Neill Westerdale, MSc, RSCN, RGN.
Sickle Cell Nurse Practitioner
Sickle-cell disease (SCD) is one of the most common genetic blood disorders in the world and principally affects people of African, Mediterranean and Asian origin (World Health Organization, 1985; Standing Medical Advisory Committee, 1993). In the UK, there are around 9000 people living with the disorder (Streetly et al, 1997).
The main genotype of SCD includes sickle-cell anaemia (HbSS), sickle-cell disease (HbSC) and sickle-beta thalassaemia (Sb-thal). The structure of sickle haemoglobin differs from that of normal haemoglobin (HbAA). SCD is characterised by anaemia and periodic painful episodes called vaso-occlusive crises (Embury et al, 1994).
Normal red cells (HbAA) are water-soluble, biconcave, flexible and pliable discs that can easily travel through the circulatory system carrying oxygen. The sickle haemoglobin has a different structure and is insoluble - when deoxygenation occurs, the sickling process begins. The haemoglobin alters its shape and becomes sticky and the red blood cells aggregate to form long liquid crystals. These deformed cells then create vaso-occlusions, cutting the supply of oxygen to surrounding tissue, giving rise to ischaemia, pain and tissue damage. The sickling process - or vaso-occlusive crisis - is precipitated by factors that include dehydration, infection, extreme changes in temperature, deoxygenation, and physical and emotional stress (Embury et al, 1994; Okpala et al, 2002).
Typically, SCD crises will occur in areas of the body with small blood vessels: common sites include the muscles, penis and abdomen (Embury et al, 1994).
Vaso-occlusive crises affecting bones and joints result in damage to bone tissue, which is called avascular necrosis. This complication is typically associated with the development of chronic pain in SCD (Serjeant and Serjeant, 2001). Vital organs such as the kidney, liver, spleen and brain can also become affected, giving rise to life-threatening complications such as liver, renal and splenic failure, and stroke (Okpala et al, 2002; Helps et al, 2003).
SCD and adolescence
The effects of having SCD vary greatly between individuals, which is due to a number of biological reasons, including genotype, steady state haemoglobin (the level of haemoglobin when not in crisis) and fetal haemoglobin levels (Embury et al, 1994).
Adolescents with SCD experience a rise in haemoglobin level that is thought to increase the episodes of vaso-occlusive crises (Elander et al, 2003). The majority of adolescent boys will also begin to experience the onset of acute SCD priapism, which is a prolonged penile erection. A non-sexual erection lasting more than 30 minutes is considered to be a priapism (Fowler et al, 1991). These events are unwanted, painful, and in most cases occur in the early hours during sleep (Mantadakis et al, 1999). Most teenage boys with the disease fail to realise that priapism is a complication of having SCD. If left unrecognised and untreated, priapism can result in impotency (Okpala et al, 2002).
Adolescent is a difficult time for any young person with a chronic disease. While their peers become more independent, teenagers with SCD will often need help because of the illness. Adolescents with the condition will on average have a delay in puberty of around two-and-half years. This creates significant psychological issues around delays in growth and the development of sexual characteristics (Thomas and Westerdale, 1998).
In addition, yellowing of the whites of the eyes as a result of haemolysis, problems with enuresis and having a blood disorder contribute to feelings of stigmatisation (Smith and Wethers, 1994). Disruption to education owing to hospitalisation and cognition problems from cerebral infarcts can result in adolescents with SCD falling behind in their studies (Wang et al, 2001, Helps et al, 2003).
Restrictions in sports and recreational activities will naturally interfere with normal adolescent development and increase feelings of isolation, making normal peer relationships difficult. Furthermore, worries about crises, pain management and dying are a constant worry for many young people with the condition. Anxieties about these issues are frequently expressed as depression, rebellion or refusal to comply with medical treatments, advice or plans. This may manifest itself as disruptive behaviour during hospital stays. In particular, conflicts are more likely to occur if adolescents do not receive adequate pain relief and their rights are ignored or violated, for example by not having their analgesia administered on time (Thomas and Westerdale, 1997).
