Management of People with Sickle Cell Disease

There is no specific treatment for individuals with sickle cell disease; however, there are prophylactic measures that help to limit the factors that may trigger sickling crisis episodes and complications. There are national clinical guidelines for acute and community management of children and adults with sickle cell disease and these set out national standards that are measurable and will go some way to improving care (UK Forum 2006, Sickle Cell Society 2008).

Fluids

Maintenance of hydration is an important element in prevention of sickling crisis (Oni et al 2006, UK Forum 2006, Sickle Cell Society 2008). Below is the recommended daily intake of fluid, this is in addition to the fluid obtained from food:

Children:
Child’s weight Amount recommended
Below 10kg 150ml / kg body weight / 24 hours
10−20kg 80ml / kg body weight / 24 hours
20+kg 40ml / kg body weight / 24 hours
 
Adults:
Amount recommended
3 – 4 Litres in 24 hours

Prophylactic Medications

Folic acid:

Folic Acid is required for the manufacture of red blood cells. Because the diet consumed in the UK is very rich in folic acid and provided the individual is eating a normal balanced diet which contain green leafy vegetables and fortified cereals they do not need supplementation. Most Centres no longer suggest supplementation but where it is taken 5mg once a day in children and adults is sufficient and will not cause any harm.

Penicillin:

Because of poor splenic function and underdeveloped immunity people with sickle cell disease are more prone to infection and the pneumococcus is commonly implicated as the major cause of infection often leading to meningitis and pneumonia which can be fatal especially in children under the age of five (Serjeant & Serjeant 2001), the largest death rate occurs in this age group. Worldwide research has demonstrated that the daily use of a broad spectrum antibiotic e.g. oral penicillin significantly reduces mortality and morbidity (Serjeant & Serjeant 2001, Steinberg et al 2001, Riddington & Owusu-Ofori 2002, and Raghavan & Davies 2002).

Worldwide the daily use of prophylactic penicillin to reduce the likelihood of pneumococcal infection has been and continues to be recommended; this was further endorsed by the UK Forum (2006) who suggested maintaining the dosages as follows:

Penicillin dosage:
Age Dose
3 months - 1 year 62.5mg twice daily
1 year - 5 years 125mg twice daily
5 years – adulthood 250mg twice daily

Parents and inexperienced health professionals become anxious about the use of daily penicillin and the possibility of a patient developing penicillin resistance. Although there have been reported cases of penicillin resistance in North America this has not occurred in the UK and where the individual develops an infection that is not sensitive to penicillin other antibiotics may be prescribed safely for infection. Parents, individuals and health care professionals need to be reassured that research demonstrated that the long-term use of daily prophylactic penicillin does not cause an individual to become ill.

It is highly recommended that the penicillin is given throughout the vulnerable period of childhood to at least aged sixteen years and preferably continued into adulthood. Where an individual is allergic to penicillin in the UK erythromycin is often prescribed as an alternative.

Immunizations

Childhood immunisations:

It is highly recommended that children with haemoglobinopathies be given the full course of the recommended childhood immunisations these include diphtheria, Pertussis (whooping cough), tetanus (DPT), polio, Prevenar, haemophilus influenza (Hib), Meningitis C, measles, mumps and rubella (German measles) (MMR). Contracting any of these infectious diseases can prove fatal in this client group (Davies & Oni 1997, UK Forum 2006).

Other Immunisations:

It is recommended to also give Pneumovax at age 2 years (vaccination against a broad range of pneumococcal infections); this is repeated three years after the initial dose, a booster dose should be given every five years thereafter and throughout adulthood. Meningivac - is recommended for protection against meningococcus types A and C, especially for those travelling to some parts of the world where type A is most common this includes Africa and Asia. Hepatitis B – hepatitis B is a blood transmitted infection but it can be contracted in the womb from a mother to her unborn child, it is commonly contracted through unscreened blood during a blood transfusion; because of rigorous screening the latter is rare in the UK. Influenza – an annual influenza vaccination is recommended.

Management of Acute Sickle Cell Crisis Pain

Contrary to popular belief, the majority of clients manage their sickle cell pain episodes at home and only come into hospital when their pain is excruciating and they can no longer manage it at home safely or the medications they are using is not effective. 10% of the client population account for 90% of all hospital admissions (Davies & Oni 1997, Ballas 1998). How often a person has a crisis is very unpredictable, the severity of the crisis is also unpredictable.

Jones observed,

There is an overwhelming focus on Opioids and addiction linked to stereotyping of this group of patients and has led to under treatment of pain....one of the biggest problems we see (in patients with SCA) is patients being under medicated for painful episodes....and being labelled as drug seeking behaviour when they are not (Jones 1998:1055).

