Sickle Cell Disorders and Physiotherapy

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SCHOLARLY PAPER

Sickle Cell Disorders and Physiotherapy Mel Stewart

Key Words Sickle cell disease, physiotherapy.

Summary Sickle cell disorders affect a wider group of people than is commonly realised. Screening is not universal and this, with a general lacksof awareness about the condition, means that many people are inadequately treated. Physiotherapy is noticeably absent from the general medical literature and from the current health education programmes which relate to the disease. Its appropriateness has rarely been addressed, and yet some of the manifestations and potential problems are similar to other conditions which are commonly treated.

The case for physiotherapy management to include stroke and pulmonary rehabilitation, preventive care, joint protection, back care, pain control, leg ulcer management and exercise is outlined.

Finally, serious concerns are raised about the lack of awareness and knowledge of health professionals including physiotherapists regarding the condition.

Introduction Sickle cell disorders (SCD) produce varying degrees of functional incapacity or even death, yet the existing and potential contribution of physiotherapy in their management appears t o have been largely ignored in medical literature. In the UK, the number of people affected is similar t o that for cystic fibrosis, but in strong contrast the use of physiotherapy, the level of research and the organisation of health education is much less. The aim of this article is to give an overview of the condition in order to explore the ways in which physiotherapy may help to improve the functional ability and quality of life for the people affected.

Sickle cell disorders are known to affect a wider group of people than is commonly acknowledged. They are present in equatorial Africa, Turkey, northern Greece, Eastern Province of Saudi Arabia, Italy, parts of India and America (Serjeant, 1992a). David et aZ(1993) suggest that there is a rising prevalence in the developed coun- tries and in Britain it is thought that they affect between 5,000 and 6,000 people. In certain areas, it is said t o account for approximately 40% of hospital admissions in the haematology specialty (Brozovic et al, 1989). At least 150 babies are born each year with a positive diagnosis (Brozovic and Davies, 1987).

The procedure for the identification and follow-up of people who are affected varies enormously across regions with the inevitable consequence

that many fall through the care net without receiving appropriate attention. I t is possible to test a fetus to detect a haemoglobin disorder from as early as 11 weeks of pregnancy. Mixed reac- tions surround selection and screening for genetic abnormalities at birth but the advantages are such that the Cystic Fibrosis Trust is currently campaigning for screening of all babies within the UK for cystic fibrosis, and Selekman (1993) recom- mends that all new-born babies are also screened for SCD. Beazley (1991) states that although universal screening has been successfully employed in Cyprus to combat thalassaemia, in the UK with the ethnic minority population standing a t less than three million, this would be inappropriate. Franklin (1990) indicates that there would have to be a major investment in counselling facilities if such a programme were to be implemented.

The term SCD encompasses a group of disorders or diseases including sickle thalassaemia. The pathology is due to the presence of sickle haemoglobin. I t may result from the inheritance of haemoglobin S (HbS) from both parents, giving rise to homozygous sickle cell disease ( S S disease),

USUAL SICKLE HAEMOGLOBIN CELL TRAIT

USUAL SICKLE CELL USUAL SICKLE CELL HAEMOGLOBIN TRAIT HAEMOGLOBIN TRAIT

Fig 1: If one parent has the usual haemoglobin (HbA) and the other has sickle cell trait (HbAS), of the four possibilities for their children, two are for HbA and two for HbAS

BOTHPARENTS SICKLE CELL TRAIT (HbAS)

USUAL SICKLE CELL SICKLE CELL SICKLE CELL HAEMOGLOBIN TRAIT TRAIT" DISEASE

Fig 2: Both parents have sickle cell trait (HbAS) so each parent has one gene for sickle haemoglobin and one for the usual haemoglobin

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or - in the case of thalassaemia - the inheritance of HbS from one parent with one of the thalassaemia genes from the other (Serjeant, 1992b). Alternatively, if one parent has the usual HbA and the other is a carrier of HbS then their children will have a 5050 chance of having normal haemoglobin or sickle cell trait (HbAS - fig 1). Individuals who have sickle cell trait are carriers of HbS but remain healthy and relatively asymp- tomatic throughout their lives. If each parent has the trait then there is 25% risk of each child suffering from a form of the disease (fig 2). Among more than a hundred of the abnormal haemoglo- bins which have been identified are: HbC, HbD, HbO, and Hb Lepore Boston. When these are inherited with HbS, a form of clinically significant SCD also results. Individuals with SS disease have

Fig 3: Sickle cells

approximately 80-100% HbS instead of the normal 95% HbA in adults and experience ‘sickling’ of the red blood cells in varying degrees throughout their lives. On giving up their oxygen, the shape of these red blood cells changes t o resemble a sickle and they become stiff (fig 3).

