Hello, my name is Kristine, my husband was diagnosed with SCLS february 2016, we live in Denmark with our 2 children (6 and 3 years old). We need very much help/ support trying to cope this disease, right now, I fell we are facing more questions than answers (startting up treatment, coping mentally, what to expect and so on...) My husbands episodes (under my profile for a little more details) • First episode was in 2014, he was hospitalized with swelling legs but not diagnosed at the time. • Second episode: Spring 2015. Symptoms: swelling of both arms. After consultning doctor om call, the swelling stopped, no hospitalizing, treatment or diagnose. • Third episode: February 12, 2016. My husband was ill with influenza-A, in few hours this turned in to great stomach pain, pressure drop and myocardial infarction before he was hospitalized. At the hospital har had a second myocardial infarction, multi-organ failure and compartment syndrome i both legs, that required fasciotomies. The surgeons were able to save both legs, but had to remove muscles on the right leg (causing dropfoot). Under suspision for SCLS after 4 days (had the MGUS protein, lowered albumin on 8 g/l. Hematocrit/EVF/HCT on 0,51), but contact to the Mayo-Clinic was first made 3 weeks after, the clinic recommented IVIG 1-2 g/kg. Treatment was started up the following week (Privigen 1 g/kg (60 g), same dosis was given two days in a row). My husband was in ICU for two weeks (in coma for 10 days), in hospital for 6 weeks, followed by rehabilitiation at home. We are very lucky and grateful that he survived. Now after 5 weeks at home he is relearning how to walk with the right leg (dropfoot) in spite of challenges with heavy swelling of this leg. Fortunately all organs er well/normal, he feels however force reduction in both hands and still very thirsty. Before the last episode he was feeling ill (very tired, cognitive problems, problem with the "sleeping" and "unrested" arms) at least since the first episode in 2014 (maybe since 2010 more or less since 2010, when we think about it). Actually he was diagnosed with stress in February 2015 and later on terminated from his job. Might these symptoms be related to SCLS instead and could this be permanent? Treatment (so far): Privigen 30 g each 4-6 week. The next treatment is the 17. of May. The recommend dose of Mayo was unknown to me until yesterday (reading the patient record). Now I fear very much that the suggested dose is NOT sufficient! (My husband weighs about 67 kg.) What are the recommendation of the community? Thank you all for already sharing your history at this community, it had helped me/us a great deal already. If there should be other Scandinavian/ Northern European patients, who wish to make contact, my mail is: arkitekt.maa.loeser@gmail.com. Best of wishes for you all. Kristine

As the wife of a SCLS patient and a mother of a 4 year old my heart hurts for you because I know the fear and anxiety of this disease. My husband also has cardiac involvement. Was it a true heart attack? My husbands labs all read heart attack but they cathed him and he had no blockages. Does your husband have a low EF? My husband takes heart failure medicine but nothing for a heart attack because he actually didn't have one. You are correct he should he getting 2g/kg of weight so 30 is not sufficient. Please contact Dr. Druey at the National Institute of Health (USA) for additional medical support or questions. Although I'm in the states you can email me if you want to talk further as it sounds as though our husbands' cases are similar. b.owens396@gmail.com

Kristine, Welcome to our Community! You have come to the right place for information and support. Please read, and have your husband read, the key pages "Disorder Details" and "Disorder Resources," and then visit some of the topics of relevance in this "Discussion Forum" section. As to your question, we do not know what is the right amount of IVIG that SCLS patients should receive, and in fact we have suspicions, but are not sure, why IVIG usually works to prevent episodes of SCLS. Therefore, for several years now, doctors have prescribed a high dose of 2 gr/kg over two days, which means your husband should be receiving at least 60 gr on Day 1 and 60 gr on Day 2. That dosage was inspired by what patients with other, better-understood diseases receive. However, IVIG is an extremely expensive medication. Therefore, government and private health-insurance plans, and also hospitals and physicians, have a direct or indirect incentive to start or end with lower doses, in case they are sufficient. We also have a few patients that have had trouble tolerating the high dose of IVIG, and therefore they either receive a lower dose or else a half-dose every 2 weeks. To my knowledge, the lowest dosage being given to a small minority of patients in our community is 1 gr/kg over two days, which is the 30 gr that your husband is receiving. And I concur with Warhamer's advice that you request that your husband's principal physician contact Dr. Druey at NIH as soon as possible, to report and document this new case and consult with him on IVIG dosage alternatives and whatever else. Dr. Druey and his team are the only scientists in the world doing research on SCLS, and they are in touch with the world's largest group of patients, including all the patients in this Community. And to my knowledge, no research on SCLS has been conducted at the Mayo Clinic in the past 5 or more years. To conclude, please encourage your husband to join this Community. It is important for his mental health and emotional well-being that he should inform himself as much as possible, and that he should receive our support and contribute to our understanding of this exceedingly rate disease.

