Wednesday, 1 November 2017

Hypokalemic Estimated.

hypokalemic periodic paralysis is estimated to affect 1 in 100,000 people. Men tend to experience symptoms of this condition more often than women.

If a genetic disorder runs in my family, what are the chances that my children will have the condition?

When a genetic disorder is diagnosed in a family, family members often want to know the likelihood that they or their children will develop the condition. This can be difficult to predict in some cases because many factors influence a person's chances of developing a genetic condition. One important factor is how the condition is inherited. For example:
  • Autosomal dominant inheritance: A person affected by an autosomal dominant disorder has a 50 percent chance of passing the mutated gene to each child. The chance that a child will not inherit the mutated gene is also 50 percent (illustration). However, in some cases an autosomal dominant disorder results from a new (de novo) mutation that occurs during the formation of egg or sperm cells or early in embryonic development. In these cases, the child's parents are unaffected, but the child may pass on the condition to his or her own children (illustration).
  • Autosomal recessive inheritance: Two unaffected people who each carry one copy of the mutated gene for an autosomal recessive disorder (carriers) have a 25 percent chance with each pregnancy of having a child affected by the disorder. The chance with each pregnancy of having an unaffected child who is a carrier of the disorder is 50 percent, and the chance that a child will not have the disorder and will not be a carrier is 25 percent (illustration).

Información sobre los niveles anormales de potasio que los pacientes tienen con (HypoKpp)

Le doy información sobre los niveles anormales de potasio que los pacientes tienen con (HypoKpp)

La información que estoy poniendo se obtiene de muchos pacientes que tienen (HypoKpp) de todo el mundo que tienen el mismo problema con los niveles de potasio en la sangre.

Cuando obtenemos la sangre para saber el nivel de potasio y potasio sale con niveles normales como 4.0 4.5, pero tenemos ataques de parálisis, con niveles de potasio que son normales para los médicos, y que confunde a los médicos y las personas que están a nuestro alrededor.

Somos conscientes de que no todos los pacientes con (HypoKpp) responden de la misma manera a los tratamientos que realizan los médicos y que a veces los medicamentos no funcionan para todos, y que hay casos en los que los pacientes con (HypoKpp) Medicamentos lo hacen bien.

En pacientes con parálisis periódica no es necesario que el nivel de potasio caiga fuera del rango normal para que se desencadene un ataque: pacientes con HypoKpp si el nivel de potasio es normalmente alrededor de 4, entonces cualquier cambio por debajo podría causar un ataque.

3.5 está en el extremo inferior de la normalidad en todos los pacientes. Lo que es importante tener en cuenta es que, en el tipo de parálisis periódica, el potasio se desplaza fuera de la sangre, este es el nivel más bajo y, a medida que el ataque comienza a mejorar, los niveles aumentarán.

Los médicos están buscando el potasio disponible en el torrente sanguíneo. La parálisis periódica ocurre a nivel celular: puede tener gran cantidad de potasio en la sangre, pero si los canales iónicos no funcionan correctamente, los músculos seguirán débiles. Esa es una gran diferencia en el pensamiento del doctor,
Los doctores asumen que dado que el potasio en la sangre no es bajo, la Parálisis Periódica no puede ser la respuesta

Un  ejemplo (el tanque de gasolina puede estar lleno pero si su bomba de combustible no funciona, su vehículo no arrancará).

Los pacientes que tienen (Hypokpp) necesitan que el número de potasio sea alrededor de 4.5 *.

Los pacientes con niveles de potasio alrededor de 3.0 a 3.5 * están en parálisis total.

Information about the abnormal levels of potassium that patients have with (HypoKpp)

I give you some information about the abnormal levels of potassium that patients have with (HypoKpp) 

The information I am putting  is obtained from many patients who have (HypoKpp) from around the world who are having the same problem whit levels of potassium in the blood.

When we get the blood to know the level of potassium and potassium comes out with normal levels like 4.0 4.5, but we have paralysis attacks, with potassium levels that are normal for doctors, and that confuses the doctors and the people who are around us.

We are aware that not all patients with (HypoKpp) respond in the same way to treatments that are given by doctors and that sometimes medicines do not work for everyone, and that there are cases that patients with (HypoKpp) Medicines are doing well,

In patients with periodic paralysis the potassium level does not need to drop outside the normal range for an attack to be triggered – Patients whit ( HypoKpp ) if the potassium is normally level around 4 then any change below that could cause an attack. 

3.5 is at the low end of normal in all patients. What is important to note is that in the type of periodic paralysis the potassium shifts out of the blood, this is the lower level, and then as the attack starts to improve the levels will increase.