In a series of interviews conducted by Maxwell & Streetly a patient with sickle cell disease stated,

…they treat you as a street junkie (Maxwell & Streetly 1998)

At home and in hospital patients use a range of pain remedies depending on the severity of their pain, their preference of pain medication, local GP and hospital protocol. In the majority of cases a moderate to severe painful crisis warrants hospitalisation and medical intervention, management often include intravenous fluids to prevent or correct dehydration, pain relief (often opiates), bed rest and an investigation of the cause of the crisis and its management where applicable, for example, treating a precipitating infection.

Depending on local service structures patients enter the acute care area through one of three possible sources A&E, day care unit or direct admission to a ward area via the GP or community specialist nurse. The following care pathway is common in areas of high prevalence where specialist services are available. In low prevalence areas any or all of these modes of entry are implemented.

Admission to A&E or Day Care Unit

The day- care unit provide opportunities for patients to have rapid assessment and access to specialist care and is a useful mode of entry for those experiencing an uncomplicated non-emergency acute painful crisis. The units are often managed by highly advanced and skilled nurses, and may or may not include a specialist nurse, but in high prevalence areas they are managed by sickle cell / thalassaemia specialist nurses.

On arrival in the day-care unit an initial vital signs, general observations and pain assessment is conducted by the nurse and the approved local policy pain medication is administered following which the patient will be assessed by the medical team and specific investigations and treatment is implemented. The patient is often managed quicker when attending the day care unit compared to attending A&E.

Aggressive treatment of moderate to severe pain is consistent and the pain managed effectively. The supportive care environment of the day-care unit and provision of care by familiar members of staff can often prevent the need for inpatient admission for an uncomplicated sickling episode. Day care units provide a short term holistic approach to the management of sickle cell disease crisis.

On arrival in A&E or the Day Care unit it is vital for the triage nurse to regard patients with sickle cell disease as an acute emergency which necessitates their receiving immediate attention (Brookoff & Polamano 1992). Delay in conducting an initial assessment can lead to detrimental consequences because of the unpredictable nature of sickle cell disease. A sudden and unexpected death is not uncommon.

It is important to recognise that a patient experiencing acute sickle cell crisis pain would have made every attempt to relieve their pain at home. It is when these attempts have failed that they would come into hospital. Therefore, by the time they arrive in hospital the pain is often excruciating, hence their urgent and incessant demand for immediate pain relief. Taking detailed medical and social history on arrival in A&E can be misconstrued as the nurse’s or doctor’s unwillingness to acknowledge the severity of the pain. This can lead to conflict between the patient and the health care professional (Oni 1998). Patients in this predicament often argue that ‘doctors and nurses do not take such detailed social history from a person admitted following a major injury or heart attack until their pain has been relieved’, and severe sickle cell crisis pain is similar therefore attempts must be made to relive the pain prior to taking detailed biographical details. This is a point highlighted by Brookoff & Polamano (1992) who suggested that severe sickle cell crisis pain should be likened to cancer pain and treated with the same level of urgency and individuals treated with an empathetic understanding.

Most leading hospitals dealing with a significant population of patients with sickle cell disease have a policy which allows specially trained hospital nurses to give patients opiate analgesia prior to being seen by a medical doctor.

On arrival patients must have a basic nursing assessment, this should include:

  • Observation and recording of vital signs
  • Temperature, pulse and respiration
  • Report of when the pain started
  • Time of onset, site, nature and severity of pain
  • Medications already taken, amount and time taken
  • Routine medications patient is currently taking
  • Any known allergies especially to medications or plaster

The patient is then given the recommended pain medication approved for use by nurses in A&E in that particular hospital. For example, the local protocol may include use of Entonox via face mask, or and morphine intramuscularly. These are the most commonly prescribed medications for relief of severe acute sickle cell crisis. It is anticipated and recommended that one should aim to achieve administration of pain relief within 15 minutes of arrival in A&E.

