Video Transcription By: Lori A. Eley, RN Wife of Physician afflicted with RSD/CRPS Advocate in support of Research and Cure for RSD/CRPS RSD IN CHILDREN Sarah Young Sarah Young: I want to be a normal teenager. TV News narrator: 17 year old Sarah Young's battle with RSD, or, Reflex Sympathetic Dystrophy Syndrome, began when she was hit with a softball. Sarah Young's Mother Sarah's mother: It took a while to show up, what it was, after going through multiple doctors who did not... they were clueless. I had a couple tell me to cut her leg off, because her leg would be so blue and ice cold. TV News narrator: RSD develops after the body experiences a trauma. The Sympathetic Nerves, ones that prepare the body to react to emergencies, misfire and send constant pain signals to the brain. Dr. Kirkpatrick: The mechanism by which an injury triggers RSD is unclear. The following animation is intended to give a simplified view of how a relatively minor injury might lead to RSD. Activation of the Sympathetic Nervous System following an injury is part of the fight/flight response to an emergency situation. This response is very important for survival. For example: Firing of the sympathetic nerves following an injury causes the blood vessels in the skin to contract, allowing blood to be shifted to muscle and vital internal organs, which enables the victim to use his muscle to get up and escape from danger. Also, decreased supply of blood to the skin reduces blood loss. Thus one might expect the skin to turn from red, due to inflammation, to pale, due to decreased blood flow following injury. Also, because the Sympathetic Nervous System causes increased metabolism and heat production, there is increased sweat production to cool the body. Ordinarily, the Sympathetic Nervous System shuts down within minutes to hours after an injury. The Sympathetic Nervous System exerts control not only of the injured part, but of other parts of the body. Early in the course of RSD, this Regional and Central control is lost and there is increased blood flow to the injured area and other parts of the body. For reasons we do not understand, individuals who develop RSD the Sympathetic Nervous System appears to assume an abnormal function. The sympathetic outflow appears to cause pain by directly stimulating receptors on pain fibers. These events are believed to produce more pain, leading to more stimulation of the Sympathetic Nervous System, which triggers another response establishing a vicious cycle of pain. Thus, we might see changes over time directly attributable to a abnormal function of the Sympathetic Nervous System. These changes include: changes in skin temperature, changes in the color of the skin, red or bluish discoloration, changes in sweating, changes in goose flesh or Piloerection of the skin, and swelling. Most patients with RSD have more pain expected for the type of injury they have suffered. They usually have swelling and discoloration early in the course of the disease. Objective Findings Dr. Kirkpatrick: There are two important things to remember about the objective findings sometimes seen with RSD. First, none of these objective findings for RSD might be present in a patient with RSD, especially during the early stages of the disease. Second, these objective findings tend to be labile, meaning that they may come and go over time. Movement Disorder These abnormal changes in the Sympathetic Nervous System seem to be responsible in some patients for constant pain signals to the brain. Abnormal function of the Sympathetic Nervous System leads to Movement Disorder. Pain is not the only reason why patients have difficulty moving. Patients state that their muscles feel stiff and that they have difficulty initiating movement. Sometimes blocking the Sympathetic Nervous System with local anesthetics injected near sympathetic nerves can lead to a prolonged reversal of this abnormal sympathetic function, resulting in improved movement and prolonged relief of pain. We call this Sympathetically Maintained Pain. In other cases the pain returns after a series of sympathetic blocks. In this situation, it might be possible to obtain a permanent relief of pain and improve movement by selectively destroying sympathetic nerves. We call the selective destruction of sympathetic nerves a Sympathectomy. In some patients with RSD, blocking the Sympathetic Nervous System provides little or no relief of pain. We call this Sympathetically Independent Pain (SIP). These patients, nonetheless, might manifest all the findings of abnormal function of the Sympathetic Nervous System previously stated. As RSD progresses over time, especially without treatment, the disease tends to become more sympathetically independent and unresponsive to sympathetic blocks, hence, the importance of early diagnosis and treatment of RSD. However, there are some patients who appear to have RSD that is sympathetically maintained pain (SMP) for a lifetime, and these patients maintain a positive response to sympathetic blocks. RSD can spread RSD can remain localized to one region of the