Is Pulmonary Hypertension Common in Thalassemia Minors?

Definition
By Mayo Clinic staff

Pulmonary hypertension is a type of high blood pressure that affects only the arteries in the lungs and the right side of your heart.

Pulmonary hypertension begins when tiny arteries in your lungs, called pulmonary arteries, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, which raises pressure within the arteries in your lungs. As the pressure builds, your heart's lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and eventually fail completely.

Pulmonary hypertension is a serious illness that becomes progressively worse and is sometimes fatal. Although pulmonary hypertension isn't curable, treatments are available that can help lessen symptoms and improve your quality of life.

Symptoms
By Mayo Clinic staff

The signs and symptoms of pulmonary hypertension in its early stages may not be noticeable for months or even years. As the disease progresses, symptoms become worse.

Pulmonary hypertension symptoms include:

    * Shortness of breath (dyspnea), initially while exercising and eventually while at rest
    * Fatigue
    * Dizziness or fainting spells (syncope)
    * Chest pressure or pain
    * Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites)
    * Bluish color to your lips and skin (cyanosis)
    * Racing pulse or heart palpitations

Complications
By Mayo Clinic staff

Pulmonary hypertension can lead to a number of complications, including:

    * Right-sided heart failure (cor pulmonale). In cor pulmonale, your heart's right ventricle becomes enlarged and has to pump harder than usual to move blood through narrowed or blocked pulmonary arteries. At first, the heart tries to compensate by thickening its walls and expanding the chamber of the right ventricle to increase the amount of blood it can hold. But this thickening and enlarging works only temporarily, and eventually the right ventricle fails from the extra strain.
    * Blood clots. Clots help stop bleeding after you've been injured. But sometimes clots form where they're not needed. A number of small clots or just a few large ones dislodge from these veins and travel to the lungs, leading to a form of pulmonary hypertension that is reversible with time and treatment. Having pulmonary hypertension makes it more likely you'll develop clots in the small arteries in your lungs, which is dangerous if you already have narrowed or blocked blood vessels.
    * Arrhythmia. Irregular heartbeats (arrhythmias) from the upper or lower chambers of the heart are complications of pulmonary hypertension. These can lead to palpitations, dizziness or fainting and can be fatal.
    * Bleeding. Pulmonary hypertension can lead to bleeding into the lungs and coughing up blood (hemoptysis). This is another potentially fatal complication.

Is N-Acetyl Cysteine (NAC) Safe?

N-Acetyl Cysteine (NAC) is a stable form of the essential amino acid L-cysteine. Cysteine is a precursor for glutathione, an important antioxidant in the body. Cysteine also serves as a major sulfur source for many body components. It also may help with Alzheimer’s and also eye health.

However, recently some researchers at the University of Virginia Health System have found some potential side effects which may be troubling for some people who use this nutrient in high doses.  But…they are not sure, because these potential side effects have only been found in rats and may not occur in humans…and they did not occur in all rats.

The potential side effect is that NAC can form a red blood cell-derived molecule called nitrosothiol.  This could result in your body thinking that it has an oxygen shortage, which in turn can lead to pulmonary arterial hypertension (PAH).

Because it is likely that some of the media will jump on this ‘bandwagon’ I thought that I should mention a few important points about this first so any negative reports don’t raise any concerns for you.

1. The doses in their experiments were VERY high.  In fact, I sometimes wonder why the researchers do these experiments and then publish the results because it really is quite meaningless in the real world, unless they are trying to discredit a particular nutrient.  Almost every substance when taken in excess can cause a side effect.

2. Many body builders take very high doses of NAC.  Up to 2000mgs of more a day in 3 - 4 x 500mgs capsules.  I am unaware of any one having the side effect referred to by the researchers.

3. NAC is a valuable nutrient, and a safe one.  For more info and references concerning this please click here

Even though there is no evidence of any side effects in humans, as I say frequently you should never ingest any single ingredient in high doses.  They should always be combined with other nutrients and co-factors.

Unfortunately there is and always will be a group of consumers that feel more is better, and so when they hear some good results about a particular nutrient they want to take large doses of it and of course the manufacturers and marketers respond and give it to them.

Bottom line…NAC is SAFE. We use it in our formulas but in low amounts.  For example, we only use 35mgs of it in our Total Balance and that is with the full dose of 6 tablets per day.  It is an important nutrient and it plays a valuable role.
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