    00015 – Risk for constipation r/t immobility

  • -

    Mobility:

  • -

    00085 – Impaired physical mobility r/t cognitive impairment m/b decrease in gross motor skills

  • Sleep and rest:

  • -

    00198 – disturbed sleep pattern r/t change in normal sleep pattern m/b lack of sleep control

  • Dressing:

  • -

    01109 – Self-care deficit: dressing r/t cognitive impairment m/b impaired ability to choose clothing

  • Maintain body temperature:

  • -

    00109 – Self-care deficit: dressing r/t cognitive impairment m/b impaired ability to choose clothing

  • Hygiene and skin:

  • -

    00108 – Self-care deficit: bathing r/t cognitive impairment m/b inability to complete personal hygiene

  • -

    00047 –Risk for impaired skin integrity r/t immobility

  • Communication:

  • -

    00051 – Impaired verbal communication r/t cognitive impairment m/b inability to speak

  • Safety:

  • -

    00061 – Caregiver role strain r/t high care demand of the sick person m/b verbal expression of tiredness

  • -

    00155 – Risk for fall r/t impaired mental state

  • -

    00004 – Risk for infection r/t the use of invasive devices

  • -

    00213 – Risk for trauma r/t tonic-clonic seizures

Care planning and delivery

Based on the nursing diagnoses identified, the NOC and NIC12 patterns that were most appropriate for the patient's situation were chosen, and they were used to create a care plan (Table 1).

Table 1.

Nurse assessment of the FIRES patient in the acute phase according to the NANDA, NOC and NIC taxonomies.

