Diabetes Insipidus

What is diabetes insipidus?

Diabetes insipidus is a rare but treatable condition in which your body produces too much urine (pee) and isn’t able to properly retain water. Diabetes insipidus can be chronic (life-long) or temporary and mild or severe depending on the cause.

Diabetes insipidus is mostly caused by an issue with a hormone called antidiuretic hormone (ADH, or vasopressin) — either your body doesn’t make enough of ADH or your kidneys don’t use it properly.

People with diabetes insipidus pee large volumes of urine several times a day and drink large amounts of water because they feel constantly thirsty. If you have diabetes insipidus and don’t drink enough fluid to replace water loss through urine, you can become dehydrated, which is dangerous to your health.

Because of this, diabetes insipidus is a serious condition that requires medical treatment.

What is the antidiuretic hormone (ADH, or vasopressin)?

Antidiuretic hormone (ADH, or vasopressin) is a hormone that your hypothalamus makes and your pituitary gland stores and releases.

Hormones are chemicals that coordinate different functions in your body by carrying messages through your blood to your organs, skin, muscles and other tissues. These signals tell your body what to do and when to do it.

Your hypothalamus is the part of your brain that controls your autonomic nervous system and the activity of your pituitary gland. It makes ADH and then sends it to your pituitary gland for storage and release.

Your pituitary gland is a small gland located at the base of your brain below your hypothalamus. It’s a part of your endocrine system and is in charge of making, storing and releasing many different important hormones

ADH helps regulate the water balance in your body by controlling the amount of water your kidneys reabsorb while they’re filtering waste out of your blood. Your body normally produces and releases more ADH when you’re dehydrated or losing blood pressure. The increase in ADH tells your kidneys to hold onto more water instead of releasing it in your pee (urine).

In most cases of diabetes insipidus, your hypothalamus isn’t making enough ADH, your pituitary gland isn’t releasing enough ADH or your kidneys aren’t properly using ADH. This causes frequent and excessive water loss through your urine.

As an example, if a person without diabetes insipidus were in the desert with no access to water, their body would produce more ADH and retain as much water as possible. A person with diabetes insipidus would continue to urinate water and would quickly become dehydrated.

What is the difference between diabetes insipidus and diabetes mellitus?

Diabetes insipidus and diabetes mellitus are two distinct conditions with different causes and treatments. They share the name “diabetes” because they both cause increased thirst and frequent urination. “Diabetes” comes from the Greek word “diabainein,” which means “go through” — much like the liquids that quickly go through your body in these conditions.

Diabetes mellitus, most commonly known as Type 1 diabetes, Type 2 diabetes or gestational diabetes, happens when your pancreas doesn’t make any or enough insulin or your body doesn’t use the insulin it makes properly. Your body needs insulin to transform the food you eat into energy.

If your body doesn’t have insulin to transform glucose into energy, it starts breaking down fat and muscle for energy instead, which produces a substance called ketones. Too many ketones can turn your blood acidic, so your body tries to get rid of them through your urine. Because of this, symptoms of diabetes often include extreme thirst and frequent urination.

Diabetes insipidus happens when your body doesn’t make enough antidiuretic hormone (ADH) or your kidneys don’t use it properly. Your body needs ADH to retain appropriate amounts of water. Without ADH, your body loses water through urine.

Diabetes mellitus is much more common than diabetes insipidus.

What are the types of diabetes insipidus?

There are four types of diabetes insipidus, including:

• Central diabetes insipidus: This is the most common type of diabetes insipidus. It happens when your body doesn’t have enough antidiuretic hormone (ADH, or vasopressin). Your hypothalamus produces ADH, but your pituitary gland stores and releases it. You can get central diabetes insipidus if your pituitary gland or hypothalamus is damaged.
• Nephrogenic diabetes insipidus: This type of diabetes insipidus happens when your pituitary gland releases enough ADH, but your kidneys don’t respond to it properly and can’t retain water.
• Dipsogenic diabetes insipidus: In this type of diabetes insipidus, an issue with your hypothalamus unrelated to ADH production causes you to feel thirsty and drink more liquids. Because of this, you may need to pee often.
• Gestational diabetes insipidus: This is a rare, temporary condition that can develop during pregnancy. Gestational diabetes insipidus happens occurs when your placenta, a temporary organ that provides nourishment to your baby, makes too much of an enzyme that breaks down your ADH. Women who are pregnant with more than one baby are more likely to develop the condition because they have more placental tissue. Gestational diabetes insipidus usually goes away shortly after the pregnancy is over. It’s not to be confused with gestational diabetes, which is a type of diabetes mellitus that can develop during pregnancy in people who don't already have diabetes mellitus. Gestational diabetes mellitus causes high blood sugar.

Who does diabetes insipidus affect?

