Self-care of men with priapism and sickle cell disease

Objective: To identify self-care demands of men with sickle cell disease and priapism and describe self-care measures in light of Orem’s Self-Care Theory. Method: This is a descriptive exploratory study with qualitative approach conducted with nine men with a history of sickle cell disease and priapism. Data were analyzed using Orem’s Self-Care Theory. Results: Some demands were identifi ed: from universal self-care diffi culty in social interaction and solitude, changes in self-image, self-esteem and sexual activity; from development the experience with priapism and little knowledge about the pathophysiology of the disease; regarding health deviations pain crises. Conclusion: Orem’s theory allowed to identify self-care demands, which are essential for the nursing care provided for men with priapism. Nursing has an essential role in the measures for the different demands presented. Descriptors: Sickle Cell Disease; Priapism; Nursing Theory; Self-Care; Nursing Care. RESUMO Objetivo: Identifi car as demandas de autocuidado de homens com doença falciforme e priapismo e descrever as medidas de autocuidado à luz da Teoria do Autocuidado de Orem. Método: Trata-se de uma pesquisa descritiva, exploratória, de abordagem qualitativa, com nove homens com história clínica de doença falciforme e priapismo. A análise dos dados foi realizada por meio da Teoria do Autocuidado de Orem. Resultados: Foram identifi cadas algumas demandas: de autocuidado universal difi culdade de interação social e solidão, alterações na autoimagem e autoestima e atividade sexual; de desenvolvimento a experiência com o priapismo o e pouco conhecimento sobre a fi siopatologia da doença; em relação a desvios de saúde crises de dor. Conclusão: A teoria de Orem possibilitou identifi car as demandas de autocuidado, que são essenciais para cuidado de enfermagem a homens com priapismo, e a importância da enfermagem frente às medidas para diferentes demandas apresentadas. Descritores: Doença Falciforme; Priapismo; Teoria de Enfermagem; Autocuidado; Cuidados de Enfermagem. RESUMEN Objetivo: Identifi car las demandas sobre autocuidado de hombres con enfermedad falciforme y priapismo y describir las medidas del autocuidado a la luz de la Teoría de Orem. Método: Se trata de una investigación descriptiva, exploratoria, de abordaje cualitativo, realizada entre nueve hombres con historia clínica de enfermedad falciforme y priapismo. El análisis de los datos se llevó a cabo según la Teoría del Autocuidado de Orem. Resultados: se identifi caron algunas demandas: del autocuidado universal: difi cultad de interacción social y soledad, alteraciones de la autoimagen y autoestima y actividad sexual; del desarrollo: la experiencia con el priapismo y el conocimiento insufi ciente sobre la fi siopatología de la enfermedad; sobre desviaciones de la salud: crisis de dolor. Conclusión: La teoría de Orem permitió identifi car las demandas del autocuidado, esenciales para la atención de enfermería en hombres con priapismo y resaltó la importancia de la enfermería acerca de las medidas a ser tomadas en las diferentes demandas. Descriptores: Enfermedad Falciforme; Priapismo; Teoría de Enfermería; Autocuidado; Cuidados de Enfermería. Self-care of men with priapism and sickle cell disease Autocuidado de homens com priapismo e doença falciforme Autocuidado de hombres con priapismo y enfermedad falciforme Ueigla Batista da Silva E-mail: ueigla.sillva@gmail.com CORRESPONDING AUTHOR


