9/29/2017 – STORY UPDATE

After my first parathyroid surgery on February 16, 2016 , my PTH remained elevated and calcium remained lower , but normal. I had given myself a year in hopes that my PTH would lower and things would even out. Once I passed a year, I had decided to agree to a subtotal parathyroidectomy with my surgeon from before. My second operation was June 6th, 2017. I was given the option to stay in the hospital overnight, but felt that I was stable enough to go home and was discharged that evening. We discussed symptoms of hypocalcemia and that I was immediately to go to the emergency department if symptoms developed.

The morning of June 8th I woke up and noticed that my arms felt heavy and numb and decided to go back to bed in hopes that symptoms would improve. When I woke up feeling worse I decided to call my Dr’s office and headed to the emergency room. By the time I got to the hospital my face and entire body felt numb, my speech was slurred, everything felt heavy. I was scared that I was having a stroke. I was taken back right away and given blood tests that showed a calcium level of 6.7. I was admitted up to the surgical floor and started on IV fluids, calcium and magnesium.

About three days later I was stable enough to be off the IV but remained monitored until my numbers were stable enough to be sent home. I was put on vitamin D, magnesium, calcitrol, and tums, with dosages adjusted according to labs. There is a chance my remaining gland will not recover and I will need to continue on those medications the rest of my life and may also need natpara injections. At this point we just wait and see, but at my most recent endocrine appointment my Dr. said he felt the damage is permanent .There was a decrease in kidney function when I was put on higher dosages of calcium so I am hoping this can be leveled out before permanent kidney damage occurs.

I am still developing stones rather quickly and underwent my eighth stone procedure in September. I am following up with urology, nephrology, and endocrinology regularly, but it feels like each appointment ends with more questions than answers. I am learning to adjust to life with hypocalcemia/hypoparathyroidism, but it has been hard to get used to this new normal.

GUEST BLOGGER, BRITTANY’S STORY….

My parathyroid journey started two years ago at the age of twenty-six. I was in the third trimester of my third pregnancy when I was stricken with severe back pain. I thought it was just a pulled muscle, but wanted to be evaluated to make sure that the baby and I were okay. I went to my local walk-in clinic where they ran a urine test that came back with a large amount of blood. The doctor told me that it was most likely a kidney stone and sent me for an ultrasound to confirm. As well as having several stones, I was also diagnosed with a condition called medullary sponge kidney, also known as Cacchi–Ricci disease. It is a congenital disorder of the kidneys characterized by cystic dilatation of the collecting tubules in one or both kidneys. Individuals with medullary sponge kidney are at increased risk for kidney stones and urinary tract infection. I was scheduled to meet with an urologist and nephrologist to discuss these findings. Because one of my stones was too large to pass naturally I was scheduled for a lithotripsy. I had the procedure six weeks after my daughter was born. Sadly the lithotripsy did not break the stone down enough to pass. As a result I required a second surgery to go in and retrieve the stone.

Despite dietary changes and medications I continued to form kidney stones at an alarming rate. My stones were analyzed and found to be composed of mostly phosphorus. I did my best to manage things, believing that this was going to be my life – contending with medullary sponge kidney and the resulting stones. I educated myself as much as possible. It was when I joined a kidney stone support group on Facebook that someone suggested having my parathyroid hormone (PTH) and serum blood calcium levels checked to test for primary hyperparathyroidism (pHPT). Little did I know, but my previous nephrologist had run these tests, but didn’t inform me. I learned that the first time my labs were drawn my calcium was 10.1 mg/dL and my PTH was 63.7 pg/mL.

As I did not feel comfortable with this first nephrologist, I asked to switch to a different provider in the department. This was the best decision I could have made, because the second nephrologist I was placed with genuinely cared and listened to my concerns regarding hyperparathyroidism being a possibility, even though these initial tests indicated everything was normal. She began running blood and urine calcium and PTH labs every three months, as it was standard procedure to run these test for kidney stone maintenance appointments. My blood calcium was always normal, never higher than 10.2 mg/dL. This lab’s normal range for calcium was 8.5 mg/dL – 10.5 mg/dL. While adults 30+ should suspect and rule out pHPT as the root cause if values are over 10.1, it might be normal for an adult like me in their 20’s to have a value as high as 10.5, so my 10.2 was not alarming. Here is graph of my calcium blood tests: 

My PTH on the other hand, aside from that initial draw, continued to come back over 100 pg/mL. The normal range for PTH at this lab starting November 2015 was 14 -95 pg/mL. Prior to that the range was 14 – 72 pg/mL. As a side note, I am not sure why they changed their values, but I feel it will make diagnosis even more difficult for others. While Vitamin D was tested as well, it was not done consistently. Here is a graph of my PTH levels:

At my request, on two occasions,  my doctor ordered Ionized Calcium. The first time it was 1.36 mmol/L and the next time 1.33 mmol/L . The normal range at this lab was 1.12 to 1.32 mmol/L. 

