{"id":15914,"date":"2021-11-24T07:11:59","date_gmt":"2021-11-24T07:11:59","guid":{"rendered":"https:\/\/zeepedia.com\/?p=15914"},"modified":"2021-11-25T11:46:36","modified_gmt":"2021-11-25T11:46:36","slug":"kuvan","status":"publish","type":"post","link":"https:\/\/zeepedia.com\/medical\/medicines\/kuvan\/","title":{"rendered":"Kuvan &#8211; Sapropterin dihydrochloride uses, dose and side effects"},"content":{"rendered":"\n<p>100 mg&nbsp;soluble tablets<br>sapropterin dihydrochloride<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">What Kuvan is and what it is used for<\/h2>\n\n\n\n<p>The cube contains the active substance sapropterin, which is a&nbsp;synthetic&nbsp;copy of a body substance called tetrahydrobiopterin (BH4).&nbsp;BH4 is required for the body to use the amino acid phenylalanine, to form another&nbsp;amino acid&nbsp;called tyrosine.<\/p>\n\n\n\n<p>Kuvan is used to treat hyperphenylalaninemia anemia (HPA) or phenylketonuria (PKU) in patients of all ages.\u00a0HPA and PKU are caused by abnormally high levels of phenylalanine in the blood, which can be harmful.\u00a0Kuvan lowers the levels of these substances in some patients who respond to treatment with BH4 and can therefore help to increase the amount of phenylalanine that can be included in the diet.<\/p>\n\n\n\n<p>This drug is also used to treat a hereditary disease called BH4 deficiency in patients of all ages.&nbsp;The disease means that the body can not produce enough BH4.&nbsp;With very low BH4 levels, phenylalanine is not used properly, and its levels rise, which has harmful effects.&nbsp;By replacing the BH4 that the body cannot produce, Kuvan lowers the harmful excess of phenylalanine in the blood and increases the tolerance of phenylalanine in the diet.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">What you need to know before taking Kuvan<\/h2>\n\n\n\n<h2 class=\"wp-block-heading\">Do not take Kuvan<\/h2>\n\n\n\n<p>If you are allergic to sapropterin or any of the other ingredients of this medicine (listed in section 6).<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Warnings and cautions<\/h2>\n\n\n\n<p>Talk to your doctor or pharmacist before taking Kuvan, especially:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>if you are 65 years or older<\/li><li>if you have kidney or liver problems<\/li><li>if you have become ill.&nbsp;Contact with a doctor is recommended in case of illness, as phenylalanine levels in the blood may then rise<\/li><li>if you have a tendency to cramp<\/li><\/ul>\n\n\n\n<p>When you are being treated with Kuvan, your doctor will take blood samples from you to see how much phenylalanine and tyrosine you have in your blood and on that basis decide to change the&nbsp;dose of&nbsp;one of Kuvan or your diet if necessary.<\/p>\n\n\n\n<p>You must continue with your diet according to your doctor&#8217;s recommendations.\u00a0Do not make any dietary changes without first consulting your doctor.\u00a0Even if you take Kuvan, you can develop severe neurological problems if your phenylalanine levels in the blood are not well controlled.\u00a0Your doctor should continue to check your phenylalanine levels in your blood frequently during treatment with Kuvan\u00a0<strong>to ensure that your phenylalanine blood levels are not too high or too low<\/strong>.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Other medicines and Kuvan<\/h2>\n\n\n\n<p>Tell your doctor or pharmacist if you are taking, have recently taken, or might take any other medicines.