What is Sickle Cell Anemia?

According to the Centers for Disease Control and Prevention (CDC), SCD is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”. The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such infection, acute chest syndrome and stroke.

What is an Intellectual Disability?

DSM-V defines intellectual disability as a disorder with onset during the developmental period that includes both intellectual functioning including abstract thinking, judgment, academic learning, problem solving.  Adaptive functioning including limitations in activities of daily living, communication, social participation, and independent living across multiple environments such as home, school, work and community. Deficits are on the onset during the developmental period.

According the American Association on Intellectual Disabilities (AAIDD), Intellectual Functioning refers to general mental capacity such as, learning, reasoning and problem-solving.

Types

Although historically, the levels of severity was based on I.Q. scores, this has changed to adaptive functioning which determines the levels of support required.

Mild

• Social Domain- There may be difficulties in regulating emotions and behaviors in an age-appropriate manner. There tends to be a limited understanding of calculated risk, and social judgment.
• Practical Domain- May need assistance in independent living skills such as grocery shopping, transportation, banking and food preparation.

Moderate

• Social Domain: Capacity for relationships is evident in ties to family and friends and may have successful friendships across life and sometimes relationships in adulthood.
• Practical Domain: Can care for personal needs involving eating, dressing and hygiene and as an adult participate in all household task.

Severe

• Social Doman: Spoken language is limited. Speech may be ingle words or phrases. The individual understands simple speech.
• Practical Domain: Requires support for all activities of daily living, including meals, dressing and bathing. The person will require supervision at all times. Unable to make responsible decisions regarding self-care.

Profound

• Social Domain: Has limited understanding of symbolic communication in speech and gestures. The person expresses his or her own desires and emotions through non-verbal communication.
• Practical Domain: The child or adult is dependent on other people for basic needs including self-care and independent living including health and safety.

A sickle cell “crisis” occurs when the red blood cells decrease and the irregular sickle cells block the blood vessels leading to organ damage and pain. A person with an intellectual disability may not be able to communicate they are experiencing a crisis. signs and symptoms to look for include:

• Pain
• Tiredness
• Fatigue
• Leg Ulcers

Ways to support a student with sickle cell includes the following:

1. Offer water throughout the day including frequent small amounts of water
2. allow for accommodations during extreme weather conditions
3. Watch for signs of a stroke
4. Allow the student opportunities to make up homework if missed days from school are due to an illness or crisis.

Resources

Kids Health

Mayo Clinic

Medicine Net

NHLBI (National Heart, Lung, and Blood Institute)

WebMD

Organizations

Children’s Sickle Cell Foundation, Inc.

Foundation for Sickle Cell Research

Sickle Cell Disease Association of America