CHOP: Where hope beings!

Yesterday we spent the day at Children’s Hospital of Philadelphia. We were truly impressed, not only by the beautiful facility but by the professionalism and kindness we were shown at every turn. We had appointments with different departments (all within the special delivery unit) lined up all day but got ahead of schedule and so they just moved us around and we had completed all of the diagnostic testing, ultrasound, ECHO, and MRI before lunch. We took a short stroll to a sandwich shop across the street. I have always loved being in the city with Mickey. It seems like a life we should have lived together once. Holding hands, crossing streets, looking at buildings and art.

After lunch we met with the genetic counselor who was truly kind and went through our family histories once again, not identifying anything that would give us the answers to this, but definitely giving us insight into areas we should be watching in our own and children’s health and lifestyles.

At about 3pm we met with a team of doctors and nurses and the genetic counselor. Our doctor is Dr. Nahla Khalek. She was very knowledgeable and also very kind. She discussed her thoughts on Auguste and always called him by name. I don’t think doctors always realize that calling him by his name vs the fetus makes a big difference emotionally. It showed that she was viewing him as a person of value.

Then we hit all of the bases and all of the different scenarios we may encounter since we have chosen to continue this pregnancy.

The first is continuing to do our best to identify the problem. There is a diagnosis called chylothorax that is a essentially a leak of lymphatic fluid. She states that this is a common disorder accounting for many cases of hydrops. The only way for her to know if this is Auguste’s diagnosis is to take a sampling of his pleural fluid (by doing a thoracentesis). At this point, she feels the timing is not right. The main reason she does not feel she can do this at this time is that there simply is not enough fluid to do it safely. The procedure is done much like an amniocentesis, with a small needle making once swift pass through me, my uterus, and into a wiggly baby’s chest. She said there is not anesthetic used for either of us but it was quick and would feel like my amniocentesis. She said since the fluid is “moderate”, she would be concerned that there would be risk to hitting his lung or heart at this time. However, she now wants to see us back next week, feeling at this time there may be enough fluid to do this. If there is, they would do the thoracentesis, then wait the weekend and make sure a shunt was not necessary depending on if the fluid rapidly came back. A shunt placement is more like a surgical procedure, where I am sedated, as is Auguste, and they also use local anesthetic. We asked if it was possible to know from SC if the fluid is more, but she feels strongly that since she is the surgeon who would be performing the procedures, she needs to be the one assessing this. This makes sense and so we are set up to see her again next Thursday morning, in Philly.

We are working out the details regarding the kids, but are actually planning to bring Emily with us next time, and probably letting Oliver and Eliot spend a long weekend in Florida with my family.

They are also talking back and forth with Greenville to see if they have enough cells to test for Noonans syndrome, which is also commonly associated with hydrops.  We discussed this syndrome at length. It certainly can present in many ways, from fully functional with few noticeable differences, to significantly delayed and fairly apparent physical characteristics.

We discussed what happens if we rule chylothorax and Noonans syndrome out, which is likely. Right now Auguste’s lungs are in a very critical time of development and the risk of pulmonary stenosis is high. This basically means that they would not develop and could possibly not be as elastic thus inhibiting viability outside in the world. She said that if the fluid stays where it is currently, it is extremely difficult to say whether it is inhibiting lung development or not. She said we needed to be prepared for making it to delivery, but knowing that there is a very real possibility that we would never be able to bring him home.

She encouraged us to go ahead and meet with a neonatologist so that they know us and know what is going on, and make sure that they were prepared for very small babies, and felt that they would call and make sure there was ECMO (extracorporeal membrane oxygenation) available as that would be key to giving him a survival chance in the outside world. She felt strongly that in the absence of an emergent situation, we should consider delivering in Philadelphia, as there have been studies linking the NICU capabilities to outcomes in babies and she felt sure they were one of the best in the nation, and also touted University of Ca, San Francisco and University of Alabama in Birmingham with being up there with them.  She also let me know that if he makes it to 33 weeks, that she would not suggest we go any further than that in pregnancy as she felt they would be able to do more for him in the NICU than I could in utero at that point.