Following initial relief of pain a detailed medical and social history can then be taken. In assessing the patient the general principles of nursing will apply:

  • Assessment
  • Diagnosis
  • Planning care
  • Implementation
  • Evaluation

A detailed pain history should include:

  • Activity patient was doing when pain started
  • Environment patient was in when pain started (e.g. hot, cold etc)
  • Any factors which the patient feels may have precipitated the crisis
  • Severity of the pain, using a pain analogue (rating) scale
  • Attempts made to relieve the pain at home (actions and medications)
  • How they have been feeling for the last few days (e.g. symptoms of flu)

Once the initial acute phase has abated further nursing assessment and intervention may and often include:

  • Observations - Temp, Pulse and Respiration
  • Checking vital signs - especially whilst having opiates
  • Check oxygen saturation if warranted
  • Test Urine, to exclude urinary tract infection and to assess kidney function
  • Maintain hydration, orally if patient is able or may require intravenous fluids to prevent or correct dehydration
  • Measure all fluid input and output diligently

Nurses often fail to appreciate the importance of these basic nursing assessments especially routine assessments of temperature, pulse and respirations and these can make a difference between life and death. While the initial excruciating pain may have abated and the patient appear to be capable of being self-caring nurses and other health care professionals may fail to recognise that sickle cell disease is regarded a ‘silent killer’ for good reason, the patient’s condition can deteriorate rapidly and unexpected, vigilance is crucial at all times. A lack of knowledge of sickle cell disease is a major factor which impedes many nurses’ ability to care for this group of patients effectively. For example, failing to maintain fluid balance can lead to missing early signs of kidney failure and a fatal deterioration of the patient’s condition, especially whilst they are receiving opiate medications for pain; failing to maintain observations can result in missing the early signs of complications and the need to take remedial action. These failings can and often result in the unnecessary death of patients with sickle cell disease (NCEPOD 2008) in all age groups.

Case Study

Jeanette is a 15 year old with sickle cell anaemia she was admitted to hospital with back and hip joint pain, all infections screen proved negative however, she continued to complain of pain and required continued use of opiate medications. On the third day she complained to the nurse that she felt breathless this was recorded as being caused by her having gone to the hospital shop which was several yards away from the ward.

After four days of admission Jeanette died and post mortem revealed the cause of death as an acute chest infection and consolidated lungs.

On a review of Jeanette’s case notes it was discovered that although she had a fluid balance chart only IV fluids and urinary output was recorded only on day one, by day two only IV fluid input was recorded, oral intake was not recorded at all and output was not recorded from day two. Urinalysis was not conducted even on admission.

Observations of temperature, pulse and respirations were not recorded after day one despite being on regular opiate medications and IV fluids. There was no formal assessment of pain severity or effectiveness of the pain medications; no pain rating scales were used. Jeanette was reported as self-caring in the nursing notes and it was acknowledged that prior to her death she continued to complain of pain on a regular basis despite having regular opiate medications.

This is evidence of a lack of understanding of sickle cell disease and the care needs of this patient; assessment of kidney function is vital, measurement of hydration (fluid input and output) is crucial in this patient group, especially during an acute illness (NCEPOD 2008). Observations of temperature, pulse and respirations may have alerted the nurses to the increasing deterioration of the patient and a report of chest symptoms in a patient with sickle cell disease is regarded as serious until proven otherwise, in this instance where chest pain was reported a doctor should be called to examine the patient.

Many patients, adults especially, report that health carers do not always believe the severity of their pain and may withhold pain relief for fear of their becoming addicted to their pain medication Valler (1994). The patient’s report of pain may be poorly managed allowing break through pain for long periods before the next dose of medication is due. These experiences perpetuate development of attention seeking behaviours that may be misconstrued as drug seeking behaviours. It creates mistrust and lack of confidence in health carers and may lead to conflict between the patient and carer (Oni 1998). A multi-disciplinary approach in the management of pain in conjunction with the pain control team, doctors, nurses, social worker and psychologist is recommended.

Medical and nursing management is dependent on the nature and cause of the acute episode, the patient’s age (paediatric or adult) and reported symptoms. General management include investigations to determine the cause of the acute crisis episode, pain relief, hydration, treating any underlying infection, rest and reassurance.

The Nurses’ Role in Managing Sickle Cell Crisis and Illness

Observations and Assessments

The frequency and type of nursing observations depend on the patient’s condition and the medical/ nursing interventions required. For example, monitoring of vital signs is extremely important during the early stage of an acute episode and should be continued whilst patients are having opiates, and any change in mood or behaviour must be investigated and taken seriously. This may be a sign of worsening crisis involving vital organs such as the lungs and the brain.

Sickle lung or chest crisis is a common complication of sickle cell disease (Vichinsky et al 2000) that can cause sudden unexpected death in adults. A steadily increasing respiratory rate may be the only visible sign that this is occurring, hence the need to maintain vigilant observations even when the patient’s condition appears to be improving. Where the oxygen saturation level is < 94% immediate medical intervention is required.

Once the severe acute crisis has abated many patients with sickle cell disease become self-caring whilst they are not well enough to go home they may be well enough to require less medical attention, however, they still require care.