Management of sickle-cell disease
In the authors’ experience, it is important to treat the predisposing factors alongside the management of pain. Psychosocial factors such as diet, poor housing, inadequate central heating, educational difficulties, personal issues, lifestyle, poor concordance with treatment and a lack of access to specialist services may in part contribute to an adolescent’s instability and may precipitate crises.
Vaso-occlusive crises are common and the majority of these events are self-managed by patients in the community (Maxwell and Streetly, 1998; Dampier et al, 2002). However, over 90% of SCD-related hospital admissions are for vaso-occlusive crises (Platt et al, 1991). Acute sickle cell pain may involve any area of the body and its severity will frequently change, as will the duration and locations of the events.
The pain associated with a vaso-occlusive crisis is often described as akin to a deep gnawing and throbbing; there is also frequently a post-crisis residual aching pain (Ballas, 1994).
Management of acute pain
Many adolescents with less severe types of SCD do not experience acute painful events. These adolescents are rarely exposed to strong analgesia such as opiates and as such are considered to be ‘opiate naive’. However, those adolescents with severe SCD, and especially those with HbSS, may have experienced many vaso-occlusive crises. Subsequently, out of necessity these adolescents will have been exposed to opiates. Such adolescents naturally will become ‘opiate tolerant’ and therefore may require larger doses of analgesia and with more frequent intervals to achieve good pain control. This is compounded by the fact that the half-life of morphine in children with SCD averages about 80 minutes, 40 minutes faster than in ‘normal’ individuals (Dampier et al, 1994).
The relief of pain is the primary objective in SCD and not the complete absence of pain as this is not normally achievable because of its nature and severity. However, titration of analgesia should continue until a clinically significant decrease in pain intensity for each individual is achieved, or until side-effects become problematic (Jacob et al, 2003). In the authors’ experience, administrating ever-increasing doses of opiates in a desperate effort to control pain may increase the risk of over-sedation and respiratory depression, which can worsen a crisis and lead to life-threatening complications. Not surprisingly, adolescents with SCD often become reluctant to use any form of strong analgesia (Dampier et al, 2002). Therefore it is strongly recommended that analgesic plans should be individualised as much as possible to meet a patient’s needs (Dampier et al, 1994; Beyer, 2000). In this way, they may be safer and more realistic.
Adolescents may also have issues such as needle phobia, poor peripheral access and non-concordance, which often will determine how analgesia can be delivered. When treating vaso-occlusive crises, the ladder of oral analgesia should be used. Short-acting opiates such as oral morphine can be effective at controlling vaso-occlusive crisis pain if aggressively titrated (Jacobson et al, 1997). In addition, oral interventions can often be enhanced by the use of non-steroidal anti-inflammatories (NSAIDs). Since many vaso-occlusive crises can affect the bones, the pain will often respond well to this combination (Grisham and Vichinsky, 1996). NSAIDs should be used with caution as SCD can affect the kidneys, which can then become more susceptible to further injury from the use of NSAIDs. For this reason, NSAIDs should be avoided in those with renal impairment or their usage restricted to short intervals. If parenteral opiates are acceptable and required, morphine is the drug of choice (Okpala et al, 2002).
Morphine can be given easily subcutaneously or intramuscularly. It has a rapid onset and a very effective half-life. The intravenous route should be avoided because of access difficulties in those affected by SCD as well as increased risks of over- sedation. Patient-controlled analgesia (PCA) is considered an optimal method of pain control within SCD and could be administered subcutaneously using a subcutaneous needle (American Pain Society, 1999).
In adolescents PCA may have other advantages, which include educating those using it to take an active role in their own pain management and treatment. However, there is evidence to suggest that PCA is successful only with proper patient and staff education alongside the existence of pain protocols for SCD (Ballas, 1994; Trentadue et al, 1998).
Chronic pain and management
Chronic pain may start to manifest itself during adolescence and is principally related to the development of avascular necrosis, especially within the femoral head (Serjeant and Serjeant, 2001). Chronic pain in SCD is persistent and is often unrecognised. It is difficult to assess and not easily distinguished from the pain of frequently re-occurring vaso-occlusive crises.
