Advocacy Update- October 2021

To save the life of a cortisol deficient person in the event of an adrenal crisis, an emergency cortisol injection must be administered. But unfortunately most EMS personal in the United States are not only unware of this, but are not legally allowed to administer patient medication and most ambulances do not carry emergency cortisol injections onboard.

Adrenal Alternatives Foundation is actively working to change this on a federal, state and local level! We recently visited the campus of EMC Medical Training – Emergency Medical Consultants to provide materials and education on how to recognize and treat an adrenal crisis. This school not only trains EMS personnel but also offers CPR, First Aid, IV Therapy, Phlebotomy, EKG and Emergency Airway Management to all medical professionals.

Our goal in providing this school with copies of Adrenal Insufficiency 101 and pamphlets on how to manage an adrenal crisis is to prepare medical professionals who attend their classes to be able to recognize an adrenal crisis and how to administer an emergency injection.

Photo of EMC Instructor Lauren and AAF team member, Winslow E. Dixon

You can get involved too! Call or visit your local fire house and EMS station and advocate that they add adrenal crisis protocols. You can download full instructions on How to add Adrenal Crisis Protocols to your city’s EMS program from our website.

We appreciate all contributions which allow us to further our mission, improving access and awareness to all cortisol care options.

Donate to Adrenal Alternatives Foundation

Sign up for our member email list to get the latest updates.

Advocacy Update- No Patient Left Alone Expansion Proposal

Adrenal Advocacy Update

Adrenal Alternatives Foundation and CHronic illness advocacy & awareness Group (Ciaag) have joined together to address the serious issues COVID-19 restrictions are having on millions of citizens with rare diseases and/or chronic illnesses. Patients with continuous ongoing treatments such as dialysis, chemotherapy and IV infusion medications, now forced to be alone during these already difficult treatment sessions. This increases their suffering and has potential mental health implications, which then can adversely impact their overall health and wellbeing. 

There are great concerns regarding the increased potential for patient endangerment and medical errors in patients with rare disease protocols without caregivers and advocates present. Facilities, hospitals and treatment centers are citing COVID-19 as the rationalization behind restricting visitor access are not complying with the requirements of the Americans with Disabilities Act (ADA) which clearly states in Titles II and III that health care facilities are mandated to provide reasonable accommodations for persons with disabilities. 

These accommodations can include visitors who provide the patient with necessary support services, including communications, behavior/emotional support, and support managing the patients medications and other unique needs. There are several federal disability civil rights laws that apply to hospitals including, but not limited to, Title III of the Americans with Disabilities Act (ADA), Section 504 of the Rehabilitation Act (RA), and Section 1557 of the Patient Protection and Affordable Care Act (ACA). All of these statutes protect people with disabilities yet facilities, medical centers and hospitals across the nation are denying chronically ill patients a basic human rights of support and comfort of a loved one during medical experiences, citing COVID-19 restrictions as the reasoning. The ADA, RA, and ACA laws are still enforceable during the the COVID-19 pandemic.

The United States Department of Health and Human Services’ Office for Civil Rights issued a statement specifically reminding hospitals that they must “keep in mind their obligations under laws and regulations that prohibit discrimination on the basis of disability” and that the federal disability rights laws “remain in effect” even during the COVID-19 pandemic.

Our Proposed Solution: Standard legislation that mandates medical centers must follow existing guidelines in Title III of the Americans with Disabilities Act (ADA), Section 504 of the Rehabilitation Act (RA), and Section1557 of the Patient Protection and Affordable Care Act (ACA). There needs to be legislation that allows chronically ill patients to have a caregiver with them during medical procedures, treatments and surgeries even during a pandemic. 

Bill Proposal: Expanding the SB 730 – No Patient Left Alone Act BILL ANALYSIS: S730 contains the “No Patient Left Alone Act” which ensures the visitation rights of hospital patients during a period in which a disaster, emergency, or public health emergency has been declared.