body indefinitely. In other cases, it can spread to other regions of the body spontaneously or by trauma to other regions of the body. For example: In this illustration, RSD has spread spontaneously to the opposite upper extremity and to the lower extremity. RSD of the upper extremity can spread to the face causing problems with hearing and seeing. Also, RSD of the upper extremity is sometimes associated with difficulty in swallowing. RSD of the lower extremities can be associated with bowel and bladder problems. Thus the term Total Body RSD has evolved over the years as we learn more about this disease. Special Considerations In Children Male Narrator: Pediatric patients present unique challenges. For example: Children have not had sufficient time to develop the psychosocial skills necessary to cope with the pain and suffering due to RSD. Also, children generally fear needles more than adults do. Needles are the basic instrument used to perform sympathetic blocks. This fear and anxiety in children leads to a lowering in the children's pain threshold, making nerve blocks and other medical procedures even more painful. Another concern in children relates to cosmetics. Children might have a greater concern for scar formation following surgery. These concerns have led to a great deal of confusion and frustration among children and their families. Although there have been no large-scale studies on the incident of RSD in children, some generalizations can be made about the children who get this condition. Published case studies indicate that the incident of RSD increases dramatically between 9 and 11 years old, and it is found predominantly in young girls. During this program we will learn a great deal about RSD through the voices of children. The two children in this program are typical for children who get RSD. Both are female and both are aged between 10 and 14 years old. These two children were chosen for another important reason. These two cases illustrate that not all RSD is the same. There could be great variability in the clinical course of the disease as well as in the response of the condition to treatment. This variability in RSD has led some to speculate that there might be different subsets of patients with this disease. This videotape has been prepared in consultation with Dr. Anthony Kirkpatrick and Dr. Dennis Bandyk. Now please consider this situation: Lacey Booth A ten-year-old sprains her left ankle. Initially, the pain was confined to the child's left ankle, but over weeks, it spread to involve the entire left lower extremity. Associated with the pain the child reported cold sensation in the leg, whitish to reddish discoloration of the skin and increased sweating in the left foot. Also, the patient complained of increasing sensitivity to light touch causing pain in the effected region of the leg. The patient was evaluated by a Pediatrician, Orthopedist, Rheumatologist and a Neurologist without arriving at a diagnosis. When examined at the University of South Florida, lightly touching or stroking of the skin provoked pain and she was unable to bear weight with the left leg. Lacey was confined to a wheelchair The child was confined to a wheelchair. Using a portable infrared scanner, the child's left lower extremity was profoundly colder than the right by 2° degrees centigrade. Published data and consultant opinions indicated that a series of one to three sympathetic nerve blocks may reduce the risk of progression of RSD. Sympathetic nerve blocks are particularly urgent when the patient is immobilized with pain. In this patient's case the child was unable to bear weight with the left lower extremity and she was confined to a wheelchair. The child became progressively worse, despite administration of pain medications, aggressive Physical Therapy, and treatment by a Psychologist to help her better cope with the pain. The child underwent a series of three lumbar sympathetic blocks spaced approximately 1 week apart. The child made only slight improvement in her condition. At that point a meeting was held with the family and child to discuss the child's treatment options. At that meeting an approach to her treatment was recommended that ultimately led to a nearly full recovery from RSD. Recently, Dr. Kirkpatrick met with the child and mother for a progress report. This interview was not recorded in a studio. In fact, Dr. Kirkpatrick simply placed a camera on the corner of his desk and began recording. What the interview might lack in technical quality is more than made up for in the honest and spontaneous responses of the child and mother. (Interview Begins): Dr. Kirkpatrick: After we finished doing these blocks on you we had a meeting, do you remember, in my office? Lacey: Yes. Dr. Kirkpatrick: Yeah Lacey: Mom lost her keys. Lacey's Mother: I lost my keys. Dr. Kirkpatrick: That's right! That's a good memory, you have a good memory. You remember. That's right. Now, see if you can remember that meeting. What do you think...what was it that I was trying to emphasize? What do you think I was trying to communicate