The day I've been counting down to finally arrived!
As you all know I've been suffering from severe chronic pain in my legs. Now I've always had painful legs ever since I was a child. I've had a few diagnoses.. First of all it was growing pains, but I stopped growing (too early! 😁) but the pain continued, then it was restless legs, then it was migraines of the legs, but none of these made sense! I’ve always taken para-codeine, the strongest dosage for my body weight, but only at night, as it makes me sooo sleepy! There’s only so many painkillers I can take to stop this pain and I’ve had enough!!
I've always had low oxygen saturations in my feet, and something always mentioned was that I had a high lack of oxygen reaching my lower limbs, causing the pain. Of course with a hole in my heart and my oxygenated blood not being able to pump around my body easily due to the PH, it made sense. Anyway the pain has continued and it has recently gotten worse and worse. On bad days I can't even stand up due to the pain and some days my husband has to carry me from our bed, to the bathroom or to the sofa. If Terry isn't around, I hobble around in the house on crutches, so I don't put all of my body weight upon my legs. If need to go out, I use my wheelchair. It's all been a bloody nightmare and it's made me so miserable!!
A few months ago I watched a video (link) on Pulmonary Hypertension with Eisenmenger's Syndrome. This video covered "Musculoskeletal Complications". It stated that it could possibly be the Endothelial Vascular Growth Factor bypassing the lungs. Now, from having that explained to me today, I understand this being a factor that is supposed to stimulate the formation of blood cells to oxygenate the bone tissue in all our limbs. Obviously with our legs furthest away from our heart and having PH, it's even worse for the factor to do this. The bone tissue isn't getting enough oxygen to form the tissue properly leading to bone pain, causing inflammation of the muscles around the bone.
This has a name: PERIOSTOSIS - I finally have a diagnosis for my leg pain!!!!! I am so glad I have pushed this as even though it is another diagnosis to add to my list, it is good to know what the problem is, meaning consultants can start prescribing the best medication for this condition.
What is Periostosis...
Pachydermoperiostosis (PDP) is a primary hypertrophic osteoarthropathy, characterized by various clinical expressions with the involvement of digital clubbing, pachydermia, seborrhoea, periostosis and arthritis or arthralgiaabnormal. Periostosis is the deposition of periosteal bone, manifested by the growth of periosteoma... (long bone pain)
I am now under an Endocrinologist and he has helped me with a 3 month plan. I have started a tablet called Salicylate, aka Aspirin, 300mg. I can take up to 4g a day, which equals to 13 tablets a day. I will be taking this for 1 month. However if the Aspirin doesn't work after a month, I can change to Bisphosphonates in tablet form (dosage not confirmed yet). I will try these for 2 months and then I will be returning to my consultant to discuss how I'm getting on and if I need to move on to the final solution. This solution is having Bisphosphonates intravenously as a yearly injection. So there we have it, my plan for the next 3 months!
As everyone knows, trials are the only way we and medics can find out more about our own conditions. There isn't much evidence to show that Bisphosphonates are beneficial for patients, especially for those with Pulmonary Hypertension with Eisenmenger's Syndrome, but I can only try and it is a step forward! I've targeted PH with ES because Periostosis is more common in people with this type of PH. So please keep your fingers crossed that this is something positive for us. It is very exciting for not just myself as a patient, but for those with PH & Eisenmenger's Syndrome and my PH specialists. The Royal Brompton are keeping well informed with this trial. I will keep you all updated over the next 3 months.
Let's hope I won't be in severe pain in a few months time and my pain will be a lot less. I hope one day I will be to control pain flares that happen in the future! I know I will always have chronic pain as its part of my Pulmonary Hypertension, but being able to control it will be wonderful! 🤞
NB: If you have PH and you're already on any of the tablets for this condition, please get in touch with me on any of my social media or email me firstname.lastname@example.org - thank you!