Von woke up with a low-grade fever, and he coughed nearly the whole night long. It seems like he has the never ending cold. Everyone says that kids are sick for the first 2 years, but I never imagined it to be like this for him. He can’t seem to catch a break. A friend of mine asked me yesterday does his PHP make him more susceptible to illness. The short answer is that his immune system is fine. He cannot get ill any more easily than anyone else. The long answer is that if he is not treated properly with his medication he can get sicker than the average person, and the illness can drag out longer than the average child. I thought I would go through some more commonly asked questions about his illness to help you better understand how we treat him. When I refer to treating him, I am speaking about the amount of steroids or replacement cortisol I am giving to him. The drug that he takes is called Hydro-cortisone. The biggest factor in his illnesses making it harder on him is his adrenal insufficiency. I thought I would also detail a bit more in depth about what his adrenal insufficiency is and how it effects him. Since the first blog seemed to really help people understand in an easy format, I thought I would stick with what I know.
What is adrenal insufficiency:? Adrenal insufficiency is when your adrenal glands are not releasing enough of the steroid Cortisol in to your system. There are several causes of Adrenal insufficiency, but Von’s is caused by his lack of pituitary gland. The pituitary gland is in charge of speaking to the adrenal gland, and it tells it to release the steroid in to his system. Because Von does not have one, the adrenal glands are actually functioning glands, but they do not have the master gland telling it what to do. Von’s form is due to the lack of ACTH the hormone the pituitiary gland releases telling his adrenal gland to work. They commonly refer to this as secondary adrenal insufficiency because his glands would work if the ACTH was released from the pituitary gland.
What are the symptoms of adrenal Insufficiency? The most common symptoms of adrenal insufficiency are hypoglycemia (low blood sugar), dehydration, weight loss, dizziness, tiredness, and lethargy. Other symptoms that can occur would be low blood pressure.
What were Von’s most present symptoms? Von’s most present symptoms prior to regular medication were low blood sugar and low blood pressure. The average baby has a heart beat of about 120 beats per minute. Prior to Von’s treatment his beat around 70 beats per minute. Scary, huh? Yep, this scared the heck out of the PICU staff. His other present symptom was his hypoglycemia. When he had his near fatal episode, his blood sugar fell to 7. To give you perspective, the average person has a fasting blood sugar of 80 – 120. They consider anything below 60 critical, and the further down it goes it becomes life threatening. Von should have been unconscious when his blood sugar was 7 but he was fully alert. He should have been convulsing or seizing, but he was awake. They had never seen that before, and could not believe he was even alive at that point.
With medication does Von still deal with hypoglycemia? Yes. Von would be hypoglycemic without the medication. In fact, without the regular medication, he would not be able to live. His medication is critical to his survival. It would be comparable to a diabetic being unable to survive without insulin. He will wear a medical alert bracelet for his entire life. He is what medical folk consider “steroid dependent”.
How do you manage this condition? This is the trickiest part of his whole PHP diagnosis. The adrenal glands are your fight or flight mechanism. When you are sick, they release extra cortisol, or what you or I would call adrenaline, in to our bodies. The cortisol gives your body the extra boost it needs to fight the infection. It is not part of the immune system, but it is sort of the backbone behind the immune system. If your body cannot sustain the stress of the illness, it truly doesn’t matter that your immune system is fully functioning. In order to balance this we have to give Von more steroids depending on his symptoms. On a normal day, Von would be given a single dose of his medication, and it would be given three times per day. He gets a dose right when he wakes up, after he goes down for an afternoon nap, and then right before bed. This enables his blood sugar to stay at a normal level. Reasons we would give him more medication are for when he has a fever, diarrhea, vomits, or simply doesn’t seem to be “himself”. I’ve had to learn to understand what Von’s baseline of normal is, and I have had to determine what to give him for medication based on that baseline. The doctors told us he wouldn’t need medication for a cold or for teething. I’ve learned Von simply can’t handle these stresses without at least a double dose of his medication.
What happens if he has an emergency? An emergency for Von would be if he vomits, breaks a bone, has significant emotional stress, etc. It would be when we have provided him the oral medication and he simply cannot come out of the lethargy or the low blood sugar on his own. At this point, we would have to give him a shot of Solu-Cortef. This is an emergency shot, that is a lot more concentrated and gives him an instant boost of adrenaline. If that fails, Von would have to be transported to the hospital. They would hook him up to a glucose drip and IV solu-cortef to help his body fight the stress. Thankfully we have not had to deal with this so far. The closest we have gotten was he was really sick. He was throwing up at home. We gave him a triple dose of his medication, and his blood sugar was still only 80. It was bedtime, and it would be irresponsible and potentially fatal to put him to sleep witha bloodsugar of 80. We immediately ran downstairs and gave Von juice. He drank about 3-4 ounces of apple juice and his sugar rebound to 112 within a few minutes. We checked over the next hour and it was 144 at the last check.
So Von actually needs sugar? Yes! Some parents are very strict about giving their children sugar, but a hypoglycemic needs sugar in order to function properly. I was a very healthy and natural eater prior to Von. We kept no juice and minimal sugar in the home. Now we have to have juice, and snacks that have sugar in them on hand. If Von is sick, we give him juice vs milk. The juice gives him an instant boost of sugar for his blood. Where a normal child may get a sugar rush, it makes Von act like himself. I always feel weird giving him juice or letting him eat cookies or sugar filled kid snacks, but he has to have it to keep his blood sugar stable. We are part of a group of parents online for children with PHP. I thank this knowledge on the friends I’ve made. They taught me that to have sugar on hand is really important and can prevent adrenal crashes.
I really hope helps you better understand what we deal with on a daily basis. I can only explain it so much, I think those that see Von and know Von better understand the challenges we face. The hardest part that I deal with as a mother, is that due to this adrenal issue we are never really able to travel far from Von, and we can rarely commit to plans. Due to his age, he gets sick, and he gets sick a lot. The doctors promise me this is normal. However, illness is a lot harder on Von because we are mimicking what the adrenal glands do. The normal gland would release the steroid continuously, but Von only gets it three times per day. So it makes it challenging for him to really fight the illness like a normal child. This is the part that has isolated us the most. I know I mentioned that we have lost friends. I truly feel our inability to see people was part of the reason we have lost people. We simply cannot commit. This in turn makes both Todd and I feel like we are constantly letting people down, and it makes us both feel incredibly isolated. Right now it is pretty much the three of us against the world of PHP, and our primary focus is keeping him healthy. Most people don’t really understand his condition, and that further isolates us. My hope is that those of you that know us,are willing to meet us at our home vs expecting us to meet you someplace. It isn’t because we don’t want to travel, but because all of his medication is here, and Von is safest when he can be managed from the safety of his own home. I’m certain most mothers of children with PHP would echo that same sentiment. Thank you again for taking the time to read this today.