Measurement of temperature, pulse and respirations are crucial markers during an acute illness and may give early indications of infection, impending sickle lung or other potential complications. Infection is one of the most common causes of illness in children and adults with sickle cell disease therefore an infection screen is crucial and should include urinalysis.

Hydration

Maintenance of hydration is an important element in prevention of sickling crisis and complications. The amount of fluid required daily, which is in addition to the amount obtained from food has been described earlier. If the individual is vomiting, has diarrhoea, has a fever and or is unable to take oral fluids for any reason it is vital to maintain hydration via the intravenous or other route.

Kidney dysfunction occurs as a result of damage to the kidney tissue from early childhood and acute kidney failure is fairly common hence the need to measure fluid balance accurately especially whilst the patient is acutely ill and receiving intravenous fluids and opiate medications. Where fluid balance is not maintained adequately an acute renal failure may go unnoticed and this can contribute to further complications including death. It is important to emphasise the importance of fluid balance in management of this patient group and nurses play a pivotal role in the assessment and management of hydration including educating patients about its importance (NCEPOD 2008).

Maintaining a strict fluid balance chart is essential. Depending on the age and condition of the patient they should be encouraged to participate in maintaining the fluid balance chart, particularly recording their oral intake and urinary output when they become self-caring and ambulant.

Pain relief

The choice of pain relief depends on the severity of the pain, the site of the pain, nature of the pain, hospital protocol and individualised patient protocol. Medications tend to range from for example Paracetamol for mild pain → Distalgesic for mild/ moderate pain → opiates for moderate to severe pain. Opiates are usually given IM as an initial bolus dose and depending on the age of the individual subsequent doses may be administered IV using a Patient Controlled Analgesic (PCA) pump. Administration of opiates via PCA pump is usually only used in A&E or when the patient has been admitted to the ward as in-patient.

Example of Pain Relief Ladder

  Degree of Pain Medication and Route
 
Children Mild Oral analgesia Paracetamol / Calpol/ Disprol
Oral codeine phosphate
 
Moderate / Severe IV Morphine Sulphate
PCA Morphine Sulphate
 
+ or - Anti-inflammatory drug e.g. Ibuprofen
+ or - Anti-emetic
+ or - Anti-histamine
+ or - Antidepressant
 
Dosage of all medications in paediatrics depends on child’s age and weight.
 
Adult Mild / Moderate Oral Paracetamol
Oral Dihydrocodeine
Oral Codydramol
Oral Diclofenicl
Oral Tramadol
 
+ or - Anti-inflammatory drug e.g. Ibuprofen
 
Moderate / Severe Subcutaneous Morphine
Subcutaneous Morphine via PCA
IM or Oral Valtoral
 
If proven to be allergic to Morphine other opiates may be given.
 
+ or - Anti-inflammatory
+ or - Anti-emetic
+ or - Anti-histamine
+ or - Antidepressant

The frequency and dosage of medications is often dependent on the degree of pain and other underlying factors such as sensitivity to medications, allergies, effective functioning of vital organs e.g. lung, kidney and liver and if there is any sign of cerebral irritation, vomiting, and diarrhoea, these will also affect preferred choice of treatment.

It is important to have an understanding of the pharmacokinetics of the drugs being used. Due to poor kidney function patients with sickle cell disease have difficulty excreting the metabolite of Pethidine (nor-Pethidine) therefore frequent and high doses of Pethidine can cause a build up of nor-Pethidine, which can cause cerebral irritation and convulsions, this can prove fatal and was one of the leading causes of death in this patent group during the 1980’s and 1990’s, after which the use of Pethidine as the first drug of choice lost favour among clinicians. In view of this many specialist units now use Morphine as the opiate of choice, because it does not have the same adverse effect as Pethidine but it can also suppress respirations. Some patients are genuinely allergic to Morphine and report pruritis, nausea and vomiting, medications to relieve these symptoms are often prescribed in conjunction with the Morphine or an alternative opiate such as Omnopon or Fentanyl is prescribed.

The addictive properties of opiates are well recognised, however, the rate of opiate dependence in this client group is reported to be very low (Valler 1994), but it does occur albeit infrequently. To help reduce the likelihood of dependence patients and their families should be taught how these drugs work, how to recognise drug seeking behaviour and how to limit occurrence of dependence. Working in partnership with the patient and their families is the vital key to proper pain management of this client group (Burghart-Fitzpatrick 1989). The use of a pain assessment tool is essential for managing pain effectively. Because of the inflammation and damage that sickled red blood cells cause to the walls of blood vessels a non steroidal anti-inflammatory drug is also useful as adjunct treatment of painful crisis hence anti inflammatory drugs such as Ibuprofen is also commonly prescribed.