A sickling crisis usually results from avascular necrosis of the bone marrow (Serjeant, 1992~) . As a consequence pain is felt in the back, extrem- ities, abdomen and chest. Some of the risk factors have been identified. The most common of these are thought to be high haemoglobin levels, red cell deformability, menstruation and pregnancy.

Fig 4: Sections through small blood vessels. Top - normal blood flow. Bottom - sickling

Precipitating factors are thought to be cold, infections, acidosis, sleep and sleep apnoea, altitude and emotional stress (Serjeant, 1992d). I t is thought that under conditions of low oxygen tension the HbS molecules show a tendency to aggregate, stack or polymerise and change from a fluid to a gel-like state resulting in intravascular occlusion. The consequences of this may vary from small occlusions in the capillary bed which may pass completely unnoticed or cause only minor discomfort to a major blockage in the lungs, spleen or the cerebral circulation which may cause a life-threatening crisis (fig 4).

Various theories have been put forward to explain the pathophysiology of SS disease. Ham and Castle (1940) observed that the viscosity of sickle cell blood rose when oxygen tension fell below 40 mm Hg and this increased further with greater anoxia. Thus, a viscous circle arises in that the resultant increase in viscosity leads to further decrease in blood flow in the capillary bed which in turn leads to further deoxygenation and a further increase in viscosity. A lowered pH was also later thought to influence the development of sickling (Harris et al, 1956). Charache and Conley (1964) related the increase in viscosity to the proportion of blood in the sickled form and the square of the haematocrit in deoxygenated blood. It was calculated that oxygen tensions and cell velocity in SS disease were higher than in normal blood, but lower pressure gradients would be associated with marked falls in oxygen tension and velocity, increasing the likelihood of sickling (Berger and King, 1980).

Some individuals with the sickle cell trait, especially the young, gain some protection from malaria (Livingstone, 1971). However, most people with HbS remain at risk from malaria and protection by immunisation is still required. Prob- lems associated with the disease usually become evident after the age of approximately three to six months but the condition can be diagnosed in utero from as early as nine or ten weeks of pregnancy (Anionwu, 1994). The pathologies which are caused by SCD are comprehensively outlined by Serjeant (1992). Some of these are listed in the panel.

I t is possible to identify the conditions which can rapidly develop into medical emergencies, for example; splenic sequestration, chest syndrome, pneumococcal infections, stroke, priapism and renal complications. Others are less life-threatening in the short term but can drastically reduce quality of life over time. One of the prime concerns of physiotherapy is the achievement andlor enhancement of efficient and appropriate move- ment aimed directly a t improving functional ability and quality of life. There is clearly a need


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Common Pathological Problems Identified in SCD

Splenic sequestration Priapism Anaemia Renal complications Pneumonia Joint involvement, eg gout, referred joint

Leg ulcers Strokes Arthritic conditions Dactylitis in children Flexion deformity of the ankle Vertebral compression fractures Avascular necrosis of femoral

Pain

pain in a crisis, etc.

head + osteo-arthritis

to address the physiotherapeutic input, or the lack of it, in the holistic care of people with SCD. Even though some individuals are treated by physiotherapy, little is known about the number who require treatment but are not referred; or are referred with a confirmed or unconfirmed diagnosis and receive physiotherapy within a holistic and long-term management programme. Despite this, there are features of the disease which could be effectively managed by physiotherapy. These strategies include:

@ Rehabilitation following strokes and pulmonary crises @ Joint protection

Back care @ Pain control @ Leg ulcer management 0 Exercise

Particular attention needs to be directed towards the management of strokes which affect many children and young adults with SCD, and chest syndrome which can occur at any age. Hemiplegia is the most common major neurological compli- cation in S S disease. Estimates vary but range from 8-17% of individuals. Approximately 80% of episodes will have occurred before the age of 14. Recurrence is common and has been reported to be as high as 90% in a study by Russell e t al (1984) and as low as 43% in another by Serjeant (1992g). Although long-term transfusion is effective in preventing further strokes (Wang e t al , 19911, the management of the resulting functional deficits from initial and sometimes subsequent strokes is not addressed by the literature.

