Saturday, September 20, 2014

Growth Hormone Replacement can cause Adrenal Crisis

Health Alert: Adrenal Crisis Causes Death in Some People Who Were Treated with hGH

Doctors conducting the follow-up study of individuals treated with hGH looked at causes of death among recipients and found some disturbing news. Many more people have died from a treatable condition called adrenal crisis than from CJD. This risk does not affect every recipient. It can affect those who lack other hormones in addition to growth hormone. Please read on to find out if this risk applies to you. Death from adrenal crisis can be prevented. 

Adrenal crisis is a serious condition that can cause death in people who lack the pituitary hormone ACTH. ACTH is responsible for regulating the adrenal gland. Often, people are unaware that they lack this hormone and therefore do not know about their risk of adrenal crisis. 

Most people who were treated with hGH did not make enough of their own growth hormone. Some of them lacked growth hormone because they had birth defects, tumors or other diseases that cause the pituitary gland to malfunction or shut down. People with those problems frequently lack other key hormones made by the pituitary gland, such as ACTH, which directs the adrenal gland to make cortisol, a hormone necessary for life. Having too little cortisol can be fatal if not properly treated. 

Treatment with hGH does not cause adrenal crisis, but because a number of people lacking growth hormone also lack ACTH, adrenal crisis has occurred in some people who were treated with hGH. In earlier updates we have talked about how adrenal crisis can be prevented, but people continue to die from adrenal crisis, which is brought on by lack of cortisol. These deaths can be prevented. Please talk to your doctor about whether you are at risk for adrenal crisis. 

  • Why should people treated with hGH know about adrenal crisis? Among the people who received hGH, those who had birth defects, tumors, and other diseases affecting the brain lacked hGH and often, other hormones made by the pituitary gland. A shortage of the hormones that regulate the adrenal glands can cause many health problems. It can also lead to death from adrenal crisis. This tragedy can be prevented. 
  • What are adrenal hormones? The pituitary gland makes many hormones, including growth hormone and ACTH, a hormone which signals the adrenal glands to make cortisol, a hormone needed for life. If the adrenal gland doesn't make enough cortisol, replacement medications must be taken. The most common medicines used for cortisol replacement are: 
    • Hydrocortisone 
    • Prednisone 
    • Dexamethasone 
  • What is adrenal crisis? Adrenal hormones are needed for life. The system that pumps blood through the body cannot work during times of physical stress, such as illness or injury, if there is a severe lack of cortisol (or its replacement). People who lack cortisol must take their cortisol replacement medication on a regular basis, and when they are sick or injured, they must take extra cortisol replacement to prevent adrenal crisis. When there is not enough cortisol, adrenal crisis can occur and may rapidly lead to death. 
  • What are the symptoms of lack of adrenal hormones? If you don't have enough cortisol or its replacement, you may have some of these problems: 
    • feeling weak 
    • feeling tired all the time 
    • feeling sick to your stomach 
    • vomiting 
    • no appetite 
    • weight loss 

    When someone with adrenal gland problems has weakness, nausea, or vomiting, that person needs immediate emergency treatment to prevent adrenal crisis and possible death. 

  • Why are adrenal hormones so important? Cortisol (or its replacement) helps the body respond to stress from infection, injury, or surgery. The normal adrenal gland responds to serious illness by making up to 10 times more cortisol than it usually makes. It automatically makes as much as the body needs. If you are taking a cortisol replacement drug because your body cannot make these hormones, you must increase the cortisol replacement drugs during times of illness, injury, or surgery. Some people make enough cortisol for times when they feel well, but not enough to meet greater needs when they are ill or injured. Those people might not need cortisol replacement every day but may need to take cortisol replacement medication when their body is under stress. Adrenal crisis is extremely serious and can cause death if not treated promptly. Discuss this problem with your doctor to help decide whether you need more medication or other treatment to protect your health. 
  • How is adrenal crisis treated? People with adrenal crisis need immediate treatment. Any delay can cause death. When people with adrenal crisis are vomiting or unconscious and cannot take medicine, the hormones can be given as an injection. Getting an injection of adrenal hormones can save your life if you are in adrenal crisis. If you lack the ability to make cortisol naturally, you should carry a medical ID card and wear a Medic-Alert bracelet to tell emergency workers that you lack adrenal hormones and need treatment. This precaution can save your life if you are sick or injured. 
  • How can I prevent adrenal crisis? 
    • If you are always tired, feel weak, and have lost weight, ask your doctor if you might have a shortage of adrenal hormones. 
    • If you take hydrocortisone, prednisone, or dexamethasone, learn how to increase the dose when you become ill. 
    • If you are very ill, especially if you are vomiting and cannot take pills, seek emergency medical care immediately. Make sure you have a hydrocortisone injection with you at all times, and make sure that you and those around you (in case you're not conscious) know how and when to administer the injection. 
    • Carry a medical ID card and wear a bracelet telling emergency workers that you have adrenal insufficiency and need cortisol. This way, they can treat you right away if you are injured. 