\u00a0In particular, tell your doctor if you are taking:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>levodopa&nbsp;(used to treat&nbsp;Parkinson&#8217;s disease&nbsp;)<\/li><li>drugs for the treatment of cancer (eg methotrexate)<\/li><li>drugs for the treatment of bacterial&nbsp;infections&nbsp;(eg trimethoprim)<\/li><li>drugs that cause&nbsp;dilation&nbsp;(&nbsp;dilation&nbsp;) of blood vessels (eg&nbsp;glyceryl&nbsp;trinitrate (GTN),&nbsp;isosorbide dinitrate&nbsp;(ISDN), sodium nitroprusside (SNP), molsidomine, minoxidil).<\/li><\/ul>\n\n\n\n<h2 class=\"wp-block-heading\">Pregnancy and breastfeeding<\/h2>\n\n\n\n<p>If you are pregnant or breastfeeding, think you may be pregnant, or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.<\/p>\n\n\n\n<p>If you are pregnant, your doctor will tell you what to do to keep your phenylalanine levels under control.\u00a0If these are not strictly controlled before or when you become pregnant, it can be harmful to you and the baby.\u00a0Your doctor will check the restrictions on the intake of phenylalanine in your diet before and during pregnancy.<\/p>\n\n\n\n<p>If the strict diet does not provide a sufficient reduction in the levels of phenylalanine in the blood, your doctor will consider whether you need to take this medicine.<\/p>\n\n\n\n<p>You should not take this medicine if you are breastfeeding.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Driving and using machines<\/h2>\n\n\n\n<p>Kuvan is not expected to affect the ability to drive and use machines.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Important information about other ingredients in Kuvan<\/h2>\n\n\n\n<p>This medicine contains less than 1 mmol\u00a0sodium\u00a0(23 mg) per tablet, ie essentially &#8216;sodium-free.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">How to take Kuvan<\/h2>\n\n\n\n<p>Always take this medicine exactly as your doctor has told you.&nbsp;Consult a doctor if you are unsure.<\/p>\n\n\n\n<p><strong>Dosage\u00a0for PKU The<\/strong><br>a recommended starting dose of Kuvan for patients with PKU is 10 mg per kg body weight.\u00a0Take Kuvan as a single daily dose with a meal to increase your intake, and at the same time each day, preferably in the morning.\u00a0Your doctor may adjust\u00a0dose\u00a0one, usually to between 5 and 20 mg per kg body weight per day, depending on your condition.<\/p>\n\n\n\n<p><strong>Dosage&nbsp;for BH4 deficiency The<\/strong><br>recommended starting dose of Kuvan for patients with BH4 deficiency is 2 to 5 mg per kg body weight.&nbsp;Take Kuvan with a meal to increase your intake.&nbsp;Divide the total daily dose into 2 or 3 doses spread over the day.&nbsp;Your doctor may adjust the&nbsp;dose&nbsp;up to 20 mg per kg body weight per day, depending on your condition.<\/p>\n\n\n\n<p><strong>The table below shows an example of how a correct\u00a0dose\u00a0is calculated<\/strong><\/p>\n\n\n\n<figure class=\"wp-block-table\"><table><tbody><tr><td>Bodyweight (kg)<\/td><td>Number of 100 mg tablets(&nbsp;dosage10 mg \/ kg)<\/td><td>Number of 100 mg tablets(&nbsp;dosage20 mg \/ kg)<\/td><\/tr><tr><td>10<\/td><td>1<\/td><td>2<\/td><\/tr><tr><td>20<\/td><td>2<\/td><td>4<\/td><\/tr><tr><td>30<\/td><td>3<\/td><td>6<\/td><\/tr><tr><td>40<\/td><td>4<\/td><td>8<\/td><\/tr><tr><td>50<\/td><td>5<\/td><td>10<\/td><\/tr><\/tbody><\/table><\/figure>\n\n\n\n<p><strong>Supply of the drug<\/strong><\/p>\n\n\n\n<p>For PKU patients, the total daily dose is given once a day at the same time each day, preferably in the morning.<\/p>\n\n\n\n<p>For patients with BH4 deficiency, the total daily dose of 2 or 3&nbsp;doses&nbsp;is&nbsp;distributed&nbsp;over the day.