All about Hypopara


Hello Friends!

I’ve put together cheat sheet for my rare disease, hypopara.

Disclaimer: I am not a doctor nor should any of this be taken as medical advice. I do not represent or work for any pharmaceutical company, the Hypopara Association, or any medical facility. This page is simply my OWN PERSONAL JOURNEY and is only my personal opinion. I am in the United States and this information/availability of treatments will vary from country to country.

To find a doctor that can help you on your hypopara journey, visit Hypopara.org. To share information with your doctor, send them KnowHypopara.com.

To dive into Dr. Mannstadt’s research (considered the “Father of Hypopara”), start here: https://mannstadt.mgh.harvard.edu

Contents:

  • What is Hypopara?
  • Current News (Most current updates, also continued at bottom of blog)
  • How did I get Hypopara?
  • What are my symptoms?
  • Physical symptoms (and how physical therapy may help)
  • Cognitive symptoms (and how mental therapy may help)
  • Why does calcium matter?
  • Calcium tests and testers (ISTAT information here)
  • How is hypopara treated?
  • Supplements 
  • Medical bracelets
  • Am I at risk?
  • Do I use mobility aids?
  • What affects hypopara?
  • Information on sensitivity reading
  • Newly diagnosed information
  • Current News Updates continued
  • About me

What is Hypoparathyroidism, also known as Hypopara?

In simplest terms: Hypopara means I need to control my calcium levels from outside my body, which is difficult and dangerous in moderate to severe cases (like mine).

The details: Despite having “thyroid” in the name, hypopara has nothing to do with your thyroid! However, 80% of hypopara patients come from the inadvertent removal or damage of the parathyroid glands during neck procedures, like thyroid surgery.

Hypopara is a disorder causing lower-than-normal levels of calcium in the blood due to insufficient levels of parathyroid hormone, or PTH. Hypopara is an uncommon Endocrine deficiency disease characterized by low serum calcium levels, elevated phosphorus levels, and absent or inappropriately low level of PTH. Hypopara affects every muscle, bone, and organ in the body, as calcium is a necessary element.

Approximately 80% of the estimated 80,000 people in the U.S. and 110,000 in the European Union with hypoparathyroidism are women.


Current News

UPDATES:

5/14/2024: The FDA delays the review of TransCon PTH by three months

11/15/2023: Ascendis has resubmitted Transcon PTH to the FDA

11/7/2023: 2023 Hypopara Conference Highlights

10/20/2023: Amoylt Pharma opens trial for PTH injectable medication

10/3/2023: Ascendis presents Transcont PTH Data at the American Thyroid Association. Also Amolyt Pharma has presentation at ASBMR annual meeting

9/8/2023: Ascendis confirms plan to resubmit Transcon PTH to the FDA in October. If accepted, we will hear in November if it will receive a Class 1 or Class 2 categorization, which will determine a new PDUFA (potential approval) date of either December 2023 (class 1, shorter review) or April 2024 (class 2, more in-depth review). EAP is still enrolling.

6/8/2023: Amolyt Pharma to Host Hybrid Key Opinion Leader Event 

6/6/2023: TransCon PTH EAP enrollment opens in Germany

For more updates on current events, scroll down to “Updates Continued” at the bottom of this page


How did I get hypopara?

I had benign tumors on my parathyroid  glands, and was born with only three glands instead of four. I was diagnosed in 2002 with hyperpara due to the tumors, which meant my bones and organs were leeching too much calcium into my blood stream (the opposite but just as dangerous disease)

I had surgery to correct it on July 2, 2003. Unfortunately, the surgery overcorrected due to my missing fourth gland, and I was left with hypopara.

 

What are my symptoms?

For me, personally, the near continuous muscle spasms, brain fog, and fatigue are the heavy hitters, even when my calcium is in normal range. I use an IUD to stop my periods (and as a form of birth control as pregnancy and hypopara is risky). This has helped stabilize my calcium and lower my pain levels monthly. Note: Estrogen–like in birth control pills–can interfere with calcium absorption. 

Physical symptoms include:

Seizures, painful menstruation, cardiac rhythm disturbances, brittle nails, dry, course skin/hair, tingling/burning (parenthesis) in fingertips, toes, legs, and lips. Fatigue or weakness, tetany (involuntary skeletal muscle spasms) ranging in severity.

