This letter is going to an “independent” medical reviewer (probably yet another M.D. with a specialty in endocrinology, not a CFRD specialist as I ask for) and will also be read by a medical director at my insurance company who will make the final decision of whether or not they will assist me financially with the purchase of an insulin pump and continuous glucose monitor.

I put on my white doctor lab coat when I wrote this, so I am sorry if there is confusion. I tease that I think most CFers deserve a honorary medical degree by the age of 30 with all the medical stuff we go through!

Here’s the letter… (or click the link below)

To Whom It May Concern:

Due to the rarity of my health issues, my appeal should be reviewed by a specialist in Cystic Fibrosis Related Diabetes.

As my Endocrinologist has described, I have Cystic Fibrosis Related Diabetes (CFRD). This is a rare type of diabetes affecting a small, but growing population of Cystic Fibrosis patients. CFRD is unique and needs to be treated as such. It is also very challenging as it requires a treatment that is tailored to my specific needs. My nutritional intake and activity levels vary greatly on a day-to-day basis due to the effects of Cystic Fibrosis, therefore treatment of CFRD must be flexible. When my health is considered in its entirety, it is obvious the addition of an insulin pump and CGMS would be the best, most-cost effective option. Better treatment of CFRD will result in better health, less complications, both diabetic and with Cystic Fibrosis, resulting in fewer hospitalizations, less IV antibiotic treatments and such, and in turn save Great-West Healthcare money in the long run. These effects need to be considered in the decision of financial coverage of the devices. Please understand that growing up with Cystic Fibrosis has given me a medical education that most patients do not receive.

My nutritional needs require a high calorie diet due to my inability to digest food properly. CF causes thick, sticky secretions in the pancreas which block digestive enzymes. I must eat almost continuously to keep up with my body’s needs for nutrients. Despite this, I am underweight and have always struggled to maintain my persistently too-low weight. Over my lifetime I have found gaining weight is almost impossible. This affects my over all heath, as it is well known that the weight affects lung function in CF patients. Changing my eating habits is not an option.

Often, the complications of CF have an affect my appetite on a meal-to-meal basis. This makes it even more important for me to eat as much and as often as possible. When I am well, I usually eat three large meals a day and continuously snack in-between to meet my caloric intake goals. Because I eat so frequently it is sometimes hard for me to tell which direction my blood glucose levels are going since the insulin bolus’ overlap. Checking my levels with a standard point-in-time glucose meter does not show the trend-lines I need to know to treat myself properly.

When I sit down to eat a large meal, 2-4 times a day, I can never judge how much I am going to eat. I eat as much as physically possible, usually not finishing everything on my extra-large plate. I have attempted to take insulin after I eat, having counted the total amount of carbs I took in, but the delay does not work for my body. As well, I have attempted splitting my dose, taking half a dose before a meal and one more shot after to complete the dose for the accounted carbs, but this has proven to not work for my body. For all the reasons stated above, my use of an insulin pump, which would allow several to many smaller boluses while eating, and a continuous glucose monitoring system, to provide trend-lines, is the most logical choice of treatment.

Recently I was hospitalized for a Cystic Fibrosis exacerbation, which unfortunately is very common for CF patients. During my one-week stay, my blood glucose levels were inexplicably out of control. Even with the help of the nursing staff and doctors, we were unable to find the cause of my extremely variable levels. A continuous monitor would have greatly helped my ability to gain control, alerting me of times when I would start to go too high (reaching well over 400mg/dL), or when I would drop low after too much insulin was delivered to correct the high. After my hospitalization, I continued IV antibiotics at home, and added Prednisone to my regimen for one week. I attempted to keep the best control I could, but without the assistance of a CGMS and the flexibility of a pump, I was not able to maintain my goal BG levels.

During and since this hospitalization, I have been on many new medications for my CF. No longer am I able to feel the signs when I start to become hypoglycemic. My educated guess is that this is due to one of my new medications. I live alone and am very afraid I may have a severe hypoglycemic episode while by myself. Yet, I must aggressively manage my CFRD to avoid complications. A CGMS would help me be more comfortable with lower, healthier, blood sugars and would allow me to gain and maintain better control of my levels.

An added difficulty for patients with CFRD is sluggish insulin. Due to the thick secretions in the pancreas, insulin is not able to move into the system in a timely manner. This can cause lower BG levels than expected after a bolus; also another logical reason a CGMS is a necessary tool, to alert me of these lows. The ability to quickly disconnect from insulin when this event happens is another reason a pump would work better than my current treatment of using shots. There are other issues that sluggish insulin causes that affects BG control, but I am just learning about this myself.