INTRODUCTION
It is estimated that 40% of adult men with sickle cell disease (SCD) have at least one episode of priapism during their lifetime.This can cause sexual impotence and negatively influence sexuality, self-care and quality of life (1) .In SCD, priapism is associated with low hemoglobin levels and changes in hemolytic activity: reticulocyte count, bilirubin, lactic dehydrogenase and aspartate aminotransferase (AST) (2) .
Priapism is a condition in which men get a persistent erection, not accompanied by sexual desire or stimulation, which usually lasts for more than 6 hours and typically involves only the cavernous body of the penis.This condition is a urological emergency and its inappropriate management can lead to erectile dysfunction, sexual dysfunctions and difficulties with sexuality (2) .This condition requires knowledge from the health team to provide proper care and promote self-care.
Classically, there are two main types of priapism.Ischemic priapism (low flow), anoxic, accounts for 95% of cases and is a result of sinusoidal thrombosis and venous occlusion with little or no cavernous blood flow.A rapid treatment prevents cavernosal fibrosis and erectile dysfunction.Its causes may be hematological, pharmacological, neurological and malignant (3) .Non-ischemic (high-flow) priapism is less common than ischemic.It is associated with penile, perineal or pelvic trauma, which generates an arterial abnormality, which in turn leads to uncontrolled arterial inflow directly into the sinusoidal spaces of the penis (2) .
Among the aetiologies of priapism, sickle cell disease (SCD) is highlighted.Data from the Ministry of Health reveal 6% to 10% prevalence of SCD in the north and northeast regions.In the state of Bahia, the incidence is 1 case for every 650 live births and 1 case of sickle trait for every 17 live births (4) .The high morbidity and mortality rate of this pathology is a limiting factor for quality of life (5) .
Low flow priapism in SCD is explained by compromised venous drainage as a consequence of vascular blockage by deformed red blood cells.More than 1/4 of the episodes of priapism in urological practice are caused by SCD.The most common precipitating factors are sexual activity, including masturbation, dehydration, fever and exposure to cold temperature (6) .
In men with SCD, the occurrence of priapism causes negative feelings, low self-esteem, difficulties with sexuality and conflicts in relationships, since virility is called into question.It changes the rhythms of the affective life, limits the sexual relations, interferes in the person's self-esteem and leads to the occurrence of other complications (1) .
These restrictions cause difficulties in social relations and in self-care.In addition, obstacles caused by illness require adaptations and coping strategies to accept the body image and changes in sex life (7) .
Thus, nursing care for men with SCD who have priapism should include the possibility of negotiation, so that they become active in therapeutic care and contribute to self-care.In order to integrate the practices of health professionals, it is essential to comprehend their experiences and how they perceive their illness (8) .
In order to understand the complexity and the mechanisms involved in the self-care of men with SCD and priapism, the Self-Care Theory (SCT) developed by nurse Dorothea Elizabeth Orem will be used.SCT is one of three theories that form the Self-Care Deficit Theory of Nursing (9)(10) .It has been used in studies carried out in nursing, especially in primary care, where prevention and self-care are fundamental.SCT is based on the assumption that people are able to take care of themselves and, in cases where there is a deficit, patients are stimulated to develop self-care practices in order to preserve life and personal well-being (11)(12) .
The Self-Care Theory presents specific demands that must be fulfilled: the universal demands, such as water intake and the balance between solitude and social interaction; developmental demands, associated with processes and events that negatively influence healthy individuation; and health deviations, which can hinder people's ability to practice self-care and are evidenced when diseases are present.When these demands are not met and self-care deficits are identified, the determinants for the functioning of the Nursing Systems Theory are established (11)(12) .
Nursing has an important role in the care of men with SCD and priapism, as it promotes self-care through health education aimed at the empowerment of these individuals, identifies and develops therapies for symptom management, acts on pain control and relief and adopts a holistic approach in the care of people with sickle cell disease (13) .
Thus, the onset of priapism can significantly alter sexual and love life, self-esteem, and self-care.The construction of masculinity with the social heritage of patriarchy determines behaviors based on virility and power, which causes suffering to men when they are affected by pathologies that expose them and confront them with this reality.These symptoms and pathologies may hinder men's self-care and access to health services.Thus, we have as a guiding question: What actions do men with sickle cell disease take as self-care measures to avoid priapism and worsening of their health status and to improve quality of life?
This research is justified since it explores the health of men with SCD.This topic is still little addressed, so the study can give a greater visibility to this condition and improve the scientific knowledge in nursing and other health areas.It should be emphasized that the Brazilian population is mainly composed of Afro-descendants, which justifies the high prevalence of sickle cell disease and men with priapism.Thus, this study may encourage reflection among health professionals about the structure of services that assist men with SCD and priapism, as it can also promote the evaluation and redirection of health actions focused on care practices and survival conditions.

OBJECTIVE
To identify self-care demands of men with sickle cell disease and priapism and describe self-care measures in light of Orem's Self-Care Theory.

