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 Expert
Posts: 3815
   
Location: The best kept secret in TX | I've tried to post to the boards of the Scleroderma Foundation but feel so out of place.... Hoping I can find someone here...
Has anyone on the boards been diagnosed with Scleroderma or CREST Syndrome?
My two year old daughter has been diagnosed with both Limited & Systemic Scleroderma with CREST Syndrome. She is on a medication that may or may not help with her blood circulation. (sop far it has not and we are almost finished with one bottle with 4 refills left....) Since I know Bear will ask the medication is compounded into a liquid form and is given orally 2x daily with a 5mL dose. Pentoxifylline I think is how the medication is spelled. I was told it would open up her vessels to allow for more blood flow to keep her warmer. This raises her BP and makes her extremely dizzy, clumsy, and exhausted...
My question is: How in the world do you keep warm when you swim?? The daycare called me to say my little one played outside for maybe 10 minutes in 90 degree weather with a sprinkler and had a Raynaud's attack and had to come inside to warm up... She was wearing a surf suit. Long sleeves, water shoes, long pants.... She's going to miss out on a lot if I can't keep her warm this summer while playing outside with water....
Anyone have any suggestions.... I'm at a loss... everything made to keep people warm is either not water proof or doesn't come in toddler sizes....  | |
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 A Somebody to Everybody
Posts: 41354
 
Location: Under The Big Sky Of Texas | IRunOnFaith - 2017-05-17 5:03 PM I've tried to post to the boards of the Scleroderma Foundation but feel so out of place.... Hoping I can find someone here...
Has anyone on the boards been diagnosed with Scleroderma or CREST Syndrome?
My two year old daughter has been diagnosed with both Limited & Systemic Scleroderma with CREST Syndrome. She is on a medication that may or may not help with her blood circulation. (sop far it has not and we are almost finished with one bottle with 4 refills left....) Since I know Bear will ask the medication is compounded into a liquid form and is given orally 2x daily with a 5mL dose. Pentoxifylline I think is how the medication is spelled. I was told it would open up her vessels to allow for more blood flow to keep her warmer. This raises her BP and makes her extremely dizzy, clumsy, and exhausted...
My question is: How in the world do you keep warm when you swim?? The daycare called me to say my little one played outside for maybe 10 minutes in 90 degree weather with a sprinkler and had a Raynaud's attack and had to come inside to warm up... She was wearing a surf suit. Long sleeves, water shoes, long pants.... She's going to miss out on a lot if I can't keep her warm this summer while playing outside with water....
Anyone have any suggestions.... I'm at a loss... everything made to keep people warm is either not water proof or doesn't come in toddler sizes....
I dont know anything about this, but just wanted to offer you and your little girl a bunch of hugs and prayers that this is fixed..   | |
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Expert
Posts: 3815
Location: The best kept secret in TX |
Thank you Roxie! Currently, to my knowledge, there is no cure for Scleroderma, or CREST. I have been raising donations for research tho. I have Faith in my God that his plans for her are greater than any I could imagine. I have Hope that God has already started healing her. And I pray daily for a cure. . | |
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  Fact Checker
Posts: 16575
Location: Displaced Iowegian | I am totally ignorant regarding this syndrome but pray that you get the answers and help that you need.....it sounds like it is going to be a challenge. Hopefully, Scott will see this thread and respond if he has any suggestions. | |
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 Extreme Veteran
Posts: 336
Location: Missouri | I have no answers, but prayers. A friend of mine's sister had Scleroderma. If you would like I can pass along your email to her. She might have some insight or helpful sites, groups, etc. Feel free to pm me. | |
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Expert
Posts: 1409
Location: Oklahoma | WoW! that's so sad! Praying for your little Girl!!! | |
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Extreme Veteran
Posts: 477
Location: Lost in the swamps | I have good little barreling friend that her dad has scleroderma, thickening of the skin and organs.
look up friends of the marquettes in FB. They can get you contact info to the family.
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 BHW Resident Surgeon
Posts: 25352
Location: Bastrop, Texas | I just barely caught a glimpse of this. This is a very rare condition in kids. In fact, I really haven't seen all that many adults with Scleroderma or its variants. As the OP knows, this disease has potential effects on virtually every organ system. I'm not going to go through a detailed discussion about this, because there is a lot of information available....like that support group on FB.
