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

4.6 Montefiore Friday

dad had half a bowl of cereal in the morning. Mom called Dad at 8.20. The doctor was in talked to Dad. At that moment Dad was fine. Once he hung up the phone he started getting sick. By 8.30am an attack started and at 9am Dad called Mom to come in.

Today was a bit of trail and error. Dr. Harris, his endocrinologist, had some ideas and asked if they could try them.
They tried: Nitroglycerin orally- Nothing
Zofran and Fentanyl Lollipop orally- Nothing
They took blood at this time.
Antihistamine/Allegra- Nothing
Zantac- Nothing
Then they decided to go back to the Zofran and Dilaudid.
45 minutes passed and no meds.
Dr. Jovin, the attending physician came in:
1 unit of Dilaudid by IV- Nothing
30 min later: Zofran by IV- Nothing
5mg of Dilaudid orally- Nothing
12.30:2 more units of Dilaudid- Finally he had relief!


In the evening a team of endocrinologist paid Dad a visit.

-Paige

4.5 Montefiore Thurday

No attacks this day!! :)

Dad had a full body scan, something called an Octreotide Scan to determine if he has Carcinoid tumors. They had him take a hormone radio isotope and then have a full body scan.

In the afternoon he was seen by an Endocrinologist, Dr. Harris.

For lunch Dad had a small amount of mushroom soup. He was able to have some candy: a few M&M's, Clark Bar, and some Twizzlers. And for dinner, a small amount of a pot roast sandwich, and some mashed potatoes.

-Paige

4.4 Montefiore Wednesday

Another bad day. An attack starting at 5am. They had tried the Zofran pill again and the Fentanyl Lollipop, but it didn't help. Dad asked for the two shots, but they were in his chart, so the nurses had to call the doctor and then the pharmacy to get it. Finally he was given the first round of Zofran and Dilaudid shots which took away the nausea. He called Mom at 7.30am while she was on her way to work and asked her to come into the hospital instead. At 8am Dad received another Dilaudid which took away the rest of the pain.

And then another one at 11am before he was to go down for the CT Enterography that didn't happen the day before.
At 11.45 the pain was at a 8 on a 1 to 10 point scale.
At noon the pain was at a 9.
12.10 they gave him the shots in the arm of Zofran and 2 units of Dilaudid.
That took away the pain and they sent him down for the CT.

This day the doctors had him on a liquids only diet: water, coffee, soup broth.

-Paige

4.3 Montefiore Tuesday

Last night the Doctor said he doesn't want Dad to be eating anything. Maybe this is food related? Also he didn't want Dad to eat because he had a colonoscopy today. There was the expectation of red rash like blood vessels, which would show the Ischemia (I think). They say some inflammation and took multiple biopsies. Everything from those biopsies has come back normal.

They were also supposed to re-do the CT Enterography, but no one ever showed up to take him down.

Around 7.30pm Dad ate some mushroom flatbread from the hospital cafe.

Mom noticed that Dad's face was flushed at 8pm. At 8.10pm Dad complained of a headache in his right side of sinus.

-Paige

The Symptoms

I realized that I haven't really explained what Dad is feeling when he is having one of these attacks. Usually it is sometime after he eats.

First he'll have a bowel movement, not diarrhea, but substantial I understand. Then a warm flushing starts in his back and progresses quickly up his body to his head. Quickly nausea and pain follow. Dad feels like he needs to throw up to get relief from it all. Often he'll make himself throw up. You know when you are sick and your head gets all woozy and loose. That happens too. The pain is the biggest thing from my understanding. And it's not just a little pain. My Aunt Peggy explained it best, it's like he's writhing in pain. There is nothing that he can do to find relief from it.

It seems that the shots of Dilaudid and Zofran work the best. He's been needing various doses of the Dilaudid to get relief. Sometimes one shot or one unit in his IV works. Sometimes he'll need up to 3 or more to have relief from the pain.

Just thought you all should know what it was that we were talking about with the attacks and pain and all.

-Paige

4.2 Info from Dr.Roberts visit

This is from an email that I sent to Mom on Tuesday.

Dr. Roberts came in yesterday and said that he believes that Dad has either a Carcinoid Tumor or Ischemia of the Bowel.

