So, it’s time for me to address this. I’ve kept quite about it for over a year now. I’m more of the suffer in silence kind of person than I am a discuss issues in public kind of person. Recent events have pushed some version of this information out to more people and it’s become a talking point as I run into friends.
What is going on? This started over a year ago. I developed skin issues of a disturbing nature. When addressed by a doctor it turns out I have pustular psoriasis. “but that’s just skin issues you’ll be ok.” is one of the responses I’ve heard. However, that’s not true. It’s an autoimmune disease, and one that carries with it a significant amount of pain and additional issues. My particular diagnosis is chronic pustular psoriasis which is is distinct from psoriasis, with a different pattern of immune activation. My particular case is a combination of palmoplantar pustular psoriasis and acropustulosis. Either one is extremely rare.
If we want to talk genetic level this is associated with abnormalities in the cytokine (messenger protein) interleukin -36-receptor-antagonist signalling. This is due to recessive IL36RN gene mutations. (Thanks New Zealand for actually researching this)
How does this present itself? Well it does so in several awkward and painful ways. The cells on my hands and feet multiply at about a 10x normal rate and in a very abnormal way. I get the normal psoriasis symptoms of plaques of red skin, often covered with loose, silver-colored scales. These lesions may be itchy and painful, and they sometimes crack and bleed. In severe cases, the plaques of irritated skin will grow and merge into one another, covering large areas. Now my immune system also comes into play because it doesn't recognize this abnormal skin and attacks it. That means I also get pus-filled blisters on the palms of my hands and the soles of my feet. Along with pus-filled blisters on the tips of my fingers and toes. Often beneath the nails. The blisters can make my nails become deformed or fall off. In severe cases, the finger or toe bones may change shape or become deformed. This is all accompanied by fatigue, pain, fever, chills, headaches, and swelling in the joints, and occasional nausea from the condition. FYI the pus is non-infectious so no you are not going to catch this or anything else by shaking hands with me.
By the way, I have a severe case. Under normal conditions symptoms come and go with trauma and treatment every few months or so. Remission normally occurs within days or weeks of an outbreak. However, I have had a constant and chronic outbreak for over a year with only three weeks of relief from a very strong steroid injection that was supposed to last 2 months. There has been days over the past year I could not come into work because I couldn't walk or put on shoes.
Oh but wait there is more. Of the 21 autoimmune diseases studied, 17 were found to be linked to psoriasis, including alopecia areata, celiac disease, scleroderma, lupus, and Sjogren's syndrome. The autoimmune disease most strongly associated with psoriasis was rheumatoid arthritis (RA). It also comes with a higher risk for diabetes, high blood pressure, heart disease, metabolic syndrome, Parkinson's disease, kidney disease -- basically everything that kills people in high numbers, with some "debilitating" joint problems thrown in for fun. Not to mention I constantly have some area on my hands and feet in danger of a secondary infection.
"Are you sure that’s what it is?" Has been another question I’ve received. Yes, I’m sure this has been confirmed by various tests of;
- Laboratory tests for bacterial infection
- Skin biopsy, which shows superficial (subcorneal) pustules and neutrophilic infiltration on histopathology
- Blood count
- Kidney and liver function
- Electrolytes, calcium / phosphate levels
Generalized pustular psoriasis can be life threatening, so hospitalization is usually required. The aim is to prevent further fluid loss, stabilize body temperature and restore electrolyte imbalance. Specialist care is essential. I have not been hospitalized for this due to my constant watching of my fluid levels, and the fact it took so long to get an accurate diagnosis. By the time we figured out exactly what it was I had been jaunting all over the world dealing with it. The doctors decided that hospitalization would be invasive.
