Another Surgery

Over the past week, Hannah has re-iterated several times, “I just can’t catch a break.”  One thing a parent never wants to hear is that at the second most prestigious children’s hospital, their child has stumped the most intelligent and experienced minds in pediatric medicine.  Hannah has not been able to stop vomiting, and over this past weekend it increased to be vomiting about every hour.  While we worked hard to learn and master the feeding tube and how to handle Hannah’s nutrition in anticipation for being released, Hannah appeared to just get sicker.  It got to the point that she was so weak she could not keep her head up.  At first the doctors just felt like the collapse of her fat pad around her duodenum was so significant that there was a complete blockage except for the Nasal Jejunum tube and that all her bilious and stomach juices were being forced to come back up. The answer was to keep feeding her so that she could gain wait, and re-substantiate the fat pad.  However, the vomiting just continued to get worse.  Having to vomit repeatedly with multiple tubes in your nose is a new form of torture. She di not respond to ANY anti-nausea medication.  In fact, when they offered them to her she chuckled and said, “Don’t bother.”  They tried zophran, promethazine, Compazine and reglan amongst others.  No reprieve.  As one would expect, the more she vomited, the harder her overall health was to manage.  She developed several other issues, including re-feeding syndrome which was serious.  

As her vomiting failed to decrease and she developed other issues, the doctors called a CHOP meeting of the minds, in which several teams met exclusively to discuss Hannah and her complete overview from the moment we walked in the door of CHOP on October 13.  This conversation included the teams from Radiology, Ortho, GI, General Surgery and General Peds.  Based on Hannah and her symptoms, they even reached out to a colleague and specialist at Boston’s Children’s hospital.  They have gotten several x-rays and CT scans of Hannah and from that we have learned a great deal.

SMA syndrome is usually caused by a weight loss which results in loss of the fat pad causing compression of the third part of the duodenum between the SMA and the aorta, leading to obstruction.  All along we have been saying that we are confused because the vomiting started in traction and before the weight loss.  In fact, the vomiting CAUSED the weight loss.  In VERY rare cases, spinal fusion can anatomically cause SMA.  In the case of a fusion, the straightening of the spine can cause an anatomical shift which results in the compression of the duodenum, resulting in the blockage.  However, since Hannah’s fusion was aborted, and the surgery was reversed, removing all screws and rods, they dismissed this as a cause of the SMA.

After being hounded by worried patients (Dr. Jenean!) who did not understand the cause, and kept repeating that the vomiting started in traction along with her decline in health and physical aspect, the doctors took addition scans. 

Much to all of the doctors surprise, the fat pad is not flat.  It is still there.  But there is an anatomical cause to Hannah’s blockage.  One that they admit they have never seen before, and caused by the correction that took place while she was in traction. At this point they cannot get a clear image of exactly what aspect of her insides is pushing on her duodenum causing the blockage- but the one thing that they all agree on now is that feeding her is not going to fix this and her vomiting will not stop until this is fixed surgically. 

Right now in order to keep her comfortable, she has a sump in her stomach.  It works just like a sump pump in your basement, it pumps out her stomach juices and bile so that she does not have to throw it up. It is not always successful, but it has lessened the consistency of her vomiting.  In addition, we are still feeding her through a Nasal Jejunum (NJ) tube.  As you can see from her video, she has a tube in either nostril.  When looking at her, the tube on the right is the sump and the tube on the left is the NJ tube that feeds her. 

Our new surgeon (Dr. Blinman but we have named him Dr. Bibbity-bop) came by and spent a good amount of time with us tonight.  He explained that Hannah needs to have surgery to repair this.  While this news genuinely scared her, she is relieved to hear there is a fix that means she will be able to stop vomiting and feel better. He explained that there are two options for surgery: a mesenteric artery bypass or something called Strong’s procedure.  Strong’s procedure involves dividing the ligament of Treitz to mobilize the duodenum to be positioned to the right of the SMA, avoiding the compression.  At this point the surgeon is planning to talk to all of the MOM team tomorrow, but he is leaning towards the mesenteric artery bypass.  We were relieved to hear how many doctors have been involved in pouring over Hannah and her case the last week and that there will be another special meeting tomorrow about her exclusively including several attending docs from GI, Radiology, General Surgery, Ortho, General Peds and specialists from Boston Childrens.  All of these docs have set aside time for them and their teams to focus on Hannah and the plans for her.  Hannah responded that she hopes they will learn from her case and that there are exceptions to every medical rule and hopefully when the next child comes in with a non-traditional presentation, they will know it is possible and be able to fix it sooner. 

Her surgery is planned for Monday or Tuesday.  We need to spend a few days getting her re-feeding syndrome under control and getting her as nourished as possible to support her for surgery and healing after surgery.

I wanted to let everyone know that Hannah felt it was really important to post on her blog.  She continues to be so appreciative of the love and support that people have offered.  She was self- conscious about her looks, but she decided that it was important enough to let people know how she was – and from her own mouth.  While we have had a lot of dark and sad moments in the last week where all we could do was cry and be angry and frustrated, she continues to rally and dig deep to find her bravery and strength.  At some point, as her mom, I want to tell you all about the thousands upon thousands of ways that this amazing young woman has shown her strength and bravery- but I think we have been able to show you glimpses of it through this blog.  We never could have planned for this nightmare- but we are doing our best to navigate it and get her strong again.  The road ahead of her is long and hard- and we are not naïve to think it will be easy- but we know that the love and support of all of our friends and family has helped and will continue to help as we get her through another major surgery. One that she is petrified of because she is traumatized from waking up from her last surgery and learning that her world had completely changed and was not what it was supposed to be.  She knows better than any of us that one can go to sleep and wake up with nothing being as it should be. 

Our focus right now is on addressing her medical needs and getting her healthy.  After she is able to be medically stable and healthy we will work on her mobility.  As we constantly need to remind each other- one thing at a time.

A special thank you for all the funny videos that were sent to us in the past week.  They gave Hannah just a few moments of smiles and laughter in a really difficult week in which the bad news seemed constant.