Maggie's Story: Parent's Letter Project Congenital lobar emphysema- Maggie's mom

Maggie is home June 2012I had a normal pregnancy and a Cesarean-section delivery for breech presentation at 40 weeks with a 9lb, 4oz, baby girl. She seemed big and healthy and didn't cause me and my husband any more concern than any regular newborn, until she turned blue.

She was immediately sent to the Neonatal Intensive Care Unit (NICU) and was given blow-by oxygen. She was in the NICU for two nights and then came home with us when I was discharged. She slept a lot. She didn't have much of an appetite and tired easily after having a bottle.

At her five-day check up, my pediatrician was concerned because she wasn't gaining weight and she had retractions when she breathed. She was concerned it could be mitochondrial disease and sent her to a cardiologist for a work-up. Her heart checked out fine, but an X-ray did reveal that part of her lungs were collapsed. The NICU doctor compared the new X-ray with one immediately following birth and thought there was slight improvement. We thought it was something that would work itself out.

At Maggie's two week check-up, my pediatrician (same practice, different doctor) was also concerned with her rapid breathing pattern and retractions and admitted her to a hospital in Northern Virginia for further evaluation. She had a CT scan, X-rays, ng feeds, and oxygen while there. The pulmonologist discharged her after five days thinking that this was something she would outgrow. He wanted to see her in two weeks with a new X-ray.

Unhappy with this, I called our pediatrician who wanted to see Maggie. She agreed, there was no change and I had a right to question the doctor's decision at the Northern Virginia hospital. The nurse checked Maggie's pulse-oxidation (pulse-ox) and it read 84 percent. The doctor immediately called 911 and Maggie was taken by ambulance to our local hospital to stabilize her. Since we had tried the local hospital in Virginia and they were of no help, our next choice was Children's National.

At 2 am, Maggie was transferred to Children's National and admitted to the NICU. She was almost back to her birth weight and therefore, the largest baby in the NICU. The doctors did a spinal tap which revealed nothing significant. Maggie also had a bronchoscopy and a milk scan. It showed that she had a very severe case of bronchomalacia and reflux. She was there for two weeks and was sent home on a 1/4 liter of oxygen and an ng tube for nighttime feedings, to increase her caloric intake because she was working so hard to breathe.

Maggie had a number of follow up appointments with Pulmonary and Gastroenterology after her discharge in early November. In December, she had a VQ scan which suggested that she could have congenital lobar emphysema. Her doctor at Children's National thought it was best to wait things out and keep her healthy to see if time would help the situation.

Unfortunately, in February, Maggie got sick with human metapneumovirus (similar to RSV). She was admitted to our local hospital and then transferred to Children's National via helicopter. She was admitted to Children's National with respiratory failure and was on a respirator for six days and then in the hospital for another eight days following extubation.

In March, Maggie contracted rhinovirus - the common cold to me and you - but it landed her in the hospital at Children's National for one week. During this stay, I had decided that enough was enough. She wasn't well enough for any further testing, but we also weren't any closer to answers.

So, I wrote a letter to CHOP (Children's Hospital of Philadelphia). A doctor called me immediately after reading my letter and scheduled a full day of tests for Maggie on March 31. At 9 am, Maggie, my husband and I met the doctor. At 10 am, she had an infant pulmonary function test (IPFT). At 12 pm, she had a CT scan with contrast. At 1 pm, she had an ultrasound with cardiology and an EKG. At 2pm, we met with the cardiologist who ruled out any heart disease. At 3 pm, we went back to the doctor's office and he gave us the results of the PFT and the CT Scan - she did in fact have congenital lobar emphysema, affecting her right middle and left upper lobes of her lungs.

The right middle was so inflated that it was compressing all of the other lobes so they were not functioning well, if they were being used at all. He scheduled us to meet a cardiothoracic plastic surgeon, in the morning. Both doctors agreed that the right middle lobe needed to come out if Maggie had a chance for a normal life.

Unfortunately again, in April, Maggie was diagnosed with another illness - parainfluenza 2 (pneumonia). Again, respirator for six days and then eight more days in the hospital. During this stay, we chose a surgery day at CHOP - June 23. Maggie would need to be well for eight weeks from the day of her diagnosis with the Parainfluenza.

Our care at Children's National was turned over to the pulmonary chief, Anastassios Koumbourlis, MD. He was in contact with the doctor at CHOP, who was also in contact with Maggie's pediatrician. Surgery on June 23 went "better than expected" - the right middle lobe "wanted to come out" - these are words from the surgeon to us. Now, eight days post-op, and Maggie is off oxygen. We are weaning her ng nighttime feedings and she is doing very well.

It took awhile for us to line up a good team of experts to care for Maggie. All of the research that we had done on the disease showed that diagnosis is typically made by 37 days. It took longer for Maggie because she didn't "fit the profile." She looked good, she was gaining weight (with ng and extra calorie help), and she was not sleeping all of the time (with the aid of the oxygen).

The Pediatric Intensive Care Unit (PICU) and NICU doctors at Children's National were very helpful to us. The Pulmonary staff, while pointing at the diagnosis, wouldn't commit to it. We needed a commitment so we could pursue a plan for our baby. Children's National will continue to do her follow-up care and (knock on wood) care for her, should she need to be hospitalized. We are thankful for all that they did to help Maggie in the first six months of her life, but we did have to look at another hospital to get the answers that we desperately needed.

This is a difficult journey. Surgery day was a nightmare - I had pictured a million scenarios, but luckily for Maggie, it worked out wonderfully and now we have our baby girl back and we can concentrate on her and our family and keeping her healthy.


Maggie's Mom

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