Remember: Some people who lacked growth hormone may also lack cortisol, a hormone necessary for life. Lack of cortisol can cause adrenal crisis, a preventable condition that can cause death if treated improperly . Deaths from adrenal crisis can be prevented if patients and their families recognize the condition and are careful to treat it right away. Adrenal crisis is a medical emergency. Know the symptoms and how to adjust your medication when you are ill. Taking these precautions can save your life. 


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Page last updated September 24, 2012

Monday, September 15, 2014

Compassion Breeds Solidarity

My compassion for and interest in illnesses, especially the rare or mysterious, has exploded since my Cushing's diagnosis in 2007. I now can relate to the pain and suffering, the daily struggle, the dashed hopes and dreams punctured by mental and physical ailments. This is a hard life, we can all try out best, but wow, it's a lot to bare. I've also become friends with many mother-child pairs, as the parent fights for the diagnosis and treatment of the child's Cushing's. That experience in blood draws, midnight testing, brain MRIs, brain surgeries, and withdrawal symptoms is also my experience. I totally agree: this life is not glamorous at all.

Take a moment to read this story from a teenager who is chronically ill. As an aside, several Cushing's patients develop POTS after bilateral adrenalectomy, but we patients can not determine the pattern or relationship between the two illnesses. 

Sunday, September 14, 2014

Lessons of Alexis Shapiro: Texas Girl's Ordeal Spotlights Silent Crisis

Image: Alexis Shapiro 

Lessons of Alexis Shapiro: Texas Girl's Ordeal Spotlights Silent Crisis


A Texas pre-teen's medically induced obesity and subsequent slim-down has led her parents to a broader mission: Ensure that people with dangerous adrenal insufficiency get the right treatment in emergencies.

Alexis Shapiro's condition, caused by removal of a benign brain tumor three years ago, prompted nonstop hunger that pushed her weight above 200 pounds. Life-saving surgery in March has left her 50-plus pounds trimmer.

But her mother, Jenny Shapiro, said Alexis remains vulnerable to potentially lethal blackouts linked to adrenal insufficiency or AI — a little-known condition that a leading specialist says might be vastly underdiagnosed and could affect several million people in the U.S.

Alexis' condition must be painstakingly managed via oral steroids and 24/7 access to a needle and the drug Solu Cortef, which must be injected within 30 minutes after serious symptoms arise. Yet the vast majority of ambulances don't carry the drug.

Shapiro is banding with a network of"friends and family" impacted by AI to lobby for all EMS units to carry Solu Cortef — and to urge that all first responders learn more about the illness.

People with AI lack vital hormones produced by the adrenal glands. The paired organs sit atop the kidneys, flooding the body with chemicals that, among other things, maintain blood sugar, blood pressure and cardiac output during stress, said Dr. Michael Freemark, chief of pediatric endocrinology and diabetes at Duke University Medical Center.

Some people are born with AI. But far more — possibly "several million," said Freemark, who supports the push for EMS units to carry Solu Cortef — have secondary adrenal insufficiency caused by brain tumors (and their removals), strokes or long-term use of administered drugs like prednisone and hydrocortisone.

The worst symptoms of AI can include disorientation, shock, organ failure and death, unleashed by stress, sudden injury or illness.

"I told them she has adrenal insufficiency and was going to need her shot at the hospital. She was white as a sheet, shaking, going into crisis."

Two close calls with AI have stricken Alexis — the second aboard a jet as sheflew to Cincinnati last spring for the gastric-sleeve operation that curbed her ravenous appetite. (NBC News has followed Alexis' journey since December.)

But it was the first terrifying episode at her kitchen table about a year ago that launched Jenny Shapiro's latest fight for her daughter, who wears a medic-alert bracelet revealing she has AI. A chair accidentally landed on and fractured Alexis' pinky toe — and the injury triggered an adrenal crisis.