Twitching/jittering/spasms of muscles, particularly in the hands, arms, and throat. Bronchospam, laryngospasm, bone pain.

For muscle spasms, I see a physical therapist and chiropractor weekly, and a massage therapist bimonthly. My physical therapist manually releases my spasming muscles and then we do low impact exercises to strengthen them. While we can’t address the underlying problem, PT has helped me get out of spasms more quickly and they’re less severe. We do have to be fluid in our approach, as some weeks I can do more exercises than others. (I go once a week and do exercises at home 6 days a week). 

I also get a deep tissue massage with stretching and see a chiropractor once a week.

 

What about cognitive symptoms?

Cognitive symptoms include: memory problems, anxiety, confusion, fatigue, depression, brain fog. No two patients present in the same way, which makes hypopara very difficult to diagnose. Symptoms are often misattributed to other conditions or brushed of my patients and doctors alike.

Hypopara itself can cause anxiety, as well as low calcium. Mental health is just as important as physical health!

I am on daily anti-anxiety medication (Buspirone). I also see a cognitive behavioral therapist (CBT) for my anxiety and an EMDR therapist to work on my medical trauma and panic. Both have been instrumental in helping me move forward and heal. Of note, I do find it very difficult to have successful therapy when my calcium is low. Make sure to discuss this with your provider and ways to mitigate the scenario. 

Why does calcium matter? 

Calcium is a necessary mineral the body needs to function. As the slide says, calcium plays a key role in conducting electricity in our bodies. When calcium is out of balance, it adversely affects the nervous system. 

Calcium also plays a role in muscle contractions–including contractions of the heart muscle. It is a building block of healthy bones and teeth, and is involved in the blood-clotting process.

Calcium tests and testers 

Your total–or serum–calcium level is made up of two types of calcium levels: “Bound calcium” which is attached to proteins in your blood and “free calcium–known as ionized calcium–which is what is available for active use by the body.

The most common type of calcium test is a serum calcium venous blood test done at a lab or doctor’s office. This test can take 24-48 hours but will give a patient their serum calcium levels (faster if in ER/hospital). 

Unfortunately, calcium levels can change drastically over a number of hours and unlike other endocrine disorders (like diabetes), there is no at-home tester. 

In the US, Abbott does have a hospital-based unit called the ISTAT, which is occasionally offered to patients through the Abbott Point Of Care Physician Guided Program. This program requires a patient to work directly with their doctor to be a part of this program. With this unit, a patient can test their ionized calcium level at home. This unit is considered a loan, must have bi-annual updates, and an annual prescription from an approved physician. 

The device has a steep learning curve and requires a high level of care and training by a doctor. It’s also not available for purchase outside of this program in the US. Unfortunately, this makes it very inaccessible. 

After five years of fighting and advocating, I was approved to join the Abbott Point Of Care Physician Guided Program in 2021 with my private physician (which means I pay a physician an annual fee to be a patient of my PCP’s office, but I get 24/7 care). No other doctor except my private physician would agree to the time investment needed. To qualify, you must need ionized calcium testing no fewer than one time daily. I use Chem 8+ cartridges. 

To learn more about this US only program, reach out to:
apoc_pgprg (at) abbott (dot) com

To calculate a serum calcium estimate from an ionized calcium result: Multiply 7.507 x (Ionized calcium total) = Serum calcium estimated total. This equation isn’t foolproof, but it will give a good estimate. Ideal ionized range for me is 1.22 to 1.3 (9.1 to 9.7 serum). 

 ISTAT availability through the program varies all the time and I do not keep track of the availability as it would be impossible. I asked to be on a waiting list when I first approached them. Three years later, I was offered one but no doctor would work with me on it. When I got a private PCP, she also reached out and we were able to received a “loaned” tester one year later. It’s worth reaching out to your doctor and Abbott! Just be prepared to advocate.

In non-US countries, I’ve heard from one patient that they’ve been able to purchase an ISTAT through their hospital.

How is hypopara treated?

Everyone with hypopara is different. Some just need one calcium pill a day and it doesn’t affect their daily lives, others need active vitamin D (ex. Calcitriol), magnesium, and handfuls of calcium to be stable. Some, like me, have their daily lives severely impacted. For those with uncontrolled moderate to severe disease, we need a parathyroid hormone replacement, either by direct injection or a pump. 

