Hi Everyone, I was notified as of yesterday that I will be going to NIH for the study of this diseasse. I am looking forward to this but with some worry. For the ones who have been to NIH do you have any advice and what may happen while I am there. Thanks, Annamarie

Annamarie: There is no reason to worry. Indeed, you should consider yourself very lucky, and really look forward to the opportunity. At NIH they will question you, take blood and other samples from you, put various devices on you, take various measurements, and so forth. You'll feel a little like a guinea pig. On the other hand, this may be the first time that a whole medical team that knows a lot about SCLS will take your problems seriously, make a big-picture evaluation of your case, and give you and your own doctor the best advice that is available. And they do that at no cost to you! Thus, you will feel very grateful for the experience, and you will join a distinguished line of SCLS patients who have inconvenienced themselves a little bit in order to learn more about their medical problem -- and to advance medical knowledge of use to all of us SCLS patients from around the world. Arturo SCLS NIH Patient Volunteer #1 PS: You can also do some sightseeing while in Washington.

I have been fortunate to visit NIH on two occaisons. First piece of advice. Please give yourself about 15 minutes to go through security. As this is a Federal Institution, an airport type screening process is used. You will be under the primary care of Dr. Kirk Druey. He is a very compassionate and warm person. He and his staff will provide you with a detailed accounting of what to expect during your time at NIH. You will meet with a wide range of specialists which may include consults and examinations. Dr. Druey and his staff may elect to perform several tests (blood work ups, etc). I was one of the earlier patients who went to NIH, 3 years ago. Since then the prototocols and testing procedures have probably changed. The NIH experience in exhilirating as you will meet some of the best doctors in the world. One other tidbit, internet access was limited (Federal Facility) not certain if they have softened the firewall restrictions. You will be very happy with the NIH experience

I too went to NIH; see comments under discussion topic, "NIH" and my profile. My only "surprise" was to discover there wasn't a private room assigned to me but they were able to accomodate me upon request. It is indeed a great opportunity; I hope you will share your experience and any new insights you get from Dr. Drury about this disease with those of us on this site. Oh, and the food is pretty good, too. Good luck.

Thanks for the support. I did not think that I would be going this quick. My doctor sent info little over a month ago. And I had a power port put in this past Monday which is doing well. When I talked to Laura at NIH she was pleased to hear that I have a port for them to use. I am taking IVIG and it has hepled but still have many problems so I am hoping that NIH will have some answeres on why I have these difficulties with my legs and arms. My surgeon that placed the port is a friend of my doctor who is handling the disease, he now is interested in this disease. I live in a small town and now there is another doctor interested in learning about this disease. I will keep all posted on my trip to NIH. Happy Thanksgiving to All.

Hi Annamarie ! I was at NIH in July & enjoyed my time there. Dr Drury & Celeste were wonderful. They took their time, explained all procedures & were great. Yes, I liked the food as well, especially the shrimp burritos. I flew from Green Bay to Milwaukee to DC by myself. My flights were booked "with assistance" & there was someone meeting me with a wheelchair to help me get to the next flight or to the taxis. I recv'd my first IVIG treatment in the hospital during my 5th leaking episode in 3 months. I'm now trying to get my insurance company to approve the IVIG on a monthly basis, but just getting the phone run around at this time. I had just started a new job at my Dr's clinic, but was let go because I was off too many days while in my probation period. How frustrating ! Keep us updated on your NIH experience & Thanks for taking the time to go ! Dawn - Green Bay, WI

Hi Annamarie ! I was at NIH in July & enjoyed my time there. Dr Drury & Celeste were wonderful. They took their time, explained all procedures & were great. Yes, I liked the food as well, especially the shrimp burritos. I flew from Green Bay to Milwaukee to DC by myself. My flights were booked "with assistance" & there was someone meeting me with a wheelchair to help me get to the next flight or to the taxis. I recv'd my first IVIG treatment in the hospital during my 5th leaking episode in 3 months. I'm now trying to get my insurance company to approve the IVIG on a monthly basis, but just getting the phone run around at this time. I had just started a new job at my Dr's clinic, but was let go because I was off too many days while in my probation period. How frustrating ! Keep us updated on your NIH experience & Thanks for taking the time to go ! Dawn - Green Bay, WI

I do appreciatre everyones input. My husband and I will be driving to MD about a 11 hour drive. We enjoy spending time together traveling. With the way things have been I just dont go out much. To tired and aching to much to go anywhere, This disease has been a huge struggle for me. I was always full of energy and worked 60 hours a week or more. Now I am on medical leave until sometime in Jan. I am praying that things will get better soon. And I want to be apart of helping with science on finding a cure for this disease. If it can't help me maybe it will save someone else from going through this pain and struggle. I have been in the medical field for over 20 years and been on the other side figting for breast cancer patients to have their needs met by insurance. Now I am fighting for myself with insurance companies to have my needs met by this very rare disease Thanks for the support all of you have made me feel so much better about going to NIH. I am looking forward to going.

A couple of more things came to mind; you might want to write down all the questions you have befor hand so you can record the responses, including any your doctor may have had. The current literature doesn't begin to cover what effect your day to day activities may have on SCLS (for example, I wanted to know if alcholhol consumption was a trigger--it wasn't but I went two years with out so much as a beer because my doctors were concerned about CLS and the "blood-brain barrier!") so you may have questions of your own based on your own experience and lifestyle. I also found talking to Dr.s who did the tests informative; Dr. Gorbach is the infra red measurement guru and a facinating individual. Very helpful in sharing results (your and others). Again, good luck.

