Our boy is two and half years of age and is an IPEX kid with multiple problems of just about every organ in his body and the dysregulation of his immune system is the main cause of this from the FoxP3 which is needed to produce a special type of T lymphocytes named "regulatory T cells". Without these functioning properly then there is very critical imbalance in the system ultimately causing multiple problems.
How do you control Type 1 Diabetes with a child with IPEX? Meaning there absorption rate on food is different every time he eats as his GI tract does not function correctly.
The second question is who would like to help me with posting specifically about IPEX. So that parents with this disease can converse about the issues that they are having and what we have already experienced with IPEX in direct relationship to diabetes, diabetes control and many other issues. To include the different types of medications the kids are on, outcomes, the issues, and what has each done to try and resolve the issues.
To answer your second question first. I know of no Parent Association that is specifically for IPEX/XLAAD/XPID; but I think that you might find what you need by exploring the Children's Immune Deficiency Foundation at HEALINGKIDS.NET and also the Jeffrey Modell Foundation.
Blood glucose control for your small son must indeed present great difficulties with the triple problems of stress, the enteropathy and the diabetes itself. I assume though that you already have a dietitian and a gastroenterologist to help the diabetes doctor. So that I you have probably already been able to make progress in finding out which foods minimise the diarrhoea, whether what he does absorb is meeting the nutritional requirements for growth and ultimately of course whether intermittent home parenteral nutrition is an option. Also there has probably been a decision on whether the enteropathy needs to be contained with drugs like MMF or Infliximab and Tacrolimus. For the specifically diabetes component my only suggestion would be for a maximally flexible insulin adjustment that is to say, by using bedtime Lantus (insulin glargine) basal insulin and either Humalog or NovoLog immediately after meals. For the best results you will probably have to do more blood sugars and to offset the discomfort of this you might consider the FreeStyle monitor
linked to a Palm Pilot Tracker which could also be programmed for so that you could adjust the meal time insulin dose to the premeal blood sugar and the number of 'carbs' actually consumed
I certainly wish you well in all of this and for encouragement you should ask the hospital's medical librarian to get you a copy of Autoimmune enteropathy in a child: response to infliximab therapy by Vanderhoof JA and Young RJ in the Journal of Pediatric Gastroenterology Vol 34,page 312 in March 2002. Most importantly the 'child' is now 19 and getting better!
[Editor's comment: IPEX is a very rare X linked autoimmune disorder with components of Immunodysregulation, Polyendocrinopathy, And Enteropathy, X-linked. It does not have the same underlying pathology as type 1A (autoimmune) diabetes. See Clinical and molecular features of the immunodysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome at PubMed. WWQ]
Original posting 11 May 2003
Posted to Daily Care
Last Updated: Tuesday April 06, 2010 15:09:44
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