Parathyroid hormone replacement options: 

Forteo: Forteo (Teriparatide) is an osteoporosis medication used off-label for people who need a PTH replacement but do not have an approved alternative available. This is the most commonly used medication outside of the United States for hypopara if oral medications are not enough. Because of its short-acting nature, it is often dosed multiple times a day or micro dosed in a pump.

Some patients have had success with the CeQur delivery system, cross between a patch and pump. If you have issues getting this medication through insurance, visit this website for guidance: https://mannstadt.mgh.harvard.edu/research-news-item-3/

TransCon PTH: (palopegteriparatide) by Ascendis Pharma is an investigational long-acting PTH replacement injection. It is open for Expanded Access Program (EAP) with hopeful FDA approval by April 30, 2023 and drug distribution by mid-second quarter. For more information on the EAP, visit https://ascendispharma.com/patients

Patients must stop all other PTH replacement immediately upon starting TransCon PTH. Titrating/overlapping medications causes a dangerous and uncomfortable side effect called Hungry Bone Syndrome (prolonged hypocalcemia).

NATPARA: (Takeda Pharma) The only FDA approved PTH replacement was recalled in the US on September 5, 2019. I’m a lucky patient who, along with around 400 others, was able to continue receiving medication through the Special Use Program. As of the 2024, NATPARA–including the special use program–will be discontinued globally. Natpara will not be returning to market.

For more information about current and future drug therapies, check out the 2022 conference section of Hypopara.org  (Note: I’m really excited to learn more about Amolyt Pharma’s new drug that’s in Phase II trials)
 
 
For all of Dr. Mannstadt’s research during the Natpara Recall, visit:
 

Do you have supplement suggestions?

Every person with hypopara may need different supplements, including different formulations. Some patients do best with calcium carbonate, while others do better with calcium citrate. 

The main things to look for with supplements:

  1. How many pills do you need to take to reach the strength your doctor suggested? It can be 1 pill or multiple pills. Ask a pharmacist or doctor if you’re struggling to read the bottle
  2. What are the inactive ingredients? Some supplements add in things like zinc and copper which should not be taken in large doses. Others may have fillers like lactose, which doesn’t work for people following a dairy-free or vegan diet. 
  3. Are the supplements third party tested? Supplements like Orthomolecular are third party tested to make sure you’re getting exactly what the label says. 

When in doubt, ask your doctor, pharmacist, or visit your local vitamin store. I get my supplements from a local specialty pharmacy that I use to compound some of my prescriptions, as I’m allergic to dairy. 

For calcium, I take Citrical petite. I take the petite because the regular strength has added minerals that should not be taken in large doses. I also keep Nature Made Calcium gummies on hand for emergencies when I’m unable to swallow a pill.

I travel with a powered calcium citrate/vitamin D packet by Fit Formula in case of emergency. There are also chewable tablets available as well. 

Should I wear a medical bracelet?

While it’s up to each individual person, I strongly suggest yes! Also, in an emergency, the team will look in your purse/wallet/phone. Make sure to carry a medical card that explains hypopara, your medications, your doctor’s information, and your emergency contact. It’s a good idea to also have your current medications and any allergies listed.

If you’re in the US, my favorite place to get medical bracelets is Lauren’s Hope. They’re cute, affordable, and have a good size tag. If you’re not sure what to put on your tag, Lauren’s Hope can help guide you, and it’s a good idea to also check with your doctor.

What’s on my tag? Since many people don’t know what hypopara is, I have hypocalcemia listed. My tag reads:
Heather, Hypocalcemia
1MG/KG Calcium IV
(Allergies)
Use epi-pen/inhaler 
Emergency contact name and number

On my cellphone screen, I use an app called Lockscreen to add text that reads:
Heather
Hypopara–medical bracelet
KnowHypopara.org
Emergency contact name and number

I have an iPhone, and I keep the health information up-to-date so it can be accessed in the emergency screen. I also utilize the medication log in the health app. Every time I take any medication, I log it. 

 

Am I at risk of getting hypopara?

Likely not, unless you have neck surgery or a family history of nonsurgical calcium/parathyroid disorders. The occurrence rate in the US population is 0.0002%Purple background with blue shapes and white text. Occurrence rate 75k patients in the US have hypopara. 41k of those have moderate to severe disease aka 0.0002% of the us population

Do you use mobility aids?

