Self‐management recommendations for sickle cell disease: A Ghanaian health professionals' perspective

Abstract Objective To describe self‐management recommendations for sickle cell disease (SCD) care among health professionals who manage SCD in Ghana. Method Nine health care professionals (nurses, doctors, and physician assistants) who work in SCD were interviewed. The semistructured interviews were recorded, transcribed, and analysed using the qualitative content analysis method. Self‐management recommendations were conceptualised as preventive health, self‐monitoring, self‐diagnosis, self‐treatment, and self‐evaluation. Results Preventive health recommendations were the commonest, where the professionals described similar topics including avoidance of cold temperature, frequent oral hydration, and healthy nutrition. Self‐monitoring recommendations included regular checks for pallor, urine colour, and splenic enlargement. Self‐diagnosis recommendations were captured as warning signs and included pain, fever, unusual feelings, and enlarged spleen. Pain and fever management were the focus of most self‐treatment advice, and there were some self‐treatment recommendations for dactylitis, anaemia, and priapism. There was considerable variation in the strategies recommended for the management of individual SCD‐related problems. Conclusion Ghanaian health professionals involved in SCD care provide limited and inconsistent self‐management recommendations. There is a need for the development of SCD standards and guidelines that support effective self‐management. Health professionals working in SCD require continuing education in self‐management.

the foundation of self-management, health professionals should provide self-management support, including education and skills, social facilitation, and equipment. [20][21][22] Effective support by health professionals optimises patients' self-management, thereby contributing to improvements in patients' health outcomes, the rational use of health resources, and a reduction in health care costs for LTCs such as SCD. 23,24 Internationally, SCD self-management research has generally been conducted among patients [25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42] and little has been done to elicit the perspectives of health professionals. Currently, no internationally agreed best practice standards have been published to guide health professionals to support SCD self-management. However, it is argued that providing advice to patients on certain lifestyle factors, such as avoiding cold weather, rigorous exercise, and dehydration, and engaging with health professionals, is helpful in reducing crisis and complications. 7,12 In Ghana, as no structured patient-focused education is provided for SCD self-management, the education patients receive from health professionals is dependent upon the professionals' knowledge and skills. Until now, no study has examined the selfmanagement education Ghanaian health professionals provide for SCD care. This paper reports on a study which investigated self-management recommendations for SCD care among health professionals in Ghana. Understanding self-management from health professional's perspectives is important to establish what advice is given, whether there is consistency in the messages provided, and to identify areas for further education.

| Design
This study forms the second stage of a 4-stage sequential mixed method study that investigated self-management strategies among people living with SCD in Ghana. 43 The study used a descriptive exploratory approach with interviews and qualitative content analysis. 44,45 This method was useful, as little is known about SCD self-management in Ghana and no suitable instrument was located in the literature that could be contextualised to the Ghanaian situation. The study was conducted in a teaching hospital and 3 district hospitals in the Ashanti region of Ghana. The Ashanti region was chosen because until 2016, it remained the only region in Ghana screening newborns for SCD in the public health system and held organised SCD clinics in district hospitals. Ethical approval was obtained from Victoria University of Wellington, the Ghana Health Service, and the Komfo Anokye Teaching Hospital. Self-management was conceptualised as actions involving preventive health, self-monitoring, self-diagnosing, self-treatment, and self-evaluation.

| Participants
The participants included nurses (n = 6), doctors (n = 2) and a physician assistant. To be eligible for selection, participants had to have at least 3-month experience in providing clinical care and patient focused education or psychosocial care to patients and families at SCD clinics or the SCD Association ( Table 1). The 9 participants were purposively selected. They were the only health professionals available who met the inclusion criteria in the Ashanti region.

| Procedure
As phone interviews from New Zealand were the means to conduct the interviews, a local coordinator assisted with recruitment. The coordinator first contacted the potential participants to inform them about the study and distributed consent forms and information sheets.
The principal investigator (PI) then followed up with phone calls and e-mails to solicit agreement to participate in the study.
The interviews were all carried out by the PI and followed a semistructured interview guide. The interview began with an overarching question, "what do you tell your clients with SCD and their families to do for SCD care at home?" Specific questions were asked to capture responses that reflected the topics (preventive health, self-monitoring, self-diagnosis, and self-treatment) in the conceptual framework of the main study. Questions included (1) what they ask patients to do to keep well, (2) how they ask patients to monitor their health, (3) how they advise patients to recognise a sickle cell-related problem, (4) what actions they advise patients to take when there are problems, and (5) what self-management practices they have observed in their clinical practice. Each interview continued until information redundancy was observed or when a participant had no more information to share. The interviews were audiotaped and transcribed verbatim.

