A Life-Threatening Sickle Cell Crisis

AbstractBackground and aims:Acute pain episodes associated with sickle cell disease (SCD) are very difficult to manage effectively. Opioid tolerance and side effects have been major roadblocks in our ability to provide these patients with adequate pain relief. Ketamine is cheap, widely safe, readily available drug, with analgesic effects at sub-anesthetic doses and has been used in wide range of surgeries, pediatric burns dressing change and cancer related pain however, literature concerning its use in sickle cell crises is still limited in our setting. This study aimed to establish if 1 mg/kg of intravenous ketamine is non inferior to intravenous morphine 0.1 mg/kg in severe SCD-associated pain.Methods:We performed an institutional review board-approved randomized, prospective, double-blinded, active-control, non-inferiority trial at the national referral sickle cell center. Children between 7 and 18 years of age with severe painful sickle cell crisis, defined by numerical rating scale score of greater or equal to 7 were enrolled. Patients were consented and randomized to receive, either IV ketamine (LDK) 1 mg/kg or IV morphine (MOR) 0.1 mg/kg as an infusion over 10 min. The primary endpoint is maximal change in Numerical Rating Scale (NRS) pain score. Secondary outcomes were, incidence of adverse effects, optimal time to and duration of action of ketamine and incidence of treatment failures by treatment group. A clinically meaningful difference in validated pain scores was defined as 1.3 units. Assuming both treatments are on average equal, a sample size of 240 patients (120 per group) provided 95% power to demonstrate that IV LDK is non-inferior to IV morphine with a 0.05 level of significance and a 10% non-inferiority margin. All analyses were based on a modified intention to treat. This trial was registered with clinicaltrials.gov NCT02434939.Results:Two hundred and forty patients were enrolled (LDK120, MOR120). Demographic variables and baseline NRS scores (8.9 vs. 9.2) were similar. LDK was comparable to MOR in the maximum change in NRS scores, 66.4% vs. 61.3% (MD 5.5; 95% CI −2.2 to −13.2). Time to achieve maximum reduction in NRS pain scores was at 19.8 min for LDK and 34.1 min for MOR. The average duration of action for LDK was 60 min. MOR had more patients still at maximum effect at 120 min (45.8% vs. 37.5%; RR 1.2; 95% CI 0.9–1.7). LDK patients were 11.3 times more likely to develop side effects, though were transient, anticipated and non-life threatening (37.5% vs. 3.3%). MOR had significantly more treatment failures 40% vs. 28.3% (RR 0.7; 95% CI 0.5–1.03,p=0.07) Vital signs and sedation scores were similar in both groups.Conclusions:Intravenous LDK at 1 mg/kg provides comparable analgesic effectiveness as IV MOR in the acute treatment of severe painful sickle cell crisis in children in the day care sickle cell center. However, it is associated with a high incidence of several transient, non-life threatening mild side effects.Implications:Intravenous ketamine at 1 mg/kg can be a reliable alternative to morphine in the management of severe painful sickle cell crisis especially in a resource limited area where morphine is not readily available.

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Hyperhaemolysis in Sickle Cell Disease Is Not Necessarily a Transfusion Reaction

Blood ◽

10.1182/blood.v126.23.4595.4595 ◽

2015 ◽

Vol 126 (23) ◽

pp. 4595-4595 ◽

Cited By ~ 2

Author(s):

Elizabeth A Jones ◽

Louise Smith ◽

Russell D Keenan

Keyword(s):

