In November 2020, a 10-year-old girl with sickle cell disease and respiratory failure was intubated in the pediatric intensive care unit (PICU) at Children’s Medical Center Dallas, part of Children’s Health℠. A few months later in April, she was readmitted with acute chest syndrome and persistent hypoxemia (low blood oxygen levels).

The pulmonology team at Children’s Health treated the patient with an innovative, two-part treatment protocol that combines the use of a bilateral positive airway pressure (BiPAP) machine with respiratory physical therapy. This was the first time our team used this two-pronged treatment approach to help a pediatric patient with sickle cell disease.

In addition, a review of studies yielded no published research on this approach for pediatric patients with sickle cell disease.

The results of this combination treatment have been so promising that our team is in the process of standardizing the treatment protocol. Our goal is for more children with sickle cell disease and recurrent hypoxemia to benefit from this two-pronged approach.

“Pulmonary disease is associated with increased morbidity and mortality among children and adults with sickle cell disease,” says Devika Rao, M.D., Pediatric Pulmonologist at Children’s Health and Associate Professor at UT Southwestern. "We're excited about how this new protocol can prevent hypoxemia and help more children with sickle cell disease stay out of the hospital."

A team approach to caring for children with sickle cell disease

Many factors affect breathing when a child has sickle cell disease, says Dr. Rao, who oversees a pulmonology clinic at Children’s Health that she founded to help children with respiratory problems brought on by sickle cell disease. She also directs the Pulmonary Function Testing (PFT) Lab at Children’s Health.

Dr. Rao initiated care for the 10-year-old with sickle cell disease, which included a sleep study that showed she had low oxygen levels at night. Dr. Rao prescribed the nighttime use of a BiPAP machine. Still, she knew it would take a team effort, including partnership with parents, to get a pre-teen patient to regularly use a respiratory device.

“Tweens and teens have a lot going on,” she says. “They don’t want to bother with hooking up to a machine at night, or they fall asleep before doing so.”

Treatment part 1: The BiPAP machine

Respiratory therapists fitted the patient with a comfortable breathing mask and showed her and her family how to set up and use the BiPAP machine. Child life specialists met with the patient and family to educate them about the importance of following through with the treatment.

“Child life specialists are key to easing children’s anxieties,” says Dr. Rao. “Pediatric patients won’t use the machines if they’re anxious, don’t understand why they need it or simply because they don’t want to change any part of their routine.”

Treatment part 2: Respiratory physical therapy

Patients with sickle cell disease often experience chest and back pain, which leads to shallow chest breathing instead of deeper diaphragmatic breathing from the lungs. In addition, pain medications like opioids reduce respiratory rates, causing slow breathing.

Dr. Rao was concerned that the BiPAP treatment wouldn’t be enough to prevent rehospitalization for hypoxemia. It was during a conversation with a respiratory physical therapist about the poor breathing mechanics of pediatric patients with cystic fibrosis that Dr. Rao decided to see if respiratory physical therapy could improve breathing in this pediatric patient with sickle cell disease.

The patient participated in weekly respiratory physical therapy sessions for six months. The sessions focused on helping the patient learn how to engage their diaphragmatic muscles to allow the lungs to expand more fully and take in more air.

“Diaphragmatic breathing is more efficient compared to using chest and neck muscles to breathe,” says Dr. Rao. “But it’s a learning process to change the way you breathe after breathing a certain way for so long.”

The promising results

Within a few weeks of regularly using the BiPAP machine, the patient reported she was sleeping more soundly and had more energy during the day. A follow-up sleep study conducted six months later showed a significant improvement in nighttime oxygen levels. The patient’s daytime oxygen levels also improved.

Respiratory physical therapy was also a success. In a six-minute walk test conducted six and nine months into the treatment plan, the patient was able to walk farther while maintaining healthy oxygen levels.

“The combination treatment worked as we’d hoped with the BiPAP machine providing the necessary support and serving as a bridge until the patient had mastered diaphragmatic breathing,” says Dr. Rao. The patient was able to stop the BiPAP treatment after nine months.

Implementing a quality improvement initiative

Now, Dr. Rao and hematologists with the Pauline Allen Gill Center for Cancer and Blood Disorders (CCBD) are working together on a quality improvement (QI) initiative to standardize the treatment protocol. The goal is to quickly identify patients hospitalized with sickle cell disease and recurrent hypoxemia who can benefit from this new treatment approach. Dr. Rao is also collaborating with a pediatric pulmonology fellow on a research paper to report their findings.

“We’ve submitted research protocols to the Institutional Review Board and continue to refine the treatment based on our findings and what the data shows,” says Dr. Rao. “In the two years that we’ve been using this dual treatment approach, several patients have seen significant improvements in their breathing, which has contributed to reduced hospitalizations.”

However, Dr. Rao notes that there is still much to learn. She understands the need to analyze the data to confirm what she sees anecdotally as beneficial. The patient who was first treated with this dual approach is now 12. Two years after the intubation that started this treatment journey, the patient was hospitalized again with persistent hypoxemia.

“Upon evaluation, we discovered that she had reverted back to chest breathing,” says Dr. Rao. “This suggests the potential need for more regular follow-ups with respiratory physical therapists and ongoing breathing exercises at home.”

Dr. Rao is optimistic that this dual treatment approach can help children with sickle cell disease enjoy a better quality of life.

“We know that by helping children with sickle cell disease improve their breathing mechanics, we can potentially improve their oxygen levels and prevent respiratory problems that lead to hospitalization,” says Dr. Rao.

Learn more about our nationally ranked Pediatric Pulmonary Program at Children's Health. >>