1.3 Copper (Cu)
The
Excess Copper (ExCu) or, more specifically, the excess of
Copper (Cu) ions in the Pompe affected Nervous System (PaNS), is the primary factor responsible for symptoms in the Pompe Disease (PD). The reason it is listed after
Nerve Toxicity (NT) is related to structural text decisions, not rational ones.
The
Excess Copper (ExCu) in Pompe Disease (PD) affects the entire body. Even if most of the time the
Excess Copper (ExCu) only traverses through the Pompe affected Nervous System (PaNS), also the Brain (B) is affected to some extent because of the
Soaking-In/Pumping-In Process (SIPIP), detailed in
1.1.2.1 Soaking-In/Pumping-In Process (SIPIP). By affecting the Pompe affected Nervous System (PaNS),
any physical activity in the body is a high burden for the body. This makes
Excess Copper (ExCu) the most disabling factor in Pompe Disease (PD).
An important point regarding the
Myozyme Cycle (MC) and
Excess Copper (ExCu) is that, by the author's own experimentation, it is impossible to damage the nerves of the Pompe affected Nervous System (PaNS) during a
Myozyme Cycle (MC). This does not come to say it is impossible, just that the necessary overburden for the Pompe affected Nervous System (PaNS) would be so high that it was deemed to be practically impossible. This highly contrasts to the effects of
Excess Copper (ExCu) outside of the
Myozyme Cycle (MC) where it can easily damage any section of the Pompe affected Nervous System (PaNS).
Excess Copper (ExCu) is accumulated as a consequence of the intake of food. If this food has higher amounts of
Copper (Cu), more
Copper (Cu) will be accumulated, and vice versa. In general, the same dietary recomendations for patients of Wilson Disease (WD) should be followed by every patient with Pompe Disease (PD).
Having gone through the whole range of
Excess Copper (ExCu) amounts, from 100%* in 2011 to 0% as of 2023, the following 5 stages can be observed:
- Stage I: 100% (2004-2011)
The first stage predates the
Myozyme Therapy (MT). During this stage, symptoms were only due to
Excess Copper (ExCu) accumulation and movement across the Pompe affected Nervous System (PaNS). In the author's case, the
Excess Copper (ExCu) led to bedriddenness. Movement of
Excess Copper (ExCu) is detailed in
1.3.4 Copper Dynamics (CDs).
- Stage II: 100% to 58% (2011-2016)
The second stage starts with the full amount of
Excess Copper (ExCu) at the time the
Myozyme Therapy (MT) is started. This must not be necessarily the same amount than in Stage I (2004-2011), because of other possibilities of binding
Excess Copper (ExCu), as was the author's case. Relative to the
Myozyme Therapy (MT), at the start of this stage, symptoms are at its highest intensity. The author needed approximately 5 years of regular Myozyme Infusions (MIs) to make the first big leap: being able to walk 5 minutes in a row, that is, without having to sit down, or lay down in between.
As indicated by the percentages, almost 42% of total accumulated
Excess Copper (ExCu) were needed to be removed before reaching such a seemingly "meager" step forward. The reason for this is because
Copper (Cu) is highly toxic and disabling with regards to normal body activity. For the body to be able to resist its negative effects during activity, a big amount of it needs to be removed first out of the Pompe affected Nervous System (PaNS). Under this perspective, being able to walk 5 minutes in a row without having to sit down for a rest is a
huge step forward.
- Stage III: 58% to 25% (2017-2020)
Being able to resist the effects of
Copper (Cu) on the Pompe affected Nervous System (PaNS) implies the body must still struggle with these effects. That is what this stage was for, for stabilizing the previous achievement of Stage II (2011-2016) and further decrease the negative effects of
Copper (Cu) on the Pompe affected Nervous System (PaNS) by a continued reduction of it.
- Stage IV: 25% to 0% (2020-2023)
Lastly, the negative effects from
Excess Copper (ExCu) became low enough for the author to be able to sit, move with some difficulty and even speak. Symptoms did not disappear completely when reaching 0% of
Excess Copper (ExCu). The reason being that the
Nerve State (NSt) still accounted for its share of all symptoms, and now without
Excess Copper (ExCu), it was responsible for 100% of the remaining symptoms. The predominant factor among the Nerve State (NSt) that was responsible for the symptoms was the Nerve Pressure (NP).
Once all
Excess Copper (ExCu) is removed, a reaccumulation occurs during the
Myozyme Cycle (MC). As stated above, the amount of
Copper (Cu) reaccumulated depends on the amount of
Copper (Cu) intake with food. The author applies a low
Copper (Cu) diet since 2005/2006, which reduces
Copper (Cu) intake to a minimum. Nonetheless, there is still a small amount that reaccumulates during each
Myozyme Cycle (MC). This is a high burden for the body. Refer to
Copper Burden Effect (CBE) in
1.3.4.1 Copper Burden Effect (CBE). The
Copper Burden Effect (CBE) has been experienced mostly during Stage I (2004-2011).
To represent this extremely low amount of reaccumulated
Copper (Cu), the symbolic value 0.1% was chosen. It does not reflect any real measured value.