GOAL: Proposing expansion on the No Patient Left Alone Act, originally passed in North Carolina. We are proposing a standard protocol outlined in a legislative bill that will mandate nationwide protocols that allow chronically ill patients to have a caregiver with them during medical procedures/treatments. 

If you would like to support our efforts please sign: Petition to Expand the SB 730:The No Patient Left Alone Act

If have been denied these rights: Print the DISABLED COVID RIGHTS PDF and provide it to the medical facility who has denied you.   

Remember, It’s Important to Know Your Rights!

SIGN THE PETITION, CLICK THE LINK HERE

Further reading can be found at the links below:

Covid Rights Initiative- Adrenal Alternatives Foundation

Hospitals Must comply with ADA rights

Supporting Family Caregivers in Providing Care

Supporting Family Caregivers in the Time of COVID-19 – State Strategies 

Hospitalized Adults need their caregivers – they aren’t visitors 

Caregivers are missing from the Conversation

SB 730 – No Patient Left Alone Act

This information has been brought to you by the Adrenal Alternatives Foundation and is not to be used to provide medical care or legal advice.

We appreciate all shares, contributions and donations which allow us to continue our mission, advocacy and access for all cortisol care.

Adrenal Advocacy Update – Summer 2021

Adrenal Advocacy Update: Summer 2021

Adrenal Alternatives Foundation is proudly representing cortisol deficiency in the rare disease congressional caucus. We are also proud to be a part of Rare Disease Week on Capitol Hill.

During Rare Disease week on capitol hill 2021: We are voicing our support of the following legislation:

The STAT Act

The Safe Step Act

The Benefit Act

we are also asking for attendance, endorsement and support FRom congressional represenatives foR OuR Upcoming disability inclusion event.

You can get involved too!

Contact your local representatives and ask them to co-sponsor the STAT Act, Benefit Act and the Safe Step Act to benefit all rare disease patients!

You can also invite them to attend the Disability Inclusion Event!

This information was brought to you by the Adrenal Alternatives Foundation for educational use only and is not meant to provide medical care or legal advice.

Sign up for our member email list to get the latest updates.

Adrenal Disease- Rare or Untested for?

When most people are diagnosed with adrenal disease, they are told it is a rare disease. Even though over approximately 1,000,000 people in the US and Europe have been diagnosed with cortisol related conditions.

Though it may be true that specific adrenal diseases are rare in occurrence, the incidence of diseases that impact cortisol and require the need for cortisol replacement medications are not as rare as endocrinologist believe. There are many forms of adrenal disease, but the treatment for all cortisol deficient conditions requires steroid replacement medications, leading us to believe that the cortisol deficient population is a large demographic.

Cortisol Deficient Conditions[1]

The following statistics indicate the prevalence of all cortisol related conditions that require cortisol replacement. All statistics are based on an annual scale in the United States unless otherwise noted. Research references are located in footnotes.

