June 30, 2022

Sickle Cell Disease Symptoms and Treatments

Specialty Infusion Blog Jess

Sickle cell disease manifests as a genetic blood condition known as sickle cell anemia. The change in the red blood cells usually observed forms when you have sickle cell anemia, going from being round, flexible discs to rigid, sticky sickle cells that obstruct blood flow. About half of those with sickle cell anemia survive into their 50s through early identification and the latest therapies.

Compared with healthy red blood cells, sickle cells have a shorter lifespan. Anemia is called sickle cell anemia since a patient doesn’t produce enough healthy red blood cells.

Red Blood Cells

Children born with sickle cell disease hardly ever make it to adulthood. Nearly half of those with sickle cell anemia now reach their 50s owing to early identification and modern therapies. Sickle cell anemia patients continue to remain at risk for possibly fatal medical issues. Furthermore, medical professionals have procedures that lessen the likelihood of problems and soothe symptoms. Still, no appropriate medical therapy for sickle cell disease is accessible in many parts of the world.

Symptoms of Sickle Cell Disease 

The symptoms of sickle cell disease often appear in newborns around the age of five months. Each person’s symptoms are unique and evolve gradually. The following are the symptoms of SCD.

Anemia:

Sickle cells are more delicate than normal red blood cells and often disappear or die after 10 to 20 days. Almost 120 days is the average cell life span in this way. This leads to the loss of red blood cells, called anemia.

Fatigue results from a lack of red blood cells because it affects the transport of oxygen throughout the body.

Pain crisis:

Pain attacks are a common sign of sickle cell disease. Pain in your chest, belly, joints, and bones is brought on by red blood cells with a sclerotic form that obstructs blood flow via small blood arteries. The pain’s level and frequency might change, and sometimes, you might need to visit the hospital.

Hands and feet swelling:  

Swelling results from sickle-shaped red blood cells obstructing blood flow to the hands and feet.

Risk of infections:  

Ulcers may develop when sickle cells harm your tissues. Also, it’s possible you might get infections if they affect your spleen. Healthcare professionals usually give SCD patients immunizations and medications to avoid potentially harmful infections.

Jaundice or Yellowing of Skin and eyes: 

It may happen due to damaged sickle RBCs.

Vision issues:

Sickle cells may get lodged in the blood arteries that nourish your eyes, negatively affecting your retina and impairing your eyesight.

Growth Issues:   

Children with SCD may develop more slowly than other kids. Teenagers affected by SCD may reach puberty later than similar-aged teenagers.

Stroke:

One more severe and unexpected consequence affecting sickle cell patients is the chance of a stroke. The damaged cells might block the main blood arteries that provide oxygen to the brain. Severe brain injury may occur if the blood and oxygen supply to the brain is eliminated. A stroke caused by sickle cell anemia increases your chance of a second and third stroke.

Treatment of Sickle Cell Disease

Hydroxyurea (Droxia, Hydrea, Siklos)

Using daily hydroxyurea lowers the incidence of painful crises and may lessen the requirement for hospitalization and blood transfusions. However, it could enhance the chances of infections. If you are pregnant, avoid using the medication.

L-glutamine oral powder (Endari)

This drug recently got approval from the FDA to treat sickle cell anemia. The likelihood of pain can be minimized by using this drug.

Voxelotor (Oxbryta)

Adults and children over 12 may take this drug to treat sickle cell disease. This medication, when taken orally, may increase blood flow throughout the body and reduce the risk of anemia. The possible adverse effects are headache, nausea, diarrhea, exhaustion, rash, and fever.

Pain-relieving medications

For sickle cell-related pain issues, your doctor may also prescribe narcotic analgesics (painkillers) to reduce pain severity.

What is Adakveo?

Adakveo is a brand-name prescription drug called Crizanlizumab. This drug is FDA to avoid vaso-occlusive crisis (VOC) in adults and children 16 years of age and older with sickle cell disease. VOC is another name for a pain crisis.

The hereditary disorder sickle cell disease alters the structure of your red blood cells. Most often, sickle cell disease is discovered in newborns.

Few individuals with sickle cell disease have a pain crisis. Pain crises often result in intense pain. Additionally, this may result in a blood clot, raising the danger of organ damage and stroke.

Administration of Adakveo

Adakveo is administered as an intravenous (IV) infusion into your arm—it is a liquid solution. (An infusion is an injection that is slowly injected into your vein.) The dosage of the drug is 100 milligrams of the drug per 10 milliliters of solution.

Adakveo may be administered in an infusion suite, a medical facility designed exclusively for infusions. It can also be given to doctor’s clinics and hospitals. Rarely, a nurse may be able to provide the infusion to you at home.

Adakveo is a member of the class of drugs known as selectin blockers. Crizanlizumab is the active ingredient of Adakveo.

FDA approval of Adakveo

In November 2019, the Food and Drug Administration (FDA) authorized and approved Adakveo. The first targeted therapy for a pain emergency is adakveo. This indicates that it functions differently from other pain relief drugs. Adakveo identifies blood cells and prevents blood clots as opposed to alleviating the pain.

Important Adverse Reactions of Adakveo

Some adverse effects include nausea, joint discomfort, back pain, and fever. Fewer than 10% of patients receiving ADAKVEO noted clinically significant adverse effects such as oropharyngeal pain, infusion site reaction, diarrhea, vomiting, pruritus (including vulvovaginal pruritus), musculoskeletal chest pain, and myalgia.

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