METHOD Ethical aspects
The research was approved by the Research Ethics Committee of the Nursing School of the Federal University of Bahia (EEUFBA).The study meets the recommendations proposed in Resolution 466/2012 on research involving human beings.
Self-care of men with priapism and sickle cell disease Costa DO, Araújo FA, Xavier ASG, Araújo LS, Silva UB, Santos EA, Ferreira SL.

Study type
It is a descriptive exploratory study, with qualitative analysis.

Study scenario
The population of the state of Bahia is mainly Afro-descendant, which justifies the high prevalence of sickle cell disease.For this reason, the study was carried out in two outpatient clinics: the Municipal Outpatient Clinic for Viral Hepatitis and Sickle Cell Disease in Salvador and the Municipal Outpatient Clinic for People with Sickle Cell Disease in Feira de Santana, both located in the state of Bahia.

Data sources
The analysis of the medical records found 09 men with a clinical history of priapism.The following aspects were adopted as inclusion criteria: being over 18 years old, having confirmed diagnosis of SCD, presenting priapism as a complication and being registered in one of the services for people with SCD in the state of Bahia.The men selected were identified through the chart numbers in the appointment agenda and in the minutes book.They were then personally contacted during their consultation in these services for the invitation and scheduling of the interviews at the clinics, according to the participants' availability.

Data collection and organization
Empirical data were obtained through semi-structured interviews.The collection instrument contained questions about the characterization of participants and questions regarding the episodes, their frequency, duration, treatment and complications associated.
The researchers were trained to collect the data, to approach the participants, and to conduct extension activities in the study scenarios.Then, a semi-structured interview with a mean duration of 30 minutes was recorded with those who agreed to participate in the study after reading and signing the Informed Consent Term (TCLE).The interviews took place in the outpatient clinics from April to August 2015.

Data analysis
The statements were treated by the Bardin content analysis method, under the category of Thematic Categorical Analysis, based on three stages: pre-analysis, exploration of the material, treatment of results and interpretation (14) .This method is a valuable framework for analyzing diverse contents, as it allows reliable inferences to the reality and the health needs of the communities studied.
During the analysis, the importance of using Orem's Self-Care Theory to categorize and group the data became evident, since the demands of self-care are extremely important for patients with sickle cell disease.Initially, universal, developmental and health deviation demands were identified and determined, and then the demands raised and the respective self-care actions were analyzed.

RESULTS
Nine men with diagnosis of SCD participated.The predominant age group was 30-40 years old.None of the interviewees lived alone at home, but three were single.Regarding race, all participants declared themselves black.Regarding place of birth, five are from Salvador and four from Feira de Santana.Of those interviewed, two did not finish elementary school and two finished high school.The painful episodes and other complications of the disease in daily activities, especially for the younger, directly interfered in attendance to classes and sometimes led to loss of the school year or even dropout.
Family income ranged from one to four minimum wages and only one had an income above five minimum wages.Due to a series of complications related to SCD, these men are subject to several limitations, which may force them to resort to informal work.Low schooling due to the interruptions of school years during childhood and adolescence and also due to the large number of hospitalizations leads to labor market exclusion.
Only one interviewee experiences parenting, despite the fact that SCD causes conflicts and insecurities about procreation.The predominant religion was Protestant.All the interviewees reported an episode of priapism and had homozygous hemoglobin S (HbSS).This complication in the life of a person with sickle cell disease is due to the pathophysiology of the disease, which is characterized by vasoconstriction.
Three categories were identified from the analysis of the findings: Universal self-care demands, Developmental Self-care demands, and Health deviation self-care demands.
Universal self-care demands Difficulties in social interaction and loneliness were reported by the participants and associated with priapism, which revealed negative feelings about life and interpersonal relationships.In one report, the experience with priapism led the participant to think of suicide as a way to ward off suffering.

I try not to leave the house, when it happens during the day I stay at home, because if you try to put on underwear it really bothers you, because there is no way to lose the erection. It is very difficult to talk about priapism, very difficult. (E03)
Priapism is humiliating; when you arrive somewhere and people make fun of you it's humiliating, and if you are weak in the head you can do something stupid.I've tried to take my life, but God is faithful.We learn slowly.(E06) The episodes of priapism brought changes in self-image, selfesteem and in affective relationships and caused insecurity, since participants reported that there is no way to predict the occurrence of a new episode.If the crisis occurs in the work environment or other environment that does not allow them to take routine care actions, these men are exposed to jokes and embarrassment.