Over 1,000,000 people in the US and Europe have been diagnosed with cortisol related conditions such as:
Adrenal adenoma- According to the American Cancer Society[2], adrenal adenomas are found in 1 in every 10 people who have an imaging tests of the adrenal gland.
Adrenocortical carcinoma- It is estimated [3] that diagnosed 600 people are diagnosed in the United States each year.
Addison’s disease[4]– Prevalence estimated to be between 40 and 60 cases per 1 million in the general population.
Antley-Bixler syndrome- (ABS) has been described[5] in more than 100 patients.
Adrenoleukodystrophy[6]– According the Stop ALD Foundation, this condition affects 1 in 18,000 people.
Cortisol Dysregulation[7]– The WHO states that conditions where in which cortisol dysregulation is indicated, form a part of a group of illnesses termed as “noncommunicable diseases” (NCDs) accounts for the majority of deaths with 17.9 million people dying annually. Noncommunicable diseases (NCDs) kill 41 million people each year worldwide, equivalent to 71% of all deaths globally[8]. In the Americas, 5.5 million deaths are by NCDs. Research[9] also highlights the fact that cortisol dysregulation is present in up to 40% of stroke patients.  
Cushing’s disease – An estimated [10]10-15 per million people are diagnosed with this condition in the United States every year.
Congenital adrenal hyperplasia (CAH)[11]– The most common form of CAH, 21 hydroxylase deficiency, affects approximately 1:10,000 to 1:15,000 people in the United States and Europe. Among the Yupik Eskimos, the occurrence of the salt-wasting form of this disorder may be as high as 1 in 282 individuals.
11-Beta hydroxylase deficiency[12]– It is estimated that 11-beta-hydroxylase deficiency occurs in 1 in 100,000 to 200,000 newborns. This condition is prevalent in Moroccan Jews living in Israel and occurs in approximately 1 in 5,000 to 7,000 births.
17a-hydroxylase deficiency[13]– 17a hydroxylase deficiency has an estimated prevalence of 1 in 50,000 to 100,000.
3-Beta-hydroxysteroid dehydrogenase deficiency[14]– Human 3 beta-hydroxysteroid dehydrogenase deficiency (3b-HSD) resulting from HSD3B2 gene mutations has an estimated diagnosis of less than 1/1,000,000.
Congenital lipoid adrenal hyperplasia[15]– Exact statistics for Americans diagnosed with this condition are unknown, however recent studies estimate the rate for this mutation seen in Asian populations to be between 1 in 200 and 1 in 300 people yearly.
PORD (P450 oxidoreductase deficiency)[16] – Estimated annual incidence of 1/100,000-200,000.
Hypopituitarism[17]– Globally, the prevalence is estimated to be 4.2 cases per 100,000 per year, and the prevalence is approximately 45.5 cases per 100 000 people.
Panhypopituitarism[18]– Estimated annual prevalence of 45.5 cases per 100,000 people.
Pheochromocytoma[19]– The prevalence is estimated at 1:2,500 to 1:6,500 patients annually.
Pituitary Adenoma[20]– 9.3 cases per 100,000 population yearly.
Secondary Adrenal Insufficiency[21]– Yearly estimated prevalence is 150–280 per million.
Sheehan’s syndrome[22]– Incidence of Sheehan syndrome is estimated to be 5 patients out of 100,000 births.
Lymphocytic Lypophysitis[23]– The prevalence is approximately 1 in 9 million.

The WHO states that conditions where in which cortisol dysregulation is indicated, form a part of a group of illnesses termed as “noncommunicable diseases” (NCDs) accounts for the majority of deaths with 17.9 million people dying annually. Noncommunicable diseases (NCDs) kill 41 million people each year worldwide, equivalent to 71% of all deaths globally. In the Americas, 5.5 million deaths are by NCDs. Current Research also highlights the fact that cortisol dysregulation is present in up to 40% of stroke patients.

Though cortisol testing is inexpensive and easily performed, in emergency room situations it is not a current standard of care.

This information begs the question, how many unknown causes of death are truly caused by cortisol deficiency?

Another factor in current endocrine diagnostics is that early detection is key to quality of life for those with cortisol deficient diseases. Research shows that cortisol deficient patients diagnosed post adrenal crisis suffer from lowered quality of life and most are declared disabled. Diagnosis prior to adrenal crisis is essential to preventing impairments.

Despite research supported, legal, medical advances, cortisol deficiency patients remain a poorly served demographic with high mortality rates and low quality of life.

