The New York Trip .... part three!

Ok, hopefully the "last of the NY sections" ... hoping to get anything remaining in this last one!

Monday 2nd Nov:

Had the "parent training" session with Melissa Chaikin Kahn and she went over the exercises that I am to be doing at home with Alyssa, to help strengthen her abdominal muscles (which will hopefully also help reduce the gut pain!) and correct her overall posture - as well as help Alyssa with her many sensory issues. Alyssa was less than co-operational, but it still went pretty well. Got lots of "homework" sheets to bring away with me, and reminded (or perhaps ordered, lol!) by Melissa that I was to contact her by e-mail often. Melissa also told me that she expected Alyssa to change pretty quickly so the plan would need to change with her, so was pleased to hear that!

Tuesday 3rd Nov:

Did some last-minute shopping, then headed to the NYU Medical Center to catch up with Dr Axelrod again; and go over everything that had happened, and everyone I'd seen ... to formulate a plan of action for when I got home. We went over the original letter she'd given me as there were a few things that were inaccurate and I felt she needed to know the fine details of Alyssa's history - given she was basing Alyssa's potential diagnosis on it! Dr Axelrod told me she'd managed to contact the genetic research lab and find out if they were prepared to go ahead and start gene searching for CAD (Congenital Autonomic Dysfunction with Universal Pain Loss) which she believes Alyssa has ... and they are happy to proceed, now she has a small group of patients to compare. So Alyssa and I both had blood samples taken, which were sent to the genetics lab for studying and we'll hear in due course how they get on.

The aims:

are obviously to maximise Alyssa's potential by improving her muscle strength and decrease her discomfort, as well as her self-injurious behaviour. And to monitor her for problems which can occur later - such as blood pressure problems, contractures (abnormal shortening of muscle or scar tissue - usually permanent - which results in deformity) as well as the things that the Pain Insensitivity can bring, like fractures which go undetected. Un-noticed fractures can lead to severe bone sepsis (infection), which can be fatal if not found in time.

The plan of action:

* To try a couple of antihistamines which Dr Axelrod has suggested, that we have not tried as yet - which she seems to have had some success with. The one we will be starting with is also good at helping with gut pain, which would obviously be beneficial for Alyssa.

* Doing Sensory Integration Therapy - which I was given by Melissa Chaikin Kahn, who is specialised in this field - and doing the exercises and therapies suggested by her, at home whenever possible

* To attempt to get Alyssa swimming - which will require the aquisition of a wetsuit (which I'm still looking into) that is small enough for her, covers most of her body to protect the open wounds she has, as well as protect her from the water temperature which she obviously hates - because this is the most beneficial exercise for someone with HSAN. Being realistic, it's the ONLY exercise/sport recommended for HSAN patients because it is non-impact and therefore does not put added stress on their joints and limbs.

* To prevent (or at least reduce) Alyssa sitting on the floor, which puts unnecessary pressure on her hyperextensible (very loose/lax) joints and puts them at an abnormal position. As she also does not feel the discomfort and HSAN patients are at an increased likelihood of joint problems, we need to be pro-active in areas such as this.

* To correct her from "weird" positions whenever noted (which we already do, but some places - such as her daycare establishment may not realise the importance of this) and not to allow her to simply sit in awkward positions ... "just because she can"

* To continue to protect her eyes from danger. Because she has no pain perception in her eyes as well as her external body, she is at risk of eye ulcers, corneal abrasions and blindness from dirt/hair etc getting in her eyes. We also need to watch for the "little things" such as too much sunlight, air blowing in her eyes (like on the plane etc) and when playing with sand at daycare etc.

* To continue to monitor the nerve and motor conduction in regards to the Thalidomide - which is accepted as doing more good than harm at this point in time (and that there is no other "safe" alternative to reduce the sweating) - but to bear in mind the problems it can cause, and remember that she may well have to come off it at some point. It is not really a longterm option, but will do for now.

* To always trust my instincts when I am worried something is wrong with her, and never accept an answer of "it will probably be fine" when dealing with medical professionals who are not experienced with the HSAN disorders. To remember that WE are the experts in our HSAN children, and that we know when to be concerned and that we are not being overly paranoid, but simply protecting our children - a lot of times, from themselves! - but in the only way we can

I think you will agree that the trip was very worthwhile, and that it was worth the absolute fortune it cost! ;-) I ended up with an unscheduled (and unbudgeted!) trip to the orthopaedic specialist, but was happy to have Alyssa assessed by him considering he is an expert in the HSAN children and knows what to be concerned by. I also now have to find the cost for a Home Listening Program which Melissa feels will be the most beneficial to Alyssa, as far as the Sensory Integration Therapy is concerned ... so the fundraising will definitely be ongoing.

I have to say though, that the money would be found even if the fundraising didn't manage it. If you can't find the money for your child when it's their health that matters, what's the point in anything? I would happily end up in debt for the rest of my life if I thought that Alyssa was benefitting from it, she's my baby and she's definitely worth it! :-)

Since returning from the NY trip, I have already been back to the paediatric hospital and seen both the pain specialist and dermatologist. The dermatologist is currently hunting down the antihistamine Dr Axelrod recommended and will let me know when she has managed to source it. The pain specialist (specialist consultant in anaesthetics - to give him his proper title!) has agreed that the Gabapentin is no longer effective (which he did think before we went anyway) and we have withdrawn Alyssa from it. With no adverse effects, I'm delighted to say! She had actually gotten it a bit sporadically while we were in the States, just due to the hectic schedule, the adjusting to time difference and the excessive amount of sleeping Alyssa did! And she was no worse ... so I figured it really wasn't doing much now anyway.

We also discussed the TSE (Transcutaneous Spinal Electroanalgesia) machine - which I forgot the charger for when I went to the States - so Alyssa didn't receive it at all, and was not any worse from that aspect either. The pain specialist said he was not surprised to hear it was not having much/any effect now as he has found that this does tend to be the case in chronic pain. Everything works for a little while, then it's time to find something else. So we have also given up the TSE for now ... and she's doing remarkably well! I'm not sure if it's the OT exercises we've been doing which are helping already (although there is already a noticeable difference in her posture) or some other spontaneous reason, but she is doing pretty well at the moment with regards to her guts and is really only obviously painful just at defaecation again now.

Of course *sighs* this automatically means she is scratching more (the gut pain distracts her from the itching to a certain extent) and they are a bit yin/yang in relevance to each other. Whenever one gets better, the other gets worse ... so hopefully the dermatologist can find the new antihistamine for us to try soon ... and even more hopefully, it will work!

Signing off for now ... yet again it's late and I've done hardly anything on my "to-do" list! But at least all you "impatient" folks know how NY went - in detail! ;-)

xx