If you are unfamiliar with all that Duke Hospital is, let me share a bit of information about it. Duke Hospital is in the top 10 hospitals in the country and has been for more than twenty years. Duke Children’s Hospital is consistently ranked among the top pediatric hospitals in the country. It’s Neonatology Unit (where we were going) is ranked # 16 in the nation by US News and World Report. It’s a research hospital with cutting edge technology and innovative treatments.
We’d heard very good things about Duke from other parents of children with similar disabilities. Dr. Marcus, the pediatric plastic surgeon, has an incredible reputation. Duke has pediatric specialists that we just wouldn’t have access to without transferring to a world class facility.
This was a huge adjustment for our family. My mom lives a couple of hours from Duke and took time off work to keep the boys while we stayed with Hermione. Once again, time stopped.
The first two days after arrival, Hermione was in isolation, so we had to wear masks and paper gowns and gloves. Everyone wears gowns in the NICU at Duke, it’s much more controlled than things were at Mission – the rules are very strict about visitors and in fact about everything. As a teaching hospital, there were a lot more people involved in her care. Rounds every morning were essential, as this determined her course of care. Hermione had a nurse, a nurse practitioner and a neonatologist all directly attached to her. We knew immediately that it was the right decision.
TESTS, TESTS, TESTS!
Hermione has had every test imaginable. She developed hypertension during transport (it still hasn’t gone away). Renal, Cardio, Urology, Neuro, Speech, OT, Plastics, ENT, the list goes on and on. CT, spinal tap, literally dozens of blood tests and cultures, 3D-CT, ultrasounds, ECHOs, x-rays, etc, etc. We were fortunate enough to have a room at the Ronald McDonald House of Durham for the entire duration of Hermione’s stay. We did laundry, watched football games, played with the boys in the playroom, met the Rockettes and were served dinner by all kinds of generous people from lawyers to the Duke Lacrosse team. We connected with other families and were able to stay three blocks away from our little girl. We knew people who lost their children. It really does put it all in perspective – there were so many children there who had more serious challenges than Hermione did. There was always help for the mortal challenges with Hermione. This was not always the case for children who came here for care. We still stay in touch with many of the families that we met there.
The Ronald McDonald House in Durham housed people from as far away as France and New Zealand. People come from all over for the amazing care that Duke as to offer. We knew families who had been there for months and even years with their children.
Hermione got a cold within three days of our arrival, which proved to be hugely problematic for her. She went into isolation and suffered partial lung collapse. This put off the exploratory surgery (bracheostomy) which would determine her course of treatment. She didn’t see a human face without a mask or feel touch except through gloves for two weeks. It seemed like an eternity. We’d been there for almost three weeks before we even began to explore the issue with her jaw.
She finally got her bronch! The doctors told us that what she really needed given the results was a jaw distraction. Cue more tests and further consults with doctors. Even though the initial evaluation didn’t show that her jaw was far enough back to warrant a distraction, internally it was much further back than it looked. JAW DISTRACTION Hermione got her jaw distraction on October 1st. The plastic surgeon did a fantastic job, the techniques are very advanced and only a little screw showed on the outside. Surgery lasted for about 4 hours and Hermione did very well. The surgeon cuts the bone in 4 places on each side and inserts titanium distractors, devices that move the jaw forward.
Over the course of a week, the screws are turned by either a plastic surgeon or a nurse, slowly pushing the jaw forward. The space will fill in with bone and the distractors taken our after a few months. Hermione’s jaw was .8cm too far back after the 3D CT scan. The distraction process actually moved her jaw 1.3cm to allow room for it to settle back. Sometimes distractions have to be repeated if the jaw settles too far back and blocks the airway again.
After surgery, Hermione needed to stay intubated and as still as possible for several days while the distractors did their work. She did notrespond well to the drugs that were needed to keep her sedated. For two days she convulsed and fought, it was a constant battle. It took two nurses to change her diaper in order to keep her from jumping out of the isolette. She cried and cried and cried and struggled. She had and episode of respiratory arrest – her lungs seized up and she completely stopped breathing, then started again a few seconds later with her heart rate dropping rapidly.
Finally, on the second day after surgery, the pharmacist found a drug combination that would keep her relatively quiet, however it had to be kept at as low a level as possible in order to avoid long term damage. This was one of the worst parts of everything that she went through. We’d sit next to her bed and watch her shake or touch her and feel how limp she was. We didn’t hold her at all for a week. The only comfort we had for her was to talk to her and stroke her little body. She had a music player with Mozart that we played almost constantly to soothe her. I can’t imagine what it was like for this little girl, how scared she must have been.
