In 2007, we found out that my grandson (age 13) had a Pheo. He had cut his knee at school and my daughter took him to the ER for stitches when the doctor noticed his blood pressure. It was way too high. 180/110...it should have been 100/60.
The ER doctor told my daughter to take him to see the pediatrician. Never saying this is serious or critical. So she made an appointment and didnt get a day for about 2-3 weeks. As soon as they saw his blood pressure they were calling local hospitals to see if they would take him. The closer ones wouldn't...they sent him to CHOC (Children's Hospital of Orange County).
I went with them to the hospital. I told anyone who would listen that this may be a Pheo as he has VHL. No one would listen. They had to run tests etc. Pheo's are rare, I'm guessing they didn't think it was a Pheo. After 6 hours of tests and waiting for the results...Guess What? It's a Pheo.
My grandson was nervous. None of us knew what to expect. I went to the VHL Family Alliance boards and asked for help. I was given amazing advice and so thankful for the words and support they gave me...and the advice.
I was able to ask the doctors all the right questions.
They had to remove one of my grandson's adrenal glands. Adrenalectomy is what it is called. They took pictures, the tumor was about the size of a golf ball...we named it Phil the Pheo.
VHL in the Adrenal Glands
The adrenal glands are approximately 3 x 2 x 2 cm (1 inch long) perched on top of each of the kidneys. (See Figure 8.) VHL may be associated with a kind of tumor of the adrenal glands called a pheochromocytoma, (“pheo”). These tumors occur more frequently in some families than in others. In families that have adrenal involvement, they are quite common. They are rarely malignant among people with VHL (3%). Detected early, they are not difficult to deal with, but they are potentially lethal if not treated because of the damage they can cause to the heart and blood vessels and the potential for dangerously high blood pressure occurring during stresses such as surgery, accidents, or childbirth.
Pheos produce so-called “stress hormones” (noradrenaline and adrenaline) that your body uses to gain speed and strength in an emergency. The pheo secretes excessive amounts of these stress hormones into the bloodstream. The primary symptom is high or variable blood pressure, especially spiking blood pressure, that puts strain on your heart and vascular system and can cause heart attack or stroke. Patients may notice headache, increased cold perspiration, irregular or rapid heartbeat, or what feels like a panic attack, fear, anxiety or sometimes rage.
New research indicates that adrenal tumors are as much as four times more common among people with VHL than previously thought, and that traditional blood and urine tests alone are inadequate to find most pheos. It is recommended that all people with VHL be screened for pheos. Usually an initial test is done with blood and urine tests, and if additional information is required, or if there are symptoms of pheo but the blood and urine tests are negative, imaging tests or PET scanning may be used. It is particularly important to be checked for a pheo prior to any surgery, pregnancy, or childbirth. If a pheo is present, complications may be avoided by blocking off the effects of stress hormones with drugs, beginning about seven days before the procedure.
The accuracy of the urine and blood tests for pheochromocytoma activity will be determined in large part by your own cooperation in preparing for the test. Even if no instructions are provided, you should avoid smoking, alcohol, and caffeine for at least four hours before the test. Be sure to tell your doctor and the technician if you are taking any anti-depressant medication. You might want to prepare a list of all the medications you are taking, and discuss this list with the doctor before the test. Where other instructions are given, they may differ from center to center, sometimes due to different methods of analysis. Follow any instructions carefully to avoid a false reading.
If these chemical tests indicate the presence of a pheo, but it cannot easily be located on CT or MRI, an MIBG or PET scan may be recommended. These tests help to localize, or locate, a pheo, even if it is outside the adrenal gland. When they are outside, they are sometimes called paragangliomas. They may occur anywhere on the sympathetic nervous system, anywhere along a line drawn from your groin to your ear lobe. Multiple tests may be needed to find them.
If surgery is required, the standard of care these days is partial adrenalectomy. Studies have shown that keeping even a small amount of the cortex of the adrenal gland will make it much easier for you to manage after surgery. Even if you still have another healthy gland, remember that there may be another pheo in the future that could put that second gland at risk, so your goal should be to keep a portion of each gland working for you.
I am not a doctor or an expert by any means. Please visit VHL Family Alliance website for more information. www.VHL.org