Sickle cell is a hereditary blood disorder where red blood cells take on an abnormal crescent shape. This causes complications throughout the body including painful vaso-occlusive crises, acute chest syndrome, and severe anemia. Long term complications involve damage to organs like the lungs, liver, kidneys, eyes, heart and bones. Treatment focuses on managing pain and infections through medications and transfusions, but the condition requires life-long management as it can impact growth, mental health and nearly every system.
2. 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
3. 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”
4. 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).
6. 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.
•
7. Sickle Cell Complications (cont.)
Vaso-Occlusive (Acute Pain)
Crisis
Pain 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
8. Sickle Cell Complications (cont.)
• Severe Anemia: On infants or children, the possible causes could be:
• Splenic sequestration crisis
• Aplastic crisis
9. 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)
11. 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
12. 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
13. 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
14. 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).
15. 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.
16. 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.
17. 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.
18. 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
Hinweis der Redaktion
There are 6 types of Sickle Cell Disease: Hemoglobin SS:
Hemoglobin SC
Hemoglobin Sβ0 thalassemia
Hemoglobin Sβ+ thalassemia
Hemoglobin SD
Hemoglobin SE
But the most common are: Hgb SS, Hgb SC, and Hgb Beta Thalassemia.
Patients can start developing symptoms as early as 5-6 months of age ( as we have seen in our practice).
We are very familiar with some of this complications, others are not so common but we should be aware of what to expect for the benefit of our patients and families.
The pain episodes are the results of the sickle cells blocking the blood flow and the decreased of the oxygen transported throw out the body. We have heard the description of a sharp, intense, stabbing, or throbbing pain. Possible triggers for this pain are: Illness
Temperature changes
Stress
Dehydration (not drinking enough)
Being at high altitudes
We have to be aware of this because, we tend to overview this on our admission assessment.
“A splenic sequestration crisis occurs when red blood cells get stuck in the spleen, making it enlarge quickly. Since the red blood cells are trapped in the spleen, there are fewer cells to circulate in the blood. “ ("National Heart, Lung And Blood Institute", 2015). This Aplastic Crisis “ is usually caused by a parvovirus B19 infection, also called fifth disease or slapped cheek syndrome. Parvovirus B19 is a very common infection, but in SCD it can cause the bone marrow to stop producing new red cells for a while, leading to severe anemia.” ("National Heart, Lung And Blood Institute", 2015).
Because of the damage that sickle cell can cause on the spleen and the importance of this organ in the immunologic response of the body, our sicklers are more susceptive to infections. We need to be very active in our education toward parents about the importance of having this kids updated on their inmunizations.
When the sickle cells deprive the oxygen of the lungs and damage a part of it, we face Acute Chest Syndrome. Generally starts after a pain crisis and is accompany by an infection. We need to educate our parents to recognize the early symptoms' so they can be able to seek medical help immediately.
In children with sickle cell, is more common between 2-9 y/o. Also studies have showed that adults and children with Hgb SS and Beta Thalassemia of silent brain injury or silent stroke ( damage to the brain without stroke symptoms). ("National Heart, Lung And Blood Institute", 2015).
Red cells when hemolyze produce hemoglobin that breaks down into bilirubin. This substance is what create the stones in the gallbladder. The liver can be affected by the Iron Overload.
The patient with avascular necrosis may be needing pain medications, surgery or joint replacement if symptoms continue.
As healthcare professionals we should include mental health screenings on our assessments in order to discover on time symptoms of depression or other mentalhealth problems that can affect the compliance with the treatment needed for controlling sickle cell crisis.
