Senior Consultant, BMT, Haematology & Paediatric Haemato-Oncology, Artemis Hospitals, Gurugram
Part 3 of 11 in Diagnosis and Management of Sickle Cell Disease
Recognizing the Symptoms and Complications of Sickle Cell Disease
November 9, 2025
Chronic anaemia from the shortened red cell lifespan causes persistent fatigue, while blocked small vessels cause the defining feature of the disease: painful vaso-occlusive crises in the bones, joints, chest and abdomen. Pain intensity, frequency and duration vary considerably between patients who share the same HbSS genotype, from short acute episodes to debilitating chronic pain.
Dactylitis, priapism and organ complications
Swelling of the hands from small vessel blockage, called dactylitis, is more common in children, since adults have usually developed collateral vessels that reduce its frequency. Young male patients can develop priapism from the same vaso-occlusive process, and progressive splenic vessel damage can shrink the spleen over time, weakening the humoral immune protection it normally provides and raising the risk of infection from encapsulated organisms such as pneumococcus and meningococcus. Delayed growth is another important sign to watch for in affected children.
Acute chest syndrome and other crises
Acute chest syndrome presents with chest pain, fever and breathing difficulty from blocked pulmonary vessels, sometimes triggered by a lung infection, and can progress to pulmonary hypertension over time. Continuous red cell breakdown raises bilirubin and can cause gallstones and leg ulcers. Acute stress, high altitude or fever can trigger a hyperhaemolytic crisis, a sudden drop in haemoglobin from excess breakdown that can compromise kidney blood flow. A parvovirus infection can trigger an aplastic crisis, since the bone marrow in sickle cell disease is already vulnerable to the marrow suppression parvovirus causes on its own.
This article is based on a Jivo Masterclass session conducted by Dr. Sukriti Gupta, Senior Consultant, BMT, Haematology and Paediatric Haemato-Oncology, Artemis Hospitals, Gurugram. The article has been summarised with the assistance of an AI tool from the original masterclass recording. Watch the full Masterclass recording
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This guide is based on a live Jivo Masterclass — Dr. Sukriti Gupta taught doctors across Africa on November 9, 2025.
FROM THE LIVE Q&A
Dr. Mercy
What types of stroke are sickle cell patients most likely to suffer from, and how can it be prevented?
Dr. Sukriti Gupta
The middle and posterior cerebral artery territories are affected most commonly, similar to the pattern seen in adults, though the anterior territory can occasionally be involved too. Regular transcranial Doppler screening from two years of age is the key prevention tool: it uses ultrasound to measure blood flow velocity in the cerebral vessels and identifies children at higher risk of stroke early, so that preventive medication and closer monitoring can begin before a stroke actually happens.
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Frequently Asked Questions
What is your comment on the use of hydroxyurea in pregnancy, and what are the alternative options?▼
In pregnant women who have generally done well and had infrequent pain crises up to childbearing age, hydroxyurea is usually held during pregnancy since it is not a good option for fetal development. Good hydration and avoiding any other stressful conditions are advised instead, and folic acid supplementation must continue.
At what age do we commence hydroxyurea?▼
As early as the disease is detected, sometimes as young as two months of age.
What is the upper age limit for bone marrow transplant, and what are the cost implications for full match versus half match donors?▼
The best outcomes are under 16 years, 16 to 25 is still viable, and beyond 25 the risks rise and require much more detailed pre-transplant workup, including cardiac and kidney function assessment, though age is not always a fixed barrier if organ function is well preserved and the family is fully committed. A full-match sibling transplant typically costs around $24,000 to $25,000, rising for older patients closer to adult body weight, while a half-match or unrelated-donor transplant through a registry costs around $33,000 to $34,000, since registry and donor-related costs are higher even though complications are reduced.
In terms of treatment, how is gene therapy done, and is it possible for all patients?▼
Gene therapy uses the patient's own stem cells rather than a donor, mobilised and collected the same way as for a transplant, then modified in a laboratory using either a viral vector for gene addition or CRISPR-Cas9 editing to correct the underlying genetic change, a process that currently takes around six months. Because the cells being returned are the patient's own, recovery is generally faster than with a donor transplant, but the therapy currently has FDA approval only for patients aged 12 and older, and it remains expensive and not yet widely available outside the US, Europe and a handful of other countries.
In developing countries where finances are a barrier for most families, would you recommend gene therapy or bone marrow transplant?▼
If finances are genuinely not a barrier, gene therapy is worth choosing once it is better established, since it avoids the donor-related risks. But where finances are a limiting factor, an experienced allogeneic bone marrow transplant programme should still be trusted: it has decades of mature outcome data behind it, and families should not exhaust all their resources chasing a gene therapy process that could stall partway through. If a patient is having an acute crisis such as a recent stroke, there is also often not enough time to wait the six to eight months gene therapy currently requires, and transplant should be pursued instead.
Why does sickle cell disease cause painful crises?▼
Sickle-shaped red cells clog small blood vessels, causing vaso-occlusive crises with pain in the bones, joints, chest and abdomen. The intensity, frequency and duration vary considerably even between patients with the same genotype.
Why are sickle cell patients at higher risk of certain infections?▼
Progressive damage to the spleen's blood vessels can shrink the organ over time, weakening the humoral immune protection it normally provides and raising the risk of infection from encapsulated organisms such as pneumococcus and meningococcus.
In This Series: Diagnosis and Management of Sickle Cell Disease
- 1.Diagnosis and Management of Sickle Cell Disease
- 2.Understanding Sickle Cell Disease: Genetics and Pathophysiology
- 3.Recognizing the Symptoms and Complications of Sickle Cell Disease
- 4.Stroke Risk and Screening in Sickle Cell Disease
- 5.Diagnosing Sickle Cell Disease: Screening and Prenatal Testing
- 6.Supportive Care and Pharmacological Treatment for Sickle Cell Disease
- 7.Bone Marrow Transplant for Sickle Cell Disease: Indications and Process
- 8.Finding a Donor Match for Bone Marrow Transplant in Sickle Cell Disease
- 9.Bone Marrow Transplant Complications and Post-Transplant Monitoring
- 10.Gene Therapy for Sickle Cell Disease: CRISPR Editing and Gene Addition
- 11.Sickle Cell Disease in Pregnancy