A personal journey through the wonderful world of cortisol.
When I was a child, I clearly remember, my parents told me “I could change my moods very quickly” and I sometimes was tired “by doing nothing”. Typically this was in the evening or afternoon … In hindsight, with my current knowledge, I wonder if these symptoms were the first signs of ‘Cushing’, or just ‘adolescent behavior’?
From as early as 17-years, I often experienced pain in my legs, which were very tired and tingled. After I was married, my mother-in-law insisted on visiting a homeopathic doctor in Germany. She was the first who said: “this cannot be right”.
Much later, when I was already the mother of three young children, I regularly saw my GP with all kinds of ‘vague’ complaints. And never something concrete came out, certainly not a diagnosis. Often I was sent away with a reference to do some blood testing. It was so frustrating. It felt like not being taken seriously. Or if implicitly: it was not a physical illness, but just ‘between the ears’. After a lot of consultations, I didn’t visit my GP anymore. He never could say what it was, and I decided ‘to deal with it’.
Then, six years ago, we moved to a neighboring village. For practical reasons, our family switched to the local ‘village doctor’. A golden action! He immediately saw that with such a high blood pressure and a no. of vague complaints, there had to be something really wrong. He was the first doctor who told me to visit an internist in the regional hospital.
After my first consult with the internist, she had my blood tested on ‘cortisol’. Immediately I wrote it down, because I felt “this word is important and I have to search for it on Google”. It was a real eye-opener … I recognized all complaints!
I recognized directly many symptoms, but kept my cards during the following consultations. I wanted the doctor to have an open mind. To my fortune, the doctor acted appropriately. When she had ruled out her specialization – cancer – she sent me to a colleague. This colleague had an internship at the Department of Endocrinology in the UMC Saint Radboud. Her diagnosis was that I had ‘a disease’. To my question what disease, because I was keen to finally hear a name, she wrote ‘Cushing’ on a post-It and gave that to me. I was so happy that my suspicions were confirmed now. It was hard to explain to people that I was ‘happy’. But I finally had a ‘label’! Only then finally the treatment and cure could start.
In the Saint Radboud University Medical Center, all tests were repeated because there were varying results. At the end I was diagnosed with ‘Cyclic Cushing’. Unfortunately, after several scans of the pituitary and the adrenal glands, there were no signs of an adrenal adenoma. A diagnosis and a name, but still no root cause to all complaints.
A ‘sinus petrosus sampling’ indicated clear increase of cortisol levels on one side of the pituitary. This indicated – despite the absence of a clear adenoma – that the pituitary continuously sends signals to the adrenal glands to make more Cortisol.
The consequence was a disturbed energy management (overweight), an upset water balance (respiration and low potassium level) and a high blood pressure (my body worked at full capacity, even at rest).
Soon after, the neurosurgeon performed a pituitary surgery through the nose (transsphenoidal surgery), a piece of the pituitary gland was taken away. Since the operation I was extremely thirsty, which kept me awake at night. I thought to myself ‘diabetes insipidus’. Shortly afterwards I got a Thirst trial. In the morning only a mini little moisture in a glass to take the medication, nothing else! As if you are an addict whose drugs are taken away … The conclusion was indeed ‘diabetes insipidus’. The question was: temporarily or permanently? If I forget the medication desmopressin, I am very thirsty, so there is my answer …
Sometime later I got an Insulin Tolerance Test (ITT) , where during a day insulin, via the drip, is injected. The reaction of the body indicates if there is a deficiency of the adrenocortical hormone cortisol. In the beginning I started to read my e-reader, but soon I had to stop. From that moment the nurse came sitting next to me to keep an eye on me, because of complaints such as excessive sweating, hunger, but also heart palpitations. After the test I was very hungry.
More disappointing was that 6 months after surgery – unfortunately – we had to conclude that the Cyclic Cushing was still raging. In other words, the operation had failed.
Now that plan A (cure by removal of the pituitary gland) was failed, we had to rethink the possibilities of healing. We discussed all the options (do nothing, ketaconazole or irradiating SOM230 known as pasireotide or pituitary gland removal). After some consultations with my endocrinologist and many doubts, we decided to ‘remove both adrenal glands’.
It remains contradictory: remove healthy organs to heal something that is caused by the pituitary gland. Back to the ketoconazole with the associated inconveniences and much tension. The operation is performed by the urologist, via the back. These are actually 2 operations, in total 3 hours. The advantage is that this way other organs remain wrapped undisturbed in the peritoneum. In an operation via the ventral side, these organs are moved while getting at the adrenal glands. After surgery I could lie right back on my back. Very surprising!
Unfortunately during the recovery I got a novice pneumonia that delayed my discharge from the hospital with more than a week. When I finally was released, I had to take it real easy at home. Just lift something which is to heavy, or move too fast … sensitive! After 6 weeks ‘mechanical’ recovery from the surgery itself, now we are in what we call the ‘chemical’ recovery, which will last on average of 1 to 2 years. After that, I am officially cured of Cushing and automatically will be an Addison patient. Cured from Cushing I constantly suffered from, to the chance of an Addison crisis.
Lots of help
In my hospital room with 3 more patients, the other patients were surprised when my endocrinologist visited me and spoke with me about my surgery and recovery. They were very surprised, because he discussed it WITH me. Most doctors do not talk with their patients but TO them. A good relationship between doctor and patient and trust is so incredibly important. In addition, the Endocrine nurse gave very much additional information, about the recovery and above all practical matters. She really takes the time for me during an extended appointment.
And of course the ‘Carers’. Like my mother who came in the house … just ‘to be there’ for my family. The children who were washing and folding, cooking or getting wood. But Frank (my husband), who comes along with all major hospital appointments, constantly rearranging his schedule, but also cooks, shops, supports our family and me, as a patient. That makes ‘care giving’ a very special meaning!
Now for me to recover, to discover where my limits are and start my regular life, again.
Muriël Marks-de Korver