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medications for treatment

The recommended medications for treatment of Alport syndrome interfere with several hormones that together make up what is known as the renin-angiotensin- aldosterone system, or RAAS. The RAAS normally plays a very important role in maintaining the body’s fluid balance and blood pressure, helping to make sure that the kidneys get the blood flow necessary for good kidney function. The RAAS is overactive in various chronic kidney diseases, including Alport syndrome, and has been shown to promote scarring of the kidneys.

Medications that interfere with RAAS hormones protect kidney function in animals and people with chronic kidney diseases. Medications that interfere with RAAS hormones include:

1

Angiotensin converting enzyme

(ACE) inhibitors — these medications block the production of angiotensin II, the active form of angiotensin

2

Angiotensin receptor blockers

(ARBs) — these medications block the action of angiotensin II

3

Aldosterone inhibitors — these medications block the action of aldosterone

ACE & ARBS

Both ACE inhibitors and ARBs have been shown to slow down the loss of kidney function in mice with Alport syndrome. People who start taking an ACE inhibitor while their kidney function is still normal are older when they develop kidney failure than Alport patients who dont receive ACE inhibitors or are started on ACE inhibitors after they have started to lose kidney function. ACE inhibitors, ARBs and aldosterone inhibitors all reduce elevated urine protein levels in people with Alport syndrome.

ACE inhibitors that have been used to treat Alport syndrome patients include, but are not limited to:

  • Benazepril
  • Cilazapril
  • Enalapril (Vasotec)
  • Fosinopril (Monopril)
  • Lisinopril (Zestril, Prinivil)
  • Perinopril
  • Ramapril
  • Quinapril (Accupril)
  • Trandolapril

ARBs that have been used to treat Alport syndrome patients include, but are not limited to:

  • Candesartan (Atacand)
  • Epresartan
  • Irbesartan
  • Losartan (Cozaar)
  • Telmisartan
  • Valsartan

Spironolactone is medication used for aldosterone inhibition.

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. These medications have relatively few and minor side effects, they are not expensive and they have been used safely in many children with kidney disease.

Diagnosis in the older child enabled earlier diagnosis in the younger sibling, resulting in earlier therapy and delayed onset of ESRD by 13 years.

MONITORING

In addition to taking ACE inhibitors and/or ARBs to control proteinuria (protein in the urine), Alport syndrome patients need to control their blood pressure and be monitored regularly. Monitoring tests include urine and blood chemistry testing. As a general recommendation, Alport syndrome patients without any kidney function problems should be monitored yearly, patients with moderate kidney function problems should be monitored every 6 months, and those with advanced kidney failure should be monitored every 1 to 3 months, or more frequently if directed by their medical professional.”

SIDE EFFECTS

Both ACE inhibitors and ARBs can cause lightheadedness, especially when a person stands up quickly. Sometimes these medications need to be stopped or their doses lowered because of persistent lightheadedness or fainting. ACE inhibitors and ARBs should not be taken by females who are pregnant or who can become pregnant because they can injure a developing fetus. ACE inhibitors, ARBs and aldosterone inhibitors can cause elevated blood potassium levels, but this is not a common problem in people who have normal kidney function.

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).

The Clinical Practice recommendations

The Clinical Practice recommendations advise testing of urine protein levels in children with Alport syndrome, starting at 1 year of age and then at least annually. The test is called the urine protein creatinine ratio. Children with urine protein creatinine ratios above 0.2 should be treated with an ACE inhibitor with the goal of reducing the protein creatinine ratio as much as possible. This may require a gradual increase in the dose of the medication, and may be limited by side effects such as lightheadedness. If a child is receiving the maximum dose of the ACE inhibitor and still has high urine protein levels, the Clinical Practice recommendations suggest starting either an ARB or an aldosterone inhibitor (the choice is up to the child’s doctor). Some nephrologists may prefer to use an ARB initially and add an ACE inhibitor if necessary to suppress proteinuria.

Patients with Alport syndrome should avoid drugs that are nephro-toxic or harmful to the kidneys.

This includes over the counter medicines such as
non-steroidal anti-inflammatory drugs (NSAIDs) containing aspirin, ibuprofen and naproxen, as well as some decongestants. Patients should speak with their nephrologist to get guidance on medicines that should be avoided.

Hearing and vision should also be monitored every one to two years beginning in children, particularly boys, at 7 to 8 years of age and continued regularly. Hearing aids should be prescribed as needed.

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.

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