Yes! The mobility aid depends on the outing and how I feel. I can do short bursts of energy and walking without an aid on good days. On other days, I use everything from a cane to a rollator to a wheelchair. 

My favorite mobility aid is the Rollz Motion Performance. It’s a combination rollator (walker with wheels) and wheelchair. While expensive, it’s perfect for my needs. 

 

What are some other things that affect hypopara?

Hypopara patients have a running joke: everything affects calcium levels. It’s why so many of us are one a strict diet (plus, in my case, I have digestive disorders that came hand-in-hand).

Some contributing factors:  Weather (extreme hot or cold), infections, changes in medication, strenuous exercise, anxiety and stressful situations, changes in diet, abnormal magnesium and phosphorus levels, menstruation, intestinal conditions (including constitution and diarrhea

Note: Viruses, especially fevers, can affect calcium levels. A endocrine journal earlier this year (forgive me, I don’t have the link) stated that hypopara patients are more likely to have severe and longterm complications from Covid.

I want to include a character with hypopara in my book. Can you sensitivity read?

Contact me via social media or my contact form and we can discuss! My rate will depend on how much work is needed, the time frame, and my availability. 

Please note that what I’ve posted here is only a brief overview regarding hypopara. Use this information as a starting point only.

I’m a newly diagnosed hypopara patient and don’t know where to begin. What now?

Know that you’re not alone!

If you’re in the US visit: KnowHypopara.com (by Ascendis Pharma)

Don’t miss the conference (usually virtual) by the Hypopara Association in October annually. You can find out more at hypopara.org and watch past conference workshops

Search Facebook for support groups–there you can get personal advice and doctor recommendations. I don’t always have the time or energy to provide one-on-one consultations, but if you reach out to me, I can help point you in the right direction!

Updates Continued:

3/6/2023: Ascendis Pharma has opened up the Expanded Access Program (EAP) for TransCon PTH, a new parathyroid hormone replacement. If a patient and their doctor meet the EAP qualifications, they may be able to receive the medication before suspected FDA approval April 30, 2023. This is especially exciting, as NATPARA SUP will be discontinued in 2024.

Ascendis did have label talks with the FDA and according to the transcription from their investors call, there will not be a black box (osteosarcoma) warning, like there is for Forteo and NATPARA. Ascendis has also started clinical trials internationally in hopes to get the medication distributed globally.

After community and physician complaints, Ascendis parted ways with the third party in charge of the EAP program and are now doing it in-house. They have begun shipping medication to the first approved patients. 

I applied for EAP on February 24 after my doctor was certified to be a physician ambassador for Ascendis Pharma, but I have not received any updates as of 3/6/23.

The first patient I know personally has transitioned from Forteo (TID) to TransCon PTH (QD). They were advised to stop Forteo after the first injection of TransCon PTH, despite TransCon PTH taking 5 days to reach full strength. They said the “transition was easier than going from Natpara to Forteo.” They have needed supplemental oral calcium but not IV calcium. They are still adjusting to their new dose.

There are still reports of Calcitriol shortages in specific areas worldwide. 

4/3/2023: TransCon PTH review has officially been put on hold by the FDA for “deficiencies in the New Drug Application“. The FDA did not specify what those deficiencies were at this time. A follow up conference call between Acendis and Investors was recorded. This conference call was recorded and is currently available for playback.

5/1/2023: TransCon PTH was NOT approved by the FDA.

The FDA sent Ascendis Pharma a Complete Response Letter (CRL) explaining that they would NOT be approving TransCon PTH at this time due to manufacturing concerns. 

Ascendis’ conference call today said that the issue is not safety and efficacy related and they do not expect an additional clinical trial to be needed. They will set up a Class A meeting with the FDA within 3 months to review details.

After this meeting, the FDA will re-review the drug application within 2-6 months. Minimum, we are looking at a 3-7 month delay.