Death can result from cardiorespiratory failure during the acute eruptive phase of generalized pustular psoriasis so it is very important to treat it as early as possible. Elderly patients are at greatest risk. Other complications include:
- Secondary bacterial infection
- Blood count may show anaemia, neutrophilia, lymphopaenia
- Raised inflammatory markers such as C-reactive protein (CRP)
- Disturbed protein and electrolyte balance, especially low albumin, low calcium and low zinc
- Renal and liver impairment
- Malabsorption of nutrients and therapeutic drugs
Now this is the point where my concerned and caring friends will want to offer advise, treatment tips, or other words of how I should take care of this. Please don't. I have tried a lot over the last year. Both eastern and western and even some woowoo shit for the hell of it. This will not be solved by oatmeal baths or even bland topical compresses using emollients and low potency topical steroid creams. (Or high potency topical steroid creams for that matter I've tried both.) The possible treatments are;
- Acitretin (oral retinoid aka liver killer)
- Systemic corticosteroids (Very dangerous and controversial)
- Ciclosporin (immunosuppression)
- Methotrexate (Chemotherapy)
- Biologic agents (Targeted immunosuppression)
What I am taking now is a new drug on the market called Otezla (apremilast) which is a inhibitor of phosphodiesterase 4 (PDE4). Basically, it blocks my body from making the enzyme that a plays a major role in regulating immune cells' production of mediators of inflammation. It's way safer than the other drugs listed above. Also while all the other drugs only address the unsightly skin issues. This one addresses both the skin issues and the joint pain. However, side effects include:
- Tension Headache
- Rapid Weight Loss
- Suicidal Depression
I have experienced all but the last two. Feel free to speak up if you notice me in a depressive state. I do not expect this to happen as I have carefully read the white paper results on the clinical trials. The worst instance of this happening was a 1.0% during clinical trials. At average it was 0.2% chance.
This is not all that I’m doing. Daily exercise with tracts for working my way forward are already firmly in place. Smoking cessation plans are in place as well. That is a arduous process believe me I know. I did it once I can do it again. I stopped drinking alcohol, this is not due to the condition or the medication it kinda happened. I’m not opposed to a drink, but I have raised my standards on what I find acceptable to drink. Perfect cocktails only, and only when the occasion calls for it. My food intake has also changed, not that I’m on a diet. At the recommendation of my GP I’m being more mindful of my food and focusing on a Mediterranean lifestyle. Which is a high consumption of vegetables and olive oil and moderate consumption of protein primarily from fish.
I also have a great team of medial professionals working with me on this. A new GP and Dermatologist with their team of great nurses are providing me with great care and information. Even my insurance company is getting in on the action by providing me with a dedicated on call Treatment Manager that comes with 24 hour on call to nurses for questions and coordination between my various doctors and specialized pharmacy.
On the macabre side, currently there is no cure for this. This or complications from this is what will kill me barring no unforeseen accidents. The only way it won’t is if science catches up to where we can correct or turn off the recessive IL36RN gene mutations. I however will not loose any sleep over this. I plan to live life to the fullest as much as I can while taking care of my responsibilities, daily business, and self care. If we look at the odds here this cuts my expected life span by ten years. If I work hard to avoid comorbidity factors I will not compound that. If I don't then we can start subtracting five years for each comorbidity factor of circulatory disorders, genitourinary conditions, and respiratory disease.
That being said my life before this was full to the brim with work, family, love life, and various artistic projects. My new focus on self care and the various tracts of self improvement to help my quality of life maintain or improve take up a lot of my time. You may have noticed over the past year I haven’t been doing much in the realm of photography. This was partially due to mobility issues but even moving forward I will not be focusing on that. Photography was a way to have an artistic outlet when I moved on to the business side of music. I’ll still be open to actually special projects that I care about and will make a difference. When it comes to creating the same images that thousands of others online are creating I don’t have time.
The artistic side of my life is important to me. But, out of all the things I have in my life, work, family, love, it’s the artistic projects that will have to make room for my self care. This means a various amount of things will change. I will address them with those I'm working with these artistic projects on. I will still create and support where I can. Yet the result will be a large step back from everything that doesn't involve my work, or family
If you feel the need to reach out, connect, or set up some time to come see me I encourage it. I have great friends I never get to see enough of. Scheduling will have to fit around my daily work and exercise times. I will encourage healthy Mediterranean food and events that do not revolve around drinking.
Love you all and hope that this helps you understand what is happening.