Oxygen Therapy

Patients experiencing a sickle cell crisis are not routinely prescribed oxygen unless there is a specific indication, for example, if there is an acute sickle chest involvement, short or long term pulmonary damage that is limiting the patient’s ability to maintain oxygen saturation at 95 - 98%. Long term complications due to repeated assaults on the lung as that caused by sickle chest syndrome may result in poor oxygen perfusion. In such situations the patient may require oxygen this may involve use of Continuous Positive Airway Pressure (CPAP), the aim is to maintain oxygen saturation at 95 - 98%.

Monitoring the effect of oxygen therapy including measurement of vital signs is essential as well as basic nursing care such as maintaining oral hygiene including lip care, especially in this patient group noting that Black people tend to have drier skin when compared to white people.

Warmth, Comfort and Reassurance

Keeping the body warm but not hot will often relieve pain especially in a limb. Extremes of temperature may precipitate a sickle cell crisis and sudden changes to body temperature should be avoided, for example, jumping into a cold swimming pool. Many patients find a warm bath, massage or application of warm towels or a heat pad to affected limbs can be very soothing and helps relieve pain. Some patients have found the use of transdermal nerve stimulation (TENS) effective in relieving pain, especially where the pain is localised such as in a limb or in the back.

Reassurance develops the patient’s confidence in the carer, allays anxiety and helps to relieve pain, recognising that pain is not merely a physical but also a psychological experience. General nursing management is similar to management of any acute illness but recognising the potential mortality and morbidity associated with sickle cell disease is crucial.

The Psychological Dimension

By adulthood memories of inadequate management of sickle cell crisis pain may have had a major psychological impact on the individual (Midence & Elander 1993). Most adults know someone who has died as a result of having sickle cell disease, either in their immediate family, extended family or through relationships formed in the sickle cell clinic or hospital.

Therefore, each episode of sickle cell crisis, illness or hospitalisation creates fear and anxiety,

  • “will the nurses and doctors believe I am in pain”
  • “will they give me pain relief regularly, and enough to relieve the excruciating pain or will I have to suffer long periods of pain”
  • “will they think I am a ‘drug addict’ because I am in pain”
  • “will I die this time”

These anxieties and fears perpetuate the patients focus on the pain and the morbid fear of death can be all consuming. This psychological dimension exacerbates the physical experience of the pain and the stress associated with this may compound the sickling crisis. Maxwell & Streetly (1998) and Maxwell et al (1999) illustrate the socio / cultural difficulties experienced by this client group in health care settings, especially in respect of pain management. Conflicts can arise between patients and carers because of a lack of trust, misunderstanding of care needs and loss of mutual respect (Valler 1994, Oni 1998). The patient in severe pain will not understand why the carer is accusing him of being ‘selfish’ and failing to appreciate that there are other patients in the ward who also need the nurse’s attention. A busy and stressed health carer perceives that the patient’s demands are unrealistic especially when other patients need his / her attention just as much or more, perhaps the time to give further pain medications has not arrived and the patient’s incessant demand for pain relief becomes an irritation when attempts have been made to explain this to the patient and or his family and they fail to understand the issue.

Further work needs to be done to examine the most effective way of dealing with this patient group’s pain, especially those with chronic intractable pain where high doses of pain medication will not be effective in relieving the pain and is not advisable due to the unwanted side effects of long term use of such medications.

The introduction of alternative therapies, such as acupuncture, aromatherapy and massage are proving beneficial in relieving pain in this client group and reducing the tensions inherent in the carer / client relationship. The use of Cognitive Behavioural Therapy (CBT) is emerging as a useful therapeutic intervention in managing acute and chronic sickle cell pain and is proving a viable and effective alternative or adjunct to medications, provided clients can be encouraged to sustain their efforts to learn and use the technique (Anie & Fotopoulos 1998). See the Coping with Sickle Cell Disease & Pain – A Self-Help Manual for Children and Coping with Sickle Cell Disease & Pain – A Self-Help Manual for Adults manuals.

Management of People with Sickle Cell Trait

Sickle Cell trait (Hb AS) is a healthy carrier state which does not give rise to a significant clinical presentation Haemoglobin S - Sickle Cell. However it is reported that those with sickle cell trait are less able to concentrate their urine (hypostheuria) in view of this there is an increased tendency and risk of dehydration and these individuals should be advised to maintain an adequate fluid balance. There is increased rate of bacteriuria, pyelonephritis and frank haematuria especially during pregnancy, screening is essential and a positive result needs to be treated with antibiotics promptly and rigorously.

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