Yesterday was our first appointment with the hand surgeon. He had consulted Elias while he was in the NICU, but we had not seen him now for several months. We explained that there was a new diagnosis, Fanconi Anemia. Right away he perked up and acknowledged knowing exactly what that was. This was comforting and he proved his knowledge to be accurate with some of his questions. He evaluated Elias and watched as he used his hands naturally. He really liked the fact that Elias was able to get his hand to his mouth. After some time evaluating and observing we were sent to get some X-rays. Again he reaffirmed our comfort by acknowledging he will only take the minimum number of x-rays needed and he understood the need to limit Elias exposure to this radiation. We were very grateful not to have to explain this to him. Some of the items he observed are as follows:
- Elias seems to be left hand dominant
- The clubbing of his hands seems to be acting as a "natural rotation" allowing him to get his hand to mouth and head.
- Elias' left hand and arm visibly appear to be larger than the right.
- Elias left Ulna bone has a bow shape to it. This will need to be watched. However the right ulna shows signs of staying straight.
- at this moment in time we should not rush into a decision on what to do just yet.
What all of that means right now is that the doctor has defined his role as a cheerleader and watchdog. As of this moment in time doing the wrist centralization does not seem to be the best option, but doing the thumb pollicization is still a very realistic option. We never even considered the possibility of doing the thumb pollicization without the wrist centralization. He had very sound reasoning behind the suggestion. I will attempt to describe this in a manner that makes since. First, you want to work with what you have. Elias is already having no difficulty reaching his mouth and the top of his head. By centralizing his wrist there is the potential that Elias would begin to have difficulty reaching them. The wrist would essentially be turned away from the point of contact making the reach further away. Since the centralized wrist is not quite as mobile and agile he could loose range. With the way his hand and wrist behave at the moment he would be able to do basic things like feed himself, brush his teeth, comb his hair, etc. By centralizing he could potentially not perform these task as easily. This was certainly an eye opening option for us to consider. We go back in February for a follow up. If we did only the thumb pollicization we would postpone doing this until Elias was about 2 years old. This would allow the hand to grow as it will double in size between now and then. If we had to make a spot decision at this exact moment we would probably go with this option. However there are still some things that could change between now and then. How the ulnas are growing, if the left ulna bowing continues to push further out as it grows, his flexibility in his hands and muscle tone in the joints of his fingers. How his arms and hands change as they grow. This will guide us down the right path. For now, just watching, stretching and developing is the best thing we can do. Hopefully, all of that made sense. This is a very visual topic, so describing this can be a touch tricky. All in All we left the appointment with a sense of relief, confidence and understanding of what was going on. Of all the choices we have to make for Elias, this particular one has the greatest impact on his future. So having gained some insight like we did yesterday certainly took a load off our minds.
After three lovely hours at the doctors office we took a scenic route home in order to avoid the dreaded beltway traffic. Elias was so intensely focused on all the fall colored leaves that passed by the car window. He was in awe and thoroughly enjoying the scenery. Shortly after getting home we had our monthly meeting with the nursing supervisor covering our case. We took this opportunity to really stress our situation, disappointments, and concerns. She was very good at listening to what we had to say and did not patronize us with standard answers. She agreed with much of what we were offering and did return some insight on why certain things were as they appeared. The bottom line of our meeting is that we are entering the system at a very bad time. With budget cuts and nursing shortages, it is becoming increasingly difficult to staff cases. She also informed us that Elias is by far the youngest patient the agency has. The next youngest is 3 years old. So to find a nurse with neonatal nursing experience, has the skill sets required to care for him, and be comfortable with someone as young and small as he is can be difficult. She also mentioned that they are being very selective when it comes to his case because he has far more needs and things going on than any of their other patients. This certainly gave us a better understanding on what was taking so long or why this seemed to be so difficult. She admitted it is true that their is always some growing pains with transition to home nursing, but ours are magnified by Elias complicated diagnoses. The fact that we are now in a sub-contract mode with other agencies and the lack of leads from that avenue does not fair well for us. What this says is it is not an agency problem, this is global no matter which agency we are with. So we are having to accept things as they are for now and pray they get better sooner rather than later. Katharine has a saying she uses all the time, "The acceptance of mediocrity breeds mediocrity." That is the trap we might be in when it comes to nurses. For now we have to temporarily accept this mediocre scenario for hopes it will improve, but it is a vicious cycle that we will continue to fight to break. I guess now it is a battle of wills requiring endurance and patience.