At the same time I was also seeing my GP and a gastroenterologist that I was referred to for nausea and I also told them that I thought I had hyperparathyroidism. Both told me that my calcium was not high enough to have it. My nephrologist however admitted to being confused by these numbers and suggested that we have an endocrinologist look at my case.

The endocrinologist reviewed my labs and said, “There’s obviously something wrong. I just don’t know what it is.” He put me on 150,000 iu’s of Vitamin D a week split between three 50,000 iu capsules that I took on M/W/F, ordered a neck ultrasound and scheduled a follow-up in three months. I felt terrible on the Vitamin D, but continued to take it until my follow-up appointment. At the follow-up we went over my labs, and for the first time my urine calcium came back high. The last reading was 176 mg, however this time it was 482 mg. Normal range for 24-hour urine calcium is 50 – 250 mg. at this lab indicating hypercalceuria.  Blood calcium came back higher, but again, still within normal range. An endocrinologist performed the ultrasound however he was not the one following my case. The ultrasound showed swollen lymph nodes but the parathyroid glands were not located. I knew that ultrasounds were not very reliable in locating parathyroid adenomas, so I was hopeful that they would send me for a sestamibi scan, as the endocrinologist I was seeing said a positive scan or blood calcium of 11.5 was required for referral to surgery.So I asked the endocrinologist that performed my ultrasound for more comprehensive imaging, but was told it was not necessary. Instead I was told to follow-up again in six months.

In the meantime I was prescribed 25mg of hydrochlorothiazide, in an attempt to lower my urine calcium, despite already being on a different blood pressure medication. I did not feel comfortable taking the hydrochlorothiazide in that large of a dose without being monitored. I had a follow-up with my nephrologist a couple weeks after that and discussed my concerns with her. She lowered the dosage and took me off of the other blood pressure medication. While going over my numbers she noticed the elevated urine calcium and said, “I’m fairly certain you have hyperparathyroidism”. She ordered a sestamibi scan and follow-up as quickly as she could. An adenoma was located on the left side, and I was finally sent to see the General Surgeon who specializes in Vascular and Endocrine surgeries, to schedule a parathyroidectomy. I had my surgery February 16, 2016 and am so thankful my nephrologist cared as much as she did and listened to my concerns. Since I presented as normocalcemic, which means my blood calcium was never high, it was extremely difficult getting a diagnosis because many in the medical community are baffled by this biochemical presentation. Through my research I learned that patients who present as normocalcemic frequently suffer with kidney stones because the kidneys are doing their best to try to rid the body of the excess calcium. It is also quite typical for a normocalcemic patient to have PTH over 100.

I am still diagnosed with sponge kidney and am told there is no cure, just management of symptoms. I also have nephrocalcinosis, My urologist has stated that there is nothing he can do surgically to remove the calcium deposits, but has been great about getting stones out before they become problematic. I have had a total of five kidney stone procedures to date. I believe there is a link between pHPT and the kidney issues I have experienced. I will continue to follow-up with my nephrologist and urologist bi-annually. I believe that they anticipate these problems will resolve, but only time will tell.

Hyperparathyroidism has stolen two years of my life, and who knows how much longer it would have been if I hadn’t researched and found www.parathyroid.com among other sites, as well as connected with others in various on-line support groups and forums to become educated.

I used the Calcium Pro app ® that helps patients become their own health advocate. To the right is a screen shot of the values I entered.  

Since having surgery, my brain fog has lifted, and my memory has gotten much better. I’m also feeling a lot happier and more energetic. I credit the wonderful nephrologist who did everything she could to help, instead of just brushing me off like other providers had done because my calcium wasn’t high. It took a great deal of persistence on my part to get diagnosed and I’m hoping my story raises awareness and helps others like me who are struggling to get diagnosed because their doctors don’t understand the normocalcemic biochemical presentation.

ABOUT BRITTANY KENYON: Brittany is a stay-at home mom with children ages 5, 4 and 2. She graduated with honors this past December with an Associate Degree in Medical Administration. Brittany also holds Medical Reception and Medical Office Assistant diplomas. Brittany believes her coursework helped a lot in her understanding of things, especially medical terminology. Brittany invites you to follow her on twitter.com/britmasterflash and/or contact her at hello.waldorf@gmail.com.