The Use of Nocturnal Home Hemodialysis in Sickle Cell Disease.

INTRODUCTION

Despite being a rare cause of end-stage kidney disease (ESKD), sickle cell disease has broad kidney implications. Patients with both sickle cell disease and ESKD are at a greater risk of adverse events including mortality; while these patients can be managed with hemodialysis (HD), the use of nocturnal HD in sickle cell disease has not been previously described.

METHODS

Here, we report a patient with sickle cell disease and ESKD who transitioned from conventional HD to nocturnal home HD.

FINDINGS: Following the initiation of extended HD, we observed a significant improvement in her blood pressure (BP) parameters, serum potassium levels, and volume status. We describe the importance of nitric oxide (NO) in maintaining normal endothelial function, and how NO bioavailability is disrupted in both the sickle cell disease and ESKD populations. We speculated that our patient had significant vasoconstriction secondary to NO depletion causing uncontrolled hypertension and intravascular hemolysis (leading to recurrent hyperkalemia), and theorized that extended HD could have enhanced the bioavailability of NO, restoring the vasodilatory capability of her endothelium.

DISCUSSION: Having a dialysis modality that counters the negative pathophysiologic endothelial changes in sickle cell disease and ESKD could result in fewer complications in this vulnerable population.