As far as the pentoxifylline goes, my experience goes back over 30 years when the drug was first introduced as a pill for patients with peripheral vascular disease. It was supposed to be particularly good for diabetics with small vessel disease and non healing ulcers. Anyway, it was used for a wide range of vascular diseases, and the results were not really very good at all, in my opinion. As I recall, the way it worked was that it basically made red blood cells more supple and malleable, thus improving their ability to "squeeze by" tiny blockages in arterioles. Great idea.....didn't make much difference, clinically. Now in the cases of Raynaud's phenomenon associated with CREST, that's different. It supposedly works reasonably well for that.
You need to understand that your child's condition is something that 99% of docs will never see. I'm know I haven't. I also know that many patients with Raynaud's disease, either alone or as part of a disease complex like CREST syndrome, often do benefit from a class of drugs called "calcium channel blockers". They basically cause vasodilation, so this is one possibly useful suggestion you might want to look into. The commonest calcium channel blocker used for this is a drug called. "Nifedipine". Another class of vasodilator drugs includes a drug that operates by a different mechanism, "iloprost".
Ask her rheumatologist about those two drugs: nifedipine, and iloprost.
As you know, cold temperatures and emotions can precipitate the painful vasospastic episodes. This might sound extreme, but a wet suit, including gloves, might be options to consider. The combination of a different drug and wet suit just might enable her to enjoy swimming. Some of those wet suits are very cool looking!
Wish I could help more!
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BHW Resident Surgeon
Posts: 25352
Location: Bastrop, Texas | I was talking about a REAL wet suit. I'll betcha anything if you look hard enough, you can have one made for a toddler, including the gloves. Those things also improve buoyancy and they are designed to keep divers warm in freezing water. | |
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Blessed 
Location: Here | | |
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 My Heart Be Happy
Posts: 9159
Location: Arkansas | | |
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Fact Checker
Posts: 16575
Location: Displaced Iowegian | I came back to this thread this morning to bump it up. Hopefully, you will connect with some patients or parents of children afflicted with this same syndrome. Praying for you and your sweet child ......
Edited by NJJ 2017-05-18 9:37 AM
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Expert
Posts: 3815
Location: The best kept secret in TX | Thank you all so much for all the replies and prayers. I feel so out of place and uneducated on those Medical forums.... It makes people not believe you when you can't explain something correctly or can't produce every answer to every question correctly...
Bear, thank you for again, dumbing everything down for me to understand. I had asked a similar question on a forum I was directed to for Scleroderma and was very much confused when trying to read their responses. I got all sorts of questions like: What percentage was this protein, or that antibody, or did they test for this, what test did they use, what did this test read, can you post results, was it a false positive, could it be this or that and honestly.... I have no idea what most of those people were talking about. All I know is the doctor pulled 8 vials of blood and tested her for every auto immune disease you can think of and then repulled blood once she got a positive for Scleroderma and retested. That's all I know. All the symptoms fit. All the things her doctor explained to me fit. My research fits her to a T and I truly believe this is her right diagnosis as much as I don't want it to be. But again, without knowing the process or what exactly the doctor did to get a positive I can't answer most questions on that board. And when I can't people tell me it's a false positive and too go get this test for this disease or that syndrome or this. I don't need that. I just need my question answered lol and apparently I need the answer dumbed down. 
Her Rheumatologist has one other case with a 13 year old girl that is under her care that has Limited Scleroderma. She's never had a patient as young as my little one with both limited symptoms and systemic symptoms.
As far as the suit goes, I thought what I bought her from the store would work since it looked very similar to a wet suit: long sleeves, long pants, soft shell material and shoes. Makes sense she got cold because it wasn't a "real" wetsuit now that you say that Bear. I was a little worried if a "wet suit" wasn't going to keep her warm. Guess I'll be using my sweet google skills to find her a real wetsuit.
I honestly feel like a sitting duck on this medication. I can't find anyone on the boards who takes this and I couldn't find anyone with any other suggestions. I'll have to call her doctor and ask about the two mentioned. She loses her insurance in June so I'm trying to get her in to see the doctor before her plan expires. I am trying to get her on my insurance but I can't afford it and her Rheum doesn't accept my insurance so I will either have to find a Scleroderma specialist who accepts my insurance or pay out of pocket for her current Rheum.
