Dad is on his way home :]

Dad is being released from the hospital today! Hooray!

The doctors have diagnosed him with acute intermittent porphyria. According to information from Mom, porphyria is characterized by a defect in the hematin processing pathway causing an increased number of porphyrins to travel through the body, and Dad is missing the enzymes that break down porphyrins. Porphyrins effect the neurological system, and for him, specifically, the nerves in the abdomen. He has, unknowingly, has had this all his life, and it was probably triggered recently for several reasons.

Things that trigger an attack are:

- infections (Dad has had an extreme, continuous sinus infection since December)

- certain medications (doctors found that the blood medication he has been taking for 20+ years may have contributed to the severity of his reaction, specifically the flushing)

- not enough carbohydrates (glucose) in your diet

Therefore, an increased amount of glucose will help to suppress the “attack” from occurring by blocking porphyrins from coursing through the body. He will have to modify his diet to consume 400g of carbohydrates a day, which means he’ll have to eat lots of pasta, cereal, and pizza (and pizza is his favorite – poor guy!). Additionally, the doctors took him off of Zestril and he is now taking another blood pressure medication.

Over the past few days he has been on a different blood pressure medication and has had a glucose IV drip. Thankfully with these changes he hasn’t had an attack (no flushing or extreme pain) since. The doctors think that his acute stage is now over!

He’s being sent home today with Delotted (or Delautid…not sure how you spell it) to be taken orally. The doctors do not anticipate other attacks, but if he has another one, he will have to go back to the hospital to receive medicine to relieve the pain.

The website below was given to my mom by the doctors and has some good information on porphyria if you want to learn more:

www.uq.edu.au/porphyria

I’ll try to keep you guys posted if I hear of any more updates.

GOOD NEWS!

Dad's being released today (4.12)! I just got a phone call from my mom (3:30 pm) and she said that within the past half hour, they have told her that it is Porphyria and have explained to her the cause and steps need to be taken to help this. Abbie will be updating the blog in a few hours when she gets off work so she'll have the full scoop for everyone then.

-Grant

4.10 Montefiore Tuesday

Dad had an attack at 6am this morning. He was given 1 unit of both Dilaudid and Zofran via IV. Dad had some relief but the pain came back. He was then given one unit orally and one unit in the IV of Dilaudid. He finally had relief around 8.30.

The test they used isn't very accurate. So this 24 hour urine collection that they are doing now will be the Dr. Roberts visited and said Porphyria is rare, only 1 is 100 thousand people has it. The screeningdefinitive test as to whether he has it or not. He also said it could still be the Ischimia. If this urine test comes back negative for the Porphyria, he will push for an angiogram. There is still a .1% chance that it could be a rare Carcinoid tumor.

Comments and Prayers

I just wanted to encourage people to comment. Dad would love to hear from anyone and everyone. This is a way for you to do that! I've set the comments up so that you can comment anonymously. You can also email me directly if you want to send on a personal message without everyone else reading it.

Dad loves sarcasm and paying people out! So let it rip people! :) We'll make sure he gets the message.

Also, we would love prayers of support. I would be lying if I said this is super easy to be going through. Please pray for the doctors for help in figuring out what is happening and why. That they may be through with their diagnosis. And for my parents for strength to continue through all of this craziness. There are so many people praying for us and it gives us lots of hope that this will be resolved in God's time.

-Paige

4.9 Montefiore Monday

I'm finally up to date with the posting. Whew.

One of the things I forgot to mention about yesterday's update is that Dad thinks the sinus infection is returning. He is having more headaches in his sinus'. Also, they were considering that he may be suffering from migraines. Still trying to figure it all out.

Today was another bad day starting at 7am. They gave Dad Imitrex, Zofran and Tylenol at first. Imitrex for the possible migraine diagnosis, Zofran for the nausea. Talking with Mom today I found out that Dad has been having a headache with the pain and nausea. The Tylenol was for that.

Mom called at 7.30am and didn't initially get through. At 7.45am when she was walking into the Cathedral of Learning Dad called her back and told her that he was in pain. She was able to make it over to the hospital by 8.30am.

9.00am they gave Dad one unit of Dilaudid through his IV. That took the pain down to a 1 of 10, but the pain was still nagging and never went away. The nurses attempted 3 oral doses of Dilaudid, which did nothing to help. It seems that nothing orally is working for him once he get to a certain threshold.

Finally at 1.00pm they gave him another unit of Dilaudid, by 1.15pm the pain was gone. It only took over 6 hours to get relief.

More news. A Hematologist came in today. They tested the urine sample that sat in the fridge for a few days and it came back positive for Porphyria, a blood disorder. This is a potential diagnosis. Some highlights from the link:

"The acute porphyrias affect the nervous system. Symptoms of acute porphyria include pain in the chest, abdomen, limbs, or back; muscle numbness, tingling, paralysis, or cramping; vomiting; constipation; and personality changes or mental disorders. These symptoms appear intermittently."

"Porphyria can be triggered by drugs (barbiturates, tranquilizers, birth control pills, sedatives), chemicals, fasting, smoking, drinking alcohol, infections, emotional and physical stress, menstrual hormones, and exposure to the sun."

They started a fourth 24 hour urine collection. Dad did one over the weekend, since the second one was forgotten in the refrigerator. We'll see if this shows for sure. I'm pretty sure they will do other tests and things. It makes some sense with Dad having that sinus infection for close to 4 months. And some of the symptoms agree with what he is suffering from.

On a TOTAL side note. I was watching Law & Order: Special Victims Unit on USA. One of the characters has Porphyria. Way strange that I would watch that show today, the day that the doctors think my Dad my have this diagnosis.

Any questions or comments, comment by clicking on the comment button. I've opened it so that anyone can make comments without signing up for blogger.

-Paige

4.8 Montefiore Easter Sunday

Surprisingly illnesses don't know when holidays are. Kinda not fair.

Dad called Mom at 7am and asked her to come in. He woke up at 3am having an attack. He had another one around7am and a third around 10.30am. When I spoke to Mom today she said Dad was pretty drugged up with the Dilaudid.

On the good side, the flushing didn't happen and the pain was around a 4 instead of the usual 8-10. So that is a good thing. Small victories.

Abbie and Eatai were in all weekend and spent a good deal of time with Dad. Also he had some visits from family members. I would recomend that if you want to go in a visit him, to call ahead of time. If he's had an attack and was given Dilaudid, he may be super out of it. But I know he really enjoys the visits and calls and all of that. :)

-Paige

4.7 Montefoire Saturday

No attacks today.

New possible diagnosis: Systemic Mastocytosis.

A ray of hope?
Dr. Rao, an endocrinologist specialist, visited. He thinks this may be a reaction to Zestril the blood pressure medicine that Dad has been on for about 20 years.
He took Dad off the Zestril tonight. We'll just have to wait and see.

-Paige