<\/p>\n\n\n\n<p><strong><em>Use for all patients<\/em><\/strong><\/p>\n\n\n\n<p>Put the prescribed number of tablets in a glass or cup of water as described below and dissolve them while stirring.<\/p>\n\n\n\n<p>It may take a few minutes for the tablets to dissolve.\u00a0To make the tablets dissolve faster, you can crush them.\u00a0Small particles can be seen in the solution, but they do not affect the effectiveness of the drug.\u00a0Drink the reconstituted\u00a0preparation\u00a0of one of Kuvan at mealtime within 15 to 20 minutes after completion.<\/p>\n\n\n\n<p>The capsule with desiccant contained in the jar must not be swallowed.<\/p>\n\n\n\n<p><em>Use in patients weighing more than 20 kg<\/em><\/p>\n\n\n\n<p>Put the tablets in a glass or cup (120 to 240 ml) with water and dissolve them while stirring.<\/p>\n\n\n\n<p><em>Use for children with a bodyweight up to 20 kg<\/em><\/p>\n\n\n\n<p>Dose&nbsp;one is based on body weight.&nbsp;It changes as the baby grows.&nbsp;Your doctor will tell you:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>how many tablets Kuvan is required for a&nbsp;dose<\/li><li>the amount of water needed to mix a&nbsp;dose of&nbsp;Kuvan<\/li><li>the amount of solution you need to give the child for his \/ her prescribed&nbsp;dose&nbsp;.<\/li><\/ul>\n\n\n\n<p>The child should drink the solution with a meal.<\/p>\n\n\n\n<p>Give the child the prescribed amount of solution within 15 to 20 minutes after dissolution.&nbsp;If you can not give the child a&nbsp;dose&nbsp;within 15 to 20 minutes after dissolving the tablets, you must prepare a new solution as the unused solution should not be used after 20 minutes.<\/p>\n\n\n\n<p><em>Items you need to prepare and give your child a&nbsp;dose&nbsp;of Kuvan<\/em><\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>The number of tablets Kuvan required for one&nbsp;dose<\/li><li>A medicine cup with markings at 20, 40, 60 and 80 ml<\/li><li>A glass or a cup<\/li><li>A small spoon or a clean tool to stir with<\/li><li>Syringe&nbsp;for&nbsp;oral&nbsp;use with gradation at every 1 ml) (10 ml&nbsp;syringe&nbsp;for&nbsp;administering&nbsp;volumes up to 10 ml or 20 ml&nbsp;syringe&nbsp;for&nbsp;administering&nbsp;volumes above 10 ml)<\/li><\/ul>\n\n\n\n<p>Ask your doctor for a medicine cup to dissolve the tablets and a 10 ml or 20 ml&nbsp;syringe&nbsp;for&nbsp;oral&nbsp;use if you do not have them.<\/p>\n\n\n\n<p><em>Steps to prepare and take\u00a0a dose\u00a0one<\/em><\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Put the prescribed number of tablets in the medicine cup.&nbsp;Pour water into the medicine cup according to the doctor&#8217;s instructions (eg the doctor has told you to use 20 ml to dissolve a tablet Kuvan).&nbsp;Check that the amount of fluid matches the amount specified by your doctor.&nbsp;Stir with a small spoon or a clean utensil until the tablets are dissolved.<\/li><li>If your doctor has told you to&nbsp;administer&nbsp;only&nbsp;part of the solution, place the tip of the syringe for&nbsp;oral&nbsp;use in the medicine cup.&nbsp;Slowly pull back the plunger to draw up the amount instructed by your doctor.<\/li><li>Transfer the solution by slowly pressing on the plunger until all the solution in the syringe for&nbsp;oral&nbsp;use has been transferred to the glass or cup to be used for&nbsp;administration&nbsp;(eg if your doctor has told you to dissolve two tablets of Kuvan in 40 ml of water and&nbsp;administer&nbsp;30 ml to the child, you must use the 20 ml syringe twice to draw up 30 ml (eg 20 ml + 10 ml) solution and transfer it to the glass or cup to be used for&nbsp;administration&nbsp;).