Welcome to HPP and Me
HPP or Hypophosphatasia is an ultra rare progressive metabolic bone disease with lifelong impact. I live with this condition and wish to share my experience of the condition and also the NICE Managed Access Agreement to 'Strensiq' (Asfotase Alfa) in the UK. My aim is to raise awareness of HPP through my blog HPP and Me so more people in the UK can access this medication.
I am a lay person and not medically trained. All views are personal and I am not able to give advice on the condition. Always see your Health Care Provider.
Pain has been part of my life for as long as I can remember. As a child I didn’t know I had a rare condition all I knew was that I was in pain most of the time. The pains used to move about and I remember complaining to my Mum about it. At birth I was a floppy baby and the doctors recommended traction to help. I couldn’t sit up without propping as a youngster. I remember visits to the surgery for ‘growing pains’ and ‘foot issues’. To cut a long story short I had an operation on my back at 13 and more recently I have become a ‘pin cushion’ with all the cortisone injections I have had throughout my body. I have had bony spurs and inflamed tissue removed. I’ve had invasive operations in both shoulders and also a total shoulder replacement. I have also survived dark times of depression. Apart from that I am ‘fine’!
I always wanted to work so despite the challenges I have faced I have managed to adapt my situation and create a lifestyle to work with my difficulties. For example because I wasn’t able to work for others due to tiredness, pain and stamina I have been self employed so that I can plan my days or even work from the sofa or bed if needs be.
I have two daughters and 4 wonderful grandchildren. I am relieved that at the moment none of them seem to have symptoms of HPP. My family have been my rock over the years and they know how I am feeling by just looking at me. My husband is extremely supportive, we have been together since we were teenagers and so he understands my needs before I even mention them.
For many years I didn’t complain in public about my problems and would try and just carry on regardless but then ‘repent at leisure’ for days. More recently I have found that I can no longer hide my symptoms and in some ways that has been helpful as now some people will offer help or consider that I need to sit down, help me with my coat, open doors etc.,
Living with a long term health condition often means you have some very difficult times. Some of the hardest issues to come to terms with are losing your independence and having to rely on others. Likewise losing the ability to join friends doing activities or just looking after your grandchildren on your own. To deal with this I have found I need to take antidepressants all the time and it helps. Volunteering has also played its part in distracting me from dwelling too much on my restrictions as I have gained much satisfaction through helping others and my community. I have learned that taking time for myself is essential too and I spend quite a lot of time creating artwork. I work with hot wax and hot tools (encaustic art) as I find that the heat helps with the pain. I become so engrossed I can lose all track of time. If you are interested in my artwork you can view my website.
My husband is retired and this now means that I can do more than I used to as we can share the burden of the housework, cooking, etc., which used to sap me of all my energy. It also means that we have more time to do ‘fun’ stuff. One of my biggest concerns has been losing my muscle tone, being overweight and generally being unfit. I have done a lot of research and have just bought myself an electric recumbent bike. There is a proper seat and the steering is at hip level. Now I can get outside in the sunshine, do some exercise and get some vitamin D. I can even just sit there in comfort if I get tired. Exercise has been a huge problem for a long time as so many activities are too strenuous or painful. Having found I can cycle is a massive boost and a joy.
The future will be challenging but I hope I can retain my ‘glass half full’ attitude to life, keep busy and find ways to adapt.
Thanks for your interest in HPP and Me.
For support groups and useful links in the UK see below:
Raising awareness of HPP, to determine early diagnosis, and to support and advocate those diagnosed with HPP and their families in the UK.
Support services to patients and families
Rudy is a study in Rare diseases aiming to transform clinical care for participants through patient driven research.