What men value the most is their masculinity. A person that doesn't have a good head can do something stupid, you understand?! (E06)
But even if your partner understands you, you still have that feeling of impotence, you feel you do not measure up to your partner, understand?(E08) Difficulties in sexual activity were reported by most of the interviewees.The occurrence of priapism led to sexual and Self-care of men with priapism and sickle cell disease Costa DO, Araújo FA, Xavier ASG, Araújo LS, Silva UB, Santos EA, Ferreira SL.
social problems, such as reduced frequency of sexual practices and fear of sexual impotence.
After priapism the frequency of sexual relations reduced, it got less often, the erections were failing.In the old days, well before I had priapism, I had normal relationships, it was okay... now it's changed.(E02) Because I'm in a situation where I can not find a girlfriend, so I gave up having a relationship for a while, I gave up dating, I gave up trying to raise a family.(E06) Developmental self-care demands Developmental self-care demands are related to the actions and experiences carried out by the individual to promote selfcare.Thus, the little experience with priapism evidenced in the participants' reports resulted in few self-care measures taken at the time of the crisis.These cares were mostly the use of analgesics, followed by cold and warm baths, hydration, local massage and masturbation.The last one I had, it stayed for 72 hours; it used to come and I would take a shower, after a little while it would calm down, but not now (E06).

Hot bath and analgesics
Knowledge about the pathophysiology of the disease was weak.The lack of knowledge about priapism and pharmacological and non-pharmacological treatment is still frequent among people with sickle cell disease.In addition, it was evidenced in the reports that men sometimes confuse erection due to sickle cell disease with sexual stimulation and adopt inadequate care for clinical improvement.In addition, the lack of knowledge provoked a feeling of shame regarding these situations, which, for this reason, are hidden from their relatives.

Health deviation self-care demands
The pain that accompanied the erections described by men occurred more often in the night time, while they were asleep.Participants reported severe pain, with an average duration of one hour and sudden onset, with no association with fever or other symptoms.
The first crisis occurred in the middle of the night, between 2 and 2:30 in the morning and it is an involuntary erection.You wake up and you don't know what's happening to your body, then you start to feel the pain.It's a pain that's kind of like a stitch; it feels like a needle piercing the urinary tract.

It hurts too much, it really hurts. (E01)
It's usually more at night when I'm sleeping.Then when I wake up there it is, that strong, intense pain.It takes about an hour or so, sometimes it takes longer, it depends.(E02) For me the episodes are short, but it's very daunting and I feel a lot of pain.(E07)