at that meeting? Do you remember? Lacey: No. Dr. Kirkpatrick: You don't remember? Okay. Well, I think one of the things is that your response to the sympathetic blocks was nothing to write your mother about. You seemed like you were doing better, but we weren't really sure and I said it's better not to guess about these things...Let's just say that your response to the blocks was not very good. (Lacey nods her head) Now, you don't remember what I suggested what we needed to emphasize at that point, do you remember what I said we need to work on, some ideas we need to try to develop...do you remember? Lacey and her mother discuss RSD Lacey: You said something about Physical Therapy. Dr. Kirkpatrick: Okay. Lacey: You said something about needles. Dr. Kirkpatrick: Okay. What did I say about Physical Therapy? Do you remember? Lacey: You said I might need to start walking. Dr. Kirkpatrick: Yes, and what else did I say? Lacey: I don't remember. Dr. Kirkpatrick: Okay, alright. I don't know if your mom can remember. (To Lacey's mom) Do you remember some of the ideas that we talked about? Lacey's Mother: I think we talked about her getting in the pool...um...I'm not sure I remember... Dr. Kirkpatrick: Yes, that was an important meeting to me, and so it was an important meeting because I wanted you to get better. And I had a feeling that I knew what was going to be required to make you better. And let me tell you what I recall having said to you and your husband and to Lacey and that's this: It is understandable why you're very reluctant to put weight on that foot. It's very understandable, and your parents needed to understand, because when you hurt with RSD it is like no other pain you can imagine, wouldn't you agree with me? Lacey: Uh huh. (Shakes her head yes) Dr. Kirkpatrick: And when you complain of pain everyone has to respect that. I think your mom remembers me saying that, it ... Lacey's Mother: Uh huh. Dr. Kirkpatrick: ...that it's not that you're not being a big baby or anything like that, but that it hurts, and it hurts like no other pain that anybody can experience, especially a child. And I said that it's normal not to want to move on it, it's normal. It's normal not to put weight on it because, you know, that's how we protect ourselves when we're hurt, right? Lacey: (Nods her head yes) Dr. Kirkpatrick: If we kept stepping on glass on the floor, we would bleed to death, right? So that was very normal for you to do that. But do you remember me saying something to the effect that, something along the lines that... Lacey: It wouldn't hurt you. Dr. Kirkpatrick: Very good Lacey. You do have a good memory. You do have a good memory. Lacey's Mother: You said, To hurt is not to harm. Dr. Kirkpatrick: That's right, we said that. To hurt was not to harm. But, I also said that you shouldn't be forced to do that when you're hurting, that it has to come from you. You have to understand that, and it isn't easy is it? Be honest? Lacey: (Shakes her head no) Dr. Kirkpatrick: It's not easy is it? Lacey: No. Dr. Kirkpatrick: It's not easy because...especially for a child. I mean, we...as adults...we can put our minds and say, I believe Dr. Kirkpatrick, and he's saying it's not going to harm me, it's not going to break my bones and ruin my tissues and everything. I could probably talk you mother into that idea, but try to talk a child into that idea? Wouldn't you agree with me? It's not as easy to do a child? Lacey: (Nods her head yes and smiles) Dr. Kirkpatrick: Because you haven't had a lot of experience in life. I mean like 10 years. You know what I mean? Right? Lacey: (Nods her head yes and smiles) Dr. Kirkpatrick: So, what did you do? I mean, apparently you did something. How did it happen that you started moving? Give me an idea, what happened? Lacey: Well, umm... mom said we were going to move from our house if I didn't get out of the wheelchair, because our house has lots and lots of stairs and I couldn't do that in a wheelchair. So, in the pool, I started walking in a pool. And at first I couldn't do that, and then I could do that. I started walking up the steps in the pool with mom holding on to me. Then me and daddy started walking up the steps in the pool. And then, one day I got out the crutches and tried to walk, try doing one foot first then the other. It wasn't really too bad, but it hurt a lot. So, I went and showed mom. And then when I was in with mom I did something and it made it hurt so bad. You remember that, don't you? Lacey's Mother: Uh huh. (Nods her head yes) Lacey: She was in the bed. She was watching me going around the bed, and I was on the corner of the bed, I'll go, OW! And I guess I hopped a little, because when I go on crutches I kind of hop. So, I guess I hopped a little and that really hurt it, but I just kept doing that, and eventually I got to where I could walk, slowly. Dr. Kirkpatrick: Slowly? Lacey: Um, like just little teeny steps. And I got to where I could walk bigger steps cause I said to myself, Why walk teeny steps cause it's going to take you longer to get there, and it's going to