Patient and Family Education

Patient and Family Education

As a parent with a child with sickle cell disease, we encourage you to learn as much as you can about the condition. Your child’s health care providers are here to help you make informed decisions about the treatment and management of your child’s sickle cell. We encourage you to attend appointments and ask questions. For families who require language assistance, we provide in-person and telephonic interpreter services at no cost.

What is Sickle Cell Disease?

Sickle cell disease is the most common inherited blood disorder and is found during a routine newborn blood test. It affects the hemoglobin in red blood cells. Hemoglobin is a protein that carries oxygen to all parts of your child’s body. People with sickle cell disease produce an abnormal type of hemoglobin, for which there are many different types. Normal red blood cells are round and smooth. In a child with sickle cell disease, their red blood cells are hard, sticky, and crescent shaped.

The most common types of sickle cell disease are hemoglobin SS disease (Hb SS), hemoglobin SC disease (Hb SC), and sickle beta thalassemia disease (HbS beta-thalassemia).

Sickle cell disease affect families from a variety of ethnic backgrounds, but primarily affects those of African descent, Hispanics, and individuals of Caribbean ancestry. It can also affect people of Middle Eastern and Indian descent. It is rarely found in those of European ancestry.

Sickle Cell Symptoms

The type of sickle cell that your child has will determine the symptoms they can experience including disease-related complications. Most children will start having symptoms around 6 months of age. Symptoms may be mild or severe and can include the following:

Anemia caused by a lack of healthy red blood cells and is the most common symptom associated with sickle cell disease.

Jaundice yellowing of the skin, eyes, and mouth caused by bilirubin which is released when red blood cells die. Sickled cells do not live as long as healthy red blood cells.

Pain when sickled cells move through small blood vessels through out the body, they get stuck and stop blood flow. This is what causes your child to experience pain. Sudden pain can happen anywhere but is most commonly reported in the arms, legs, and chest. Babies may experience painful swelling in their hands and feet, also known as dactylitis.

Splenic Sequestration the spleen helps filter bad bacteria out of the blood. These bacteria are what cause infections. When sickled cells get stuck in the spleen, the spleen becomes enlarged and can cause your child pain. This can also cause a sudden drop in your child’s hemoglobin which requires immediate treatment.

Living Well with Sickle Cell

Advances in preventive care and new medications have reduced the rate of complications from sickle cell disease, but it is still a severe, chronic illness. You cannot completely prevent your child from developing complications, but helping your child live a healthy lifestyle can reduce the risk of some problems.

Make sure your child:

• Has regular eye exams and stroke screening tests
• Eats a healthy diet
• Gets enough sleep
• Drinks plenty of fluids

Avoid things that may trigger a sickle cell crisis for your child, including:

• High altitudes
• Extremely cold/hot weather
• Cold water swimming

Help your child avoid infections by:

• Staying away from people who are sick
• Washing hands often
• Getting all recommended vaccinations and screenings