Endocrine Visit - June 20

On June 20th we took Haven in to meet with her Endocrine doctor - he was the doctor that saved her life by finding her cancer and diagnosing her with ROHHAD Syndrome.  My main concerns have been Haven's weight and height ratio and the fact that I knew she hadn't grown much over the past year.  I had a few emails back and forth with him over the six months we hadn't seen him.


Haven has maintain her 80 lbs since January 2009.  Her Hi CY treatment for ROHHAD stopped her weight gain and her stealing of food.  Immediately following Hi CY Haven jumped up about 3 inches in height - this was a make up from the year on steroids (which stops growth).  But since that initial jump Haven hasn't done much.

So our main discussion with him was regarding her growth and if she is deficient.  Growth Hormone Deficiency - is a disorder that involves the pituitary gland, which produces growth hormone and other hormones.  Growth hormone is necessary for normal growth and development in children.  Haven's last IGF-1 test was done in May while in Oncology.  This came back low.

Haven has grown about 1 1/2 inches in the past 18 months.

Growth hormone deficiency does more than just keep them from growing - it causes serious problems with the heart strength, lung capacity, bone density, immune system and more.

Her doctor ordered a bone age x-ray to be done and we are now waiting for the results of that to see where she is falling.

We asked about the growth hormone treatment that the ROHHAD children end up on due to the damage done this area.  Obviously there is not much research done since ROHHAD is extremely rare and not much has been written about it.  So we got some of the main concerns for Haven - which are what children on growth hormone are informed of.  These are a few but not all - and again there is no telling what it could do with a ROHHAD child and especially one that also had cancer:

1. Higher risk of cancer as an adult --- but with Haven already having cancer there is no research as to how this could affect a child like her 
2. Higher risk of adult diabetes
3. Shorter life span
4. Good possibility of required surgery due to Slipped Capital Femoral Epiphysis 
5. Possibility of thyroid issues requiring medication
6. Severe migraines

There is also an age limit to start this.  Haven MUST start it within the next 6 months or so or we could be too late.  You want to get all the time in before puberty hits or it becomes ineffective.  Considering Haven has an extremely high Prolactin level we don't see puberty hitting anytime soon. 

Growth hormone treatment is a five (5) year treatment and requires Haven to get a daily shot for the next five years.  She will undergo constant testing and monitoring for her bones and anything that could go wrong (as listed above). 

Before treatment she will go through a test requiring insulin to be given through IV to stimulate growth hormone secretion - this is measured over a 12 hour period.  Haven's doctor says he already knows Haven will fail this test but it is a required step that he must have data on before beginning her treatment.

As Haven's dad and I are still not in complete agreement about this treatment and her doctor doesn't like to just give this treatment (as it is abused by parents that just want their kids taller) we have agreed to the doctors desire to wait until December to start treatment.  UNLESS her bone age x-ray and insulin test tell us she needs to do it now.  Haven's doctor said for Haven to be on track she should grow 2 inches by December BUT considering she has only done 1 1/2 inches in 18 months he doesn't see this happening.

So for now we will continue to watch Haven's growth and will proceed with any testing needed. In December if she has not grown 2 inches she will begin growth hormone treatment for the next five years.  Her dad and I will be trained on how to give her the shots and dosage.  The next six months are very important for Haven and will require us to once again make a decision that leads her in to the unknown as an adult.

Endocrine

We met with Endocrine on Monday. I was concerned about this meeting - this would've been one to cause arguments. But instead we received happy news!!!!

Haven is growing - however slow - but she is growing. She is in the 5th percentile right now and showing signs of continued growth. The doctor was pretty happy with that. I was concerned we would have to start giving her growth hormone shots as this is what was said in May prior to Hi CY. But since then she has grown almost an inch!

He also stated with her growing and growth hormone levels at least not below normal she did not have to do the cortisol testing right now. He would put a request in to Oncology to have both growth hormones checked in December during labs.

So we are very happy and excited we do not have to give her a shot 6 days a week! :)

Hospital visits

Well I have been concerned about Haven going to the hospitals since the outbreak of the Swine Flu. We have 10 possible cases here in MD but no one has confirmed them yet. With Haven living at Johns Hopkins I'm not a fan of her going in the hospital.

I kept telling her last night - Do NOT touch the elevator buttons and wear your mask!

She argues with me about both of these all the time. We really argue about the elevator buttons more than anything. I do not like her to touch them - everyone that's sick is going up and down the elevators all day long. I don't need her to touch them and rub her face and get sick. I don't like her touching the door handles or anything in the bathroom. I follow her around with hand sanitizer - which she gets pretty irritated at me. But I've had a child without an immune system make it an entire year without ever getting sick!

Kris and Haven got to Oncology but had to fill our paperwork regarding the flu and if they had any symptoms before coming in. I know Johns Hopkins is really keeping that hospital clean and sanitized - which is awesome because the hospital is so huge.

Haven currently is weighing in at 71 pounds and heigh of 41.5 inches.

I almost had a party last night - Haven's heart rate got down to 88 for about 5 minutes while she was sleeping. It ultimately went back up to 120 and stayed around there but we did go down for a bit. So that was cool. She also slept the entire night. Even the night before she slept from 6pm until 7am. That was a shocker.

We have Endocrine on Monday afternoon. I am looking forward to this appointment. I have lots of questions and we haven't seen him in a while. A few of the topics of discussion will be Cushing's Syndrome and growth hormones.

This weekend our plan is to finish our garden! We'll show photos when we finish! :)

Love to all
M