take you longer to get off your feet. So I started walking big steps, and it wasn't really bad. Dr. Kirkpatrick: Uh huh. Lacey: And that's how it happened. Dr. Kirkpatrick: How long ago was it roughly? How long ago was it that you were doing these teeny...because I didn't see any teeny steps today. I didn't see any teeny steps. How long ago was it that you were doing teeny steps, roughly, would you say? Lacey: Um...I don't know. Lacey's Mother: It was in July. Dr. Kirkpatrick: In July? Lacey's Mother: Uh huh. (Nods yes) Dr. Kirkpatrick: So, here we are in October...so, we're talking...3 months ago, roughly? Lacey: (Nods her head yes) Dr. Kirkpatrick: So, you've made some significant improvements since then. Now, how about this. What about...umm...tell me about some of the things you're doing nowadays. I mean, I heard some things about some things that you've tried that are pretty challenging physically, right? Lacey: (Nods her head yes) Dr. Kirkpatrick: Okay. Lacey: Um...me and mom have been riding our bikes a lot. Dr. Kirkpatrick: You...you ride a bike a lot? Lacey makes a full recovery Lacey: We have a long dirt road and it's got a big hill, and I'm the only one that can get up the hill without stopping now, so... And I do a lot of running with my friends. And I do Girl Scouts. And sometimes in Girl Scouts there's stuff where you run and jump, so that kind of helps. Dr. Kirkpatrick: Uh huh. Lacey: And... Lacey's Mother: Jump Roping. Lacey: I jump rope a lot. Dr. Kirkpatrick: Do you? Good for you. (Interview ends) Dr. Kirkpatrick: It is difficult to know exactly how much the recovery in Lacey's case was due to the series of sympathetic blocks, and how much was due to the psychological conditioning that she received. The adage to hurt is not to harm is an important principle, however, this principle should be applied keeping in mind this: First: The cornerstone in the treatment of RSD is normal use. The patient needs to mobilize the effected extremity as much as possible. Second: Often the Physical Therapist will treat the patient with RSD with passive manipulation, causing failure due to extreme pain, even possible further injury. Third: learning the non-protective nature of pain takes time. Fourth: While encouraging the use of the effected extremity is critical and may cause increased pain, it is essential to avoid re-injury. In contrast to the minimal response to sympathetic blocks observed in Lacey's case, the next child illustrates how crucial sympathetic block aid is to the rehabilitation of a child with Reflex Sympathetic Dystrophy. (New case, Amanda) Imagine that your child is a National Champion in Gymnastics, even offered a scholarship to a major University, when, at the age of 10, all that comes to a crashing end due to a gymnastics accident. That is the case of Amanda. Amanda first suffered an injury to her right wrist; it was a minor sprain of the wrist. The RSD spread up her arm to include her shoulder. She required a series of sympathetic blocks. Unfortunately, the sympathetic blocks did not provide her with a permanent remission; therefore, she had to have a Sympathectomy to the right upper extremity. Subsequently, the patient injured her left lower extremity, a simple sprain to the ankle. Again, the symptoms spread up the leg to include the buttock region as well. A series of sympathetic blocks were offered. Again, the patient did not sustain a permanent remission and had to undergo a sympathectomy to the left lower extremity as well. More recently, the patient injured her right lower extremity and required a series of sympathetic blocks. Currently, the patient is undergoing these sympathetic blocks to sustain mobilization of her right lower extremity. (Anatomy of nerves demonstration) Dr. Anthony Kirkpatrick discusses nerve blocks Dr. Kirkpatrick: In order to perform a sympathetic block to the upper extremity, a needle needs to be inserted near the neck in order to block the nerve, the Stellate Ganglia, which sits down, actually, in the chest. For the lower extremity, the sympathetic nerves, again, lie along the spine and it is necessary to insert a needle into the back, and come down and block the nerve in this location here. It is important to recognize that the sympathetic nerve, indicated here by these little red dots, are separated from the nerves that you feel and you move with, your Sensory and Motor nerves. Therefore, it is possible to block the sympathetic nerves without blocking the motor and sensory nerves. Therefore, after a sympathetic block, the patient should experience simply a warming of the extremity and not any change in movement, or in their ability to feel things. (Sympathectomy explained) Dr. Kirkpatrick: Sometimes a series of sympathetic blocks is not sufficient to maintain a permanent improvement in the patient's condition. Under these circumstances a permanent block, or sympathectomy, is necessary. For the upper extremity, one can come in between the ribs with a scope, find the nerve along the spinal column, and remove it. For the lower extremity we have two choices. We