People of all ages can develop diabetes insipidus. Gestational diabetes insipidus specifically affects pregnant women, though it’s rare.

How common is diabetes insipidus?

Diabetes insipidus is a rare condition. It affects about 1 in 25,000 people worldwide.

What are the symptoms of diabetes insipidus?

The main symptoms of diabetes insipidus include:

• Needing to pee often, including through the night.
• Passing large amounts of light-colored or clear urine each time you pee.
• Feeling very thirsty and drinking liquids very often.

While most people produce one to three quarts of urine a day, people with diabetes insipidus can produce up to 20 quarts of urine a day.

If diabetes insipidus isn’t treated or if a person with diabetes insipidus stops drinking fluids, it can quickly lead to dehydration.

Symptoms of dehydration include:

• Feeling dizzy or lightheaded.
• Feeling tired.
• Having a dry mouth, lips and eyes.
• Difficulty performing simple mental tasks.
• Nausea.
• Fainting.

If you’re experiencing extreme thirst and frequent urination and/or dehydration, it’s important to go to the hospital as soon as possible.

While diabetes insipidus is rare, it’s not the only condition that causes these symptoms. Extreme thirst and frequent urination are significant signs that something in your body isn’t working properly and needs medical treatment.

What causes diabetes insipidus?

In general, diabetes insipidus is caused by problems with how your body makes or uses antidiuretic hormone (ADH, or vasopressin), which helps your kidneys balance the amount of fluid in your body. The exact causes vary for the different types of diabetes insipidus. Sometimes, healthcare providers can’t determine the cause.

Causes of central diabetes insipidus

Central diabetes insipidus happens when there’s an issue with your hypothalamus or pituitary gland. Specific causes include:

• Damage to your hypothalamus or your pituitary gland from surgery.
• Damage to your hypothalamus or pituitary gland from a head injury, particularly basal skull fractures.
• Inflammation (granulomas) from sarcoidosis or tuberculosis.
• Tumors that affect your hypothalamus or pituitary gland.
• An autoimmune reaction that causes your immune system to damage healthy cells that make an antidiuretic hormone (ADH).
• An inherited gene mutation on chromosome 20.

Causes of nephrogenic diabetes insipidus

Nephrogenic diabetes insipidus happens when your kidneys don’t use antidiuretic hormone (ADH, or vasopressin) properly. Specific causes include:

• Certain medications, such as lithium and tetracycline.
• Low levels of potassium in your blood (hypokalemia).
• High levels of calcium in your blood (hypercalcemia).
• A blocked urinary tract.
• An inherited gene mutation. The hereditary form of nephrogenic diabetes insipidus can be caused by mutations in at least two genes. About 90% of hereditary nephrogenic diabetes insipidus cases are due to mutations in the AVPR2. Most of the remaining 10% of cases are due to mutations in the AQP2 gene.
• Chronic kidney disease (this is a rare cause).

Causes of dipsogenic diabetes insipidus

Dipsogenic diabetes insipidus (also known as primary polydipsia) happens when there’s an issue with your hypothalamus unrelated to ADH production that causes you to feel thirsty and drink more liquids. Specific causes include:

• Damage to your hypothalamus from surgery, infection, inflammation, a tumor or a head injury.
• Certain mental health conditions, including schizophrenia and obsessive-compulsive disorder.

Causes of gestational diabetes insipidus

Gestational diabetes insipidus is a rare condition that affects pregnant women. It happens when your placenta makes too much of a certain enzyme that breaks down your antidiuretic hormone (ADH, or vasopressin). If you’re pregnant with more than one baby, you’re more likely to develop gestational diabetes insipidus because you have more placental tissue.

How is diabetes insipidus diagnosed?

As other conditions, including diabetes mellitus, cause frequent thirst and urination, part of the process of diagnosing diabetes insipidus involves ruling out other conditions. Because of this, your healthcare provider may order several tests if you’re experiencing these symptoms.

A diagnosis of diabetes insipidus also involves determining the type and cause of it.

What tests will be done to diagnose diabetes insipidus?

A water deprivation test is the simplest and most reliable method for diagnosing diabetes insipidus. If your provider orders this test, they’ll make sure you’re under constant supervision during the process, as it can cause dehydration.

A water deprivation test involves not drinking any liquid for several hours to see how your body responds. If you have diabetes insipidus, you'll continue to pee large amounts of watery (dilute), light-colored urine when normally you'd only pee a small amount of concentrated, dark yellow urine.

Your provider may also order the following tests to help diagnose diabetes insipidus or rule out other conditions:

• Blood test to check antidiuretic hormone (ADH, or vasopressin) levels.
• Blood test to check glucose levels to rule out diabetes mellitus.
• Urinalysis to check osmolality (the concentration of your urine) and/or to check for ketones, which could indicate diabetes mellitus.
• Imaging tests, such as an MRI, to see if issues with your pituitary gland or hypothalamus are causing your diabetes insipidus.

