A doctor will use several treatments to treat sickle cell disease, depending on the symptoms of your child and his or her age. A hematologist specializes in blood disorders. Treatments will include IV fluids and pain medicines to manage any symptoms, including pain crises. Treatment may also include blood transfusions for anemia, chronic pain, acute chest syndrome, splenic sequestration, and a stroke. Early diagnosis is key to effective treatment.
There are countless benefits of blood transfusions for patients with sickle cell disease, including improved oxygen delivery and fewer blocked blood vessels. Often administered in a hospital or infusion clinic, blood transfusions may also be life-saving. They may prevent the degeneration of brain tissue or even reduce the risk of stroke in patients with sickle cell disease. However, blood transfusions aren’t appropriate for every patient.
Some side effects of blood transfusions for sickle cell disease treatment are not good. These include fever and alloimmunization. Often, blood transfusions are combined with iron chelation therapy. Patients should consider all these factors before committing to a long-term transfusion program. The risk-benefit ratio will vary from person to person and may change over time. Some patients may experience fewer or no side effects and others may experience adverse reactions.
Stem cell transplants
Stem cell transplants are effective treatments for sickle cell disease, a debilitating inherited disorder of the red blood cells. Sickle cells are abnormal in shape and are the cause of downstream pathological changes such as abnormal rheology and vaso-occlusion. Hemolysis also leads to an abnormal rheology, which causes organ dysfunction. However, hematopoietic cell transplants can treat sickle cell disease, providing long-term symptomatic improvement and a cure.
During a stem cell transplant, the patient undergoes pre-transplant tests and blood transfusions until their own immune system is strong enough to make healthy blood cells. In addition, the patient may undergo radiation therapy to weaken their immune system so that it will not attack the new stem cells. A donor’s cells replace the sickle cell patient’s bone marrow. They should begin making new healthy blood cells soon after the transplant.
A diagnosis of priapism is not difficult to make, but there are certain diagnostic tests that must be performed to determine the underlying cause. Diagnostic tests, such as a sickle solubility test, should be performed as early as possible and concurrently with treatment. The penile blood gas measurement is one such test, in which a needle is inserted into the penis to measure the gases in the blood. A black-coloured penile blood reflects an ischemic priapism, whereas bright red blood indicates a nonischemic priapism.
During this procedure, penile aspiration was combined with an extracavernous a-agonist injection and dorsal nerve blockade. The patient received cyproterone acetate 50 mg/day for three weeks, which was recommended by existing literature on ischemic priapism in general population. The erectile tissues were ischemia-prone. The ischemic penile tissues were deoxygenated and erectile dysfunction resulted.
In infants, sickle cell disease is usually asymptomatic. By six months of age, the red blood cells start to produce abnormally shaped fragments called sickle cells. These fragments can block blood vessels, causing pain and a potentially life-threatening situation known as a sickle cell crisis. These crises are also more likely to happen in children who are dehydrated or injured. Early diagnosis and treatment are essential to prevent major complications and a life of chronic transfusions.
Fortunately, mortality and morbidity rates of children with sickle cell disease have decreased over the past 25 years, due in part to improved screening tests. Prophylactic penicillin administration following birth and timely administration of H. influenzae type b and pneumococcal vaccines have reduced sepsis and other complications. Infants with sickle cell disease may benefit from optimal nutrition and screening to detect potential complications.
Some complications of sickle cell disease treatment include lung damage, stroke and seizures. The sickle cells stick to vessels, causing blockages. As a result, blood flow is restricted and oxygen can’t reach nearby tissues. Because of this, pain can suddenly become severe, and an attack can be fatal. People with sickle cell disease also have more heart problems, including heart attacks and irregular heart rhythms. A bone marrow transplant is one treatment option for patients with SCD.
Some children with sickle cell disease experience severe bleeding and other complications. If a child has an infection, antibiotics are usually used. Other complications include leg ulcers and acute chest syndrome. These conditions require prompt referral to pediatric tertiary care. Acute chest syndrome and severe pain may require blood transfusions. Some children also develop cholelithiasis. While there is no cure for sickle cell disease, treatment options can be life-threatening.