This was taken a few months after photo 2, as Prof Hindmarsh lowered and adjusted the rates to what I needed. I no longer have a weight issue afterbattling with my weight all my life.
Spring 2008 Newsletter
CARES Foundation, Inc.
Continuous Subcutaneous Hydrocortisone Infusion-Pump Therapy as a Way to Mimic Normal Cortisol Profiles
by Peter Hindmarsh, MD, SM Bryan, MD, David Brown, MD and Chris G.
Dr. Peter Hindmarsh, a member of the Development Endocrinology Research Group at the Institute of Child Health at University College London in the United Kingdom, has used continuous hydrocortisone infusion therapy to treat congenital adrenal hyperplasia (CAH) in one of his patients, Chris. He has reported on this experience at a number of endocrinology meetings. The treatment involves a continuous subcutaneous (under the skin) injection of cortisone through a small needle that is left in place 24 hours a day. The equipment is identical to that used by diabetics who are treated with a continuous insulin infusion and is adapted to inject hydrocortisone instead of insulin. The rate of injection can be varied to mimic the natural cortisol secretion rate (see chart) and can also be increased to cover times of stress or illness. Besides controlling the infusion rate, the patient must periodically change the injection site.
Hourly pump infusion rates are shown in black. Hourly blood cortisol concentrations are shown in white.
This treatment is considerably more complicated and intrusive than taking a few tablets by mouth each day. However, for someone like Chris, with serious CAH control problems, it can be useful. Chris’s problems included debilitating headaches, excessive weight gain, anger, depression, acne and gastritis, depending on which oral treatment he was taking. He was treated first with increasing doses of hydrocortisone, next with prednisolone and then with dexamethasone. Control was never satisfactory, and he always experienced some combination of the unwanted side effects. Finally, during puberty, he was switched back to hydrocortisone, but then he experienced weight loss, weakness, and dizziness and was unable to attend school. Although at first reluctant to try the continuous injection treatment, he was so desperate, he decided to give it a try. He is now a university student and is delighted with the results.
To quote Chris, “I have been on the pump for over two years: I think I probably have the best controlled CAH in the world. Another bonus is that I started to grow again… and am over 5’9”. I go to the gym as often as I can. My weight is stable and everyone says I am very lean. I do battle with accepting that I am not fat and worry about my weight, but I realize now that my weight was not my fault. Of course you have to be careful with what you eat, but I am now certainly eating more than I ever did in the years where I battled with my weight, even though I am on the same dose. I think it is the way the hydrocortisone is delivered, slowly and continuously at a rate which has been specially tailored to suit my body’s needs. It took some getting used to having it attached to me all the time, especially at night, but I am now so used to it that I feel strange without it.”
“You have to be disciplined in changing the site regularly, but really it’s a very small price to pay when I consider what advantages this method has given me. The pump gave me my life back, and I will always be grateful to Professor Hindmarsh and my mum, as I could not have got through any of this without her constant love and support.”
This was before I started using the pump and I was not getting enough cortisol.
The pump was so efficient that after 2 months, I started to show signs of over suppression with a puffy face, so we reduced the rates.
© 2008 CARES
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Disclaimer: Any communication from CARES Foundation, Inc. is intended for informational and educational purposes only and in no way should be taken to be the provision or practice of medical, nursing or professional healthcare advice or services. The information should not be considered complete or exhaustive and should not be used in place of the visit, call, consultation or advice of your physician or other healthcare provider. You should not us the information in this or any CARES Foundation, Inc. communication to diagnose or treat CAH or any other disorder without first consulting with your physician or healthcare provider. The articles presented in this newsletter are for informational purposes only and do not necessarily reflect the views of CARES Foundation, Inc.