About Sofia

Sofia was diagnosed on October 29th 2007, at 19 weeks gestation, with a Congenital Diaphragmatic Hernia (CDH) during a routine ultrasound.
CDH is a life threatening birth defect with a 50-60% survival rate. The muscle that separates the chest and abdomen (diaphragm) does not form properly during fetal development, allowing abdominal organs (stomach, liver, intestines, etc.) to move into the chest and compress the heart and lungs.  This causes the growth of the baby's lungs to be greatly inhibited.  Not only will the baby's lung be very small (hypoplastic lungs), the lungs will also not function as well as normally developed lungs. Often times the baby's lungs will not function well enough after birth for the baby to survive. The cause of CDH is unknown. CDH occurs once in every 2,500 births.

A sono pic from the day Sofie was diagnosed with CDH - 10.29.07

Sofie was born March 10th 2008 with labor induced at 38 5/7 weeks gestation. Studies show that babies born during the 38th week of gestation have the highest rate of survival. She weighed 6 lbs, 4.5 oz. A neonatal intensive care unit (NICU) team was present in the delivery room, and she was immediately placed on a ventilator to help her breathe. Within the first hour of life, she required high frequency ventilation and inhaled nitric oxide. We were very fortunate to have known of Sofie's CDH prior to her birth, so we could choose the best facility for her to be born in. She was stabilized at this delivering hospital, and then transported to a Children's hospital when she was about 4 hours old.
On day 3 of life she had her first surgery to be placed on ECMO (heart and lung bypass). Without this, she would not have survived. She spent the next 12 days on ECMO and on March 25th, had her second surgery to come off ECMO. Two days later, at 17 days old, she was able to have her diaphragmatic hernia repair surgery.

Sofie in the delivery room - 10 minutes old

Sofie @ 24 hours old - on high frequency vent + inhaled nitric oxide + 2 chest tubes + many lines + many meds

Sofie, 8 hours after being placed on ECMO - 3.14.08

The day Sofie came off ECMO - 3.25.08

The day of Sofie's initial CDH repair surgery - 3.27.08

90% of her left diaphragm was missing at birth. Her entire stomach, most of her intestines, her spleen and part of her liver were in her chest. During the repair surgery, those organs were moved into her abdomen, and a large Alloderm patch was used to create a new diaphragm, finally allowing adequate space in her chest for her heart and lungs.

She remained intubated and on the ventilator for the first 5 weeks of her life. At various times she was on a conventional ventilator, a high frequency oscillating ventilator, inhaled nitric oxide, and many medications. She had a total of 4 chest tubes during this time. We were able to hold her for the first time when she was 34 days old.

Mommy holding Sofie for the first time - 4.13.08

Daddy holding Sofie for the first time.

She "ate" for the first time at one month of age by an NG feeding tube. A few weeks later, she was able to start learning to eat by mouth, in addition to her NG feedings. She battled feeding aversion, aspiration, sensory issues, and narcotic withdrawals. We were finally able to bring our baby girl home after 81 days in the NICU.

Sofie going HOME!!! - 5.29.08

She came home on 1/8 L oxygen by nasal cannula and remained on that until the age of 10 months. After 3 days at home, her NG tube was removed by her pediatrician, and she was eating 100% by mouth. She continued to do so for a little over a month, and then suddenly started refusing to eat. After three weeks, we discovered why...

At 4 months of age, Sofia experienced a recurrence of her diaphragmatic hernia (reherniation). She underwent a 2nd repair surgery to close a hole in the original Alloderm patch. On her 3rd post-op day, Sofie reherniated again. This was debated among her doctors, who ultimately decided there was NOT another recurrence and sent us home after a 10 day hospital stay.

Sofie, after the repair of her 1st CDH recurrence - 7.22.08

At 8 months of age, it was discovered that she had indeed experienced a 2nd recurrence, and it likely happened just after her last surgery, as initially suspected. She had been reherniated for a total of 4 1/2 months at that point. She had a 3rd repair surgery, and was hospitalized for 5 days.

Sofie, the day after her 2nd CDH recurrence was repaired - 11.13.08

At 13 months of age, we discovered she had experienced a 3rd recurrence of her diaphragmatic hernia. For this repair she underwent a Reverse Latissimus Dorsi Flap Repair, a more invasive type of repair, since the last three had failed. It was estimated she would be in the hospital 2-3 weeks for this surgery. She was only hospitalized for 6 days!

Sofia, the day after her Latissimus Dorsi Flap repair - 4.30.09

Sofia received regular occupational therapy (OT) for gross motor skills related to decreased muscle strength after her eventful first year, as well as sensory integration and feeding therapy from 5 months to 3 years of age. She also saw her dietitian regularly from 5 months to 3 years to ensure proper nutrition and growth.  Her OT and dietitian came to us through Missouri's Birth to Three program, called First Steps. They were invaluable in her recovery process, and she has a wonderfully normal quality of life because of the help they were able to give her.

She suffered from gastroesophageal reflux (GER), a common long-term complication of CDH, until after two years of age, which was well controlled with medication. Her GER is now resolved.  We continue to watch her for the two of the most common long-term complications of CDH, late onset progressive sensorineural hearing loss and small bowel obstruction.

Despite all she's been through, she is developmentally appropriate for her age, and has made a full recovery.  She is truly a miracle.

Sofie, at 3 1/2 years old.