Treatment FAQ

which of the following agents is used for the maintenance treatment of sickle cell disease

by Dr. Kacey Botsford Published 3 years ago Updated 2 years ago

Medication

Nov 18, 2019 · Although most of the agents that have been discussed to this point involve a preventive approach to sickle cell disease, poloxamer 188, a nonionic block copolymer surfactant, has been shown to improve microvascular blood flow in sickle cell disease by decreasing blood viscosity. 112-115 How it does so is not well understood. However, …

Procedures

Abstract. Sickle cell disease (SCD) complications begin with the polymerization of sickle hemoglobin (HbS). Thus, SCD therapies are focused on preventing HbS production or reducing the circulating amount of HbS. Hydroxyurea treatment has become more widespread, whereas the number of evidence-based indications for erythrocyte transfusion is small.

Therapy

Apr 17, 2022 · The drugs used in treatment of sickle cell disease (SCD) include antimetabolites, analgesics, antibiotics, vaccines, and nutritional agents. Read More.

Self-care

Objectives: To synthesize the published literature on the efficacy, effectiveness, and toxicity of hydroxyurea (HU) when used for treatment of sickle cell disease (SCD); and to review the evidence regarding barriers to its use. Data sources: Articles cited in MEDLlNE, EMBASE, TOXLine, and CINAHL through June 30, 2007. Review methods: Paired reviewers reviewed each title, …

Nutrition

Feb 16, 2021 · 1.1. Rationale. Sickle-cell disease (SCD) is the most common hematologic inherited disorder and has been identified by the World Health Organization (WHO) as a major public health problem (Alaa Al-Anazi et al., 2017).It is caused by an inherited hemoglobin S gene which associated with a substitution of amino acid valine for glutamic acid in position number …

What drugs are used in the treatment of sickle cell disease (SCD)?

Refer to p. 888 of the 2018 RxPrep Course Book. NSAIDs and acetaminophen can be used for mild-moderate sickle cell pain. IV opioids, including PCA, are only needed for severe cases. Constipation is a common side effect of chronic opioid use (see Pain chapter); fluids and laxatives can prevent constipation.

What is the FDA approved drug for sickle cell anemia?

Required vaccines in SCD. Routine childhood series: - Haemophilis influenza type B (Hib) - Pneumococcal conjugate (PCV13, Prevnar) Vaccines for Functional Asplenia. - Meningococcal …

How are red blood cells used to treat sickle cell disease?

Sep 30, 2021 · Goal. To inform and educate hematology/oncology, managed care, and specialty pharmacists on current and emerging Sickle Cell Disease (SCD) therapies, including their mechanisms of action, efficacy, and safety, and improve outcomes by enabling pharmacists to incorporate these agents into clinical practice and ensure timely access to treatment.

What is the role of selectins in sickle cell disease (SCD)?

What is the known treatment for sickle cell disease?

Stem cell transplant. A stem cell transplant is the only known cure for sickle cell anemia. Clinical trials are ongoing to address stem cell transplantation in adults and gene therapies.Mar 9, 2022

Which medication is the drug of choice for treating sickle cell disease?

Morphine is considered the drug of choice for the treatment of acute sickle cell pain (Table 2), whereas meperidine (Demerol, Sanofi-Synthelabo) should be avoided because of the increased risk of seizures in patients with renal dysfunction, which can occur in patients with SCD.

Why is aspirin used as a treatment for sickle cell disease?

We hypothesize that daily, low-dose aspirin therapy will safely diminish the incidence and progression of cognitive deficits as well as the predisposition to overt and silent stroke in children with homozygous sickle cell disease (Hgb SS) or hemoglobin S Beta Zero Thalassemia (Hgb SB-0 Thal).

What is hydroxyurea used for in sickle cell?

Hydroxyurea makes your red blood cells bigger. It helps them stay rounder and more flexible — and makes them less likely to turn into a sickle shape. The medicine does this by increasing a special kind of hemoglobin called hemoglobin F.

What is the best treatment for sickle cell disease?

The National Institutes of Health advises that optimal care for patients with sickle cell disease (SCD), including preventive care, is best achieved through treatment in clinics that specialize in the care of SCD. All patients with SCD should have a principal health care provider, who should either be a hematologist or be in frequent consultation ...

What is the best medicine for sickle cell pain?

Morphine is the drug of choice. The United Kingdom's National Institute for Health and Care Excellence (NICE) guidelines on sickle cell acute painful episodes (published in in 2012 and confirmed in 2014) include the following recommendations [ 46] :

What are the goals of a syringe?

