"It's like going through life at a mediocre level". A qualitative study of the meaning and impact of fatigue in children and young people with sickle cell disease

doi:10.21203/rs.3.rs-4980034/v1

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Research Article

"It's like going through life at a mediocre level". A qualitative study of the meaning and impact of fatigue in children and young people with sickle cell disease

https://doi.org/10.21203/rs.3.rs-4980034/v1

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Background

Fatigue is increasingly recognised as a prevalent and debilitating symptom for young people (YP) with long-term conditions (LTCs), significantly affecting their family, social and educational participation. In sickle cell disease (SCD), fatigue is the most frequently reported symptom, surpassing pain related to vaso-occlusion. However, understanding of fatigue’s nature and impact on YP with SCD remains limited. This qualitative study explores the meaning and consequences of fatigue for YP with SCD to inform services and treatments.

Methods

This exploratory research interviewed 12 YP with SCD aged 12–23 years, five parents and ten healthcare professionals across England. Participants were recruited through convenience sampling from an NHS Trust, SCD-focused charities and social media. Data were generated using audio-recorded online semi-structured or art-elicitation interviews. Interviews were transcribed and analysed using coding, constant comparison and thematic categorisation to identify key themes.

Results

Six thematic categories were constructed from the data: (1) constant state of reduced energy, (2) the daily struggle, (3) the invisibility of fatigue, (4) being socially isolated, (5) managing fatigue, and (6) the future while negotiating fatigue. SCD fatigue was seen as a persistent, inescapable daily struggle, with reduced energy for day-to-day activities. This was often unnoticed or misunderstood by others. It hindered YP's daily routines, caused frequent school absences, reduced social participation, and promoted social exclusion. To meet social expectations and avoid stigma, YP described constantly pushing themselves to conceal their fatigue, exacerbating their difficulties with SCD. Fatigue was invisible in clinical settings, leading to a lack of standardised/formalised support and increasing uncertainties and precarity about the future. YP and parents employed energy economisation and recharging strategies to cope with and control fatigue.

Conclusions

Fatigue dominates YP’s experience of living with SCD, significantly impacting their physical, social and educational functioning and leading to isolation and stigma. Often overlooked in clinical settings, addressing fatigue should be integral to SCD care and research. This includes incorporating fatigue assessments, developing targeted self-management programmes, and furthering research on its management. The findings emphasise recognising fatigue as a primary symptom in YP with LTCs, given its severe impact on social and educational development and future stability.

Trial registration

Not Applicable

children

experiences

fatigue

qualitative

sickle cell disease

young people

Fatigue is increasingly recognised as a prevalent and debilitating symptom for children and young people (CYP) with long-term conditions (LTCs) and those treated for life-threatening illnesses like childhood cancer. Systematic reviews consistently highlight high fatigue prevalence in CYP with LTCs such as juvenile idiopathic arthritis [1], multiple sclerosis [2], and cancer [3], as well as across various chronic conditions [4]. This fatigue, often comparable to chronic fatigue syndrome [5], encompasses physical, cognitive, and emotional dimensions [1, 6]. Fatigue significantly impacts CYP’s quality of life, well-being, and daily participation—affecting learning, mood, school attendance, and relationships [1–2, 5, 7–9]. Several studies link fatigue in CYP with LTCs to various biopsychosocial factors, including pain, treatment side effects, low mood, sleep disturbances, and poor school performance [8, 11, 12]. However, fatigue in CYP with LTCs is not associated with disease severity [5, 13, 14] and often persists despite treatment of the underlying condition.

Despite its prevalence and significance, fatigue is often overlooked within interventions like self-management programmes [15] and under-acknowledged by clinicians [16, 17]. Research on fatigue in CYP with LTCs is largely focused on cancer-related fatigue. A recent systematic review on the prevalence of fatigue in CYP aged 0–19 years with LTCs found that studies on paediatric cancer-related fatigue were the most common, comprising 28% of included studies across 12 LTC categories [4]. Notably, research on sickle cell disease (SCD) fatigue is limited.

SCD is the most common genetic haemoglobin disorder, affecting about 300 births annually in the United Kingdom [18] and over 300,000 children globally [19]. The condition can lead to severe complications such as vaso-occlusive crises, recurrent infections, acute chest syndrome, stroke, and multi-organ failure [20]. Patients' common symptoms include fatigue, bone aches, and headaches, with fatigue frequently reported [21, 22]. A biobehavioural model suggests that factors such as hypoxemia, chronic haemolytic anaemia, inflammation, pain, stress, depression, and anxiety associated with SCD can cause substantial fatigue [11, 23]. Despite fatigue being a prevalent symptom, there is a glaring lack of data on its prevalence, frequency, severity, and daily life impact. This oversight may be related to the perception that fatigue is inevitable and unresolvable or because SCD affects people of black and ethnic minority backgrounds who are at increased risk of health research disparities [24].

Understanding fatigue in SCD is crucial as it may be both chronic and acute, predict crises [25, 26], and correlate with poorer quality of life (Ameringer et al., 2014; Anderson et al., 2015). Fatigue is also linked to increased acute pain episodes, depression, anxiety, behavioural difficulties, and higher hospitalisation rates in CYP with SCD [13, 23, 26]. CYP are particularly vulnerable to fatigue and its disabling consequences due to increased activity demands and expectations as they pursue education, relationships, and employment [23]. A recent qualitative study with adolescents with SCD in Ghana found that fatigue in SCD is highly stigmatising, severely disrupting their sense of normalcy and significantly limiting their educational, social, and biographical pursuits [27–29]. Yet fatigue remains underrepresented in self-management interventions for CYP with SCD [30]. Understanding the nature and consequences of fatigue for CYP with SCD is crucial to inform services and treatments. This study aimed to explore the perspectives of CYP with SCD, their parents, and health professionals on the meaning and impact of SCD-related fatigue.

