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Inhaled drugs for opening up the airways in cases of acute chest syndrome in people with sickle cell disease

We reviewed the evidence about whether using inhaled, short‐acting bronchodilators for acute chest syndrome reduces illness and mortality in people with sickle cell disease and to assess whether this treatment causes adverse effects. This is an update of a previously published Cochrane Review.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

Treatments for long‐lasting and painful erection of the penis in boys and men with sickle cell disease

Priapism (the prolonged painful erection of the penis) is common in males with sickle cell disease (SCD). The length of time priapism lasts differs for different types and so does the medical treatment for it. Self‐management approaches may be helpful. We looked for randomised controlled trials of different treatments to find the best option. We found one study set in Jamaica in 1985 with 11 people. In the study, stilboestrol was compared to placebo. The study only reports one of the outcomes from the review, how often stuttering priapism occurs. There is no difference between treatment groups for this outcome. Due to lack of evidence, we are not able to conclude the best treatment of priapism in SCD. Alpha agonists have become the treatment of choice for acute fulminant priapism and to prevent stuttering priapism. However, this is not supported by a randomised controlled trial. More research is needed. One trial is under way in the UK at this moment in time.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2010

Replacing fluids to treat acute episodes of pain in people with sickle cell disease

Sickle cell disease is a common genetic disorder characterised by periodic episodes of pain which usually happen again and again throughout life. These episodes occur when sickled cells obstruct blood vessels. The degree of pain may range from a mild discomfort to a severe disabling pain where the person needs treatment in hospital. A drop in body fluid levels promotes and sustains the sickling process. Routine treatment includes the use of drugs to relieve pain and the maintenance of adequate fluid levels. Fluid levels will fall if insufficient fluids are taken in compared to the amount of fluid lost. The kidneys of people with sickle cell disease do not concentrate urine properly, which results in poor control of bodily fluids. Additional fluids are given to try and slow or stop the sickling process which should reduce the amount and duration of pain. Fluids may be given in many ways, but orally or intravenously are most common. Fluids should be given with care so as to prevent fluid overload which may in turn cause adverse events such as heart failure or fluid building up in the lungs. We looked for randomised controlled trials to show the best way to replace fluids, which type of fluids and how much fluid to replace to treat acute episodes of pain in people with sickle cell disease. We did not find any such trials. We conclude that there is a need for large multi‐centre trials to examine these questions.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Removing spleens from people with sickle cell disease after a splenic sequestration compared to blood transfusions to prevent further attacks

We reviewed the evidence to see whether removing all, of part of, the spleen to prevent acute splenic sequestration improved survival and decreased illness in people with sickle cell disease, as compared with regular blood transfusion. This is a 2015 update of a Cochrane review first published in 2002, and previously updated in 2013.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Regular antibiotics for preventing pneumococcal infection in young children with sickle cell disease

People with sickle cell disease (SCD) are especially prone to respiratory infections and blood poisoning. These infections are often caused by pneumococcal bacteria. Infections occur partly due to the spleen not working correctly, but also because damaged tissue and bone resulting from SCD can harbour bacteria. The highest risk of infection occurs in children under three years old, but the usual pneumococcal vaccines are of limited use in these patients. Therefore regular antibiotics are needed to prevent infection. As risk of infection decreases with age, there might be a time when preventative antibiotic treatment can be halted. Three trials with over 800 children are included in the review. All three trials showed a reduced rate of infection in children with sickle cell disease receiving penicillin preventatively. Two trials looked at whether treatment was effective. The third trial followed on from one of the early trials and looked at when it was safe to stop treatment. Adverse drug effects were rare and minor. However, there were problems with children keeping to the treatment schedule and with the development of antibiotic resistance. We conclude that penicillin given preventatively reduces the rate of pneumococcal infections in children with sickle cell disease under five years old. The risk of infection in older children is lower, and the follow‐on trial did not show a significant increase in risk when regular penicillin was halted at five years old. Further research should look at how common and how clinically important resistant bacteria are.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Pneumococcal vaccines for sickle cell disease