can go in with a needle and dissolve the nerve with a chemical called Phenol; or surgically, we can do an excision by going in and actually removing the nerve from the patient's body. Another issue of special concern to children is fear of nerve blocks. In this portion of the interview, Amanda discusses her experience with Intravenous Sedation and nerve blocks. In children, sedation is usually provided during sympathetic blocks. Combinations of Midazolam and Propofol have successfully been employed to relieve anxiety. Unfortunately, larger doses of these agents are required to prevent movement during the procedure. This can leave the child with an unpleasant feeling of a Hang-Over. We have successfully added small doses of Ketamine, which intensifies the sedation, decreases the amount of Midazolam and Propofol required, and thus prevents the feeling of hang-over. In addition, recent studies showed that small doses of Ketamine actually increased breathing, thus increasing the safety margin for deep sedation. Let's hear from Amanda about her experience with deep sedation and nerve blocks. (Interview with Amanda about sedation and blocks begins) Dr. Kirkpatrick: Now, one of the things that you know, again we're dealing with children, is the fear, the fear of having treatments and you've been through a lot, a lot of nerve blocks. I mean, you added them up young lady, we're talking big time. I mean, if you said, I had 50 or 60, I mean, I didn't do all those, but you know, you add them up. There's been a lot of nerve blocks, sympathectomies, upper and lower. Amanda: Yeah. Amanda Alberigi and mother discuss RSD Amanda's Mother: Yes, Right. Dr. Kirkpatrick: I mean, there's been a lot going on here. I'm just wondering do the nerve blocks scare you? Amanda: No. The first time maybe, yeah, but... Dr. Kirkpatrick: After a while... Amanda: I trust you. Dr. Kirkpatrick: Tell us, not about the trust; tell us about the block. You know, when we treat you we use a medication. We don't use it alone; we use it with a sedative, okay? Amanda: Uh huh. Dr. Kirkpatrick: Versed, Midazolam... Amanda's Mother: Versed. Dr. Kirkpatrick: And we use Ketamine. And the reason we use Ketamine is because it helps keep you breathing. And one thing we know about children, young people, they need a lot of oxygen. And I mean they burn oxygen like crazy and if they don't get oxygen, they go blue real fast, you know? So, Ketamine is the drug we use. Now, when we give you Ketamine is there anything about it that you, uh...for example: some people dream, some people don't. Do you ever dream with it? Amanda: No. Dr. Kirkpatrick: Do you ever have any...do you recall any...uh...well...not dreams, but just the funny feelings like colors or anything like that? No? Amanda: No. Dr. Kirkpatrick: No? You're just out? Amanda: Out for the count. Dr. Kirkpatrick: You're out for the count. (Interview discussion about sedation and blocks ends) Dr. Kirkpatrick: It is worth emphasizing that Ketamine should never be used alone. It should be used with other sedatives; otherwise the child may awaken in an uncomfortable state. The IV should be inserted in an area other than the procedure room. The procedure room can be a very threatening environment. In addition, parents may unintentionally generate fear in the child if they fear needles themselves. Therefore, it might be best not to have the parent present during the insertion of an IV. Cosmetics, or scar formation after surgery, is another concern to children. Amanda addresses some of these concerns during the interview. (Interview with Amanda about cosmetics/scar formation begins) Dr. Kirkpatrick: Lets talk about another thing that you, only you can...probably more than anybody else I've ever seen...perhaps help understand a concern that children have. And that is, you eventually...after the Phenol didn't take long enough...you ended up getting a surgical, a surgical sympathectomy done. And of course, young people, appropriately fear a scar. I mean, you know, bikini's are sort of like in and that sort of thing. Amanda: (Nods her head yes) Dr. Kirkpatrick: So Amanda, tell me about that. Amanda: My, uh...the scars don't bother me really. I don't even realize they're there sometimes. I mean, the ones that I have under my arm, they're healed pretty well, except the chest tube one. It's still there, but... Dr. Kirkpatrick: It's still there? Amanda: ...and they don't bother me. Dr. Kirkpatrick: Now suppose you want to make an appearance. I'm sure if it was you or some other child, that there's a way you can cosmetically conceal it a little bit. Uh...I've been told, of course I'm no expert at this, but I've been told... like... for example when women have breast implants, that they can put this little tape and stuff on it. Amanda: Uh... that's the most painful thing I've ever had to go through in my life. Amanda's Mother: We've tried it. Dr. Kirkpatrick: Oh, you've tried it. Okay. Amanda: We're not doing that. Dr. Kirkpatrick: Well tell us. What happened? Amanda: I did it with my chest tube one and it hurt. Dr. Kirkpatrick: Oh, it's painful? Really? Amanda: When you have to rip it off it hurts. Yeah, it hurts! Dr. Kirkpatrick: Oh, in other words you put it on temporarily for whatever the... Amanda: Right. 