Thursday, December 2, 2010

Diabetes Insipidus & Desmopressin Shortage


Desmopressin: Two doses a day keeps the urine away!

I was shocked and disappointed to recently discover that there was a national shortage of desmopressin acetate, a medication commonly used to treat diabetes insipidus (DI). After undergoing transsphenoidal surgery to remove a pituitary tumor this year, pathology revealed that the offending mass had damaged part of my pituitary gland. As a result, I was diagnosed with DI and prescribed desmopressin therapy to manage my symptoms.


What is diabetes insipidus?

Although there is more than one type of DI, for the purpose of this blog post, I am referring specifically to CENTRAL diabetes insipidus, which can result from a head injury, infection, tumor or neurosurgery. DI is a relatively uncommon condition where the patient’s body is unable to retain enough water. This can result in excessive thirst and frequent urination, which is very diluted and pale or colorless instead of being the usual yellow, and does not reduce in volume even if the patient decreases fluid intake. DI symptoms can also include nocturia (the need to get up from sleep at night in order to urinate). Indeed, before being given desmopressin therapy, I often visited the bathroom about three or four times every night…. Certainly not something that is conducive to a good night’s sleep, which is necessary to aid the recovery process. Another one of my symptoms included a strong craving for icy cold water and I literally drank liters of water faster than I could chill it (thank goodness for the bags of ice cubes kept in the freezer). Although the word “diabetes” generally refers to diabetes mellitus (“sugar diabetes”), diabetes insipidus is not specifically related, although both conditions are characterized by extreme thirst and polyuria (excretion of considerable quantities of urine). For this reason, it is sometimes called “water diabetes”.


Some science behind diabetes insipidus

DI generally occurs because of a deficiency of an important antidiuretic hormone called vasopressin (also known as ADH), which is responsible for controlling the release of urine from the kidneys. Vasopressin is made by the hypothalamus in the brain and is stored by the pituitary gland (the master gland of the endocrine system). When the amount of water in the body becomes too low, ADH is released from the pituitary gland – This helps to maintain water by instructing the kidneys to produce less urine (and to instead return water into the bloodstream). When there is insufficient vasopressin, the kidneys are unable to properly conserve water whilst they carry out their function of filtering blood, and too much fluid gets passed from the body in the form of diluted urine. A shortage of vasopressin is usually caused from damage to the hypothalamus or the posterior (back part) of the pituitary gland.
  


The good news

The good news is that DI can usually be managed effectively with the synthetic antidiuretic hormone replacement therapy of vasopressin (desmopressin, DDAVP), which may be taken in nasal spray (intranasal), tablet (pill), or intravenous (injection) form. The manufactured version of ADH works similarly to natural ADH in your body by preventing the kidneys from producing urine when the level of water in the body is low. When treated, DI does not cause any major problems or reduced life expectancy. However, if fluids aren’t replaced DI can cause severe dehydration and other serious conditions (especially in children, the elderly or in those who have other complicating health issues). It is therefore, important to have your electrolytes monitored to ensure that you are on the correct dose of medication and to carry an adequate supply with you when you are away from home. I have also read on many medical websites that it would be pertinent to wear a medical alert ID bracelet or necklace to inform caregivers of your condition in an emergency situation.

I was prescribed the tablet form of DDAVP, although some patients prefer the nasal spray because it can be more quickly absorbed into the bloodstream. The spray, however, may not be suitable for some patients, such as those who have recently undergone transsphenoidal or sinus surgery, or for those who have a cold, as the nasal passages will likely be congested or blocked. In some cases, patients may switch to the intravenous method (e.g., those who have poor intranasal absorption or because they are undergoing surgery). Since taking desmopressin, my DI symptoms have improved dramatically and I no longer need to lug a large flask of water around with me, as if it were an oxygen tank. I am also able to enjoy a good night’s sleep and don’t seem to experience any significant or bothersome side effects (although I must admit that it is difficult to isolate some of the side effects from some other conditions and medications that I am currently encountering).


The bad news

The bad news is that there currently appears to be a national shortage of the drug desmopressin. The U.S. Food and Drug Administration (FDA) recently announced that such a shortage was due to increased demand and manufacturing delays (See here for more information). Although this shortage is said to specifically relate to the intravenous (IV) form, a few patients also seem to be having trouble obtaining other types of the drug. I contacted my own pharmacy and the person I spoke to appeared oblivious to any shortages. However, I’m not overly confident in the information (or lack thereof) provided by this particular individual. Despite the fact that he had said he was a qualified pharmacist, he’d never actually heard of the medication desmopressin and was unresponsive when I asked if he would check with their supplier for availability of the drug. I did, however, directly contact Ferring Pharmaceuticals who said that desmopressin vials were indeed on back order, although the nasal spray was still available (Ferring do not have any dealings with the tablet form).