We employed an exploratory qualitative approach, drawing on the principles of constructivist grounded theory [31]. The approach acknowledges that knowledge is socially constructed and situated through social interactions and that research data are co-constructed between the researchers and participants. This allowed us to bring our research, clinical and community expertise, knowledge and lived experience of SCD to shape the inquiry while reflecting on how these may influence data collection and analysis [32].

Recruitment and Sampling

We involved three participant groups: Young people (YP) with SCD, parents or primary caregivers, and healthcare professionals. Table 1 presents the sample eligibility criteria.

Table 1

Sample Eligibility Criteria
Inclusion Criteria	Exclusion Criteria
• Young people: • Aged 12–24 years • Diagnosed with SCD. Parent: Mother, father or legal guardian of a young person (12–24 years) with SCD• Healthcare professionals: Providing services to young people aged 12–24 years with SCD.	• Young people with an additional long-term condition. • Young people who are pregnant • Young people living in foster care. • Young people/parents assessed by the healthcare team to be inappropriate to include in the study, e.g. recent bereavement, current child protection issues, receiving end-of-life care. • YP (16 years and above) and parents without the capacity to consent

Eligible participants were recruited from an NHS Trust in Northwest England and through SCD-focused charities (Sickle Cell Society, OSCAR Sandwell, Sickle Cell Care Manchester, and the Congenital Anaemia Network). Health professionals were recruited through NHS England Haemoglobinopathy Coordinating Centres, the British Psychological Society Special Interest Group in Haemoglobinopathies and the Sickle Cell and Thalassaemia Association of Nurses, Midwives and Allied Health Professionals.

Convenience sampling, supplemented by snowballing, was primarily employed. As the study progressed, purposive sampling was introduced with the aim of including participants with specific characteristics (e.g., male and Asian ethnicity) and from different professional disciplines. Study information was given to eligible participants by clinicians at the Trust and key contacts at the charities and professional networks. Those interested in participating in the study contacted the first author (BAP) to discuss the study further. A time and format for an interview (in-person or remote) were scheduled with those who agreed to participate in the study. BAP also attended monthly clinics at the participating NHS Trust during the study recruitment period to meet eligible YP and parent participants who indicated interest in participating. For YP under 16 years old, parents were involved in the recruitment process and provided consent for their child’s participation. We aimed to recruit 30 participants (15 YPs, five parents and ten healthcare professionals) for the study. However, theoretical sufficiency was reached with 27 participants.

Data Collection

Data were collected by BAP using semi-structured interviews and art-based approaches. The latter were included to give YP participants greater flexibility in choosing how they wished to represent and communicate their experiences [33]. Interviews were conducted with 12 YP, five parents and ten healthcare professionals. All the interviews were conducted between June and November 2022 via videoconferencing (Zoom or MS Teams based on participant’s preference) and lasted between 30 and 120 minutes. Seven YP expressed interest in art-based methods and produced artworks. Four produced drawings and/or photographs, two produced poems and one produced a video diary. These artworks were used to stimulate and facilitate data generation. Topic guides were developed for each participant group to guide the interviews. Table 2 presents the main topic areas in the topic guides. The guides and interviews focused on participants’ perspectives on the meaning and impact of SCD-related fatigue in YP; the strategies and resources used by YP and parents to self-manage fatigue; and current service provision for SCD-related fatigue and potential facilitators and barriers to fatigue management in routine care. Informed consent and assent (from those under 16) were obtained from all participants before participation in data collection.

Four YP-parent dyads participated in the study, and the child and parent were interviewed separately in each instance. All interviews were audio-recorded and conducted in English. Although provision was made for interpreters where needed, all the YP and parent participants chose to have their interviews in English and were competent in conversational English. With participants’ permission, interviews were audio-recorded and professionally transcribed. Consent was sought to use the interview excerpts and artworks anonymously during research dissemination. Participating YP and parents were offered a £20 gift voucher as a token of appreciation.

Table 2

Interview topic guides
Participant group	Main topic areas
Young people	1. Experience of living with SCD in general a. Daily life with SCD and severity of condition (symptoms, hospital stays) b. Impact on daily life (physical, social, educational/academic, emotional/mental, employment) c. Influence on relationships – parents, siblings, peers, intimate partners d. Self-management – practices, challenges/difficulties, challenges in different contexts (home, school, work, other) e. Support (sources, types, helpful/unhelpful) and support needs 2. Experience of fatigue a. Nature of fatigue – description, explanation of feeling of fatigue b. Impact of fatigue – physical, social, educational/academic, emotional/mental, employment c. Factors influencing fatigue – contributory factors, alleviating factors, contextual (personal, family, social, educational, employment) d. Concerns and worries about fatigue 3. Self-management of fatigue a. Day-to-day self-management – practices, experiences and concerns b. Challenges in different environments – home, hospital, school, work, other c. Views about current support (forms, sources, helpful/unhelpful) d. Support needs and perceptions of unmet support needs
Parents	1. Experience of SCD in general a. Severity of child’s condition (symptoms, hospital stays) b. Impact of SCD on child’s and family’s life 2. Management of child’s conditions a. Practices and strategies b. Concerns about managing child’s condition c. Challenges/difficulties in different contexts (home, school, work) d. Support (sources, types, helpful/unhelpful), support needs (perceptions and concerns) e. Perceptions of child’s self-management skills f. Views about transferring management responsibilities to the child g. Experiences and concerns regarding role transfer 3. Child’s fatigue experience a. Perceptions of the nature of child’s fatigue – description of child’s fatigue b. Impact of child’s fatigue on child and family’s life c. Factors influencing child’s fatigue d. Concerns and worries about child’s fatigue e. Strategies for managing child’s fatigue f. Experiences of providing support g. Experiences of healthcare provision for child’s fatigue 4. Management of fatigue a. Day-to-day self-management – practices, experiences, concerns b. Challenges in different environments – home, school, work c. Views about current support (forms, sources, helpful/unhelpful) d. Support needs and perceptions of unmet needs
Healthcare professionals	1. SCD care in general a. Organisation of services and standards of care b. Current treatments and support programmes c. Views and concerns about current care 2. Self-management among CYP a. Preparation of CYP for self-management b. Current programmes used to support self-management c. Barriers/facilitators to providing self-management support 3. Perspectives on SCD fatigue a. Causes- biological, psychological, social b. Influencing factors – contributing and alleviating factors c. Impact of fatigue on CYP d. Vies and concerns about fatigue in CYP 4. Fatigue management a. Current treatment/support programmes for fatigue b. Experiences of providing fatigue support to CYP and families c. Barriers/facilitators to providing fatigue support and self-management interventions

