In the hospital today, my husband's doctor has recommended he start using Terbutaline as Theophylline has many side effects. Anything I've read thus far indicates using both of these medications. Am I misreading the info out there? Do you usually start with one, and then switch to the other if it doesn't work? Does anything else need to be taken in addition to Terbutaline, or is it standard to start with just the one medication? Thanks.

I should mention my husband is still in hospital recovering from 4-limb fasciotomies, and his delirium has only recently cleared. He's about to go into an extensive rehabilitation program as an in-patient.

I'm no medical doctor, but this is my understanding from my own experience and that of many other fellow SCLS patients: 1) Most of us don't have a 2nd episode until many weeks or months or even years have passed after the 1st episode, so first of all, don't worry yourselves sick about having to go through what you just went through all over again. Right now, your husband's physical rehabilitation, and your own mental and emotional rehabilitation, should take 1st priority. In many ways, care givers such as yourself suffer as much or more than patients, so feel free to get medical or psychological help for yourself and your children, if you have any, and lean on your family and friends for emotional support. 2) It's not a bad idea to start on one or both of these medications now, while he's still in a hospital setting, under watch. Theophylline tends to be given first, with or without Terbutaline, starting at a low dose to see how the patient reacts. Nothing is written in stone, however. Both medications have side effects, as you will see by searching the Internet, so fasten your seatbelt: Look for an increased heart rate, nervousness, irritability and insomnia as the dosage rises -- and Terbutaline is also prone to induce shaking (in one-third of patients). 3) As the dosage will probably keep getting hiked after you both return home, I advise you to insist that your MD prescribe a tranquilizer for your husband, or at least something to help him fall asleep. In case they don't tell you this, it's best to take these medications in the morning and afternoon -- preferably never in the evening, just like you would never want to take 20 cups of coffee after 8PM. 4) These medications don't always work in preventing the severity or frequency of future episodes, so early detection and better management of any additional episodes that may come along will need to become a priority going forward. Good luck! You deserve to have some after what you've been through.

Thanks Arturo. This information is of great value to me. I guess I feel panicked because we've already been in hospital over two months, and it will likely be another two months before we are out. So I'm worried about another leak while in hospital. I should properly introduce myself. My real name is Liesa Evans, and we've spoken a few times through e-mail when this first happened. I would change my user name to my real one, but I'm not sure if that can be done. I have sought the help of my own physician and will likely request her help in finding me a therapist to talk to. I am a writer, so for now I use writing as my personal therapy. I keep a Facebook Group on my husband's progress called "Jim Evans Recovery Update". I haven't yet put Jim's diagnosis on the Facebook group as I'm a bit worried journalists. I had one contact me in ICU when H1N1 was a possibility so I tend to keep quiet publicly regarding diagnosis. Although I don't think Jim's condition would start any media-storms here locally, I did just complete a two-year Journalism course and have a slight bit more exposure to writers and journalists than others might, although it is a minor exposure as most writers I know are young and just starting out in the field. But perhaps when this has all calmed down, I can find a way to use my writing skills to bring some more attention to SCLS. Even if it's minor in nature, I'm willing to help in any way I can. Thanks for having this community. I'd really be lost right now without it. Liesa

As usual arturo you have done a great job in explaining all of this. You are a wonderful freind to all in need and thankyou for all you do! Just wanted to add that dr greipp had my brother take both meds to begin with like arturo said. Then due to side effects has just taken theophylline for several years. He had a major episode 6 years ago with fasciotomies and was in hospital for a couple months. Was great till last sept when he tried to reduce his theophylline because of the side effects. Bad idea! Had another episode but only a few days in hosp, no coma, no fasciotomies. He is lucky because the theophylline works well for him. He takes it in morning and late afternoon like arturo said and mostly he tolerates it. Every drug has side effect and he has learned that taking the meds and dealing with that is a better choice than having another episode. Its been around for while so probably a fairly safe drug. He has used ambien if needed for sleep. He also talked to a counselor after his first episode just to make sure he was ok after he nearly died You both have been through a lot but you are doing a very good job of being an informed patient advocate. Recovery to begin with it is a lot of baby steps but befor you know he will be up and running. We are cheering for you! Take care. Linda

As always, Arturo provided you with a comprehensive answer to your question. I want to share with you that my physicians started me on a regimen of Terbutaline and Theophylline over one year age. Dr. Greipp confirmed that taking both meds, in concert with each other, was good based on his examination when I was at Mayo. The side effects are usually tolerable. It took about one month to get used to the dosage. Over time my theophylline dosage has been increased. Each person responds differently. Ask your doctor to confirm that monthly blood tests (initially) are done to measure the levels. New research indicates that the normative range is in the high teens. Once stabilized, I now go for a blood work up every three months.

I agree with all, thank you Arturo, and everyone else. I will ask Jim's doctors why we are starting with just one or the other medication instead of both. I suspect it may be because he's in hospital still dealing with secondary infections and more side effects could really complicate things. I've also just found out Terbutaline may not be available in Canada in pill form, and inhaler form is too weak for a dosage, so our choices may become limited. Muara, what are they looking for in your bloodwork? Is it the Hgb levels, the Albumin levels, or both? Thanks.