Summary

Diabetes insipidus can be a serious medical condition and often needs to be treated with desmopressin therapy. It is important that you see your general physician if you think you may have DI. He or she will ask you about your symptoms and carry out the appropriate tests (which should include the water retention test) or refer you to an endocrinologist for further examination. If you have already been diagnosed with DI and are having problems obtaining your medication, you may find it helpful to contact your own pharmacy for additional information. The Pituitary Network Association (PNA) are advocating on behalf of DI patients who are unable to obtain desmopressin and request that those in difficulty contact them accordingly (See PNA link below for more instructions). 


Sources/links



17 comments:

  1. Just a slight correction: you mean intramuscular (injected into a muscle), not intravenous. :-> An intravenous treatment would be tricky to administer at home.

    ReplyDelete
  2. Thanks for your valuable comment - It is much appreciated. To the best of my knowledge, desmopressin formulation may be administered subcutaneously or by direct intravenous injection. Presumably, such methods would mainly be reserved for hospital inpatients that may not benefit as favorably from other forms of the drug. Some medical professionals may even decide to fit a catheter to the patient before going home though (I almost had one fitted yesterday, but we eventually decided against it... for now anyway!)

    ReplyDelete
  3. Hi, thanks for all the good information. My 14 year old doggie Sophie also has DI and is extremley dependant (as am I due to the side effects of DI if not medicated i.e. urinating throughout the house and drinking excessively). I am having a very difficult time finding the nasal spray Desmopressin. I actually transfer it into a small eye dropper bottle (I know, not verry sterile) but is the only way to administer to a dog in one eye twice a day. I did manage to find a 50 dose bottle of the DDAVP, but it is significantly higher in price, usually I would pay $80 per month, now $126 per month. One 50 dose bottle lasts about 1 month. I live in BC, Canada. I do hope the shortage is soon rectified, especially for you. Good luck and again thanks for the info.

    ReplyDelete
  4. Thank you for all the info... I just found out my 9 yr old daughter has diabetes Insipidus central, We think she stopped making the hormone because she fell and hit her head 2 yrs ago but not sure. she has to take 4 pills a day of desmo. for the rest of her life:(

    ReplyDelete
  5. I am so sorry to hear that your young daughter is suffering with CDI and wish her well in all her future health endeavors. Although I am unaware of any real cure for this disorder, I recently started taking an adaptogen herb called Rhodiola Rosea (from Amerinden). I’m not sure if it helps everyone, but you may wish to talk to her doctor about trying it. There is also a book about this wonderful herb called “The Rhodiola Revolution”, if you are interested. Perhaps when I get a chance I will write a blog post about it. Thank you for taking the time to write. Best wishes to you and your daughter.

    ReplyDelete
  6. This blog is really a great source of information which is very useful for me. Thank you very much for such important information.

    FOR BEST SOLUTIONS ON MYELOMA PROBLEMS.

    ReplyDelete
  7. I recently had a pituitary tumor removed and with in 24 hrs of the surgery I was having issues with dehydration. They started me on desmopressin .02 it took some time to get it right but I now take this every 8 hours and I make sure not to miss a dose because I feel very bad very fast. They also have me on cortef 20mg once a day and methylpredisolone. They say all this is temporary that my pituitary is in shock. Has anyone had these problems? akminton@gmail.com

    ReplyDelete
  8. This is a comprehensive article on diabetes insipidus, which is a disease often confused with normal diabetes.

    ReplyDelete
  9. Diabetes insipidus occurs when the body can not regulate the amount of fluid taken. The amount of fluid excreted in the urine is largely affected by the production of antidiuretic hormone (ADH), also called vasopressin. ADH is made in the pituitary gland. There are no known ways to prevent diabetes insipidus. However, it is necessary to seek medical attention as soon as they appear two typical symptoms, and excessive urination and excessive thirst.

    ReplyDelete
  10. I am happy to have found this site. Many people do not understand what we go through on a daily basis and to find a group of people who suffer from the same medical bs and can support each other is huge to me.

