It could have been so much worse...

I see stories like this, and I feel grateful to have been diagnosed as "early" as I was (although it felt like a very long time to me).  Many people live with Cushing's for years, and receive much more damage to their bodies.

http://www.today.com/health/after-massive-weight-gain-cushings-disease-diagnosis-saves-mans-life-2D80527683

ACTH Stimulation Test

This morning I had an ACTH Stimulation test conducted at an infusion center.  I am trying to focus on feeling grateful these days for the things that are going well and improving.  So today was an opportunity for that.  I am trying to feel grateful that I was simply at the infusion center to have a test conducted.  It was a room with two nurses and probably 12-15 lounge-type chairs for patients.  People were there getting infusions for all sorts of things, many of them seemed very familiar with the nurses and so were probably there a lot.  I think one of the women had cancer, as she had on a hat.  Another woman was incredibly thin.

It was depressing to be there, to feel like a "patient" - a feeling I am trying so hard to fight - I am trying to just feel normal.  It's a hard emotional balance between giving myself a break and allowing myself to be sick, and trying to be optimistic and celebrate the successes and the indications that I am geting better.

So today, instead of being depressed, I need to focus on feeling grateful that my experience in an infusion center was only for a test, that my disease is curable, and I just need to be patient and give it time.

Learnings

First, thank you friends for your kind and encouraging comments.  They mean a lot to me - more than you can imagine.  I'm physically and emotionally different than I was a few months ago, so it means a lot to feel the love coming in.  Thank you.

My last post was the first page of my powerpoint presentation as I shared my story about my journey through cushings.  The following is the traditional advice or "what I learned" that makes it a valid health moment to share with my colleagues.  Of course these are just the main points, the details are in the talking points.

Managing Your Health
Tips for navigating difficult to diagnose medical conditions

•Find a good internist…now

–Internist vs. family practitioner

–Referrals to specialists will be like-minded

•Trust yourself / knowledge of your body

–Get second / third opinions

•Research doctors

–Google

–Rate MDs*

–Health Grades

–Vitals.com

•Schedule new patient appointments with 2-3 doctors/specialists simultaneously

–Ask to be placed on cancellation lists

–Follow-up with appointment schedulers regarding cancellations

•Get copies of your medical records, particularly labs

–Follow-up regarding lab results

–Recognize that labs can be conducted in various levels of detail

•As your condition progresses, repeat labs

–Look for trends

•Don’t give up!

The Changes

At my work, we regularly have to present "safety" moments - each meeting is supposed to start with a moment to share a safety, health or security learning.  I was assigned a safety moment to kick off a team meeting in December, and decided it would be a good opportunity to share my learning from "navigating a hard to diagnose medical condition" - and to tell the team about my disease, and the fact that I would soon be out of the office for several weeks for surgery.

It is therapeutic for me to share my story, as it helps me unravel my misplaced guilt and mistaken assumption that I am a hypochondriac and I am hopeful it will help others who also get written off as "anxious" by their doctors.  As I share my story, this happens all too often - particularly for women.  So I offered to share my story and learning as the topic for the Working Mother's Group, which holds a brown bag lunch every month to discuss different topics.  I put together a two-page PowerPoint presentation, which I'll try to cut and paste below.

 My Story: Diagnosing Cushing's Disease

"Normal", June 2010

I put this picture up to show what I used to look like.  I chose this one as it's when I'm fairly newly postpartum.  I think Katie is about 2 months old here.  I attributed a lot of my difficulty with weight to just being older and not being able to lose the babyfat from my pregnancy with Katie - but when I look back at pictures after she was born, I can see that's not true.  I gained weight and started getting the "moonface" months AFTER she was born.

October 2011

Weight gain, first anxiety symptoms
Consult with psychologist

January 2014

First physical symptoms (cardiac)
Consult with family doctor

August 2014

First onset of continuous physical symptoms
Consult with family doctor, cardiologist, psychiatrist

October 2014

Begin seeking specialists on my own

Diagnosed mid-November

January 2015
One day prior to surgery

January 2016

Average 12-18 months
for full recovery

Hormonal Gal

Wow, it's hard to believe that it's been over a year since we've posted on this blog.  Life has sure thrown us through the fire medically (and thus emotionally) over the last couple of years.  But,finally, I have hope that things will get better - partially because they are all.  With a lot of help from a lot of people.
I had the title of "Hormonal Gal" or "Adrenaline Rush" (although the latter would have been medically false) for an entire blog in my head for a while now.  But realistically, where is a mother of three who still has 2-3 medical appointments per week going to find the time to start a new blog?  I wanted to start at the beginning of my Cushings journey - which I realize now would have been several years ago.  I wanted to document my physical changes, the psychological impact, what I've learned, how I think I have changed, and how I still want to change.  But it's been too overwhelming on where to start.  So instead, this is going to be a hodge podge of what I feel like sharing, when I have the time to share it.