Data Analysis

Interview transcripts and artworks were anonymised[1] and transcripts were imported into NVivo 12 Pro to organise and manage the data. Using the constructivist grounded theory approach (Charmaz, 2014), data were analysed iteratively through coding, constant comparisons, thematic categorisation and concurrent data collection and analysis. BAP coded the transcripts. BAP, KA and SK met regularly to discuss the emerging codes, category development and interpretation. Coding and category development guided subsequent data collection. Regular meetings among the authors were also used to reflect on the data and developing themes, seeking alternative and possible themes and interpretations. This helped to increase the credibility of the analysis and identify how our experiences and insights about the research area might influence data analysis and interpretation. The reporting of the findings below has prioritised the YP accounts and used the experiences of parents and healthcare professionals as a commentary on the YP’s accounts. Six thematic categories were generated from the analysis: a constant state of reduced energy, the daily struggle, the invisibility of fatigue, being socially isolated, managing fatigue, and the future while negotiating fatigue.

Ethics

Ethical approval

for the study was received from the UK’s Health Research Authority (REC reference: 22/SW/0036; IRAS ID: 310855) before recruitment. All study participants were provided with a tailored participant information sheet. Audio-recorded verbal consent and/or assent was obtained before the start of every interview. For YP under 16 years, consent was obtained from parents before they were approached about participation in the study. Parents also provided proxy consent before their children were interviewed. If the YP did not give assent, they were not asked to participate in the interview, even if their parents consented to their participation. Consent was regarded as a continual process, with attention paid to any indication that participants might wish to discontinue the interview. Participants were fully informed about the purpose of the art-based methods, the potential copyright and anonymity issues that might arise, and their consent and/or assent were sought for the use of the artwork for data generation and research dissemination. As there was a risk that participants might become distressed during the interviews, a distress and debrief policy was developed to ensure participants were supported both during and after participation in the study. Procedures were also established for any safeguarding disclosures, and confidentiality limitations were highlighted in the participant information sheets.

Public and Patient Involvement

We involved three young people with SCD (RO, AM and EK) as project advisors who contributed to various stages of the research. Notably, two (EK and RO) were actively involved in developing the grant application, shaping the research from its early stages. The young advisors were also instrumental in developing participant-facing recruitment materials – study information sheets, consent/assent forms for YP participants and a recruitment video. They contributed to developing topic guides for data collection and were involved in data analysis and interpretation.

The sample for our study comprised 12 YP, five parents (all mothers), and 10 healthcare professionals from various disciplines (Table 3). The YP ranged in age from 12 to 23 years old, with five aged between 12 and 15 years old and seven between 16 and 23 years old. Eight YP identified as female and four as male. It is worth noting that there were no YP or parent participants from an Asian background, which is not unusual as SCD predominantly affects people of African and Afro-Caribbean backgrounds in the UK [34].

Table 3: Participant Characteristics

YP characteristics

Number

Number of participants

Sex

Male

Female

Ethnicity

Black-British (African)

Black-British (Caribbean)

Black African

Mixed (White & African)

Age

12-15 years

16-23 years

SCD diagnosis

HbSS

HbSC

Disease-modifying treatment

Hydroxycarbamide

Exchange blood transfusion

Perceived SCD severity

Mild

Moderate

Severe

Current Educational level

Primary School

Secondary School

College

Postgraduate

Parent characteristics

Number

Sex

Female

Ethnicity

Black-British (African)

Black-British (Caribbean)

Black African

Black Caribbean

Child’s SCD diagnosis

HbSS

HbS-Beta Thalassaemia

Perceived severity of child’s SCD

Mild

Moderate

Severe

Educational level (Child)

Primary

Secondary

Special Education

HCP characteristics

Number

Number of participants

Discipline/Job title

Consultant haematologist

Specialist nurse (hospital care)

Specialist nurse (community care)

Clinical psychologist

Specialist physiotherapist

Youth worker

The findings are presented around the six categories generated from the analysis. The categories present an overall line of argument about how fatigue is experienced as a constant daily struggle of reduced energy, which is invisible to others, promotes social exclusion, is difficult to manage, and creates uncertainties about the future.

A Constant State Of Reduced Energy

Across all the interviews, participants clearly described fatigue and how it influenced their everyday lives. They described their fatigue using metaphors related to low energy:

It’s like having the appearance of a Land Rover but the energy capacity of a small Fiat (YP10, male, aged 16 years)

It’s like going through life constantly on a low battery (YP08, male, aged 12 years)

YP regarded fatigue as a fundamental feature of SCD and, therefore, inescapable, with one YP emphasising how it was, thus, my fate, I cannot escape (Fig. 1, poem by YP02, female, aged 23 years). They identified biological factors as being the underlying cause of SCD fatigue, using terms such as low haemoglobin, chronic anaemia, sickled red cells, and hypoxaemia. Therefore, SCD fatigue was externalised as something they had no control over. However, they were aware that their fatigue was exacerbated by dehydration, exertion (physical, emotional, cognitive or mental), pain crises, strong pain medications, not taking SCD medication on time, and not eating well. YP associated fatigue with feelings of exhaustion, dizziness, shortness of breath, forgetfulness, concentration difficulties and an overwhelming urge to sleep. Indeed, for YP, there appeared to be a close and intertwined relationship between fatigue and SCD pain, with fatigue being described as a trigger, a consequence and a warning sign of an imminent pain crisis. This caused anxiety as the YP felt they could not always mitigate the risk.

