Sickle cell anemia–Causes-Symptoms-Diagnosis-Treatment–Prognosis

The treatment of SCA depends on the symptoms presented by the affected individual. As a genetic disease it is not possible to cure SCA. Hence treatment is almost always focused on managing the disease and preventing crisis.

Fatigue and Anemia- fatigue is very commonly associated with anemia. In an SCA patient the RBCs are very brittle and this results in a severe drop in blood cells. Hb levels in individuals with sickle cell anemia are usually between 6 to 8 gms/dl (normal value is between11 -13gms/dl). When the body is unable to makes up for the loss it results in ‘crisis’. A blood transfusion may then be required to compensate the loss.

Pain Crises- Excruciating pain occurs due to impaired blood circulation in SCA patients. This usually requires hospitalization and narcotic medications for relief. Hydroxy urea is now a popular medication to tackle pain and to increase the amount of fetal HB, which is resistant to sickling. Hydroxyurea is also given to prevent pain and not after it arrives. This medication has a few side effects, such as increasing risk for infections.

As a preventive measure SCA patients must avoid the following pain triggers-

Dehydration,

Infection/ injury,

Exposure to extreme heat / cold,

Emotional stress,

Strenuous exercise.

Pain crises require medications for pain and increased fluid intake. Sometimes hospitalizations may be required when pain is excruciating. Dehydration must be prevented to avoid further injury to the tissues and intravenous fluids may be necessary. Along with the fluids clotrimazole and magnesium are also given.

Medications for pain relief include-

Acetaminophen,

Nonsteroidal Anti-Inflammatory Drugs (NSAIDs),

Narcotics like meperidine, morphine, oxycodone,

Other methods to tackle pain include self-hypnosis, bio feedback and electrical nerve stimulation (ENS).

Dactylitis and Arthritis -Swelling and inflammation of the hands and feet is a prominent feature of sickle cell anemia and this can be relieved by administering anti-inflammation medications, such as ibuprofen and aspirin.

Bacterial Infection – Lung infection, Osteomyelitis and meningitis are common among the SCA-affected. This must be detected and treated early with antibiotics. Hospitalization may also be required.

Splenic Sequestration - Transfusion of blood may be required. Sometimes removal of the spleen may be imperative.

Lung and Heart Injury –This problem is usually treated depending on the type and degree of damage. This may include oxygen supplementation, medications, blood transfusion or treatment with antibiotics.

Leg Ulcers in SCA patients can be managed by medications, dressings, elevation of the leg, topical therapies and sometimes skin grafting.

Aseptic Necrosis / Bone Infarcts – Rest and Pain managing medications are necessary to manage this condition. In some cases joint replacement is necessary.

Priapism –This condition in male patients can be treated with fluids, medication, aspiration of blood from the penis or by a surgery.

Eye Damage- If bleeding into the eye or retinal detachment is detected early, appropriate measures such as laser therapy can be carried out to prevent blindness.

Gall stones –If gall stones results in gall bladder disease then surgery is required to remove the affected bladder.

Strokes - Transcranial doppler ultrasound is routinely taken in children with SCA to check blood flow in the brain. This method helps to detect patients who are at a high risk for stroke.

In a small number of very young patients, with severe form of the disease, bone marrow transplants could help reduce the pain episodes.

Gene therapy, wherein the abnormal gene of the patient is replaced by normal gene, is another modern treatment that is being considered to alleviate the sufferings of SCA patients.

Newer medicines used in SCA treatment-

Butyric acid - Increase the level of normal hemoglobin in the blood.

Nitric oxide- keeps sickle cells less sticky and helps to open blood vessels.

Decitadine –increases fetal hemoglobin levels.