Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi
Part 7 of 11 in Diagnosis and Management of Sickle Cell Disease
Priapism, Infections and Aplastic Crisis in Sickle Cell Disease
November 24, 2024
Priapism
Priapism is a sustained, unwanted, painful erection lasting more than four hours, caused by venous occlusion reducing venous outflow from the penis. It is extremely painful, and severe or recurrent episodes can lead to permanent erectile dysfunction; the lifetime prevalence in boys and men with sickle cell disease has been reported as high as 20 to 30%, and in some series up to 89%. Treatment is supportive, with IV hydration and pain management, and vasoactive agents have been used; if the episode is prolonged, a urology consultation is needed, since some cases require surgical intervention for relief.
Severe Bacterial Infection
Functional asplenia typically develops by two to three years of age, as recurrent splenic infarcts destroy the spleen's tissue. Once this happens, the risk of infection with encapsulated bacteria, particularly Streptococcus pneumoniae, rises sharply, and these infections can present as septicaemia, meningitis or osteomyelitis. Patients are also more susceptible to Salmonella, Haemophilus influenzae, Meningococcus and Mycoplasma infections. This is why the conjugate pneumococcal vaccine, routine childhood vaccination, and penicillin prophylaxis, started as early as possible, are all standard parts of care.
Acute Aplastic Crisis
Acute aplastic crisis is caused by parvovirus B19 infection and is common in children with HbSS disease. It presents as sudden-onset fatigue, shortness of breath and fever, with haemoglobin sometimes falling suddenly by 3 to 6 g from baseline and reticulocyte counts dropping. Treatment is supportive care together with red blood cell transfusion. A useful feature of this complication is that it does not recur: infection with parvovirus B19 confers lifelong humoral immunity.
This article is based on a Jivo Masterclass session conducted by Dr. Divya Doval, Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi. The article has been summarised with the assistance of an AI tool from the original masterclass recording. Watch the full Masterclass recording
This guide is based on a live Jivo Masterclass: Dr. Divya Doval taught doctors across Africa on November 24, 2024.
FROM THE LIVE Q&A
Moderator
Is sickle cell trait a disease?
Dr. Divya Doval
Sickle cell trait itself I don't consider a disease, since it doesn't manifest with any problems in most people. But sickle cell disease has a whole spectrum, and once the pain crises and other manifestations begin, we definitely have to treat it as a disease.
Frequently Asked Questions
What is the success rate of managing sickle cell disease with stem cell transplant?▼
Stem cell transplant has a very good outcome, especially in younger children. Even with a haploidentical stem cell transplant, we have reported outcomes between 70 to 80 percent overall survival, and matched sibling data was close to 90 percent. It is a one-time treatment and the cure is for life, whereas managing sickle cell disease is an ongoing process, one complication after another, and cognitive dysfunction from silent strokes can build up over time even with careful management. Doing the transplant at a younger age gives a better outcome.
What is the management of vaso-occlusive crisis in sickle cell disease? Is there a curative treatment for it?▼
There is no definitive curative treatment for vaso-occlusive crisis. We can only support with oxygen, fluids and painkillers, and if the pain is excessive and not controlled, a blood transfusion.
Under what age is stem cell transplant effective?▼
There is no age limit. We have transplanted adults as well. The risk simply increases with age and the outcomes are not as good, but otherwise transplant can be done at any age if the organ systems are fine. With age, the risk of stroke and of the liver and kidneys being affected increases, which then reduces eligibility for transplant.
What is the overall cost of bone marrow transplant at your facility?▼
If it's a matched sibling donor, an HLA-matched sibling, it is somewhere between 20,000 and 24,000 US dollars. If a matched sibling is not present and it is a half-match transplant, the price goes up to around 35,000.
What is the role of urine alkalisation in the management of vaso-occlusive crisis?▼
Alkalinisation of the urine prevents acidosis and infections, and once the urine is alkaline, crystallisation is prevented as well. Overall, it helps with the pain.
In This Series: Diagnosis and Management of Sickle Cell Disease
- 1.Diagnosis and Management of Sickle Cell Disease
- 2.Sickle Cell Disease Explained: Inheritance, Haemoglobin S and Why Cells Sickle
- 3.Diagnosing Sickle Cell Disease: From Newborn Screening to Confirmatory Testing
- 4.Pain Crisis and Acute Chest Syndrome: The Two Most Common Sickle Cell Emergencies
- 5.Stroke Risk in Sickle Cell Disease: Screening and Preventing a Devastating Complication
- 6.How Sickle Cell Disease Damages the Spleen, Liver, Kidneys and Eyes
- 7.Priapism, Infections and Aplastic Crisis in Sickle Cell Disease
- 8.Hydroxyurea and Newer Drug Therapies for Sickle Cell Disease
- 9.Blood Transfusion and Iron Overload Management in Sickle Cell Disease
- 10.Bone Marrow Transplant and Gene Therapy: Curing Sickle Cell Disease
- 11.Sickle Cell Disease and Pregnancy: Managing a High-Risk Combination