Challenging cases: Managing chronic lung disease after stem cell transplant

Helping our patients survive and thrive after stem cell transplant.It’s Donate Life Month, and at Boston Children’s Hospital’s Division of Pulmonary and Respiratory Diseases, we’re focused on helping our lung transplant recipients survive and thrive year-round.

We give these most critically ill children the best chance by working across disciplines to develop a long-term care plan, using new and innovative therapies when available. We are one of the few pediatric centers to offer photopheresis, a therapy that has been beneficial in treating graft vs. host disease (GVHD) and graft rejection in lung transplantation.

Craig Gerard, MD, PhD and team manage a challenging case.
Craig Gerard, MD, PhD

“As many stem cell transplant (SCT) patients later battle chronic lung disease, they are well served by our integrated team approach involving specialists in pulmonology, endocrinology and dermatology as well as experts in oncology from Dana Farber/Boston Children’s Cancer and Blood Disorders Center,” says Craig Gerard, MD, PhD, chief of the Division of Respiratory Diseases and the Leila and Irving Perlmutter Professor of Pediatrics.

Read the case of a patient treated with a bilateral lung transplant for pulmonary complications after SCT for refractory acute lymphoblastic leukemia who is now thriving.


CASE STUDY:

In 2005, a 6-year-old male patient received a matched unrelated donor stem cell transplant for refractory acute lymphoblastic leukemia. The patient’s post-hematopoietic stem cell transplantation (HSCT) course was complicated by chronic cutaneous and pulmonary graft-versus-host disease (GVHD). The patient also experienced some issues with skin lesions including squamous cell carcinoma.

In June 2007 due to progressive pulmonary symptoms, the patient had an open lung biopsy showing chronic fibrosing/obliterative bronchiolitis and acute and organizing bronchopneumonia. By November 2007, lung function had declined to FVC 45 percent and FEV1 26 percent.

In early 2012 — after several years of stable lung function — the patient started to experience a cough, shortness of breath, and decrease in activity. He responded to outpatient oral steroids, and then developed a right-sided tension pneumothorax with respiratory failure in April 2012. The patient had a chest tube placed and was intubated at a local ER and transferred to Boston Children’s Hospital.

Once at Boston Children’s, the patient initially required high frequency oscillatory ventilation (HFOV) support. He failed to respond to therapy, was evaluated for lung transplantation and was listed in May 2012. Additionally, the patient was treated with sildenafil after a cardiac catheterization showed secondary pulmonary hypertension. He was converted to a conventional ventilator and had a tracheostomy and GJ-tube placed in early June 2012 and was discharged home three weeks later. At the time, lung function had declined further with an FVC 13 percent and FEV1 10 percent.

For the next 17 months, the patient did relatively well with home ventilation and outpatient rehabilitation with the occasional admissions for pneumothoraces and respiratory decompensations.

Due to abnormal chest wall growth and small thoracic cavity, the patient waited for appropriate lungs for over a year, ultimately receiving a bilateral lung transplant in October 2013. Due to large donor lungs (in particular the left), he had left diaphragm plication along with partial resection of the donor lung. The chest was closed two days after transplant and he was discharged 2.5 weeks later, having been weaned from ventilator support and with the tracheostomy tube removed. The GJ-tube was removed in March 2014.

Images before and after lung transplant in child.

The patient has done well post-transplant and is followed by our multidisciplinary team of specialists
in Pulmonology, Pharmacy, Nutrition, Dermatology, Endocrinology and Hematology/Oncology. Lung function has remained stable to date, although since chest wall issues are not uncommon in this population, we continue to see a restrictive pattern with FVC and FEV1 ~ 50 percent. Despite therapy for osteoporosis, the patient’s active lifestyle led to a wrist fracture and left femoral neck fracture requiring surgery.

We have been able to anticipate various issues common to this unique population of HSCT patients who have later received lung transplants, such as skin cancer, dermatologic issues, variability in immunosuppressive requirements and a high infectious disease risk. The breadth and depth of our team’s combined experience allowed us to address some of these common issues early on with
this particular patient — including immunosuppression adjustments to reduce skin lesions — contributing to his post-transplant success and overall health.

Now a senior in high school, the patient participates in school activities and is looking ahead to college.

Learn more about Boston Children’s Division of Pulmonology and Respiratory Diseases.