Saving Kobe

“I’m late”

“No, I’m late”

Denial

• Home test x 4 (+)– Expired– Ovarian tumor

• New home test (+)• Blood (+)• Ultrasound

• Denial plays a big role

The situation

• Parents over 40• 3 kids (15,14,10)• Busy life

• Reality show

Thoughts

• How could this happen• Finding time for a baby• Why is everything my

fault• Mother’s health• Baby’s health– Chromosomal– Down’s syndrome

Statistics

• There is a 1 in 30 chance the baby will have a chromosomal abnormality

• The baby will have a chromosomal abnormality

The pregnancy

• No problems• Pitting edema– My fault

• Screening tests• Amnio

Kobe James Bixler

4 week check

• Yellow skin?• Yellow eyes?• Yellow tears

We have a problem

• Labs– Total bili 9.5– Direct bili 5.4

• Ultrasound– Liver normal– Spleen normal– GB functioning

• “He doesn’t have biliary atresia!”

Oh thank God

What the heck is biliary atresia

Assume that the I know nothing about medicine

Even if I am a healthcare professional

Diagnosis

• Hopkins (CHOP)• Traffic • Finding building• Parking• Finding clinic• Waiting room

Please understand that I am stressed out

I might do or say things that I would never otherwise do or say

Neonatal Hepatitis

• Idiopathic hepatitis• Hepatitis A• Hepatitis B• EBV• CMV• Enterovirus

• Alpha-1-antitrypsin deficiency

• Wilson disease• IEM• Cystic Fibrosis• Biliary atresia

Diagnosis

• More labs• HIDA scan• Next week• Your baby is yellow

If there is any chance that something is wrong, please find

out ASAP

Diagnosis

• Phone calls– Ped surgeons– Transplant surgeons

• BA – Kasai procedure• Liver transplant• Practice makes perfect– Shouldice clinic– Kasai procedure

• Go to CHOP

I want the best for my baby. Please send me to the doctor where you would take your

baby.

Try not to let outside forces influence your decision

Children’s Hospital of Philadelphia

• Called Saturday• Hours • Fellow on call– I’m busy call back later

• ER Sunday morning

My baby is sick and I don’t know why.

Why won’t anybody help me

Children’s Hospital of Philadelphia

• Traffic• Parking• H1N1• 2 hours• ER 8 hours• “your baby is really

yellow”

Help us

• 5 days• 3 hospitals• Severe distress• No diagnosis• Admitted by Peds

resident• Yellow baby

What can you possibly be doing that is so important that you

can’t at least pick up the phone and let me know you care

Do what ever you can to let the family know that they are being

cared for

Someone help me

“I really dig Hannibal. He had real guts. He rode elephants into

Cartilage”

“I guess I’m going to fade into Bolivia”

“Everybody has a plan until they get hit in the mouth”

If it important enough to test, itis important enough to let me

know the results

NOW

The camp

• Don’t mess with mamma bear

• Studies– labs

• Rounds• Thought• Sleep deprivation• Decision making

Diagnosis weekneonatal hepatitis

• More labs• Ultrasound • HIDA scan– technician

• Liver biopsy– Severe cirrhosis

Biliary atresia

• 1 in 15,000 births• Progressive obliteration

of bile duct system• Cause unknown• Liver failure • Death • Kasai procedure

The surgeon

• Met for 3 min• Surgery plan• Was once a transplant

surgeon• Hung crepe• No Q and A time• Experienced Kasai

surgeon

Dr. Morio Kasai1922-2008

• Pediatric Surgeon• Fellow at CHOP• Dr. C. Everett Koop• Kasai procedure• A chance• A bridge

Kasai results

• Best before 6 weeks• Rule of thirds• 80% liver transplant• Malabsorption• Portal hypertension

Pre-op

• Extreme tension• Surgeon (1 min)• Plan• Cholangiogram• Kasai • Staff

I can hear you.I feel like I’m going to be sick.

I don’t want to hear about your tequila shots

Surgical waiting room

• No word after cholangiogram

• 3 hours – doing kasai• 8 hours– Its done– Liver was a rock– We will see– Gone in a flash

This is the most important day of our life.

Please let me know how surgery is going.

Base camp

• Ilius• Cried for days• Mom would not leave• Stress!!!!!!• Eyes cleared• Liver labs improved• Going home

Last night

• 2.5 weeks• Uncertainity• Surgery• Fear, stress,

helplessness• Sleep deprivation• “High K incident”

This has been very hard for me.Please use common sense and

respect me just as I respect you.

I am still a momma bear

December• Fever• Temp 102• Screaming• Lethargy• Ascending cholangitis

Ascending cholangitis

• Iv abx• Fever gone• Go home• 2 days• $18,000 dollars for

flight

Bounced back

• Fever returned• Liver enzymes high• Ascending cholangitis• ER – same story• Fellow at desk• Ultrasound• PICC line

How are we now?

• 2nd birthday• No cholangitis• Bili normal• Liver enzymes normal• Thriving • 80 % height and weight

Biliary atresia

• Never goes away• Intrahapatic?• Kasai is temporary• Cholangitis• Liver– Portal hypertension– Esophageal varicies– Failure

• Transplant

Domestic Violence Syndrome

Summary

• No one is perfect• Try as hard as you can

to be perfect• Having a sick kid is no

fun• Life is never the same• Ask these “parent

questions” yourself