Welcome to PICU Doc on Call, the podcast where we break down real-life cases from the pediatric intensive care unit and share practical insights for clinicians everywhere! I’m Dr. Monica Gray, and I’m joined by my co-host, Dr. Pradip Kamat.

Today, we’re diving into a fascinating case: a 13-year-old boy who suddenly developed muscle weakness and was found to have severe hypokalemia. After some detective work, he was diagnosed with familial hypokalemic periodic paralysis, a rare but important condition to recognize in the PICU.

We’ll discuss the genetic underpinnings, classic clinical features, and common triggers associated with this disorder. Additionally, we’ll guide you through the differential diagnosis, key management strategies, such as potassium supplementation, and explain why genetic testing is so crucial. We’ll also cover essential considerations for anesthesia and cardiac monitoring in these patients.

Whether you’re a pediatric intensivist or just interested in acute neuromuscular care, stick around for some practical pearls you can use on your next shift!

Show Highlights:

• Clinical case discussion of a 13-year-old male patient with muscle weakness and hypokalemia
• Diagnosis and management of familial hypokalemic periodic paralysis
• Genetic basis and mutations associated with hypokalemic periodic paralysis (CACNA1S and SCN4A)
• Physiological mechanisms underlying hypokalemic periodic paralysis
• Common clinical presentations and triggers for episodes of muscle weakness
• Differential diagnoses for muscle weakness and hypokalemia in pediatric patients
• Laboratory investigations to confirm hypokalemic periodic paralysis
• Treatment options for hypokalemic periodic paralysis, including potassium supplementation and prophylactic medications
• Importance of avoiding triggers and coordinating care with anesthesia

References:

• Fuhrman & Zimmerman - Textbook of Pediatric Critical Care Chapter 68: Weimer M et al. Acute neuromuscular disease and disorders page 840
• Rogers Textbook of Pediatric Intensive Care Medicine: Management of Sodium and Potassium Disorders. Pages 1876- 1883
• Reference 1: Weber F, Lehmann-Horn F. Hypokalemic Periodic Paralysis. 2002 Apr 30 [Updated 2018 Jul 26]. In: Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2025. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1338/
• Reference 2: Channelopathies. Clin Exp Pediatr. 2014;57(1):1-18. Published online January 31, 2014**DOI: https://doi.org/10.3345/kjp.2014.57.1.1**
• Reference 3: Statland JM, Fontaine B, Hanna MG, Johnson NE, Kissel JT, Sansone VA, Shieh PB, Tawil RN, Trivedi J, Cannon SC, Griggs RC. Review of the Diagnosis and Treatment of Periodic Paralysis. Muscle Nerve. 2018 Apr;57(4):522-530.

Learn how to sharpen your pediatric intubation skills and make evidence-based decisions at the bedside. Today, Dr. Pradip Kamat, Dr. Monica Gray, and Dr. Rahul Damania expertly dissect the nuances of selecting optimal induction agents for critically ill children in the PICU. Through engaging, real-world case scenarios, our hosts guide you through drug choices in complex situations such as cardiogenic shock, septic shock, and elevated intracranial pressure—always prioritizing hemodynamic stability and patient safety. Gain valuable insights into the advantages, limitations, and clinical pearls of agents like propofol, fentanyl, ketamine, and midazolam, along with practical strategies for rapid sequence intubation, neuromuscular blockade, and individualized patient care. Don’t miss this high-yield discussion, packed with actionable knowledge!

Show Highlights:

• Induction agents for endotracheal intubation in critically ill children
• Clinical scenarios highlighting optimal choices of induction agents and neuromuscular blockers
• Importance of maintaining hemodynamic stability during intubation
• Pharmacology and clinical considerations of various induction agents (e.g., propofol, ketamine, fentanyl, etomidate)
• Use of neuromuscular blocking agents (NMBAs) in pediatric intubation
• Differences between depolarizing and non-depolarizing neuromuscular blockers
• Risks associated with specific induction agents in patients with cardiac dysfunction or septic shock
• Modified rapid sequence intubation (RSI) techniques for unstable patients
• Key takeaways for managing critically ill pediatric patients requiring intubation
• Practical tips for optimizing intubation conditions and minimizing complications

References:

