Below is a list of other medications and procedures that PCers have tried.
Botulinum Toxin and PC
(Botox, Dysport)
COMMENTS: “No one fully understands why Botulinum toxin works for PC at all. It helps people with hyperhydrosis (too much sweat), and so the thought is that it reduces the sweat and may, therefore, reduce the blistering. It doesn’t affect what is really going on with the keratin genes. There is no information on the amount of the drug needed for PC injections versus that used for cosmetic (wrinkles) or other treatments. As far as we know, there is no published information on how the drug is stored or retained in the body following treatment for PC. There are studies for other uses of the drug.”
COST: In Sweden, you pay a yearly fee and then everything is free. In the USA, the cost estimate is over $100,000 for a single treatment because of the need to have a general anesthetic administered in a hospital.
Drug Data
Wikipedia has an excellent article on Botulinum toxin, its purpose, and other uses.
Botox — Allergan, Inc.; [1]
Also see wikipedia article on Allergan
Dysport — Ipsen ispen.com
Dysport website
US National Institutes of Health Articles:
Botox
Dysport
Best Practices
We have learned there is a specific way that works best for Botox. Including:
- It is not possible to do the injections with only cryosthesia because the pain for PCers is horrible. The injecting physician must employ nerve blocks before injecting the toxin.
- The dose should be 200 per foot.
- Other measurements will need to take place.
The following is the best treatment protocol to use for those with PC. The last author has a lot of experience working with botulinum toxin and PC. If your dermatologist would be willing to be instructed, we can connect them.
Title: A treatment protocol for botulinum toxin injections in the treatment of pachyonychia congenita-associated keratoderma.
Journal: Br J Dermatol Authors: Koren A, Sprecher E, Reider E, Artzi O
Abstract:
BACKGROUND:
Severely debilitating plantar keratoderma pain is the most distressing clinical feature of pachyonychia congenita (PC). Several earlier publications reported therapeutic success with botulinum toxin (Btx) plantar injections.
OBJECTIVES:
To describe our 4-year experience during which we administered a total of 30 plantar Btx injections to five PC patients following an optimized protocol.
METHODS:
Five patients with PC (age 21-54 years) who were treated at our medical center from 4/2015 to 6/2018 were included in the study. After an ultrasound-guided nerve block performed by an anesthesiologist, the patients received plantar intradermal injections of Btx A. To ascertain the effect of the treatment, we used a dedicated quality-of-life questionnaire for PC patients (PCQoL) and asked the patients to evaluate the intensity of eight parameters pertaining to their symptoms at baseline and before every treatment session. At study closure, patients were asked to evaluate the maximal improvement in the same eight parameters throughout the study period.
RESULTS:
All patients demonstrated a decrease in PCQoL scores during the follow-up period. All patients showed a significant improvement in PCQoL after the first treatment session and at the last evaluation (P = 0.043). The best improvement scores concerned morning feet burning and long-distance walking (> 500 m). The scores were significantly lower if the intervals between Btx injections were <100 days.
CONCLUSIONS:
Btx treatment of PC-associated keratoderma following an optimized protocol leads to a major change in patients’ quality of life.
Other Patient Experiences
K6a patient: “I have had the treatments every 2-1/2 to 3 months for the past 2 years. It is not 100% (sometimes works better than other times). One time, the anesthesia didn’t work and it was terribly painful. From that time on, I was put to sleep (general anesthesia) to have the treatment because it hurts too much otherwise. My sister also has the treatments, but cannot use the general anesthesia as it makes her sick. My sister waited a little longer this last time as it is so painful to have the treatments. Our cousin has chosen not to have the treatments.
Side Effects: I cannot read fine print very well, my eye doctor thinks I am too young to have this condition and they think it could be from the Botox treatments as my sister is also having this same effect.”
K17 patient: “Here is some information, from my point of view. There are 3 people in my family who have been treated. My sister and two others have done the treatment several times. I have only done it once. It lasted for about 6 months for me, but it took about 3 weeks before I felt any difference in the feet.
I had to start the treatment with only one foot, and the second foot was treated a little more than one month later. The good thing with that was that you could actually SEE the difference: The treated foot had less blisters than the untreated foot!
The treatment was no miracle. My feet didn’t become painless, but it sure reduced the sweat (the feet got really dry…). It took a longer time before I reached the worst “pain level” after a day of work. I got less blisters and my feet produced less calluses during the time the (drug was) in the feet.
And the needles… in Sweden you get anesthetic. There are two types, either you get full anesthetic (you’re “asleep”) or you get a local one, where they block the ankles and the feet are pointed upwards. I did the last one, and I thought that was the worst part of the treatment! Next time I do the treatment I would rather have the full anesthetic, but the good thing with the ankle-anesthetic is that it is possible to walk afterwards without any problems!
(This) is a drug and it might not be the best solution for the body, but eating painkillers all of your life is not good either.”
Early Studies
Although other patients with conditions ‘like’ PC have been included in some treatments, we know of only a few patients with confirmed PC who have been treated. Five were treated in Sweden and one was treated in Ireland. In the study in Sweden, Dysport or Neurobloc (not Botox) was used. We do not know what product was used in Ireland.
