New Information

In my previous post I mentioned that Kai would be heading back to U of M on Friday...our home away from home...

We headed back because of some complications and issues including low oxygen saturation levels (and cyanosis), even after his open heart surgery this past June. Kai’s cardiologist has said that he hasn't encountered his symptoms post-op before, so he had consulted other institutions, such as Boston Children’s Hospital and CHOP, regarding his situation.  Friday's procedure would be diagnostic in nature - to see if there were any abnormal arterial or venous connections. The procedure was also expected to be intervention. The surgeon was expecting to place coils in these, to stop the growth and transportation of blood where it should not go.  All of this is especially important as we were recently told by the Emergency Room doctors in Grand Rapids that Kai was, once again, in heart failure.

Friday’s heart procedure was positive. It clearly showed that the heart defect repair done in June was functioning ideally. While Kai’s heart will never work like a "normal" heart and he will deal with his heart defects his entire life, all of the data collected showed that his heart was functioning as well as can be expected. It also ruled out the heart failure that we were recently told - -most emergency room doctors deal with adults, not children with congenital heart defects, which are vastly different. Most ER doctors deal with issues like chest pain and heart blockage, many do not have experience with complex heart deformities. We believe that the ER diagnosis of heart failure was based on a lack of knowledge of a single ventricle, single atrium heart functioning.

As thrilled as we are that his heart is functioning well, we are equally as devastated about what that means for his lungs. As we met with doctors and surgeons on Friday, we discussed the likelihood that Kai has a serious pulmonary disease.  While we are waiting on his official diagnosis from the pulmonologist, doctors feel that Kai has a lymphatic disease known as Plastic Bronchitis. There is a possibility that he also has a disease called Protein Losing Enteropathy.  Both diseases are directly related to his heart defects; specifically, both diseases develop after the Fontan procedure, one of the surgeries that Kai has had to repair his heart. Plastic Bronchitis is a lymphatic flow disease, meaning that there is abnormal circulation of fluid through the lymph nodes. This causes serious respiratory complications. Children with this disease experience lymph fluid buildup in the respiratory system and airways and forms bronchial “casts”.  These casts can cause life-threatening airway obstructions and cause asphyxia (suffocation).

Here is some of the information that we received from the University of Michigan about these two diseases:

PB is an extremely rare and potentially fatal disease.

Lymphatic flow disorders refer to a group of diseases characterized by abnormal circulation of lymph fluid. Lymph vessels carry lymph fluid to veins, where it returns to the bloodstream, playing a crucial role in immune function and fat and protein transport. (Doctors have told us that this disease results in decreased immunity and makes patients more susceptible to a variety of illnesses, which requires increased hospitalizations).

Very little is known about what causes the two complications known as plastic bronchitis (PB) and protein losing enteropathy (PLE). Both complications are severe and incurable. Someone with PLE loses protein from their blood, which leads to leaky blood vessels and fluid build-up. Patients experience growth problems, blood clotting issues and develop infections and immune abnormalities. People with PB develop large protein casts in their airways, causing breathing difficulties.

Obviously, we will be seeking treatment immediately.  Kai’s has an appointment with the pulmonologist early Monday morning at the University of Michigan. We anticipate that he will undergo extensive testing, including a broncoscopy and lymphangiography.  Because of the rarity of the disease, we have been told that treatment options are limited. We will be working to control symptoms by using medication to help keep airways open, as well as to break apart the bronchial casts. This will lessen the chance of suffocation or choking. We will also be consulting with CHOP (Children’s Hospital of Philadelphia), the premier pediatric pulmonary hospital. CHOP is the only hospital in the country to offer a new surgical treatment called lymphatic duct embolization, which may possibly be an option for Kai. 

At this point, we have definitely not become “experts” on what this means for Kai and our family yet. To be honest, it’s quite overwhelming to consider a new set of complications to consider and treat. But, we absolutely are in awe of Kai's medical team. Not only are they talented medical professionals, but they continue to be wonderful friends. We appreciate prayers for wisdom and guidance as we, along with doctors, begin this new chapter in our journey.