Chronic Lung Disease
Your baby has been or is being transferred to the Newborn/Infant Intensive Care Unit (NI/ICU) at Children's Hospital of Philadelphia for evaluation and treatment by the Newborn and Infant Chronic Lung Disease Program.
What is chronic lung disease?
Infants who are born prematurely and require respiratory support can develop chronic lung disease (CLD), also known as bronchopulmonary dysplasia (BPD). CLD means that the baby cannot breathe well without either the ventilator or oxygen by 36 weeks corrected gestational age. Infants with CLD often have scarring and cysts in the lungs, which prevents normal lung function. They can also have problems with the blood vessels that supply the lungs. These problems make it difficult for the heart to pump blood to the lungs and to the body. This is called pulmonary hypertension.
Our Team
Neonatologists – The Chronic Lung Disease Team is led by neonatologists experienced in the care of infants with CLD. Our CLD attending neonatologists work closely together to assure that your baby receives consistent care. The team will meet with you to discuss plans for treatment after the initial evaluation. The CLD neonatologist, specialists, and multidisciplinary team meet weekly to discuss your baby's progress.
Consultants – We work closely with other specialists also interested in CLD.
Cardiology/Pulmonary Hypertension Team – Your child may have developed pulmonary hypertension as a result of the lung disease. We work closely with the Pulmonary Hypertension Team from the Division of Cardiology to evaluate and treat pulmonary hypertension. The team will continue to follow children with pulmonary hypertension after they are discharged from the N/IICU.
Pulmonologists – We often seek input from our pediatric pulmonologists. This team, also known as the pulmonary BPD Team, specializes in lung disease in infants and children. They will help manage your child’s lung disease after they are discharged from the N/IICU.
Bone Health Team – Premature babies have low amounts of calcium and phosphorous. When combined with medications used to treat lung disease, they can develop “thin bones.” Our Bone Health Team will evaluate your baby and help us develop a plan to improve bone health and prevent fractures. This team, led by endocrinologists, may prescribe specific testing and medications for your baby’s bones.
Nutrition Support – Many premature infants with CLD have challenges with growth. A neonatal dietitian will help decide the best way to promote growth while protecting your baby’s lungs. You will see the dietitian on daily rounds, providing information to the team on nutrient intake, feeding choices, and feedback from the evaluation with CHOP’s Bone Health Team.
Psychosocial Developmental Team – The Chronic Lung Disease Program offers developmental and psychosocial support services to families while their child is being cared for in the N/IICU.
These services may include, but are not limited to:
The clinical staff are well trained and certified in their specialty They are available to work with you and your baby to create an individualized care plan for:
The staff will work with you and show you how your baby communicates when they are tired, ready to play, eat, or upset. They will also show you safe ways to bond with your baby through touching, holding, and calming.
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When your baby is medically ready, a speech-language pathologist may help with early communication and oral feeding skills. The Speech-Language pathologist will assess oral motor skills and provide recommendations for oral feeding or stimulation depending on whether your baby can take breast milk/formula by mouth.
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Baby’s motor skills are evaluated by either an occupational or physical therapist. They are skilled in assessing your baby’s use of large and small muscle groups and will recommend the best position for your baby. These specialists will also provide ongoing therapy to promote the development of motor skills, play, social interaction, and self-calming skills.
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Each family is assigned a social worker available to support you during this emotionally stressful time. They realize that as a parent, you are struggling to balance your time between the hospital and home. They can help you find ways to stay connected to home while supporting your child in the N/IICU. They can also support you around issues regarding adjusting to the N/IICU, exploring housing options, transitioning back to your home hospital when the time is right, and understanding the long-term impact of this medical condition.
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Our child life specialists (CLS) are here to provide your baby with comfort support during procedures and developmental play opportunities when they are ready. If your baby has siblings, the CLS can help you to prepare them for their first visit to the hospital and assist in supporting and educating them while your baby is here.
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Music therapists and art therapists are also part of this team and can work with you to find creative ways to express yourself, as well as bond with your baby.
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The developmental psychologist will assess your older infant and work with the other developmental specialists who know your baby to create a developmentally appropriate schedule of activities. The psychologist is also available to discuss stresses you may have while parenting your baby in the hospital.
What can I expect upon arrival at CHOP?