Acute chest syndrome (ACS) is the most frequent cause of death in adolescents and young people

with SCD (Davies, 1991). Its manifestations include chest pain with fever, cough, and dys- pnoea with abnormal clinical and radiological chest signs. It is possible that these features may be due to infection, infarction and acute pulmonary sequestration; but current data suggest that most episodes do not show evidence of infection (Serjeant, 1992 f). Consolidation of the lungs develops with an associated drop in saturation of arterial oxygen (Davies, 1991) and there may be multiple lobe involvement. Hough (1996) remains one of the few people to suggest that physiotherapy may be indicated for some of these problems but gives no clarification of the term ‘acute chest syndrome’ and little guidance to its management. Incentive spirometry has been indicated by Bellet e t al (1995) as a means of preventing some pulmonary complications, for example atelectasis, in patients who are hospi- talised with chest and back pain above the diaphragm.

The rehabilitation of people whose lives have been affected by strokes and pulmonary pathologies forms a major part of the work of physiotherapists, yet in SCD where both conditions often have a major impact on functional ability and quality of life there appears to be little awareness.

Preventive Care Yang et a1 (1995) have shown that there is a gross discrepancy between the cost of caring for patients who attend comprehensive health care clinics with preventive care programmes, and for those who do not attend. Patients enrolled in these clinics use emergency rooms and in-patient units less frequently (p < 0.0001 and p = 0.006 respec- tively). The development of preventive care in SCD would need to take account of the common pathologies and address the issue of secondary problems arising as a consequence of long- standing disease. Diminishing lung compliance, disturbed ventilatiodperfusion balance, reduced exercise tolerance, and tendedswollen joints including back pain, are just a few of the common presenting problems. Preventive care could encompass advice on maintenance or improvement of lung function including pulmonary function tests, monitoring of cardiovascular fitness, joint protection, maintenance and/or improvement of joint range and muscle power, and back care. It could be incorporated into existing community management programmes or administered through roqtine physiotherapy referrals via general practitioners or hospital consultants. Alternatively, it could be introduced into existing health education programme in schools, health centres, community groups and specifically targeted locations.

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Exercise The advice given t o individuals with SCD regarding the extent to which they should exercise appears to be non-specific and this may be due to a number of reasons. I t is known that in SCD the response to exercise is abnormal (Serjeant, 1992d) and that a crisis can be trig- gered by an excess of exercise (Diggs, 1965).

Sudden death associated with intensive exercise of individuals with the sickle cell trait has been highlighted by Binder and Jones (19701, Eichner (1993) and Kark et al (1987). I t has also been suggested by Ready (1993) that, if the trait is present, symptoms which mimic SCD can develop when dehydration occurs as a result of exercise. However; it should be noted that the number of these deaths has been relatively small.

The advice normally given is that individuals should be allowed to find their own limits. Although this is useful, some people may find it difficult to weigh up the extent to which they should challenge their cardiovascular system in order to gain the benefits of exercise against the risk of triggering a crisis.

The utilisation of exercise as therapy is a funda- mental part of the practice of physiotherapy. Guidance on exercise under supervision should be offered through carefully scheduled programmes t o help in the achievement of the desired levels of fitness. I t needs t o be specific and carefully targeted towards the different client groups, for example pregnant women, children, athletes, individuals prone to chest complications, etc. The type of exercise, for example low impact or high impact, and the amount of exercise would need to be carefully tailored and monitored to help patients gain control over their own programme. I t is suggested that children with SCD may be disadvantaged in competing with normal children in short-distance racing and in swimming (Millis et al , 1994). Allowances have to be made in the programming of such activities.

Joint Protection Advice on the protection of joints in situations where they are prone t o destruction, such as in rheumatoid arthritis, is considered part of normal physiotherapy practice. SCD renders many joints susceptible to the same fate, therefore similar advice should be given. Dactylitis (‘hand-foot syndrome’) is a frequently presenting feature in children and requires special attention. Damage to joints in SCD may occur secondary to avascular necrosis in bone, and as a result of related arthritis, gout, deformity and so on. The recogni- tion of abnormal joint changes and attention to these problems when they arise may help in

preventing future joint complications. The hip, shoulder, ribs, sternum and vertebral bodies are the areas most commonly affected.