24 hours. Amanda's Mother: Right. It's a patch and you wear it for 24 hours and you have to take it off. It did reduce the swelling and the color, because it was a raised scar... and it healed from the inside out...so it was raised. To where the one on her stomach, which is still very sensitive, we haven't even attempted that. But she kept Vitamin E as well as 50 sun protector, being out in the sun during the summer... Amanda: I show my scars. Amanda's Mother: ...and she doesn't hide them. She doesn't let them bother her. Dr. Kirkpatrick: Yeah, yeah...well let me ask you this now, if I understand, because I've never seen this technique. Amanda: It's called a silicone patch. Dr. Kirkpatrick: That's what it's called, a silicone patch? Amanda: Uh huh. Dr. Kirkpatrick: Now, does it leave like...you put it on ...it has to be on for 24 hours and you take it off? Does it leave something there on your skin? Is that what it's supposed to do? Amanda's Mother: I don't know. Amanda: You have to replace them every 24 hours. Dr. Kirkpatrick: Every 24 hours? Amanda and her Mother together: Yes, yes. Replace them for 30 days. Dr. Kirkpatrick: So, it's not a patch you put on and leave on to cover it up, but when you take it off, that's the final product? Amanda: It reduces the scar more and more. Amanda's Mother: It shrinks it. Dr. Kirkpatrick: Oh. It shrinks the scar. Oh, I see. But what you're saying when you gave it a try just for fun, whatever, it was painful taking it off. Amanda: Yeah. It feels like ripping a band-aid off, just ten times bigger. Dr. Kirkpatrick: Right, right... Amanda's Mother: Because the patch is about that square, 5 by 3 square. Dr. Kirkpatrick: I see. So the Sympathectomy to the left lower extremity was done about 14 months ago... Amanda: No. Amanda's Mother: No, no, April 2nd. Dr. Kirkpatrick: April 2nd... Amanda's Mother: April 2nd, 2001. Seven months ago. Dr. Kirkpatrick: 7 months ago. Now, can you show your scar here so we can see it? Amanda: Yes. Amanda's Mother: Don't show the belly button ring. Dr. Kirkpatrick: Oh yeah. Let me see if we can get a little close-up of that. There we go. Alright. So, that's the scar and you did say that you tried to apply that... Amanda: Not to this one. Amanda's Mother: Just Vitamin E and sunscreen. Dr. Kirkpatrick: Yeah. Without getting too private, is there a...can you show the other one? Amanda: Here's one. Dr. Kirkpatrick: Yeah, let's see. That's only one, there should be three. Amanda: There's one under my breast. Amanda's Mother: But you can't hardly see it. Dr. Kirkpatrick: You don't have to show us the one under ... Amanda's Mother: You can't hardly see them. Dr. Kirkpatrick: You don't even see them. Put your finger on the one there. There's one there. Let me see if I can zoom in on that. Let me go up there. Let me see. There's one up there; that little guy. Amanda's Mother: That's where the chest tube was. Dr. Kirkpatrick: That's where the chest tube was... Amanda: ...and then there's...you can't see the other ones... Amanda's Mother: No it's... Amanda: You can't even see it. Amanda Mother: No, it's...you can't even see it. Dr. Kirkpatrick: So she's only left with 2 scars. Well, that's interesting. So, the one that leaves most of the scar appearance is really the one that is... Amanda: On my stomach... Dr. Kirkpatrick: On your stomach, yeah. So that's important you know. I mean, that scar appearance is really the one that is... Amanda: On my stomach. Dr. Kirkpatrick: On your stomach, yeah. So, that's important you know. I mean that we recognize and be able to tell young people, Hey, if you're going to have a surgical sympathectomy, you're going to get into something a little more substantial. Amanda: Uh huh. Dr. Kirkpatrick: Now tell us this, you know you've had both. When you look back at your recovery from the two, which one was the more difficult one after surgery to deal with, would you say? Amanda: The one in the upper area. Dr. Kirkpatrick: It was? Why is that? Amanda: Because I was in ICU for 24 hours with a chest tube. Dr. Kirkpatrick: With a chest tube? Amanda: And that was rough. Dr. Kirkpatrick: That was rough? Amanda: That was very rough. Dr. Kirkpatrick: And they gave you pain medicine for that? Amanda: They gave me Morphine, but I didn't want it anymore. So, I was just taking Tylenol. Dr. Kirkpatrick: Yeah, because it makes you kind of dopey? Is that the reason? Amanda: No. It made me sick. Dr. Kirkpatrick: It made you sick, like nauseated? Amanda: Oh yeah. Dr. Kirkpatrick: Yeah, um...but... well of course, we all know that for the lower one...for the lower one...on the lower part...that one, you were out the next day I believe, out the door. Amanda: Uh huh. Dr. Kirkpatrick: You know, this is kind of interesting, because you know what? In adults, it's the other way around. Amanda's Mother: That's what Dr. Bandyk told us. He said, My patients don't usually go home for 4 