&nbsp;Use a 10 ml&nbsp;syringe&nbsp;for&nbsp;oral&nbsp;use to&nbsp;administer&nbsp;volumes up to 10 ml or a 20 ml&nbsp;syringe&nbsp;for&nbsp;administration&nbsp;of volumes above 10 ml.<\/li><li>If the child is too small to drink from a glass or cup, you can give the solution via syringe for&nbsp;oral&nbsp;use.&nbsp;Withdraw the prescribed volume from the solution prepared in the medicine cup and place the tip of the syringe for&nbsp;oral&nbsp;use in the baby&#8217;s mouth.&nbsp;Aim the syringe tip at one cheek.&nbsp;Slowly press the plunger to&nbsp;administer&nbsp;a little at a time, until all the solution in the syringe is exhausted.<\/li><li>Discard the remaining solution.&nbsp;Remove the plunger from the cylinder on the syringe for&nbsp;oral&nbsp;use.&nbsp;Wash both parts of the syringe and the beaker with warm water and allow them to air dry.&nbsp;When the syringe is dry, put the plunger back in the cylinder.&nbsp;Keep the syringe for&nbsp;oral&nbsp;use and the medicine cup until the next use.<\/li><\/ul>\n\n\n\n<h2 class=\"wp-block-heading\">If you take more Kuvan than you should<\/h2>\n\n\n\n<p>If you take more Kuvan than you were prescribed, you may experience\u00a0side effects, such as headaches and dizziness.\u00a0Contact a doctor or pharmacist immediately if you have taken more Kuvan than you were prescribed.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">If you forget to take Kuvan<\/h2>\n\n\n\n<p>Do not take a double\u00a0dose\u00a0to make up for a forgotten\u00a0dose.\u00a0Take the next\u00a0dose\u00a0at the usual time.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">If you stop taking Kuvan<\/h2>\n\n\n\n<p>Do not stop using Kuvan without first consulting your doctor, as phenylalanine levels in your blood may rise.<\/p>\n\n\n\n<p>If you have any further questions on the use of this product, ask your doctor or pharmacist.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Possible side effects<\/h2>\n\n\n\n<p>Like all medicines, this medicine can cause\u00a0side effects, although not everybody gets them.<\/p>\n\n\n\n<p>A few cases of allergic reactions (including rash and severe reactions) have been reported.&nbsp;The frequency of them is not known (the frequency can not be calculated from the available data).<\/p>\n\n\n\n<p>If you have red, itchy, and raised areas on the skin (\u00a0hives\u00a0), runny nose, rapid or uneven\u00a0pulse, swelling of the tongue and throat, sneezing, wheezing, severe difficulty breathing, or dizziness, you may have had a severe\u00a0allergic reaction\u00a0to the medicine.\u00a0If you notice these signs, contact your doctor immediately.<\/p>\n\n\n\n<p><strong>Very common\u00a0side effects\u00a0are<\/strong>\u00a0(can occur in more than 1 in 10)<\/p>\n\n\n\n<p>Headache and runny nose.<\/p>\n\n\n\n<p><strong>Common&nbsp;side effects&nbsp;are<\/strong>&nbsp;(may affect up to 1 in 10 people)<\/p>\n\n\n\n<p>Sore throat, nasal congestion, cough, diarrhea, vomiting, stomach pain, too low levels of phenylalanine in blood tests, indigestion, and nausea (see section 2: &#8220;Warnings and precautions&#8221;).<\/p>\n\n\n\n<p><strong>Side effects&nbsp;without known frequency<\/strong>&nbsp;(can not be calculated from the available data)<\/p>\n\n\n\n<p>Gastritis&nbsp;(&nbsp;inflammation&nbsp;of the stomach mucosa),&nbsp;esophagitis&nbsp;(&nbsp;inflammation&nbsp;of the esophagus&nbsp;mucosa&nbsp;).<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">How to store the Kuvan<\/h2>\n\n\n\n<p>Keep this medicine out of the sight and reach of children.