She said her first concern is lung development, and her second concern is brain development. At this point his brain looks beautiful, but although she could definitely monitor brain development, there is no way to monitor brain function. She wanted us to be prepared for anything, from completely normal to significantly delayed. We feel that we are more prepared than most for some of these things, but since there is no way to tell one way or another until birth, we realize there may be many more hurdles to clear.

We discussed Mirror syndrome. Mothers and fetuses are very intimately linked and in 10-15% of cases, mothers will develop similar symptoms, and get very sick very quickly, she stated that it was life threatening and she wanted to make sure we were ready to induce labor at any point if this occurs, no questions asked. She said I should be followed extremely closely and have my urine checked for protein and frequent blood pressure checks, etc. I understand this and although it is a very emotional subject, I do know that Oliver, Eliot and Emily need their mother, and agreed that if this happens, we will induce labor and do whatever we can for Auguste at that time, but to keep my life safe as well. Mickey feels skeptical because he thinks I tend to blow off my own health in certain situations, primarily going back to when I was in labor with Oliver and didn’t tell anyone right away because I wanted to finish making frozen meals at “Dinner Done”. I promised him and Dr. Khalek that I can be trusted and that  I love my children, and will do all I can to be there through their life. BUT, that I felt that my odds (90%) seemed more favorable than Auguste’s (10%) and that at this point we should proceed with him in the forefront.

I then asked the question I hate to ask, and I asked her if she had seen any baby’s in which the diagnosis is never found, who presented the way Auguste is presenting, who survived. Her answer was an unfortunate ‘No”. Unfortunately, they usually die in utero and I will need to be prepared for that. They talked to us about how to prepare ourselves and the other children and let us know they would assist in any way.

But she added that we have our own story and that just like every story, they are all different and that the Losinski family’s story is our own unique story and that certainly, all hope is not lost and that they would travel this journey with us and do all they could do help us each step of the way.

A lot of crying happened at the end of our visit, after most of the group had cleared and we were left with Dr. Khalek and our nurse, Laura. I talked about all my plans and how perfect my life was just a few weeks ago, and how everything seemed to be going according to plan. How I had never thought I may actually have to bury a child. I just never thought this would happen to us. Dr. Khalek said “well, this IS happening to you, but you are never going to be given something you can’t handle, and you will make it through this journey despite the outcome. You will find that no matter the outcome, it needs to make sense to you”.

I explained that we are not unreasonable people and that we know the facts and we know that we will very likely lose Auguste. But that my faith makes me focus on the survivors and the stories of miraculous healing, and that if 10% survive, then why not Auguste? Why not?! He is alive today and we will take it one day at a time and believe that he will be alive in January (his new due date) and that he will be alive when we take him home.

I truly like the team at CHOP and I feel we did the right thing in traveling to them, because despite the outcome, they seem to be on board with giving us the best possible chance for Auguste.

We decided to go ahead at that point and head towards SC. Mickey feels less confident that he did yesterday, but agrees he feels we are doing all of the right things and that no matter what, we want Auguste to be able to give hope and chances to other baby’s facing this. We turned the music up and I did a lot of crying, and we just drove. We made it to Fredericksburg, VA, which is where one of the schools Mickey has applied to is located, University of Mary Washington. This morning we stopped at the campus and looked around. It was quiet, I think they are on fall break. We ate at a little diner called 2400 diner and it was wonderful. We stopped for lunch in Elon, NC and checked out Elon University, and liked that area so much we may have moved it up on the list. Very nice town.

There is still a lot to coordinate regarding work and time off, medical visits, bills, kids school, etc over the next few months, and it is scary and will be challenging both emotionally, and financially. Our family is definitely going to face a lot, but we are going to do the best we can for all of our kids and do things one day at a time.  We have a good support system, but our families still work and are also having to travel, so there is definitely a lot of challenges ahead. I emailed the doctor tonight to find out some more answers to logistics such as timing of surgeries, etc. Hopefully we can get the next two weeks planned in the next few days.

Please keep Auguste's healing in your prayers as we are going to maintain our hopes, and trust in miracles. They do happen and they can happen. There is always a chance. The CHOP slogan is: "Where HOPE begins".  My hope is that after Auguste is born, Dr. Khalek will be able to tell the next mother who asks her if she had seen survivors, and she will be able to give a confident, YES!