After four days, we were finally able to decrease the drugs keeping Hermione sedated and unresponsive. While we were working to get a feeding tube in so that she could eat for the first time since surgery, Hermione had a ten minute long seizure. I was there when it began, in fact I was watching her and saw her eyes roll in the back of her head. I tried talking to her and she was completely unresponsive. I asked the nurse what was going on and as I did she started shaking violently, her eye rolling and her tongue moving, mouth foaming. It seemed like eternity. My husband had literally just stepped out for a cup of coffee and I texted him to come back ASAP. There must have been a dozen people around her bed by the time it ended. Movement didn’t come back in the right half of her body completely for three hours. Since she was still intubated, she had no breathing problems. She was on an EEG for two days, and while her brain activity was slightly unusual, it wasn’t abnormal enough to be problematic.
Two seizures gets a diagnosis of epilepsy. So far she hasn’t had another one and she is set to be on seizure medication for at least a year to ward off any future activity. A terrifying moment but hopefully an isolated one.
We had a huge battle to get her extubated and eating again. The ICN doctors just did not have the same vision of how this should go for her and kept putting of drawing down the medications and getting the tube out, despite the all clear from the surgeon. We appreciated their caution, but we were very concerned about the incredible amount of medication and the side effects that she was experiencing. Hermione is listed as having an opiate sensitivity and a morphine allergy and she has had the physical reactions to back those up. It took us calling a family meeting to have anyone listen to us about her intolerance for the level of medication. It’s still not clear how much of a role the huge level of medication played in her seizure. She had 3 different heavy narcotics in her system at the time.
The distraction completely cured her breathing problems!! Once extubated, Hermione has been breathing on her own ever since. It really was a miracle surgery. It was like having a new baby, a new lease on life and an incredible victory.
This is video of her just minutes after extubation, breathing on her own for the first time (the nasal cannula only lasted for 12 hours and was just precautionary). Compare it to the video of her breathing in this post and you can see the amazing difference!
Now the only thing between us and home was the bottle. From the beginning we had known that this would be a long process. Everyone had warned us that after being exclusively tube fed for so long it would be an uphill battle to get her eating by mouth. Just as at the beginning of this journey, we were prepared to be in it for the long haul. Hermione was now almost three months old, and babies lose their sucking instinct between 2 and 6 months. We had consistently worked with her on the pacifier and had given her as much positive oral stimulation as possible throughout her stay. In retrospect we could have perhaps done more, but we were just so concerned with keeping her breathing.
We had a huge battle with the feeding team at Duke, who insisted that she would not be able to swallow and so would need a gtube. This seemed a little insane as she was in full blown withdrawal at the time that they made this assessment. She was finally weaned off of the narcotics and when that happened she went through two days of shaking and crying and didn’t sleep for 36 hours. We didn’t feel as though she was really given a chance. She had been bottle fed at Mission when she was just two weeks old, though she’d only gotten up to half over her feeds and that was only for three days. She had also been taking drops of milk on her pacifier from us after we insisted.
Suddenly people were telling us that Emanuel Syndrome meant that would probably need a gtube. This is just crazy considering how many children with Emanuel eat just fine. It is astonishing how many doctors look at a diagnosis without looking at the patient. Emanuel doesn’t mean anything unless she shows us something and we are not going to allow her care providers to make assumptions about her based on her chromosomes. The feeding team was completely unsupportive, only offering us one chance at the bottle per day and one week to make a decision about surgery. That’s just bad behaviorism! Of course she’s not going to want to work at eating out of a bottle if she’s accustomed to the food just going in without any effort on her part. If you feed her in the tube seven times a day and then put this weird thing in her mouth only once she’s just not going to get it. Besides that, how can you tell us over and over again that this is a long process and then not give her any time? If it was going to take potentially two weeks to get her on the bottle or two weeks surgery scheduled, completed, her recovered and us taught how to use the gtube, then why not go for the bottle instead of both putting her through surgery and losing out on all of the developmental advancement that she would get from eating by mouth. This is obviously not what we expected from a place like Duke.