Liesa, These replies are very good, Arturo's in particular. They show the strengths of the forum. Informing and supporting. If you wish, my office number is 507 319-2904. Leave a number where I can reach you if I am busy when you call. Phil Greipp

Arturo's comment is very accurate. There are differences on how the meds affect us all. And it can get to you at first until you find what works for you. I started with both Terbutaline and 800 mg Theophylline. It started out fine but then I became sleepless and very hard to live with. Dr. Greipp and my family Doctor spoke so we dropped the Terbutaline. Later my Doctor switched from Theophylline to Aminophylline. This has done wonders. The strange thing is that I am now on 1200 mg a day and sleep very well. The weekly leaks have minimized. Dr. Greipp suggested Melatonin (3mg) before bedtime to help with sleep. I only have to use this 1 or 2 times a month. This can be purchased at any pharmecy. Just to give one SCLS point of view. Find what works for you.

My 16 yo daughter has just reported to me that lately she has a little insomnia lasting for 45 mins to1 hour. I am not sure if I should start her on ambien due to her age. She is currently on both terbutaline 5mg 4 x daily , and theophylline 300mg twice daily. Dispite the many attempt by her primary to increase the dose, the therapeutic level for theophylline is still very low. I am so worried for next month. She is starting to show some early symptoms, and very very horrible mood swing @ Clswalt, you mention that your primary switch to aminophylline. Is that due to your insomnia?.

Aminophylline and theophylline are very similar. Please look them up and compare the effects. By accident, the aminophylline provides better sleep habits for me. But like Arturo warns us all, we are not the same in all our reactions to meds. I believe that Terbutaline makes a person a little mean and my sleep habits were better when I quit. For whatever reason, my sleep also improved when my Doctor increased my daily dosage of aminophylline. My breathing also improved. I just returned home 3/22/10 from my 4th major episode where I passed out, rushed by ambulence and then ICU. I returned home in two days. I believe the Aminophylline is doing it's job for me to prevent the critical life risk episodes. I did not take meds for the first two episodes because I was not diagnosed yet. The 3rd and last weekends were as follows: Low blood pressure 60/40, passed out and could not get up, did not come to for over 5 min., regained blood pressure after albumin, and the kidneys did not fail. So I think the meds work. I was wanting to switch back to theophylline, but my Doctor is not sure that it will improve my recent rash of episodes. They are minor, but we are looking at IVIG. It was very difficult watching myself on Mystery Diagnosis on Monday and actually being an ICU patient for CLS the same week. I have planned another trip to visit Dr, Greipp in June. Walt Breidigan

I started my IVIG treatment a week and a half ago (during a major attack while I was already at the ICU) and I've had no minor attacks since (I expect at least one to two per week). I start my regular treatments starting the 31st of this month. My Doctor also said that Anti-TNF Therapy could have an impact on the syndrome and is something to look at in the future.

Jeff I have you on my timeline spreadsheet as having your first episode the same month as mine, Feb 2005. Your reply to history was several ER visits. How many ICU visits have you had? This is strange that you and I had ICU visits the same month again. What meds did you take prior to IVIG and are you taking meds with the IVIG treatment? What kind of syptoms and how frequent have they been lately. If you don't mind, e-mail me at wnbreidigan@verizon.net Walt

The "Mystery Diagnosis" TV show provided an interesting hypothesis for why most of us tend to have just a couple of episodes in the first couple of years, but then many of us transition to having more and more frequent episodes: _The capillary walls get weaker and weaker the more they are shot full of holes._ [Another worrisome thing is the collateral damage caused by each episode to our hearts and kidneys. We have several members of this Community with serious, permanent organ damage caused by the beating that our organs take with each episode of SCLS.] Albumin injections or other medications like steroids may help plug the capillary wall holes that open up during a particular episode, but just like what would happen to auto tires that are punctured again and again, patched-up tires eventually just give out and are not worth repairing. They must be replaced\ with new ones. So, what's the answer? _To move on to a medication that strengthens the capillaries or prevents the episodes that trigger further capillary deterioration._ And from my personal experience and that of more and more patients in this site -- from Europe, Canada and now the USA -- monthly IVIG infusions are the best answer we currently have. So I'm glad Jeff that you finally got your first infusion of IVIG, and I'm sorry it took one more major attack for you to get it. And you, Walt, what are you waiting for?

Walt So sorry to hear about your recent scls attack. I can't imagine how difficult that must have been to watch the mystery diagnosis episode while battling thru another attack. The show is obvioulsy a huge step in getting the word out about scls and we are all grateful to you for voluteering to share your experience. Thankyou so much for doing that. I guess the only silver lining is that people will be educated and lives will be saved and perhaps more research will take place so maybe you ( and everyone else) can find a way to stay episode free. The most important thing is that you are ok. Take care Linda (allens sister)