Rare Mitochondrial Disease, Destruction of Muscle Cells –
Rhabdomyolysis
(răb′dō-mī-ŏl′ĭ-sĭs)
.
Carnitine Palmitoyl
Transferase Deficiency,
is a rather rare
metabolic insufficiency,
caused by
a Genetic Inborn Error;
symptoms
are best managed
by dedicated prayer.
.
A Rhabdomyolysis Crisis,
or flare,
needs immediate,
Emergency Care!
.
Destruction of Muscle
is severe;
renal failure,
is a justifiable fear.
.
Emotional and
sustained stress
can trigger a crisis
of Rhabdomyolysis.
.
Myoglobin turns
urine dark red
due to bleeding out
of muscle tissue that is dead.
.
Open-line I.V. fluids,
to the veins are wired;
medication for acute pain-control,
is immediately required!
.
Upon late arrival of opioids,
severe pain will ensue the scream;
thus, being knocked-out,
is a glorious dream!
Rare Mitochondrial Disease, Destruction of Muscle Cells
Muscular Dystrophy’s rarer diseases include Inborn Errors of Metabolism. These complex mutations can cause havoc on the human body.
Carnitine Palmitoyl Transferase Deficiency causes an energy deficiency to the muscles, thus when certain stressors arise (including: lack of calories; high-fat diet; emotional stress; sustained physical exertion; menstruation; dehydration; lack of sleep; certain medications; and/or, infections), severe Rhabdomyolysis can occur.
Rhabdomyolysis is a crush injury to the muscles, as one might receive from being crushed underneath a building during an earthquake, being hit by a semi, or falling off of a tall building. However, with CPT-Deficiency, the Crush Injury (muscle destruction) is created from within one’s own body, but is still as equally painful as from an external force causing the destruction of muscle tissue.
Rare Mitochondrial Disease, Destruction of Muscle Cells
Rhabdomyolysis requires immediate emergency care at the hospital, as well as hospitalization in the Intensive Care Unit in many cases. Pain Control, Open-Line Intravenous Fluids, (to prevent kidney failure) and Heart Monitoring is crucial during a crisis.
Creatine Kinase Levels (evidence of muscle destruction) can go up, easily, to well-over 250,000-500,000 (normal range 50-150).
Due to the rarity of CPT Deficiency, it is important to have a standing protocol waiting at the hospital, at all times, so that when the patient experiencing severe Rhabdomyolysis comes into the ER, the hospital staff will know immediately what measures need be taken to secure adequate treatment, along with a favorable outcome.
It is not advisable to treat Severe Rhabdomyolysis at home due to its life-threatening nature (along with Electrolyte Disturbances, Shock, Heart Rhythm Alterations, Uncontrollable and Excruciating Pain, ETC.,.); however, some do treat it at home (though, it’s not advisable), because hospitals trigger fear and anxiety for many.
Rare Mitochondrial Disease, Destruction of Muscle Cells Summary:
Destruction of muscle tissue which can cause kidney failure; excruciatingly painful; spasmodic muscle contractions; weakness; inability to walk (or even move, in severe cases); paralysis of muscles during severe flares; confusion; nausea; projectile vomiting (like in The Exorcist movie); possible shock.
Rare Mitochondrial Disease, Destruction of Muscle Cells
Myoglobinuria:
Rust-colored urine due to deceased muscle tissue being released into the bloodstream, and through the kidneys
Malignant Hyperthermia:
People with metabolic myopathies are at high risk of developing Malignant Hyperthermia during the use of certain anesthetics. It’s a good idea to wear a Medical Alert bracelet stating this susceptibility. My bracelet accidentally fell through the air conditioner vent, so I need to purchase a new one.
Muscle Destruction (Rhabdomyolysis) Treatment:
1). Standing Protocol;
2). Massive, Open-Line IV Fluids For Hydration (Kidney Function);
3). IV Pain Control (Morphine; Dilaudid; OxyContin; Demerol; Fenatyl; ETC,);
4). Electrolyte Replacement via of IV Fluids;
5). Possible Ongoing PTSD Therapy due to Excruciating Pain;
6). Monitoring of Renal, Cardiac, & Liver Fuction;
7). Severe Nausea Control
8). Continuous Blood-Monitoring of Renal Function
9). Avoid certain medications and anesthestics;
10). MedicAlert bracelet
.
Isaiah 35:6
Then the lame man will leap like a deer, and the tongue of the mute will sing; for waters will break out in the wilderness, and streams in the desert.
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You did a beautiful job of getting in your facts, but presenting everything in a creative and interesting way that really highlights your feelings and experiences as a person with these conditions! Through your words, I can begin to imagine what it must be like for you living with such a condition, and I can certainly sympathize with what you experience despite my condition being so radically different from yours. Thanks for putting this out in the world. It’s important work and I’ll share it on my social media. Xx
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Awe, thank you, Michelle. That’s very kind of you. ♥️ ♥️ ♥️ Hugs!
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