The doctor’s are looking at the available potassium in your bloodstream. Periodic Paralysis happens at the cellular level  - you can have lots of potassium in the blood but if the ion channels aren’t working correctly, the muscles will still be weak. That’s a big difference in doctor’s thinking ,
The doctors assume that since the blood potassium isn’t  low, Periodic Paralysis can’t be the answer

Like ( Your gas tank can be full but if your fuel pump isn’t working, your vehicle Still isn’t going to start. )

Patients whit ( Hypokpp ) needs potassium number to be around 4.5*. 

Patients whit Potassium levels around 3.0- 3.5* is in full paralysis.

New Update in Hypokalemic periodic paralysis Wikipedia


Autosomal dominant - en.svgHypokalemic periodic paralysis (hypoKPP) is a rare, autosomal dominant channelopathy characterized by muscle weakness or paralysis when there is a fall in potassium levels in the blood. In individuals with this mutation, attacks often begin in adolescence and most commonly occur on awakening or after sleep or rest following strenuous exercise (attacks during exercise are rare), high carbohydrate meals, meals with high sodium content, sudden changes in temperature, and even excitement, noise, flashing lights and cold temperatures. Weakness may be mild and limited to certain muscle groups, or more severe full-body paralysis. During an attack reflexes may be decreased or absent. Attacks may last for a few hours or persist for several days. Recovery is usually sudden when it occurs, due to release of potassium from swollen muscles as they recover. Some patients may fall into an abortive attack or develop chronic muscle weakness later in life.

Some people only develop symptoms of periodic paralysis due to hyperthyroidism (overactive thyroid). This entity is distinguished with thyroid function tests, and the diagnosis is instead called thyrotoxic periodic paralysis.[








Wednesday, 22 February 2017

Hypokalemic periodic paralysis

Hypokalemic periodic paralysis (HOKPP) is a condition that causes episodes of muscle paralysisassociated with a fall in blood potassium levels (hypokalaemia). Episodes typically involve a temporary inability to move muscles in the arms and legs. The first attack usually occurs in childhood or adolescence. Attacks can last for hours or days, and the frequency of attacks varies among affected people. The frequency is usually highest between the ages of 15 and 35, and then decreases with age. Some people with HOKPP also develop late-onset proximal myopathy.

HOKPP can be caused by mutations in the CACNA1SSCN4A, or KCNJ18 gene. Inheritance is autosomal dominant. Treatment varies depending on the intensity and duration of attacks. Minor attacks may resolve spontaneously, while treatment for moderate or severe attacks may involve ingesting oral potassium salts or intravenous potassium infusion.


Symptoms......Continued Here:
https://rarediseases.info.nih.gov/diseases/6729/hypokalemic-periodic-paralysis

Tuesday, 26 April 2016

Why Do I Use a Power Wheelchair When I Can Walk?

Someone asked me recently why I use a power wheelchair when I have the ability to walk. Why don’t I force myself to walk? Why have I given up?
The following was my answer:
Please do not judge me until you live a day in my life and understand the disabling medical condition I endure daily. I am able to walk a few steps with the assistance of a walker or cane, but due to a rare, invisible, chronic, hereditary, debilitating mineral metabolic disorder called periodic paralysis, my skeletal and breathing muscles are weak and progressively getting weaker, permanently. 
I have developed exercise intolerance. This means I am unable to do physical exercise or exertion on a level those others my age can. This condition also causes extreme pain and fatigue after exercising or exertion and other debilitating symptoms such as a feeling of heaviness in the muscle groups. 

Tuesday, 23 February 2016

Meet Jodie: Hypokalemic Periodic Paralysis

Jodie was diagnosed with hypokalemic periodic paralysis, a rare disorder that affects about one in 100,000 people. Despite adversity, he finds that life is good. He is a husband. A father. He knows the Diplomat Difference.



The Fight Against Hypokalemic Periodic Paralysis

20 Things Not to Say or Do to Someone in a Wheelchair

I don't necessarily agree with all of the responses, but I can relate to some of them and I really can't blame her for being frustrated.................

Please continue Here:  http://fightinghkpp.blogspot.co.uk

Myopathy of Hypokalemic Periodic Paralysis An Electron Microscopic Study

EREDITARY intermittent flaccid paralysis of skeletal muscle is at present classified into three types on the basis of the changes in the serum potassium during the paralytic attack. The hypokalemic form was the first to be clinically recognized, and is the most common of the three variants, more than 600 cases having been described in the literature by 1959.1 The clinical manifestations of this disease will not be detailed here as many reviews are available.2-5 Worthy of emphasis, however, is that permanent proximal muscle weakness may occur. This feature was first noted by Oppenheim6 in 1891 and was later thought to be a variant of spinal muscular atrophy occurring in conjunction with periodic paralysis.7 However, it has now become apparent that permanent myopathic weakness is not uncommon, and not dependent upon severe or repeated paralytic attacks.

Please continue Here: http://archneur.jamanetwork.com/article.aspx?articleid=568933