Sadat-Ali (1993) has identified a method of clinical evaluation which assists in the early detection of avascular necrosis of the femoral head and which includes analysis of the patient’s history and radiological examination. He suggests that early diagnosis and proper management could delay further deterioration.

Screening for the disease may be one of the few ways to prevent worsening of progressive arthropathy, and Tismit and Bardin (1994) suggest that it is the only way.

Closely associated with general joint protection is the advice which should be given on care of the back. One of the most common pathological features of the back in SCD are vertebral compression fractures and pain (Serjeant, 1992). Referrals for the care of low back pain may account for 60% the waiting list in a physiotherapy out-patient department (Newton and Waddell, 1991). The experience of physiotherapists in back care should be made available to individuals with SCD as part of a health education and joint protection programme.

Pain and its Control Of all the symptoms experienced by individuals affected by SCD, pain is the most common clinical manifestation (Serjeant, 1 9 9 2 ~ ) and ranks as one of the most distressing. The greatest inten- sity of pain is probably experienced during a crisis but pain can and does occur from a multitude of other pathologies. Alcorn et al (1984) have suggested that exercise and physiotherapy may decrease the length of hospital admission for a painful crisis. One of the most common trigger factors for a crisis is cold. This fact should be brought to the attention of all physiotherapists since the use of ice is one of the most common modalities in many departments.

Work has been carried out on the use of opiates and other drugs for the control of pain (Serjeant, 1992e). However, the same cannot be said of the modalities used in physiotherapy which are known to help in its control and have fewer known side-effects. Transcutaneous electrical nerve stimulation (TENS), pulsed electromagnetic energy (PEME), ultrasound, short-wave diathermy, infra- red irradiation, and acupuncture are just a few of these. Very little is known about their impact in SCD although Co et al (1979) and Wang et al (1988) have shown acupuncture and TENS to have only a placebo effect. The further investigation of some of these modalities could bring to light some useful alternatives for the control ‘of pain.


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Leg Ulcers It is well recognised that individuals with SCD often suffer from leg ulcers. These are often located over and above the malleoli and the ante- rior aspects. Numerous remedies have been tried to encourage healing but with limited success. Sergeant (1992) suggests that perhaps the most effective of these has been rest with elevation and bandaging. Unfortunately, this approach has not always proved practical for everyone and treatment remains unsatisfactory. PEME, ultrasound, laser and ultra-violet irradiation in controlled doses, with some form of exercise and rest in elevation have all been usefully applied to ulcers within physiotherapy practice (Thompson et al , 1991). However, there is little reference to the treatment of leg ulcers in SCD. The possible benefits to these individuals should be explored.

In seeking to develop further a programme of physiotherapy care for individuals with SCD, consideration should be given to: @Facilitation, delivery and exchange of information between those charged with managing the condition - ie the individual, the physiotherapist and other members of the existing health care team. @Assessment of the real and potential benefits of physiotherapy. @Provision of a functional assessment of patients including assessment of the contexts in which they have to function. @Provision of a programme of preventive care. 0 Treatment. @Regular review and assessment.

This programme would need t o ensure that it takes into account the factors which may influence multicultural and community care needs.

Historically, the management of SCD has been co- ordinated from haematology departments within large teaching hospitals and specialist NHS community centres (Eboh and van den Akker, 1995). The main carers have been the family, general practitioner, haematologist, and genetic counsellorhurse counsellor. Eboh and van den Akker (1995) suggest that the majority of this care including counselling, screening and the dissemination of information has been carried out by the specialist nurses. The information dissem- inated from these centres has included little or no mention of physiotherapy. The need for its integration with other areas of care is vital if individuals are to benefit from holistic care.

Midence et al (1994) and Holmes et al (1992) report that, so far, the targeting of specific health education programmes t o individuals with SCD

has fallen short of achieving their objectives. Eboh and van den Akker (1994), Anionwu and Beattie (1981) and Midence et aZ(1994), have all drawn attention to the lack of awareness and knowledge of SCD, especially among general practitioners, and the confusion which exists about the signifi- cance of the disease among patients, health-care professionals and the general population. Because of this general lack of awareness, in many cases the diagnosis is made only when a child is brought to hospital in crisis and sometimes not even then (Midence and Elander, 1994a). Thus many of the needs, including physiotherapy, of individuals with SCD have been generally neglected (Midence and Elander, 199413).

