Diabetes Insipidus
Diabetes insipidus is a rare condition that makes your body lose its ability to balance water properly. While most people make 1 to 3 quarts of urine a day, people with this condition can make up to 20 quarts, leading to constant thirst and frequent trips to the bathroom day and night.
Table of contents
- What is diabetes insipidus?
- Other names for this condition
- Symptoms
- Causes and types
- How doctors diagnose diabetes insipidus
- Treatment options
- Possible complications
- Living with diabetes insipidus
What is diabetes insipidus?
Diabetes insipidus is an uncommon condition that causes the fluids in your body to become out of balance[1]. This happens when your kidneys are unable to prevent too much water from leaving your body[5]. The condition affects about 1 in 25,000 people in the general population[2].
The disease gets its name because it causes you to produce large amounts of urine, similar to diabetes mellitus. However, diabetes insipidus is completely different from type 1 diabetes or type 2 diabetes (also called diabetes mellitus). In diabetes insipidus, your blood sugar levels are normal, but your kidneys can’t properly balance the amount of water in your body[2].
The condition is caused by problems with a hormone called arginine vasopressin (AVP), also known as antidiuretic hormone (ADH)[4]. This hormone plays a key role in helping your kidneys retain water in your body. It’s produced in a part of the brain called the hypothalamus and stored in the pituitary gland, a small gland at the base of your brain[4].
arginine vasopressin deficiency, arginine vasopressin resistance, water diabetes
Other names for this condition
Healthcare providers may refer to diabetes insipidus by several other names. It is also called arginine vasopressin deficiency and arginine vasopressin resistance[1]. Some doctors call it “water diabetes” because, like diabetes mellitus, it results in large amounts of urine, but unlike diabetes mellitus, this frequent urination is not from high blood sugar[15].
Symptoms
The two main symptoms of diabetes insipidus are extreme thirst and making large amounts of urine[4]. These symptoms can significantly affect daily life and sleep patterns.
In adults, the common symptoms include being very thirsty, often with a preference for cold water, and making large amounts of pale or clear urine[1]. Many people need to get up to urinate and drink water often during the night[1]. While most healthy adults pass urine 4 to 7 times in a 24-hour period, people with diabetes insipidus may urinate much more frequently[4].
Most people make 1 to 3 quarts of urine a day. People with diabetes insipidus can make up to 20 quarts of urine a day[2]. The urine is very diluted and pale in color[5].
In babies and young children, symptoms may appear differently. A baby or young child who has diabetes insipidus may have large amounts of pale urine that result in heavy, wet diapers, and may experience bed-wetting[1]. Children may show extreme thirst, with a preference for drinking water and cold liquids[1]. Other symptoms in children can include weight loss, poor growth, vomiting, irritability, fever, constipation, headache, problems sleeping, and vision problems[1].
If you or your child experiences constant thirst or urinates much more than usual, you should see your healthcare provider right away[1]. These symptoms are important signs that something in your body isn’t working as it should, and it’s essential to get medical help as soon as possible[3].
Causes and types
Diabetes insipidus happens when there’s a problem with how your body makes or uses arginine vasopressin (AVP). The pituitary gland releases AVP when the amount of water in your body becomes too low. It helps retain water in the body by reducing the amount of water lost through the kidneys, making the kidneys produce more concentrated urine[4].
There are several types of diabetes insipidus, each with different causes:
Arginine vasopressin deficiency (AVP-D), formerly known as central diabetes insipidus, is the most common type[4]. This happens when your body doesn’t make enough AVP. It can be caused by damage to the hypothalamus or pituitary gland from a head injury, brain tumor, brain surgery, infection in the brain such as meningitis or encephalitis, loss of blood supply to the pituitary gland, or inflammation from conditions like sarcoidosis or tuberculosis[5]. Some cases are caused by an inherited gene variation[3]. In about one-third of cases, no obvious reason can be found for why the hypothalamus stops making enough AVP[4].
