In the first of a two-part article, Dr Claudine Matthews speaks to Deborah Fletcher on perspectives from a specialist dietitian on sickle cell disease.
The nutrition landscape in sickle cell disease (SCD) is changing, thanks to pioneering research focused on integrating nutrition and nutritional care into sickle cell patient pathways in the NHS.1 SCD is a marginalised, genetically inherited red blood cell disorder characterised by several clinical features and complications affecting medical and nutritional problems in this patient population.
The aetiology of the nutritional problems in SCD is as a direct consequence of the main clinical features, namely chronic haemolysis, vaso-occlusion, impaired immunity and chronic inflammation responsible for the malnutrition risks, growth delays and multiple nutrient deficiencies commonly experienced by this patient population.2
Despite the plethora of existing scientific research on the role of nutrition in SCD, very little has been conducted specifically looking at the integration of nutrition into standard care. In addition, a significant paucity exists about the nutritional management of sickle cell patients across the life course. Furthermore, key nutrition knowledge care gaps have been identified in a recent qualitative study1,3 linked to the lack of nutritional care provision available to sickle cell patients in the NHS.
More research is needed to inform the development of tailored sickle cell nutrition policy and guidelines to support clinical practice. The creation of the first ever specialist dietitian in sickle cell role speaks to the strides made as sickle cell nutrition is recognised as an emerging speciality in the profession of dietetics. This is a significant breakthrough towards integrating nutrition and nutritional care into sickle cell patient pathways.
Perspectives from a specialist dietitian in sickle cell in her role as part of the SCD MDT
The implementation of the national nutrition standards4 calling for dietitians to work as part of the SCD MDT signals a change in the dietetic management of sickle cell patients in the NHS.
This role forms part of a community sickle cell improvement project, where the dietitian works alongside a specialist sickle cell physiotherapist and sickle cell pharmacist. Here, dietitian Deborah Fletcher, shares insights and perspectives about the dietetic management of sickle cell patients and how this role adds value to patients’ access to and outcomes of tailored nutritional care that is integrated into standard SCD patient pathways.
Dr Claudine Matthews (CM): What sparked your interest in applying for the job?
Deborah Fletcher (DF): My interest developed while I was working in the community with head and neck oncology patients. My manager informed me about a new pilot post in sickle cell care and encouraged me to consider applying. I had reached a point in my role where I felt ready for a new challenge and a change in direction.
The opportunity to move into a different specialism really appealed. I was keen to broaden my clinical experience, develop new skills and step outside my comfort zone. This offered the chance to grow professionally while contributing to an area of care that is both complex and deeply meaningful.
CM: Did you have prior knowledge of sickle cell disease before coming into this role?
DF: My understanding of sickle cell disease came mainly from personal exposure rather than formal clinical experience. A school friend had this condition, which left a lasting impression and gave me an early awareness of its seriousness. I also knew sickle cell predominantly affects people of African and Caribbean heritage and had some understanding of the pain crises associated with it. Clinically, my dietetic knowledge was limited, although I was aware of common medications and their potential side effects.
CM: What were the types of patients referred to your service and the main reasons for referrals?
DF: We receive referrals for both adults and children with a confirmed diagnosis of sickle cell anaemia (most common genotypes being Hb SS and Hb SC). Unfortunately, we are not funded to take on thalassaemia patients. Patients are generally referred to the MDT (for physiotherapy, pharmacy and dietetic review) and are referred for a variety of reasons, including optimising medication management, improving physical activity or receiving general healthy eating nutritional advice.
From a dietetic point of view, the main referral criteria are those requiring nutrition support advice for those with a BMI below 18kg/m2 (for adults), significant weight loss (>10%) and/or prolonged inadequate intake (five days or more). I also review those seeking nutrition education and advice to optimise nutritional status to better manage their sickle cell and signpost to local weight management programmes for adults with a BMI over 27.5kg/m2.
CM: What were some of the key factors you considered when undertaking a nutritional assessment of sickle cell patients?
DF: I aim to build a comprehensive picture of their clinical status as well as the impact the condition has on their day-to-day life. Working with the pharmacist and physiotherapist, I review medications, mobility and any recent complications or treatments. This includes exploring issues such as gastrointestinal side effects from opioids, current hydroxyurea treatment, recent biochemistry results, transfusion history, the presence of leg ulcers, sleep patterns, fatigue, recurrent infections or hospital admissions. Frequent pain crises and hospital stays can affect appetite and eating patterns, so I try to understand how often these occur and how they influence nutritional intake.
It is well documented that people with sickle cell disease have increased resting energy expenditure and higher nutrient requirements because of chronic inflammation, accelerated red blood cell turnover and increased cardiac output. Nutritional assessment therefore focuses on assessing overall nutrient intake, alongside monitoring height, weight, BMI, and growth velocity in children.
With the physiotherapist, we also assess handgrip strength and may carry out a sit-to-stand test. Renal impairment can be an issue, with some patients experiencing proteinuria, so I consider what the recommended guidelines for this are, while still aiming to optimise nutritional status.