"Before the ambulance got there, she said everything was going dark," Sharpiro said.

The mother gave Alexis five steroid pills to swallow — what's known as a "stress dose." (She normally takes one pill in the morning, one at night.) She hoped her daughter would keep the meds down. Vomiting is a common symptom of adrenal crisis. Sharpiro also agonized about giving Alexis an emergency Solu Cortef shot in case her stomach heaved up the tablets. As the EMS team arrived, Shapiro urged them to rush Alexis to the hospital. They urged her to "calm down."

"A lot of paramedics don't know what it is. When they came to my house when Alexis was in crisis, they kind of blew me off when I kept asking if I should inject her. They didn't know what I was talking about," Shapiro said. "That, plus not having the medication on ambulances, creates a dangerous situation."

Image: Philip Hart, who died in 2009 at age 22 of an adrenal insufficiency crisis.

Philip Hart, who died in 2009 at age 22 of an adrenal insufficiency crisis.

Many people likely wouldn't learn they have AI until they lapse into a disorienting adrenal crisis, said Eric Hart. He lost his 22-year-old son, Philip, to an adrenal crisis caused by undiagnosed Addison's disease. Philip's adrenal crisis was triggered by a bout of flu.

His death spurred Hart to start, a nonprofit in the Raleigh, North Carolina, area that has persuaded a local EMS training school to list adrenal shock as an illness paramedics must consider when assessing patients.

"Like Alexis' parents discovered, Solu Cortef is not carried on ambulances — something we seek to change," Hart said. "This needs to go nationwide."

Adrenal Insufficiency United is spearheading the drive to raise awareness among medics and, perhaps eventually, equip every ambulance with a $7 vial of Solu Cortef, a corticosteroid that restores the body's crashing hormonal chemistry and helps people in adrenal crisis to start recovering.

The nonprofit, based in Eugene, Oregon, was formed by Jenny Knapp, whose 18-year-old daughter was born with AI (Knapp's daughter, who wears a medic-alert necklace, prefers not to be named).

Knapp's daughter has a horror story similar to Shapiro's. The triggering incident was a car accident.

"I did not have the Solu Cortef injection with me because my (daughter's doctor) told me EMS squads carry it and would give it to her," Knapp recalls. "The ambulance got there. They didn't have it. I told them she has adrenal insufficiency and was going to need her shot at the hospital. She was white as a sheet, shaking, going into crisis. They barely looked at her."

On Tuesday, Knapp's group launched anonline petition to establish new emergency policies to treat adrenal crises, emphasizing "timely treatment prevents needless disability and death." By Friday, more than 3,000 people had signed the petition.

"You have a $7 injection versus someone becoming permanently disabled or dying from this," Knapp said. "Is this a third-world country where people are dying because of (the lack of) a $7 injection on ambulances?

"I don't blame EMS (emergency medical services) for this," Knapp said. "What they hear in training about adrenal insufficiency is maybe a couple sentences. They want to help. I'd love all EMS to carry Solu Cortef. However, that's not going to work for (all EMS units). Our solution is: 'Why don't you (paramedics and EMTs) just inject the patient-carried meds?'"

But that option is unsafe, contends Dr. Alfred Sacchetti, an emergency-room physician in Camden, New Jersey. He also speaks for the American College of Emergency Physicians (ACEP), an organization that includes EMS directors.

"If they want the ambulance squads to give their kids Solu Cortef that someone pulls out of the refrigerator, that's not fair to an EMS squad," Sacchetti said. "The paramedics would think: 'What's really in this vial? How much do they get?' The whole thing is very suspect."

The better choice, Sacchetti asserts, is to treat people in adrenal crisis with Solu Medrol, a glucocorticoid carried by every EMS responder. That drug will "buy you time," stabilizing the patient until they reach a hospital, he added.

"I've talked to some of these parents at length: 'I understand, you've got a child with a special problem and I sympathize.' But I can't say to every EMS squad: 'You must carry all of these very unusual drugs for the three people in the state who have this disease.' You couldn't justify the cost," Sacchetti said.

Freemark, the AI specialist from Duke University Medical Center, disagrees with that stance — and with the number of people perhaps afflicted. He believes all EMS units should carry Solu Cortef — and should include AI among the assessment protocols used to evaluate patients in distress.

"It is a potentially life-threatening condition," Freemark said.