An important step in the establishment of physiotherapy into a life-long, holistic pattern of care, would be to increase the awareness of all these groups of its relevance. McLaurin (1986) and LePage and Maynard (1994) are a few of the individuals who have addressed this issue. In Facing up to Difference, a toolkit for creating a more culturally competent health services, the DOH has recognised the haemoglobinopathies as a priority area for action (DOH, 1996).

If ‘physiotherapy is a health care profession which emphasises the use of physical approaches in the promotion or restoration of an individual’s physical, psychological and social well-being, encompassing variations in health status’ (CSP, 1996), then its relevance to SCD should be inves- tigated. The main routes for referral to treatment in physiotherapy are via general practitioners and hospital consultants. Therefore the awareness of its relevance in SCD needs to be raised with these particular groups. Health education in in-service programmes, schools, community groups, and undergraduate education are some of the most obvious avenues by which the awareness of others may be addressed.

Currently, the number of individuals who are affected and the extent of intermarriage of people from different ethnic backgrounds within the UK indicate that, unless there is an effective programme of screening, accurate predictions of individuals a t risk and the level of care will be difficult. The greater need for counselling will also have to be addressed (Chandra, 1996).

Conclusion The reasons for the past and present neglect of the relevance of physiotherapy in SCD in the literature and perhaps in practice are complex. I t appears to be a relatively untapped resource for individuals seeking to improve their -functional capacity and quality of life. Comprehensive inves- tigations are needed in order to seek out reasons


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for this omission, find ways of identifying the growing numbers of people who are likely to be affected, investigate and direct its management, and find effective ways of improving the knowledge base of health professionals about sickle cell disease.

Acknowledgments The author would like to thank Dr Anne Parry for her helpful comments and advice.

All the illustrations to this paper were provided by the Sickle Cell Society, 54 Station Road, London NW10 4UA (tel 0181 961 779514006 ). This is a patients’ organisation which provides information and counselling for those with sickle cell disorders and their families.

Author and Address for Correspondence Me/ Stewart MEd MCSP DlpTP is a lecturer in the Department of Physiotherapy, School of Health Sciences, Morris House, University of Birmingham, Edgbaston, Birmingham B15 2TT.

This article was received on August 5, 1996, and accepted on May 14, 1997.

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grants and awards Volvo Awards for Low Back Pain Research 1998 In order to encourage research in low back pain, the Volvo Company of Goteborg, Sweden, again this year has sponsored three prizes of US$9,000 each. Awards will be made competitively on the basis of scientific merit in one or more of the following areas: 1. Clinical studies. 2. Bio-engineering studies. 3. Studies in other basic science areas.

Papers submitted for the contest must contain original material, not previously published or submitted for publication.

A multiple authorship is acceptable. The manuscript, in English, should be in the form of a complete report, including original illustrations, marked with names, not exceeding 30 type- written pages - references and tables included - double spaced, in 10 pt type or larger, and in a form suitable for submission as an original paper for publication in a scientific journal. Ethics committee approval is neces- sary for all animal studies as well as controlled clinical studies.

One original and five copies of each

paper in full - including illustrations - must reach the address given below no later than January 15, 1998. Arti- cles sent by fax will not be accepted. Please give complete address with telephone and fax numbers. Winners will be informed by the end of March.

One of the authors should be prepared, at his or her own expense, to go to Brussels, Belgium, to present the paper and to receive the award at the meeting of the International

Society for the Study of the Lumbar Spine, June 9-1 4, 1998.

The board of referees will be chaired by Professor Alf Nachemson and will contain members from the fields of clinical medicine, bio- engineering and bio-chemistry.

Please direct all correspondence to Professor A Nachemson, Department of Orthopaedics, Sahlgrenska Univer- sity Hospital, S-413 45 Goteborg, Sweden.

Winston Churchill Memorial Trust Travelling Fellowships All British citizens are eligible to apply for a Churchill Fellowship, covering travel and living expenses overseas.

Categories for 1998 include deliv- ering health care, projects in the Indian sub-continent, recruitment and use of volunteers in the community, promoting parenting skills, and sport and the fitness of schoolchildren.

Applicants may propose a study project related to one category, and they must be able to demonstrate that

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Sickle Cell Disorders and Physiotherapy