Children's Check-Up

Thumbs up for a good check-up!

Ella had her four month check-up at Boston Children's hospital last Friday. This visit marks 18 months since we switched to Dr. Holm. We have made a lot of adjustments on medications and doses and we are finally seeing the benefits of those changes. Dr. Holm has been very deliberate and diligent with Ella's medication. When we first met with her in January 2012, she explained to us how small changes can make a big difference. We have seen the results of rapid medication changes to her health. It is not good. At times it seems like we have been on a slow pace and little change, TIME to adjust to the proper dose has proven to be the most effective approach.

We were excited to learn that for the first time, Ella's levels are perfectly normal. Her T4 and TSH (both thyroid hormone) as well as her growth factor all register right in the middle of the normal range. She gained 4lbs and grew more than an inch since January. So as we approach her 4th birthday she is 34 lbs and 36 inches and finally wearing 4T clothes. This would certainly explain her recent need for a whole new wardrobe!

Ella is still on a pretty high dose of growth hormone, but the plan is to let her continue to grow into this dose. She is responding very well and catching up. She jumped from the 11% on the growth charts to the 18%....all of this since January! When Ella was first diagnosed with hypopituitarism, she was not on the growth chart. That initial diagnosis also came with disheartening news at the time that there was "nothing we could do" to help her grow and that growth hormone "probably wouldn't help" (quotes from our endocrinologist at the time). It is gratifying to know that sticking with my intuition and remaining dedicated to finding help for Ella has paid off.

We return to Dr. Holm in September. We will have her annual bone age x-ray and a complete lab work up. If all goes well, we may be able to bump her visits to every 6 months!

When It Rains, It Pours....

Ella and Auntie Jess...one of my favorite pictures :-)

January shaped up to be a rough month in our house. It was made up of a lot of sadness and sickness. We lost a dear family member, Jason's sister Jessica. This has been a difficult time for all of us, as she was not only a beloved sister, but a wonderful friend, a fantastic auntie, and someone who always knew how to make you laugh. I will write a blog for Jessica soon, but right now the emotions are too raw. It is so important for me though to let Ella know how much Jessica loved her and how special she was.

As we were dealing with our loss, I traveled to Kelowna, British Columbia (Jason's hometown) to be with Jason and his family for the services. Jason arrived a week earlier. It is a long flight and last minute tickets meant we were flying what seemed like around the world to get there. Ella had been fighting a nasty cold since Christmas, so she didn't make the trip. We figured it would have been just too much for her.



A hug after a long ordeal meant so much
to Ella and Jason.

 It's a good thing she did not travel because a day after I arrived in Kelowna, Ella arrived at the hospital to be treated for pneumonia. She was admitted to Winchester hospital (near my mom's house where she was staying) and later transferred to Boston Children's hospital. She spent nearly a week in the hospital on antibiotics to fight the infection. It was so scary and sad to be far away and have a child in the hospital. Jason and I are very thankful to our extended family who dropped EVERYTHING to help. My mom, sister, aunts, and friends all stepped in to make sure our little Pnut was getting the best care possible. While modern day travel dealt us delay after delay and even a few cancelled flights trying to get back to Boston, modern day technology kept us somewhat sane. We were able to facetime with Ella on my iphone a few times...we even got to speak to the nurse at Children's!

I am happy to say that Ella is finally much better. She is out of the hospital and getting back to herself! She is back to school and will get back to all her regular activities this week.

Ironically, the stay at Children's coincided with our long scheduled visit with the genetic endocrinologist there. We were discharged just in time to make it to our appointment and because Ella was already a patient, Dr. Holm was pretty familiar with Ella and her case. Jason and I were very impressed with her. She had lots of good information for us and seems to be very comfortable treating Ella's hypopituitarism. She drew some blood and is running extensive lab work to determine exactly the right dose and combination of medication for Ella. Some of her levels have been out of whack lately because of inconsistent blood work. We left her office feeling confident that switching to her care would be the best situation for Ella. We have another visit in four months, but we actually should know more about the latest round of labs this week. Score one for mom intuition (and a little luck getting an appointment)!

Tall and Skinny

It's been a busy week getting back into our routine after the holiday's. We had a check-up with Endocrinology on top of everything yesterday. I have been anxiously anticipating this visit for a few weeks now, but not because I was so curious about Ella's gains. I have been going back and forth with the doctor since before Christmas about changes to Ella's meds and some other issues I feel they have overlooked. I need her records to take to Children's Hospital in a few weeks and it seems all of a sudden the doctors here in Albany want to be extra vigilant. I appreciate the efforts, however this is the way I feel we should have been treated from the beginning. I am constantly calling for lab results and expressing my concerns about every little issue. I understand they see several patients, but I also think they are not doing their job well when I can't even get a phone call to let me know that my daughter's blood work shows her levels are low and her medicine doses need to be adjusted significantly!