Barriers In current cortisol care

  1. Cortisol testing in emergency room situations is not a current standard of care.
  2. There is currently no center in the United States soley dedicated to managing cortisol care. Vital testing to manage cortisol deficiency such as 24-hour cortisol day curve testing is not available. This test evaluates the effectiveness of a patient’s cortisol replacement medication by drawing a cortisol blood lab once an hour for 24 hours and provides insight on a patient’s cortisol metabolism and absorption.
  3. Patient’s cortisol levels are tested initially at diagnosis but typically are not tested after diagnosis to evaluate effectiveness of their steroid replacement.
  4. Comparative assays of blood, urine and saliva testing to help establish a correct steroid dosing milligram and regiment are not offered in standard endocrinology clinics.
  5. Cortisol deficiency patients are not typically given multiple options to manage their care. The lack of cortisol replacement options is likely due to poor clinician awareness on the latest research showing standard administration of hydrocortisone 2-3x daily is inadequate to replicate the body’s natural cortisol production.
  6. Advances in cortisol replacement medications are available in the United States, but awareness of options such as all steroid medications and alternative cortisol delivery methods such as subcutaneous injections and the cortisol pumping method are not prominent.
  7. There is currently no way to check blood serum cortisol levels outside of a laboratory setting.
  8. Education on the proper management of cortisol deficiency is scarce within the endocrine community, who are mainly educated on diabetes and thyroid conditions. Advances such as circadian rhythm dosing can make massive improvements in quality of life for adrenal patients, yet many endocrinologists are unaware of this advancement.
  9. All EMS personnel are not authorized to administer the life saving cortisol injection in the event of an adrenal crisis.

Solutions to CUrrent Barriers in Cortisol Care:

  1. Early detection and diagnosis prior to experiencing adrenal crisis.
  2. Access to and awareness of all steroid options, which includes oral medications, subcutaneous cortisol injections and the cortisol pumping method. 
  3. Access to and awareness of testing that can evaluate cortisol deficient conditions and provide clinicians with insight on better cortisol regimens and replacement methods.
  4. Education on the importance of medication compliance and the need to increase dosing in times of stress, sickness or injury.
  5. Advancements in research to create biotechnology such as a cortisol meter.

How ADrenal Alternatives Foundation intends to solve these problems

Adrenal alternatives foundation is in the process of obtaining the funding to open a cortisol care center to provide appropriate cortisol testing and all possible care options to manage conditions that result in cortisol deficiency or dysregulation. Advances in cortisol care are available, but awareness and access to these options are limited and not widely offered.

Adrenal Alternative’s Cortisol Care Center will be a centralized location for all conditions that result in cortisol deficiency and cortisol dysregulation to have access to all steroid options, which includes oral medications, subcutaneous cortisol injections and the cortisol pumping method. Our center will provide diagnostic testing to screen for cortisol deficiency and dysregulation and also provide comparative lab assays of blood, urine and saliva cortisol levels, testing protocols such as the Cortisol Day Curve and the testing of all adrenal hormones to help patients find better quality of life.  

Our goal is to save and improve lives.

We appreciate all contributions which allow us to further our mission, Education, Advocacy and Awareness for all adrenal disease and will help us get closer to our goal of making the cortisol care center a reality.

Donate to Adrenal Alternatives Foundation

This information was brought to you by the Adrenal Alternatives Foundation for educational use only and is not meant to provide medical care or advice.

Sign up for our member email list to get the latest updates.

Sources:

[1] Types of Adrenal Gland Disorders

https://www.nichd.nih.gov/. (2020). Types of adrenal gland disorders. [online] Available at: https://www.nichd.nih.gov/health/topics/adrenalgland/conditioninfo/types

[2] American Cancer Society. https://www.cancer.org/cancer/adrenal-cancer/about/key-statistics.html#:~:text=Adrenal%20tumors%20(most%20of%20which,any%20age%2C%20even%20in%20children.

[3] Adrenal Gland Tumor: Statistics. American Society of Clinical Oncology (ASCO) Cancer.Net Editorial Board, 01/2021

[4] Addison’s Disease Rare Disease Database. National Organization for Rare Disease (NORD)  https://rarediseases.org/rare-diseases/addisons-disease/

[5] Antley-Bixler syndrome Orphanet Clinical genetics review (2017) https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=83

[6] Stop ALD Foundation.  Available at: http://www.stopald.org/what-is-ald

[7] Cortisol level dysregulation and its prevalence—Is it nature’s alarm clock? Physiol Rep. 2021 Jan; 8(24): e14644.

