It’s very important for people with Alport syndrome to visit a nephrologist (kidney specialist) regularly.
The following are typical things that occur during your visit:
- Your blood pressure will be taken with a blood pressure cuff around your arm
- Your height and weight will be measured.
- You’ll be asked about how you’re feeling physically and emotionally
- You’ll be asked to urinate in a medically sterile cup to measure levels of blood and/or protein in your urine.
Sometimes you’ll need to have your blood drawn from your arm.
Because it can affect hearing and vision, it’s important that people with Alport syndrome also regularly be evaluated with hearing and vision tests.
This may mean seeing an audiologist (hearing specialist) once a year, and an optometrist/ophthalmologist annually.
Several different kinds of medications can slow down loss of kidney function in people with Alport syndrome. They are generally referred to as “ACE inhibitors” and “ARB” medications. ACE stands for “angiotensin converting enzyme” and ARB stands for angiotensin receptor blockers. Researchers are still learning about the ways in which these medications protect the kidneys of animals and people with Alport syndrome. Researchers believe there are at least two effects. First, these medications may directly prevent the formation of scar tissue in kidneys of animals and people with Alport syndrome. Second, by lowering urine protein levels these medications may prevent the harmful effects of high urine protein levels on kidney cells.
Because these medications protect kidneys and slow disease progression, it’s extremely important that people with Alport syndrome take these medications exactly how their nephrologist prescribes them.
Some examples of ACE inhibitors used to treat Alport syndrome are:
Some examples of ARBs that have been used to treat Alport syndrome are:
Spironolactone is medication used for aldosterone inhibition.
Possible Side Effects of Medicines
Both ACE inhibitors and ARBs can cause lightheadedness, especially when standing up or increasing heart rate through exercise quickly. Sometimes these medications need to be stopped or doses lowered because of persistent lightheadedness or fainting. ACE inhibitors and ARBs should not be taken by females who are pregnant or who are planning to become pregnant because they can injure a developing fetus. ACE inhibitors, ARBs and aldosterone inhibitors can cause elevated blood potassium levels. If your nephrologist notes that your blood potassium is higher than it should be, you might be asked to restrict the amount of potassium rich foods you eat.
A common side effect of ACE inhibitors is a cough, which may take up to a month to subside. If one ACE inhibitor causes cough, it is likely that the others will too. Coughing occurs less often with ARBs, which may be used instead of an ACE inhibitor. The most serious, but rare, side effects are allergic reactions, a decrease in white blood cells, and swelling of tissues (angioedema).
Important medications to avoid
People with Alport syndrome should avoid medicines that are harmful to the kidneys. This includes over the counter medicines containing aspirin, ibuprofen and naproxen, as well as some decongestants. Your nephrologist can provide guidance on medicines that should be avoided. Never let a school nurse or well-meaning adult give you an over-the-counter pain or cold medicine that you have not checked with your doctor.
Maintaining a healthy lifestyle and a balanced diet is also beneficial. Discussion of nutritional considerations, such as sodium reduction and moderating protein consumption, should be discussed with your doctor.