Sickle cell disease complications: Understanding the prevention and management.

Question 1

What are the acute complications of sickle cell disease in children?

Answer 1

Infections, severe anemia and vaso-occlusive problems causing pain.

Question 2

What are the most common chronic complications and sickle cell disease?

Answer 2

Pain and anemia

Question 3

Why are children with sickle cell disease at higher risk of infection?

Answer 3

Secondary to functional hyposplenism and asplenism.

Question 4

What are other reasons in children with sickle cell disease causing increased infections?

Answer 4

Altered cellular and humoral immunity
Hypoventilation
Reduced tissue perfusion
Splinting
Presence of indwelling catheters

Question 5

What are the most common infectious organisms in children with sickle cell disease?

Answer 5

Malaria is the most common morbidity and mortality worldwide
Encapsulated organisms such as strep pneumoniae and haemophilus influenza
Other causes include E. coli, Staph aureus, and Salmonella species
Mycoplasma and Chlamydia pneumoniae cause pneumonia and acute chest syndrome

Question 6

What are some preventative measures that should be taken in patients with sickle cell disease?

Answer 6

Prompt evaluation in any child develops fever or other signs of infection.
Information about prophylactic penicillin and vaccines against encapsulated organisms.

Question 7

Because sickled RBCs undergo hemolysis after about 17 days what type of anemia do patients with sickle cell disease have?

Answer 7

They have a chronic compensated hemolytic anemia, with baseline hemoglobin levels between 8 to 10 g/dL and hematocrits between 20 to 30%. Reticulocyte’s are elevated at 3 to 15% range.

Question 8

What classic finding on peripheral blood smears occur in individuals with sickle cell disease?

Answer 8

Sickled RBCs and Howell-Jolly bodies

Question 9

What is the management for patient with chronic compensated hemolytic anemia?

Answer 9

Folic acid supplementation and regular CBC monitoring.

Question 10

What occurs in an aplastic crisis in a individual with sickle cell disease?

Answer 10

Erythropoiesis transiently stops which abruptly decreases RBC precursors with resulting drop in patient’s reticulocyte count and hemoglobin levels.

Question 11

In a patient with sickle cell disease what is the most common infectious causes of an aplastic crisis?

Answer 11

Parvovirus B19.

Question 12

What is the management of the patient with aplastic crisis?

Answer 12

A blood transfusion.

Question 13

What is a splenic sequestration crisis in a patient with sickle cell disease?

Answer 13

When the patient’s RBCs are pooled into the spleen, presenting with an enlarged spleen and decreased hemoglobin level which can lead to hypovolemic shock and death.

Question 14

If a patient survives from splenic sequestration crisis what is the treatment?

Answer 14

Splenectomy

Question 15

What are some of the common triggers for occlusive pain in patients with sickle cell disease?

Answer 15

Cold temperature, low humidity, wind, stress, menses, dehydration.

Question 16

What are some of the common locations for vaso-occlusive pain crises in patients with sickle cell disease?

Answer 16

Chest, back, abdomen or extremities.

Question 17

How are the vaso-occlusive pain crises best managed in patients with sickle cell disease?

Answer 17

Pain assessment is best done with patient’s own report of pain levels.
Management for pain is an individualized pain protocol that can allow for pain to be managed at home. If not managed at home with oral opioids then they need to go to the hospital.

Question 18

What medication is used to prevent vaso-occlusive pain episodes in patients with sickle cell disease?

Answer 18

Hydroxyurea is used to prevent vaso-occlusive pain episodes and may be given at home.

Question 19

What major complication can occur with an acute pain episode that would require immediate exchange transfusion therapy in a patient with sickle cell disease?

Answer 19

Multiorgan failure.

Question 20

With CNS complication can occur in individuals with sickle cell disease at a higher rate than those without?

Answer 20

Stroke with ischemic strokes being more common in children over hemorrhagic strokes.
They are also at high risk of seizures

Question 21

In patients with sickle cell disease what can be done to help with stroke prevention?

Answer 21

A transe cranial Doppler can be used for risk assessment and then MRA can be used for selective screening.
These individuals can be managed with chronic prophylactic transfusions

Question 22

How is acute chest syndrome diagnosed in the patient with sickle cell disease?

Answer 22

Any new pulmonary infiltrates in the setting of
Fever
Chest pain
Cough
Wheezing
Respiratory distress
Hypoxia

Question 23

What management should be done for patient with acute chest syndrome who has sickle cell disease?