Published online 2020 Dec 19. doi: 10.14814/phy2.14644 PMCID: PMC7749606 PMID: 33340273. Available at:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7749606/

[8] Chronic noncommunicable diseases (NCDs) Americas of the World Health Organization. Pan American Health Organization. Available at: https://www.paho.org/en/topics/noncommunicable-diseases

[9] Olsson, T. , Marklund, N. , Gustafson, Y. , & Nasman, B. (1992). Abnormalities at different levels of the hypothalamic‐pituitary‐adrenocortical axis early after stroke. American Heart Association Journal: Stroke, 23(11), 1573–1576. [PubMed] [Google Scholar]

[10] Cushing’s Syndrome/Disease. 2021 American Association of Neurological Surgeons. https://www.aans.org/en/Patients/Neurosurgical-Conditions-and-Treatments/Cushings-Disease

[11] Congenital Adrenal Hyperplasia. Rare Disease Database. National Organization for Rare Disease (NORD) Available at: https://rarediseases.org/rare-diseases/congenital-adrenal-hyperplasia/#:~:text=The%20most%20common%20form%20of,as%201%20in%20282%20individuals.

[12] Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency. Genetics Home Reference. Available at: https://medlineplus.gov/genetics/condition/congenital-adrenal-hyperplasia-due-to-11-beta-hydroxylase-deficiency/#references

[13] A case of 17 alpha-hydroxylase deficiency. Clin Exp Reprod Med. 2015 Jun; 42(2): 72–76. Published online 2015 Jun 30. doi: 10.5653/cerm.2015.42.2.72 PMCID: PMC4496435. PMID: 26161337 Sung Mee Kim1 and Jeong Ho. Saint Mary’s Women’s Hospital, Daegu, Korea. Department of Obstetrics and Gynecology, Keimyung University College of Medicine, Daegu, Korea. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4496435/

[14] Human 3 beta-hydroxysteroid dehydrogenase deficiency associated with normal spermatic numeration despite a severe enzyme deficit. Endocr Connect. 2018 Mar; 7(3): 395–402. Published online 2018 Feb 2. doi: 10.1530/EC-17-0306. PMCID: PMC5827574. Available at:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5827574/#__ffn_sectitle

[15] Lipoid Congenital Adrenal Hyperplasia: Genetic Steroid Disorders, 2014. ScienceDirect ® Elsevier B.V. Available at: https://www.sciencedirect.com/topics/neuroscience/lipoid-congenital-adrenal-hyperplasia

[16] Cytochrome P450 Oxidoreductase Deficiency. Jan Idkowiak, MD, PhD, Deborah Cragun, MS, CGC, Robert J Hopkin, MD, and Wiebke Arlt, MD, DSc. GeneReviews® Adam MP, Ardinger HH, Pagon RA, Seattle (WA): University of Washington, Seattle; Initial Posting: September 8, 2005; Last Update: August 3, 2017. Available at: https://www.ncbi.nlm.nih.gov/books/NBK1419/

[17]  Hypopituitarism Epidemiology. Yolanda Smith, B.Pharm, Dr. Liji Thomas, MD. Medical News Life Sciences. News-Medical.net. Available at: https://www.news-medical.net/health/Hypopituitarism-Epidemiology.aspx#:~:text=Hypopituitarism%20is%20considered%20to%20be,cases%20per%20100%20000%20people.

[18] Panhypopituitarism. Bernard Corenblum, MD, FRCPC Professor of Medicine, Director, Endocrine-Metabolic Testing and Treatment Unit, Ovulation Induction Program, Department of Internal Medicine, Division of Endocrinology, University of Calgary Faculty of Medicine, Canada. Medscape.

Available at: https://emedicine.medscape.com/article/122287-overview#:~:text=Internationally%2C%20hypopituitarism%20has%20an%20estimated,per%20100%2C000%20without%20gender%20difference.

[19] Pheochromocytoma In: Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000–.