I am almost 12 weeks post-op - the time which I am announced "medically clear" to resume all normal activities - ie. I don't have to worry about breaking the patch in the membrane that separates my brain from my sinuses.  Which is really encouraging for me, as I really need to begin exercising again to feel good.  I still feel weak and out of shape and a bit depressed that my body is not changing back as quickly as I would like for it to.  But over the past week or so, I have begun to feel physically better, and more importantly, I feel like I am coming out of a rather dark place (aka depression) that seemed to overtake me a few weeks after the operation.

I had on-and-off insomnia for a few years prior to my surgery (yes, another side affect of Cushings), and last night and again tonight I find myself wide awake at 3 AM.  Mark says this is encouraging, as hopefully it means my steroid dose is becoming too high, which would mean my pituitary and/or adrenal glands are beginning to function again, so I have a total sum of too much cortisol.  That would be nice.  I have a test on Wednesday to test their function, and I am hoping, hoping, hoping that we get good results so that we can begin tapering or even eliminating the hydrocortisone.  But my negative self worries that the Cushings is just returning.  Unlikely at this stage, but still something that is a possibility, which would require another surgery...

My story with Cushing's involved a lot of people (myself included) believing that I had cumulative stress from managing Adam's heart condition, Mark's detached retina, and just living the day-to-day life of a working mother of three.  But when my symptoms continued to get worse, I fortunately had two advocates who encouraged me to continue to seek a cause for my symptoms (thank you Christina and Dad!), when my family practitioner rather coldly told me over the phone that my diagnosis was complete: hypertension & anxiety.  Our conversation took place in mid-October, and I had not spoken with him since.  I felt the need to provide him with some feedback, partially for self-vindication (I wanted him to know that I was NOT a crazy hypertensive lady that he believed me to be), but also partially so that maybe he will pause just a little before he writes another patient off who experiences a hard-to-diagnose condition.  So yesterday I wrote him the following letter, which I delivered to him through the patient portal.  My mom thinks that he will not respond due to legal concerns.  But at least he'll know:

Dr. Giglio,

It has been several months since I last spoke with you, and perhaps you already know about my condition from Dr. Berman, since I had to receive cardiac clearance from him prior to surgery in January.

I believe that you have the best of intentions as a doctor and want to treat your patients successfully, and so for that reason I am writing you this note.

I began seeing you regularly last August (2014) after experiencing episodes of orthostatic hypotension.  These symptoms quickly escalated into an overall feeling of being unbalanced and “foggy,” and soon I was also experience very intense anxiety.  I had seen you in January after experiencing episodes of chest tightening, which we both thought were potentially panic attacks. So, reasonably, we thought I might just be suffering from anxiety.  I began seeing a psychiatrist, but other symptoms continued to progress.  I began also experiencing periods of heart palpitations, even when I was relaxed.  You agreed to a 24-hour holter monitor test, and when it presented some unusual results, you referred me to Dr. Berman.  At my appointment with Dr. Berman, he observed that my blood pressure was high, so we agreed to monitor it for a month.  During the course of that month, my blood pressure rose to dangerously high levels, so I weaned the baby and began blood pressure medications.  At my prompting, you also agreed to run another round of labs to find out why my blood pressure was so high.  My potassium levels came back low, and so Dr. Berman ran a lab to test for Hyperaldosteronism.  When this test came back negative (around mid-October), you called me to inform me of the results and told me that my heart was fine, I no longer needed to see Dr. Berman and added a beta blocker to my medications to help address my shaky hands.  I told you that something was just not right --- it is not “normal” to go from a healthy 36 year old to someone with exceedingly high blood pressure over the course of a month, and that I would like to see an endocrinologist.  I asked for a recommendation, and you told me that I did not need to see an endocrinologist. You instructed me to take the blood pressure medication, continue to see my psychiatrist and check back with you in three months.

