I'm new here - hello! Anyone else with Hemoglobin H?

Hi everyone!

Looks like I found the right place!

I'm nicknamed Zadkhi around the web and amongst my friends and I'm an early to mid-twenties female who has the type of alpha thalassemia called Hemoglobin H Disease.

I've known about my blood disorder ever since I could remember as I was diagnosed with it when I was 12 months old or so.

My pediatrician originally mistook my anemia for iron-deficiency anemia and when I didn't get better with iron therapy, he took me off the iron and sent me to the hospital for a thorough blood workup.  The results came back and that's how my family and my doctor found out that I have HgH Disease.

My typical Hg count is in the high 7 and I have tried brief transfusion therapy in an effort to boost my energy levels and to hopefully give me more energy to combat my chronic depression. Because my Hg levels only went to 9.6 with little noticeable results, the therapy was discontinued though I've been encouraged to seriously think this option over.

My thalassemia is not asymptomatic and my primary issues related to my thalassemia is chronic fatigue and an unhealthy penchant for getting sick at all the worst times.  A side effect from the fatigue, my doctors and I suspect, is my chronic depression that was always sort of floating around when I was younger that finally escalated into fullblown depression (possibly bipolar in nature, though without the manic symptoms) after I went through a really rough spot during my university schooling.

I oftentimes feel caught in between - having a chronic illness that isn't severe enough to warrant constant intensive medical attention, yet unable to 'catch up' and 'compare' to my healthier peers - and have discounted the validity of my symptoms time and time again because on the outside, I look rather healthy and I'm even nicely tanned, but inside... sometimes I feel like I'm nothing but a jumble of broken parts inside.

Hence, the reason I'm here now.  I'm experiencing things that... my older non-thalassemic sibling and peers haven't faced before and may never face - physical healthwise and otherwise - and it's been... difficult coping.

When I was a kid, everyone treated me like I was normal and that was how I wanted to be treated.  Invariably, though, when I got stressed out or sick, I would get REALLY sick and suddenly, I would become so weak and have to miss a lot of school and PE.

It wasn't so bad when I had my mother to take care of me since she always knew how to take care of me and took care of my school issues as well, but when I grew older and started facing 'adult' situations, concerns, and responsibilities, everything sort of fell apart.

It's tiring to have to explain why I -need- health insurance.

It's tiring to have to explain that I simply CAN'T STAND to run a mile.

... Or stand/be on my feet for longer than an hour without any breaks.

It's tiring to have to explain that I get very easily tired when doing physical activities of ANY kind.

It's also tiring and embarrassing to have to explain to my professors that I sometimes fall asleep in class because I am simply exhausted from all the running around I had to do earlier and mean no disrespect.  (Thankfully, all my profs have been understanding but it's still embarrassing!)

It's frightening as hell to be sick with mono and pneumonia and constantly feel like I'm about to pass out and be unable to see a doctor because I can't afford the damned health insurance because I got kicked out of school for being sick in the first place!

It's discouraging to talk to my significant other sometimes about building a family and going on adventures and whatnot because I'm not one of those people who can 1) just decide to have a kid and have a kid 2) just decide to pack up shop and leave for a camping trip to the middle of nowhere even though I'd love to do it.

It's discouraging to have my 'bad days' and have no outward symptoms of it besides the general feeling of malaise and fatigue and have people behaving as if it's all in my head or that I'm simply exagerating.

... And it goes on and on.

For a while, I thought these issues were all in my head indeed since I couldn't recall having such problems as a kid, but after talking with my hematologist and psychiatrist about this at length, it seems that the issues I'm dealing with are almost to be expected.

Is this true?

And this is why I'm here.

To learn more about others who live with thalassemia and how they live with it and what works for them and what doesn't.

I'm very interested in finding out if there's a link between chronic depression (or a tendency towards depression) and symptomatic thalassemia because I believe the constant fatigue might play a big role in controlling one's moods.

Right now, I'm struggling with severe chronic depression and am wondering if there are other avenues of treatment that I have not yet looked at from the thalassemic point of view.