SCD fatigue was described as being present even at rest, and YP felt that they never had sufficient energy for day-to-day activities. Fatigue was characterised as constant and dynamic, with YP moving between a state of low energy and a state of no energy (YP02, female, 23 years). SCD fatigue was seen as unpredictable and often not proportional to recent activity, which meant that what YP could tolerate energy-wise varied from day to day. They found it difficult to make plans and commitments, as they were unsure they could see them through. This created uncertainties and added to the precarity created by having a long-standing chronic condition (this is further discussed below). Fatigue appeared to increase in frequency, severity and impact as YP transitioned through educational and social stages, took on more responsibilities, and pursued interests and opportunities.

The Daily Struggle

SCD fatigue was experienced as a physical and mental ‘heaviness’ that presented YP with a daily struggle to navigate and overcome. YP described it as having your bones replaced with lead (YP11, female, 16 years), wearing a puffer coat filled with rocks (YP06, female, 18 years) and a big grey cloud that sits over you (YP12, female, 14 years). Consequently, extreme mental and physical effort was needed for daily activities such as getting out of bed in the morning, keeping their eyes open and remaining attentive in school. YP07 explained the constant physical and mental struggle:

With sickle cell anaemia, there’s the constant physical agony of fatigue we go through and getting up is difficult …not only is it a physical struggle, but it’s also a mental struggle ... having to gain the strength to get up. Like it’s a constant, oh, how am I going to get up now from this exhaustion? Or where do I get the strength to get up from my bed every morning? (YP07, female, aged 20 years)

The representation of SCD fatigue as a daily struggle likened the YP’s lives to being in a war zone, where they had to fight against the odds (YP02, female, 23 years). They described how they were involved in a daily fight against their fatigue (and themselves) to engage in daily life. For instance, they described fighting to stay awake to concentrate in school, a fight they mostly lost. This daily struggle set SCD fatigue apart from ‘normal’ tiredness, which, as expressed in YP11’s account below, was described as a transient experience associated with extreme exertion and relieved by sleep/rest. The YP were aware that their reduced energy and the daily struggle were different from that of peers:

My fatigue is present when I wake up, present throughout the day, and present when I go to sleep. It can also come alongside a pain crisis. I just ignore it and push through it every day. It’s not like normal tiredness that people normally experience, which happens after a long day or intense exercise and is relieved after sleeping (YP11, female, aged 16 years)

YP described pushing themselves physically and mentally to navigate and overcome the daily struggle, as seen in YP11 and YP07’s accounts above. They explained how this was an important skill for mastering everyday school, social and family activities that were limited by fatigue in order to maintain a sense of ‘normality’ even though they were aware that ignoring and pushing through came with the risk of triggering a pain crisis. This notwithstanding, fatigue was seen as always having the upper hand; while YP could feel trapped in this fight, they persisted, hoping the next day would be better. One young person extensively captured this sense of battling fatigue in a poignant poem (Fig. 1, poem by YP02, female, aged 23 years).

The school was particularly singled out as the site of a constant battle with fatigue (see Fig. 1). YP reported frequent school absences, non-participation or reduced participation in schoolwork, and overwhelming tiredness, leading to sleeping in class. Consequently, YP found it difficult to ‘keep up’ with school/college work, which could negatively impact their educational achievements. As YP10 explained:

It has sometimes made it difficult to concentrate in class. And last year, this was happening a lot. I would just get home and sleep when I had a lot of schoolwork to do, but I couldn’t stay awake at the end of the day. So, I started to fall behind a little bit, so I had to spend the whole summer just trying to catch up. (YP10, male, aged 16 years).

The unpredictability, inevitability, constant battle, and pervasive nature of SCD fatigue meant that participants had normalised its presence. There was a sense of resigned acceptance for the YP, with one YP alluding that is how it is with sickle cell (YP05, male, aged 14 years). The normalisation of SCD fatigue contributed to its invisibility to others. However, for several of the parents, the inevitability and invisibility of SCD fatigue led to feelings of disempowerment and a sense of helplessness – this is further discussed below.

The Invisibility Of Fatigue

Maintaining the invisibility of fatigue was perceived as important by YP in order to ‘pass’ as ‘healthy’ and ‘normal’ within their social settings. YP described aiming to achieve this by pushing through the daily struggle and hoping no one sees (YP11, female, 16 years) their struggle with fatigue. However, their efforts to hide and mask SCD fatigue were not always successful, which could ‘out’ YP, raising suspicions about their normality and exposing them to stigma. According to YP, SCD fatigue betrayed their daily efforts to prevent their illness from being their identity.

Indeed, SCD fatigue was seen as a discreditable attribute and an enduring identity feature for YP with SCD. In particular, others could not distinguish SCD fatigue from ‘normal’ tiredness and thus failed to appreciate YP’s daily struggle.

When you tell people you are tired, they think it’s normal tiredness, and when you tell them it’s not, they say, okay, so what have you done today? And you narrate to them that, oh, I’ve just been here and there, just gone to this place because I have to do this today or I have to do that today… to them, that is not supposed to make you tired because it’s not stressful when you tell them about it. But they don’t realise that the energy you need to use to power yourself is double what they would use to do one activity, and this is energy that you normally don’t have too (YP01, female, aged 20 years)

Having their explanations for their lack of energy discredited and contested by others forced YP into silence and to accept the label of lazy. This further supported their strategy of masking their fatigue, as YP10 explained:

I’ve become quite good at making the fatigue less noticeable, even if it’s there. I don’t want to bring it up, it gets frustrating from all the interrogations. Because I think to them it looks like me not wanting to do much. I guess it looks like being lazy or just putting in no effort. And I could tell them what’s going on, but I think it would just be a lot easier for me to keep quiet and go on with things (YP10, male, 17 years)

The daily struggle with reduced energy levels and the stigmatising responses to their fatigue highlight the precarious situation that fatigue creates for YP. To maintain a sense of normalcy, YP often push themselves to function despite their fatigue, striving to keep their struggle invisible to others, as Y11 presents in her poem:

You want to lie down, wait for it to pass

But life waits for no one

So, you push through, knowing your body will push back

But you do it anyway

Hoping no one sees

The awfulness of fatigue

(excerpt from a poem by YP11, female, aged 16 years)

This effort requires a persistent approach, as they must continuously manage their energy levels and maintain ‘normal’ outward appearances. The need to conceal fatigue and maintain normalcy underlines a form of resilience that YP develop. This resilience emerges as a response to the vulnerability, uncertainty, and precarity that fatigue creates, as YP navigate these challenges while seeking to uphold their daily routines and responsibilities.

