Living with PV for 47 years after father diagnosed days earlier.

In 1968-9 my father was diagnosed with PV and Spherocytosis by VA. His doctors suggested his direct family members also be test to see if disorders were hereditary. Out of 6 people, his mother, older brother, older sister, oldest daughter, son, and youngest daughter-me I was found to also have the same disorders. My poor Mom had the two if us undergoing splenectomies within days of each other at two hospitals blocks apart. After we were released my Dad underwent scheduled phlebotomies. I on the other hand visited my hemotologist at Univ of Illnois Hospital in Chicago for a few years for observation. My Dad died in 1977 of unrelated arterioschlerosis since he smoked for many years. My Mom died 10 years later of same issues, also a smoker.

In 1992 I began my history of PEs. Doctors initially contributed my first PE in 1992 from driving to Colorado from Chicago in a little over a day. I was prescribed Coumadin for 6 months. In 1993 after my 2nd PE my local doctors became cautious and verified I had PV/Spherocytosis. I started my life prescription of Coumadin. In 1997 I encountered my 3rd PE and was assigned a new hematologist who performed my second bone marrow test to verify Polycythemia Vera/Spherosytosis. I began having regularly scheduled phlebotomies. After several phlebotomies my hematologist decided it was best to stop phlebotomies since my body was fighting to keep my cell production at the level. I began using Hydroxyurea/Hydrea. I am still using this drug today. I was sent for a second opinion to Rush Medical Center and saw Dr. Stephanie Gregory who once again verified my having PT/Spherocytosis. She introduced me to a genetic specialist that ran a series of test over a couple months and he also verified my conditions. After a couple a years my Hematologist prescribed Anagrelide to my daily regime since I was not feeling well. I still am taking this drug and seeing the same Hematologist.

In 1997 I was also introduced to a Pulmonologist so to summarize his findings from 1997-present here it goes:

1. Because of my PE issues it was suggested that I receive a Greenfield Filter since results showed no exact point to which my PEs began. At lease with the filter the chance of having PEs from my lower extremities would be covered.

2. I was diagnosised with asthma. Still prescribed Pulmicort, Serevent and albuterol.

3. I have been diagnosed with Sleep Apnea for several years so therefore use of a CPAP is in my daily routine.

4. My oxygen level was low so for several months I used O2 throughout the day.

5. In the last couple years I began having further breathing issues and chest pains.

6. I was referred to see an expert Cardiologist who verified my Pulmonologist's assumption of Pulmonary Aterial Hypertension, PAH. He determined best drugs for myself, Sildenafil and Toprol XL and then recommended I see a nationally known PAH expert at the Univ of Chicago. I still visit him on a regular basis.

My PV is fine and steady but the side effects related to my previous PEs are my issues. I am currently very fatigued and continuously have pain in my lower back and hips. I keep on moving because I must. Over the years I have also trained myself what not to do to irritate my PAH. Once you determine your limits on distance and incline everything is good and you can go on with life!

[piggie50]

Hi penguinlover,

You really have gone through the mill! I'm sorry but I don't know what PE is? It's nice to hear from someone who has been taking Hydroxy for a great number of years. I think we'll worry about long term medication. I also agree with you, determine tour limits and get on with life.

Best wishes

Judy

[Stevesmum42]

My goodness, you have had more than your share of problems, but appear to have good medical care. We do have to know our capabilities regarding our health issues.

I wish you well.

Regards Sandy

[Mazcd Partner MPNVoice]

Hello Penguinlover, welcome to our forum, and thank you for sharing with us all your family history, it seems that you and your family have been through quite a lot. I hope you find this forum is of help, the people on here are very helpful and supportive, if you need any printed information about PV and Hydroxy please email me at [e-mail] with your postal address. Best wishes, Maz

[wormwood]

Gordon Bennet, you have been through it, keep going!!!

You sound like a fighter, I sometimes think a positive mental attitude is needed to cope with all that we suffer with an MPN !!!

Take care x

[blulou]

Genetics? ANK1? As one who ate hydroxy for decades, I do commiserate. I do believe most of us are JAK2+, but you are definitely in the family here. Thanks for sharing the story and stay strong. (Note: the JAK1 and JAK2 mutations are not [knock wood] hereditary.)

[mol56]

Hi.

My nephew was diagnosed with sphyerocytosis when he was 8. He had his spleen removed and has been healthy since and is a fine strapping lad of 40.

Unfortunately, two of his children have inherited the blood disease (now aged 6 and 4) but his last child has not.

Neither my sister or my brother-in-law nor my other nephews and nieces or my own children have it.

We believe it started with him.

I was diagnosed with Essential Thrombocythaemia 10 years ago, have been on Hydroxycarbamide for 10 years. I asked a consultant about the fact that my nephew has spherocytosis but he said that there was no connection.

My great niece and nephew are always very pale but otherwise seem healthy. They may have to have their spleens removed later on.

Wish you well

Judith

[Hidden in reply to mol56]

Judith, Thank you for letting me know that your grand niece & nephew were diagnosed with Spherocytosis at an early age. I always contributed my PV/Spherocytosis diagnosis to my Dad's great doctors. It looks like you also had great doctors. My view on life is I rather know now than later since it helped my doctors quickly diagnose my Emboli. I wish you and your family the best.

[tracey13]

HI penguin lover,

You certainly have been through the mill with your health issues!!

They say pv isn't hereditary, but I don't believe it.

My husband has had pv now for 2 years his father suffers high blood pressure itchy legs short of breath. He's asked his gp if he needs testing and he said no!!

It's quite scary to think your father had pv and you have got pv.

May this needs to be brought to light. It's a blood test that determines the pv so it's nothing complicated!

This site has helped myself and husband so much.

[Hidden]

I have heard the same. Here is a link from the (US) National Library of Medicine that may answer your question. ghr.nlm.nih.gov/condition/p...

I hope it helps. Teresa