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Sickle Cell Disease: Symptoms, Treatment and Management

Sickle Cell Disease: Symptoms, Treatment & Management

In this Fox 8 House Call series, Cone Health experts discuss sickle cell topics, including:

What is Sickle Cell Disease?

Sickle cell disease is an inherited disorder of the red blood cells. Instead of having normal flexible round red blood cells, those affected have sickle-shaped and rigid cells, which can cause blockages in circulation when they stack together. They also have a shorter lifespan than normal, caused by a shortage of red blood cells, or anemia.

Some of the most common signs of sickle cell are periodic episodes of pain, anemia and jaundice. Individuals with sickle cell disease can have life-threatening complications, and often experience severe pain crises in various parts of the body.

Fortunately, sickle cell can be managed to prevent symptomatic episodes, decrease the occurrence of commonly related health conditions and increase red blood cell production. Long-term management of sickle cell disease involves a combination of proper preventative measures, such as anticipating crises before they happen, immunizations and screenings, as well as disease-specific measures, such as patient education on proper lifestyle modifications and medication.

Sickle cell is inherited and passed down from parent to child. Individuals can either have the sickle cell trait, called carriers, inheriting one normal hemoglobin gene and one gene for sickle hemoglobin, or have sickle cell disease, which occurs when a person inherits two genes for sickle hemoglobin. Individuals with a family history of sickle cell disease and planning to start a family of their own may want to consider being screened to see if they are carriers of the trait. A test known as a hemoglobinopathy panel is used to determine if an individual has sickle cell disease or if they are a carrier.

Olu Jegede, MD, MHA, FAAP, FACP, CPE, is an internal medicine specialist and the care division medical director for Cone Health’s Community Care Services. In this role, he leads the Community Health and Wellness Center, Transitional Care Clinic, Patient Care Center and Renaissance Family Medicine Center.

New Treatments in Sickle Cell

In the past, the main focus of sickle cell disease treatment was symptom management. In recent years, many advancements have been made in sickle cell treatment and new therapies are being researched.

Treatment of the disease can involve the use of a variety of therapies, including:

  • Disease-modifying drugs like Hydroxyurea. This drug helps slow the progression and complications of sickle cell disease, decrease frequency and severity of pain episodes, as well as reduce the patient’s risk of stroke throughout the course of their disease.
  • A new medication called L-Glutamine, which reduces the acute complications of sickle cell disease.
  • Bone marrow transplants.

Current treatment research is aimed at the pathophysiology of the disease, the modulators of inflammation. Gene therapy and stem cell transplants are being studied as ways to cure the disease.

Michelle Matthews, MD, is a board-certified internal medicine specialist at Cone Health Patient Care Center .

Importance of Primary Care in Patients with Sickle Cell

With sickle cell disease, early intervention and long-term management through primary care is key to fighting and/or preventing complications caused by infections in immunocompromised patients. Sickle cell is a complex disease that can affect all parts of the body, which is why successful management plans focus on the patient’s overall health as well as disease-specific treatment.

The team of specialists at the Cone Health Patient Care Center put together a sickle cell care plan for each patient that involves a combination of preventative measures and disease-specific measures, including:

  • Health screenings for diabetes, hypertension, high cholesterol or other chronic conditions.
  • Immunizations.
  • Annual preventive visits for eye exams, dental checkups, Pap smear, etc.
  • Routine health maintenance.
  • Vitamin and medication adjustments.

These treatments are focused on preventing symptomatic episodes, decreasing occurrence of commonly related health conditions and increasing red blood cell production.

Sickle cell disease can negatively affect many of the organs within the body; therefore, it is important for patients to be seen by appropriate specialists on a regular basis. Sickle cell disease can also serve as a significant source of emotional distress, therefore it is also important for patients to receive psychosocial support, such as counseling, to learn healthy ways to cope with this chronic illness.

China Hollis, FNP, is a family nurse practitioner at Cone Health Patient Care Center.

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