NANDA  NOC  NOC indicators  NIC  NIC activities 
(00039) Risk for aspiration r/t cognitive impairment  (0410) Airway patency  (41012) Ability to clear secretions(41015) Dyspnoea at rest(41019) Cough(41020) Accumulation of sputum  (3140) Airway management(3200) Aspiration precautions(6610) Risk identification  - Identify biological, environmental and behavioural risks and their (interrelationships)- Apply risk reduction activities-Maintain airway patency-Monitor pulmonary status- Place the patient in the position that allows the maximum possible ventilation potential- Keep suction equipment available 
(00195) Risk for electrolyte imbalance r/t inability to take in fluids  (0602) Hydration(0606) Electrolyte balance(1902) Risk control  (60211) Diuresis(60202) Moist mucous membranes  (2020) Electrolyte monitoring(4120) Fluid/electrolyte management(4140) Fluid resuscitation(4190) IV insertion(4200) IV therapy  -Maintain appropriate IV infusion flow rateMonitor the relevant laboratory tests for fluid balance- Watch for signs and symptoms of fluid retention- Monitor the patient's response to the prescribed electrolyte therapy- Watch for manifestations of electrolyte imbalance- Examine the patient's oral mucosa, sclera and skin for signs of impaired hydroelectrolyte balance (dryness, cyanosis, jaundice) 
(00020) Functional urinary incontinence r/t cognitive impairment m/b involuntary loss of urine(00014) Bowel incontinence r/t cognitive impairment m/b involuntary soiling  (909) Neurological status(1101) Tissue integrity: skin and mucous membranes  (090901) Neurological function: consciousness(090914) Absence of seizure activity  (610) Urinary incontinence care(1750) Perineal care(1804) Self-care assistance: urination/defaecation  -Provide aids (pads)- Provide assistance until the patient is fully capable of assuming self-care- Facilitate hygiene after urination/defaecation 
(00091) Impaired bed mobility r/t cognitive impairment m/b impaired ability to slide or change position in bed  (203) Initial body position(204) Consequences of immobility  (020311) From side to side  (740) Care of the bedridden patient(840) Position change (6486) Environmental management: safety(6490) Fall prevention  - Place in a position of correct body alignment- Identify safety needs based on the patient's physical and cognitive function and behavioural history-Keep available adaptive devices (stools or handrails) to increase the safety of the environment- Use side rails of adequate length and height to prevent falls from the bed- Review history of falls with family 
(00198) Disturbed sleep patter r/t change in normal sleep pattern m/b lack of sleep control  (0004) Sleep  (402) Hours of sleep achieved(403) Sleep pattern(407) Sleep habit  (1850) Sleep enhancement  - Determine the patient's sleep/wake pattern- Determine the effects of the patient's medication on sleep pattern 
(00108) Self-care deficit: bathing r/t neuromuscular impairment m/b inability to fully or partially wash the body  (305) Personal care: hygiene(1101) Tissue integrity: skin and mucous membranes  (110113) Skin integrity  (1610) Bathing(3540) Pressure ulcer prevention  -Record the condition of the skin on admission and daily-Ensure the patient's hygiene in bed- Perineal care 
(00047) Risk for impaired skin integrity r/t physical immobility  (1101) Tissue integrity: skin and mucous membranes  (110113) Skin integrity  (840) Change of position(3540) Pressure ulcer prevention  -Record skin condition on admission and daily-Strictly monitor any areas of redness-Use elbow and knee protectors, as appropriate-Monitor the patient's mobility and activity-Ensure appropriate nutrition-Place in position of correct alignment-Minimise friction and shearing forces when repositioning the patient 
(00051) Impaired verbal communication r/t cognitive impairment m/b inability to speak  (0902) Communication  (90208) Exchange of messages with others  (5460) Touch  -Assess the preparation of the patient when offered contact 
(00061) Caregiver role strain r/t high care demand of the sick person m/b verbal expression of tiredness  (2211) Parenting performance(2609) Family support during treatment(2202) Caregiver homecare readiness  (260917) Participates in discharge planning(220201) Willingness to assume caregiving role(220204) Participation in decisions about home care  (2380) Medication management(2680) Seizure management(7040) Caregiver support  -Determine the appropriate community resources to cover vital needs and basic health- Determine the caregiver's acceptance of their role- Inform home care staff about the patient's stay at home, health status and technologies used with the patient's or family's consent 
(00155) Risk for fall r/t impaired mental state(00213) Risk for trauma r/t tonic-clonic seizures  (1912) Falls(1902) Risk control  (191204) Falls from bed(190214) Use of personal support systems to control risk  (6486) Environmental management: safety(6490) Fall prevention(6610) Risk identification(2680) Seizure management  - Use prevention devices: bedrails-Help the family to identify and modify hazards in the home 
(00004) Risk for infection r/t the use of invasive devices  (1908) Risk control  (190218) Other  (6550) Infection protection  -Watch for signs and symptoms of systemic or localised infection 

m/b: manifested by; r/t: related to.

Since most of the problems identified were dependent on the acute epileptic status situation, nursing care focused mainly on patient safety to prevent complications. In general, attention was focused on maintaining airway patency and prevention of bronchial aspiration, with monitoring of respiratory function and discontinuation of oral diet and switching to hydroelectrolyte replacement. The short recovery period meant that other therapies such as enteral or parenteral nutrition were not necessary.

Nursing activities were also aimed at preventing skin lesions due to both pressure and moisture and possible convulsions. Skin hygiene and hydration, postural changes and the use of bedrails were used in this regard.

Hospitalisation also enabled working jointly with the family. On the one hand, listening and support were provided and, on the other, the resources provided were optimised. Education was provided on the ketogenic diet,13 which had not been tried previously, and the home hospitalisation service was contacted for intravenous administration of medication at the beginning of the seizures, thus preventing them from becoming worse and the need for admission.

Assessment of results

With planned nursing care, we were able to keep the patient free of respiratory complications, maintain his hydration and electrolyte balance until he was able to eat on his own, and hygiene and skin care substitution tasks were performed keeping the skin intact and achieving good healing of the occipital wound as an outpatient. Little by little the patient recovered the sleep-wake rhythm, and his ambulation functions, communication and leisure activities. For primary care givers, the resources provided implied a reduction in load and number of hospital admissions.