I knew something was just not right, though, so I researched doctor reviews online and had an appointment with an endocrinologist by late October.  He ran lab work which came back abnormal, had me do a 24-hour urine analysis test (also abnormal), dexamethasone suppression test, and finally ordered an MRI of my pituitary gland.  He diagnosed me with Cushing’s Disease by mid-November.  I had the tumor removed from my pituitary gland at the beginning of January.  I recovered well from surgery, and am now slowly recovering from the damage that Cushing’s has done to my body.  Full recovery is expected, but it can take many months.

After our last call in October, I felt that you gave up on helping me figure out the root cause of my symptoms and were satisfied with treating the symptoms themselves.  It was stressful and lonely needing to coordinate my care on my own without the guidance of a family practitioner, but I learned to be assertive and trust myself as the true expert of my own body.

I hope in the future you will listen more closely to your patients, and trust that they are the experts in knowing when something is “off” with their bodies.  After I was diagnosed with Cushings, I learned a lot more about the symptoms, many of which I clearly presented.

I wish you and your staff the best.

Sincerely, Katherine Griffith

Adam's Heart - Part 9

The 15^th floor of Texas Children’s Hospital is the non-ICU cardiac floor. Almost all the patients there have had surgery, done significant recovery in the CVICU, and this will be their final stop before being sent home. The tenor there was so different from the CVICU – spacious, far less nursing care, and really quiet. Everyone has a private room, and parents, rather than nurses, are the primary caretakers of the patients. In the ICU, parents were encouraged to be there as much as they liked or could, but most would leave for portions of the day – meals, taking care of other kids, and of course sleeping. In the ICU, there was constant noise from the staff chatting, alarms going off, equipment moving around, all kinds of noise. There were some private rooms in the ICU, but we were in a large room that accommodated several babies – it was hard to find a comfortable chair and breastfeeding was a big deal. All that changed when we got to our room on 15. We had a fold out bed right next to Adam’s crib. The quiet was amazing – pleasant, but lonely at times. It had been fun to chat with other parents, see how their babies were doing, and make small talk with the nurses. The only interaction with other patients or parents was limited to rare meetings  while coming or going. We also had to adjust to taking care of our own baby. We had gotten used to the nurses doubling as babysitters while Katherine and I ate meals together, spent time with Ben and Katie, and especially when sleeping at night. Overall, it was much closer to regular life with a newborn baby.

One of the main reasons patients spend time on this floor is so that parents can get some training in how to care for them. Post-surgery babies need to be handled a little differently to protect their sternums. We had to get lessons in bathing, dressing, CPR, and car seat use. It turns out the car seat and CPR stuff doesn’t change much – they just really want parents to be educated in those regards. It was really great to be able to hold Adam and nurse him whenever we wanted. At this point, he had only a pulse oximeter attached to him, and even that was removed pretty soon after we moved to the 15^th floor. The nurse would come in to check his vitals every four hours – a long way from the constant monitoring and alarms of the CVICU.

 

We met each day with the cardiologist for the floor – Dr. Reddy. He was an Indian gentleman who sounded exactly like Rahaj from the Simpsons. He was a very encouraging, kind hearted doctor. Usually he and a fellow would make their rounds in the morning, and we’d talk for a little while each day about Adam. His recovery was basically complete and they just wanted to keep an eye on him for a few more days before sending us home. Dr. Reddy said one thing in particular that we really appreciated. We were concerned about going home and not having the supervision of the doctors. What would be some warning signs that there were problems? What symptoms should we look for? At what point does normal baby fussiness evolve into an area of concern? Our concern, especially Katherine’s, was due largely to the subtle but concerning symptom that brought us to the ER in the first place – the rapid, labored breathing. Katherine had been widely praised for her correct mother’s intuition but at this point felt the burden of “what if I miss it the next time?” Dr. Reddy said confidently there really should be no symptoms or problems like that. Speaking to Katherine, he said “You focus on being a mother, we will take care of his heart.” That was immensely reassuring as our departure from Texas Children’s drew nearer.