Chronic pain is typically described as pain lasting more than three to six months (McHugh and Thomas, 2001). Therefore adolescents with SCD presenting with such persistent pain should be thoroughly assessed. If avascular necrosis results in chronic pain, these adolescents may require treatment with long-acting analgesia such as morphine sulphate slow release (MST). It is also worth considering interventions such as nerve blocks, hydrotherapy, physiotherapy, and massage (NIH, 2002). Psychological interventions such as relaxation and cognitive behaviour therapy have also been used successfully in both adults and adolescents with SCD. (Ballas, 1998; Thomas et al, 1998; Eccleston et al, 2003).
A number of complex issues create difficulties for health-care professionals when assessing and managing pain in SCD. First, the pain of SCD does not typically fit the classical models of either acute or chronic pain and most patients will normally experience both types. Frequently there is little clinical manifestation such as swelling and redness during vaso-occlusive crises (Ballas, 1998). Moreover, sickle cell pain can abruptly arrive and depart, it frequently waxes and wanes and changes sites. It therefore does not behave like other common types of pain such as that associated with trauma or postoperative conditions. This often makes evaluation of pain associated with SCD difficult for health-care staff.
Furthermore, stereotypical views still persist among some health-care professionals that many SCD sufferers are addicted or likely to become addicted to opiates (Alleyne and Thomas, 1994; Maxwell and Streetly, 1998). This is despite evidence that suggests that addiction rarely occurs in those affected by SCD (Portenoy and Foley, 1986; Elander et al, 2003). These negative perceptions and complexities frequently result in those affected by SCD pain not having their pain needs met.
Therefore an important goal for assessment of both acute and chronic pain is to characterise the adolescent’s pain to provide a basis for therapeutic decisions and to document the efficacy of pain control. In view of the subjective nature of pain, any assessment undertaken requires patient self-reports and the use of valid pain tools and measurements that are repeated over time (Jacob et al, 2003). Simple measuring tools are recommended such as the African-American Oucher and the Adolescent Paediatric Pain tool (Beyer, 2000) the McGill questionnaire (MPQ) (Melzack, 1983) and the multidimensional Adolescent Paediatric Pain Tool (APPT) for either chronic pain or acute pain (Jacob et al, 2003). Pain diaries in particular are recommended for home use (Shapiro et al, 1995; Franck et al, 2002). In addition, staff and patient education is required about the nature, management and assessment of SCD pain.
Health education/health promotion
Teenagers with SCD will need specific information about alcohol, tobacco and recreational drugs as these are known to trigger vaso-occlusive crises (NIH, 2002). In the authors’ experience many adolescents will often select unsuitable sports and activities such as judo, kickboxing, rugby, skydiving and scuba-diving, and want to go clubbing. Such strenuous and physically demanding pastimes also increase the risk of vaso-occlusive crises. Often these activities are selected in an effort to fit in with their peers and be normal. Many of these pastimes are undertaken in secret and it is common to find that parents, particular those who are over-protective, are unaware of these activities.
In addition, advice about priapism, contraception, sexual health practices, analgesia, lifestyle, studying and employment choices should also be included in any health education provided to this patient group. Ideally such educational initiatives should take place within an adolescent transfer clinic.
In such clinics adolescents should be gradually prepared for the realities of adult care and encouraged to be more responsible for caring for themselves. In addition, they are to be taught about how and when to access hospital treatment.
Adolescence is clearly a difficult time for many people and more so for those who live with a chronic condition such as SCD, where pain is a major feature. For this reason, management of adolescents with SCD requires a holistic approach, which should include a great deal of support and education. Adolescents should be encouraged to take an active role in managing their illness.
Good pain assessment is essential and will include taking a patient’s pain and analgesia history. An analgesia ladder should be used. Parenteral opiates should be avoided unless the level of pain warrants their usage or the patient is ‘opiate tolerant’. Appropriate pain tools and an individualised approach will lead to good management of acute and chronic pain. Chronic pain will require long-acting analgesia and other pain-relieving interventions must be considered. The pain needs of these patients may change over time and analgesia plan must be regularly updated and modified.
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