Micronutrient status is another key area, especially folate, zinc and vitamin D and assessing sufficient intake. Iron, however, requires more nuanced interpretation. Regular blood transfusions can lead to iron overload, so this must be reviewed cautiously, often with the medical team. Hydration is always a priority. Adequate fluid intake can help reduce the risk of vaso-occlusive crises, but many individuals struggle to meet their requirements. This can be especially difficult to monitor in school-aged children.
For young people, growth is a major focus. Delayed growth and puberty are relatively common. I also sensitively explore any signs of pica (eating non-food items such as paper, sand or soil), as this can be linked to micronutrient deficiencies. Older children may feel embarrassed discussing this, so building trust is essential. If pica is identified, the recommended pathway is to refer to the children’s psychology team and inform the consultant and sickle cell nurse. A detailed dietary history helps me understand eating patterns, appetite and nutritional adequacy.
It is also important to consider cultural, social and financial factors that may influence food choices, including access to food, family support and any barriers that may affect nutritional wellbeing.
CM: What are some of the key challenges you’ve experienced when undertaking nutritional assessments in sickle cell patients?
DF: One is the limited evidence base of the information to hand. Compared with conditions such as cystic fibrosis, the research on nutrition in sickle cell is still emerging, so you’re often relying on a mix of available evidence, clinical judgement and what you learn from working closely with patients.
Another challenge is the impact of socioeconomic and psychosocial factors. Some patients struggle to access nutrient-dense foods or maintain good hydration because of financial pressures or limited availability, so you must be mindful when making recommendations. We work closely with welfare support advisers who can delve deeper into financial constraints and seek additional support where needed to facilitate access to hardship schemes, food bank vouchers, disability badges and employment and housing advice.
For many sickle cell patients, this may be the first time they have been referred to a dietitian, so they may be sceptical about what we can provide. Building trust and a rapport with patients is vital to improve outcomes and overall patient experience. Chronic symptoms also play a huge role. Pain, fatigue, poor sleep and medication side effects can all reduce appetite and make it difficult for patients to follow nutritional advice consistently. It means you have to set very realistic goals and be flexible.
As sickle cell is such a fluctuating condition, you often need to work closely with the MDT, for example the pharmacist and physiotherapist, to help manage pain or other symptoms before nutrition can improve. It’s very much a team effort.
CM: What types of nutritional interventions have you used when treating sickle cell patients?
DF: My interventions depend on the individual reason for referral, but there are common themes. I’m often helping patients increase their energy and protein intake to meet their higher metabolic needs. That might involve suggesting calorie-dense, nutrient-rich foods, simple food fortification ideas or quick and easy meals to make at home. I also encourage limiting ultra-processed foods. Folate supplementation is common because of the increased red blood cell turnover and I often look at other micronutrients too, especially vitamin D, zinc and magnesium.
I often receive referrals for iron deficiency, although I have to be mindful of whether they are also due to receive a blood transfusion, as sometimes iron overload can also be seen. Hydration is another big area, as dehydration can cause more frequent vaso-occlusion crises. I give practical, personalised advice because, even though most patients know hydration is important, many struggle to meet their daily recommended amounts.
I also encourage an increase in anti-inflammatory foods, such as fruits, vegetables, oily fish and foods rich in polyphenols and omega 3s. For children, growth monitoring is key. If there is faltering growth or delayed puberty, I will work closely with parents and paediatric sickle cell nurses to devise suitable food fortification advice and/or consider oral nutrition supplements.
For patients with a higher BMI, the approach is more nuanced as I need to balance the metabolic demands of sickle cell with healthy weight management. I therefore focus on nutrient-dense foods, maintaining adequate protein intake and avoiding restrictive diets. Hydration is essential. I usually give advice for healthy eating and weight management.
The interventions are individualised, and I try to make them as achievable as possible.
CM: What nutritional guidance, policies and resources did you use to support your practice?
DF: In practice, I drew from a mix of national guidance, haematology standards and dietetic frameworks because there isn’t a single comprehensive manual for sickle cell nutrition – although we now have access to the first ever chapter on sickle cell nutrition, written by Dr Claudine Matthews, published in the Manual of Nutrition and Dietetics and known as the gold standard textbook for dietetic practice! I use NICE guidance, particularly around chronic disease management, nutrition support and vitamin D. NHS England service specifications were helpful for understanding MDT expectations within sickle cell services.
From a haematology perspective, I rely on British Society for Haematology (BSH) guidelines, especially around transfusion and iron overload, which directly influence nutrition decisions. I also use resources from the Sickle Cell Society and ASCAT (Academy for Sickle Cell and Thalassaemia).
My practice also uses BDA resources, Food Fact Sheets and specialist group guidance, as well as local trust policies for nutritional support and Food First advice sheets – and I signpost patients to local weight management services where appropriate.
Conclusion
Despite the gaps in nutritional management guidelines, policy and practice in sickle cell nutrition, the appointment of the first specialist dietitian role in sickle cell as part of the SCD MDT provides clear evidence for the need to integrate nutrition and dietetic care into patient pathways.
This project has given people living with sickle cell access to tailored nutritional care, to improve patients’ experience and outcomes.
