Medication

Kalium VerlaThe medication I use to keep the hypokalemic periodic paralysis attacks under control is at this moment “Verla Kalium” (Kalium=Potassium) . It comes in boxes containing 20/50/100 sachets .
Each sachet contains 20 mmol = 780 mg Kalium . I use depending on how the day goes about 6 to 8 sachets a day.

Because I don’t have a colon I have the problem that I have to eat every 3 hours, else my liver kicks in and releases glucose what triggers an attack too.
That’s why have an alarm set at night at 03:00 and 06:00 to take kalium (potassium) and to eat something. This way I prevent an attack in the morning.

If I can’t manage the attack because of whatever reason and I am just about able to move a little and very slow I use 1 sachet Verla Kalium and an Ibuprofen 600. Ibuprofen raises the Kalium level too for a little longer period of time. An half hour after I took the first sachet Kalium I take the second on and most of the time that will help to get the recovery started and then I take every 2 hours a sachet kalium until I can walk again.
An serious heavy attack takes from 6 hours up to 12 hours to recover from.

This week (28-11-2017) my doctor asked me to try Kalinor capsules.
The kalinor capsules contain 8 mmol = 315 mg Kalium and releases the Kalium more slowly. I started at 06:00 with the first capsule and at I0:00 the first attack kicked in.Kalinor

I know now for sure that 3 capsules spread out over 24 hours isn’t the solution for HYPOPP.
I know when to expect an attack I know the triggers so I can anticipate the attack with the sachets and that’s a much better solution until now.
When I feel an attack coming I take 1 sachet Kalium and take a time out and most of the time that’s enough to stop the attack from getting worse.

Jaap

HYPOPP DNA test gone bad

If you have read the other pages on my blog you know that I don’t have very much confidence in the people who I have to deal with when I go to a doctor or a hospital. I mean they studied and know what they have studied but that’s it, if you ask to think a little further it seems the bucket is empty right away there is nothing left, also in this case.

I went to a “Praxis für Humangenetik” that’s German for a for Human Genetics laboratory. I travelled for 1,5 hr to get there, spend 20 minutes in the waiting room and listened to the explanation of the DNA test (already read that on the Internet) and gave the doctor my info in about an hour .
During that hour I got an HYPOPP attack and at the end I wasn’t able to write my autograph down.

The doctor told us (my wife was with me) that it would take about 8 weeks until the results are there. We asked, will you test my DNA on all three known genes (CACNA1S, SCN4A and KCNJ18)? And the doctor confirmed they will.

Now three months later we finally got the phone call we waited for.
The results – nothing found on CACNA1S and SCN4A the doctor said “it took a while to get in contact with my colleges to ask them if we needed to test the KCNJ18 too. But the changes are so small that KCNJ18 contains the problem so we didn’t test the KCNJ18 gene” !

 

On Living with Periodic Paralysis: The Blog you can read:

The professionals say only about 50% of those tested will have a positive finding because only about 50% of the known genetic mutations have been found. Right now that covers at least five known different mutations, SCN4A, KCNJ18, CACNA1S, KCNJ2, KCNJ5

So the doctor decided for me without further investigation that the DNA failure couldn’t be found in the  KCNJ18 gene , the Human Genetics laboratory didn’t even know about the KCNJ18, KCNJ2 and KCNJ5 gene!

Well I have to be patient, I try to find a doctor who is able to make the proper decisions in rare cases and have the courage to look further.
In march 2018 I have an appointment with another neurologist (no.?), let’s hope….

In the meanwhile on the NCBI website  everyone can read:

“Consensus diagnostic criteria for primary hypokalemic periodic paralysis have been published in a Cochrane review [].
The criteria:”

OR
Three of the following six clinical/laboratory features:

  • Onset in the first or second decade
  • Duration of attack (muscle weakness involving one or more limbs) longer than two hours
  • The presence of triggers (previous carbohydrate rich meal, symptom onset during rest after exercise, stress)
  • Improvement in symptoms with potassium intake
  • A family history of the condition or genetically confirmed skeletal calcium or sodium channel mutation (We don’t know for sure)
  • Positive long exercise test (see Testing) [McManis et al 1986]

I score 5 out of 6!


Sources:
NCBI Hypokalemic Periodic Paralysis
– Living with Periodic Paralysis: The Blog by Susan Q.Knittle-Hunter