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.

Never a Dull Moment

It’s been a while since we’ve updated publicly about Kai. This is probably because a lot of things have been up in the air since this summer.  More likely, it’s because we’ve taken in so much information that our heads feel like they are spinning. As we have been working as a family to process what we’ve experienced the past year with Kai’s health, and work toward harvesting confusion and pain into wisdom, we’ll be the first to admit that it has been hard to do.  Trying to go on with life, keep track of obligations, and do “normal” things, often leaves little time to make sense of life. There are days when it feels downright overwhelming. Our days are whirlwinds. So, as it has been challenging to work through individually and as a family, it, naturally, is difficult to articulate to others what we’re facing and how we’re feeling. But, as we’re coming up to further medical procedures, I am going to attempt to give it a try. Here is a brief and condensed timeline of our past month or so.

I’ve mentioned previously in posts that Kai has experienced some complications and issues including low oxygen saturation levels (and cyanosis), even after his open heart surgery this past June. We have seen oxygen levels in the high-60s to the mid-90s, and everything in between. This has been something that we have been keeping a watchful eye on ever since. We’ve been especially attentive to this because it’s not common. 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. After consulting, it became increasingly clear that his symptoms were probably not a cardiac issue, but, rather a pulmonary issue. Because of the increasing frequency and duration of symptoms, we had been in contact with doctors throughout October, realizing that it could no longer be “let go”. So, (reluctantly) we scheduled surgery for Friday, November 20.

Because we never have a dull moment in the Timmer home, around the same time that we were trying to wrap our minds around pulmonary/lung issues and what that could possibly entail, Kai began having tachycardia issues. For non-medical readers, basically, he was having an abnormally fast heart rate.  These “episodes” would cause him to have a hard time catching his breath, profuse sweating, and nausea/vomiting.  He would also clutch his chest and say that his “heart was going too fast”. We discussed with the cardiologist different possibilities of problems with the heart’s electrical conduction system. To celebrate, tomorrow is the last day he needs to wear his heart monitor - -it’s been a month of crazy dry, itchy skin (from the “stickies” on his chest) and trying to keep a very curious 3-year-old from trying to push buttons that are attached to him!

As many of you are already aware, Kai ended up in the emergency room on Sunday. Remember what I just said about never having a dull moment?! Because of his heart defects, many people assumed that I had taken him there because of heart issues. Not specifically.

I knew he was sick with some sort of respiratory infection or virus. However, with all of his other health issues, even a common cold takes a huge toll on his little body and has been known to send him to the hospital.  So, respiratory illnesses need to be taken care of right away.  This is especially true if we’re now looking at lung problems. Plus, that morning he oxygen sats were quite low and he was struggling to breathe, even to the point of retracting and nasal flaring. After running some tests, we found out that he had bronchitis.  After being on some strong antibiotics since then, he’s now feeling much better, as far as the bronchitis goes.

Although I was frustrated that he was sick… and we needed to head to the hospital again… and he had to go through tests… and he needed to be on more meds, I realize now that it was probably a blessing in disguise, as we found some further, unexpected heart failure issues. As much as I was devastated Sunday, because really I – he—we did not need one more problem to try to work through, I’m thankful now that we discovered this and can now work toward treating it. So, we’re now managing with meds and working on developing a plan, keeping our scheduled surgery date for next week. Realistically, we know that our options to “fix” this are becoming limited. But, we’re hopeful that he’ll come through this stronger, like he always does. Hey, since Sunday, he’s lost almost 4 pounds of fluid, so that’s a start!

There is no doubt about it, Kai is a fighter. There is nothing –not even heart failure or surgery or a heartrate of 300 beats per minute-that can keep him down for long.  He’s happy, silly, and spunky even in the midst of all of this. So much so, that sometimes we forget how much his little body is going through and how miserable he probably really feels. We often have people say that “he looks so healthy” or “he acts so happy” or “you’d never know that something is wrong.” It’s easy to forget how complex his health issues are because of how full of life he is.  If only looking good was a true indicator of health! Even as parents, now that he’s feeling SO better with antibiotics and the bronchitis is clearing up, it’s hard not to be in denial that there are more concerning underlying issues going on. But, as Chris said tonight, “he looks so much better than he has in the last week, but I’m more worried than ever.”  I can’t imagine what all of this would be like if he didn’t have such an upbeat personality and demeanor!

So, the long and short of our month has involved continued and new cardiac and pulmonary issues.  We’d appreciate your prayers for:

* wisdom for doctors and us, as parents… that we thoughtfully determine the right course of treatment.

*being free from any viruses, infections, etc. so we can just focus on the major cardiac and pulmonary issues AND so that sickness doesn’t interfere with treatment.

*rest…we ALL need sleep.

*peace with considering the possibility of travel.

*Kai’s continued zest for life, in spite of all that he goes through and how he feels, even when it doesn't show on the surface.

Thanks!