Sickle Cell Complications

Sickle Cell Complications

Mrs. Grisell M Covas RN, BSNPediatric Hematology/Oncology Department

December 29th, 2015.

Objectives

• After this conference the audience should be able to:• Understand in general what is sickle cell• Recognize sickle cell symptoms• Mention the systemic effects of sickle cell

What is Sickle Cell? An overview…

• According to MediLexicon International (2015) is define as: “an abnormal, crescentic erythrocyte that is characteristic of sickle cell anemia, resulting from an inherited abnormality of hemoglobin (hemoglobin S) causing decreased solubility at low oxygen tension”

Early Symptoms of Sickle Cell

• Painful swelling of the hands and feet, known as dactylitis• Fatigue or fussiness from anemia• A yellowish color of the skin, known as jaundice, or whites

of the eyes, known as icteris, that occurs when a large number of red cells hemolyze

• ("National Heart, Lung And Blood Institute", 2015).

Early Symptoms of Sickle Cell

Sickle Cell Complications

• The manifestations of Sickle Cell Disease is as varied and unique as every patient. This manifestations are the results of the complications this condition can carry.

•

Sickle Cell Complications (cont.)Vaso-Occlusive (Acute Pain) CrisisPain is often located on:• Lower back• Legs• Arms• Abdomen• Chest

Chronic Pain• This pain is more difficult to treat and is hard

to describe. This is the result of a complication, like aseptic necrosis.

• This is more common in adults• Some medications used in the treatment of

chronic pain are:• NSAID drugs, such as ibuprofen• Duloxetine• Gabapentin• Amitriptyline• Strong pain medicines, such as opiates

Sickle Cell Complications (cont.)

• Severe Anemia: On infants or children, the possible causes could be:

• Splenic sequestration crisis• Aplastic crisis

Sickle Cell Complications (cont.)• Infections by bacteria:• Pneumococcus• Hemophilus influenza type B• Meningococcus• Salmonella• Staphylococcus• Chlamydia• Mycoplasma pneumoniae

• They can cause:• Blood infection (septicemia)• Lung infection (pneumonia)• Infection of the covering of

the brain and spinal cord (meningitis)

• Bone infection (osteomyelitis)

Sickle Cell Complications (cont.)

• Acute Chest Syndrome symptoms:

• Chest pain• Fever• Shortness of breath• Rapid breathing• Cough

Sickle Cell Complications (cont.)

• Stroke:• Weakness of an arm or leg

on one side of the body• Trouble speaking, walking,

or understanding• Loss of balance• Severe headache

Sickle Cell Complications (cont.)

• Damage to the retina:• This is the most common damage to the eye caused by

sickle cell• Detachment of the retina can lead to visual impairment or

loss

Sickle Cell Complications (cont.)

• Heart Disease• Pulmonary Hypertension:

Related to problems with the blood vessels. Symptoms: Shortness of breath and fatigue.

• Iron Overload: Related to the constant transfusions

• Kidneys Problems:• Nocturnal Enuresis• Blood in the urine• Decreased kidney function• Kidney disease• Protein loss in the urine

Sickle Cell Complications (cont.)

• Gallstones• Symptoms: • Right-sided upper belly

pain• Nausea• Vomiting

• Liver Complications• “Sickle cell intrahepatic cholestasis

is an uncommon, but severe, form of liver damage that occurs when sickled red cells block blood vessels in the liver. This blockage prevents enough oxygen from reaching liver tissue.” ("National Heart, Lung And Blood Institute", 2015).

Sickle Cell Complications (cont.)• Joint Complications:• Avascular or aseptic necrosis: is

the result of a decrease in oxygen to the bone and the damage of this bone. Usually is the hip, but can also affect the shoulder, knees, and ankles ("National Heart, Lung And Blood Institute", 2015).

• Delayed Growth and Puberty

• The development of sickle cell kids should be watch carefully, because their growth and sexual maturity can be delayed because of the anemia.

Sickle Cell Complications (cont.)

• Mental Health:• Dealing with a chronic disease can get a toll on the sickle

cell patient. The limitations on the daily life tasks, trouble coping with the pain, fatigue, medical visits and hospitalizations can produce feelings of sadness and isolation that can lead to depression.

Conclusion

• Sickle Cell is a hereditary chronic condition that can manifest symptoms in a patient as early as 5-6 months.

• This means that the treatment is for life.• This condition can affect practically every system in the

body and put the patient in risk of infections, stroke and other conditions.

• The same way we take care of the body, the sickle cell patient/family require extensive psychological support.

References

• MediLexicom. (2015). MediLexicom International. Retrieved from http://www.medilexicon.com/medicaldictionary.php?t=15875

• National Heart, Lung and Blood Institute. (2015). Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/sca

• National Heart, Lung and Blood Institute. (2015). Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/sca/signs