days. Dr. Kirkpatrick: Yeah. They're out of the hospital faster for the upper extremity sympathectomy than for the lower one. And that's a difference, and that's a difference that's very important. Amanda's Mother: We were in the hospital for 5 days for the upper and less than 24 hours for the lower. And she was up walking around the same night that she had the lower one. Dr. Kirkpatrick: Yeah. What I'm planning to do here is, you know, I have the video of that little worm they took out of you. Amanda's Mother: Yes. Dr. Kirkpatrick: Did I send you that video? Amanda's Mother: Yes you have. Amanda: Yes. Laughter occurs when the family learns that Amanda's surgery will be part of the video program Dr. Kirkpatrick: I'm going to try to get that inserted in here so that we can look at it. Now, is there anything else that we could... have we missed anything from the mother's point of view? What do you think? Is there anything else that's important having gone through all of this? Amanda's Mother: Think positive and be strong. Trust your doctor. I mean, they're the only ones that know what's really right. I mean, and if she wasn't comfortable in any of the decisions...I always let her be involved in the decisions, because that's...it was her body, it's her that it's happening to, not me. So, I can't be the one to say, well this is going to fix it, let's do it , and I let it be her decision. So I think it's important she, you know, each child needs to. But they need to know the repercussions of, you said, the scars, you know? Are they so vain they don't want the scars, and do they want to just have the pain? Do they just want to deal with it or do they want to get better. Dr. Kirkpatrick: Yeah. Amanda's Mother: And knowing that this was the best way to get better, we made the decision, and we made a very reasonable decision to have it done. Dr. Kirkpatrick: So Amanda, what's your take on it? Do you agree with that? Amanda is cured of RSD in her right upper extremity by sympathectomy Amanda: I do. You've got to be positive, you can't think negatively. Dr. Kirkpatrick: Yeah. Amanda: You can't think negatively.... (Inaudible) Dr. Kirkpatrick: Right. (Interview with Amanda ends) (Dr. Bandyk discusses sympathectomy) Dr. Dennis Bandyk explains the risks, limitations and potential benefits Dr. Bandyk: It must be emphasized when undertaking more invasive approaches to pain management such as sympathectomy, that the benefits must be carefully weighed against the risks. Recently, we reported on a large series of patients with RSD at the University of South Florida that had undergone sympathectomy. Over 3/4 of the patients had long-term benefits from the procedure. However, other patients did not obtain significant long-term benefits despite very careful selection for sympathectomy. Furthermore, it should be emphasized that there is little published data on sympathectomy in children. Therefore, extra caution is prudent when considering sympathectomy in children until more data becomes available. The selective use of these invasive treatments must be by a very experienced physician. Before considering a sympathectomy, a child should be offered a series of pure sympathetic blocks to help facilitate an informed consent about what to expect from a sympathectomy. An epidural block is not a pure sympathectic block. Before considering an invasive procedure the family should be encouraged to obtain a second opinion to ensure that all lesser invasive therapies have been tried. In Amanda's case, her multiple extremity RSD consistently forced her to be absent from school within one week after each sympathetic block. Pain medications, aggressive physical therapy and extensive psychological counseling all failed to control her RSD symptoms. Now it appears that she has been cured of RSD in both her right upper and left lower extremities following sympathectomy to those regions of her body. Children will fall into several groups. First are those who are permanently cured by treatment. The second is a group of children whose conditions are improved, but then show a high recurrence of RSD. Fortunately each subsequence occurrence of RSD seems less severe. A third and relatively small group of children with RSD get progressively worse despite treatment and require aggressive intervention, even sympathectomy. In this last group of children with the most severe form of RSD, physicians may procrastinate and wait until the skin of the extremity turns from blue to black, indicating irreversible tissue loss, before considering a sympathectomy. Once a disease is allowed to progress to this advanced stage, it may be too late for a sympathectomy to provide any improvement in tissue perfusion, or prevent the need for an amputation. A sympathectomy should be considered before such severe dystrophic changes occur in the tissues of a child with RSD. It is often difficult to know in advance if a child falls into this more severe form of RSD, therefore, vigilance is required in following the clinical course of RSD in children, in order to achieve the best