We spent a total of four days on the 15^th floor. They let us know on Wednesday that Adam would be discharged the next day. We still had to take our car seat class, and there was a myriad of documentation to be done before we could leave. The day seemed to go by slowly. We had a meeting with Amy Hemingway, the surgical nurse practitioner and one of our very favorite people that we had worked with. She was our liaison to the operating room during Adam’s surgery, and was the one who educated us about how to care for him during his recovery, both in the hospital and at home. She walked the line perfectly between being a realist about risks, dangers, and cautions, but also encouraging about excellent surgical results, and living at home with school age kids and a post-op baby. She was one of those medical professionals who, after speaking with her, you didn’t have to do any interpretation of what was said, because she had explained things so clearly and realistically. She told us Adam would be fully recovered six weeks from the date of his surgery. His heart was fine, but it would take that much time for his sternum to heal. In addition, his immune system would be a little weak – a byproduct of the heart-lung bypass machine. We would need to limit his exposure to crowded places and other kids. Realistically that meant not taking him to the grocery store or church and not having Ben and Katie’s friends and neighbors over to play – definitely a tough pill for the older kids to swallow.

And so on Thursday, August 1^st, at about 5pm, we left Texas Children’s to bring Adam home for the second time. He hated the car ride, crying for most of the long drive home. It was really pretty amazing to be home – no nurses or doctors, no monitoring equipment, no way to check his blood pressure. When we first left the CVICU, we thought about this time arriving home and it made us uneasy. But it turns out the process worked, and we felt vigilant but mostly relaxed. One of the biggest challenges during this whole experience was being away from Katie and Benjamin. We missed them a lot, and it was tough for them to have Mom and Dad gone most of the time, spending their nights at the hospital. Katie was vocal about missing us. Ben was less vocal, but had some behavior issues at school a little while later that we think are a result of this. It felt so good to be at home with those two sweet kids again. It was just fantastic to be together as a family, to do normal family type things like tucking kids in bed at night, reading together, and eating meals together. We really appreciated the simplest things.

Adam's Heart - Part 8

Thanks so much for reading our blog! It’s rewarding for us to share all these details with our friends and family, it helps us remember the details enough to write them down, and hopefully Adam will appreciate knowing his story someday.  

The day after surgery was the start of Adam’s journey, God willing, toward being a normal baby. While that first day seemed long and without progress, it really was an amazingly brief journey considering the complexity and significance of his surgery. The doctors and nurses were well versed in how this process goes, and we were continually encouraged by their calm nature and lack of worry. The first day, they just wanted Adam to rest, keep his pain under control, and maintain stability with his vitals, especially blood pressure. He was getting hefty doses of Fentanyl, a strong pain killer, that pretty much kept him asleep. He opened his eyes a few times that first day, but that was it. We weren’t able to hold him anymore. There was so much he was connected to, and some of it was delicate – namely the arterial and aortic lines. We tried to spend time next to him, chatting with each other or the nurses, hoping maybe he could hear our voices and find them comforting.  

Now that we’re up during the night feeding Adam or walking laps around the house to get him to sleep, we look back fondly on those nights In the Ronald McDonald House. It was really kind of nice to have nurses looking after him so we could get a full night’s sleep. Mostly full night sleep – Katherine was still getting up every three hours to pump breast milk. I was getting up early to be back with him in time for the 6:30am rounds of the surgery team. The surgeons are the “big dogs” in the CVICU, and we really wanted to hear what they had to say about how he was doing. Over the first five days or so, he was generally doing well, but his blood pressure was often a concern. They tried several different medications, and each of them responded for a time, but then something different would be required, presumably because his condition was changing rapidly. At one point, I found it humorous that he responded real well to Inderal, the drug used by many performers, including lots of orchestra musicians, for stage anxiety.  

Day two was exciting because he started getting disconnected from a little more equipment. First were the chest tubes which drained fluid from his chest cavity. We had one night nurse in particular, for three nights in a row, who was very proactive about removing the equipment. There was a different dynamic at night – the daytime staff was  more cautious, always checking with layers of superiors before wanting to do anything. At night, the nurse would feel it was safe to remove tube such-and-such or step down the ventilator, would check with the attending cardiologist, and then just do it. It was fun to come in the mornings and see what progress had been made. Slowly, it all came off, and the pain medicines were reduced.