Case study: Seven-year-old male, telephone review with mum
Reason for referral
-
Pharmacist and dietitian
-
Mum requesting advice on additional nutritional supplements, healthy eating advice
-
Attended pill school – lead by MDT pharmacist to help encourage children to transfer from liquid medications to tablets
Diagnosis
Sickle cell disease. Genotype: HB SS
Medical history
-
Nocturnal enuresis
-
Bacterial urinary infection
-
History of recurrent tonsillitis
-
Asthma
Past medical surgical procedure
Lumbar puncture (2022)
Medications
-
Hydroxycarbamide – Take ONE 500mg capsule with TWO 100mg tablets once daily (Total dose = 700mg)
-
Phenoxymethylpenicillin 250mg/5ml liquid – 250mg (5ml) BD
-
Folic acid 2.5mg/5ml – 5mg (10ml) once daily
-
Paracetamol liquid – PRN for pain
-
Ibuprofen liquid – PRN for pain
-
Macrogol sachets
-
Movicol Paediatric Plain oral powder 6.9g sachets
-
(Norgine Pharmaceuticals Ltd), 1–2 sachets daily, when required if the stools are hard as per haematology letter on 02/12/25, 30 sachet
-
Salbutamol 100micrograms/dose inhaler CFC free, 1–2puffs PRN 2–6 hourly as needed
-
Over the counter (OTC) medicines and supplements: Multivitamin Optibac probiotic
Social history
-
Lives with parents and two older siblings
-
Attends school. Doing well. Good attendance. Enjoys playing football. Has a sickle cell care plan. However, mother is concerned about the school’s hygiene standards and does not feel school manages his sickle cell well, especially in extreme weather temperatures
-
Last MRI: No signs of progressive disease
-
Mobility: Usually active but can suffer with fatigue a bit
-
Bowels/gastro issues: No issues reported
-
Known to paediatric sickle cell nurse and care plan in place
Anthropometry
Weight: 28.9kg (23/06/2026) +1.12 SD 86.9%. Mum reported from last review that weight is increasing.
Height: 1.32m (23/06/2026) +1.30 SD 90.4%
BMI: 16.6kg/m2 +0.59 SD 72.2%
Weight history
10/03/26 27.4kg (84%, Z= 1.00)*
02/12/25 25.8kg (79%, Z= 0.80)*
HEIGHT HISTORY
10/03/26 1.304m (91% Z= 1.35)*
02/12/25 1.282m (90% Z= 1.26)*
Diet history
-
Mum reported that the patient can be really fussy, but feels more recently eating more varied foods.
-
Confirmed not eating non-food stuff (no evidence of pica)
Diet recall
-
Breakfast: smoothie – Greek yogurt with handful of blueberries and almond and peanut butter
-
Lunch: School meal – canteen foods – unsure of exact foods eaten
-
Evening Meal: Rice or pasta with chicken goujons/breaded cod and vegetable (broccoli/cauliflower/corn on the cob)
-
Fluids: 2 x 500ml water at school (but sometimes comes back with only one 500ml completed). Blended apple and carrot and ginger juice (homemade)
-
NB: Mum reported to be upset with school as during heatwave – yesterday extremely hot and went to school with x1 bottle of water where he should have had x2 bottles. Had a meeting with teaching assistant to ensure he drinks lots
Discussion
-
Discussed food sources for folate, zinc, vitamin C and protein
-
Overall, originally described by mum that patient is a fussy eater, but feels that very recently is now experimenting with new foods and flavours, which is encouraging
Nutrition diagnosis
Impression: Recently including new foods and having a varied diet
Aim: To continue to offer nourishing foods to meet requirements
Plan
-
Encouraged to continue with offering varied foods. Mum doing well with encouraging a varied diet and trying to include more fruit and vegetables in the day
-
Noted that sickle cell paediatric nurse will be in contact with school for care plan and reinforce need to encourage drinking water
-
Invite to attend education and play session – provided by the sickle cell community team
-
Agreed to review again and aim for face-to-face review in clinic. If progress made with increasing food variety with no additional concerns to diet, then agreed to discharge from MDT review
References
1 Matthews C. Co-developing a health literacy framework to integrate nutrition into standard care in SCD. Cambridge: Anglia Ruskin University; 2023.
2 Hyacinth H.I., Gee B.E. and Hibbert J.M. The role of Nutrition in Sickle Cell Disease. Nutrition Metabolic Insights, [pdf], 2010, 3, pp. 57-67.
3 Matthews C. Sickle Cell and Other haemoglobinopathies. In Manual of Dietetic Practice, 7th Edition (eds M. Hickson, K. Maslin, L. Wedlake, D. Mellor and A. Brown); 2026. https://doi.org/10.1002/9781394206148.mdp0083.
4 Sickle Cell Society. Standards for the Clinical care of adults with sickle cell disease in the UK. [pdf], 2018, London: Sickle Cell Society. Available at: <https://www.sicklecellsociety.org/wp-content/uploads/2026/06/Standards-for-the-Clinical-Care-of-Adults-with-Sickle-Cell-in-the-UK-2018.pdf>.
