A House with a Weak Foundation

       I’m not even sure where to begin this week’s update.  Khadees remains positive and in good spirits, but we have continued to notice the decline in his overall health and abilities to do daily things.  Most days, he has the most energy in the morning, able to do a few things before the overall exhaustion sets in.  Occasionally, he will have a good day, even though he is exhausted.  However, he is quickly dizzy, tired, and short of breath.  

At his appointment with the transplant team this week, due to the decline Khadees has been feeling at home, they got him in right away for a heart catheterization to see exactly how his heart was doing.  Depending on what the heart cath showed, he would either be transferred to the CVICU as a Status 3 and would remain in the hospital until transplant, or he would be able to be discharged home and continue to wait for transplant at home.  In between his appointment and his heart cath, we both analyzed how he has felt, his vitals, and our overall luck and knew that this would be a flip of the coin, 50/50 chance of either outcome.

This heart cath wasn’t like the others, we both felt extremely nervous.  He wasn’t nervous about the procedure itself, as he has had so many he is a pro now.  But more so, for the outcome.  We were both relieved when the doctor said it was still stable enough to remain at home.  But then that brought further questions, what exactly are our parameters for being worried?  He has days where he is exhausted walking to and from the bathroom, takes 4 hour naps, needs frequent naps throughout the day, can’t concentrate on conversations, and just isn’t himself.  When should we be worried?  

Khadees already has Stage 4 Heart Failure.  Worsening heart failure would just be evidenced by deteriorating vital signs (low MAPs), lethargy, and arrhythmias.  We are thankful for the milrinone (IV medication) running through his veins (helping his heart pump) and the life vest that will hopefully protect him from lethal arrhythmias.  

At our appointment, we were told that the gene mutation Khadees has has a 50% chance of being passed to our children.  His doctor described the mutation as a house built with weak foundation.  The house will stand for so long, but eventually the foundation will crumble, and the house will not be able to stand.  A portion of the heart muscle is weak.  This is why Khadees’s body didn’t respond to most medications they tried in the beginning, and that transplant is the most viable option.  

When we first received the results, we were told that the gene mutation doesn’t normally present until age 10 (Kellan will turn 10 in November, Stella is 6, and Alice is 2).  There are certain lifestyle modifications we can take to prolong this, but if they have this, they will probably be looking at the same prognosis.  He suggested we see a genetic counselor, and the kids will receive further genetic testing.  As you might remember, the kids saw a Pediatric Congenital Cardiologist earlier this summer and all were cleared with healthy hearts.  They will be monitored yearly by cardiology.

Since we were already testing the kids and I’m not sure I can handle any other surprises being thrown my way, I also had Kellan and Stella tested for the Type 1 Diabetes antibodies.  Kellan came back negative, but Stella tested positive for one out of the three they tested for.  From working in the endocrinology clinic, we usually tested for 5.  So for now, I am watching Stella extra close.  (She is not aware of the positive antibodies or what this potentially means.)

We are hopeful that Khadees will get his call soon.  The doctors on his team are hopeful and we continue to pray daily for our family, as well as the donor’s family.  We know that post-transplant will present a new set of challenges, but also more hope.  After transplant, they get him up and walking soon after he wakes from anesthesia.  From what I’ve read, many transplant patients do not remember their first few days.  He will take high dose steroids and many immunosuppressant drugs in hopes of his body not rejecting the new heart.  

We are so appreciative of everyone who has supported us through this journey.  The sweet text messages, phone calls, and sweet cards and notes in the mail have kept us all smiling.  So many people have stepped in to help with fixing things in the backyard, visiting Khadees when we’ve had a lapse in coverage, sending us meals, random acts of kindness, gift cards, and donations.  We are lucky to have friends and family that have flown in to see Khadees.  (This week, his childhood friend, Jason came in to see him.)  We are forever thankful to everyone.