Once admitted to the N/IICU, your infant will be evaluated by our CLD Team. In addition to chest x-rays and echocardiograms, which your baby likely had at your home hospital, many infants in the CLD Program will have further testing of their heart and lungs:
CT angiogram – low-dose CT angiogram provides a 3-dimensional picture of your child's lungs. Our radiologists have worked to reduce the radiation dose for this procedure to less than a tenth of the amount that is used for standard CT scans.
Pulmonary function tests (PFTs) – Pulmonary function tests can give us the data we need to develop the best plan for ventilating your child's lungs. PFTs also help us to understand your baby’s response to pulmonary treatments such as bronchodilators.
Ventilation/perfusion scan – This study is less often needed, but it helps determine whether there is a match between areas of the lung that are ventilated (exchange air) and the areas that are perfused (have blood flowing through them to pick up oxygen and release carbon dioxide).
Cardiac catheterization – Again, this is less often needed. It may help show the team the amount of pulmonary hypertension and helps them to decide whether certain medicines might be useful. Cardiac catheterization is done by a cardiologist with extensive training in this procedure.
Bronchoscopy – Many infants with chronic lung disease have problems with their airways that delay their ability to come off the ventilator. Potential airway problems include:
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Subglottic stenosis: areas of narrowing and swelling that result from prolonged intubation
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Tracheobronchomalacia: weak or floppy airways
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Lower respiratory tract infection
If we think that your baby has one of these problems, we will consult our Neonatal Airway Team and pulmonologist. A bronchoscopy, a test to look at the airway with a bronchoscope, can be performed at your baby's bedside or in the operating room. This test is performed by our ENT or pulmonary physicians.
Evaluation for gastroesophageal reflux and aspiration – Some infants with chronic lung disease have gastroesophageal reflux and they aspirate their feedings into their lungs. When the contents of the stomach go into the lungs, your child’s oxygen levels may fall. This can cause further injury to the already damaged lungs. To test for reflux and aspiration, your baby might need an upper GI study and a milk scan. For the milk scan, we give your baby a “milk” containing medicine that is placed into the stomach. A series of pictures is then taken to follow the path of the “milk” through the body. If we are concerned about reflux and aspiration in your baby, we may feed them into the duodenum or jejunum instead of the stomach to protect the lungs and allow them to heal. Your baby may need medications to block the acid production in the stomach.
Some infants with severe reflux or aspiration may require an operation called a fundoplication in which a portion of the stomach is wrapped around the esophagus to stop reflux. These infants will also have a gastrostomy tube (G tube) placed as well. A G tube is a tube inserted through the abdomen that delivers nutrition directly to the stomach.
Medications used in the management of CLD
The overall goals of therapy for CLD are to protect the lungs while providing for enough gas exchange for normal growth and development of your baby, and their lungs and lung blood vessels. We will discuss the risks and potential benefits of the medications with you.
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Diuretics – Many babies with CLD are on diuretics such as chlorothiazide (Diuril®), furosemide (Lasix®), or spironolactone (Aldactone®). Your baby was likely started on one or more of these medications before transferring to CHOP.
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Bronchodilators – Many babies with CLD have airway problems similar to those seen with asthma. Often, these babies are treated with bronchodilators such as albuterol and inhaled ipratropium.
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Corticosteroids – Most infants transferred to CHOP with CLD have had at least one course of steroids for their lung disease. This may have been referred to as the DART protocol. We will discuss if your baby might benefit from another course of steroids.
Medications used to treat pulmonary hypertension
If your baby has pulmonary hypertension, they may be treated with a medication that opens up the pulmonary blood vessels. These medications include:
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Nitric oxide (NO) – a gas that can be given through the ventilator or by a nasal prongs. It can only be given in the hospital
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Sildenafil – a medication that is given by mouth (or NG or G tube). It is possible to give this medication at home.
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Flolan® (epoprostenol sodium) and Remodulin®(treprostinil ) – these medications are given by IV as continuous infusions. Infants have been sent home or to home hospitals on these medications, but they require extreme care as they cannot be stopped suddenly. These medications require careful planning and discussions
For more information about our program, please visit our website at https://www.chop.edu/centers-programs/newborn-and-infant-chronic-lung-disease-program
Reviewed July 2024 by Kathleen Nilan, RN