The 24 hour urine test should show if there is a carcinoid tumor. The Doctor said that if it is the carcinoid, the reason that Dad gets sick every time he eats (which usually isn't the case with carcinoid) is maybe the tumor is around or surrounded by muscle which squeezes the tumor when the muscle is working on Dad's food, which then causes the carcinoid to secrete hormones, and that causes Dad to get sick. The test results should be back in a few days since they had to send it somewhere to get tested. Now the tumors are slow growing and small. Dr. Roberts said that he has been involved in cases where they have diagnosed carcinoid tumors but hadn't been able to find them for a while, until they grew larger to see. He said that is pretty rare, but to be aware of it. It could be one tumor or a few they aren't sure. And since they are slow growing and small that would account for the carcinoid not being seen in the CT scans that Dad has had done.

Speaking of CT Scan, the one that he had done ion the 26th wasn't done to the specification that Dr. Roberts ordered. He seemed pretty angry about this, but didn't seem as though he wanted Dad to do it over again. They didn't do some of the cross sections that he had ordered.

They have been taking blood from an artery when Dad has these episodes and they've found an increase of lactic acid and white blood cells. Lactic acid is produced when a muscle isn't getting enough oxygen. That would lead them to go with the escarpic bowel, but it's not a very high amount of lactic acid. Escarpic bowel is when the blood vessels to a certain part of the bowel aren't supplying it correctly. An angoigram would give them a better idea if this is the cause of Dad's episodes. Dr. Roberts seemed to want to have done this from the beginning, but the team and radiology have been against it as an initial test because of the invasive nature of the test. Dr. Roberts said to Dad yesterday that he is pushing for them to do the angoigram even before they get the results from the urine test. But then another doctor came up a few hours later and said they aren't going to do it yet. If it is this escarpic bowel, that doesn't account for the flushing and sweating that Dad keeps having. The treatment for the escarpic bowel is to remove that section of bowel.

Dr. Roberts said that they aren't 100% sure that either one of these are what Dad is suffering from. And if he had to pick he would want it to be the escarpic bowel, and not the carcinoid tumor.

-Paige

4.2 Montefiore Monday

A bit of a rough day. Dad had 2 attacks. The first day that he's had more than one attack in a day. One was around 8am to maybe 10am. It was comforting to him and the family that my Aunt Peggy was there. She's a nurse that trains people diagnosed with diabetes how to handle their treatment, I think. She had stopped in to see how he was doing since her husband, Vince, was in the hospital to have his gallbladder removed. (He's doing fine.) She told my Dad that he was in good hands with his nurses.

In the first attack they tried different ways to combat the pain and nausea. He was given a prescription earlier the week before for a suppository to help with the nausea (I think). That didn't work. They ended up giving him the 2 shots, Dilaudid for the pain and Zofran for the nausea. They also took blood from an artery in his arm, around his wrist.

I arrived around 11:30 and he promptly had another attack. They tried a Fentanyl Lollipop and an Zofran pill. It took about 40 min, but finally the pain went away.

Dr. Roberts, Dad's PCP, came in after all this and discusses what may be going on. They are also looking into not only the Ischemia but also the possibility of Carcinoid Tumors. The next web entry is from an email I sent to Mom about the Doctor's visit.

-Paige

3.30 Montefiore Friday

In the hospital, no "attacks" this day. He did another 24 hour urine collection since the first one was lost. *We later find out they had this one in the fridge 5 days before sending it out to be tested...grrr*

-Paige

3.29 Thursday

And again to the hospital not 15 hours after he came back from the night before. Dad had some of a Meat Lovers personal pan pizza for lunch. Around 3 he started feeling bad with throwing up and pain. It subsided a little. Then came back with full force around 4ish. I took him to the hospital at 4:30pm. Mom met us there.

Dad was admitted to Montefiore, a spill over for Presby. He's in 8 North room 882 bed 2. I'm sure he would love a visit.

-Paige

3.28 Wednesday

Wednesday evening, I made dinner. It was turkey, stuffing and spinach. Pretty much within the hour Dad started feeling poorly. Instead of waiting until the pain was full force, Mom took Dad into Presby right away. They waited in the waiting room for an hour before being seen. Dad got the 2 shots and was discharged. They got home around 1 or 2 am.

-Paige

3.26 Monday

Dad went back to Dr. Roberts on Monday to start some of the testing that Dr. Roberts wanted done. He dropped of the 24 hour urine collection. The CT Enterography happened. And they were supposed to do a blood draw, but the paperwork wasn't there.

