Rolf and I had another early start Tuesday morning to make it to UCLA for a 9am appointment with Rudy’s pulmonologist Dr. Pornchai Tirakitsoontorn. We were eager to talk with him and hear his thoughts about Rudy’s heart cath results. Like us, he is surprised by the lack of pulmonary progress which doesn’t leave him feeling very positive. The fact that there hasn’t been any change since January’s cath is not a good sign in his opinion. The lungs can regenerate and see rapid growth in the first two years of life…regeneration can happen up to age nine but at a much slower rate. He is not, however, without hope. He said he had patients in the past who, at age 3 or 4, all of a sudden saw pulmonary growth so he is not giving up hope for potential improvement for Rudy but there isn’t anything we can do to promote that growth. There isn’t a drug he isn’t already on or a lung treatment that will improve the issues of O2 sats and pulmonary pressures.
Since the Glenn is not an option at this time, he is recommending decannulation and would like to start the process of setting up a sleep study at either Chilren’s L.A. or Orange County to make sure Rudy can breathe without the trach overnight before we begin weaning him. Rudy’s ENT, Dr. Shapiro, will also need to weigh in and let us know if the scar tissue in his airway needs to be removed before or after the sleep study. Either way, it will need to be removed before we can wean him so his will be a slow process of decannulation.
After our appointment with Dr. Pornchai, we met with Dr. Rick over coffee (and a diet coke!). He reviewed the cath results and talked with Dr. Dan so he was up to date on the latest. As always, we appreciated his time and found it comforting to talk through the “big picture” with him. He couldn’t offer us any new alternatives or a “plan B” but he was reassuring. At one point I asked him “What do we do, Dr. Rick?” and his reply was “just what you’ve been doing!”. Rudy’s heart surgeon, Dr. Brian, met up with us in the hallway and confirmed that there is nothing surgically he can do for Rudy at this point but that he could put in another shunt if need be. So, we didn’t get news of options for Rudy but we were comforted to touch base with key members of his team. We just kept asking the same questions to everyone hoping someone would give us a different answer…but everyone is in agreement.
I met with Rudy’s SB Endocrinologist today and updated him. At one point he said to me sympathetically…”The heart surgery is no longer an option (That’s right) and you understand what this means (Yes, I do) Well, you are doing remarkably well. Rudy is strong and you continue with his palliative care”. When we talk about keeping it all in perspective, I think this is key to remember because nothing has really changed in Rudy’s condition or care. His treatment plan was never “curative care” – his has always been “palliative” (Medical or comfort care that reduces the severity of a disease or slows its progress rather than providing a cure). I guess “palliative” feels alot more secure and less threatening when there’s a plan in place (i.e. The Glenn procedure) but our goal with or without a plan remains the same…just like Dr. Rick said! We do all we can to keep him healthy, comfortable and happy. And whether we are waiting for that “touch of God” pulmonary healing, new medical advancements or letting go, the reality is God is in control of it all and we’re all in His hands.
When we left yesterday, Dr. Rick walked us to the elevator, gave me a big hug and told me not to worry about Rudy…just love all the kids. It struck me as we were driving home just how good his advice is. When you are stripped of a plan of action, it’s real easy to get caught up in the disappointment and obsess over the lack of a plan…we need to resist this temptation and live life in the moment…loving our kids and believing wholeheartedly that we will navigate through this no matter what. Rolf and I both feel we need to face the harsh reality but do so with deep hope. Whatever will be WILL BE and we have no less control over what is to come than we did yesterday. My mind is adjusting but my heart is slow to follow…our hearts are breaking.
To add to the drama this week, Rudy has discovered how to pull off his oxygen mask and freely demonstrated this new skill throughout the day yesterday!! A little frustrating since we’re dealing with a two-year-old who suddenly has an ornery streak!! We certainly don’t need him sabotaging his own treatment at this point. It was a little frightening to wake up this morning and find he pulled the mask off during the night…we’re not sure how long he was without oxygen but he seemed ok today. When we wean him off the trach, he’ll need to get his oxygen through a nasal cannula…yeah, that will go over real well!!! Ok experienced heart moms…HELP! The crazy boy has and always will keep us on our toes…thank you for your continued prayers. Your prayer support is essential to us and we are grateful!!!