People with sickle cell disease are particularly prone to pneumococcal infection, which may be fatal. Children aged up to 23 months are at high risk, but usual polysaccharide pneumococcal vaccines may not work in this age group. New conjugate pneumococcal vaccines may help to reduce the rate of infection in people with sickle cell disease of all ages. We searched for trials which compared a polysaccharide or conjugate pneumococcal vaccine schedule with a different schedule or no vaccination in people with sickle cell disease. The review includes five trials with a total of 547 participants. One trial showed that the polysaccharide vaccine did not reduce the risk of infection very much in children younger than three years old, but it was linked with only minor adverse events. Three trials of conjugate vaccines showed increased antibody responses compared to control groups in people of all ages, although clinical outcomes were not measured in these trials. This review did not show if the vaccines prevent infection or decrease death rates. We recommend that conjugate pneumococcal vaccines are used in people with sickle cell disease. Randomised trials will be needed to determine the best vaccination schedule when further, potentially more effective vaccines become available. Such trials should measure clinical outcomes of effectiveness.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2012

Transplantation of blood‐forming stem cells for children with sickle cell disease

We reviewed the evidence about the cure rate and risks of hematopoietic stem cell transplantation for people with sickle cell disease.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

Piracetam for reducing the incidence of painful sickle cell disease crises

We reviewed the evidence to assess the effectiveness of piracetam for reducing the incidence of painful sickle cell disease crises. This is an update of a previously published Cochrane review.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Treatments for people with sickle cell disease in whom poor blood supply to an area of bone leads to bone death

We reviewed the effects of treatments for avascular necrosis of bone in people with sickle cell disease.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

Psychological therapies for sickle cell disease and pain

Sickle cell disease is a group of blood disorders. It can cause anaemia, which if severe can reduce mobility. It may also allow small blood vessels to become blocked causing pain in muscle and bone and it can damage major organs such as the spleen, liver, kidneys, and lungs. People with sickle cell disease are more vulnerable to severe infections. Treatment is usually in response to symptoms and designed to ease pain. Psychological treatment to help people cope with sickle cell disease might complement current medical treatment. There are four types of treatment: patient education; cognitive therapy (to do with thoughts and feelings); behavioural therapy (to do with actions); psychodynamic psychotherapy (talking to relieve emotional pain). We searched for randomised or quasi‐randomised controlled trials which compared psychological treatments to each other or to no treatment in sickle cell disease. We included seven studies in the review, of which five, with 260 people, had data we could enter into the review. One study showed that cognitive behaviour therapy reduced the affective part of pain (feelings about pain), but not the sensory part (pain intensity). Another study of this therapy had inconclusive results for coping strategies and showed no difference on how different groups used the health service. A study using cognitive behavioural therapy with teenagers and their families at home did not show any difference when compared with education about sickle cell disease. One education study did not show a reduction in depression. Furthermore, one study in patient education helped improve attitudes to healthcare workers and medication use in adolescents and young adults. The authors believe that some patient education seems relevant for children, adolescents and young adults, while methods to improve the ability to cope in both children and adults are important. Nonetheless, these results may not apply to across all ages, clinical severity, types of pain (acute or chronic) that people with sickle cell disease have, or which country they live in. More research needs to be done in this area.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Vaccines for preventing severe salmonella infections in people with sickle cell disease

Salmonella organisms are probably second only to pneumococcus among bacterial causes of infection in people with sickle cell disease. Infection with these bacteria can lead to complications and reduce the quality of life of people with the disease and sometimes result in death. Immunization with salmonella vaccines is one of the interventions available to reduce infection by these bacteria. There are different types of vaccines available: the inactivated vaccines and the oral vaccines. We did not find any randomized controlled trials assessing these vaccines in people with sickle cell diseases. We therefore conclude that there is a need for a randomized controlled trial to assess the benefits and risks of the different types of vaccines to evaluate the potential for improving survival and decreasing mortality from salmonella infections in people with sickle cell disease.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Disclosing to parents newborn carrier status identified by routine blood spot screening

No guidance is available on the best approach to disclosing to parents newborn carrier status inadvertently identified by routine newborn blood spot screening.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

Hormone contraceptives for women with sickle cell anemia

Whether women with sickle cell anemia should use hormonal birth control is unknown. Sickle cell anemia is a blood disease. This type of anemia also causes bone pain known as sickle pain crises. A concern is that women with this disease using hormonal birth control may have blood vessels blocked by blood clots or have more bone pain. Clinicians often do not prescribe these types of birth control due to these concerns. However, many women with sickle cell anemia are sexually active, are able to get pregnant and are interested in contraception.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2011

Antibiotics for treating osteomyelitis in people with sickle cell disease

We reviewed the evidence to determine whether antibiotics (alone or in combination) given to people with sickle cell disease who have osteomyelitis (a bone infection) before the specific bacterium causing an infection is known is effective and safe as compared to bacterium‐directed antibiotic treatment and whether this effectiveness and safety is dependent on different treatment regimens, age or setting. This is an update of a previously published Cochrane Review.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