Thanks I will start to write down all questions I have and will talk to my doctor here. I am dissapointed that when I emailed Laura who sets all this up that I had asked if there was any private rooms She said no that I would have a roomate. This is making it very hard for me to want to go and be a lab rat and have to deal with a roomate. I have always been a private person and even more so since this disease has attacked me. I am thinking about what I should do, I am not happy about having a roomated during such a stressful time with testing and talking with the staff that will be involved. Annamarie

Hi Annamarie, My visit to the NIH was this past March 2011. When my wife and I arrived, we had to complete paperwork and id badge stuff in admissions. Once we were taken up to the floor where I was to stay for the week, we were met there by a nurse who began to explain the process / procedures. She said there were no private rooms, but she was able to accommodate me with a room with out another patient. There were not many patients on the floor during my stay, multiple rooms were empty. This is not a general hospital, any patient that is there was referred and approved to be a part of a study. This place is a huge campus setting with many buildings. They were able to accommodate my wife in the family lodge (nice place). We were warned that we should be prepared to pay for lodging for her at a local hotel if a room in the lodge was not available. Family members of patients who are more critical get priority of the lodge. My stay there was quite pleasant and not difficult at all. I was able to get day passes so my wife and I were able to leave the campus to have lunch or dinner in the local town. We went to meet up with Arturo one day...and had some very good conversation about his experience and knowledge of SCLS. If you get the chance to meet up with him that will be very beneficial. Hopefully this helps with some of your questions/concerns. Please let me know if you have any other questions about my stay there. Glenn

annamarie, my husband was diagnosed with cls about six months ago after probably suffering with it for almost 3 years. its a long story as he has had other problems. it was fortunate we were with a hemotologist for about three to four months and she read a article in the medical journal that suggested my husband, her patient might have the desease. that was in march 2011. we have since seen a immunologist, cardiologist and both are agreeing with our hemo doctor.our paperwork was sent to NHI and we are waiting to hear from them. my question to you is how long did you wait for a reply? what type of doctor are you being treated by? if you have the time please reply as we are trying to be patient. mrs. custom

Hi Custom It took us about a month to get the appt. My Dr. was very proactive in getting me in. He first called NIH and spoke with Dr Durey office. Laura there handles all appts. Ater he talked with them we sent all medical records and cover letter. he had sent another letter 2 weeks later. After that I got a call from Laura to set appt. They were able to set me up for Nov. 28. Because I am on medical leave until mid January. My doctor is a cardiologist he was the one who saved my life in the hospital. And he sent me to Mayo once I revovered from the first attack. I live in a small town and my doctor decided to educate himself on this dissease. I started IVIG in August of this year. There has been some improvement but I suffer from some dissabilities from it. You and your doctors have to be very proactive on this. If there is anything else I can do to help you please let me know. I wish the best fot you both, I will keep all posted on how things go at NIH. Annamarie

What side effects do you have from the IVIG, Annemarie and what do you mean by 'you and your doctors have to be very proactive about this."? I am starting IVIG in 3 days and wish to be as well informed as possible

With IVIG I have had sick to my stomach and headaches. I started with a rash after 1st IVIG, not sure if this is caused by IVIG i will find out more when I am at NIH. After the 2nd treatment my hair has been thinning again not sure if it is coming from this. I do know that I take Tylenol 2 days before treatment and during and 2 days after. This dose help, and I drink plent of fluids while getting the IVIG. Being proactive with my doctors is that I keep well informed of all information on SCLS and make sure my doctors have this info. Most doctors have never heard of SCLS. When I went in for my power port I made sure that the surgeon understood the disease. Because we have low blood preasure and a high pulse. I then talked with the anesthesia team tp make sure they understood and not to load me with fluids. The surgeon gave me Lasiki by IV 2 hours before surgery. My surgery went great and the surgeon did tell me that my BP did start to bottom out but they were finished and was able to wake me quickly. I wear a flash drive bracelet that has all my info on it so if something happens away from home the ER will beable to find out what to do. Bottom line just be as educated about this disease that you can. The more you know the better. My heart doctor who at that time was a friend and he was the one who saved my life on the first attack. The hospital and other doctors were doing the wrong things and was making me worse. He figured out what it was by researching the web that night after the ER. Then he sent me to Mayo to be sure we had the right diagnosis. Oh one more thing when getting IVIG they should start you out at a slow rate and bump it slowly. You will know once you have had your first on wether you need to slow it down more as you go through the months. By the 3rd IVIG I know what rate I should be at because of how I feel. Keep me posted on how things go for you and good luck I am so glad to hear you are able to get IVIG. God Bless

annamarie thank you very much for your reply. my husband's paperwork was submitted about one month ago. I will have his allergist continue responding with NIH. she kind-of-took control after our first visit with her. we were getting frustrated not knowing what could be wrong and tried several specialist before the allergist became involved. she was just so interested in his situation she talked to his hemotologist and they started brainstornimg together. she called mayo then was referred to NIH. she has gone far and surpassed her involvement in this disease. we cannot thank her enough. i agree the patient has to be pro-active. we will get in touch with her again on monday and see if she would call and maybe facilitate a quicker response. again thank you. you put a light at the end of this very dark tunnel. mrs. custom

annamarie, my husband was diagnosed with CLS. He had a episode right in the doctor's office. he immediately checked him into Cornell and was given the IVIG treatment. he had slight pressure problems and slight blood problems but all is well. I think the best things was being at the right place at the right time. the doctor's office and treatment immediately. Looking forward to NIH for the research work and am feeling "great" overall. slight headache but just "great". You are right you have to be insistant and aggressive with follow ups and information. Second treatment is January 3. We are optimistic and looking for a somewhat back to normal life. custom(his wife)