If anyone has any advice or stories and experiences of their own to share, I'd love to hear.

In particular, does anyone else here have HgH Disease and has it affected your life?

Hello to everyone again and thanks in advance!



~ Z

Hi Zadkhi,

Welcome to the site.

I think that your depression and lethargic condition is related to low HB. Try to keep it above 10 rather let it float around 7.

Plus are you taking folic acid? If not then do it. It can do wonders and hopefully you will feel better with continued use.

Take care, Peace!

So perhaps the depression really is linked to the low Hg count then?

My hematologist advised me on that and told me that it isn't unusual, but...

I've never been on constant transfusion therapy before and to be very honest, I am quite scared to do so! ^^;;;

And yes!

I DO take folic acid; thank you for asking! ^^

But funny enough, I had one doctor tell me that folic acid isn't of any help and another doctor tell me that I should consistently take folic acid supplements - what gives?

I've had my vitamins and minerals levels checked during my last hematologist appointment and everything seems to be fine so far, though it's been a while since my last appointment.

Any type of anemia can be a cause of depression. I have become depressed when I was going through iron deficiency anemia and after talking to many thals for the past 4 years I would say that thals tend to have more problems with depression than the general public. I think there are a combination of physical, psychological and emotional factors involved. Having low blood and feeling tired all the time and the helpless feeling it leaves you with because there isn't much you can do about it, does get depressing. Feeling like you can't have a normal life...having kids or careers are prime examples...is one of the most common causes of depression and anxiety in thals, and unfortunately, much of this is instilled by others who tell thals they can't have normal lives. I have talked to many thals who have told me this and have also seen this alluded to in posts.

In your case, I would definitely get more opinions. I feel that you may need to consider the pros and cons of transfusions. Please read some of the posts Jean has written about being an intermedia and how much she wishes she had begun transfusion many years before she did. You can read her most recent discussion about her life as an intermedia at http://www.thalassemiapatientsandfriends.com/index.php?topic=520.msg3955#msg3955

Hemoglobin H disease is very much like beta intermedia as far as symptoms and some people need to transfuse and some don't. If you can get on insurance again, it will make transfusing much more feasible too, as exjade the new iron chelating drug has made iron chelation much easier to do and insurance in the US does cover it.

I do think that if your Hb is higher you will feel much better and that will also lead into more positive feelings about the rest of your life.

Folic acid helps build good red blood cells and is universally recommended for thal patients. It is the one thing that all experts on thal have agreed about for many years. When a thal specialist speaks at conferences about thal treatment, folic acid is always mentioned as essential. Every single medical reference will also tell you to take folic acid for thal. I wonder where the one doctor got his information.

Do you still have your spleen? Also, do you know if what you have is Hemoglobin H disease or Hemoglobin H-Constant Spring? You can read some more about both at

http://www.thalassemia.com/alpha_thal_3.html

Therapy for individuals who have Hgb H disease includes folate, avoidance of oxidant drugs and foods, genetic counseling education and frequent medical care....Children with Hemoglobin H-Constant Spring have a more severe course than children who have Hgb H. They have a more severe anemia, with a steady state hemoglobin ranging between 7 and 8 gm/dl. They more frequently have splenomegaly and severe anemia with febrile illnesses and viral infections, often requiring transfusion. If anemia is chronically severe and the child has splenomegaly, a splenectomy may be performed.

Thanks so much for your input, Andy, and hello! 

My doctor definitely has talked with me about considering more long term transfusion therapy options but without the current insurance, it's something I've been thinking about, but not too hard on since it isn't possible at this particular moment.

I'm sad to hear that other thal patients also deal with depression, but it makes me feel less alone in experiencing it.  Thank you for sharing your own experience with it, I really really appreciate it.

A question:

I've heard of Hemoglobin H being classified into Thalassemia Intermedia because of its intermediate form, but only recently have I realized that Thal Intermedia refers mostly to Beta Thal Intermedia and not the Alpha Thal intermediate forms.

I'm guessing that HgH Disease falls into its own category/classification even though it shares many similar symptoms and issues with Beta Thal Intermedia?