We called a family meeting and made our case in front of the head of neonatology. He was in our court and gave us his full support. He gave us the go ahead to try her on the bottle at as many feedings as we wanted. The feeding team specialist (who incidentally we will see again as she’s on the cleft palate team) still didn’t give us her support. She was unsupportive at best and passive aggressive at worst. By this time we had been moved out of the ICN and into the Transitional Care Nursery because she was doing so well.
Originally she was on a Haberman feeder, but she just wouldn’t take it anymore. Looking at the nipple on the Haberman, we realize that it was very similar in feel to the suctioning device that was the bane of her little existence. We approached the team and said that she need to move to another of the cleft palate nursers as we thought that her aversion to the bottle might have to do with her fear of the suctioning device. The feeding specialist then gave us Pigeon bottle and valve with a regular nipple. It made a huge difference. Hermione would latch and suck for thirty to forty minutes. The problem was that at most get 20 ml of a 65ml feed, usually more like 10ml. It was hard work for us all. My husband and I stayed at her bedside, giving her 6 of her 8 feeds a day so that she could work on the bottle and just leaving to spend a little time with the boys, eat and sleep. After a week of us having to come up with ideas about what kind of bottles to use with her and not feeling like we were getting any guidance from the experts, we were exasperated.
At last we decided that we just weren’t going to get there without more help. We asked to transfer back to Mission, who’s feeding team had her on the bottle at least for a few days right after she was born. We clear to the head of neonatology that the reason that we were leaving was that we believed that the feeding team at Mission was superior to what we’d found at Duke.
On a fun note – Hermione had her first airplane ride. She flew by fixed wing from Duke back to Asheville. We didn’t even find out until she landed and we did the math on how long it took her to get there. When we asked how she got there on Saturday instead of Sunday, we found out that she had flown! That baby has had so many adventures.
At Mission we found a VERY supportive team.
CLEFT PALATE FEEDING
Cleft palate babies can’t create suction, so they can’t drink out of a normal bottle. Mission worked with us and the Mead Johnson Cleft Palate bottle in order to get volume in. We mixed and matched tips and finally got something that worked. It was amazing to get her eating on her own!
The feeding team listened to our ideas but also offered feedback. They told us that of course a feeding tube was still possible – which was fine with us, we just wanted to give her an honest chance. They came to see us three times a day for the first few days that we were there. She made consistent progress and (though that took a bit of a battle as well) was offered the bottle at every feeding, even when we weren’t there. Our caregivers at Mission were amazed at her transformation, what a difference!
Finding the right people who supported our vision was essential to her feeding success.
After a week at Mission we moved to transition, where I stayed with her 24/7 for a week, giving her all of her feeds. Mission has as beautiful transition ward. The rooms are private, with a sofabed, crib and beautiful mountain views. It was so good to be near her. She finally went to on-demand feeding a couple of days in and lost her feeding tube on day 4. One week to the day she moved to transition, our daughter finally came home on October 29th.
As you can see, her big brothers (ages 3 and 5) were VERY excited to have their baby sister home. They still are over the moon to have her – we have remarkably never seen a trace of jealously, though this journey was rough on them. She could not have better big brothers.
It has been a long, hard road – this is true for every parent of a child with a disability. You are dropped into a world unknown and unwanted with no way out. Our experience in special education has been both a blessing and a curse. We certainly know how to navigate the system, where to go for help and how to help her developmentally. We also are keenly aware of what choice boards and walkers look like. We understand that this will be a lifelong struggle. It’s hard to accept this, but she amazes us daily.
If she is like Peter Pan and never grows up, that will be just fine. We’ll ride the teacups with her at Disney World when she’s forty. Of course we’d do that if she went to college.
We would not have made it through those dark days without the immense support of our family and friends. The words of kindness, cards, prayers and good wishes all made it possible for us to get her what she needed. THANK YOU to everyone who was there for us through this entire ordeal!
Hermione is doing very well at home. We thought that we’d be bringing home tubes and machines, at home nurses and around the clock care, but other than her medications for blood pressure, seizures and extra saliva (a cleft palate thing), Hermione is a normal kid. She is very small for her age, in the .2 percentile on weight, but is growing with the curve. She’s developmentally behind, but only by a month and consistently follows the developmental curve so far.
Special needs children need a chance to be the most that they can be! There is a fine line between hope and denial, between pushing her to be all that she can be and accepting who she is. We demand the best of her, just as we do of our other children.
Hermione is proof that a diagnosis is not a sentence to a life without hope. We are excited about her future.