Thursday, August 17, 2017

Detailed SOLU-CORTEF actovial instructions with Photos

Detailed SOLU-CORTEF actovial instructions with Photos. These are the best and most thorough that I've ever seen. 

Add this to your emergency kit. Place it on your refrigerator. Add to service dog vest.

Train loved ones to save you! I mean, if they like is, they want to keep us around, right?! 💙💛💙

Tuesday, August 15, 2017

Choosing a BLA surgeon

(Updated September 5, 2018) How do you choose a BLA surgeon? What do you need them to say that will prove to you that will get every last one of those adrenal cells out of your body when they do your BLA? I share the questions I asked my BLA surgeon three years ago.

The internet is loaded with articles about how to choose a surgeon. 
Consumer Reports gives its suggestions. So does Forbes.

Even Medscape posted a study in early 2017 about How do patients choose surgeons (51% based on who accepts the insurance!). It also seems that Cushies have surgery with whoever their endocrinologists work with regularly and recommends, often describing those surgeons as "the best around."

I would argue that we need to be considering other things as well -- more medically-specific issues.

When the time comes for bilateral adrenalectomy or BLA, a Cushie has to choose the right surgeon. A google search didn't turn up much other than basic information from medical universities about Cushing's and BLA as a treatment. I did find this 2016 article Adrenalectomy for Cushing’s syndrome: do’s and don’ts, but doesn't help us when interviewing a BLA surgeon. The article tells us more when BLA is the best treatment, which is not what we are seeking.

I share so much information here in my little corner of the internet because the patient experience after BLA is nearly non-existent online. When Cushies search for information, I want them to find it. So I just post the list myself. My friends in the BLA group always help me learn more about this disease, so some of the knowledge I share here is as a result of learning from them.
I asked my BLA surgeon these questions in 2013. Yes, I asked all of these! Access to Information before making a decision is my right as a patient, and no surgeon is cutting into me without a thorough but friendly interrogation.

-- How do you reach the adrenal glands? Which operative method do you use to perform BLAs: posterior retroperitonial laparoscopic approach or anterior laparoscopic approach? What are the benefits and risks of each method?

Retroperitoneal refers to a posterior or back approach, while anterior refers to a front or side flank approach.  The posterior approach allows for direct access to your kidneys and adrenal glands. The traditional anterior surgical approach requires incisions in your abdomen and requires surgeons to work around your liver, spleen, pancreas and colon. 

I had my BLA done via posterior approach.
Here is one 2012 article I found about retroperitoneal BLAsI did not suffer much pain pain post op so I didn't take many pain killers. I could move around easily because our bodies bend forward, not backwards, in our daily lives.  I know many people who have had their adrenal glands removed from the front or side. They experience a reasonable amount of pain and discomfort from the abdominal surgery, but they manage with cautious moving and pain killers. They survive and would do it again.
JUST REMEMBER: Many doctors have been trained to perform BLAs either using the posterior or anterior approach, and they like to do it that way. Let them. You don't want them practicing on you. If you prefer one method, then find a surgeon that specializes in that method.

-- How often and under what circumstances have you converted from a laparoscopic approach to an open BLA? 

-- How many BLA procedures do you perform each year?
A Cushie friend P asked me who performs adrenalectomies. Answer: general surgeons or bariatric surgeons. This 2009 article entitled Which matters more: volume or specialty? reviews the outcomes of over 3,000 surgeries performed by nephrologists and general surgeons throughout the United States It is recommended to go with the surgeon that does a high volume of these surgeries for a better outcome.

-- Will you perform the surgery or do you have anyone else assist you? 
I prefer you to do it. I do not give consent for a resident or fellow to participate in my surgery.

-- Will a robot assist in my surgery?

This is a real thing. You better ask. Then click here to see how many adrenalectomies are listed under the 'robot' column.

-- Will my weight cause any surgical concern?

-- How much exploration will you do to search for ectopic adrenal tissue?
Give permission and encourage surgeon to really explore the area. Causes for persistence or recurrence of endogenous cortisol production were incomplete removal of the adrenal glands and ectopic adrenal tissue, which could be identified in locations like the retroperitoneal fat, the gonads, or the mediastinum.

-- How do you handle the adrenal gland once detached? Do you bag the adrenal? 
My surgeon said he doesn't cut into the adrenals. He cuts them away from the blood vessels, bags it, removes it, and sends it to pathology. This keeps him from leaving any adrenal cells behind.

-- How do you ensure that you remove even microscopic adrenal cells? Do you scrape the adrenal beds?
I drilled my surgeons about rest tissue and making sure they got the fat surrounding the adrenals. How does he/she make sure all adrenal tissue is gone, out of hiding. You want them to remove all the surrounding fat. My left adrenal slid south below the kidney!! My surgeon said it was a "normal" congenital defect"--that's an oxymoron!  Your surgeon needs to explore and track it down.

-- The adrenal glands are fed by many vessels and nerves. How do you navigate around these to avoid damage to the kidneys and other structures?
The arteries supplying the suprarenal glands are numerous and of comparatively large size; they are derived from the aorta, the inferior phrenic, and the renal. They subdivide into minute branches previous to entering the cortical part of the gland, where they break up into capillaries which end in the venous plexus of the medullary portion.

-- Incidence of rest tissue or remnant tissue after you perform BLAs? (informal study from BLA group of 200 patients suggest 10%)
-- Incidence of Nelson's of patients (med lit says 20%)

-- Have you had any of your patients get sepsis? die 30 days after BLA?