I feel like her doctor gave her so many refills so that she doesn't have to see her as often. I also feel like a sitting duck just waiting for another symptom to show up. It's like Roulette adn I hate it. So far she has the Raynaud's associated with CREST, shortness of breath while playing, esophegeal dysmotility (She chokes a lot when she tries to swollow, no matter how little the bites are), and she has many gastrointestinal problems. I caught her hands the other day on the table and she couldn't stretch her fingers out all the way and cried when I tried to help her. She looked like her hands were arched ready to play a piano. I think I still have a picture. They were red because the blood was starting to return to her hands. She had just had a Ray's attack because she dropped her sandwhich on the floor and Dad wasn't fast enough to get her another....
Thanks for letting me vent guys....  | |
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 Shelter Dog Lover
Posts: 10277
| Many prayers, seeing our children suffer is so hard. I don't know anything about this disease but my niece was born with a very severe debilitating seizure disorder. My sister became a warrior for her daughter and fought any obstacle to give her the best life she could and my niece lived a wonderful life that even the doctors never thought was possible. Over time she became a walking encyclopedia about her daughters condition, she had copies of every medical exam, X-ray. You sound like the same kind of mom and your daughter is going to do better because of it. Hugs and prayers.
Edited by rodeomom3 2017-05-18 9:11 AM
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 Miss Laundry Misshap
Posts: 5271
| Hugs my dear! | |
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BHW Resident Surgeon
Posts: 25352
Location: Bastrop, Texas | IRunOnFaith - 2017-05-18 8:51 AM
Thank you all so much for all the replies and prayers. I feel so out of place and uneducated on those Medical forums.... It makes people not believe you when you can't explain something correctly or can't produce every answer to every question correctly...
Bear, thank you for again, dumbing everything down for me to understand. I had asked a similar question on a forum I was directed to for Scleroderma and was very much confused when trying to read their responses. I got all sorts of questions like: What percentage was this protein, or that antibody, or did they test for this, what test did they use, what did this test read, can you post results, was it a false positive, could it be this or that and honestly.... I have no idea what most of those people were talking about. All I know is the doctor pulled 8 vials of blood and tested her for every auto immune disease you can think of and then repulled blood once she got a positive for Scleroderma and retested. That's all I know. All the symptoms fit. All the things her doctor explained to me fit. My research fits her to a T and I truly believe this is her right diagnosis as much as I don't want it to be. But again, without knowing the process or what exactly the doctor did to get a positive I can't answer most questions on that board. And when I can't people tell me it's a false positive and too go get this test for this disease or that syndrome or this. I don't need that. I just need my question answered lol and apparently I need the answer dumbed down. Her Rheumatologist has one other case with a 13 year old girl that is under her care that has Limited Scleroderma. She's never had a patient as young as my little one with both limited symptoms and systemic symptoms.
As far as the suit goes, I thought what I bought her from the store would work since it looked very similar to a wet suit: long sleeves, long pants, soft shell material and shoes. Makes sense she got cold because it wasn't a "real" wetsuit now that you say that Bear. I was a little worried if a "wet suit" wasn't going to keep her warm. Guess I'll be using my sweet google skills to find her a real wetsuit.
I honestly feel like a sitting duck on this medication. I can't find anyone on the boards who takes this and I couldn't find anyone with any other suggestions. I'll have to call her doctor and ask about the two mentioned. She loses her insurance in June so I'm trying to get her in to see the doctor before her plan expires. I am trying to get her on my insurance but I can't afford it and her Rheum doesn't accept my insurance so I will either have to find a Scleroderma specialist who accepts my insurance or pay out of pocket for her current Rheum.
I feel like her doctor gave her so many refills so that she doesn't have to see her as often. I also feel like a sitting duck just waiting for another symptom to show up. It's like Roulette adn I hate it. So far she has the Raynaud's associated with CREST, shortness of breath while playing, esophegeal dysmotility (She chokes a lot when she tries to swollow, no matter how little the bites are), and she has many gastrointestinal problems. I caught her hands the other day on the table and she couldn't stretch her fingers out all the way and cried when I tried to help her. She looked like her hands were arched ready to play a piano. I think I still have a picture. They were red because the blood was starting to return to her hands. She had just had a Ray's attack because she dropped her sandwhich on the floor and Dad wasn't fast enough to get her another....
