June 22nd, 2011 by

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 to 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 maintained 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 stop 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.

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One Response to “Endocrine Visit – June 20”

  1. avatar Jess says:

    I just wanted to let you know that even though I don’t comment on many posts, I have been following miss Haven’s story. I have learned so much from your blog, not just about an illness I had never heard of before but also what it truly means to persevere- especially what it means to persevere as a mother.Our son has special medical needs, nothing like ROHHAD, he has a genetic syndrome called Digeorge syndrome that causes various medical complications. They have been relatively minor, but still hard for our family to go through. Your blog and Haven’s story has been such a huge help and inspiration. I wish her and your family all the best

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