Hi, My name is Melanie, I live in Sydney Australia and I have a a 9 year old son who was diagnosed with SCLS in February of this year. My son was completely healthy prior to this admission. His first attack presented early on as normal flu, in fact Ryan tested positive for rhinovirus at the time. He had a runny nose, sore throat and complained of sore legs that he described as not feeling like they were his 2 days before he had his acute episode. During the night, right before he was rushed by ambulance to the hospital he had begun vomited which I assumed was from the virus. I encouraged him to drink water to stay hydrated, which Ryan was only happy to do, it seemed that he couldn't get enough, nor could he keep it down. At 8am we were talking in bed he seemed fine, but tired from vomiting all night. He complained of being hot, but when I tested his temperature he was low at 34.8. I had never dealt with a low temp, so I decided to take him to the doctor which was only a 10minute drive. Ryan walked into the surgery and collapsed, his blood pressure was too low for their machines to get a reading, so an ambulance was called. At the hospital they could not access a vein, his blood pressure was approximately 70/36, he was transferred to ICU and later diagnosed as having SCLS. He survived this severe attack but has been unwell every since. He started IVig in March and undergoes treatment every 4 weeks, which I am extremely grateful for, however he does suffer side effects from this treatment such as severe headaches, muscle cramps, bone aches and occassional high blood pressure. He also shows signs of swelling in 4th week before treatment is due again, although he shows no signs of haemoconcentration in blood work which is collected monthly. Basically he is very sick a week out of every month, with occassional swelling throughout every month even though he is undergoing IVig. Occassionally he is given 50mg of prednisolone to ease swelling and cramps, although this only provides temporary relief. We have taken him to hospital because of the pain where the have given oxycontin, which again provides temporary relief. He suffers from pain in legs and stomach cramps, and he has put on 12kg in 7months. Is this normal for SCLS sufferers? During the last month Ryan complained of similar symptoms in the 4 week, when he had his blood collected it did show elevated haemoconcentration. His team of doctors have now reviewed Ryan's treatment, he is having a port put in this week and will undergo treatment every 3 weeks. Could Ryan be a chronic sufferer? Can I expect him to feel better if he has his treatment every 3 weeks? Is the port the best way to go? Does anybody else have similar symptoms? There is another little girl who has this disease being treated by my doctors, she apparently has no other symptoms. I feel like a crazy mother, but I know my son is not making up his symptoms. My child is not who he use to be, he was happy go lucky, and the youngest of 3 boys. He is not prone to wingeing and was very active until this year. We do fun things as a family so that his mind is not focused on being sick, but even then he will bow out because he isn't feeling well. Why has he always got some form of pain? Is this normal? Please comment with your experiences, and if there is anything else I can try, or any questions I should ask his doctors. Fairly new to this disease but desperately wanting guidance.

Hi Melanie and Ryan, I can clearly imagine: the experience of à life threatening event and the ongoing problems afterwards makes you desperate, and generate a lot of questions. Within the group on this site, more reports of young children exist. I'm sure some of them will answer you as well. Because of the impact of the disease and due to the small count of known cases in the world, most of us feel lonely, and misunderstood to some extent, especially when the diagnosis is not clear. SCLS is à disease of which à lot is still unknown. So it is hard to tell you what in future will happen. At this moment a therapy that is able to cure the disease is not known. So we have to stick with therapy which suppresses, even the mechanism it not completely understood. So new attacks can be prevented. A lot react very well. But your report of the last week being worse is more often reported, as well as a discussion how many weeks should be scheduled between infusions of IVIG. Once monthly is the most used schedule.(Also dose dependent) Also some report of chronic and sometimes severe changes in energy(as in my case) as well combination with other problems. I think it is wise to put not too much strain on Ryan and to prevent him from being triggered by activities that exceed his energy-level in this period ( even not knowing how long (!!) that can last.As you stated hè has a positive mind. It is still not known if the acute and chronic complaints are due to the same mechanism. The pains can be due to oedema in organs and tissues. Your description of his weight gain and his complaints could be a sign that suppression by IVIG is a partially succes. Other therapeutic possibilities, are mainly a combination terbutaline and theofylline ( both normally used for astma) and some others drugs being used incidentally but IVIG is rather -state of the art- therapy. If it is possible for you to read them, I would advise you the articles meantioned in the "disease resources" capital on this site. Of which the headlines are mentioned there as well on the site. A lot of questions may follow. I think you should discuss them one by one with your doctors as well as on this site. I hope it is a start for you... Hiltjo

