What’s New in Pulmonary Care for Children With Sickle Cell Disease?
What’s New in Pulmonary Care for Children With Sickle Cell Disease? https://pediatricsnationwide.org/wp-content/uploads/2021/02/AdobeStock_126797930_lung-header-1024x575.gif 1024 575 Erin Gregory https://secure.gravatar.com/avatar/?s=96&d=mm&r=g
A recent publication reviews the impact of pulmonary complications on children and youth with sickle cell disease (SCD) and offers suggestions to improve outcomes.
Pulmonary complications are a leading cause of morbidity and mortality in children with sickle cell disease (SCD), yet they remain underrecognized and undertreated. A recent review published in Clinics in Chest Medicine highlights the impact of conditions such as acute chest syndrome (ACS), lower airway disease (asthma), sleep-disordered breathing (SDB) and pulmonary hypertension (PH). The publication addresses critical gaps in research and clinical practice, emphasizing the need for early screening, multidisciplinary care models and targeted interventions to improve outcomes.
Michelle Gillespie, MD, pulmonary medicine expert at Nationwide Children’s Hospital is the lead author of the review.
Key Pulmonary Complications in Pediatric Sickle Cell Disease
Major pulmonary conditions affect children with SCD and require evidence-based strategies for early detection and management.
| Condition | Impact | Recommendation |
| Acute Chest Syndrome (ACS) | The second most common cause of hospitalization in pediatric SCD, ACS results from vaso-occlusion in the lungs, often triggered by infection, fat embolism or hypoxemia. Treatment includes oxygen therapy, antibiotics and transfusions when necessary.
|
Non-invasive ventilation is a critical potential therapy to prevent acute chest syndrome in hospitalized patients with SCD. |
| Asthma and Lower Airway Disease | Children with SCD have higher asthma rates, increasing their risk for vaso-occlusive episodes (VOE) and ACS.
|
Clearer diagnostic criteria and proactive asthma management to reduce complications. |
| Sleep-Disordered Breathing (SDB) | Conditions such as obstructive sleep apnea (OSA) and nocturnal hypoxemia worsen cardiopulmonary function and increase hospitalizations. | Standardized screening protocols to improve diagnosis and treatment.
|
| Pulmonary Hypertension (PH) | A leading cause of mortality in SCD, PH is often underdiagnosed in children. | Routine echocardiogram screenings by age 8 and highlights hydroxyurea and transfusion therapy as key treatments.
|
Closing Gaps in Pulmonary Care
“Pulmonary care in pediatric SCD is often fragmented, leading to delays in diagnosis and treatment,” says Dr. Gillespie. “By offering a combined clinic, we can ensure that all patients receive appropriate screening.”
For example, even though there is no recommendation for routine pulmonary function testing (PFTs) in these patients, many physicians who care for these patients including Dr. Gillespie recommend screening PFTs every 2-3 years for asymptomatic children and at every visit for symptomatic patients to improve early detection of lung disease. The combined clinic model helps support the implementation of this testing recommendation.
In Nationwide Children’s multidisciplinary Sickle Cell Disease-Pulmonary Clinic, pulmonologists and hematologists collaborate to provide comprehensive care.
A retrospective analysis of the combined clinic’s successes showed:
- Reduced ACS episodes and acute care visits
- Higher rates of asthma diagnosis and prescriptions for medications including hydroxyurea and inhaled corticosteroids
“Patients with SCD have historically been underserved in both access and quality of care,” Dr. Gillespie says. “We need dedicated pulmonary specialists who understand the complexities of this disease.”
The Future of SCD Pulmonary Care
While there are no new emerging disease-specific therapies for pulmonary complications in SCD, gene therapy and hematopoietic stem cell transplantation (HSCT) show promise in the management of SCD. However, accessibility and affordability remain barriers. The study also calls for environmental risk screening, as air pollution and secondhand smoke significantly worsen pulmonary outcomes.
“Health care policies may have the most potential to positively impact children with SCD,” says Dr. Gillespie. “Expanding funding and improving the transition to adult care for adolescents could significantly improve long-term outcomes.”
Reference:
Gillespie M, Afolabi-Brown O, Machogu E, Willen S, Kopp BT. Updates in Pediatric Sickle Cell Lung Disease. Clin Chest Med. 2024 Sep;45(3):749-760.
Image credit: Adobe Stock