Gene therapy for sickle cell disease

We reviewed the evidence about the effect and safety of gene therapy on survival and on preventing symptoms and complications associated with sickle cell disease.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

Antibiotics for treating acute chest syndrome in people with sickle cell disease

Sickle cell disease affects millions of people throughout the world. Acute chest syndrome is a major cause of illness and death in people with sickle cell disease. Symptoms include fever, chest pain and a raised white blood cell count. Acute infection of the lung tissue is a major cause of acute chest syndrome. Antibiotics are often given to treat these lung infections, but there is no worldwide standard treatment. We searched for randomised controlled trials which compared antibiotics (alone or in combination) with other antibiotics, placebo or standard treatment. We wanted to know if the different antibiotic treatments were effective, if they were safe, and which doses worked best for acute chest syndrome in people with sickle cell disease. This update was unable to find any trials to include in this review. We conclude that a randomised controlled trial should attempt to answer these questions. Until there is firm evidence, clinicians should treat acute chest syndrome on a case by case basis and according to the diagnosis and the treatment available.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Phytomedicines (medicines derived from plants) for sickle cell disease

Sickle cell disease is an inherited blood condition caused by defects in the production of haemoglobin. Haemoglobin is the part of the red blood cell that carries oxygen across the body. Sickle cell disease occurs when people inherit faulty genes responsible for producing haemoglobin from both parents. A variety of complications and a reduced life expectancy are linked with sickle cell disease. Phytomedicines are medicines derived from plants in their original state. People with sickle cell disease may come across them in terms of plant‐remedies from traditional healers. Their benefits have not been evaluated systematically. Laboratory work has long suggested that these medicines may help to ease the symptoms of sickle cell disease.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Testing newborn babies for sickle cell diseases

Sickle cell diseases are inherited and affect mainly people of African origin. The red blood cells are abnormally (sickle) shaped, which can lead to life‐threatening complications. They are most likely to be fatal in the first few years of life since affected children are at higher risk of serious infections. Regular antibiotics and immunisations reduce the risk of infections, and if sickle status is known, can be started early. Screening babies allows early diagnosis and therefore early treatment. Screening may also have disadvantages. This review aims to assess whether screening compared to diagnosis from symptoms leads to less morbidity and mortality. The authors were not able to find trials that assessed the benefits and harms of screening. There is evidence that starting treatment early is of benefit. Early treatment is made possible by screening in the neonatal period. There are some reports in non‐trial literature which suggest that newborn screening is appropriate based on currently available evidence. Healthcare providers must assess whether these reports are relevant to their practice and situation when deciding whether to screen for SCD in the neonatal period. Practice recommendations could be made from the results of a prospective randomised controlled trial. Such a trial may be thought to be unethical given the proven benefit of early preventative treatment of children with penicillin. There are no trials included in the review and we have not identified any relevant trials up to July 2008. We therefore do not plan to update this review until new trials are published.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2010

Inhaled nitric oxide for acute chest syndrome in people with sickle cell disease

Sickle cell disease is an inherited blood disorder affecting approximately 250 million people worldwide. Sickle‐shaped red blood cells which are characteristic of sickle cell disease may block blood vessels causing pain, tissue death and even severe damage in the major organs. Similar blockages in the blood vessels of the lungs can lead to lung injury and a complication known as acute chest syndrome which occurs in approximately 30% of people with sickle cell disease. Common symptoms include fever, coughing, chest pain and shortness of breath; some of which can be life‐threatening. Treatment is mainly supportive and given when an individual experiences symptoms and may include antibiotics, drugs to help prevent the clotting of blood and other conventional treatments. Interest has been shown recently in inhaling nitric oxide, a soluble gas. This gas is known to play a role in expanding blood vessels and clumping platelets, to relieve some of the symptoms of acute chest syndrome. It is also used for treatment in similar conditions, namely pulmonary hypertension (high blood pressure in the blood vessels of the lungs) in babies up to four weeks old. The authors of the review did not find any trials showing how effective inhaled nitric oxide is for acute chest syndrome in people with sickle cell disease. The authors concluded that future research should provide evidence for people to make informed decisions about whether nitric oxide is effective.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

Antibiotics for treating pneumonia caught outside of hospital or care homes in people with sickle cell disease

We reviewed the evidence on the effect and safety of the antibiotic treatment approaches (one drug alone or combined drugs) for people with sickle cell disease with from community‐acquired pneumonia. This is an update of a previously published Cochrane Review.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

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