Discussion

Since this is a rare epileptic syndrome, but with devastating consequences for the patient, all professionals involved in its treatment must pool their knowledge to address it, at all times from an individualised perspective.1,4

Nursing care should be aimed primarily at preventing complications or major injuries caused by acute situations. In the assessment, special attention should be paid to the primary caregiver, since the chronic phase is characterised by cognitive and behavioural deterioration,4 increasing their psychological and emotional burden.

The approach to the disease should be multidisciplinary, from the neurologist with the pharmacological and therapeutic management of the disease, the nurse with the planning and implementation of care, to the endocrinologist with the establishment and monitoring of the ketogenic diet.13

Conflict of interests

The authors declare that they have no conflict of interests.

References
[1]
R.H. Caraballo, G. Reyes, M.F. Avaria, M.C. Buompadre, M. Gonzalez, S. Fortini, et al.
Febrile infection-related epilepsy syndrome: a study of 12 patients.
[2]
J. Ramos-Lizana.
Encefalopatías epilépticas.
Rev Neurol, 64 (2017), pp. S45-S48
[3]
L.J. Hirsch, N. Gaspard, A. van Baalen, S. Demeret, T. Loddenkemper, V. Navarro, et al.
Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions.
Epilepsia, 59 (2018), pp. 739-744
[4]
K.L. Hon, A.K.C. Leung, A.R. Torres.
Febrile infection-related epilepsy syndrome (FIRES): an overview of treatment and recent patents.
Recent Pat Inflamm Allergy Drug Discov, 12 (2018), pp. 128-135
[5]
Van Baalen A. Síndrome epiléptico por infección febril: 2012 [informe en Internet]. Portal de información de enfermedades raras y medicamentos huérfanos: Orphanet. Recovered from: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=ES&Expert=163703 [accessed 05.07.19].
[6]
N. Moreno.
Crisis febriles simples y complejas, epilepsia generalizada con crisis febriles plus, FIRES y nuevos síndromes.
Medicina (B. Aires), 73 (2013), pp. S63-S70
[7]
C.A. Pardo, R. Nabbout, A.S. Galanopoulou.
Mechanisms of epileptogenesis in pediatric epileptic syndromes: rasmussen encephalitis infantile spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES).
Neurotherapeutics, 11 (2014), pp. 297-310
[8]
C. Minardi, R. Minacapeli, P. Valastro, F. Vasile, S. Pitino, P. Pavone, et al.
Epilepsy in children: from diagnosis to treatment with focus on emergency.
J Clin Med, 8 (2019), pp. E39
[9]
A. Younas, J. Sommer.
Integrating nursing theory and process into practice; Virginia's Henderson need theory.
Int J Caring Sci, 8 (2015), pp. 443-450
[10]
B. Romanos, N. Casanova.
La escala de Norton modificada por el INSALUD y sus diferencias en la práctica clínica.
Gerokomos, 28 (2017), pp. 194-199
[11]
M.J. Bueno-García, M.T. Roldán-Chicano, J. Rodríguez-Tello, M.D. Meroño-Rivera, R. Dávila-Martínez, N. Berenguer-García.
Características de la escala Downton en la valoración del riesgo de caídas en pacientes hospitalizados.
Enferm Clin, 27 (2017), pp. 227-234
[12]
NNNconsult. Elsevier; 2019. Recovered from: www.nnnconsult.com [Cited during the month of July 2019].
[13]
M. Palanca Cámara.
Aspectos dietéticos en el paciente epiléptico7.
Rev Cient Soc Esp Enferm Neurol, 42 (2015), pp. 4-9

Please cite this article as: Palanca Cámara M, Güell Baró R. Plan de cuidados de enfermería en el síndrome epiléptico relacionado con infección febril: a propósito de un caso. Rev Cient Soc Esp Enferm Neurol. 2020. https://doi.org/10.1016/j.sedene.2019.10.001

Copyright © 2019. Sociedad Española de Enfermería Neurológica
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