Red blood cells are round in shape, and they supply oxygenated blood throughout the body.

Sickle Cell Disease/Anaemia has red blood cells in the shape of a sickle.

They are deformed  red blood cells with decreased oxygen. Sickling of the red blood cells causes thickening of the blood,  and decreases oxygen flow. Small blood vessels in different organs, bones and joints become occluded putting the individual in a painful crisis.

See further information at http://nhlbi.nih.gov/health-topics/sickle-cell-disease.

General Symptoms in Infants.

In infants, symptoms do not usually appear until late in the baby's first year. Most commonly, they include:

• Fever
• Swelling of the hands and feet
• Pain in the chest, abdomen, limbs, and joints ( Pain is as intense as cancer pain)
• Nosebleeds and frequent upper respiratory infections

General Symptoms in Children. Pain is the most common complaint. It can be acute and severe or chronic, usually from orthopaedic problems in the legs and lower back. Other symptoms include:

• Fatigue and shortness of breath (signs of anaemia)
• Irritability
• Jaundice (yellowish discoloration of the skin and eyes)

Additional Symptoms in Adolescence or Adulthood. Symptoms from childhood continue in adolescence and adulthood. In addition, patients may have:

• Delayed puberty (in young teenagers)
• Severe joint pain
• Progressive anemia
• Leg sores
• Gum disease
• Vision problem

  Complications of Sickle Cell Disease

  1. Stroke: A stroke can occur if sickle cells block blood flow to an area of your brain. Signs of a stroke include seizures, weakness or numbness of your arms and legs, sudden speech difficulties, and loss of consciousness. If your baby or child has any of these signs and symptoms, seek medical treatment immediately. A stroke can be fatal.
  2. Acute Chest Syndrome: This life-threatening complication of Sickle Cell Anemia causes chest pain, fever and difficulty breathing. Acute chest syndrome can be caused by a lung infection or by Sickle Cells blocking blood vessels in your lungs. It may require emergency medical treatment with antibiotics and other treatments.
  3. Pulmonary hypertension: People with Sickle Cell Anemia can also develop high blood pressure in their lungs (pulmonary hypertension). Shortness of breath and difficulty breathing are common symptoms of this condition, which can be fatal.
  4. Organ damage:  Sickle Cells can block blood flow through blood vessels, immediately depriving an organ of blood and oxygen. In Sickle Cell Anemia, blood is also chronically low on oxygen. Chronic deprivation of oxygen-rich blood can damage nerves and organs in your body, including your kidneys, liver and spleen. Organ damage can be fatal.
  5. Blindness: Tiny blood vessels that supply your eyes can get blocked by Sickle Cells. Over time, this can damage the retina — the portion of the eye that processes visual images — and lead to blindness.
  6. Skin ulcers: Sickle Cell Anemia can cause open sores, called ulcers, on your legs.
  7. Gallstones: The breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in your body can lead to gallstones.
  8. Priapism: Men with Sickle Cell Anemia may experience painful, long-lasting erections, a condition called priapism. As occurs in other parts of the body, Sickle Cells can block the blood vessels in the penis. This can damage the penis and eventually lead to impotence.

It is very painful for families to watch their family member endure these long periods of painful crisis that can last up to (14) days. Ask your doctor for recommendations to a therapist you can and your family can talk with to maintain emotional stability.
Fluids: Take lots of water and juices. Extra fluids reduce the risk of dehydration and reduce the possibility of a crisis.
Clothing: Wear fleece, flannel shirts and jackets in winter with thick wool socks. This keeps the body warm and reduces the workload of the heart. Extreme in temperatures requires more oxygen to keep the body warm. Use flannel and wool linen for beddings and sleepwear.
Skin Care: Lotion the body daily with natural lotions such as those made from Shea butter of Cocoa butter. Use natural oils such as olive oil to keep skin moist. Dry skin cracks easily and can become infected. The infection leads to a painful crisis.
Reduce Stress: Exercise should be done as tolerated. Talk about how you feel about your illness. Talk to a therapist about your illness, depression sets in easily for anyone with a chronic illness. Join yoga classes, meditation or metaphysical classes to learn techniques of using the mind to control the physical body.
Medications: Take the prescription recommended by your MD. Consult with your doctor about over the counter medications, some can cause constriction that will set off a painful crisis.
Students in Elementary to High School diagnosed with sickle cell disease should be under the 504 Program.

What is the 504 program:

The determination of whether a student has a physical or mental impairment that substantially limits a major life activity must be made on the basis of an individual inquiry. The Section 504 regulatory provision at 34 C.F.R. 104.3(j)(2)(i) defines a physical or mental impairment as any physiological disorder or condition, cosmetic disfigurement, or anatomical loss affecting one or more of the following body systems: neurological; musculoskeletal; special sense organs; respiratory, including speech organs; cardiovascular; reproductive; digestive; genito-urinary; and lymphatic; skin; and endocrine; or any mental or psychological disorder, such as mental retardation, organic brain syndrome, emotional or mental illness, and specific learning disabilities. The regulatory provision does not set forth an exhaustive list of specific diseases and conditions that may constitute physical or mental impairments because of the difficulty of ensuring the comprehensiveness of such a list.
 Source: http://www2.ed.gov/about/offices/list/ocr/504faq.html

Children with Sickle Cell Disease are qualified for the 504 programs:

At the elementary and secondary educational level, a "qualified student with a disability" is a student with a disability who is: of an age at which students without disabilities are provided elementary and secondary educational services; of an age at which it is mandatory under state law to provide elementary and secondary educational services to students with disabilities; or a student to whom a state is required to provide a free appropriate public education under the Individuals with Disabilities Education Act (IDEA).
 Source: http://www2.ed.gov/about/offices/list/ocr/504faq.html

Typical Provisions your child should have in school under the 504 Program

• Allow the student to have fluids throughout the day.
• Allow for unlimited trips to the bathroom.
• Allowance for self-limit in involvement in all physical activities including physical education without penalty.
• Provide the student with door to door transportation to and from school.
• Avoid exposure to extreme temperatures.
• Allowing the student to wear extra clothing within the school building as needed.
• Provide the student with a second set of textbook.
• Assisting the student in making up missed work during absences.