*is a bit confused about classifications*

 

--

In response to the second post:

When I was first diagnosed, I was told consistently by my pediatrician and the referring blood specialist that I needed to be on constant folic acid.

When I went to university and had to go in for a routine blood checkup, I clearly remember being told very emphatically that taking folic acid is a waste of money in my case.  For what reason, I have no idea, but the advice struck me at the time as very very odd.  I actually DID stop for a while until I started seeing a hematologist and then went back on it and I do believe that it's helped me over the years.

I do still have my spleen and my iron count is approximately 2.5 times that of a normal person's, but this was based on blood work and no biopsies or anything like that.

As to whether I have Constant Spring or regular HgH, I'm honestly unsure because when I last saw my doctor, he was concerned that I might have Constant Spring because apparently, my Hg levels are considered fairly low, even for a HgH patient.

Thank you very much for the literature!

Zadkhi,
     I think it is possible you have depression from having a low hemoglobin; seven is very low, and I think gets harder to live with as you get older. I think you would feel so much better at a higher hemoglobin. I hope you can somehow get back on insurance.
     There is also a possiblility that you have what they call major depression, which can afflict anyone and seems to be genetically predisposed. Maybe you have developed depression as a result of low hemoglobin combined with recent events in your life: no insurance, not in school, chronic illness, pressures from your etc. People with thal can also have other conditions that complicate diagnosis and treatment. I also have fibromyalgia and it is hard at times to know what symptoms are from that or from thal.
     I know what you mean about looking OK and feeling so bad. I used to get so sick of talking about my health at work, because people do not understand chronic illness and its effects, and yet you have to try to explain why you are sick all the time. It's hard for relatively healthy people to believe that anyone can be sick so much of the time and not be in a cast or wheelchair, etc.   
     Anyway, are you getting any support from family? Is there any way you could get on insurance again? What about disabilty insurance? Is your spleen enlarged? If you could get back on some kind of insurance, start regular transfusions, you could maybe have the energy to go back to school, if that is what you want to do.  Most universities have services to help those who are medically challenged, and you would fit that category. You may be able to get some educataional accomodations which would make it easier for you at school. I'm not sure if you want to go back to school or not. 
     It's good to talk to you. Wish I had more knowledge of HgH.   Jean

Jean,

Please pardon me if I ramble in different directions.  I've been so caught up in trying to write an Incomplete paper that my brain is in 'paper writing mode' which means I might talk strangely as if I were writing a paper! :

My biggest issue with the depression is is actually comparable to what you said in regards to your fibromyalgia.  Like you, sometimes (heck, most of the times!) I (and my doctors) can't tell exactly where the depression is coming from.  Is it coming from too much fatigue, which is depressing my body and mind in general or is it coming from chemical imbalances not necessarily caused by the thalassemia or is it coming from both?

Treating depression that is stemming from naturally occurring 'depression' with meds won't help while leaving chemical imbalances untreated won't help either.

That's why I did a trial transfusion run, but like you said, perhaps it wasn't enough to make a difference (yet) because my body is simply used to dealing with what it 'normally' has.

Of course, having a low Hg level more or less equates to having a lower energy level and thus equates to an overall 'depressed' state in the sense that the body can't do as much or is pushed harder to do what might be considered to be 'normal' levels of work.

But yeah... because of this, I've definitely been seriously thinking about transfusion therapy.

I DO get support from my family, still, thank goodness, but I feel bad about not being independent yet and being caught between graduating from university and getting a job and working.

I don't have insurance anymore, but I have looked into disability insurance and special needs insurance available in California.

I go to the University of California - Berkeley, and my school was AWESOME about helping me get the care that I needed and I was indeed in a special program at my school that allowed me to take classes more at my own pace to make room for my frequent illnesses and such.

I'm grateful for the help they gave me and now that I am without it, I realize just how difficult it is to get necessary treatment and help without the proper authorities to back you.

Health insurance is definitely a big deal and it is the biggest factor holding me back from any current treatment at the moment, but hopefully, that will change soon!

Thanks so much for the response!