Okay rant over (for now) -- As for yesterday's appointment, it was overall positive. I have seen some improvements in Ella since we increased her Synthroid and growth hormone doses. She has more energy, her skin is getting better, and she is coping with things more appropriately. She now stands at 32.5 inches tall....a jump of 2 inches in just three months! We are all very happy to see such gains. I knew she grew. She certainly looks much taller and she can get into more trouble (like pulling plates off the counter and opening doors on her own!) Her weight is another issue. We affectionately refer to her as our little rack of ribs these days. She weighs 23 lbs...a gain of only 3 ounces in three months. This too was an expected outcome. She is a skinny kid. It's not for lack of trying. She eats pretty well and we are constantly feeding her snacks throughout the day. Part of the issue may be from the fact that her meds were off for a bit. The other issue is the jump in height. She is growing so quickly that it is hard to pack on the pounds (I wish I had that problem these days, haha!) Dr. Gleason was not concerned. Ella is finally sneaking on to the growth charts in the 1% range these days.

We head to Boston in a few weeks for a consultation with Dr. Holm and Dr. Hirschorn. Hopefully we'll get some more answers. In the meantime, ice cream sundaes for everyone to celebrate progress (and maybe fatten Pnut up!)

No Wonder!

To say that I am frustrated with Ella's endocrinologist is an UNDERSTATEMENT! I have been questioning whether her thyroid hormone levels are correct for several months now (actually since June!) I have taken her for at least three blood draws to check and every time, the doctor seemingly brushed me off and said her levels were fine. I called again last week for another blood draw because Ella just seems to be a little off. She is sleeping lots, her skin is super dry (more than just winter dry), and she had a few other signs that I noticed a year ago before she was even diagnosed. At first I was told the results of the latest draw showed her thyroid levels to be borderline...and that they wanted to re-test. 24 hours later I got another phone call from the nurse who was backtracking big time. She said after "thorough review", the doctor decided to increase Ella's synthroid, without doing another blood draw. The history showed a decline in levels and she needs to go to one and a half pills a day...WHAT!! No wonder she hasn't seemed like herself. In one day she went from being borderline low to a significant increase in meds. I am thankful that I was at least persistent in having her tested multiple times. I hate to put her through so many needle sticks, but clearly it is best for her health at this point.

This was the final straw, solidifying my reasoning for heading to Boston for a second opinion. We have another check-up in Albany January 3rd. I am not expecting much from it. We head to Boston Children's hospital January 23rd where I am confident we will get more answers. In the meantime, I am optimistic that the new dose of synthroid will boost Ella's energy. She will hopefully be feeling good just in time for Santa's arrival.

And The Results Are.....

Six months....5.7 lbs....4 inches....these are Ella's stats from her Endocrinology visit today!

Weighing in October 4, 2011...22.7 lbs

Ella now tips the scales at 22.7 lbs and stands 30.5 inches tall. AMAZING! Today's visit went very well. Ella was happy to show off how much she has grown and developed. Since her last check up in July, Ella has gained 1.7 lbs and grown 1.5 inches. Her growth is still going very well and the growth hormone therapy is working. Dr. Gleason admitted she would have liked to have seen a little more of a weight gain with this visit, but attributes this to all of the activity and walking, etc that Ella is doing now. She's not too concerned about our little skinny minnie. Dr. Gleason did however change her dose a little. She bumped up the shots to keep up with Ella's growth. We also had routine blood work done to check her other hormone levels. Her thyroid medication may actually have to be adjusted as well. Of course, Ella was a trooper in the blood lab. We should have the results back in about a week.

30.5 inches!

Thank you to everyone who wished us well with today's visit. I can't say enough how much the incredible love and support from family and friends means to all of us!

Good, Good, Good!

Good...it's the word of the week at our house! Ella has lots of new words, like "good job" and "good girl". She loves to hear you tell her how good she is doing. She will even remind us nicely if we forget once or twice to tell her.

We also got some very good news from the endocrinologist. The results of the Cortisol (nightmare) test that we had done a few weeks ago came back perfect. Ella's numbers were normal. Dr. Ridick and Dr. Gleason were very happy to see this, but not happier than Jason and me. After a summer of lots of changes/medicines/procedures for Ella, it was wonderful to hear that her cortisol is fine. She will not have to repeat the test any time soon. The plan moving forward now will be to monitor Ella's cortisol levels every three months and this can be done with the blood work that she has done routinely. Yay!