    My original transnasal resection was two and a half years ago. The pituitary macro prolactinoma had pushed through the sella and was growing so my neuro and I decided on surgery (after cabergoline failed). In surgery, the ENT surgeon cut my left coratid artery and I nearly bled to death. That produced a pseudo aneurysm which has grown itself since and I now have 3 different metals and a stent holding that together. I had several csf leaks in hospital which lead to 3 additional surgeries and more lumbar drains etc. Was in hospital for 2 months and have had chronic sinusitis and sleep seizures since. The post op care was horrible. They didn't want to see the person they almost killed reminding them that they make mistakes. My care has been almost non existent except for ct angiograms to make sure that the ticking time bomb they gave me is not expanding anymore. I too have extreme weakness, DI, dizziness, fatigue, short term memory loss, tinnitus, headaches, body pain and a healthy case of I no longer trust doctor ism. My vision is getting worse and I have had hearing tests proving that I am losing my hearing. The best part? They didn't get it all because dr happy hands cut the coratid before my neuro had a chance to finish.

    I do not want to scare anyone but I would never recommend this surgery to anyone. My results are not common, however please research alternatives to surgery. My post op life is a shell of life before surgery. Went from working a dream job to being bankrupt and on disability. Has anyone else had any of this happen to them? I have been looking for a support group and have not been able to find anything remotely close until seeing this page.

    ReplyDelete
  11. Hello Bridget and welcome! I’m very sorry to hear about your dreadful struggles following pituitary surgery but I’m glad you find this site to be of some support. I agree with you that it can be helpful to share our stories, especially because we have what is considered to be a rare disorder where medical professionals are still finding their feet. Indeed, it is not uncommon for pituitary patients to know more than some doctors about pituitary disease – A scary place for us patients to be in!

    I can relate to a number of your symptoms including fatigue, weakness and cognitive deficits as well as the chronic sinusitis and the seizures, although my epilepsy existed prior to the TSA from another mass in my temporal lobe. Still, I can understand how disturbing such symptoms can be for you. And like you, I am also not surgically cured and intolerant to Cabergoline, so I am living on a ‘wing and a prayer’ that the tumor doesn’t grow back. I don’t think I would want to consider another transsphenoidal surgery now that my sinuses are so badly damaged. Are your hormone levels and MRIs stable since having the surgery? I’m just curious, have you also been checked for adult growth hormone deficiency?

    ReplyDelete
  12. No doctor has ordered a post op MRI to date, but my old endocrinologist recommended that I be put on growth hormone. The endo I saw a couple of times after realizing that the university hospital had no intention of delivering quality post op care refused to put me on growth hormone saying "I don't think you need that".

    Thankfully I will finally be seen by the Mayo Clinic in mid February and together we will start picking up the pieces and putting them in order.

    I been meditating and doing my best to self heal, however that alone is not the answer.

    Are your tumors malignant? I will meditate on your health and recovery. Bless you for creating this blog. This is like finding an oasis after being lost in a desert. Namaste!

    ReplyDelete
  13. Hi Bridget. That’s terrible! I do hope that you get things sorted out in the foreseeable future though. With regards to growth hormone deficiency, it is an extremely expensive drug and insurance companies will only agree to cover costs if patients meet specific criteria. Usually, a stimulation test is required, such as the glucagon or GST (the insulin tolerance test or ITT is the gold standard but it can be dangerous). It is important that you get tested by a board certified endocrinologist and preferably by a pituitary endocrinologist if possible. This will likely save you a lot of headaches down the line, even if you need to travel to see one. I would definitely request a stimulation test if you are experiencing symptoms of AGHD (FYI, some patients who are deficient have normal IGF-1 values). And even if your insurance company does deny you, it’s important that you appeal (such a scenario is unfortunately not uncommon because of the high cost of HGH).

    My MRIs usually incorporate the whole brain. Although the pituitary tumor isn’t malignant, my neurosurgeon is not entirely sure what the TL mass is. In 2011, it was suspected that it was an astrocytoma but following a craniotomy biopsy, doctors now seem much more optimistic (I had two neurosurgeries and one sinus surgery within a 7-month period). It will likely take another year or so and a couple more MRIs before I am completely “out of the woods”.

    Are you familiar with the Pituitary Network Association and their guidebook?

    It’s great that you have been meditating and I hope it will help in your healing process.

    Thank you for your very kind words. Many blessings to you!

