Connecticut Children’s is one of the few institutions that offer a sickle cell pulmonary clinic. The clinic is devoted to sickle cell patients and is held once every other month.
Frequently Asked Questions
It has been found that a significant number of children with sickle cell disease have abnormal lung function starting as early as infancy. Although those who have lung problems like acute chest syndrome are more likely to have abnormal lung function, a significant number of children with no history of lung complications also have abnormal lung functions.
Development of chronic lung disease in adults has led people to believe that lung function abnormalities start in infancy and childhood. Our hope is that catching these lung function abnormalities by diligently screening during childhood will help identify problems sooner and perhaps decrease an incidence of chronic lung disease in adults.
Doctors frequently refer to pulmonary function tests as PFTs. To obtain a PFT, your child will need to go the pulmonary function laboratory at Connecticut Children’s main campus in Hartford. Currently, we recommend that all patients with sickle cell disease who are 5 years and older get a PFT at least once a year.
PFTs should be repeated sooner if your child has an episode of acute chest. Children who have history of wheezing or asthma and allergies should also get a PFT, and be followed by a pulmonologist three to four times per year.
The most frequent lung problem in patients with SCD is acute chest syndrome (ACS). This may occur both in children and adults with SCD, but is much more frequent in children and patients with SS disease.
Approximately 50 percent of all patients will have at least one episode of ACS. Causes of ACS may be infection, infarction or embolism. In patients who have asthma, an acute asthma attack may aggravate ACS.
Asthma occurs more commonly in patients with SCD. Both patients with SS and those with SC disease have an increased likelihood of developing asthma.
The cause of this increased frequency is unclear. The likelihood of having exercise-induced asthma is also increased in patients with SCD. Asthmatics who do not have sickle cell disease are more likely to suffer from allergies, which is not the case with patients with SCD and asthma.
This is a condition in which patients develop high blood pressure in the arteries that supply blood to the lungs. Symptoms include fatigue, shortness of breath and dizziness. This is known to occur in approximately 30 percent of children and adults with SCD.
The exact mechanism that causes pulmonary hypertension in patients with SCD is not known. It is thought that the development of chronic lung disease as well as the release of hemoglobin when red blood cells break down contribute to development of this condition.
This complication is a major concern because of higher risk of death in people with SCD and pulmonary hypertension as compared to people with SCD who do not have pulmonary hypertension. This diagnosis can be confirmed by a cardiology consultation. It is recommended that all patients have a pulmonary hypertension evaluation prior to transfer over to adult care.
Good quality sleep is an important part of a person’s wellness and wellbeing. Sleep disorders are common in patients with SCD. There are many reasons for this, including pain, lower oxygen saturations which may result from anemia, and scarring in the lungs from repeated episodes of ACS.
Poor quality sleep may also result in fatigue, poor attention span, hyperactivity or daytime sleepiness. All of these can interfere with school and job performance.
We usually recommend a sleep study to better evaluate your child’s sleep. This can be done at Connecticut Children’s Sleep Center. Your pulmonologist or hematologist can order this test.