Ella enjoying breakfast track side at Saratoga.

We celebrated the good news with a day at the Saratoga race track. My parents came to visit for the weekend and we decided to spend the day at the track. We have been going to the track just about every summer that Jason and I have lived in New York. We even took Ella when she was just 6 weeks old (looking back, we were nuts!) It was a beautiful morning to sit right at the edge of the track and enjoy breakfast and watching the horses warm up. We walked off breakfast with a trip to downtown Saratoga and a ride on some pretty pony's in the carousel. 

Taking a spin on the carousel

We have all been so busy, and then on vacation, so this was the first time Nan and Pa had the chance to see the progress Ella has made. She was a show-off...strutting her stuff. They were amazed with her new strength and especially her confidence. Our little pnut even brought a little good luck with her this year...both my parents won at the track!

Ella strolling around Saratoga with Pa.

I have so many good feelings...proud to see Ella gaining so much independence, happy to spend time with my parents, and love that they enjoy these special moments with their grand daughter. My mom has always told me to think positive. I guess this weekend is just living proof that once again mom is always right :-)



The List

We have been on a roll, with one thing after another for several months now. We started synthroid, growth hormone, ear tubes, endless doctors visits...it's exhausting! Everything we are doing for Pnut is working and all the stress leading up to each test, appointment or procedure pays off ten-fold. However, the other day Jason said to me he feels like we are constantly checking things off "the list" and our list seems never ending. 

Ella checking things off her grocery list!

Once the ear tube surgery was scheduled, the hope and expectation (for me anyway) was that things were finally slowing down. Then we got the call from our endocrinologist just before her surgery. The blood work that Ella had done in early July shows that her cortisol levels are low. Cortisol is commonly known as "the fight or flight" hormone. It helps our bodies fight stress....everything from a common cold to a traumatic injury. Ella's levels came back at 2.5 (normal range is above 4). For this reason, she had to have a hydrocortisone shot prior to surgery. This ensures that her body has enough cortisol. It was a crucial precaution because a person can not live without enough cortisol. Ella's apparent deficiency is due to the hypopituitarism.

A synthetic form of cortisol is available. It comes in a pill form and would go along with the hormone replacement therapy that Ella is already on. The issue with cortisol replacement is that once you start this therapy, it can not be stopped. As soon as you give the body the synthetic version, the body stops producing the hormone all together. We have known since Ella's initial diagnosis that we would constantly be monitoring all of her hormone levels and cortisol has always been on the radar for our doctors, however even Dr. Gleason (pediatric endocrinologist) felt we wouldn't be going down this road just yet. It's not a bad thing, just one of the unknowns we will continuously battle.

On Tuesday morning, we will head to Albany Med (again) for an ACTH stimulation test. It takes about two hours and requires several blood draws. This will help us determine if Ella's cortisol is low or if the first results were a circumstance of when the blood was drawn. Cortisol levels fluctuate throughout the day. We have checked cortisol several times when Ella has had a nasty virus, etc and everything has been fine. Jason and I are hopeful that things will be fine this time as well. The longer we can hold off on cortisol replacement, the better.

We are confident that the doctors will help us make the best decision possible for Ella. We'll check another thing off the list....and stay optimistic that the list takes a break for a while! I hope that Ella has little memory of any of these tests and doctors visits. She is such a happy kid and takes everything so well. We are so lucky and learn new strength from her every day!

Who needs toys when you can have fun with pot holders!

Catching Up!

"Her progress and growth is remarkable."....that's what Ella's endocrinologist said to us today and it was music to our ears :-)


7/5/11 - Ella on the scale.
9.5 kg translates to 21 lbs!

After just three months on growth hormone Ella has gained 4 lbs and grown 2.4 inches!!! She now tips the scales at 21lbs and is 29 inches tall. Jason and I couldn't be more excited. We had a great check up with the endocrinologist. She feels her "catch-up" growth is right on target and at a pace that Ella is handling very well.


This is a shot of the device they use to measure Ella's height.
It's important to get an accurate measurement.



 All this growing has had a tremendous impact. Ella is a lot more active. In 3 months she has started walking, talking and communicating more, she is more interested in her toys, she sleeps better, and she actually has an appetite. She wouldn't sit still during her exam...and we all had a good laugh about what a difference three months makes.

This is just the beginning. The doctor has told us that it will take about 6 months to see the full effects. We will have another check-up in October. We will also have the results of her blood work in about a week. The doctor will then be able to determine if her dosage stays the same. It's a careful balance, but so far the hormone replacement is working. Big cheers for Pnut!