For Question 1 related to the adequacy of the Introductory Text to the Endocrine Disorders other than Diabetes mellitus, the discussion and guidance are still relevant, appropriate, and clear; and I have no suggestions on changes.
For Question 2 related to the adequacy of the Introductory Text specifically for Diabetes Mellitus, additional guidance and consideration of adding a listing for Diabetes Mellitus in adults at least should be considered as below.
Patients, especially with Type I DM at times can have extremely difficult to control blood sugars despite even multiple insulin injections or use of insulin pump Rx and despite compliance with Rx. Some of these patients have been termed patients with “Brittle Diabetes Mellitus,” where their blood sugars may rise into the 300-500mg/dl range or higher and then drop precipitously into the 30-40mg/dl range or lower, such that they have recurrent DKA as well as severe hypoglycemia and hypoglycemic unawareness or neuroglycopenia. Sometimes this can be seen in patients with underlying CKD as well as autonomic neuropathy with gastroparesis, but at other times, no clearly discernable etiology can be found. Even patients, who appear initially to have Type II Diabetes Mellitus, may later develop features typical of Type I DM with need for insulin and develop “Brittle Diabetes Mellitus.”
These patients typically have frequent physician and ER visits or hospitalizations requiring interventions including IV fluids and insulin given parenterally for repeated bouts of DKA or parenteral glucose infusions or glucagon for severe hypoglycemia/neuroglycopenia despite compliance with Rx. These patients, although uncommon, are not rare.
Therefore, although in rating these individuals, one can use another listing as potentially an equals, because these admissions are directly due to their Diabetes Mellitus and effects of their response to their Rx with insulin, consideration of introducing a Listing to cover these individuals seems to be warranted and important in considering the severity of these complications and would not be a reference listing to another Body System. Therefore, I would propose the following be considered:
Listing: With diabetes mellitus that is insulin requiring and despite compliance with treatment, repeated attacks of DKA (can be defined in the preamble) or severe hypoglycemia with hypoglycemia unawareness or neuroglycopenia (again defined in the preamble) requiring physician intervention and parenteral insulin and IV fluids for DKA or parenteral glucose or glucagon for hypoglycemia occurring six times during a consecutive 12 month period at least 30 days apart; or
With diabetes mellitus that is insulin requiring and despite compliance with treatment, repeated attacks of DKA (can be defined in the preamble) or severe hypoglycemia with hypoglycemia unawareness or neuroglycopenia (again defined in the preamble) with three hospitalizations within a consecutive 12-month period and occurring at least 30 days apart. Each hospitalization must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization.
Again, these claimant cases may be uncommon but are not rare and would help the adjudicators understand that there are patients with diabetes mellitus that due to their underlying disease may have repeated complication of glucose control despite following prescribed Rx and that these individuals do have recurring bouts of poor glucose homeostasis over which they have no control. THIS IS NOT ALWAYS A COMPLIANCE ISSUE!
For Question #3, patients with islet cell pancreas transplantation; these may be done in conjunction with patients with CKD-5, who are in need of renal transplantation. However currently, more are being done in patients without significant CKD, but who have difficult-to-control diabetes mellitus despite multiple insulin injections of various formulations of insulin that are both short and long acting or with insulin pumps. These patients are those, who have been termed “brittle,” who despite compliance with dietary and insulin regimens, have blood sugars that can wildly swing from very high in the 300-500mg/dl range and then rapidly plummet into the 30-40mg/dl range predisposing these patients to both frequent bouts of DKA and severe hypoglycemia including hypoglycemic unawareness and neuroglycopenia that are actually more common in these patients with chronically widely fluctuating blood sugars.
These islet cell transplants represent allogenic transplants, since Type I patients basically have lost their islet cells and require transplantation of islets from another person. It has only been in the last 8-10 years that with the development of certain preparatory procedures to the islets, that long-term independence from insulin injections has occurred, whereby 50% or more of patients may be insulin independent after 2 years or more post-transplant. Even those, who are not insulin independent, many times can be controlled with simpler insulin regimens without the wide swings in blood sugars. Also, most importantly, many of these patients, who prior to islet cell transplantation have hypoglycemic unawareness, have marked improvement in this phenomenon after islet cell transplant; so that they can discern when they become hypoglycemic in time to treat it, before they develop neuroglycopenia.
The issues with any allogenic transplant are success of the transplant and importantly the side effects, which with islet cell transplants include the side effects of immunosuppressive agents that are required to prevent rejection of this allogenic transplant. The actual technical part of the transplant is relatively straight-forward and with low risk overall. However post-transplant, there can be issues with management of blood sugars as well as the immunosuppressive agent side effects. For these reasons, if islet cell transplants become a therapeutic modality widely available to patients with Type I DM, then just as with other allogenic transplants including, for example BM transplants, consideration of a year of disability from date of transplant (since the need for a period of pre-transplant procedures to the patient is usually not needed as with some other allogenic transplants such as allogenic BM transplants) should be considered as an additional Adult ENDO Listing addition due to the issues for a period with glucose control post-transplant; issues with need for ongoing immunosuppressive agents; and complications that can occur in any patient with Type I DM, who by having Type I DM itself is considered immunocompromised by many experts; and complications that then may occur post-transplant from both the Type I DM and immunosuppressive agents used to prevent rejection of the transplant.