    ReplyDelete
  14. :). Nice to find some decent info for a change.
    I am 3 weeks Post op now. And its so easy to find lots of info on pituitary tumor removal, but there is precious little info out there for people who have had their whole pituitary gland removed. I have had severe CDI that started with in 1 hour of surgery ( while i was still in recovery). I was passing 900 mls of urine every 15 minutes. They couldnt pump fluid into me fast enough. I was started on desmopressin and it did settle some what. I had an indwelling catheter in for 9 days and was still passing 600mls an hr every hr til they removed the catheter. Once removed i was going to the toilet every 15 minutes. I too had and still have a craving for Iced water !!! Big time. I am on the tablet form of desmopressin and my endo wants me to swap to the nasal form, but my nose is still sore and the left nostril still blocked and when i told her that she just brushed it away. So i havnt started on the nasal form yet. Im seeing the Neuro and ENT team in 4 days so ill see what they say first. Im really not comfortable putting medication up my nose when im still using nasal flushes and Nozoil for comfort.
    At this stage im not on any hormone replacement ( cept the desmo), im having a full range of tests done in a few weeks to see what my levels are before they start me on replacements. I was on hydrocortizone while in hospital but they ceased it the day i was discharged. Something about wanting to see just how low i go and what function if any the tiny bit of pituitary gland they left on the stalk has !!!
    Im a bit in the dark about what to expect now i have no pituitary gland to speak of.
    Oh the reason they took the whole gland was because my cortisol levels were so incredibly high pre surgery ( causing mega high blood pressures and blood glucose levels), the plan was if no tumor could be detected, to remove the whole gland. I had an over abundance of ACTH cells ( overactive pituitary).
    Im 3 weeks post op. Fatigue after doing 5 minutes of anything, cant be more than 15 minutes away from a toilet at any given time. I still carry around a tank of water lol. I have major trouble with memory, both long and short term, but especially short term. I have trouble finding words, i can look at pictures of people and i know who they are but no way can i think of their name.
    And the worst thing. I had an OT come to my house 2 days ago to devise a return to work plan. And work just seems so far away right now....... I dont know what to do !

    Anyway ive rambled enough

    Thanks for taking the time to write this for those of us desperate to find any info to help us thru difficult times :)

    ReplyDelete
    Replies
    1. Hello Debs. I am so sorry to hear of your ordeal and understand your plight. Pituitary disease can be difficult to deal with. It is important to make sure that your endocrinologist is fully cognizant with pituitary disease and isn’t merely a general endocrinologist who is “finding” his or her way in such complex issues. It is essential that your doctor will help you find the right balance of hormone replacements, which may take some time and effort. I cannot emphasize how crucial this will be to the quality of your life (QOL). It took me a long time to learn this and even though doctors don’t yet have all the answers, a specialist in pituitary disorders will likely give you the best outcome. I would recommend some suitable pituitary endocrinologists in the US but I understand that you are in Australia? The PNA does offer a physician list, so it may be worth you contacting them at info@pituitary.org for a suitable doctor in your area (or perhaps, like some pituitary patients, it may be worth some travel to see a good doctor). However, if you are confident in your doctor’s expertise in pituitary disorders (or have insurance restrictions), then remain proactive as a patient so that you are able to ask the right questions and request the relevant tests. For example, some doctors don’t think it necessary to test for adult growth hormone deficiency (AGHD). Even though my IGF-1 levels were normal post-op, I nevertheless requested a stimulation test because I felt so lousy (there are a number of associated symptoms). It turned out that I was indeed growth hormone deficient and required supplementation. There are a number of different hormone supplementations available depending upon your specific needs and hopefully, in time, they will help you feel much better. Here is a link to Dr. Friedman’s PowerPoint presentation on Pituitary Hormone Replacement, which you may find useful: pit_hormonal_replac-MAGIC-13n.ppt

      With regards to the Desmopressin, I understand your concerns about switching to the nasal spray if you are still congested. Perhaps your doctor(s) will decide to keep you on the pill form until the inflammation has completely gone. I hope your appointments with the neurologist and ENT will go well in a few days.

      Again, I understand how stressful your current situation may be and wish you well in your recovery.

      Delete
  15. Thank you So much for your reply :)
    I will check out Dr F's presentation now :)

    ReplyDelete
    Replies
    1. You're welcome. I hope you will find it helpful.

      Delete

Thank you for your comment – I appreciate you taking the time to write to me, and hope you will find my blog helpful. All comments are moderated, so your post will appear soon. You may also email me directly at: gallantstrides@me.com if you are concerned about leaving any personal information.

Wishing you and your family good health and happiness for the future!