Table 1.

Major clinical complications of SCT and proposed counseling recommendations

Complication	Recommendations for counseling
Exertion-related injury	The vast majority of individuals with SCT will never experience an exertion-related injury event
	Even among those involved in high-intensity competitive sports or training, the absolute risk of exertional injury with SCT is low
	Exertional injury in SCT does not seem to occur with usual exercise or low- to moderate-intensity exertion
	Modifiable factors such as activity duration/recovery, hydration, and climate acclimation likely influence the risk of exertional injury in SCT
	Universal precautions, such as those employed by the military,* seem to mitigate the risk of exertional injury in all individuals, irrespective of SCT status
	Screening for SCT, even in high-intensity settings, is not necessary, especially when universal precautions are instituted
CKD	CKD occurs in ∼15%-35% of adults with SCT age >45 y, which is approximately twice the risk of those without SCT
	The risk of progression to ESRD in carriers of SCT is unknown
	Not all SCT carriers are at risk for CKD, and those with coinheritance of α-thalassemia mutations may be protected from developing CKD
	Modifiable factors such as coexisting diabetes may influence the risk of CKD in SCT
	Therapies for the prevention and treatment of CKD in SCT are unknown
	Research is needed to determine the benefit of screening SCT carriers for CKD
RMC	RMC is an exceedingly rare cancer; therefore, the vast majority of individuals with SCT will never develop RMC
	RMC can occur in children or adults, with ∼50% of cases occurring at <21 y of age
	Hemoglobinopathy testing should be performed in any child or young adult with micro- or macroscopic hematuria
	Prompt referral to urology or renal imaging should be performed in any individual with SCT and unexplained hematuria
	Routine screening for RMC among known SCT carriers without concerning symptoms is not recommended given the rarity of the diagnosis
VTE	SCT is a low-risk thrombophilia, similar to factor V Leiden or prothrombin gene mutation
	Screening for SCT in individuals with VTE is not recommended, and its presence should not influence anticoagulation duration
	D-dimer results in SCT carriers should be interpreted with caution and may not be reliable for guiding anticoagulation discontinuation or ruling out VTE
Stroke	SCT does not seem to be a risk factor for ischemic stroke
	SCT should not be assumed to be the etiology of stroke in an SCT carrier, and additional workup for underlying cause should be pursued

ESRD, end-stage renal disease.

^*More information provided in Table 2.