The goals of treatment are symptom control and management of disease complications. Treatment strategies include the following seven goals: 1 Management of vaso-occlusive crisis 2 Management of chronic pain syndromes 3 Management of chronic hemolytic anemia 4 Prevention and treatment of infections 5 Management of the complications and the various organ damage syndromes associated with the disease 6 Prevention of stroke 7 Detection and treatment of pulmonary hypertension

When was Siklos approved?

In December 2017 , the FDA approved Siklos (hydroxyurea) to reduce the frequency of painful crises and the need for blood transfusions in children 2 years of age and older and adolescents with sickle cell anemia who have recurrent moderate to severe painful crises.

What are the stages of pain management?

Pain management should include four stages: assessment, treatment, reassessment, and adjustment. While considering the severity of pain and the patient's past response, follow consistent protocols to relieve the patient's pain. The goals of treatment are symptom control and management of disease complications.

What is the FDA approved drug for SCD?

Crizanlizumab, a P-selectin inhibitor, was approved by the FDA in 2019 to reduce the frequency of vaso-occlusive crisis (VOC) in adults with SCD. Binding P-selectin on the surface of activated endothelium and platelet cells blocks interactions between endothelial cells, platelets, red blood cells (RBCs), and leukocytes.

What is voxelotor used for?

It is indicated for treatment of sickle cell disease in adults and adolescents a ged 12 years or older. Voxelotor is a hemoglobin S (HbS) polymerization inhibitor that binds to HbS with a 1:1 stoichiometry and exhibits preferential partitioning to RBCs.

How to prevent sickle cell?

Drink plenty of water. Dehydration can increase your risk of a sickle cell crisis. Drink water throughout your day, aiming for about eight glasses a day. Increase the amount of water you drink if you exercise or spend time in a hot, dry climate. Avoid temperature extremes.

How does a blood transfusion help sickle cell anemia?

Blood transfusions. In a red blood cell transfusion, red blood cells are removed from a supply of donated blood, then given through a vein to a person with sickle cell anemia. This increases the number of normal red blood cells, which helps reduce symptoms and complications.

Why is sickle cell anemia important?

They're even more important for children with sickle cell anemia because their infections can be severe. Your child's doctor will ensure that your child receives all of the recommended childhood vaccinations, as well vaccines against pneumonia and meningitis and an annual flu shot.

What to do if your child has sickle cell anemia?

If you or your child has sickle cell anemia, your doctor might suggest additional tests to check for possible complications of the disease. If you or your child carries the sickle cell gene, you'll likely be referred to a genetic counselor.

How old do you have to be to take penicillin for sickle cell anemia?

Children with sickle cell anemia might receive penicillin between the ages of about 2 months old until at least age 5. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to children with sickle cell anemia.

How can a doctor learn which children have a higher risk of stroke?

Using a special ultrasound machine, doctors can learn which children have a higher risk of stroke. This painless test, which uses sound waves to measure blood flow, can be used in children as young as 2 years. Regular blood transfusions can decrease stroke risk.

Can you take hydroxyurea while pregnant?

It can also increase your risk of infections. Don't take the drug if you're pregnant.

Diagnosis

Treatment

Lifestyle and Home Remedies

Coping and Support

Your provider will work with you to develop a care plan that may include one or more of these treatment options.
Treatments, such as medications and blood transfusion, aim at managing the symptoms and preventing crises.
Medication

Analgesics: To reduce pain during crises.

Ibuprofen


Antimetabolite: Reduces episodes of pain; it is also said to stimulate production of normal hemoglobin.

Hydroxyurea


Amino acid: Reduces acute complications of the disease.

L-glutamine

Procedures

Bone marrow transplantation: Also known as stem cell transplant, the therapy involves introducing healthy bone marrow stem cells from a donor.

Therapy

Intravenous therapy:Rehydration with intravenous fluids.

Oxygen therapy:Given through a mask to improve breathing.

Self-care

Always talk to your provider before starting anything.

  • Use hot pads to relieve pain.
  • Drink plenty of water.
  • Exercise regularly.
  • Get the child vaccinated as per the doctor’s recommendations.

Nutrition

Foods to eat:

  • Foods rich in folic acid: e.g. green leafy vegetables, legumes, orange juice, and fortified cereals.
  • Foods rich in calcium: e.g. low-fat or fat-free milk, yogurt, and cheese, leafy green vegetables and calcium-fortified Foods such as soymilk, orange juice, and tofu.

Foods to avoid:

  • NA

Specialist to consult

Hematologist
Specializes in the study of the blood and blood disorders.

Preparing For Your Appointment

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