YP felt that SCD fatigue was invisible to teachers, healthcare professionals, and services. According to YP and parents, it was not considered in routine health assessments, patient/parent education, transition services/support, and health consultations.

I have never talked about it with them because they don’t ask about it. I don’t know if there’s anything they can do about it (P05, child aged 15 years)

They just ask do you get tired at school. And obviously I say yes and then they’re like, next question, let’s measure you, let’s do this. (YP06, female, aged 18 years)

Rather, YP and their parents said SCD care and service provision appeared to focus on SCD pain management and monitoring/managing complications. As a result, YP and their families normalised and minimised the symptom and rarely raised it during healthcare consultations even when it significantly impacted on YP’s quality of life. This created a sense that fatigue was an unimportant and unresolvable problem that families had to cope with alone, disempowering YP and parents in seeking help for fatigue.

From healthcare professionals’ accounts, the invisibility of SCD fatigue appeared to relate to there being a limited evidence-based understanding of the symptom, a lack of evidence-based treatment interventions, and the absence of validated fatigue assessment tools for YP with SCD.

I think that we don’t realise how important fatigue is. And I think we don’t have an understanding of just how important it is. If you ask a haematology doctor about sickle cell, fatigue would be one of the things that they mention when they talk about it. But I think there’s no way to measure it, and I think that is what we’re not very good at. When we can’t measure something physically, it’s harder to quantify, and it relies more on qualitative conversation. ….. we tend not to give it enough airtime, it’s not even thought of. (HCP05, Clinical Psychologist)

Indeed, some healthcare professionals struggled to explain SCD fatigue, sometimes conflating it with ‘normal’ tiredness or associating it with supposedly typical teenage behaviours of being lazy, being bored, and having poor sleep etiquette. As HCP05 suggests above, the subjective nature of fatigue means that it can be perceived as unmedical and, therefore, an untreatable symptom. Indeed, many healthcare professionals reported feeling unequipped and unconfident in supporting YP with this symptom:

It's the one symptom you know is present, but you don’t ask a lot about it because you don’t know what support to provide (HCP10, haematology consultant).

If a child said in a clinic appointment that they were really struggling to manage their tiredness or fatigue, I don’t know where that would lead to. I don’t know if we have a pathway in the same way that we have a pathway for pain management or recovering from a stroke. (HCP05, Clinical Psychologist)

It was notable that when fatigue had been discussed during a consultation, YP had found it helpful even without an ‘objective’ fatigue assessment, as it validated their experiences and acknowledged that fatigue was a significant part of their lives with SCD. Indeed, the opportunity to discuss fatigue during consultations was identified as fundamental to bringing legitimacy to SCD fatigue and as a helpful intervention in its own right.

Being Socially Isolated

Fatigue was found to influence YP’s ability to engage in social activities and to develop and maintain social relationships. During interactions with peers, YP described how their fatigue made them feel being in a bubble and separate from their peers, as YP04 conveyed in a drawing:

This drawing is about me in a video game, and I’m separated from everyone else. I can’t like play because I’m tired and yeah, and I’m low in energy. I’m that figure in the circle, which is like a bubble. Everyone else is kind of doing something in the game except me (YP04, male, aged 12 years)

Figure 2: Drawing by YP04

This sense of detachment and YP’s tendency to regularly cancel social plans could be interpreted by their peers as YP having an attitude problem and being unreliable:

Socially, I’m the friend that’s always tired, and I cancel plans a lot if I’m just tired. Or if I’m already out, or I’m surrounded by my friends, or whatever, I’m just, sometimes I’m just quiet, and literally don’t sometimes have the energy to speak. Like I can be observing what’s going on, my friends might think I’m in a mood or something because I don’t really respond. But I’m literally just in my own world because I’m so tired. And then, I’m there, but I’m not really there: I’m kind of like a blank canvas (YP02, female, aged 23 years)

On days when I’m struggling with fatigue, say I’m organising a day out with my friends, and at the last minute, I have to cancel. I think things upset me because this time and age, I really want to experience life. Just have fun and do normal teenage things, but that is not always possible. (YP11, female, aged 16 years)

Consequently, bringing their authentic selves to social relationships and interactions was challenging as they struggled to sustain the expected level of engagement. Fatigue creates a barrier between YP and what they want to do, encouraging social isolation and withdrawal and a preference for solitary activities as YP found it exhausting to interact with others and engage in social activities. This made them feel they were losing their best years (YP02, see Fig. 1) as they struggled to experience life and do normal teenage things (YP11, female, 16 years). One YP described life with SCD fatigue as like being a dead tree (i.e., being socially dead).

The tiredness makes me feel like a dead tree, like there’s no life in me because I can’t do a lot of things, and I’m mostly on my own. There’s no sparkle in my life; it’s just dull and boring (YP08, male, aged 12 years)

Figure 2: Drawing by YP09

Parents felt that SCD fatigue limited their ability to encourage and support their child’s social participation and independence:

It’s hard to even get him to go out and be with his friends or engage with people. He says, ‘mummy, you don’t understand, I’m tired’. It’s hard for me to see him struggle in that way. He’s at the age he should be physically and socially active, and he just can’t because of the fatigue (P01, child aged 14 years)

The difficulties YP experienced in social participation due to fatigue led them and their parents to describe feelings of guilt, fear, worry, anger, sadness, frustration and despair. As will be discussed later, participants were concerned about the future implications of social exclusion for YP’s future.