Arginine vasopressin resistance (AVP-R), formerly known as nephrogenic diabetes insipidus, occurs when your body makes enough AVP, but your kidneys don’t respond to it properly[3]. This type can be caused by certain medications, particularly lithium used to treat bipolar disorder, kidney disease, an inherited gene variation, a blocked urinary tract, or imbalances in body minerals such as too much calcium (hypercalcemia) or too little potassium (hypokalemia)[3]. Long-term lithium use can damage the cells of the kidneys so they no longer respond to AVP, with up to 2 in 5 people on long-term lithium therapy developing some degree of AVP-R[8].
Gestational diabetes insipidus is a rare form that occurs only during pregnancy[3]. During pregnancy, the placenta may make too much of an enzyme that breaks down AVP. This condition is more likely if you are pregnant with multiples or have a condition that affects your liver function, like preeclampsia[3]. Gestational diabetes insipidus usually goes away two to three weeks after the pregnancy is over[3].
Dipsogenic diabetes insipidus results from a problem with your hypothalamus that affects your sense of thirst. It causes you to be abnormally thirsty and drink a lot, which then makes your body produce more urine[15].
How doctors diagnose diabetes insipidus
When you come to your first appointment, your healthcare provider will ask about your medical history and symptoms[14]. It’s important to share as many details as possible about your symptoms, how long you’ve been feeling this way, and other medical conditions you have.
Your doctor will perform several tests to diagnose diabetes insipidus and determine which type you have. These tests help rule out other conditions that can cause similar symptoms[10].
A urinalysis examines the contents of your urine. If the water content is high and the salt and waste concentration low, it could be due to diabetes insipidus[9]. Your doctor will check the urine’s specific gravity, which measures how concentrated the urine is[7].
Blood tests can check the levels of certain substances in the blood, such as sodium, potassium, and calcium. These can help with a diagnosis and may be useful in identifying the type of diabetes insipidus[10]. Paired early morning urine and serum osmolality tests can show if your serum osmolality is high with inappropriately dilute urine[16].
The water deprivation test is the main test for diabetes insipidus. For this test, you stop drinking fluids for several hours, sometimes up to 12 hours. During the test, your healthcare provider measures changes in your body weight, how much urine your body makes, and the concentration of your urine and blood[10]. If you keep making large amounts of dilute urine despite not drinking anything, you may have diabetes insipidus[6].
After a water deprivation test, your doctor may give you a shot of vasopressin (also called desmopressin) to see how your body reacts[6]. This can help show if your body is making enough AVP and if your kidneys can respond as expected to AVP[10].
A magnetic resonance imaging (MRI) scan can look for problems with the pituitary gland or hypothalamus, such as tumors or other structural issues[10]. If your doctor suspects you have the inherited form of the disease, genetic testing may be recommended, particularly if other family members have had similar problems[10].
Treatment options
Treatment for diabetes insipidus depends on the type and severity of your condition. The goal of treatment is to reduce the amount of urine your body produces, relieve thirst, and prevent dehydration[1].
If you have mild diabetes insipidus, you may only need to drink more water to avoid dehydration[10]. Your doctor may advise you to drink a certain amount of water every day, usually at least 2.5 liters[11]. Most patients with diabetes insipidus can drink enough fluid to replace their urine losses[13].
For arginine vasopressin deficiency (AVP-D), the main medication is desmopressin (DDAVP, Nocdurna)[10]. Desmopressin is a manufactured version of AVP that’s more powerful and more resistant to being broken down than the AVP naturally produced by your body[11]. It works just like natural AVP, stopping your kidneys from producing too much urine when the level of water in your body is low[11].
Desmopressin can be taken as a nasal spray, in tablet form, or as a form that melts in your mouth between your gum and lip[11]. If you’re prescribed desmopressin as a nasal spray, you’ll need to spray it inside your nose once or twice a day, where it’s quickly absorbed into your bloodstream[11]. If you develop a cold that prevents you from using the nasal spray, your doctor may suggest switching your treatment to tablets[11].