The US expanded access program (EAP) is still accepting new patients, and review and EAP programs in multiple countries continue. (As we saw with the US-only Natpara recall, other countries have different restrictions)

Listen to this call here

5/3/2023: This is subjective as I’m not a medical professional, but based on the numerous articles I’ve read and the people I’ve spoken with, this is my review on what’s going on:

The FDA CRL letter stated:

  • FDA cited concerns related to the manufacturing control strategy for variability of delivered dose in the TransCon PTH drug/device combination product
  • No new preclinical studies, or Phase 3 clinical trials to evaluate safety or efficacy, were requested in the letter

Ascendis is using a dosing device that has already been approved with other medications. Therefore, the general though is the onus is not on Ascendis to prove that this device works through a separate trial, they’re only responsible for proving the device works accurately with TransCon PTH. This data may include records of manufacturing techniques and verifying that every dose from each strength from several batches of medication remain consistent. 

This is a way easier thing to gather data on, some of which they may already have or can readily get per their statement:

“We are committed to working collaboratively with the FDA and, because the agency did not suggest that additional Phase 3 studies may be needed to demonstrate the product’s safety and efficacy, we believe we are well prepared to address their concerns,” said Jan Mikkelsen, Ascendis Pharma’s President and CEO.”

What happens now? (This is all to the best of my understanding):

  • Ascendis has 90 days to request a Type A meeting with the FDA. (Learn more about the Type A meeting process here)
  • This meeting will take place within 30 days of request.
  • After this meeting, a transcript will be released from the FDA within 30 days. Most companies wait to receive this transcript before resubmitting.
  • Per the Ascendis conference call, a review will take one of two tracks, a 2 month or 6 month resubmission. Per the call, Ascendis believes they will receive the 2 month track.
  • If TransCon PTH fails this review, Ascendis will have to start the entire New Drug Application review process over.

From The Pharma Letter:
“Ascendis said it believes it is well prepared to address the FDA’s concerns, and said it will request a Type A meeting with the FDA as soon as possible to agree on the best path forward.

According to FDA guidelines, Type A meetings are aimed at resolving disputes and helping stalled product-development programs proceed, with scheduling targeted to occur within 30 days of receipt of a written request.”

Most investor articles expect TransCon PTH to only have a few month delay and will be out by the end of the year. Obviously, it’s too early to tell, but this is looking much more promising than I initially expected. EAP continues to enroll patients in the US and are moving forward in other countries.

5/12/2023: I’ve been accepted into EAP for Transcon PTH, and was told I’m the first Natpara SUP patient scheduled to make the transition. I’m still working with my doctor and Ascendis on next steps but hopefully will transition in the next four weeks.

Amolyt Pharma moved to phase 3 trials on their PTH injection Eneboparatide

Extend Bio announced a successful phase 1A trial on their new once a week PTH injection EXT608

Bridge Bio is in phase 3 trials with their drug to treat hypocalcemia type 1 (ADH1)

5/24/2023: I will be starting TransCon PTH EAP on Wednesday, June 7. I have been informed I will not be titrating Natpara, but will have to stop taking Natpara upon my first injection per EAP guidelines. [Update 5/31/23: Patients must stop Natpara or Forteo immediately upon taking TransCon PTH. Titrating/overlapping medications causes a dangerous and uncomfortable side effect called Hungry Bone Syndrome (prolonged hypocalcemia).]

I will report back on how this goes, as TransCon doesn’t reach full potency for 5 days. As of today, I will be the first Natpara SUP patient to transfer directly to EAP, but I do know more have been approved. 

My doctor is confident that after his research, the FDA and Ascendis Pharma will solve the CRL letter/data request quickly and TransCon PTH should be approved in the next few months.

I have heard reports that some (US ONLY) doctors have struggled to get in touch with Ascendis in regards to applying to EAP. Here are some additional contact details:

Email: medinfo (at) ascendispharma (dot) com or 1-844-442-7236. If your US doctor is still unable to reach Ascendis, please contact me via social media or email via my contact page and I will do my best to assist. 

NOTE: My natpara-to-transcon blog is currently unavailable. I apologize for any inconvenience 


About Heather

When she’s not pretending to be a rock star with purple hair, award-winning author Heather Novak is crafting sex positive romance novels to make you swoon! After her rare disease tried to kill her, Heather mutated into a superhero whose greatest power is writing stories that you can’t put down.

Heather tries to save the world (like her late mom taught her) from her home near Detroit, Michigan, where she lives with Mr. Heather and a collection of musical instruments. She is part of the LGBTQ+ community and believes Black Lives Matter.

Special thanks to IG Kady.Hancock who made these comprehensive hypopara slides! 

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