The more tools I have in managing my BG levels, the better my health in general will be, and the less of a financial burden I will be on my health insurance. I have suffered from dehydration many times since I was diagnosed with CFRD due to my body trying to flush the excess sugar out of my body. Dehydration causes the already plentiful amounts of thick, sticky mucus, to become even more like cement in my lungs, causing a perfect breeding ground for bacteria. I have a copious amount of mucus plugs in my lungs, which is common for someone with CF, but I also believe that the amount of plugs I have is increased due to dehydration. It is well known that patients with CFRD have decreased lung function, as compared to the already decreased function of a “normal” Cystic. I would appreciate your help in assisting me to take better control of my diabetes, by giving me the tools necessary to help me, in every way, to take better control of my blood glucose levels, to prevent further destruction to my lungs and the rest of my body’s organs, which is in my interest as well as in Great-West Healthcare’s interest.

Sleep is ever so important for CF patients when both healthy and ill. This may be a less major point, but it adds to the overall reasons that a pump and continuous monitor could assist in improving and maintaining my control. When I am ill, my need for long term insulin is even more important. While I am sleeping I can not give myself a shot of Lantus, there for I must interrupt my much needed sleep to give myself a shot. If I had a pump, I would not need to do this, which would help my body recover faster from illness, possibly even preventing illness and preventing the worsening of my Cystic Fibrosis.

I am not expecting to replace my point-in-time meter with a CGMS, I understand that both are necessary and very valuable tools that can only help me gain better control and maintain that control. I would appreciate the ability to take the best care of myself to prevent unnecessary illness because of all that affects my body with two chronic diseases.

With all the complications of diabetes, as well as the complications from Cystic Fibrosis, and all of the unknown complications diabetes can cause on CF. I think it is in the best interest of my health, and the best financial interest of Great-West Healthcare to provide myself coverage of both an insulin pump and CGMS.

I appreciate your consideration of my unusual case. Please contact my doctor or myself with any questions you have.

Sincerely,

me!

My doctor said we are finally ready to pursue my final appeal of insurance coverage for an insulin pump and continuous glucose monitor. It took a while to find and compile research studies since simply there are so few (we even had to wait for one to complete!). CF related diabetes is very rare, considering the size of the general population and it is also a fairly newly discovered complication of CF only since us cystics are living so much longer now days. I have figured there are only approximately 4,000 people in the US with CF related Diabetes. Finding info on it, none the less, hard data and research studies is very complicated.

Regardless of this, my insurance companay doesn’t care that I need to take up to 8-9 insulin shots a day, if I were to follow my prescribed nutritional needs. That is on a rough day. At a minimum I take 5 shots a day. Most diabetics with Type 1 diabetes take about 3-4 shots a day, minimum. My nutritional needs are much different than someone with Type 1, which makes things even harder to balance. My insurance company is not allowing coverage for an insulin pump because I do not have Type 1 diabetes…. now does this sound fair to you?!

I sent in my paperwork the other day, and my doc is sending in her stuff soon. Please wish good thoughts and send positive vibes for me and my future pump. Hopefully this time the reviewers will actually listen to my doctor and to common sense!

I spoke with the insurance co, they believe their independent reviewers are up to date with all the latest info in their field. I approximate 4,800 people in the US have CFRD. How is every single endocrine specialist supposed to know the ins and outs of such a rare disease? My insurance “assured” me that their independent reviewers are up to date with diabetes (no mention of CFRD). Why am I not convinced?! Ha!

I am able to request my next appeal to be done by a specialist in CFRD, but I will not be assured that my request will be granted.

Learned something new today thanks to Dr. J. I have a normal c-peptide level, which the insurance is using to deny my claim. If I had an abnormal c-peptide level, this would diagnose Type 1 (low level) or Type 2 (high level) diabetes. CFRD is neither, which is why my levels are “normal” and why I have CFRD and not Type 1 or Type 2.

I want to send out a special “thank you” to my CF clinic nurse. I can’t thank her enough for all the help and support she has given me over the years. I know I am getting the best care because she always goes over and beyond to make sure of it.

This is an interesting fact. Colorado is a state that allows health insurance discrimination. A healthy friend of mine, “healthy” with exception to a small shoulder pain he had last year (which was treated with a cortisone shot and has not reappeared), was denied an individual policy because of the shoulder issue. So I really doubt I could get a self-insured policy in this state. I may have to consider something else.

Just started shaking, dizzy, and slightly confused, feeling like I am in a tunnel…. checked sugars, 74… off to go get a snack! Yum yum chocolate covered pretzels here I come!

P.S. Thanks for your support Jesse!!