Intensive Care ◽

Sickle Cell Disease ◽

Blood Transfusion ◽

Organ Failure ◽

Sickle Cell ◽

Red Cell ◽

Cell Disease ◽

Sickle Cell Crisis ◽

Life Threatening ◽

Red Cell Transfusion

Abstract Hyperhaemolysis is a rare life threatening complication in sickle cell disease with rapidly dropping haemoglobin, intravascular haemolysis and haemoglobinuria leading to multi organ failure and death. The literature reports that hyperhaemolysis in sickle cell disease is a complication of red cell transfusion (Aragona et al., 2014 J. Pediatr. Hematol. Oncol.) and suggests management based on with holding further transfusion to avoid aggravating the haemolysis and using immunosuppression (Win 2009 Expert Rev. Hematol.). In the literature, all cases of hyperhaemolysis in addition to a recent blood transfusion, were in or had had a recent sickle cell crisis. We report a case of life threatening Hyperhaemolysis in a 5 year old child following a sickle cell crisis who had never previously been transfused. We suggest that, at least in this case, the hyperhaemolysis cannot be transfusion related. The theoretical case for management of withholding transfusion may not be sound and potentially dangerous. A female child with known sickle cell disease presented with temperature and chest pains, she had a Hb 72g/L (stable over a few years). She initially improved with oxygen, fluids and antibiotics. 36 hours after admission she acutely deteriorated with increasing pallor and dropping oxygen saturations. She started passing frank red urine which initially was considered to be haematuria but on investigation was haemoglobinuria. Her Hb dropped to 47g/L with no evidence of blood loss. Within hours of developing haemoglobinuria she required intensive care for respiratory support. She rapidly developed multi-organ failure requiring oscillatory ventilation, inotropes, and haemofiltration for renal support. She was managed with emergency red cell transfusion (her first ever) and within 12 hours of haemoglobinuria received a full red cell exchange transfusion. There were ongoing antibiotics for clinical respiratory infection and she was later confirmed to have influenza B. No steroids or other immune suppression were given. There was no evidence of acute bleeding to explain a drop in haemoglobin at any point. With maximum intensive care support including further transfusions she gradually improved and has made a full recovery. No deterioration was observed following transfusion. She has remained well since. She is now 13 years old and following such a dramatic episode she has remained on a transfusion programme with successful oral iron chelation. She has not experienced any further episodes of hyperhaemolysis and no red cell antibody has been detected at any time. This case demonstrates that hyperhaemolysis in sickle cell disease does not require a previous transfusion. We suggest that it is possible the previous reported cases are also not due to blood transfusion but are an acute form of haemolysis seen on the background of a chronic haemolytic disease. An increase in the rate of haemolysis may be related to other acute complications of sickle cell disease. We propose that the optimum management of hyperhaemolysis should include full supportive care including maintaining haemoglobin by transfusion. Immunosuppression in this case could have led to a worse outcome as influenza pneumonia was the likely initial trigger of the episode. Disclosures No relevant conflicts of interest to declare.

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Fluosol in the treatment of sickle cell crisis

Transfusion Science ◽

10.1016/s0955-3886(96)90130-7 ◽

1996 ◽

Vol 17 (2) ◽

pp. 309-313

Author(s):

E LEEWADE

Keyword(s):

Sickle Cell ◽

Sickle Cell Crisis

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SICKLE CELL CRISIS PRESENTING AS A NUMB CHIN SYNDROME IN A CHILD

Neuropediatrics ◽

10.1055/s-2006-943659 ◽

2006 ◽

Vol 37 (S 1) ◽

Author(s):

M Srour ◽

S Abish ◽

D Mitchell ◽

C Poulin

Keyword(s):

Sickle Cell ◽

Numb Chin Syndrome ◽

Sickle Cell Crisis

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Concurrent Bilateral Central Retinal Artery Occlusion Secondary to Sickle Cell Crisis

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/23247096211028392 ◽

2021 ◽

Vol 9 ◽

pp. 232470962110283

Author(s):

Gowri Renganathan ◽

Piruthiviraj Natarajan ◽

Lela Ruck ◽

Roberto Prieto ◽

Bharat Ved Prakash ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Vision Loss ◽

Central Retinal Artery Occlusion ◽

Retinal Artery Occlusion ◽

Artery Occlusion ◽

Cell Disease ◽

Sickle Cell Crisis ◽

History Of ◽

Retinal Artery

Vascular occlusive crisis with a concurrent vision loss on both eyes is one of the most devastating disability for sickle cell disease patients. Reportedly occlusive crisis in the eyes is usually temporary whereas if not appropriately managed can result in permanent vision loss. A carefully managed sickle cell crisis could prevent multiple disabilities including blindness and stroke. We report a case of a 24-year-old female with a history of sickle cell disease who had acute bilateral vision loss during a sickle crisis and recovered significantly with a timely emergent erythrocytapheresis.

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Young child with painful edema and purpura: a case report

Italian Journal of Pediatrics ◽

10.1186/s13052-021-01001-y ◽

2021 ◽

Vol 47 (1) ◽

Author(s):

Sarah Contorno ◽

Giorgio Cozzi ◽

Irene Berti ◽

Egidio Barbi ◽

Andrea Taddio

Keyword(s):

Differential Diagnosis ◽

Child Abuse ◽

Case Report ◽

Young Child ◽

Sickle Cell ◽

Benign Condition ◽

Sickle Cell Crisis

Abstract Background We reported the case of a two-old-year boy with a painful acute hemorrhagic edema. This is a self-limited benign condition: usually, affected children are well appearing and this strongly support the diagnosis. In the opposite, in our case, we observed a painful presentation of the edema. Therefore, we demonstrated that rarely, this condition could have also a painful presentation. Conclusions This case report helps clinician to know that also acute hemorrhagic edema could have a painful presentation, so we must considered it in the differential diagnosis with sepsis, sickle cell crisis and child abuse. We believe that these findings will be of interest to pediatricians.

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