<\/p>\n\n\n\n<p>Do not use this medicine after the expiry date which is stated on the can and carton after \u201cEXP\u201d.&nbsp;The expiration date is the last day of the specified month.<\/p>\n\n\n\n<p>Do not store above 25 \u00b0 C.<\/p>\n\n\n\n<p>Close the jar tightly.&nbsp;Moisture sensitive.<\/p>\n\n\n\n<p>Medicines should not be disposed of via wastewater or household waste.&nbsp;Ask your pharmacist how to dispose of medicines no longer required.&nbsp;These measures will help to protect the environment.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Contents of the pack and other information<\/h2>\n\n\n\n<h2 class=\"wp-block-heading\">Content declaration<\/h2>\n\n\n\n<ul class=\"wp-block-list\"><li>The active substance is sapropterin dihydrochloride.&nbsp;Each tablet contains 100 mg of sapropterin dihydrochloride (equivalent to 77 mg of sapropterin).<\/li><li>The other ingredients are mannitol (E421), anhydrous calcium hydrogen phosphate, crospovidone type A,&nbsp;ascorbic acid&nbsp;(E300), sodium stearyl fumarate and riboflavin (E101).<\/li><\/ul>\n\n\n\n<h2 class=\"wp-block-heading\">What the medicine looks like and contents of the pack<\/h2>\n\n\n\n<p>Kuvan 100 mg soluble tablets are off-white to light yellow with \u201c177\u201d debossed on one side.<\/p>\n\n\n\n<p>The medicine is packaged in jars with a child-resistant closure of 30, 120, or 240 soluble tablets.\u00a0Each jar contains a small plastic tube with desiccant (silica gel).<\/p>\n\n\n\n<p>Not all pack sizes may be marketed.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Marketing Authorisation Holder<\/h2>\n\n\n\n<p>BioMarin International Limited<\/p>\n\n\n\n<p>Shanbally, Ringaskiddy<br>County Cork<br>Ireland<\/p>\n\n\n\n<p><strong>Manufacturer<\/strong><\/p>\n\n\n\n<p>BioMarin International Limited<\/p>\n\n\n\n<p>Shanbally, Ringaskiddy<br>County Cork<br>Ireland<\/p>\n","protected":false},"excerpt":{"rendered":"<p>The cube contains the active substance sapropterin, which is a synthetic copy of a body substance called tetrahydrobiopterin (BH4). BH4 is required for the body to use the amino acid phenylalanine, to form another amino acid called tyrosine.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"cybocfi_hide_featured_image":"","footnotes":""},"categories":[2200,12118],"tags":[15088,15082,15083,15087,15086,15077,15081,15080,15084,15078,15085,15079],"class_list":["post-15914","post","type-post","status-publish","format-standard","hentry","category-medicines","category-k","tag-benefits-of-kuvan","tag-benefits-of-sapropterin-dihydrochloride","tag-kuvan","tag-kuvan-side-effects","tag-kuvan-tablet","tag-sapropterin-dihydrochloride","tag-sapropterin-dihydrochloride-side-effects","tag-sapropterin-dihydrochloride-tablet","tag-what-is-kuvan","tag-what-is-sapropterin-dihydrochloride","tag-what-kuvan-is-used-for","tag-what-sapropterin-dihydrochloride-is-used-for"],"_links":{"self":[{"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/posts\/15914","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/comments?post=15914"}],"version-history":[{"count":4,"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/posts\/15914\/revisions"}],"predecessor-version":[{"id":15949,"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/posts\/15914\/revisions\/15949"}],"wp:attachment":[{"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/media?parent=15914"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/categories?post=15914"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/zeepedia.com\/medical\/wp-json\/wp\/v2\/tags?post=15914"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}