1. Fuhrman & Zimmerman - Textbook of Pediatric Critical Care 6th Edition. Chapters 127 - 135, Pages 1510 - 1610
2. Hendrix JM, Regunath H. Intubation Endotracheal Tube Medications. [Updated 2025 Jan 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459276/
3. Agrawal, Dewesh. Rapid sequence intubation (RSI) in children for emergency medicine: Medications for sedation and paralysis. UpToDate. Last updated Dec 4, 2024.
4. Vanlinthout LE, Geniets B, Driessen JJ, Saldien V, Lapré R, Berghmans J, Uwimpuhwe G, Hens N. Neuromuscular-blocking agents for tracheal intubation in pediatric patients (0-12 years): A systematic review and meta-analysis. Paediatr Anaesth. 2020 Apr;30(4):401-414. doi: 10.1111/pan.13806. Epub 2020 Mar 9. PMID: 31887248.
5. Tarquinio KM, Howell JD, Montgomery V, Turner DA, Hsing DD, Parker MM, Brown CA 3rd, Walls RM, Nadkarni VM, Nishisaki A; National Emergency Airway Registry for Children; Pediatric Acute Lung Injury and Sepsis Investigators Network. Current medication practice and tracheal intubation safety outcomes from a prospective multicenter observational cohort study. Pediatr Crit Care Med. 2015 Mar;16(3):210-8. doi: 10.1097/PCC.0000000000000319. PMID: 25581629.
6. Conway JA, Kharayat P, Sanders RC Jr, Nett S, Weiss SL, Edwards LR, Breuer R, Kirby A, Krawiec C, Page-Goertz C, Polikoff L, Turner DA, Shults J, Giuliano JS Jr, Orioles A, Balkandier S, Emeriaud G, Rehder KJ, Kian Boon JL, Shenoi A, Vanderford P, Nuthall G, Lee A, Zeqo J, Parsons SJ, Furlong-Dillard J, Meyer K, Harwayne-Gidansky I, Jung P, Adu-Darko M, Bysani GK, McCarthy MA, Shlomovich M, Toedt-Pingel I, Branca A, Esperanza MC, Al-Subu AM, Pinto M, Tallent S, Shetty R, Thyagarajan S, Ikeyama T, Tarquinio KM, Skippen P, Kasagi M, Howell JD, Nadkarni VM, Nishisaki A; National Emergency Airway Registry for Children (NEAR4KIDS) and for the Pediatric Acute Lung Injury and Sepsis Investigators...

Ready for a deep dive into a real-life pediatric ICU situation? Today, Dr. Pradip Kamat, Dr. Monica Gray, and Dr. Rahul Damania will walk you through the case of a seven-year-old girl with Hemoglobin SC (HbSC) disease, who presents with abdominal swelling, pneumonia, low oxygen, and pain.

In this episode, our team unpacks the spleen’s anatomy and its crucial role in immunity, then zooms in on how sickle cell disease can throw a wrench in splenic function. You’ll hear how they approach the diagnosis and management of acute splenic sequestration crisis, sharing clinical pearls along the way. Plus, they’ll break down why quick recognition is so important and discuss strategies for both immediate and long-term care in pediatric sickle cell patients. Don’t miss these practical insights from the frontlines of pediatric critical care!

Show Highlights:

• Case study of a seven-year-old girl with hemoglobin SC disease
• Presentation of symptoms: abdominal distension, pneumonia, hypoxia, and body pain
• Discussion of acute splenic sequestration crisis as a complication of sickle cell disease
• Anatomy and physiology of the spleen
• The role of the spleen in sickle cell disease and how sickled cells affect splenic function
• Acute splenic sequestration crisis, including clinical features and laboratory evaluations
• Management strategies for acute splenic sequestration crisis in the ICU
• Importance of blood transfusions and supportive care in treatment
• Prophylactic measures to prevent recurrence of splenic sequestration
• Educational emphasis on recognizing clinical signs and the need for timely intervention

References:

• Fuhrman & Zimmerman - Textbook of Pediatric Critical Care Chapter 88. Hemoglobinopathies. Baender, MA, Marsh Anne. Pages: 1457-1470
• Rogers' textbook of pediatric intensive care: Hematologic Emergencies. McCory MC, Bhar S, and Blaine E. Pages 2003-2005
• Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014 Jul;166(2):165-76. doi: 10.1111/bjh 12950. Epub 2014 May 26. PMID: 24862308.
• Waleed S, Aldabsa M, Gouher S. Splenic Sequestration Induced by Parvovirus B19: A Case Report. Cureus. 2024 May 23;16(5):e60937. doi: 10.7759/cureus. 60937. PMID: 38915956; PMCID: PMC11195323.
• Solanki DL, Kletter GG, Castro O. Acute splenic sequestration crises in adults with sickle cell disease. Am J Med. 1986 May;80(5):985-90. doi: 10.1016/0002-9343(86)90649-2. PMID: 3706382.
• Karna B, Jha SK, Al Zaabi E. Hemoglobin C Disease. [Updated 2023 May 29]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559043/