Hello Melanie, I am sorry that your son is dealing with this awful disease. I am not a chronic sufferer, but one that has acute attacks. I am writing to you because you mentioned that your son presents with symptoms on the fourth week after his IVIGG treatment. My doctor has split my monthly treatment into two. I do 30g one treatment and then 35g the following 2 weeks. This has alleviated any headaches and reactions to the IVIGG, and takes less time to administer. It also keeps the medicine constant in my system. IVIGG wears off at a rate of 1/2 per week. This may not be the correct treatment rate and dose for someone with chronic SCLS, but I wanted to let you know that it has been successful in my case and may work for others. A port is a good idea if Ryan's veins are hard to access. You give up certain activities if you have a port, but it provides easy access for the nurses to administer the drug. I opted against getting a port because I have young children and wanted to swim with them ,as well as I teach strength classes and was told I would not be able to lift weights over my head. I have also been told that my veins may be unable to tolerate years of being stuck with a needle, but for now that is the way I am choosing to receive my IVIGG. I hope Ryan finds some relief soon. We are all in this together. My thought and prayers are with Ryan, you, and your family. Cristina

Thank you for replying, it helps to understand different methods with dealing with this enigmatic disease. My wish is for Ryan to live a pain free life. I hope and pray that whatever treatment plan he receives he will be able to do that. I just wish we had answers. Fingers crossed. Best of luck to you all!

Hello Melanie. I'm so sorry to hear that your son has this awful disease, and especially at such a young age. Poor little guy. My husband (who is 46) has experienced many of the symptoms and side effects you describe. His first acute episode went very far because like you, we didn't know that anything serious was going on. We just thought he had a really bad cold (which was identified later in ICU as Human Metapneumovirus). Unfortunately, he also had SCLS. :( The disassociated feeling my husband had in his legs & feet during his first episode was the result of severe loss of circulation due to the capillary leakage that we didn't know was happening. Because no one knew he had SCLS, he was treated with an aggressive 18 liters of fluid to raise his very low blood pressure, which resulted in those fluids leaking into the compartments in his body, and causing severe compartment syndrome in all four limbs. It has left him disabled (reliant on crutches), and with permanent and severe nerve damage in his arms and legs that still causes pain today, three years later. My husband takes monthly IVIG, but is one of the rare cases that still experiences frequent acute SCLS episodes about every three months. He also has severe reactions to his IVIG treatment including extreme malaise and exhaustion, severe headaches, muscle cramps and spasms, and occasional bursts of elevated blood pressure and spikes in his heart rate. Like your son, he is sick a minimum of 1 week per month (but often more than that). To reduce the side effects of IVIG, the nurses give him the transfusion over 2 days, and never allow the IV to run faster than 250 ml/hour. So, he goes in for a six hour transfusion on day one, and then goes back on day two for a second six hour transfusion. He is also pretreated with a steroid and Benedryl. He still has very bad reactions, but for many others these methods have helped. Between SCLS episodes, my husband does experience some weight gain and gradual bloating that doesn't resolve until after he has been treated for an episode. His feet constantly swell off and on, and he also gets swelling in his neck. I hope that by sharing my experience, I have shown you that you are not a crazy mother, and your son is not making up his symptoms. SCLS has a very wide range of symptoms and triggers. It can vary greatly from patient to patient, so hopefully your doctor doesn't expect your son's experience to be exactly like that other little girl he/she treats. Feel free to let me know if you have any other questions about our experience with SCLS (liesaevans@live.com). I hope your son feels better soon.

Hi Melanie I live in Canberra and also have SCLS. In my case however I am 63. Although I have had the disease for more than 15 years I was only diagnosed around 3 years ago. My doctor and I put in submissions to the NBA and now IVIG is authorised and paid for in the treatment of SCLS by the NBA in Australia. I assume you are receiving the treatment free under this scheme. I have changed my treatment from every 4 weeks to half dose every 2 weeks and this is working much better. I do not suffer the same post treatment headaches etc and I do not suffer leaks between treatments especially in the last week If I can be of any help please let me know. Warren

Hi Melanie, I originally was administered IVIG 2 days every 4 weeks & have switched to 1 day bi-weekly. I feel more stable on this schedule. I have a Power-Port in my chest. This was originally placed b4 the IVIG infusions began because the hospital couldn't find my veins during episodes. With the Power-Port, it is located completely under the skin & I can still swim. It was the "Hickman" port here that I refused because it would be placed in the upper arm & the connectors needed to be taped to the skin. Thinking & praying for you & your son. Dawn