Managing Fatigue

In the absence of service provision for SCD fatigue, YP and parents relied on their own creativity and ingenuity to manage the symptom. Participants described using a range of self-management strategies informed by lived experience that centred around energy management (i.e., energy preservation and ‘recharging’). These self-management strategies included rest/sleep, activity pacing, hydration, multivitamin supplementation, physical exercise, solitude/solitary activities and healthy eating. As one YP explained:

For me, drinking a lot of water, eating well and doing calm things on my own is how I deal with fatigue, like the way you charge a phone, an electric car and water a flower (YP08, male, aged 12 years)

Self-management was the YP’s way of asserting control over fatigue and their lives. It involved YP making daily decisions on how to effectively use their energy, which involved prioritising activities and planning ahead. When making these decisions, YP evaluated potential activities in terms of their costs and benefits and the potential energy consequences of their actions. This required strict self-regulation, self-discipline, and stringent prioritisation of energy expenditure. As one participant explained, it also involved YP coming to understand their own bodies and personal limits:

I recommend just knowing your limits and knowing how to get rest. Because when you go over your limit, you’ll usually notice because that’s when the severe fatigue and tiredness will kick in. When I didn’t understand my body and fatigue, my fatigue was pretty bad; I felt tired all the time until I understood what was happening in my body and how to deal with it. Now, I keep track of everything I do, and that has helped me to come to know my body. At first, I just kept pushing myself because back then, I didn’t know my limits, so I was pushing myself even more, which caused me to be tired when I wasn’t even doing anything. Because then in my body, I just felt tired all the time, and I felt lightheaded, and I always had to sleep (YP05, male, aged 14 years)

In relation to rest, YP described having to balance the need to be ‘productive’ whilst making time for rest to avoid overexertion and the risk of triggering a crisis. This requires them to make calculations different to those of their peers, which further contributes to their sense of difference:

The struggle is moderating how much rest I take and balancing it with what I have to do. Sometimes, I’ll be like resting now, and then I rest a bit too much, and then I’ll have to rush to get on top of my work and get the work done. So, it’s finding the balance between making sure I have enough energy but not taking too long with the rest. That’s hard (YP10, male, aged 16 years).

It's difficult to find the balance between being productive and not overexerting yourself. When your baseline energy is biologically low, you seem to overexert even when you’re doing less (YP11, female, aged 16 years)

The wider social context influenced the use of fatigue management strategies. For instance, family finances influenced the ability of YP to eat healthily, engage in exercise (e.g., gym membership), access solitary and less energy-demanding activities (e.g., art/music programmes), and purchase multivitamin supplements and energy fluids. Family housing circumstances could restrict the availability of comfortable spaces to promote quality rest/sleep. In addition, the demands of life and sociocultural expectations created barriers to using rest and pacing as strategies. YP perceived these strategies as unrealistic for their day-to-day lives, particularly in school/college, due to inflexible routines, lack of conducive rest spaces and the need to ‘keep up’ with schoolwork:

It's not everywhere that you can rest and pace yourself. And you can’t do it all the time, too. If I have a wave of fatigue that hits me while I’m at school or something, there’s not really a lot I can do to rest. I can’t just stop everything I’m doing. So, I just try and continue with what I’m doing, knowing that when I get home, that could impact me feeling worse the next couple of days or even with some sort of pain crisis (YP06, female, aged 18 years)

Indeed, rest and pacing were perceived to be antithetical to youthfulness and productivity. As one young person explained, rest and pacing could have a detrimental effect on their education, employment, finances and social life:

That advice of rest and pace yourself is given without consideration for how realistic they are. You can’t just rest and pace yourself without detriment to your education, employment, finances or social life. I just shake my head when they tell me that because life doesn’t wait for you to rest. Resting and pacing means you’re always playing catch up or missing out or going through life at a mediocre level (YP02, female, aged 23 years)

The Future While Negotiating Fatigue

SCD fatigue created significant fear, concern, and uncertainty amongst YP and parents over whether YP would have the necessary energy and physical capacity to assume adult roles and be ‘successful’ independent adults. The YP and parents highlighted similar concerns. However, the YP appeared calmer when talking about them, while the parents seemed distressed. The lack of attention given to fatigue in clinical care exacerbated YP's and parents’ fears, concerns, and anxieties for the future.

I am worried about when I get older. How will I cope when I start having a family of my own? I’m already struggling with work due to the fatigue (YP02, female, aged 23 years)

I wish it would stop. He’s going to have to go through puberty, and I don’t know where he’s going to get the energy from. I’m afraid he will struggle in life and not have the energy and the strength to do the things every child is supposed to as they grow (P03, child aged 12 years)

These concerns contributed to the YP’s sense of precarity and uncertainty. Fatigue was seen as reducing YP’s capacity to build the personal, social and educational capital for adulthood as it impaired their social, educational and physical functioning during childhood and adolescence. The disruptive nature of fatigue required YP to consider its future consequences, which required negotiation. In terms of future career plans, fatigue had forced some YP to alter these in order to find a career that would fit a future with constant fatigue. YP06 explained how she had been forced to abandon her career aspirations of being a psychologist and instead pursue a business apprenticeship due to SCD fatigue:

I’m going to go to college to do an apprenticeship. I chose an apprenticeship instead of uni because of the fatigue. The apprenticeship will be a bit more manageable for me. It works for me as an individual rather than what I want to really do in a career. I’d have studied psychology at uni if I was going. So, fatigue affects my decisions about what I want to do in the future. (YP06, female, aged 18 years)