Hello all, thank you so much for all your support and good thoughts. It has given us a good starting point. Latest update. We have contacted my husbands two principal physicians, and they have agreed to contact Dr. Druey at NIH this week. Actually it turns out that they have tried getting in contact earlier but with no success (probably wrong email). Our hope is that Dr. Druey will guide our physicians, especially on the matter of dosage af IVIG, which is still to discussion. Our physicians informs us, that until now they are not convinced that we will get something extra, in addition to potential side effects, by going up in the very high dose. They are still under consideration but thinks so far, that 30 g is correct. I really hope everything turns out into our advantage... I will contact you directly, Warhamer, to hear more about your situation. Thank you so much for sharing again, it means a lot. Best to all Kristine

Hi Kristene,I feel compelled to comment since the 3rd episode your husband endured has brought back some rather unsettling memories of my own attack,they are practically identical in every way except the dropfoot occurred to my left side. I consider myself extremely fortunate to still be here. Thus far it has been my only attack(although I admit I am still unsure as to what would constitute a mild attack). For myself I have been on a 4 weekly course of ivig (octagam) which seems to be doing the job. In the matter of footdrop,I have recently undergone a tendon transfer operation which has brought movement back to my foot(I am now able to lift my foot again). It is still in the process of healing but the results have been fantastic. I'm based in the UK and it was pure chance that I spoke to a surgeon with the knowledge to perform the operation (not common knowledge, apparently) if it will assist you I will gladly pass his details to you. I wish you all the best and stay on the ivig.

Status Update. Thank you all very much for your response, it really helped us a lot. Lately is a lot happened, both good and bad. We managed to increase IVIG dose to 60 gr. and Cristianos SCLC doctors have made contact with NIH, where they asked Cristiano, if he wanted to participate in the research by Dr. Drury. He has agreed, but we do not yet knows what it implies, but hope that he can get consultation i DC also. Cristiano is now receiving treatment at a hospital closer to our home every four weeks. For 2 months ago, he had unfortunately an allergic reaction to IVIG, and the subsequent treatment of the reaction with antihistamine (Tavegyl) he received, gave a violent reaction (severe pain). In addition to this his albumin level is very high. The local haematologist is therefore very nervous to be responsible for the further treatment and have contacted Cristianos SCLS doctors. I have informed them that some SCLS patients are receiving 30 gr. every 14th day instead, perhaps it may be an option. Further the treatment was given much faster than normal, perhaps that may be the answer to the allergic reaction, as the treatment for 1 month ago happily went just fine. We are currently awaiting replies. Does anyone know what Albumin level must be when you are in IVIG treatment? - And Greg, thank you very much for your response, sometimes one forgets that you are not alone, it really means a lot. I'm sorry to hear that you have been something similar through, but I am very pleased that your healing is progressing and that your operation has been successful. I'd like to hear more about this operation and whether you have nerve pain in the foot and get medical treatment for pain. Can I contact you directly? Best to all. Kristine

Hello Kristine I would reinforce that the rate of transfusion is critical in some. Our survey, though very small reflects this. A true allergic response would be good to document with tryptase levels. John

Hi, Kristine I'm sorry to hear of the difficulties you're experiencing, I am sure solution can be found,please keep us updated as much as you are able. You can certainly contact me directly if you wish. My email is gregwilkin@hotmail.co.uk As to nerve pain and all that,I would say after the initial operations and subsequent skin grafts,there was significant nerve pain,treated with a dizzying array of pain killers. I would say this lasted approximately 5-6 months. The footdrop situation has taken a significant amount of time since there is some pre-operative preparation required,not to mention 3 months in plaster(making showering something of a challenge). But I have to say it's been worth it,although I have regained some nerve pain whilst it heals properly. I can give you a much more detailed description via email if you wish. Hope to hear from you soon. Take care.