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• How would you evaluate and manage a pediatric patient who has a painful swelling of the hands and feet, fatigue, or fussiness? Which diagnostic studies would you recommend for this patient and why?
• What physical exam findings and diagnostic results would be concerning to you and why? What would be three differentials in this case?
• In SCD (SPLENIC CELL DISEASE), the spleen doesn’t work properly or doesn’t work at all. This problem makes people with SCD more likely to get severe infections. What is the treatment for Mia and education for the family?

SOME HELPFUL INFORMATION:

To evaluate this patient, I would perform a few tasks as below:

1. Observation

Standing and sitting (Pelvis heights, lower limb lengths and alignment, hind foot position , forefoot position, foot arch, toes (claw toes, overriding fifth toe, Morton’s foot with first ray shorter than the second), swelling, and redness.)

2. Study the range of motion

The resting position of the foot and ankle is slight ankle plantarflexion (10°), subtalar neutral.

Passive movements and angular inclinations between the ankle and the knee will show the extent of the effects.

3. Special tests

1) Tests for rigid or flexible flat feet (Observe patient’s medial longitudinal arch standing, standing on his/her toes, and sitting. Presence of an arch while on one’s toes or sitting and absent with standing suggests flexible flat feet. Absence of an arch in all positions suggests rigid flat feet.)

2) Thigh foot angle (Measure of tibial torsion. Patient prone with knee flexed to 90° and foot in relaxed position; measure angle between the axis of the thigh and foot. The thigh foot angle rotates laterally with increasing age; the upper limit of normal is 30°. Negative values are common in infants. Negative and positive values beyond the normal range are referred to as medial (internal) tibial torsion and lateral (external) tibial torsion respectively.)

3) Forefoot adduction correction test [Patient supine or prone. The lateral border of the foot is usually straight. If forefoot adduction is correctable to neutral or beyond with abduction it is “correctable” and if “fixed” it likely requires orthopedic intervention and casting.]

4. Flexibility

The ankle, subtalar, forefoot and joints should be moved through active range and then placed through full passive ranges of motion. Muscles that span two joints are important for functional range of motion and should be tested independently.

5. Spine, hip and knee joint exam

Check for the range of motion.

6. Neurovascular exam

Sensation of the lower legs and hands can be examined too.

6. Spleen test (splenomegaly)

I may confirm the diagnosis of an dysfunctional/enlarged spleen with one or more of these tests:

• Blood tests, such as a complete blood count to check the number of red blood cells, white blood cells and platelets in your system
• Ultrasound or computerized tomography (CT) scan to help determine the size of your spleen and whether it’s crowding other organs
• Magnetic resonance imagining (MRI) to trace blood flow through the spleen

7. Gait

Normal heel toe gait requires ankle dorsiflexion of at least 10°, subtalar pronation and supination of 5° and 1st joint dorsiflexion of 40°. In normal walking, 60% of time is spent in the stance phase (25% double stance) and 40% in the swing phase. The stance phase includes heel strike, foot flat, heel rise and toe off. Altered gait may be due to truncal or lower limb misalignment, lower extremity muscle weakness, joint instability, limited joint range of motion, and/or pain.

I would recommend the tests above for the patient to evaluate the symptoms and then decide how to manage them.

Physical exam findings and diagnostic results that would concern me:

• Fever
• Lethargy
• Abdominal pain
• Irritation in the limbs
• Joint pains

In Miah’s case, the spleen my not be functional in the body and therefore can be a liability as it would risk causing infections. A procedure called laparoscopy or another called splenectomy can be performed on Miah. This would remove the spleen, and then the cleansing functions of the spleen are taken up by other organs like the liver. Miah’s family can be informed about the little risk this poses when removed compared to the bigger risk when left in the body. If the spleen is removed, she cannot effectively clear certain bacteria from the body and so vaccines or other medications are needed to prevent infection. This is better than leaving the non-functional spleen which would cause effects like fatigue, frequent infections, weight loss, jaundice and even anemia.