Results

To really explain the results that we received in Cincinnati last week, I really need to start back at this past spring.  In late April, Keyan started suffering from additional abdominal pain.  It was several times a day and we struggled with getting her relief.  Her body was also putting out greater amounts of drainage from her stomach and the stuff that we were getting out changed from her normal pale yellow to sludgy green.  We tried a lot of things and had several conversations with the doctor in Cincinnati.  In July, the GI doctor in Cincinnati asked us to be seen by her local GI doctor because she really wasn't doing well.  That appointment led to her being admitted here in Grand Rapids for testing.  I agreed to admit her for 24 hours with the understanding that they better be doing something….not just observing her.  To my surprise, they were able to coordinate quite a bit of testing over the next 30 hours or so.  Nothing was showing much (typical for Keyan), until we did an upper GI.  The first problem, was upon viewing the live upper GI, Keyan’s stomach had no peristalsis (contractions).  That is not the way your stomach is supposed to behave.  Upon further investigating, we left there with a diagnosis that made some sense but didn’t quite settle right with us.  Regardless, because there was nothing to do about it, we went home having her stay on her right side as much as possible to help her stomach drain and called Cincinnati.

We had a regular check-up with Cinci in August where they discussed with us that while Grand Rapid’s diagnosis was possible, it was not very probable.  It would be on their list of things to look into, but not one of the first 20!  No surprise to us.  At that point in the appointment, the doctor asked to see what was coming our of her stomach into her Ferrell drainage bag.  AS SOON as he saw it, he looked at us and said, “That is bile, which means there is a GI blockage.  We just need to find it.  We can fix this.”  We were relieved and reminded how important it is that they actually “see” Keyan.  We went home and waited for all of the moons to align (read insurance)  so that we could take her back to figure it all out.  She and I headed back down for her to be admitted the third week of October.

Those four days of testing were absolute hell for Keyan.  The testing that they put her through kept her in a constant state of pain and retching.  It was horrible to watch and I felt horrible for agreeing to putting her through it.  In all reality, I was actually very unprepared for what they were going to do and felt like I was being tricked into consenting…I suppose that is a whole different post though!  All along, my only saving grace was the benefit of these tests if they actually found something.  We left the hospital at 8pm when all of the test were done and she had all of her tubes back in place.  They did their best to convince me to stay the night but Keyan and I both needed to get out of the hospital as soon as we could.  She is not an infant any longer. She knows what is happening.  She looks to me to help her and when I have to hold her down through a terrible procedure, it is confusing to her.  When she screams , “no”,over and over again, and I let things continue, she is scarred.  By the time we left, she would not make eye contact with anyone and she had stopped talking.  I knew we were doing emotional damage to her and I was DONE!

It was very clear on my trip home the next day that she had pneumonia from aspirating all of the stuff they put into her stomach.  It was a horrible feeling to hear every one of those coughing spells and to watch her heart rate keep climbing as she was drowning in secretions.  We got her to her wonderful pediatrician when we got home and with the use of strong antibiotics and high doses of steroids, we were able to work at getting her back to her baseline. 

Ten days after we returned home, I received a call from our nurse in Cincinnati.  She called to get an update and to let us know that they had some preliminary test results to discuss with us.  The doctor requested that we come back down so that we could discuss the findings in person.  I scheduled the appointment and hung up the phone.  It was than that I realized I was shaking and having a hard time breathing.  I knew then that he was not bringing us back down to tell us good news.  The next three weeks passed by with this constant weight of worry hanging over our heads.

Our appointment was last Thursday. The doctor, who always has a large group of people with him, walked in alone.  He sat across from us and stated, “This is a conversation amongst friends.  I am not giving a lecture.  I want you to feel comfortable to ask questions and have emotions.”  I found myself holding my breath with the reality that I was right all along….not good news.

• The test showed that her stomach and intestines are grossly abnormal.  The do not work.  They do not digest, they do not absorb well, they do not do anything.  She has what is called neuropathic dysmotility, otherwise known as chronic pseudo obstruction disorder. Fancy words for the GI system does not work.  It is either that the nerves are not getting the right messages from her brain, or the nerves are not giving the right messages to the muscles.  They have no way to determine which it is, just that it involves the nerves.
• The doctors will no longer view her GI tract as a means of nutrition, simply a means of drainage, which it barely does on it own to begin with.  Right now, we are using a bag to drain her stomach, which is keeping her comfortable enough.  That will not always be the case and the doctors began discussions about other ways to drain, none of which are wonderful.
• The doctors will switch from investigative care to comfort and quality of life care.  There is no cure for this disorder.  We can only manage her symptoms and keep her comfortable. We will have additional fluids to run here at home so that she can remain hydrated, and we will use some pain meds to help her through the rough spells.
• All of this leaves her TPN dependent for the rest of her life. TPN is her IV nutrition and it is what will sustain her life.  TPN comes with many complications like septic infections, organ failure, and the risk of not having IV access to get her what she needs.

None of this was shocking to us.  We have lived through eight years watching her GI system digress.  We have been focused on her quality of life for 2 years now.  However, when the best GI team in the country suddenly agrees with you and tells you there is nothing more that can be done to fix this, you suddenly realize how much hope you truly had been holding on to.  I have lived with the understanding that we may not have Keyan with us for as long as we would like, but to be told that this will only get worse was very hard to hear. 

We will continue to pray for a miracle, but it is time that we as a family face the reality that Keyan will only live as long as her body can endure the TPN.  We have no idea how long that will be but it is time that we speak honestly about it. You need to understand that NEVER in 8 years have we been told there is no hope.  This is the first time.  We may have already known that in our hearts, but hearing it and accepting it are very difficult  We are heart broken, angry, in a fog, thankful for the tubes that are keeping her with us, and yet so frustrated by the reality that those tubes bring us.  We are not afraid to talk about it, but we honestly don’t even know what to say.  We have a lot of work in front of us.  We need to meet with her other doctors to see if this information helps answer questions for them.  We need to help our other kids navigate this and give them a better understanding and language for their questions.  I am sure they are feeling confused by our hushed conversations and tears that come too easily.

Again, thanks so much for caring about our girl.  I have always said that while I would never wish our situation on ANYONE, I would wish what we have learned from Keyan on EVERYONE.  We have learned to take nothing for granted.  To embrace all sorts of “normal” and that one little girl can change your life in an instant.