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SICKLE CELL DISEASE: NEED FOR REFERRAL TO TERTIARY LEVEL CARE?
- Naveen Eipe, Gabrielle La Rosee (26 November 2005)
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Alloimmunization in Sickle cell disease
- Mahindra G Chincholkar (27 August 2005)
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Naveen Eipe, Anaesthetist Padhar Hospital, Gabrielle La Rosee
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To the Editor: We read with interest Dr Firth’s review of the evolution of thought and practice on the perioperative management of Sickle Cell Disease (SCD) 1. The new concept of this disease described; as being one of chronic inflammatory and immunological abnormalities, appears to result in a paradigm shift of the focus from the “peculiar cells” to the “vascular endothelial damage and dysfunction”. This may have far reaching consequences in the anaesthetic management of SCD in the years to come. We have recently managed two teenaged patients with SCD presenting for cleft lip surgery in a rural secondary level hospital (unpublished). We followed the “traditional principles” involved in the perioperative management of SCD - avoiding anaemia, dehydration, hypoxia, hypothermia, hypercarbia and stress responses. With modifications applicable to this level of Hospital, we achieved uneventful and successful outcomes in these patients with SCD. Other reviews describe the management of SCD in tertiary and referral level hospitals2- 4. Here at our level of care, from the preoperative evaluation to the postoperative care, many diagnostic and therapeutic modalities were unavailable. While one study5 reported a 45% overall perioperative complication rate, intra-operative death due to SCD has been reported6. This may not have been acceptable for elective corrective/ cosmetic surgery in a teenager at this level of care (a Rural Secondary Level Christian Mission Hospital in Central India). We find Dr Firth’s assertion of the maxim primum non nocere—first do no harm interesting and coincidental in this context. This very philosophy was the basis of our discussions and consultations, since the decision to accept these patients was difficult. Though we may have simply rejected them or advised referral (to tertiary- level hospital), we decided to accept these teenaged patients for anaesthesia. This we felt was justified in the light of them not having had surgery till date (probably due to poverty, ignorance, inadequate medical coverage and co-existing medical illness) and that further referral could only increase the chances of their not getting operated. Intraoperative medical management is only one element of the Anaesthetist’s skills7. As Anaesthetists, we have a primary responsibility to safeguard our patient’s health in the perioperative period. But by rejecting such patients because of SCD for anaesthesia, we may be denying them an opportunity for corrective surgery. This is therefore a conflict between our professional duties and social responsibilities. Dr Firth’s advice “to concentrate on the basic standards of generally accepted anaesthetic practice, rather than adopting potentially harmful but unproven strategies”, makes us wonder- should all patients with SCD be referred to tertiary level hospitals? REFERENCES 1. Firth PG. Anaesthesia for peculiar cells- a century of sickle cell disease. BJA 2005; 95: 287-99. 2. Marchant WA, Walker I. Anaesthetic management of the child with sickle cell disease. Paediatr Anaesth. 2003; 13(6): 473-89. 3. Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology. 2004; 101(3): 766-85. 4. Tobin JR, Butterworth J. Sickle cell disease: dogma, science, and clinical care. Anesth Analg. 2004; 98(2): 283-4. 5. Gross ML, Schwedler M, Bischoff RJ, Kerstein MD. Impact of anesthetic agents on patients with sickle cell disease. Am Surg. 1993; 59(4): 261-4. 6. Intraoperative death during caesarian section in a patient with sickle- cell trait. The Anaesthesia Advisory Committee to the Chief Coroner of Ontario Canadian Journal of Anesthesia 1987; 34: 67-70. 7. Gaba D. What Makes a Good Anesthesiologist? Anesthesiology 2004; 101(5): 1061-3. Conflict of Interest:None declared |
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Mahindra G Chincholkar, SHO Anaesthesia Cumberland Infirmary, Carlisle
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Editor-I read with interest the recent review by Firth 1 who rightly highlighted the dangers of transfusion reactions in Sickle cell disease, in particular the problems associated with alloimmunization. The haemolytic transfusion reactions associated with alloimmunization may present with distinctive features which include haemolysis, reticulocytopaenia and development of a more severe anaemia than was previously present and have been termed as Sickle Cell Haemolytic Transfusion Reaction syndrome.2 The proposed mechanism for haemolysis includes Bystander immune cytolysis caused by activated complement components that are formed because of an antigen-antibody reaction. These activated complement components may then attach to red blood cells not involved in the original immune reaction, resulting in their lysis.2 Haemolysis may be associated with back pain, malaise, chest pain and fever. These findings may be misinterpreted as sickle cell crisis, delaying the diagnosis which may result in significant morbidity and mortality. Reticulocytopaenia often accompanies this syndrome, in contrast to other haemolytic disorders, further exacerbating the anaemia resulting from haemolysis of transfused red blood cells.2 Patients develop a more severe anaemia than prior to transfusion. The critical point to keep in mind is that subsequent transfusions may further exacerbate the anaemia. Withholding transfusion where practical and treating with methylprednisolone and intravenous immunoglobulins may be preferable.3 Recovery manifested by reticulocytosis and gradual improvement in haemoglobin may occur only after the withholding of further transfusion. An interesting aspect of this disease is that foetal haemoglobin is not incorporated in the sickle cell haemoglobin polymer, providing the rationale for treatments aimed at increasing the foetal haemoglobin concentration.4 Hydroxyurea, which increases foetal haemoglobin levels, has been used with variable success. Other potentially useful drugs which enhance the expression of gamma globin genes include decitibine and short chain fatty acids. Oral magnesium and clotrimazole also have been shown to have a beneficial effect.4 1. Firth PG. Anaesthesia for peculiar cells-a century of sickle cell disease. Br J Anaesth 2005; 95: 287-99 2. Rosse WF, Narla M, Petz LD, Steinberg MH. New views of Sickle Cell Disease pathophysiology and treatment. Hematology 2000;:2-17 3. Win N, Yeghen T, Needs M, Chen FE, Okpala I. Use of intravenous immunoglobulin and intravenous methylprednisolone in hyperhaemolysis syndrome in sickle cell disease. Hematology 2004; 9: 433-6 4. Buchanan GR, DeBaun MR, Quinn CT, Steinberg MH. Sickle cell disease. Hematology 2004;:35-47. Conflict of Interest:None declared |
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