| Analysis
The analysis process involved a 10-stage iterative process of deductive and inductive approaches informed by 2 publications on qualitative content analysis. 45,46 The analysis was undertaken by the PI under the supervision of the other 2 authors. The process involved (1) using the conceptual framework (preventive health, self-monitoring, self-diagnosis, self-treatment, and self-evaluation) to describe selfmanagement; (2) identifying

| Results of self-management recommendations
All 9 participants reported having discussions with patients about selfmanagement, but the extent of discussions varied. Preventive health and self-treatment of painful episodes were the areas that were comprehensively described by the participants. Healthy nutrition "And then we also advise them to take well-nourished diet … They need good nutrition to be able to develop well because their red blood cells are being destroyed at a faster rate almost every 21 days so they need to replenish whatever they are losing." Frequent oral hydration "I think we generally advise them to take a lot a lot of fluids because most of the common complications they come with is VOC." Personal hygiene "We advise them that at home what they should observe is their personal hygiene. You know when they observe personal hygiene, it will help them. So that it will not trigger them." Supportive medicines "When their condition is stable we give them their routine folic acid and penicillin V … we educate them to take it every day. Even when they cannot come to the clinic they should go and buy some and continue taking it." Interaction with health professionals "So, if we take the vaso-occlusive events it can happen in the form of painful crisis where the patient complains of pains in the joints and … it is very obvious because the person will be screaming and when that happens depending on the level of pain, we advise them to the hospital for hospital management." Avoiding extremes of temperature "we advise them to avoid extremes of temperature, not to take too much cold water, very cold water, not to play in the rain when it's raining." Avoiding overactivity "We also advised them of course to desist from strenuous work, they should know their limit and should know when they have reached their limit" Avoid diseases that complicates SCD "and they should also avoid infection, for example malaria and worms so that they wouldn't get anaemia." Avoid injuries "And when they are playing football, the boys they should avoid contact as much as possible... When you playing ball make sure nobody touches your stomach. They should protect themselves." Abbreviations: SCD, sickle cell disease; VOC, vaso-occlusive crises.

Self-monitoring
Pallour check among children "they [parents] should be observing the child from time to time for example the conjunctiva and the palm for colour change so that when the baby is getting pale they can compare the baby's palms to theirs they can see the difference in terms of colour." Pallour check among adults "We educate them on how to check the conjunctiva for anaemia that is how they will get to know the warning. They will stand in the mirror and check if your eyes are yellow and you check your conjunctive and it's pale like you look in your palms and you are pale you can report to the hospital." Splenic palpation (when there are no enlargements) "watch out for the spleen. If the child is a new-born their mothers are taught how to palpate the spleen. If he is a young adult or an adult, again we teach them how to palpate the spleen so that they will be able to determine when their spleen enlarges so they can act appropriately … Then we also teach them how to palpate the spleen of the baby so that they can look out for splenic sequestration which is one of the major complications of SCD." Splenic palpation (when there are enlargements) "those who have splenomegaly, we tell them that they should take note of where the size is, so when they see that it's becoming bigger or its becoming tender they should report to the hospital." Growth monitoring "their [child's] general growth, because, some of them have delayed growth so especially those that are screened, the new-borns we tell the mothers to be observing their development if by one and half years the child is not walking, they should report to the hospital. Then we will follow her up and see if anything wrong or just the sickle cell."

Self-diagnosis
Fever "And then if it comes to the infections, of course the infections always exhibit by high fever and once the temperature is beyond 37 degrees [Centigrade] parents should know that their child is running temperature. And we make them aware of the seriousness of temperature and … that there may be an underlying infection such as pneumonia." Pallour "The skill of detecting pallor, they've been taught to look under tongue, look under oral mucosa, look at the conjunctiva, the palm of their children and whenever they see their children are pale, from the usual … steady state like pallor they should bring the child to hospital … the mothers will come, doctor, 'mehwԑԑ na ni ase no, na ni ase ayԑ fitaa' [when I look at the eyes they are pale]. We examine and do the HB [haemoglobin], you will be surprised some of them having 4.0, 3.5 and they are walking about. Because of their chronic anaemia … they are able to walk about any how with low HB." Abbreviation: SCD, sickle cell disease.

Health professionals recommendations
Heat application for pain management "They [children] may not know which parts of the body is paining so they can … keep the baby warm or put some hot compresses around every part of their body or they can even make some warm water and put the baby in the warm water." Fever and wound management "We teach them how to tepid sponge which is one of the first aid in the house is when the child has fever...administration of the drugs, we teach them and they have that skill. Sometimes we teach them a little about … how to treat the wound in the house." Priapism management "So, the male patients we tell them that as soon as they see something like that [priapism] they should drink the fluids and come to the hospital, then we manage."

Herbal and traditional practices by patients
Use of herbs "When they are in pain they boil some herbs and give it to the child … they will give some enema before they come to the hospital especially those who have abdominal distention." Weight application for pain "They will ask that a weight be put on that joint or that part or joint which again, sort of numbs the part of the body and reduces the pain that they are experiencing." Scarification to cure disease "They have also what we call the scarification that are done by traditional medicine men with the belief that sickle cell disease is a bought disease, a spiritual disease, and, therefore, by making the scarification on patients and putting in some black quotient can reduce the effects of the disease on the patients through evil manipulation. So they do the scarification and then they sort of give to the spirits." Local haematinics for anaemia "And another person told me something about a drink that is made with 'Kwawunsusua' (Turkey Berries). She boils the Kwawunsusua … boil till its green and she drinks it with anything, like she adds sugar, if you want milk you add to make it nice. According to her that is her blood tonic so she takes it like tea every morning." Conventional haematinics for anaemia "We discourage [haematinics] because most blood tonics contain iron and therefore they may be adding more iron to what they already have because we know that when the cells are getting destroyed the iron is stored in their system for use. So if you have not tested to actually show that they are lacking iron, then they are actually adding more iron by taking the blood tonic."

| Preventive health
Preventive health covered health maintenance and preventive care strategies. All 9 participants provided recommendations on aspects of preventive health (Table 2).