Healthcare professionals were similarly concerned about the future of YP. One haematology consultant highlighted their concerns about how fatigue could limit YP’s future economic potential and independence due to their difficulties in taking up opportunities during adolescence that build economic and social capital:

Fatigue is going to limit their economic potential. These young people are struggling at school due to fatigue. I’m worried that they are going to grow up trapped in the economic deprivation and hardship most are already growing up under. How are they going to attain true independence? This will significantly affect their health in adulthood, increasing the burden on the health services. Due to fatigue, they can’t take advantage of many opportunities and roles necessary for their development. (HCP10, Haematology Consultant)

This study offers comprehensive insights into SCD-related fatigue in YP with SCD, drawing from the perspectives of YP, parents and healthcare professionals, thereby contributing to the currently limited understanding of fatigue in SCD. The findings illuminated how fatigue shapes the lives of YP with SCD, portraying it as a pervasive, unpredictable and omnipresent symptom that significantly impacts their daily functioning across physical, social and educational domains. YP vividly described fatigue as an ongoing battle with exhaustion, comparing it to carrying burdensome weights like having bones replaced with lead and wearing a coat filled with rocks, which hinders basic tasks such as waking up and staying attentive in school. These descriptors echo similar themes found in studies involving adolescents with chronic conditions such as multiple sclerosis and chronic fatigue syndrome (Carroll et al., 2016; Parslow et al., 2018). The school was singled out as the site of the battle with fatigue, causing frequent school absences, reduced participation in schoolwork and activities, and sleeping during lessons.

The distinction between SCD fatigue and normal tiredness was underscored by YP’s accounts, emphasising the constant presence and debilitating nature of SCD fatigue throughout the day. Despite this, peers and teachers often fail to recognise the severity of YP’s fatigue, leading to their experiences being contested, discredited, or labelled laziness, even when they try to explain it. Consequently, YP feel compelled to conceal and mask their fatigue, pushing themselves beyond their limits to conform to societal expectations, as seen in other chronic illness contexts [2, 27, 35–37]. Throughout the YP’s accounts, there seems to be little room for them to contest expectations and demands posed by others (and themselves) on their level of performance and engagement. If they resist or fail to assimilate, they risk developing a stigmatising sense of personal laziness and irresponsibility [27, 28, 37].

A unique contribution of this study is the revelation of the invisibility of SCD fatigue within clinical care settings. Healthcare professionals appear to often overlook or minimise discussions of fatigue during routine assessment and consultations due to the lack of standardised assessment tools and treatment protocols, coupled with over-normalisation and a broader misunderstanding that conflates SCD fatigue with everyday tiredness or typical adolescent behaviours (e.g., the propensity to sleep in, reticence to engage in activities). These issues sidelined fatigue as a less prioritised concern. The oversight perpetuates the normalisation of fatigue among YP and parents, fostering a sense of disempowerment in seeking appropriate support, consistent with findings in studies of chronic fatigue syndrome [35, 38]. However, where health professionals did acknowledge and discuss fatigue with YP, it validated their experiences and provided a crucial opportunity for supportive intervention. This highlights the importance of integrating discussions about fatigue into routine care to enhance understanding, support and quality of life for YP with SCD.

The theme of being socially isolated highlighted how SCD fatigue impedes YP’s social participation and relationships, resulting in the cancellation of social plans, forsaking cherished activities and diminished ability to engage authentically with peers. YP described feeling detached and often misunderstood by friends, echoing experiences documented in other chronic illness contexts [6, 9, 35, 36, 38, 39]. Being socially isolated was likened to feeling like a dead tree, symbolising a lack of vitality and connection with others. Parents also echoed these social challenges, expressing emotional distress about their children missing out on normal social experiences and the difficulties in supporting their independence. As other studies have noted, the unpredictability, variability and lack of formalised information and support complicated parents’ efforts to support their children to have a normal social life [40].

The self-management strategies employed by YP and parents, such as energy conservation techniques and lifestyle adjustments, underscored their proactive approach to coping with SCD fatigue. These strategies mirror those reported in studies of adolescent cancer survivors and other individuals with SCD, emphasising the importance of pacing and prioritisation in managing daily activities [27, 37, 41, 42]. Being able to economise their reduced energy capacity efficiently meant YP needed to understand their bodies and personal limits, constantly weighing the cost and benefits of activities and being able to discipline and regulate themselves strictly. However, this was not always realistic for the YP due to a lack of understanding, acceptance and support within their social domains coupled with the wider demands of excellence and competence. As they often told us, being the responsible and disciplined YP who rests, paces themselves and avoids overexertion to self-manage fatigue was in tension with being productive and youthful. This aligns with another qualitative study, where YP with SCD in the UK were conflicted in juxtaposing self-disciplined with self-actualised/productive identities [37]. This forced the YP to push themselves constantly. Pushing yourself was considered a positive (and inevitable) self-management strategy [41]. This was an important strategy to meet education and social responsibilities, avoid stigmatising social responses, as well as helping the YP handle social discourses surrounding being young, productive, responsible and self-sufficient, irrespective of the dire consequences for their health [29, 37].

One significant finding pertains to the future implications of SCD fatigue, which pose substantial challenges to YP’s personal, social and economic development. Fatigue not only compromises their current educational and social participation and achievements but also influences their career aspirations, forcing some YP to rethink their vocational and professional choices to align them with their reduced energy capacity. This adaptation reflected a strategic and resilient response to mitigate the impact of fatigue on their professional prospects and long-term career trajectories in order to work towards becoming neoliberal citizens – responsible, productive, competent and autonomous [27, 29, 37]. There was a palpable sense of fear, uncertainty and anxiety among YP, parents and healthcare professionals regarding the future implications of SCD fatigue, such as the ability of YP to manage family responsibilities, sustain meaningful employment, achieve economic independence and avoid dependence on healthcare services. Seeing their children juggle the immediate consequences of fatigue and its future projections heightened parents’ concerns and anxieties. The lack of recognition and management of fatigue within clinical care also exacerbated these fears as YP and parents grappled with uncertainties about long-term outcomes and quality of life.