Thanks for letting me vent guys....
I don't like to use the term "dumbing down" when explaining things to people, but I suppose we all need to have things "dumbed down" for us at some point or another. Diesel mechanics and electricians always have to dumb things down for me, because I know so little about those things. If some people seem to enjoy talking over your head, then you are better served by avoiding them. One good thing about the internet is things like support groups that can link people with one thing in common, even if it's rare, and it can be an enormous source of ideas and information, as well as emotional support.
As to those medications, I think you should ask your daughter's doctor about adding nifedipine. That might help with her Raynaud's symptoms. You probably already know there are a lot of strategies used to control those awful symptoms. | |
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Extreme Veteran
Posts: 336
Location: Missouri | IRunOnFaith - 2017-05-18 8:51 AM
Thank you all so much for all the replies and prayers. I feel so out of place and uneducated on those Medical forums.... It makes people not believe you when you can't explain something correctly or can't produce every answer to every question correctly...
Bear, thank you for again, dumbing everything down for me to understand. I had asked a similar question on a forum I was directed to for Scleroderma and was very much confused when trying to read their responses. I got all sorts of questions like: What percentage was this protein, or that antibody, or did they test for this, what test did they use, what did this test read, can you post results, was it a false positive, could it be this or that and honestly.... I have no idea what most of those people were talking about. All I know is the doctor pulled 8 vials of blood and tested her for every auto immune disease you can think of and then repulled blood once she got a positive for Scleroderma and retested. That's all I know. All the symptoms fit. All the things her doctor explained to me fit. My research fits her to a T and I truly believe this is her right diagnosis as much as I don't want it to be. But again, without knowing the process or what exactly the doctor did to get a positive I can't answer most questions on that board. And when I can't people tell me it's a false positive and too go get this test for this disease or that syndrome or this. I don't need that. I just need my question answered lol and apparently I need the answer dumbed down. Her Rheumatologist has one other case with a 13 year old girl that is under her care that has Limited Scleroderma. She's never had a patient as young as my little one with both limited symptoms and systemic symptoms.
As far as the suit goes, I thought what I bought her from the store would work since it looked very similar to a wet suit: long sleeves, long pants, soft shell material and shoes. Makes sense she got cold because it wasn't a "real" wetsuit now that you say that Bear. I was a little worried if a "wet suit" wasn't going to keep her warm. Guess I'll be using my sweet google skills to find her a real wetsuit.
I honestly feel like a sitting duck on this medication. I can't find anyone on the boards who takes this and I couldn't find anyone with any other suggestions. I'll have to call her doctor and ask about the two mentioned. She loses her insurance in June so I'm trying to get her in to see the doctor before her plan expires. I am trying to get her on my insurance but I can't afford it and her Rheum doesn't accept my insurance so I will either have to find a Scleroderma specialist who accepts my insurance or pay out of pocket for her current Rheum.
I feel like her doctor gave her so many refills so that she doesn't have to see her as often. I also feel like a sitting duck just waiting for another symptom to show up. It's like Roulette adn I hate it. So far she has the Raynaud's associated with CREST, shortness of breath while playing, esophegeal dysmotility (She chokes a lot when she tries to swollow, no matter how little the bites are), and she has many gastrointestinal problems. I caught her hands the other day on the table and she couldn't stretch her fingers out all the way and cried when I tried to help her. She looked like her hands were arched ready to play a piano. I think I still have a picture. They were red because the blood was starting to return to her hands. She had just had a Ray's attack because she dropped her sandwhich on the floor and Dad wasn't fast enough to get her another....
Thanks for letting me vent guys....
