February 19, 2009

On my last update here I mentioned that Sadie had an issue with her ankle and that it turned out to be a fracture. Well, that was quite some time ago (feels like forever) and since then we have had many more issues. I truly dont' know where to start... since then she has been seen in clinic 3 additional times and has be in the ER twice including our visit of almost nine hours yesterday. The problem was that Sadie wasn't getting better, she was getting worse and symptoms were changing. She went from having a fractured ankle to "well, we can't see a healing fracture and after all this time we should, so were not sure why there is inflammation in the bone and we hate to do it, but we need to treat the symptoms and hope it gets better". Treating the symptoms included not wearing the boot (because it never gave her ankle a resting position as her ankle isn't shaped like the rest of us), but also not weight baring at all. The not weight baring was by crutches which created it's own set of problems including issues with her hip and wrist and even back at times. So, a wheelchair was given to us for walks and days long days where I couldnt' really monitor her crutching. The pain continued and there were other problems that had come up - like the fact that every time she took a shower her right leg, from the knee down, would turn bright purple. The coloring would go back to normal in about 30 minutes, but it was odd enough that I took pictures of it. In the midst of all this was a major break down of communication between doctors and patience, I mean major... I found myself back to where I was when she was diagnosed with JRA. I could not seem to get anyone to listen to me, take me serious, call me back, talk to me, but I knew, as did Sadie, that something was not right. The pain she was feeling was not an arthritic pain... it was nothing she was use to feeling. So, fast forward to this last weekend. Sadie's right leg now has started to turn cold from the knee down. If you started touching her and the knee and worked your way down towards her ankle the temperature dramatically dropped and by Tuesday night her leg coloring was almost a grey color. At this point I was extremely concerned about her circulation and although we were suppose to have had a schueled appointment with her rhematologist on the books (per my conversation with her on Friday); that never happened. Wednesday morning I called our family doctor out of desperation for someone to see her... I knew that once someone saw what I saw, they would be concerned too. Rather then seeing her, her family doctor made calls and pretty much got no where as I did. Between the two of us it was decided that if I made it the ER during the day, when all specialists were in house maybe we could get some answers. So, we went to the ER.

We arrived at 12:30 and were in a room very quickly (I hadn't even completed the consent form yet). We were seen by two docs in the ER who were both very concerned with the swelling and the temperature of her leg. The asked orthopedics to come down, they did and they pushed it back to Rhematology. When Rhematology finally got there we went over everything and the two docs said they were pretty sure they knew what was going on, but needed to meet with the Radiologist the following day to further rule out any other issues. They gave Sadie and idea of what they thought it was, but told us to hold off on that until they confirmed today.
Today... here is the issue. Sadie has something called Reflex Neurovasular Dystorphy (RND) here is a bit of information on this:

What is Amplified Musculoskeletal Pain?
Amplified Musculoskeletal Pain or Reflex Neurovascular Dystrophy (RND) is a very painful medical condition. It usually affects a limb (a foot or leg more commonly than a hand or arm), but can cause pain anywhere on the body. Some children have pain all over and a few have intermittent attacks of pain. It has multiple manifestations and each form can be named separately, but herein I will use the term RND to encompass the spectrum of amplified musculoskeletal pain syndromes. The pain these children experience, however, is much more intense than one would normally expect because the pain signal is amplified.

How is the pain signal amplified?
First, look at the figure to see how we normally feel pain. Usually pain is in response to tissue damage, such as stepping on a tack. The damage sends a signal through the pain nerve, to the spinal cord, that then sends the signal up to the brain. The brain then recognizes the signal as being painful.

In RND there is an abnormal short circuit in the spinal cord. Therefore, the pain signal not only travels up to the brain, but also goes to the neurovascular nerves that control blood flow through the blood vessels. These nerves, however, cause the blood vessels to constrict thus decreasing blood flow. The decreased blood flow deprives the skin, muscles, and bones of oxygen and also leads to a build-up of acid waste products such as lactic acid. This lack of oxygen and acid build-up causes pain. This new pain signal also goes across the abnormal reflex and causes a further decrease in blood flow, thus leading to more pain. Thus, the pain is greatly amplified. RND is a very painful condition.

Frequently the part of the body with RND will become cold, blue or purple, and even swollen due to the decreased blood flow. Some children will have a test called a bone scan that may show this decreased blood flow (although this test may also be normal – there is no specific test for RND). Other findings frequently seen are osteoporosis due to a decrease in the calcium from the bones and muscle wasting (atrophy). Rarely, the skin will become thick and waxy, a so-called dystrophic change that gives RND part of its name.

What kind of illness leads to RND?
What can be done to help my child?
Illness is an infrequent cause of RND. Most commonly I have seen it in conjunction with inflammatory illnesses of the musculoskeletal system such as arthritis, tendinitis, or enthesitis. Other illnesses may be the initial cause of pain, most are infections such as mononucleosis, influenza, gastroenteritis, or streptococcus.

Most children will need to have a formal exercise program. It just hurts too much for him or her to do it on his or her own. Most require daily out-patient therapy. A few children require hospitalization. The exercise program lasts 5-6 hours a day and involves physical and occupational therapy as well as pool therapy.

What will my child be doing in the program?
Your child will have an exercise program designed specifically for him or her. It will be intense and will focus on the body areas that are painful or do not function properly. If there are body areas that are painful to touch, these areas are desensitized with rubbing and massage. In the beginning this exercise program will take up to six hours a day. Physical and occupational therapists that are experts in RND direct this therapy. A program of exercises to do at home is also part of the exercise treatment.

How long does the program take?
What is the long-term outcome?
The average is 2 to 3 weeks but an occasional child will require many weeks. It is impossible to predict until we see the rate of progression once in the exercise program. Function comes back first; the pain usually takes longer to decrease. Most children are fully functioning by two weeks and pain-free by four weeks.

There are few studies of long-term outcome. In one study where the children were treatedexercise program, 88% of the children were pain free and fully functioning after an average of five years.

Where we are now:
Okay, so is that a ton of information or what??? So, there is more. The radiologist found what seems to be active arthritis in the joint and that said they can't put Sadie into an intensive thapapy program becasue it could agrevate the arthritis further. Double edge sword if you ask me. So, here is the plan..

Sadie is going to take Prednisone (this is against all my wishes and many, many concerns were brought to the doc by both myself and Sadie). The have given her a very low dose for 10 days to see if it can minimise the swelling and help with pain (this should help both the arthritis pain and the RND pain - although almost all pain she is feeling is likely RND related). She will start moving again, meaning by Monday she needs to be fully walking. They warned this will be very painful for her and she will have struggles with this. She also has a few exercises we will do at home and she will also have to start waking up her blood vessels again by rubbing her leg with wash clothes, etc. On Tuesday we go she her regular Rhematologist where a longer plan will be set forth and in the mean time the referral for the people that deal with RND has been put in so we could hear from them sooner than Tuesday or they may wait to call until after they talk with Dr. W on Tuesday.

That's all I know.. which is A LOT more then I knew when I woke up yesterday and although we would never have wanted any of this I am super glad to have some type of plan of action to getting her well. I have never seen Sadie hurt as much as she has these last few weeks and it has been heartbreaking, sleep disturbing, emotion provoking weeks. I ask that you keep Sadie in your prayers for her recovery, for the treatment program, for the doctors to have the right information at the right time, for healing as we have been very frustrated with the lack of response to legitimate concerns, for peace, and for overall healing of body and spirit