IMPLICATIONS FOR SERVICES AND RESEARCH

This study highlights the multidimensional impact of SCD-related fatigue on young people’s lives, shedding light on its pervasive nature, social and educational consequences, and implications for their future development. It underscores the urgent need for healthcare professionals to systematically recognise and address fatigue within clinical care to empower YP and their families to manage fatigue effectively and enhance their overall quality of life. Our research indicates that fatigue should be integral to SCD care and included in (a) health assessments (e.g., annual reviews, psychological assessments, admission, and discharge plans), (b) patient and parent education programs, (c) healthcare professional education and training, (d) school education and care plans, and (e) public education and SCD advocacy. Study participants emphasised the importance of improving knowledge and understanding of SCD-related fatigue among care providers, schools, employers, third-sector organisations, and the wider society. This awareness is crucial in raising the profile of the symptom and supporting YP with SCD.

Our research has highlighted the limited understanding and resources available to identify and manage SCD-related fatigue. Consequently, addressing fatigue should be a research priority in SCD. Research on cancer-related fatigue suggests that a systematic approach is necessary for understanding this phenomenon in SCD across the lifespan. As noted by several researchers [10, 13, 43], more research, including longitudinal studies, is required to fully quantify the burden of fatigue in CYP living with SCD. There is a need to develop treatment pathways for fatigue that incorporate assessment tools, treatment, and self-management interventions/technologies. Therefore, future research should (a) examine factors that contribute to or exacerbate fatigue in CYP to identify specific causal mechanisms, (b) optimise existing patient-reported outcome measurement tools for SCD-related fatigue, (c) assess fatigue as an endpoint for existing and future disease-modifying therapies and self-management interventions/technologies, and (d) develop self-management tools and programmes that specifically address fatigue.

STRENGTHS AND LIMITATIONS

This study’s strength lies in the diversity of its sample, which included YP, parents and healthcare professionals from various disciplines and locations across England. Another key strength is the involvement of three young people with SCD in designing and conducting the study. They participated in all stages of the research, from developing the initial research idea to ensuring the study’s relevance and meaningfulness to YP and their families by providing diverse perspectives. Additionally, using art-based approaches enhanced the comprehensiveness of the data collected, supporting the young participants in sharing their experiences more effectively. However, there are some limitations. The interviews were conducted at a single time point, so the findings do not account for changes in fatigue or its management over time and at different stages of a child’s development. Additionally, excluding children younger than 12 years may be considered a limitation. Fewer male YP were interviewed than female YP; however, the in-depth interviews generated rich data from both sexes.

This study reveals the significant impact of SCD fatigue on YP, affecting their physical, social and educational lives. Often misunderstood by others, SCD fatigue leads to isolation and stigma. It is frequently overlooked in clinical settings due to a lack of standardised assessment tools. Addressing fatigue should be integral to SCD care, including health assessments, education programmes and public advocacy. Future research should focus on factors contributing to fatigue, optimising measurement tools, developing self-management programmes and including fatigue as an outcome in treatment evaluations. A comprehensive approach will improve support and quality of life for YP with SCD. These insights also have broader implications for all YP dealing with fatigue, highlighting the need for a comprehensive and empathetic approach across various LTCs, with fatigue addressed as a significant symptom rather than a secondary concern.

Abbreviation	Definition
CYP	Children and Young People
HCP	Health Care Professional
LTC	Long-Term Condition
P	Parent
SCD	Sickle Cell Disease
YP	Young People

Ethical approval and consent to participate

Ethical approval for the study was received from the National Research Ethics Service (Reference: 22/SW/0036). Informed consent/assent was obtained from all study participants. All methods were carried out in accordance with relevant guidelines and regulations.

Consent for publication

Not applicable.

Availability of data and materials

The data generated and/or analysed during the current study are not publicly available due to privacy or ethical considerations. The point of contact regarding the availability of data and materials is the corresponding author.

Competing interests

The authors declare that they have no competing interests.

Funding

This project is funded by the National Institute for Health and Care Research (NIHR) under its Research for Patient Benefit (RfPB) Programme (Grant Reference Number NIHR2021169). The views expressed are those of the authors and not necessarily those of NIHR or the Department of Health and Social Care.

Authors’ contributions

All authors, except A.M., were involved in conceptualising the study. B.A.P. collected data and led the data analysis. All authors contributed to data analysis and interpretation. B.A.P. produced the initial draft; all authors read and approved the final manuscript.

Acknowledgements

We want to thank the participants who gave time to contribute their perspectives to this study and the clinicians, nurse specialists and charities who facilitated their recruitment.

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Participants were identified by their unique identifier, plus sex and age for the YP participants (e.g., YP08, male, 12 years = YP participant 8) or plus child’s sex and age for parent participants (P01, male child, 14 years = Parent 1), or plus disciplinary background for healthcare professional (HCP01, specialist nurse = Healthcare Professional participant 1)

No competing interests reported.

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"It's like going through life at a mediocre level". A qualitative study of the meaning and impact of fatigue in children and young people with sickle cell disease

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Version 1

Abstract

Figures

BACKGROUND

METHODS

Recruitment and Sampling

Data Collection

Data Analysis

Ethics

Public and Patient Involvement

RESULTS

A Constant State Of Reduced Energy

The Daily Struggle

The Invisibility Of Fatigue

You want to lie down, wait for it to pass

But life waits for no one

So, you push through, knowing your body will push back

Hoping no one sees

The awfulness of fatigue

Being Socially Isolated

Managing Fatigue

The Future While Negotiating Fatigue

DISCUSSION

IMPLICATIONS FOR SERVICES AND RESEARCH

STRENGTHS AND LIMITATIONS

CONCLUSION

Abbreviations

Declarations

References

Footnotes

Additional Declarations

Status:

Version 1