outcome. Recently, the surgical technique for sympathectomy has been modified to minimize scar formation. For example, the need for insertion of a post-operative chest tube is being eliminated in some patients. And the laparoscope is now being used to perform Lumbar sympathectomy of the lower extremity as well. (Dr. Bandyk's discussion ends) (Dr. Kirkpatrick's discussion begins) Dr. Kirkpatrick: Some children with RSD improve with conservative treatment. Indeed, the available pediatric literature supports the notion that a high percentage of children will improve with active mobilization of the affected extremity and psychosocial conditioning alone. There is a definite art to guiding a child through a step-wise return to weight bearing and mobilization of the effected extremities, and for helping them to understand the difference between protective and non-protective pain. As shown in this video program some children benefit significantly from pool exercises. According to a recent study at Harvard Medical School, one needs to remain vigilant for a recurrence when a child goes into remission following a bout with RSD. In that study, recurrent episodes of RSD in children occurred in up to 40% of patients, however, most of these recurrent episodes were milder than the initial episode. A variety of medications are helpful for individual children, but there is no perspective pediatric literature on specific effectiveness of most of these agents for this type of pain found with RSD. Trials of anti-depressants such as Nortriptyline might be useful as adjuncts for sleep in many patients. Anticonvulsants such as Gabapentin are sometimes helpful. Several patients have had good responses to a short course of Prednisone early in a flare-up; others have not. Clearly, further research is needed to determine what is the most effective treatment options for children. Another important point, in several case series, a high percentage of children with RSD have been very involved in sports, dance and gymnastics. Indeed, the thought of gymnastics competition often conjure images of major trauma as shown in these short video clips. It must be emphasized, however, that most children that engage in gymnastics or similar sports do not get RSD. The fact is, RSD can be triggered by a relatively minor injury. We would like to give special thanks to Lacey and Amanda for the significant contribution they've made to educating us about RSD. Male Narrator: Some key points made in this video are worth emphasizing: The pathophysiology of RSD is uncertain. The animation presented during the early part of the video program is at best an over simplification of how RSD develops following injury. In fact, some refer to RSD as a Complex Regional Pain Syndrome to avoid the implication that the disease has to involve a reflex of the Sympathetic Nervous System. These two pediatric cases illustrate that there is great variability in the clinical course of the disease as well as variability in the response to treatment. The importance of time in treating RSD needs to be stressed. Do not expect change overnight. Active participation of the child is crucial. It needs to be emphasized that this video is a case-report about two children who have had to struggle with RSD. The video program is not intended to be a comprehensive review of the treatment options available for children with this disease. The video glossed over fairly quickly medication trials, cognitive and behavioral approaches to pain, which are the meat that help the majority of children with RSD. As with sympathectomy, there are other invasive treatments such as Spinal Cord stimulation that might be applicable to only a small minority of children with RSD. Because everyone is different, the information expressed in this video program cannot and should not be used to diagnose or treat individual health problems in patients with RSD. The treatment must be individualized by a health professional. This video program underwent extensive peer review at the international symposium held at the University of South Florida A 30-minute version of this video was presented and peer-reviewed by an international panel of experts on RSD, at the International Update on RSD/CRPS, held at the University of South Florida. As a result of the comments received by this panel, the video was expanded to 40 minutes in order to cover a broader range of issues in the diagnosis and treatment of RSD. For complete guidelines for the Diagnosis and Management of RSD, please refer to the Clinical Practice Guidelines available on the Internet in English, Spanish and French published by the Reflex Sympathetic Dystrophy Syndrome Association of America and by the International Research Foundation for RSD/CRPS. The Scientific Advisory Committee on RSD wrote these guidelines. We hope you found this overview of RSD in children helpful. HOME  |  MENU  |   DONATE  |   CONTACT US Copyright © 2003 International Research Foundation for RSD/CRPS. All rights reserved. For permission to reprint any information on the website, please contact the Foundation.