 

We had been warned that the recovery from surgery didn’t often go completely smoothly. Most babies have some hiccups along the way. Adam’s came on the second night after his surgery. When I came for the morning rounds early on Wednesday morning, the nurse told me he had had some “seizure activity” during the night – a rhythmic firing of his leg muscles that was definitely not normal baby twitching. It was concerning because it looked like seizures. But it didn’t have some of the hallmarks of typical seizures – a spike in heart rate and blood pressure. He had seemed otherwise calm through these two or three episodes lasting several seconds each. The doctor ordered an EEG – a procedure where they place electrodes all over the head and measure electrical activity in the brain. The procedure took place later that day. That afternoon, I went home to spend some time with Katie and Ben, then stopped at the grocery store to get some fruit and snacks for us at the hospital. While I was there, I got a text message from Katherine saying to please come back immediately – the neurology doctors wanted to consult with us about Adam possibly having epilepsy! Wow, that made for a very fast, stressful drive back to the med center. Neither Katherine or I knew much about epilepsy, including that it has a spectrum of severity. We imagined a child prone to grand mal seizures. Would he be able to drive? Play sports? This was the first time since getting his original diagnosis that there was a real fear of Adam having a long term, life-altering limitation, and it was very scary.  I got there quickly, but it turned out the neuroligists were not in a hurry to meet with us, and things were probably not as dire as we had imagined. The cardiology docs told us they had found “an area of focus.” It could be something, it could be nothing, but it was not uncommon. We met with neurology the next day, and they reiterated the information the cardiologists had told us. An area of focus is a spot of unorganized electrical activity in the brain. It can be a seizure, but it can also just be random. They said if they were to give everyone in the room an EEG, probably many of them would have these areas of focus. They weren’t overly concerned, but they did want to do a brain MRI. In the week that followed, there would be some hospital drama surrounding this MRI. The neurologists wanted to have it done, and the cardiologists did not. Initially, we were told that Adam’s surgeon, Dr, Fraser, was strongly opposed to it. Adam’s blood pressure was still high, and he was still in a pretty fragile post-operative state. Regardless of what the MRI told them, no immediate action would be taken anyway. As Dr. Fraser is one of the bigwigs at the hospital, his opinion prevailed. And as the days progressed without any other neurological concerns, the neurology folks acquiesced, and we were told to schedule a consultation two to three months after we were discharged. Whew. 

From there, the pace of recovery really picked up. On day three, the dialysis tube came out. Day four, the brain oxygen patch, urine catheter, and aortic line.  I think day four also saw the removal of the ventilator. This was significant in that if there was a problem and he had to be reintubated, that would be a quick and unpleasant procedure. The nurses (especially at night), had been proactive in stepping down the frequency and pressure of the ventilator. The idea is that by the time it gets shut off, the patient is doing almost all the breathing on their own. Adam’s breathing tube was removed uneventfully. He was given a nasal cannula for some extra oxygen, but that was removed a day later. 

Day five was awesome, as this was the day the arterial line was removed and we were able to hold Adam again. As I look back through the pictures we took, the one below is my favorite – Katherine holding him for the first time since his surgery. He got a lot of cuddling time that day. Day six saw plans to remove his feeding tube. Like the ventilator, there were some steps along the way. First, they delivered  a small amount of breast milk into his belly via the feeding tube. The next feeding, a little more was added, and this continued throughout the morning. He responded well, and that afternoon he got his first bottle of breast milk. Amazingly, we never had to give him any formula through his entire hospital stay! Just breast milk and IV nutrition. That night, the cardiologist gave the okay to try some breastfeeding. Katherine was extremely excited, and extremely anxious. We are big believers in the importance of breast milk for a growing baby, and that bond between mother and baby is a BIG deal. He did have six days of breastfeeding experience, and had developed good skills before he was admitted to the hospital. But would he remember how? All the medical staff was real confident but Katherine was nervous. 

 

 

 

The staff insisted that all these curtains and barriers be set up around this “scene.” Apparently it’s a privacy law or something. We didn’t really understand – you’re allowed to breastfeed anywhere in public, but apparently not in a hospital. Regardless, we were excited to give it a shot. I helped get Adam and his remaining tubes in place, and sure enough, he latched right on. Katherine was tremendously excited and relieved. From that point, Katherine would head up to the CVICU during the night rather than the hospital’s milk bank where she had been pumping. The nurse would just call our Ronald McDonald room when he started to get fussy from hunger, and she’d head up. The next day, the feeding tube was removed, and all that was left was some standard monitoring equipment (blood pressure, pulse oxymeter, etc) and his PICC line. 

 

Over the weekend, the doctors told us they were expecting to send Adam down to the cardiac floor of the hospital on Monday. The weekend went by slowly, but eventually Monday came and we took the next big step toward having a “normal” baby – out of the CVICU.