Universal self-care demands and self-care actions
The negative feelings provoked by the episodes of priapism resulted in impaired social interaction and risk of loneliness, according to the reports of the participants.The impaired social interaction is due to the fact that episodes happen suddenly, so it is not possible to predict when they will happen.As a result, men were insecure about the possibility of a new episode and, in some cases, opted for a lack of social life.They also mentioned difficulties to have affective relationships and long lasting bonds necessary to form a family.
One of the interviewees reported that during a crisis, one of the people waiting for health care questioned his erection, associating with acts of indecent assault.Although the prolonged erection is involuntary, some men opt for isolation and a more domestic life, since the looks and accusations of people who do not know the cause of the problem interfere negatively in the social life.
Some nursing interventions should be adopted to help improve social interaction and eliminate loneliness, such as: providing support for maintaining basic social skills and reducing social isolation, offering group support therapy, helping the family to understand and offer support, reducing or eliminating causative and contributing factors, reducing barriers to social contact and encouraging the patients to talk about their feelings (15) .Also as a consequence of priapism, men's self-image and selfesteem were altered (16) .The reports evidence the participants' sense of powerlessness, since it is socially constructed that men should be strong and virile.However, this fragility is related to the change in their image and the decrease in self-esteem.A survey carried out in three cities in Bahia revealed that, behind the stereotype of a strong man, these men see themselves as fragile and weak because of SCD and its marks (17) .
Low self-esteem may be related to the fact that they present a dysfunction or alteration in their sexual performance, failing to meet the expectations of the society for men.Men with priapism fear self-assertion and are afraid of becoming sexually impotent, which would hurt their principle of being virile and potent and make them lose their manhood.In Brazilian society, with its heteronormative patterns, sexuality happens through the attraction between opposites, and the construction of masculinity is crossed by insecurity, represented by the fear of impotence.
Overcoming established social paradigms may be a difficult task, since most of the time these men suffer vexatious treatment and are often confronted about their virility.These circumstances can damage even more the patients' self-esteem and self-image, which can go to extremes, such as in the report of possibility of suicide.
Therefore, nursing care should be designed with the aim of increasing self-esteem and improving the patient's self-image.Thus, a trust relationship must be established between the nurse and these patients, and the nurse should have attitudes such as encouraging the patients to express their feelings, promoting social interaction, supporting the family through adaptation, stimulating patients to share their feelings with significant people, trying to reduce anxiety, identifying strong areas of the patients' personality and involving them in group therapy support (15) .
More than half of the men reported deficits in sexual activity.It was possible to perceive that priapism can men end affective relationships due to the complications of sexual dysfunction, such as difficulties related to erection, desire and orgasm.Sexual impotence causes feelings like insecurity and anxiety before the sexual act and limits the maintenance of relationships.
The occurrence of priapism may delay the experience of sexuality, as was the case of one of the men who reported he hadn't have any sexual experience due to sexual dysfunction related to priapism.In this sense, care becomes even more necessary, since the consequences of priapism involve areas such as self-esteem, self-image, social isolation, loneliness, insecurity, feelings of impotence, unsleep, among others (1) .
To address this deficit, some measures should be established, such as investigating the causative or contributing factors, reaffirming the need for a frank discussion between sexual partners, teaching possible changes in sexual practices to deal with the limitations and encouraging the patients to share their concerns (15) .

Developmental self-care demands and self-care actions
In some cases, priapism episodes can be treated by the individual himself, with the use of analgesics, oral hydration, hot bath and physical activity before seeking specialized care (17) .No literature was found to support masturbation and cold bath as self-care measures against priapism, as reported in some discourses.
The reports evidenced that the patient needs support and nursing education to acquire skill and competence to take selfcare actions according to their needs.It should be clarified that priapism is a clinical manifestation that may appear at some point in the life of men with sickle cell disease.The first episode of priapism can happen suddenly and without knowledge about the pathological event, causing discomfort, fear and insecurity.This initial experience may, however, be important for a better understanding and recognition of the following episodes.
The learning process includes a gradual extension of the repertoire of self-care practices and related skills.This happens through observation of the care provided by the nurse or through specific and planned learning experiences.The professional and the patient must agree on the development of self-care activities and the main objective is to promote the patient to caregiver agent.
With this, the patient with this symptom can acquire enough knowledge to provide self-care in a way that alleviates the priapism episodes.
Knowledge about the pathophysiology of the disease was weak.The lack of knowledge about priapism and pharmacological and non-pharmacological treatment is still frequent among people with sickle cell disease.In addition, it was evidenced in the reports that men sometimes confuse erection due to SCD with sexual stimulation and adopt inadequate care for clinical improvement.
In one of the reports, sexual intercourse was a precipitating factor for priapism, which did not allow the couple to identify the event.Besides sexual activity, which increases blood circulation and cause retention of erythrocytes in the penis, authors point to dehydration, fever and cold temperatures as other possible causative agents (1) .
Health deviation self-care demands and self-care actions Pain during the occurrence of priapism was reported by all men, and it was characterized as sudden and intense.This fact corroborates the findings of a North American study that points to painful erection in ischemic priapism, which should be treated as soon as possible to avoid injuries (18) .
A period of more than four hours of ischemic priapism may result in sexual impotence and fibrosis of the corpus cavernosum (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) .Therefore, rapid measures must be taken in order to avoid or minimize the risk of complications.As a starting point for the intervention, the health professional must evaluate the needs of the patient with SCD and recognize him as the subject of care.
Some self-care measures should be taken in the first three hours after onset of priapism at home, such as: using pain assessment scales; hydration by mouth; not having sex at the time of the episode to avoid any further blood supply; taking analgesics according to medical prescription and pain threshold; taking a warm bath to promote vasodilation; paying attention to worsening of symptoms; seeking multi-professional hospital care after a period of more than four hours of erection (19) .
In the hospital environment, other care actions are performed, such as: venous hydration; use of pain assessment scales; administration of intravenous analgesics; checking the type of priapism, whether ischemic or not ischemic or recurrent; drainage of the corpora cavernosa (19) .Health professionals must understand the treatment manuals and consider the individuality of the patients, since each one responds to the experience in a very peculiar way (20) .With a holistic approach, the health professional can contribute to improving symptoms and diseases caused by SCD.
The men interviewed reported difficulty to communicate the occurrence of priapism to health professionals and to their relatives.This can result in complications due to lack of adequate treatment, impairing the quality of life of this public.A qualified listening must be used by health professionals who work to promote self-care.
Self-care of men with priapism and sickle cell disease Costa DO, Araújo FA, Xavier ASG, Araújo LS, Silva UB, Santos EA, Ferreira SL.
The measures adopted by nursing professionals should provide knowledge about priapism, clarifying how it works: there may be painful and prolonged penis erections unrelated to sexual desire; it occurs due to obstruction of the vessels by sickle red cells that irrigate this organ; usually the penis becomes reddish, swollen and extremely painful.
Priapism is an urological emergency and if it is not properly and quickly managed it can lead to sexual impotence.It is important to pay attention to the approach, which must be very careful and ethical, since the situation involves the sexuality of a patient.It should not be treated with irony, severity or jokes, as it can be detrimental to the psycho-emotional development of the patient.
This approach helps the patient to acquire knowledge, vides greater psychological and physiological comfort and helps creating strategies to cope with the episodes and to overcome fear and shame in situations that many cited.