A few weeks later we find out they lost the urine collection and whoever did the CT Enterography, didn't do the test correctly.

-Paige

3.24 Saturday

On Saturday the 24th, Mom and Dad went to my cousin Karl's final tamburitzans concert. There was a banquet type of thing afterwards and Dad had eaten a hot dog, fried chicken, haluski and then later in the evening he had some Popcorn.

9pm the pain and nausea started again. They went into the hospital around midnight and once the pain was under controll he was discharged.

-Paige

3.21-3.23

3.21 Dad went to his Ear/Nose/Throat doctor and learned that his sinus infection that had been hounding him since December is cleared up! Great news! No more Antibiotics.

3.22 Mom and Dad went to his primary care physician, Dr. Roberts, to find out what is going on. Dr. Roberts wants a few tests done to check for Ischemia. He wants a CT Enterography, Colonoscopy, Angiogram, blood work and a 24hour urine collection.

One of the things they think Dad may have is Mesenteric Ischemia.

3.23 Dad calls Dr. Roberts on Friday to set up tests for the following Monday.

-Paige

3.20

Dad called Mom at work before lunch to take him to the ER again. He had gone to McDonald's and gotten a Sausage/Egg/Cheese Bagel. Paige was out doing job search stuff so she couldn't drive him. Mom came out to Shaler and picked him up, then drove back to Oakland to take him again to Presby. They have him the 2 shots and sent Dad home after a while. Before they sent him home they did a chest X-ray and a different CT scan of his lower abdomen checking the blood vessels to see if something was constricting the bowel. Everything came out normal.

No hospital stay this time.

-Paige

3.13 - 3.16

Tuesday the 13th was a super beautiful day, temp wise in the 70's and lots of sun! Mom came home and made steak, mushrooms, noodles and spinach for dinner.

Around 11pm I was sitting on the couch doing some stuff for the kids at work since the 14th was my last day. Dad came downstairs and asked me to move so that he could sleep on the couch. He said that he had thrown up a few times and didn't want to be upstairs with Mom, in case he woke her. He got sick a few more times. Around 12am he fell asleep and I went to bed.

I guess at 1am, Dad woke up Mom to take him to the hospital again. They got there at 2am. He was admitted after they took care of the pain and he was sent to Magee.

Yes Magee Womens Hospital. They are an overflow for Presby. They wanted to check his gallbladder again. He got to have an ultrasound. I don't know too many men (read:none) who have had an ultrasound at Magee. He also had an endoscopy, stomach emptying test and a scope. All came out good.

On Friday they sent him home...again.

-Paige

Starting from the begining....March 8th through the 11th

So when I was growing up, Mom loved The Sound of Music and would sing songs from it. Right now as I'm about to try to explain the past month of hospital visits and all the craziness that has been going on, one of the songs pops into my head..."Let's start at the very beginning/the very best place to start...."

Starting in December, my Dad had a sinus infection that refused to go away. They tried lots of different antibiotics, different nose sprays, he had been using a neti pot for the past few years and they were trying different things to put in the water. Nothing was working. So he's been feeling sick for a while now.

Also for the previous few months he had been loosing his appetite for sweets (!) and other foods. A few rare times right after eating he would feel like he needed to throw up. Sometime he would and sometimes he wouldn't. Then he would feel better.

On the 8th of March Mom and Dad went out to eat at a Italian restaurant. Dad had shrimp. Around 9pm he started feeling really bad. He was vomiting and had pain in his stomach. They figured that he maybe had a slight case of food poisoning. Around 1am it went away and he went to sleep.

The next morning, when Dad awoke, he started having the pain in his abdomen all over again. Mom took him to the Presby ER. The reason that they went to Presby was beacause that is where his Ear/Nose/Throat doctors are and his PCP.

In the ER they gave him Dilaudid for the pain and Zofran for the nausea. This helped take the pain and nausea away. In the ER they performed a CT Scan to check for Gallstones. They were pretty sure that was what the problem was, but they kept him overnight in Presby because he had complained of some tightness in his chest.

Saturday the 10th he had a chemical stress test (beacuse of his Muscular Distrophy they couldn't do the treadmill type) and then sent him home. The stress test came back ok.

We went to see 300 with a few of my friends that Sunday. The movie rocked, in case anyone is wondering.

-Paige