Despite today being a milestone for James, it was a very hard day. I am reposting my Facebook status because I don’t have the energy to write about all of the things that I am feeling right now.
I am sorry to leave everyone in the dark for so long. I am utterly exhausted.
First, along with it being New Year’s Day, today also marks 5 wonderful months with our beautiful boy. That in itself is cause for celebration.
Today has been a hard and emotional day. I will try to use layperson terms to explain what happened.
First, a physiology lesson. The heart is pump. All it does is pump blood in circles through your body – to the lungs to pick up oxygen and then to your body to hand off the oxygen to the tissue. Then back to lungs for more oxygen and round and round. The heart has 2 different sides – the left and the right. The left side sends oxygen rich blood to the body and needs to be high pressure to do so. The right takes the oxygen depleted blood from the body and sends it to the lungs, and this is a low pressure system.
James has a hole between the lower 2 chambers – so blood flows across the 2 chambers. Since the left has high pressure, it pushes blood over to the right side. The right side goes to the lungs. So the lungs are getting all the blood from the body that needs oxygen, but they are also getting extra blood from the left side (this is called shunting). This leads to too much blood in the lungs. This is called overcirculation.
One way the body deals with overcirculation is that the blood vessels in the lungs thicken/become more muscular and contract. This will prevent so much blood from flowing into them. When this thickening leads to increased pressure in the lungs, it is called pulmonary hypertension (high lung blood pressure).
James also has a heart condition called a PDA which is contributing, but I will skip that explanation, as it’s not terribly important to understanding.
Despite being on medications to keep his lungs from being overcirculated, James is still getting too much blood into his lungs, his right heart is now very thick (what was originally called HCM is now thought to be just thickening of the heart muscle because of the shunt), and his pulmonary blood pressure is VERY high. It is actually higher than his systemic blood pressure.
He has developed severe pulmonary hypertension. It is not curable. It can be managed, but it cannot be fixed.
This morning, James went into a hypertensive crisis. His lung blood vessels clamped down, and he could not push blood from his heart into his lungs. His right heart started to fail.
The treatment for this is a drug called nitric oxide – it is an inhaled gas that will dilate the pulmonary vessels. This was started late last night and at max dose, it has had minimal effect. We have also had to significantly increase James’s oxygen support. Ideally, his inspired oxygen levels should be <60%. Last night, he was at 100% (obviously maxed out).
This morning, his condition was critical. He was also anemic. The doctor suggested a drug called Flolan. It is a prostaglandin that will help dilate his blood vessels. Unfortunately, whereas nitric oxide works ONLY in the lungs, Flolan works on both the lung pressures and systemic blood pressure. This combined with
James’s need for a blood transfusion meant that he needed more IV access then his 1 line jugular catheter.
They placed a large catheter with 3 lines into his femoral artery. The procedure went very smoothly and quickly, thankfully.
And now we wait. He is maxed out on his nitric oxide. His oxygen is currently at 80%. This is too high long-term, but we cannot turn him down, as his oxygen saturation is only at 90%. We have a lot of room to go up on the Flolan – but that’s it. Flolan is the last thing short of ECMO (a type of bypass) – which we already know that he is not a candidate for and which we would not choose to do regardless.
I know Jim’s post seemed upbeat, but this is not good news. We are walking a thin line here. It is possible that his pulmonary hypertension is irreversible at this point, and if so, the drugs will only do so much. So now we wait. I am trying to be optimistic, but the prognosis is not good.
Our nurse today has been amazing, and we love her. She was able to get us a big bed again so that I can lie down beside James. Having been unable to hold him since Wednesday morning has been its own sort of torture.
We are facing some hard decisions in the next 48-72 hours if James doesn’t respond the way he needs to. So far, he has responded some, but it isn’t as profound as it needs to be, and I fear from what I am seeing that his pulmonary hypertension is irreversible/permanent and progressive.
Thank you for all of the phone calls, visits, texts, messages, etc. I am here reading them, but I haven’t really been responding. We will try to update as we can.
Love you all, the Ashes