2013 Jun 10. PMID: 25905204 Bookshelf ID: NBK278970 Available at: https://pubmed.ncbi.nlm.nih.gov/25905204/

[20] The Journal of Clinical Endocrinology & Metabolism, Volume 91, Issue 12, 1 December 2006, Pages 4769–4775, https://doi.org/10.1210/jc.2006-1668

[21] Adrenal Insufficiency. National Center for Biotechnology Information, U.S. National Library of Medicine

Nicolas C Nicolaides, M.D., George P Chrousos, MD, MACE, MACP, FRCP, and Evangelia Charmandari, M.D. Available at: https://www.ncbi.nlm.nih.gov/books/NBK279083/#:~:text=Secondary%20adrenal%20insufficiency%20occurs%20more,life%20(18%2C%2019).

[22] Sheehan Syndrome. Mark P. Schury; Rotimi Adigun. StatPearls [Internet]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK459166/

[23] Hypophysitis: Evaluation and Management. Clin Diabetes Endocrinol. 2016; 2: 15. Published online 2016 Sep 6. doi: 10.1186/s40842-016-0034-8 PMCID: PMC5471685.PMID: 28702249, Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471685/#__ffn_sectitle

Adrenal patients and diabetics do not have equal access to life saving infusion pumps

Type 1 Diabetes is the disease where the pancreas fails to produce the correct amount of insulin, thus rendering someone insulin dependent.

Adrenal insufficiency is a disease where the adrenal glands fail to produce the proper amounts of steroid hormones and requires lifelong steroid medication for cortisol replacement.

Unlike diabetic patients who have glucometers, adrenal disease sufferers have no meter to check their cortisol levels. They must be constantly vigilant of their own low cortisol symptoms. The excitement from a happy event, the sadness from a death of a loved one or the strain from exercising are examples of things that would cause the body to release more cortisol. In an adrenal insufficient person, this does not happen. They must artificially manage their cortisol, which can differ from day to day. They require an emergency cortisol injection if their levels drop to a critical point.

Hydrocortisone is the standard cortisol replacement medication, which only has a blood serum half-life of 90 minutes and must be taken multiple times a day. This medication must be processed through the stomach and the liver before reaching the blood stream.  This causes an abnormal rise and fall of cortisol levels, which results in subpar function, increases mortality rate and decreases quality of life.

However, advances in research have found a solution for adrenal patients. The concept of Cortisol Pumping is the use of solu-cortef (inject-able version of cortisol when mixed with saline) used in an insulin pump programmed to disperse cortisol according to the natural circadian rhythm. This method bypasses the gastric passage and delivers cortisol in a more natural way. With this method, an adrenal insufficient patient can receive cortisol constantly. Side effects due to malabsorption are decreased and patients have been reported to have improved sleep, weight management and experience an overall improvement in their quality of life. This method has also been proven to lessen the prevalence of adrenal crises and lessen hospitalizations due to low cortisol.

Despite the vast amount of research showing the efficacy of this treatment, infusion pumps are not offered to adrenal insufficient patients. Unlike diabetics, they do not have easy access to infusion pumps and supplies.

The Right to Try Act which was passed in 2018, mandated that patients now have legal rights to access life-saving treatments which are not yet FDA approved. Adrenal Alternatives Foundation is a 501c3 organization dedicated to providing adrenal insufficient patients with the resources to safely and legally begin the cortisol pumping method. The nonprofit works to educate physicians on how to manage this protocol. They also assist patients in obtaining insurance approval and their “Pumps for Purpose Program” helps provide pumps and supplies to adrenal patients, with or without insurance coverage. They have also created the resources: Cortisol Pump Guide Book and the Cortisol Pumping website to help guide patients in their pumping journey. Their hope is that one day, there will be disease equality and both adrenal patients and diabetics will both have easy access to infusion pumps.

This information was brought to you by the Adrenal Alternatives Foundation for educational use only and is not meant to provide medical care or legal advice.

Sign up for our member email list to get the latest updates.

We appreciate all contributions which allow us to further our mission, Education, Encouragement and Advocacy.

Donate to Adrenal Alternatives Foundation