Answer 23

Analgesia, oxygen, transfusion, antibiotics, bronchodilators, and incentive spirometry

Question 24

To reduce risk of an acute chest syndrome what prevention measures are considered in the patient with sickle cell disease?

Answer 24

Prophylactic antibiotics, vaccines, hydroxyurea, transfusions if hydroxyurea fails, hematopoietic cell transplantation and asthma exacerbation prevention which increases the risk of ACS.

Question 25

In children with sickle cell disease what baseline lung study and at what age(s) should that study be done?

Answer 25

A transthoracic Doppler echocardiogram should be done to determine baseline for all children at least 8 years of age then continue routine screening for pulmonary hypertension every 1 to 3 years

Question 26

How is pulmonary hypertension diagnosed?

Answer 26

A high pulmonary arterial pressure

Question 27

What are some of the symptoms of pulmonary hypertension and child with sickle cell disease?

Answer 27

Chest pain, reduced exercise tolerance, chronic dyspnea, or presyncope.

Question 28

For renal insufficiencies or complications in children with sickle cell disease when should baseline studies be obtained and what should they include?

Answer 28

Screening should be obtained and patient 3 to 5 years of age including baseline chemistry and urinalysis looking for protein and albumin.

Question 29

To help prevent renal complications in a patient with sickle cell disease what medications should be avoided and why?

Answer 29

Nephrotoxic medications including NSAIDs, treat hypertension with medications that are not diuretics to avoid dehydration which can trigger vaso-occlusive episodes and then maintain good hydration during hospitalizations especially if contrast studies are done.

Question 30

What is dactylitis in a patient with sickle cell disease?

Answer 30

A vaso-occlusive event of the hands and or feet.

Question 31

What is the typical age for dactylitis in patient with sickle cell disease?

Answer 31

Infants and children up to 4 years of age

Question 32

How is dactylitis managed in a patient with sickle cell disease?

Answer 32

Hydration, warm packs, analgesics and consider hydroxyurea initiation.

Question 33

Why should patients with sickle cell disease have vitamin D levels checked annually?

Answer 33

They are at increased risk for vitamin D deficiency as well as osteoporosis due to increased erythropoietic activity.

Question 34

What medications should patients with sickle cell disease take to help prevent osteoporosis?

Answer 34

Vitamin D and calcium supplements.

Question 35

How often should bone density scans be done in patients with sickle cell disease

Answer 35

Bone density scan should be done every 1 to 3 years starting at 12 years of age.

Question 36

For patients with sickle cell disease what is the most common pathogen causing osteomyelitis?

Answer 36

Salmonella species

Question 37

What is the most common cause of osteomyelitis in a patient without sickle cell disease?

Answer 37

Staph aureus

Question 38

What study should be done to help differentiate osteomyelitis versus a vaso-occlusive pain crisis in a patient with sickle cell disease?

Answer 38

Bone imaging and cultures

Question 39

What are the 3 common skeletal complications in an individual with sickle cell disease?

Answer 39

Dactylitis, osteoporosis, osteomyelitis

Question 40

What other organ systems can can have complications in patients with sickle cell disease?

Answer 40

Retinopathy
Priapism

Question 41

In children with sickle cell disease how often should they have their eyes exam done and what age to rule out retinopathy from ischemia of the retinal artery?

Answer 41

This should have annual ophthalmologic exam starting at 10 years of age.

Question 42

When should patients with sickle cell disease be given anticipatory guidance regarding priapism?

Answer 42

Before the patient is 2 years of age.

Question 43

How long does a episode of priapism last in patients with sickle cell disease and what should be done for a major episode?

Answer 43

Typically an episode last greater than 30 minutes
for major episodes lasting greater than or equal to 4 hours they should be taken to the emergency room because there can be ischemia leading to permanent damage of the penis.

Question 44

How does sickle cell disease affect growth and development in children?

Answer 44

They commonly have delayed puberty, delayed menarche and impaired growth.

Question 45

If a patient with sickle cell disease has decreased growth trajectory what evaluations should be done?

Answer 45

Nutritional and environmental history and an endocrinology evaluation.

Question 46

What are some of the psychological and psych social issues in patients with sickle cell disease?

Answer 46

Because of the chronic disease, chronic pain, functional impairment and mental health issues they can have anxiety and depression. As a result patient should be given support, healthy coping strategies and resources for mental health help.