How is diabetes insipidus treated?

In some cases, diabetes insipidus can’t be cured, but it can be managed with medications. Treatment for diabetes insipidus depends on what type you have.

Treatment for central diabetes insipidus and gestational diabetes insipidus

Desmopressin is the first-line treatment for central diabetes insipidus. It’s a medication that works like an antidiuretic hormone (ADH, or vasopressin). You can take desmopressin as an injection (shot), a pill or in a nasal spray.

Healthcare providers also sometimes use desmopressin to treat gestational diabetes insipidus.

Treatment for nephrogenic diabetes insipidus

Treatment for nephrogenic diabetes insipidus is more complicated and sometimes involves a combination of approaches.

Healthcare providers often treat it with medications called thiazide diuretics, which reduce the amount of urine your kidneys produce. Your provider may also suggest taking nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, to help further reduce urine volume when they’re used in combination with thiazide diuretics. If your medication causes nephrogenic diabetes insipidus, your healthcare provider can sometimes treat it by changing your medication.

It might take a while to find the right combination that works best for you.

Treatment for dipsogenic diabetes insipidus

Researchers haven’t yet found an effective way to treat dipsogenic diabetes insipidus. If an underlying condition causes it, such as a mental health condition, treatment might be directed at that cause. If you wake up many times at night to pee, your provider may recommend you take a small dose of desmopressin at bedtime.

What are the side effects of desmopressin?

Desmopressin is generally very safe to use and has few side effects.

Possible side effects can include:

• Headache.
• Abdominal pain.
• Nausea.
• A blocked or runny nose.
• Nosebleeds.

If you take too much desmopressin or drink too much fluid while taking it, it can cause your body to retain too much water, which can cause the following symptoms:

• Headaches
• Dizziness.
• Feeling bloated.
• A low level of sodium (salt) in your blood (hyponatremia).

Symptoms of hyponatremia include:

• A severe or prolonged headache.
• Confusion.
• Nausea and vomiting.

If you’re experiencing symptoms of hyponatremia, call 911 or go to the nearest hospital as soon as possible.

What can I do to manage my diabetes insipidus?

If you have diabetes insipidus, aside from medical treatment, it’s important to drink water regularly to make sure you don’t get dehydrated.

If you have a mild case of nephrogenic diabetes insipidus, your healthcare provider may recommend reducing the amount of salt and protein in your diet, which will help your kidneys produce less urine. It’s important to always talk to your provider before drastically changing your diet.

If you have diabetes insipidus, it’s also important to see your provider regularly to make sure your treatment is working and that you have the right dosage of medication.

What are the risk factors for developing diabetes insipidus?

You’re more likely to develop diabetes insipidus if you:

• Have a family history of diabetes insipidus.
• Had brain surgery or a major head injury.
• Take medications that can cause kidney problems.
• Have certain metabolic disorders, such as high blood calcium levels or low blood potassium levels.

If you’re pregnant, you’re at a higher risk of developing gestational diabetes insipidus if you:

• Are pregnant with more than one baby.
• Have a condition that affects liver function, such as preeclampsia and HELLP syndrome.

What is the prognosis (outlook) for diabetes insipidus?

The outlook (prognosis) for diabetes insipidus is generally good, and it usually doesn’t cause serious problems as long as it’s treated properly and you drink enough water.

The risk of complications and death is higher for infants, seniors and those who have mental health conditions because they may have trouble recognizing their thirst or they can’t do anything about it.

What are the complications of diabetes insipidus?

The main complication of diabetes insipidus is dehydration, which happens when your body loses too much fluid and electrolytes to work properly. If you have diabetes insipidus, you can usually make up for the significant volume of fluids you pass in your urine by drinking more liquids. But if you don’t, you could quickly become dehydrated.

Dehydration is dangerous and can be life-threatening. If you’re experiencing symptoms of dehydration, such as feeling dizzy, nauseous and sluggish, get to the nearest hospital as soon as possible.

When should I see my healthcare provider?

If you have diabetes insipidus, it’s important to see your healthcare provider regularly so they can run tests to monitor your condition and make sure your treatment is working. If you’re experiencing any concerning symptoms, be sure to contact your provider.

What questions should I ask my healthcare provider?

If you’ve been diagnosed with diabetes insipidus, it may be helpful to ask your healthcare provider the following questions:

• What type of diabetes insipidus do I have?
• What caused my diabetes insipidus?
• Is my diabetes insipidus chronic or temporary?
• What are my treatment options?
• What are the benefits and risks of different treatment options?
• How much water should I be drinking in a day?
• Is there anything else I can do to manage my condition?
• Are my family members at risk for developing diabetes insipidus?