I know it has taken a few weeks to get posted, but there was certainly a lot of information to organize and collect in the process. This is a comprehensive update not only for informational purposes, but for record for ourselves. This is something that may not occur until sometime in the future, if at all, and it is important to be able to recall the things we learned for a decision that will have a profound impact on Elias either way.
As you all are aware Elias is missing the radii bone in both of his arms and has absent thumbs on both hands. Due to this his hands are clubbed. He also has shortened ulnas with the left side actually bowing inward. A few weeks ago we had a wonderful consultation with the orthopedic hand surgeon that works with the FA clinic, Dr. Cornwall. After looking at some films of Elias hands, wrists – or lack there of- and arms he sat down with us and laid out possible scenarios. It was quite an education actually. We certainly identified a few misconceptions we had about this be it self created or implied through our research. He did agree that there is a wide spectrum of opinion that tends to have a pendulum effect among the professionals specializing in these types of hand differences. It seems currently the pendulum is swaying on the more conservative side of less is more. One of the big misconceptions we had in our mind was that the centralization of the wrist meant to straighten the wrist. While this is often the case it seems that straight as you or I envision it is not translated the same in someone like Elias. The other piece of information we found to be interesting is that centralization of the wrist in most cases does have a tendency to digress to some degree and turns back, if you will. As he put this it happens quite frequently. Most surgeons and parents often view this as a failure, but he sees it differently. As with most things it is all in how you look at it. He gave us this example. If a wrist is clubbed 120 degrees and centralized to zero degrees –or “straight” over time that wrist could naturally evolve back, but not necessarily back to the 120 degrees. It may only rotate back to say 60 degrees or half of the original clubbed level. As we said most parents and surgeons would see this as a negative and consider redoing the surgery again, but the same result may or may not be achieved. However, in his mind that specific scenario would be a success because you still gained 60 degrees out of the process. True it is a glass is half full versus half empty thought process, but when it has been seen time and time again that the centralization process is not perfect and often unpredictable if it will work as we envision it because we perceive the outcome to be straightened, then we do have the potential of being disappointed. There is no right or wrong viewpoint, but being prepared helps process how we ultimately feel about it should it not turn out exactly corrected.
On Elias left side he of course has no true wrists, but the hand is actually already centralized on the ulna. His hand is clubbed because of the way his ulna bows inward. It hard to describe, but seeing it explained on an x-ray it makes complete sense. At this point there would not need to be a centralization surgery done. One thing he did recommend was to do a tendon transfer from the bottom to the top pulling the hand upward even with the arm. Right now the tendon is pulling the hand downward. Another possibility in addition to that is a series of surgeries to lengthen and straighten the bowed ulna. The process is very involved and honestly we are not sure how we feel about it. In a nutshell the bone is basically broken and allowed to heal with a small gap in between so that the bone elongates and fuses together. Then the process is repeated again and again until the desired outcome is accomplished. This is not something that will dramatically lengthen his arm, it will be a matter of inches when all is said and done, but it can be a painful and tedious process. That is just not something we want him to endure if at all possible. He has already had enough pain and there is unfortunately more to come. We do not need to add to his list. Still it is a recommended course to increase the function of the hand and arm. Elias arms will be shorter than they should and as he grows that will become more noticeable. However, with the bowing of this arm’s ulna it will make it even shorter and potentially much more debilitated depending on the growth.
Elias right side is a little less complex. That hand would need a centralization because of the way it is positioned. His index digit is actually turning inward and as it was described “auto-pollicization.” This is a positive, according to the hand surgeon, because the ability of the created thumb depends greatly on the digit used, it’s strength, mobility, etc. That sums up the right side, as we said less complex. He is certainly advocating that we do thumb pollicization on both hands. There is not a strong argument to support not doing it. The bottom line is thumbs are needed for certain functions. You can adapt without, yes but there will be limits. To address the concern over timing, again this is a shifting school of thought, but the overall picture shows there is no rush to do this. Elias has enough going on right now, this is not something we need to throw on the plate anytime soon. He has seen children 6 or 7 years old that have adapted as Elias has with the hands and after the thumb pollicization it takes them no time to adjust. His words were, “we simply make their hands better able to perform.”`
Overall it gives us some things to consider and think about, but it was reassuring to hear his opinion that we are not against a ticking time clock to get this done, should we choose to do so. It would certainly not all be done at once of course. Rather, a series of surgeries and in Elias case depending on the route we took it could be at least half a dozen. We are meeting with an Occupational Therapist that specializes in hands next week. The hand surgeon himself recruited her and brought her to Children’s Hospital specifically to work with him and his patients. She will be assessing what things we can do that are proactive to making Elias best prepared for the time we embark on this series of surgeries and optimize the potential for success. Of course we have other more pressing issues that are needing to be addressed so that also relieves the pressure of the timely decision. Regardless of our final decision this appointment really gave us more information to make a truly informed choice and it may even be at a point where Elias himself can be included in the discussion and decision process. As always please feel free to ask us any questions about this or any other aspect of Elias care.