Limitations of the study
The evaluation of the behavior of nursing professionals regarding reproductive and sexual issues in the context of sickle cell disease, based on the reports of the participants of this study, led to the conclusion that there is still a deficiency in professional training regarding the disease and its complications and management.More qualified professionals lead to more quality in the care provided, in professional-user communication and in the information transmitted to the family.These matters can improve the self-care taken by men with sickle cell disease and, above all, tend to reduce the episodes of priapism and its complications.
Contributions to the area of nursing Through Orem's theory, this study evidenced factors that interfere in the care and self-care of men affected by priapism.This study can contribute scientifically to the nursing team that directly cares for patients affected by health problems, since nursing professionals must recognize that their practice needs to be based on scientific knowledge so that their actions can lead to the improvement of the health of the individual and of the community.The construction of theories in nursing seeks to relate facts to form a scientific basis, abandoning empirical actions and facilitating the work of the health care team.

FINAL CONSIDERATIONS
Orem's Self-Care Theory allowed identifying the universal, developmental and health deviation self-care demands.Considering that sickle cell disease is chronic, the self-care proposed by Orem's theory is essential in the care of men with priapism.
The lack of knowledge about priapism interferes in self-care during episodes and results in complications and aggravation of sexual and social symptoms, such as sexual dysfunction, sexual impotence, changes in self-esteem and self-image, fear of sexual rejection and insecurity in the face of a new imminent crisis.The lack of knowledge hinders the identification of the erection originated from priapism, which can be seen as a sexual stimulus and lead the individual to adopt inadequate care for the situation.
During the episodes, the men adopted pharmacological and non-pharmacological care, such as analgesics, warm bath, oral hydration, cold bath and masturbation, some of which had no recommendations in the literature.
It is possible to notice that the insecurity during the episodes of priapism is related to the lack of knowledge on the subject.In this sense, health professionals play an essential role in the care of this public, especially the nursing team, which is directly involved in patient care and can promote care and self-care in acute priapism events and other diseases related to SCD.
The assistance provided to these users must be based on a qualified listening, sensitive to their needs and with attention to the affective and sexual dimensions impaired by the priapism episodes, and it must be aimed at improving quality of life and providing a comprehensive the care.
are indicated.There is no formula.It's just that, hot bath and if it stays there you have to drain it.Then you run the risk of becoming impotent... (E01) Usually when I have crisis I get up, I go under the shower, I shower, I drink a lot of water and I just sit there waiting, you know?(E 02) [...] I never took medication for that.I tried to take a cold shower to see if it would get better and sometimes I would masturbate, then after the ejaculation it went down.It's such an inexplicable, very painful erection.(E03) I massaged it and took medication.(E05)