Desmopressin is very safe to use and has few side effects. However, possible side effects can include headaches, stomach pain, feeling sick, a blocked or runny nose, and nosebleeds[11]. If you take too much desmopressin or drink too much fluid while taking it, it can cause your body to retain too much water, leading to a low level of sodium (salt) in your blood called hyponatraemia[11].
For arginine vasopressin resistance (AVP-R), desmopressin usually cannot help because your kidneys don’t respond to AVP[11]. If AVP-R is caused by a medication you’re taking, such as lithium, your doctor may stop your treatment and suggest an alternative medication[11]. However, do not stop taking any medication unless you have been advised to by a healthcare professional[11].
For AVP-R, you may be prescribed a combination of thiazide diuretics and a nonsteroidal anti-inflammatory drug (NSAID) like ibuprofen to help reduce the amount of urine your kidneys produce[11]. Thiazide diuretics can reduce the rate the kidneys filter blood, which reduces the amount of urine passed from the body over time[11]. NSAIDs help reduce urine volume further when they’re used in combination with thiazide diuretics[11].
If your condition is mild, your doctor may suggest reducing the amount of salt and protein in your diet, which will help your kidneys produce less urine[11]. This may mean eating less salt and protein-rich food, such as processed foods, meat, eggs, and nuts. Do not alter your diet without first seeking medical advice[11].
Patients may require hospitalization to establish fluid needs, and frequent monitoring of electrolytes and fluid balance is recommended during the initial phase of treatment[13].
Possible complications
The main complication of diabetes insipidus is dehydration[3]. This happens when your body loses too much fluid. If you don’t drink enough fluids, you can become dehydrated, which can be dangerous[5].
Dehydration can cause several problems including dry skin, dry mouth, nose, and sinuses, sluggishness, confusion, fever, fast heart rate, eyes that look sunken, and weight loss and weakness, especially when standing[7].
You can also develop an electrolyte imbalance, which means not having the right balance of minerals in your body[7]. Electrolyte imbalance can cause tiredness, headache, muscle pains, feeling very tired, being irritable, and irregular heartbeat[7].
If you have diabetes insipidus and you stop drinking fluids or don’t get treatment, you can quickly develop severe dehydration[3]. Severe dehydration will need to be treated in a hospital[4].
The condition is particularly dangerous when a child or person is unable to access water freely, such as a very young child, someone with an altered conscious state, someone who is not allowed to eat or drink before a medical procedure, or someone who is too unwell to drink[16].
Living with diabetes insipidus
It’s vital that access to water is never limited for children or adults with suspected or confirmed diabetes insipidus, as their thirst response is what keeps their sodium level in the high normal range[16]. Children with suspected or known diabetes insipidus must always have free access to water, and fluid intake should never be restricted[16].
With treatment, diabetes insipidus does not cause severe problems or result in early death[5]. If your body’s thirst control is normal and you are able to drink enough fluids, there are no significant effects on body fluid or salt balance[5]. You can live a long and full life with diabetes insipidus[17].
It’s important to follow your healthcare provider’s directions about medicines and fluid intake to prevent problems[7]. You need to drink more water than other people do, and it’s a good idea to carry water with you at all times[17].
Patients with diabetes insipidus must take special precautions, such as when traveling, to be prepared to treat vomiting or diarrhea and to avoid dehydration with exertion or hot weather[13]. Patients should ensure access to water at their destination when traveling. Travels through deserts are best undertaken at night to avoid the excessive dehydration that can occur during day travel[13].
Follow-up care is a key part of your treatment and safety. Be sure to make and go to all appointments, and call your doctor if you are having problems[17]. Request follow-up visits with your doctor every 6 to 12 months to monitor your condition[13].
Call your doctor or seek immediate medical care if you have symptoms of dehydration, such as dry eyes and a dry mouth, feeling much thirstier than usual, feeling dizzy or light-headed, or feeling very confused[17]. If your symptoms get worse or you have new symptoms, call your healthcare provider right away[7].
