Loading…
Loading…
Sickle cell disease is an inherited haemoglobin disorder causing rigid, sickle-shaped red cells, pain crises and organ complications.
Quick Answer
Sickle cell disease is an inherited haemoglobin disorder causing rigid, sickle-shaped red cells, pain crises and organ complications. Dr. Sachita Baniya (NMC 20158, 8+ years of clinical experience in haematology and haemato-oncology) evaluates sickle cell disease at Kathmandu Neurology Clinic & Cognitive Center, 117 Lal Durbar Marg, Kathmandu, Bagmati Province 44600, Nepal — evidence-based, medically reviewed.
Sickle cell disease results from a beta-globin mutation. Sickled cells cause vaso-occlusion, haemolysis and chronic organ damage. Care includes hydration, pain management, vaccination, infection prevention and hydroxyurea in selected patients.
Diagnosis uses haemoglobin electrophoresis or HPLC. Newborn screening is available in some settings. Baseline organ assessment and family screening are important.
Care includes hydration, analgesia, vaccination, penicillin prophylaxis in children and hydroxyurea in selected patients. Acute chest syndrome, stroke and severe infection need urgent treatment. Transplant is curative in selected severe cases.
Management is individualized and discussed with benefits, limitations, and follow-up.
Carrier screening and genetic counselling reduce affected births. Avoiding dehydration, extreme cold and infection helps prevent crises.
Red flags — seek urgent care
Allogeneic transplant can cure selected patients. Gene therapy is emerging. Most care focuses on preventing and treating complications.
Dehydration, infection, cold, stress, hypoxia and menstruation can trigger crises. Avoiding triggers helps, but crises can still occur.
It increases fetal haemoglobin and reduces crises and hospitalisations in many patients. It requires monitoring of blood counts.
Sickle cell disease increases the risk of severe infection, especially pneumococcal infection. Vaccination and penicillin prophylaxis are essential.
Yes. Retinopathy can occur and requires regular eye screening.
Pregnancy carries higher risks for mother and baby and requires specialist care.
A serious complication with fever, chest pain, breathlessness and new lung infiltrates. It needs urgent treatment.
Regular blood counts, organ screening, vaccination review and pain management planning.
Specialist assessment for abnormal blood counts, anaemia, low platelets, bleeding, clotting and suspected blood disorders.
Structured investigation of low haemoglobin — iron deficiency, haemolysis and marrow causes.
Red cell and platelet transfusion support for haematological disease and treatment.
Iron deficiency anaemia is a common anaemia caused by insufficient iron for red cell production, usually from blood loss or poor absorption.
Thalassaemia is an inherited haemoglobin disorder caused by reduced or absent globin chain production.
Haemophilia is an inherited bleeding disorder caused by deficiency of clotting factor VIII or IX.
Content on this website is for general educational purposes only and does not constitute medical advice, diagnosis, or treatment. It is not a substitute for evaluation by a qualified healthcare professional. Always seek the advice of your physician or other qualified provider with any questions regarding a medical condition. Never disregard professional medical advice or delay seeking it because of something you have read on this website. In case of a medical emergency, contact emergency services immediately. No doctor–patient relationship is established by use of this site or by contacting the clinic through the site.
Reviewed by Dr. Jitendra Prasad Yadav • Last reviewed: 2026-11-10
Content is educational and aligns with standard medical references; individual evaluation may vary. External links provide context and do not imply endorsement.
Doctor — medically reviewed
Dr. Sachita Baniya
Consultant Haemato-Oncologist & Clinical Hematologist • MBBS, FCPS (Clinical Haematology) • NMC 20158
This condition guide was medically reviewed by Dr. Sachita Baniya.
On this site
Article information
Emergency
For sudden neurologic change, thunderclap headache, or seizure >5 min, seek emergency services now.