I'm sure you've already started looking, but I just did a quick search on ebay...there are tons of really awesome full body wetsuits in toddler sizes! And not as expensive as I expected. There do seem to be different thicknesses..the couple I looked at talked about level of warmth provided. Lots of hugs momma! | |
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A Somebody to Everybody
Posts: 41354
Location: Under The Big Sky Of Texas | rodeomom3 - 2017-05-18 8:58 AM Many prayers, seeing our children suffer is so hard. I don't know anything about this disease but my niece was born with a very severe debilitating seizure disorder. My sister became a warrior for her daughter and fought any obstacle to give her the best life she could and my niece lived a wonderful life that even the doctors never thought was possible. Over time she became a walking encyclopedia about her daughters condition, she had copies of every medical exam, X-ray. You sound like the same kind of mom and your daughter is going to do better because of it. Hugs and prayers.
Oh I bet she will be the mom that fights any obstacle that gets put out there just like your sister, Tess will be our Warrior Mom for sure for her baby girl.. | |
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A Somebody to Everybody
Posts: 41354
Location: Under The Big Sky Of Texas | dianea - 2017-05-18 10:18 AM IRunOnFaith - 2017-05-18 8:51 AM Thank you all so much for all the replies and prayers. I feel so out of place and uneducated on those Medical forums.... It makes people not believe you when you can't explain something correctly or can't produce every answer to every question correctly...
Bear, thank you for again, dumbing everything down for me to understand. I had asked a similar question on a forum I was directed to for Scleroderma and was very much confused when trying to read their responses. I got all sorts of questions like: What percentage was this protein, or that antibody, or did they test for this, what test did they use, what did this test read, can you post results, was it a false positive, could it be this or that and honestly.... I have no idea what most of those people were talking about. All I know is the doctor pulled 8 vials of blood and tested her for every auto immune disease you can think of and then repulled blood once she got a positive for Scleroderma and retested. That's all I know. All the symptoms fit. All the things her doctor explained to me fit. My research fits her to a T and I truly believe this is her right diagnosis as much as I don't want it to be. But again, without knowing the process or what exactly the doctor did to get a positive I can't answer most questions on that board. And when I can't people tell me it's a false positive and too go get this test for this disease or that syndrome or this. I don't need that. I just need my question answered lol and apparently I need the answer dumbed down.
Her Rheumatologist has one other case with a 13 year old girl that is under her care that has Limited Scleroderma. She's never had a patient as young as my little one with both limited symptoms and systemic symptoms.
As far as the suit goes, I thought what I bought her from the store would work since it looked very similar to a wet suit: long sleeves, long pants, soft shell material and shoes. Makes sense she got cold because it wasn't a "real" wetsuit now that you say that Bear. I was a little worried if a "wet suit" wasn't going to keep her warm. Guess I'll be using my sweet google skills to find her a real wetsuit.
I honestly feel like a sitting duck on this medication. I can't find anyone on the boards who takes this and I couldn't find anyone with any other suggestions. I'll have to call her doctor and ask about the two mentioned. She loses her insurance in June so I'm trying to get her in to see the doctor before her plan expires. I am trying to get her on my insurance but I can't afford it and her Rheum doesn't accept my insurance so I will either have to find a Scleroderma specialist who accepts my insurance or pay out of pocket for her current Rheum.
I feel like her doctor gave her so many refills so that she doesn't have to see her as often. I also feel like a sitting duck just waiting for another symptom to show up. It's like Roulette adn I hate it. So far she has the Raynaud's associated with CREST, shortness of breath while playing, esophegeal dysmotility (She chokes a lot when she tries to swollow, no matter how little the bites are), and she has many gastrointestinal problems. I caught her hands the other day on the table and she couldn't stretch her fingers out all the way and cried when I tried to help her. She looked like her hands were arched ready to play a piano. I think I still have a picture. They were red because the blood was starting to return to her hands. She had just had a Ray's attack because she dropped her sandwhich on the floor and Dad wasn't fast enough to get her another....
Thanks for letting me vent guys.... I'm sure you've already started looking, but I just did a quick search on ebay...there are tons of really awesome full body wetsuits in toddler sizes! And not as expensive as I expected. There do seem to be different thicknesses..the couple I looked at talked about level of warmth provided. Lots of hugs momma!
I just went there too, theres alot on there for the kidos and good prices too...  | |
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Fact Checker
Posts: 16575
Location: Displaced Iowegian |
^^^^^ THIS ...... most of us here know that we can't help you with anything except prayers and good thoughts! There is nothing more heartbreaking than having a sick child and trying to find answers! Just know that you can come here to get whatever support you might need anytime! | |
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