-- Under what circumstances should I seek emergency help after I leave the hospital?
The surgeon may say things like fever over 102 degrees, oozing wounds, extreme pain or redness at the incision sites, etc. Doctor should give you a number for how to reach him in an emergency.

-- How much hydrocortisone is administered before the surgery and during the surgery?
100 mg Solu Cortef via IV push at both, or before and after. You want all of that all at once, not drip drip drip via IV over a few hours. Anesthesiology team needs to watch for low blood pressure during surgery, as this is a sign of adrenal crisis. Be sure to discuss this with surgeon in the office, anesthesiologist before surgery, and surgeon before surgery. It is always a good idea to ensure everyone is on the same page (even if we are the ones who need the reassurance).

-- How long do patients stay in the hospital?

-- Do the nurses that care for me post-op have special training on adrenal insufficiency?

-- How often will you come visit me in the hospital? Daily?
I want to see you every day!

-- When are cortisol levels checked post op in hospital?

-- How will we assess the success of the surgery with lab work? 
DHEA-S is a good measure to check since this is made in the adrenals only. After both adrenal glands are removed, the blood cortisol level can be measured to see if the operation was successful if the patient is not taking hydrocortisone. In this circumstance, the patient should be given dexamethasone for several days before blood work since hydrocortisone is measured in the blood test as cortisol and dexamethasone isn't. Most adrenal surgeons don't even worry about dropping cortisol and instead send the patient for post op labs for DHEA, basic metabolic panel (BMP), and complete blood count (CBC) between days 5 and 10. That DHEA-S level should be very low. Mine was less than 0.1. My endocrinologist Dr. Friedman said that was a ZERO. 

-- Who will write the orders for my hydrocortisone while I am in the hospital? 

-- How do I contact you if I am having trouble post op?

The doctor may say just tell the nurse, and the nurse will contact his/her office. That's all good except that most nurses are very hesitant to bother a doctor with all of the patient's questions. In our case, we need direct access to the surgeon and endocrinologist.  I have called the surgeon on my cell phone when the nurse wouldn't call after repeated requests. I have called the surgeon when the doc's fellows pay a visit and try to do something other than what the surgeon and I worked out at my pre-op appt. Don't be afraid to raise a stink and get the information you need. The folks there won't like it, but what can they do? Spit in your IV? No... you will be out of there soon, so be sure you get what you went there for.

-- When will my pathology report be back?

-- How often will I see you for post op appointments?

-- Would you be so kind to take a photo with me?
Since you are going to send this Cushing's in remission, this will be one of my before photos. I want to have a nice souvenir to remember us. 

Sunday, August 13, 2017

Cushing's is as bad as Cancer

As Cushies, we often struggle to let others know how poorly we are doing. How can they possibly understand when they don't know with Cushing's is?!

It seems in some people's minds, it's not as bad as cancer. Only cancer is as bad as cancer. That proves to be a very myopic perspective and limited understanding of all the diseases of the world.

Take Cushing's for example. We Cushies know the devastation we suffer physically, mentally, and emotionally from excess cortisol.

Cushies, we now have a tool to help us. The general population knows about cancer and multiple sclerosis. The authors of MANAGEMENT OF ENDOCRINE DISEASE: The Burden of Cushing's disease: Clinical and Health-Related Quality of Life Aspects compares these well-known debilitating diseases to Cushing's.

As you can easily see, untreated Cushies really aren't very well.
  • Cushies experience a lower quality of life than cancer patients in these categories:
    general health, vitality, social functioning, and mental health.
  • Cushies suffer equally with cancer patients in these categories: bodily pain, emotions.
  • Cushies fair slightly better than cancer patients in this one category: physical.

Yet, where is the compassion? Where is the support? Where is the community to surround a Cushie and load them up with freezer meals and casseroles?

I don't take anything away from cancer patients. It is indeed terrible and life-threatening. However, they don't have the monopoly on suffering, and we Cushies wish people would stop trying to make us feel better by saying we don't have it as bad as they do.

The plain fact is that we Cushies often don't have a support community other than the ones we build to support each other online. "Cushies doing it for ourselves" is a great slogan, but we need the support of our loved ones, both family, friends, and coworkers. This makes the pursuit of Cushing's Awareness all the more important.

Wednesday, August 2, 2017

Growth hormone for $0 a month!!

I found out recently that Humatrope does not charge a copay for people who don't have the money for it!! I now pay $0 per month!! I used to pay $60 per month and I have paid as little as $50 for three month supply.

How did I find this out? Well, I complained to the specialty pharmacy that filled the growth hormone that because they require me to fill it every month and refuse to fill a 90 day rx, it costs me more... instead of paying $60 a month for two months $120 and get third month free, this would force me to pay $180 for three months. This was unfair, and she said there was a plan. Not income based. Asked if I take it every day... yes. Do I have trouble meeting the financial obligations for taking the GH? Yes... i have 20 other medications I take daily. She said, ok your copay from now on is zero. WHAT?! I asked how that can happen, why would humatrope company offer to do that? She said that the insurance company still pays them monthly so the Patients amount is very small in comparison to the big chunk they get from insurance. So in order to get money from insurance, they let the patient have it for free....

The questions are:

Are you over 18?

Do you live in the USA or Puerro Rico?

Do any of your reimbursements for growth hormone medicine come from government entities like Medicare, Medicaid, Tricare, etc? THIS MUST BE NO

I'm so happy I stumbled across this, and I want to share it with all the Cushies out there who suffer from growth hormone deficiency after pituitary tumor(s) and pituitary surgeries.