Health maintenance advice
Health maintenance advice clustered around 2 groups of actions patients were encouraged to perform on a daily basis. The first consisted of actions patients should perform consistently, including maintaining adequate hydration to prevent dehydration, maintaining adequate nutrition, maintaining personal hygiene, and using supportive medication such as folic acid and other prescribed multivitamins.
The second set of actions were health professional-directed actions patients should perform, such as adhering to prescribed medications, attending routine health checks, and seeking clinical care for problems.
All 9 participants reported advising on healthy nutrition. An aspect of the advice concerned the type of diet SCD patients should consume.
It was recommended that patients eat a normal diet, which must be "good," with a selection of a variety of food nutrients, such as carbohydrates, proteins, and fats. A good diet was expressed as "nutritious," "well balanced," and "nourishing" and contained green leaves, fruit, and meat. For children, 2 participants recommended breastfeeding and a normal diet and food supplements for those older than 6 months.

| Self-Monitoring
Six participants (DR1, DR2, NUR1-NUR3, and NUR6) outlined physical and physiological indicators that they advise patients to check periodically, including pallor, urine colour, fever, jaundice, splenic enlargement, pain, and general demeanour (for children). The commonest recommendations were checking pallor, urine colour, and fever (

| Self-diagnosis
All the participants used terms such as "emergency signs," "warning signs," and "complications" to describe self-diagnostic indicators.
These indicators included fever, changes in urine colour, jaundice, unusual feelings, enlarged or tender spleens, and prolonged crying in children (Table 3).

Fever and urine colour changes
There were objective and subjective recommendations to what constitutes the degree of fever that patients should recognise as problematic.
Participants either specified a temperature level of 37°C or higher (DR3 and NUR4) or advised patients to note when the body is warm to touch.
Change in the colour of urine was the second commonest indicator the participants recommended for self-diagnosis. Three participants (DR1, DR2, and NUR2) specified dark or "coca cola" urine as an abnormal sign that required attention. The participants related these changes as indicative of physiological abnormalities such as dehydration, vaso-occlusive crises, and kidney malfunctions and said they urged patients and parents to report immediately to hospital if these should occur.
Four participants (DR2, DR3, NUR2, and NUR5) referred to jaundice as a diagnostic sign and reported advising patients who developed emerging or worsening jaundice to seek immediate care.

| Self-Treatment
Pain and fever management were the focus of most self-treatment advice, and there were a few recommendations concerning dactylitis, anaemia, pneumonia, and priapism management ( Table 4).
All participants, except NUR5, reported advising on pain management strategies including the use of analgesics, increased oral fluid intake, heat applications, massage, relaxation, and periodic movement.  as well as recommended measures for healthy living and crisis prevention for SCD management. 3,5,7,[38][39][40]47,48 The findings indicate that recommendations for self-monitoring and diagnosis, using objective methods and measurements such as thermometers, splenic palpation, and self-assessment tools, are limited in the Ghanaian health professionals' recommendations. In contrast, researchers of SCD self-management in the United States have focused on objective measurements for pain self-monitoring to understand pain frequency, characteristics, related symptoms, and home management. 26,28,29,32,33,37 Typically, these researchers in mostly interventional studies have asked patients to rate their pain using assessment tools, interpret their pain experience, identify related symptoms, categorise their pain and related symptoms to predetermined categories, and record their findings into paper or electronic diaries. Self-monitoring and diagnoses were facilitated by e-health technologies in 2 studies. 29,33 In Jacob et al, 29  for adults and children in this study. We acknowledge that some recommendations may be more applicable to one group than the other.
Future research is required to categorise recommendations for different age groups. Finally, saturation of findings was not achieved. However, the study did recruit all health professionals in the Ashanti region who met the inclusion criteria. Expanding the study to other regions might have produced additional information and generated less variability amongst the advice given.

| DISCUSSION
The lack of international standards and guidelines for self-management of SCD results in arbitrary self-management advice being given by Ghanaian health professionals to patients with SCD. As self-management actions by patients can improve general health and wellbeing as well as minimise the likelihood of people with SCD developing crises, there needs to be a more consistent approach to advising on self-management actions and strategies. knowledge and patients' preferences for SCD self-management is also required internationally to understand what is important to teach patients.

ACKNOWLEDGEMENT
The authors acknowledge the Victoria University of Wellington for financial and other logistical support. We also wish our appreciation to all the health professionals who participated in the study.

FUNDING
This study was financed by the Victoria University of Wellington.

CONFLICT OF INTEREST
None.